All posts by Natty Jumreornvong

Global HD: Huntington’s Disease in Peru

Author: Amanda Szerdi

The National Institute of Neurological Sciences, located in Lima, is the primary medical facility in Peru that can provide patients with a definitive Huntington’s Disease diagnosis. In June of 2011, Peru passed La Ley de Enfermedades Huérfanas or the “Rare and Orphan Illness Act.” This law provides funding for additional medical treatment and awareness initiatives for individuals affected by diseases, such as Huntington’s, that result in death or chronic disability. Federal programs and the Seguro Integral de Salud (SIS) cover the costs of appointments, transportation to clinics, treatments, and hospitalizations. Additionally, the Ministry of Health established the last day in February of each year as a “Dia Nacional de Enfermedades Huérfanas o Raras en el Peru” or a “National Rare Diseases Day in Peru.” These policies have helped to raise awareness of Huntington’s disease in Peru while striving to provide better care for patients.

Important researchers:

Miriam Velez

Dr. Miriam Velez works at the National Institute of Neurological Sciences in Lima. She is one of the primary neurologists on staff who specializes in treating Huntington’s disease and neurodegenerative disorders. She, in addition to Dr. Carlos Consentino, are doing their best to offer genetic testing as well as education about Huntington’s disease to HD patients and their family members.

Pilar Mazzetti:

Dr. Pilar Mazzetti became interested in neurology while studying to become a medical doctor at the National University of San Marcos. She has served as the Minister of Health and the Minister of the Interior in Peru. She currently works at the National Institute of Neurological Sciences in Lima, the primary facility in Peru where Huntington’s disease is diagnosed and treated. Dr. Mazzetti has worked to introduce and develop the practice of genetic testing for neurological diseases in Peru. Relatively little research had been conducted in Peru in relation to genetic diseases, and researchers such as Dr. Mazzetti face many challenges while completing their work. Research typically does not receive sufficient funding and must be completed by doctors after caring for their regular patients at the clinic. Dr. Mazzetti’s recent work has focused on the psychiatric symptoms of HD patients in Peru as well as the genetic origins of HD in Peruvian populations.

Mario Cornejo

Dr. Cornejo is the director of neurogenetics at the National Institute of Neurological Sciences in Lima. He has studied neurology and genetics in both Peru and at the University of Washington, located in Seattle. Dr. Cornejo has worked on neurogenetic studies for a variety of neurodegenerative diseases including Huntington’s Disease. He has worked with Dr. Pilar Mazzetti researching the genetic origins of Huntington’s Disease in Peruvian populations.

Source: National Institute of Neurological Sciences website < http://incngen.org.pe/>

 

Resources:

Web pages with information regarding health insurance and care provided by the National Institute of Neurological Sciences can be found below:

National register for individuals with rare diseases:

Registro Peruano de enfermedades raras y huerfanas: <http://www.minsa.gob.pe/erh/normas.html>

Peruvian Ministry of Health:

Ministerio de Salud de Peru, pagina de seguros medicos: <http://www.sis.gob.pe/nuevoPortal/>

National Institute for the Neurological Sciences website:

Sitio web para el Instituto Nacional de Ciencias Neurologicas:

<http://incngen.org.pe/>

 

Acknowledgements:

A huge thanks goes out to Doctor Miriam Velez for her help in acquiring additional information regarding how patients in Canete, Peru and surrounding areas receive care for Huntington’s Disease. While living in Peru during the summer, I contacted Dr. Velez as a HOPES student researcher. She agreed to meet with me in person at the National Institute of Neurological Sciences in Lima so that I could better understand treatment options for Huntington’s Disease patients in Peru. The hospital where patients with neurodegenerative diseases are treated is not far from popular sites such as the Monastery of San Francisco and the historical district of Lima. I was accompanied by a close friend, Cindy Maximiliano, who also lives in Lima. Her help was also essential in locating the hospital and interviewing Dr. Velez.

We were extremely fortunate to briefly visit with Dr. Velez between patient visits. She described to us how her interest in neurological disorders developed as she began to study medicine. She also informed us that the Instituto Nacional de Ciencias Neurologicas is the only clinic in all of Peru that can provide the necessary genetic tests to provide a formal Huntington’s diagnosis to any individual. Similarly, she described to us the different national health insurance programs that provide transportation to the clinic and pay for the healthcare of those with Huntington’s Disease in Peru. While many policies have been developed to improve care for patients, receiving funds is often a long process that takes time. Furthermore, loosely enforced medical regulations can make it difficult to standardize care. The visit was one of the most impactful experiences during my time in Peru.

More

Role of Drama Therapy in HD Patients

Drama therapy has been used as a form of psychotherapy for those who view the more conventional “talking therapies” as less beneficial. Such groups have included people with dementia and long-standing schizophrenic illnesses. Dementia refers to neurodegeneration that results in loss of mental abilities. While Huntington’s disease (HD) is commonly thought of as a motor disorder, cognitive symptoms can be present, which can be progress to dementia. In addition, paranoid schizophrenia-like symptoms occur in 6% to 25% of HD cases.

Why Drama Therapy?

The reasoning for the use of drama therapy in HD patients is that as an action-oriented therapy, it has helpful features not present in the purely talking therapies. For HD patients, thoughts and emotions may be disordered and hard to contain. Thus, conventional psychotherapy can be problematic because it presumes at least some ability to self-regulate thoughts and feelings and to undertake basic reality checking. According to a theory (Bielańska, Cechnicki, & Budzyna-Dawidowski, 1991), the more formal structure of drama therapy and its separation between the world of the drama and the world of the group, may offer a degree of environmental regulation.

Another concept is that of aesthetic distancing (Jones, 1991). Here, the ‘make-believe’ element of the drama allows participants to work with material, which is sensitive to them. Such sensitive issues could relate to murderous or persecutory fantasies that might be so alarming that a patient would chose to deny or minimize them, rather than talk them through in therapy. The distancing effect of drama by playing the fantasy out in a metaphorical or symbolic way allows the material to be worked with, with an underlying safety net that this is, after all, ‘only a story.”

Approaches to Drama Therapy

Drama therapy has a developmental model, with increasing demands being placed on participants with greater resilience, insight and interpersonal abilities. A creative-expressive approach to drama therapy may consist of activities such as drama games and improvisational exercises to encourage spontaneity and creativity within a safe framework (Jennings 1990). No exploration of the images and material created is necessary.

Another approach to drama therapy encourages participants to recognize their own projects in the work they produce, or modify and experiment with the roles they play (Jenkyns 1996). Existing texts may be used to allow participants to explore roles that are new or challenging to them.

Finally, a third approach to drama therapy uses the archetypal myths, fairy tales and folk tales from various cultures to allow participants to engage with these socially evolved stories with dark topics and undergo emotional growth. (Bettelheim 1976).

Case Studies

There has been no research to date on the role of drama therapy on HD patients. However, case studies on the role of drama therapy on patients with complex neurological disabilities and psychiatric illnesses suggest the potential it has on improving the wellbeing of HD patients.

For example, a study on interactive drama in complex neurological disability management suggests that the majority of participants were able to engage with interactive drama therapy and that it offered residents opportunities for enjoyment achievement, challenge and experiencing meaningful experiences (Fenech, 2009).

In addition, a study analyzed five randomized controlled trials that compared drama therapy, psychodrama and related approaches with standard care or other psychosocial interventions for patients showing psychiatric schizophrenic like symptoms (Ruddy & Dent-Brown, 2007). One study had drama therapy as the intervention, one had role-playing, one had a social drama group and two used psychodrama. Two of the included studies were Chinese and it is difficult to know whether psychodrama and inpatient psychiatric care in China is comparable with the drama interventions and inpatient care in other studies.

Resources

North American Drama Therapy Association was incorporated in 1979 to establish and uphold rigorous standards of professional competence for drama therapists. The organization maintains requirements that must be met to qualify as a registered drama therapist. It also promotes drama therapy through information and advocacy. For more information, please check out its website: http://www.nadta.org/

The Living Arts Counseling Center provides high quality psychotherapy services provided by interns, trainees and fully licensed therapists on affordable basis. Its focus is on drama and expressive arts therapy. It is affiliated with Living Arts Playback Theater and Healing the Wounds of History. For more information, please check out its website: http://www.livingartscounseling.org/

 

Citations:

Bielańska, A., Cechnicki, A., & Budzyna-Dawidowski, P. (1991). Drama therapy as a means of rehabilitation for schizophrenic patients: our impressions. American Journal of Psychotherapy, 45(4), 566–575.

Fenech, M. A. (2009). Interactive drama in complex neurological disability management. Disability and Rehabilitation, 31(2), 118–130. https://doi.org/10.1080/09638280701850900

Jones, P. (1991). Dramatherapy: Five Core Processes. Dramatherapy, 14(1), 8–15. https://doi.org/10.1080/02630672.1991.9689804

Ruddy, R., & Dent-Brown, K. (2007). Drama therapy for schizophrenia or schizophrenia-like illnesses. In Cochrane Database of Systematic Reviews. John Wiley & Sons, Ltd. Retrieved from http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD005378.pub2/abstract

More

HD in Japan

Overview

Huntington’s disease (HD) is notably rare in Japan, with a prevalence of 1-4 cases per million people – about one-tenth of the prevalence in most European and European-origin populations. Even among those who do not have HD, studies consistently report that the average CAG (Perhaps briefly state around here what CAG repeats are) repeat size in western populations is larger than that found in Japanese populations. Research from Chinese Medical Journal shows that the mean CAG repeat length is 18.4 ± 3.7 in western populations, but only 16.6 ± 1.5 in Japan. It is known that larger CAG counts in the trinucleotide expansion (Perhaps briefly explain the trinucleotide expansion a little more between two commas. Maybe not. That’s a style call.) characteristic of HD diagnosis are associated with the course and severity of illness, such as the progression and magnitude of motor and cognitive decline.

Figure 1. HD is notably rare in Japan

Figure 1. HD is notably rare in Japan

HD populations in Japan experience typical symptom onset. Kageyama et al. reported that, similar to observations in Caucasian populations, adult-onset of HD in Japanese patients presents spasticity (unusual stiffness) and cerebellar ataxia (lack of muscle coordination).

It has been hypothesized that the Huntingtin mutation in Japan has a separate origin from the HD mutation in Europe or Africa. Researchers came to this conclusion after comparing the genetic sequences of the Huntingtin gene (HTT) that there are significant differences between the European, Japanese, and African HTT genes. If one HTT allele were responsible for all the HD cases in the world, there would be strikingly similar HTT alleles in all persons with HD – not the distinct varieties that are actually found. The European, African and Japanese general population chromosomes can be grouped into three different major haplogroups (defined as group of genes within an organism that was inherited together from a single parent). The majority of HD chromosomes in Europe, African and Japanese are found on haplogroup A, B and C respectively. The highest risk HD haplotypes (A1 and A2), are absent from the general and HD populations of Japan, and therefore provide an explanation for why HD prevalence is low in Japan. Such differences, therefore, suggest that the HTT allele originated independently in these three regions.

hd-in-japan-quote-2

Figure 2. Japan has a separate origin

Resources:

Japanese Huntington’s Disease Network (JHDN). The main goal of JHDN is to use online activities to provide and share information about the latest HD research and resources. Its more specific aims are: 1) to liaison with other HD societies, such as the World Federation of Neurology and International Huntington Association, to gain access the latest HD research; 2) to translate and distribute documents and publications for Japanese readers; 3) to distribute newsletters to HD families and those who are interested; and 4) to organize HD family meetings for informal talks and discussions. For more information, please visit http://www.jhdn.org/ejhdn.html or e-mail jhdn@mbd.nifty.com

The Movement Disorder Society of Japan (MDSJ). The main goal of MDSJ is to promote clinical and basic studies on movement disorders, including chorea and Huntington’s disease. They host seminars, educational lectures, symposiums, video sessions, forums to discuss controversies and poster presentations about genetic studies, biomarkers, recent advances in drug therapies, and regenerative medicine. For more information, please visit http://mdsj.umin.jp/en/index.html

Source:

Xu, M., & Wu, Z.-Y. (2015). Huntington Disease in Asia. Chinese Medical Journal, 128(13), 1815–1819. https://doi.org/10.4103/0366-6999.159359

More

“Holding Your Breath”: Interviews With Young People Who Have Undergone Predictive Genetic Testing for Huntington Disease

Introduction

In many countries worldwide, predictive genetic testing is only available to those 18 years and older. However, some argue that testing may be beneficial for young people and should be considered earlier. A qualitative study by Murdoch Children’s Research Institute aimed to explore the experience of predictive genetic testing for HD from the point of view of eight young people within the age range of 17 to 25 in Australia and documents the impact of the testing on the young people’s lives. Four were female and two young people received a genetic positive test result.

Methods:

All interviews were conducted at either the hospital or at the participants’ homes. The interviews were recorded, transcribed word-by-word and analyzed using a qualitative research software package called NVIVO. Initial analysis is based on the benefits and harms of the testing. Conceptual analysis then produced categories to enhance the first round of analysis.

Findings:

There are three themes related to the time before the test was performed: “Living as though gene-positive,” “Risk behaviors,” and “Complex pasts.” All young participants knew that objectively there is a 50 percent chance of inheriting HD, yet many held a belief that their test result would turn out to be gene-positive. Most of the at-risk participants engaged in risky behaviors such as drug use, trouble with the police or difficulty at school. Young participants are also frustrated about growing up with misinformation about HD, coping with sick family members and not being able to undergo predictive testing.

Two themes emerged related to the time after testing: “Living again” and “Identity difficulties.” Some young participants claimed that the genetic test enabled them to move forward, make behavioral changes and being enjoying life more. Some young participants who tested negative and don’t inherit the disease had difficulty adjusting to this knowledge because they are not used the identity without HD.

Conclusion:

In current debates about genetic testing, the focus has primarily been the effect after the testing has occurred. However, this study suggests the importance of the time before and after the testing. The theme of “risk behaviors” is perhaps the most notable of all the themes that emerged, as this is the least well documented in past studies. Although the study didn’t suggest a definitive casual link between the risk behaviors and the risk of HD for young participants, it is noteworthy that participants specifically referred to their risk status, or their desire to be tested, as a cause for their risky behaviors. For some, the testing removes the uncertainty of their genetic status, which is barrier in their lives and prevented them from moving forward. The theme of “living again” represents an additional focus for future research. None of the young participants, even those tested positive, regretted undergoing predictive testing. Therefore, predictive genetic testing may not only have the potential to create harms and benefits for young people at risk of HD, but may have the potential to alleviate other forms of harm in their lives.

 

More

Maladies of My Mind – Ch. 5 Lullaby of Wishes

Maladies of My Mind

Ch. 5 Lullaby of Wishes 

My brother had just died of pneumonia. Huntington’s Disease is not fatal in itself. People with HD have a shorter life expectancy and die of other life-threatening complications related to this disease. I remembered not being able to move when I was brought to the morgue to confirm his body.

It almost felt like I was floating above myself to get an aerial view of the tragic end. My first thought was “Awut. Wake up. That’s not funny. I have something to tell you.” I thought I might have spoken those words out loud to him. But of course he didn’t wake up. He didn’t answer. Didn’t move. Fear consumed me. I had dreamed this very scene many times before. Sometimes even hoped for it to happen in those moments of despair because Awut had mentioned so many times that he wanted to die. But in those brief moments, it wasn’t like this. I had imagined relief. But this is nothing like relief. I wanted to tell him I had just tested positive for HD as well. Maybe we could do this together. Perhaps we could even morbidly joke about how our odds were just bad. Awut had always thought I would get away from the disease since there’s a fifty percent chance of getting it and he already got it. But that’s just a myth. Even if your siblings already inherited the disease, you still have a fifty percent chance of inheriting it.

I’m startled from my thoughts by Sujin’s voice. “Hmm?” I murmur, as he loops his arm around mine when we find our way through the crowded street.

Sujin purses his lips in a familiar expression of concern. He can tell I’m distracted, but I’m grateful he decides to let it go. “I said, when we get back, we should meet Dr.Roongroj. He’s the director of the Motor Disorder Center in Thailand and is also a friend of mine. He told me there are many clinical trials you would be a great fit for.”

I let his words slide pass me. That seems to be happening a lot recently. Even though he is not my doctor, Sujin would tell me about the clinical trials I should sign up for or the medications I should be taking. The more he kept telling me what I should do, the less I feel like his partner and more like his patient. The control I once have is escaping me.

So I try to distract myself with the unbearable heat that surrounds me. I feel a bead of sweat slowly drip down my neck and sacrifice itself to the fabric confines of my already damp shirt. I lethargically fan my hand at my face, hoping it might grant some brief respite from the overwhelming heat. The air is so thick with moisture and humidity that I feel I could swim through it. The entire village seems to be slowly melting. I could swear those Buddha statues were standing a few inches taller a couple minutes ago. It is early evening on the longest week of the year. We are lost in the thick of a celebration in the Songkran Festival, which is a weeklong festival in April. It’s a Thai New Year and is also the most important holiday of the year. Traditionally, water is sprayed on statues of Buddha as a means of purification. But now, this festival has turned into a countrywide water fight. It sounds like fun for most, but for a trauma surgeon like me, it’s a nightmare. This year alone, Thailand’s roads claimed 364 lives over the 7-day Songkran holiday, with a further 3,600 people injured.

The sun has nearly dipped below the horizon, and the moon hangs low and plump, ripe golden orbs suspended over the lake. Sujin and I wander through the throngs of revelers, lost amid the celebration’s rainbow of colors. Both of us are dressed in Northern Thai silks tonight, my hair wrapped up and our fingers adorned with silver rings. People draped in jasmine garlands are everywhere, packed into the narrow alleys and spilling out into the squares, dancing in long lines around bathing temples. We walk past waterways swollen with cargo-laden boats carved in gold and silver with repeating circles and squares. Ornate tapestries hang from balconies in the smoky air.

I feel as if I were in a haze, the celebrations floating around me. It’s strange to look out at all of this joy when I am feeling the opposite of it. Even with his death, my brother still found a way to bother me. In his will, he wanted to be cremated back at our hometown. Little did he know that our quiet little town in Chiang Mai has now turned into a popular spot for tourists. I stay silent, letting Sujin guide us through the busy streets, as I return to my dark thoughts.

Since my Huntington’s disease symptoms start progressing, I have woken to whispers at my bedside that fade away seconds later. They are not always there, and I cannot always understand them even when they are speaking to me. But I can always feel their presence lingering in the corners of my mind. They are blades, rotations of sound and silence, lamps that burn black. They whisper, “Orasa, you should blame yourself for Awut’s death.”

At first I would argue with the whispers. But now, I’m too weak to even come up a counter. So I agree silently to the whispers. I should have taken the genetic test sooner. At least he won’t feel alone when he died.

“All of it was your fault,” the whispers in my head grow. “You killed him. Not the disease but you. You killed Awut.”

Yes. What kind of a doctor am I when I can’t even do anything to save my own brother’s life?

“We both know deep down you wanted him to die,” the whispers say. “So you can finally be free.”

“Orasa,” Sujin says. He tugs on my arm and the whispers scatter. “We’re here.”

We arrive at the temple my brother mentioned in his will. He wanted to be cremated at the same place our mother was. The undertaker emerges from inside the temple and stomp towards the gate. I recognize him from my mother’s funeral years ago. He no longer wears a youthful enthusiasm he once had when he was excited to have his first job so he could support his family. He wears an expression of a dead man who is stuck in a dead end job he despises. I don’t blame him. Undertakers are considered the lowest of the Thai society. People avoid them because they are associated with death. But I don’t care. He is one of the few familiar faces I recognize from my childhood and I can’t wait to greet him. I force my steps towards him but my legs wobble. Before I could stop myself, I trip on my own feet and fall face down to the ground. Sujin rushes to my side. I look up to find the undertaker towering over me. I force a smile but the man says, “We don’t allow drunkards in the temple.” I thought he was joking at first. Any second now he would laugh and welcome me home like what old friends do. But he doesn’t remember me and he looks dead serious about his accusation.

Sujin helps me up. “She’s not drunk.” He tightens his fists and grumbles, “She’s just sick. The way she walks is one of her symptoms

The undertaker looks unfazed. He doesn’t believe a word Sujin said. I turn to Sujin and mutter, “I’ll be by the lake. Please make sure my brother’s funeral arrangement is settled.”

He looks concerned but I slip away from him anyway. I walk towards the lake nearby the temple. It is also the lake my family loved to visit – when we were a still a family that is.

I stare silently at my reflection in the lake, studying my long dark hair and the broken look on my face, both illuminated by the orange and yellow hue of the temple’s candlelight. I scream at my reflection and try to shatter it with my fist. The water splashes. The image becomes distorted but it quickly returns to its shape. My brother blurs in and out of the lake’s reflection, gliding behind me with a dark and mocking face. I try to make him go away, but to no avail.

I look away and found an empty bottle of alcohol by the lake. I seize it and angrily smash it to the ground. The bottle shatters and my hand starts bleeding. I grab a shard of glass, ignoring the sharp pain from my wounded hand. I take hold of a lock of my hair and frantically begin to cut it off. The wound on my hand twists in protest, tearing open, but I don’t care. I hate everything about this disease. I want it gone. It has brought upon me all of the pain in my life. It has taken from me everything — my mother, my brother and even my father who lost himself in grief and alcohol. I am alone, broken and small, like a wingless butterfly fighting for air in the flooding monsoon.

I have to get rid of this disease. I slice away again and again, chopping off locks of my hair and spilling the broken strands all around me. In my madness, the shard nips at my fingers and my scalp, leaving cuts as it goes. Red blurs my vision.

“Orasa!”

Somewhere in the midst of my hysteria is a strong, clear voice. Then Sujin is here beside me, his large hands reaching for mine. But his pleas fall deaf on my ears. I jerk away from his grasp and continue to slash away my hair. “Let go of me,” I hiss, tasting salt and water on my lips.

Sujin tries to seize the shard from my hand. In blind rage, I lash out at him with it. I stop when I see an oozing flow of red seeping out of his cheek. My hands tremble and I drop the shard. The sudden rush of control leaving my body robs me of my breath. I gasp and brace myself against the floor as my body crumples. Sujin’s arms are around me, trying to calm me down. All around us are locks of my hair, painted in orange and yellow by the candle lit temple. Sujin pulls me into a tight embrace. Frightened and distraught, I cling desperately to him.

“I’m losing myself,” I whisper, my voice cut by broken sobs. “I’m a surgeon who can’t operate. I’m a daughter who couldn’t save her mother. I’m a sister who left her brother to die all alone.”

I finally begin to understand my brother’s wish to end his life. I push Sujin away and grab hold of the broken shard of glass again — tighter this time, more blood flowing out of my hand as I pull it closer to me. There will be books that I will never finish reading, patients that I will never finish treating, TV series that I’ve started and will never see the end of. There were times in medical school when these never moments overwhelm me. When I realized there were medical textbooks and journals that I would probably never read, I went to the medical school library data and created a list with every single medical related textbook and journal. There are 28 pre-modern textbooks, 21 modern textbooks and 25,400 scientific journals. So now I mourn. I mourn for the textbooks and journals that will never reach me. For the words and knowledge that lie in the eternally unopened pages. For the patients I will never see again. I mourn my 30th birthday that won’t be celebrated. The overwhelming never moments I must face are too much for me to handle alone and I just want to drown in them.

“Please.” Sujin’s soft words cut through the angry voices poisoning my mind. He hesitates. “Please don’t give up.”

I flinch away. “No,” I snap. “I don’t need your pity.”

“How could you say that?” Sujin’s eyes glow with tears. “After all these years, you must know how I really feel about you.”

I know. But I won’t allow him to feel it. “You don’t have a future with me.” Sujin studies my face. The sadness on his face breaks my heart. Finally, Sujin lets go. “Just leave me alone,” I mutter over and over again. “Just leave me al–“

My words cut off when he puts his forehead against mine.

“Orasa,” he closes his eyes and whispers. “Do you remember how we used to skip lectures and lie on top of the med school building, pretending that we could actually count the stars in the polluted Bangkok night and ignoring our responsibilities?” I nod against his forehead. “Do you remember how your patients left gifts at the hospital for you once they knew?” His voice starts to tremble. “Do you remember how we sat up late into the night, holding each other and crying when your genetic test came back positive?”

I nod, biting back my tears.

“You are not alone.” He places both his hands on my face. “I’m here for you.”

Something breaks inside me, dissipating the ugly whispers that plagued me moments earlier, and the gates holding back my tears break down. I hug Sujin fiercely, as if I might die were I to let go. Grief envelops me. I was brought back to the time my family was happy before the disease caught a hold of us.

Sitting by the lake, mom unfurled her fingers with her palm up, like a flower opening to the butterflies. Awut and I fluttered to her sides. Mom held our small hands and said she could read our futures in the lines of our palms.

“Orasa,” Mom said. “Your education line is long. Maybe you will become a doctor.” Mom traced the line with her slender finger, tickling my hand as it moved from the base of my palm up to my pinky.

Awut shoved away my hand and offered his for mom’s reading. “Look at mine, Mom!”

I pouted, but mom chuckled. “Let me see…” She held his hand, studied it for a moment and then suddenly kissed it. Awut groaned out of frustration but I could see a smile at the corner of his lips.

“This one, dear. Please tell me about this one.” Father joined us, extending his hand in front of mom. He was a whole and healthy vision of a man I used to know, with laughter in his voice and the scent of jasmine rice on his shirt instead of the familiar perfume of alcohol. Mom pointed at a line on his palm and predicted that he would live to be an old man with happy and healthy children.

It did not matter that we heard the stories before. Each story was a lullaby of wishes we were always reaching for. We were always reaching and wishing to feel mom’s touch.

I hold the memory for as long as I can. I would have held it forever, happy to lose myself in it for the rest of my life. Finally, I release the memory and open my eyes. The image of my family is replaced with Sujin, his arms still wrapped tightly around me. I lean against him, weak and exhausted.

In that moment, I realize that all I’ve ever wished for, kindness, companionship and family, come from the people I still have in my life. Not in the past where I’ve lost too much or in the future I may not have because of this disease. But in this moment, I am not alone. The people in my life give me the courage to live and overcome the overwhelming never moments.

Tomorrow morning, I will ask Sujin about the clinical trials he has been talking about. But tonight, we stay where we are, holding on, lost in the lullaby of wishes.

Author’s note: I hope you enjoyed the story! For more information about HD resources in Asia, please check out our articles. For more information about the clinical trials, please check out our articles on research updates. 

More

GyroGlove: Wearable Treatment Solution For Hand Tremors

GyroGlove: Wearable Treatment Solution For Hand Tremors

The part of the brain most affected by Huntington’s disease, the basal ganglia, are groups of nerve cells (neurons) at the base of the brain. Basal ganglia are responsible for the motor movements of the muscles in the body. When cells in basal ganglia die, a common pathological symptom of HD, a person experiences uncontrollable muscular movements similar to that of fidgetiness. Another disease that produces similar uncontrollable movements symptom is Parkinson’s disease, due to its effects on a specific area of the basal ganglia called the substantia nigra.

After treating a 103-year-old patient who suffered from Parkinson’s disease and watching her struggle to eat a bowl of soup, Faii Ong, a 24-year-old medical student from Imperial College London, asked a nurse what more could be done to help the woman. “There’s nothing,” he was told grimly. Immediately after, Ong developed an early prototype of a device, called GyroGlove, which was the first wearable treatment solution for hand tremors. The GyroGlove is a small, lightweight stability device that fits on the back of the hand. It uses a miniature, dynamically adjustable gyroscope, which sits on the back of the hand, within a plastic casing attached to the glove’s material. Gyroscopes are spinning discs inspired by bleeding edge aerospace technology, but no different than children’s toy tops. Gyroscopes do their utmost to stay upright. These spinning discs thus counter any input of force instantaneously and proportionally. When the device is switched on, the battery-powered gyroscope whirs to life. Its orientation is adjusted by a precession hinge and turntable, both controlled by a small circuit board, thereby pushing back against the wearer’s movements as the gyroscope tries to right itself, thereby dampening any tremors in the wearer’s hand. Early stage testing of glove demonstrates significant reduction of tremors of over 80%. Moreover, the company seeks to track tremors each time the GyroGlove is used, thus providing quantifiable data for that will better inform users, their family and their doctors that may help patients optimally manage their medications.

GyroGlove, source: www.technologyreview.com

GyroGlove, source: www.technologyreview.com

While Ong and his company, GyroGear, have yet to set an exact launch date and cost for the glove, they hope to launch in the U.K. before September at a price between £400 to £600 ($550 to $850). Moreover, Ong has plans to address other tremors elsewhere in the body, such as the legs. He believes that the device could be used in professional contexts where the wearer requires a steady hand, such as surgery, photography, and even sports.

In conclusion, although there is currently no direct study on the use of GyroGear on HD patients, Ong is convinced that the technology could help reduce hand tremors in patients with Parkinson’s disease. Therefore, the similarity between the motor symptoms of Parkinson’s and Huntington’s disease means that this application could also be used to help HD patients. For more information about GyroGlove and how to sign up to test or have priority access to the product launch, please go to this link.

More

Automated Navigation System Wheelchair for HD Patients

The parts of the brain most affected by Huntington’s disease (HD), the basal ganglia, are groups of neurons at the base of the brain. Basal ganglia are responsible for the motor movements of the muscles in the body. When cells in basal ganglia die, a common pathological symptom of HD, patients experience uncontrollable muscular movements like tremors and fallings. HD patients will need assistance of a wheelchair towards the end stage of the disorder when the movements become debilitating, and the falls become more prevalent. When they are unable to get up and move around on their own, wheelchairs become important mechanisms of transport.

An electric wheelchair by Dr. Yodchanan Wongsawat from Center for Biomedical and Robotics Technology Faculty of Engineering at Mahidol University in Thailand is designed specifically for disabled patients with limited hand mobility. This prototype wheelchair has an automated navigation system that could safely navigate and facilitate comfort to the disabled patients, including those with Huntington’s disease. The electric wheelchair accommodates a variety of conditions. If their hands are still functional, a patient could use their hands; otherwise, the modes can be operated by one’s chin or eye controls if the patient’s hands and chin are paralyzed or unable to function due to a debilitating movement disorder. These functions are explained further in details below. The wheelchair can be automatically navigated to the destination on the pre-located mapping. Moreover, through the automated navigation system, the obstacles can be detected and avoided safely.

The Automated Navigation System Wheelchair is made of these components:

  1. Rotating Laser Scanner: use for detecting the obstacles on the floor
  2. Laser Scanner: use for wheelchair localization, obstacle detection and mapping
  3. 7’’ LCD screen: come with user friendly interface together with command description
  4. Mini-PC: data acquisition and wireless communication device with server
Brain Control Wheelchair poster from BCI lab Mahidol, Download date 9/24/15

Source: Brain Control Wheelchair poster from BCI lab Mahidol, Download date 9/24/15

Brain Control Wheelchair poster from BCI lab Mahidol, Download date 9/24/15

Source: Brain Control Wheelchair poster from BCI lab Mahidol, Download date 9/24/15

  

Alternative Input Mode

  1. Eye Module
  2. Chin Module
  3. Hand Joystick Module
  4. Brain Module

Before initiating the automatic navigation task, the system needs to build a static map and define the users’ most frequent locations. These locations can be selected in the different choices of the automatic navigation mode. The information from odometers (which indicate distance traveled) and laser scanners are used for map building, which is based on a mapping technique, the enhanced simultaneous localization and mapping (SLAM) system. The most frequent users’ locations can be recorded, which is then used to build the static or a fixed map. In order to navigate the wheelchair from one location to the desired destination, the Robot Operating System (ROS) based navigation system will calculate a possible path using global path planning and control the wheelchair by the command velocity from the local path planning. During wheelchair navigation, the system can localize the wheelchair location by the Monte Carlo localization adaptive approach. The obstacles can be detected using the Tilt scanner. Once an obstacle appears, the ROS based navigation system can calculate a new path and safely continue navigating the wheelchair to desired destination.

Source: Brain Control Wheelchair poster from BCI Mahidol Lab, Download date 9/24/15

Source: Brain Control Wheelchair poster from BCI Mahidol Lab, Download date 9/24/15

Source: Brain Control Wheelchair from BCI Mahidol Lab, Download date: 9/24/15

Source: Brain Control Wheelchair from BCI Mahidol Lab, Download date: 9/24/15

In conclusion, the electronic wheelchair has many applications for HD patients, especially for those at the end stage of the disorder when the movements become debilitating, and the falls become more prevalent. This wheelchair is just a prototype and has not been mass-produced. However, individuals can order a customized version of it. For more information, please contact Dr. Yodchanan Wongsawat at yodchanan@gmail.com

 

 

More

HD Spotlight in Asia (Founder and Director of Movement Disorders Center in Thailand)

HD Spotlight in Asia

Professor Roongroj Bhidayasiri, M.D. – Founder and Director of Movement Disorders Center in Thailand

roongroj

Short Biography

Dr. Bhidayasiri’s major interest is in movement disorders and he also leads many national and international clinical research trials in Parkinson’s disease. Significantly, he leads a Parkinson’s disease registry in Thailand in collaboration with the Thai Red Cross society, the Ministry of Public Health, Bangkok Metropolitan and the National Health Security Office of Thailand. Moreover, he works as the chair of the scientific committee of the Parkinson’s disease guidelines in Thailand, the director for Chulalongkorn Comprehensive Movement Disorders Center and an associate professor for Chulalongkorn University Hospital in Bangkok. He is also the president of Thai Parkinson’s Disease Society and a visiting associate professor of neurology for David Geffen School of Medicine at UCLA.

In addition to his specific works on Parkinson’s disease, he is also the founder and director of the Chulanlongkorn Comprehensive Movement Disorders Center and has treated over 10 families with Huntington’s disease in Thailand. He recounted a story about a patient with Huntington’s disease, who wrote a letter to the Queen of Thailand for help because her family members thought she was possessed by a demon and made her go through traumatizing exorcism. For more information on Mental illness and Exorcism Across Different Cultures, please refer to our article.

Moreover, most physicians in Thailand don’t recognize the symptoms of Huntington’s disease and misunderstand them as Parkinson’s disease. His goal is to raise more awareness about Huntington’s disease and other rare motor disorders in Thailand through outreach events and educational online sources, using HOPES as a model.

Medical Degree and Training:

Dr. Bhidayasiri graduated in medicine at Chulalongkorn University in 1994. He received the membership of the Royal College of Physicians of London and Ireland in 1998 and was certified by the American Board of Psychiatry and Neurology in 2005. Awarded the fellowship of the Royal College of Physicians of London in 2008 and the Royal College of Physicians of Ireland in 2010.

Primary Research Interest/project:

His research interest lies in movement disorders, particularly the clinical aspects and advanced therapeutics of Parkinson’s disease. He has published over 70 articles in peer-reviewed journals and has served in the editor-in-chief of the Thai Journal of Neurology and also a reviewer of more than 10 international journals on neurological disorders. He also serves as a writing committee panel member of the American Academy of Neurology on the practice parameters of movement disorders.

Contact information:

To make appointment and direct inquiry to Dr. Bhidayasiri, please refer to his contact form on Thai Parkinson’s disease Clinic website.

More

HD Spotlight in Asia (Founder of the Parkinson’s and Movement Disorder Society in India)

HD Spotlight in Asia

Dr. Bhimsen Singhal – Founder of the Parkinson’s and Movement Disorder Society in India

Dr.Singhal

Short Biography

Dr. BhimSen Singhal is the Director of Neurology at Bombay Hospital Institute of Medical Sciences in Mumbai, India. Although there are existing organizations for neurological illnesses like Cerebral Palsy, Alzheimer’s disease, and Stroke, Dr. Singhal is concerned that there were none actively advocating for the cause of Parkinson’s or other motor disorders. During the fourth annual meeting of the World Health Organization (WHO) Working Group for Parkinson’s in 2000, he founded the Parkinson’s Disease and Movement Disorder Society (PDMS) with experts from Mumbai, Kolkata, Delhi, Hyderabad, Nashik, Pune, Goa, Bhavnagar and Bengaluru pledging to help the cause. Along with its team of neurologists, psychologists, physiotherapists and various medical, allied medical and fitness professionals, PDMDS has extended its reach to cities throughout India. From the many invitations, PDMS has received worldwide recognition.

Medical Degree and Training

After attending school at Mount Abu and premedical studies at Maharaja’s College in Jaipur, he studied medicine at the Grant Medical College and Sir. J.J. Group of Hospitals in Mumbai. He obtained his medical degree 1956 and residency in 1959. After his initial training in Neurology in Mumbai, he went abroad for further training at the UCL Institute of Neurology in London. During his training in the United Kingdom, he obtained training certificates from Edinburgh and London in the early 1960s. He was made a Fellow of the Royal College of Edinburgh in 1973 and Fellow of the Royal College of London in 1985.

Professional Work

Over the course of his professional life, Dr. Singhal has trained over 200 neurologists who are now providing care across India. He has published over 200 scientific papers in both national and international books and journals. He is known for his interest and work in Parkinson’s disease and other motor disorders like Huntington’s disease.

He was the president of the Neurological Society of India (1986), president of the Indian Epilepsy Association (2000-2002), member of the WHO working group for multiple sclerosis and Parkinson’s disease, regional director for the World Federation of Neurology (2005-2009), and chairperson of the Asian Section of The Movement Disorder Society (2009-2010). In order to update neurologists on recent advances in neurology, Dr. Singhal created the ‘Neurology Foundation’ in 1996, and holds ‘Neurology Update’ meetings every alternate year.

Dr. Singhal established the Parkinson’s Disease and Movement Disorder Society in 2001, which he now serves as Honorary Secretary. This society has been acknowledged as a model for developing countries by organizations like the World Parkinson’s Congress and the World Federation of Neurology.

Contact Information

To make appointment and direct inquiry to Dr. Singhal, please refer to his contact form on Parkinson’s Disease and Movement Disorder Society’s contact form.

 

More

Extracorporeal Shock Wave Therapy (ESWT) for HD Patients Rehabilitation

The prevalence of pain in Huntington’s disease is unknown. An initial case report describes severe pain in two HD patients (Albin and Young, 1988). In a more recent study in 2010, eleven of nineteen HD patients had pain, with altered pain perception to pinprick, touch and temperature in some subject (Scherder and Statema, 2010). Exercise-induced muscle pain has been described in a marathon runner, who subsequently developed Huntington’s disease (Kosinski et al., 2007), suggesting that it may be an early unrecognized symptom of the disease. Significantly, there is a 10% to 25% prevalence of diabetes in patients with Huntington’s disease worldwide. Diabetes is a relatively common cause of neuropathic pain in a subset of diabetics.

Professor Areerat Suputtitada, M.D. is an expert on the use of extracorporeal shockwaves therapy (ESWT) on patients with pain management problems. She applies radial shock waves to the musculoskeletal system. The process involves producing high speed and high-pressure sound waves. The waves are delivered either in a focused manner, in which targets deeper tissues, or in a radial manner, in which targets a wider area. The energy is released to the tissues whenever there is a ligament to bone surface change.

Figure 1. Dr. Suputtitada Applying ESWT to Patient

Figure 1. Dr. Suputtitada Applying ESWT to Patient

Benefits for patient include

  • Outpatient treatment
  • No medication
  • No anesthesia
  • No surgery
  • No injections
  • Non-invasive treatment
  • Quick results
  • Improved quality of life

Benefits for the physician

  • Short application time
  • High patient acceptance
  • Broad range of indications
  • Outstanding product quality
  • Modular perfection
  • Low maintenance
  • Low cost

How does ESWT promote tissue healing in HD patients?

ESWT produces a strong energy pulse for a short period of time. The energy pulse breaks the sound barrier, which results in a shockwave. The shockwave from ESWT is identical, only on in a smaller scale, to the shockwaves that occur when a plane is flying faster than the speed of sound.

  1. The shockwave exerts pressure on the afflicted tissue. It increases cell membrane permeability and increases microscopic circulation to the tissues and the metabolism within the tissues, both of which promote healing and dissolution of pathological calcific deposits.
  2. “Cavitation bubbles” are small empty cavities created when shockwave is applied. The cavitation bubbles tend to expand to a maximum size, then collapse, much like a bubble popping. As these bubbles burst, a force is created. This force is strong enough to break down the pathological deposits of calcification in soft tissues and therefore reduce pain.
  3. ESWT stimulates osteoblasts or bone cells. These bone cells are responsible for bone healing and new bone production, so stimulating them enhances the healing process of bones.
  4. ESWT stimulates fibroblasts. Fibroblasts are the cells responsible for the healing of connective tissues like tendon or ligaments.

On a molecular and cellular level, ESWT also leads to complex interactions between the removal of neurogenic inflammation and release of growth factors, changes in gene expression, new bone formation and activation of mesenchymal stem cells, in which can differentiate into a variety of cell types like bone cells, cartilage cells, fat cells and muscle cells. All of these interactions then lead to pain relief and healing of patients.

In conclusion, ESWT is a noninvasive, easy to use after training, and cost effective way to improve quality of life within a short time frame. HD patients with pain management problems could consider ESWT. For more information on Dr. Suputtitada, her work on ESWT and her contact information, please refer to our spotlight article link.

References

Durant A, Millis D. Applications of extracorporeal shockwave in small animal rehabilitation. In: Millis DL, Levine D, editors. Canine Rehabilitation and Physical Therapy. 2nd ed. Elsevier; 2014. P. 381 -392

O gden JA, Toth-Kischkat A, Schultheiss R. Principles of shock wave therapy. Clin Orthopo Relat Res 2001; (387): 8-17

Borsook, David. “Medscape: Neurological Diseases and Pain.” Medscape. Medscape, 2012. Web. 22 Oct. 2015.

More

Cueing Device For Improving Gait Ability in Parkinson’s Disease and Other Motor Disorders

The parts of the brain most affected by Huntington’s disease (HD), the basal ganglia, are groups of neurons at the base of the brain. Basal ganglia are responsible for the motor movements of the muscles in the body. When cells in basal ganglia die, a common pathological symptom of HD, patients experience uncontrollable muscular movements. Another neurodegenerative disorder that produces similar abnormal movement is Parkinson’s disease, due to the disease’s effects on a specific area of the basal ganglia called the substantia nigra. Parkinson’s disease is a neurodegenerative disorder that results in characteristic motor abnormalities including postural instability and gait impairment. It consists of short shuffling steps, decreased walking speed, increased cadence, and gait freezing.

Current research is attempting to develop visual, auditory or somatosensory cues, or signals, to improve gait of Parkinson’s disease patients. Cueing device is defined as a device that uses external temporal or spatial stimuli to facilitate movement or gait initiation and continuation. The cueing device for improving gait ability in Parkinson’s disease was researched by Professor Areerat Suputtitada, M.D.,leading Physiatrist in Thailand. The main objective is to examine the effect of cueing devices using visual, auditory and somatosensory stimuli during walking in Parkinson’s patient using motion analysis. The study hypothesizes that the use of laser point, rhythmic sound and rhythmic vibration will increase the patients’ stride length, step length and speed, and decrease gait freezing.

Twenty Parkinson’s disease patients were asked to walk at a normal speed along a 10 meter walkway eight times using different combinations of cueing devices. The spatiotemporal data were recorded with motion analysis as seen in figure 1. The conditions tried include:

  1. Walking without a cueing device
  2. Walking with visual cue
  3. Walking with an auditory cue
  4. Walking with a somatosensory cue
  5. Walking with visual and auditory cues
  6. Walking with visual and somatosensory cues
  7. Walking with auditory and somatosensory cues
  8. Walking with all three cues.

All conditions were done three times by every patient.

Figure 1. Motion Analysis

Figure 1. Motion Analysis

 

The cueing device consists of 3 components (Figure 2):

  1.  Visual: used a laser pointer made from optic fibers with a switch to project the straight line
  2. Auditory: generated a rhythmic sound
  3. Somatosensory: used vibration and a microcontroller to create a rhythmic vibration
Figure 2. Cueing Device

Figure 2. Cueing Device

Results

The researchers found statistically significantly better scores for gait freezing, step length and walking velocity in the patients group that used the cueing device compared to those who didn’t use the device. There was no statistically significant difference between each type of cues or combined cues. In figure 3, the left picture demonstrates short steps at baseline before the cueing device is used and the right picture shows an improved step stride when light and sound cues were used.

Figure 4. Before vs After Cueing Device Feet Scans

Figure 3. Before vs After Cueing Device Feet Scans

Conclusion

The study reveals the significantly effective use of visual, auditory and somatosensory cues for improving gait ability in Parkinson patients. The combined cueing device is a product designed by Professor Areerat Suputtitada,M.D. and her team. Although her team is using the cueing device to help patients with Parkinson’s disease improve their gait, it is also highly applicable to HD patients. For more information on Dr. Suputtitada, her work and her contact information, please refer to our spotlight article link.

References

  1. Suputtitada A, Sriyudthasak M, PongmalaCueing devices for gait ability in Parkinson patient using motion analysis. Gait & Posture 2009; 30 (Suppl 2): S127.
  2. Rochester L, Hetherington V, Jones D, Nieuwboer A, Willems A.M, Kwakkel G, Wegen E. The effect of external rhythmic cues (auditory and visual) on walking during a functional task in homes of people with Parkinson’s disease. Arch Phys Med Rehabil 2005; 86: 999-1006
  3. Suteerawattananon M, Morris G.S, Etnyre B.R, Jankovic J, Protas E.J. Effect of visual and auditory cues on gait in individuals with Parkinson’s disease. J Neurol Sci 2004; 219: 63-69
  4. Novak P, Novak V. Effect of step-synchronized vibration stimulation of soles on gait in Parkinson’s disease: a pilot study. J NeuroEng Rehab 2006; 3:9.
  5. Lim I, Wegen EV, de Goede C, et als . Effects of external rhythmical cueing on gait in patients with parkinson’s diease: a systematic   review.Clin Rehabil 2005;19:695-713.
More

HD in Asia

This section of Global HD aims to highlight regions within Asia in which the HD populations have been studied, and where history and significance within the HD context have been demonstrated.

asia

The prevalence of HD in the West is estimated at five to ten people per 100,000 people. However, in Asia, the prevalence of HD is approximately one tenth of the HD prevalence in Western population. The prevalence is 0.4 per 100,000 people in Hong Kong and 0.65 per 100,000 in Japan. In India, there are case reports from Punjab and northern India and until 1990, there were 69 case reports of HD in China. In Southeast Asia, the epidemiology data for HD consists mainly of case reports with HD in Singapore being reported from a Chinese family and second-generation Indian families. There is no accurate data obtained from Thailand, Indonesia and Philippines.

The reason for this wide variation in HD prevalence in different ethnic communities is unclear. Some people advocate a European origin for the mutation, suggesting that migration and inter-racial mixture of genes led to a lower prevalence in non-European countries. As mentioned in the HD in Hong Kong section, it has been proposed that the prevalence of HD in Hong Kong maybe attributed to a European origin. This assumption derives from the fact that the ancestors of the families with HD can be connected to coastal provinces with histories of strong colonial presence. Hence, it has been hypothesized that HD in other Chinese related countries may also have originated from a common Caucasian ancestry by ways of migration.

China^

Overview^

There is limited information on Huntington’s disease in China. However, in 1992, researchers at the Chinese University of Hong Kong published Huntington’s disease in China: A hypothesis of its origin. As with many other non-European populations, HD’s origins in China’s coastal provinces indicate that it likely spread as a result of interactions between European and Chinese traders.

Between 1984 and 1991, the prevalence of HD in Hong Kong was 3 to 7 patients per million. This population appeared to be predominately males who experienced an earlier disease onset. The first instances of HD were noted in 1959 and again in 1962. Historically, war and natural disasters disrupted living patterns and caused mixing of populations that increased the prevalence of HD outside of the original coastal provinces. However, compared to other regions, the spread of HD in China is rather limited and has remained predominately in these coastal regions.

This research is over 20 years old. More comprehensive statistics are needed to understand the true prevalence of the disease, particularly the prevalence of HD in women as this population is most likely underreported due to their status in society in the 1980’s and 1990’s.

Organizations^

Chinese Huntington’s Disease Network

The Chinese Huntington’s Disease Network was launched on December 10, 2011 in Shanghai. This organization seeks to help the estimated 300 affected families in the region by increasing awareness about HD and improving quality of care.

For Further Reading^

Leung, C. M., et al. “Huntington’s disease in Chinese: a hypothesis of its origin.” Journal of Neurology, Neurosurgery & Psychiatry 55.8 (1992): 681-684.

Malaysia^

An Overview^

Malaysia is a multicultural country with three main ethnic populations. The indigenous Malaysians, Chinese and Indians make up approximately 62 percent, 29 percent and 8 percent of the population respectively. The prevalence of HD is 0.0024 per 10,000 people, based on seven reported cases.

History^

To test the hypothesis that HD in Asia has a Caucasian origin, a HD registry was established in Malaysia in 1995 at the University of Malaya Medical Centre, Kuala Lumpur. Within eighteen months, the registry had identified seven unrelated patients with HD. From these individuals, there were four Chinese, one Malaysian and two Indians. Despite the previous hypothesis set forth that HD in Asian countries originated from Europe; only one Chinese patient, out of selected seven patients, had possible Irish ancestry. Moreover, there are several social and cultural reasons that the hypothesis set forth is likely to be false. The remaining three Chinese participants did not have any relatives with Caucasian features. Culturally, Chinese women were forbidden to have close contact with foreigners. Women did not travel unless they were accompanying their husbands. Prostitution as a profession for women was deeply despised and extramarital sexual contact was strongly forbidden. Hence, it was highly unlikely that female ancestors of these patients would have sexual contact with asymptomatic Caucasian HD men and therefore result in the mixing of genes. In addition, other participants also did not have European origin, since one Malaysian patient was a local while the two Indian patients originated from Tamil Nadu and Punjab in India respectively.

There is also evidence against the European migration hypothesis from the current genetic literature. Although the nature of the abnormal trinucleotide repeats of the HD gene in Chinese patients is similar to that in Caucasian HD patients, a majority of the tested Chinese patients have a small number of excess CAG repeats. Additional genetic evidence show that there are seven CCG repeats adjacent to IT15, or the Huntington Gene, in Asians but ten CCG repeats in Caucasians. The distribution of the CCG repeat adjacent to IT15 is of seven repeats in Asians and 10 repeats in Caucasians. The distribution of the CCG repeats differs among populations of low prevalence and the west. Therefore, mutation of the IT15 gene rather than the European migration hypothesis is likely to be the explanation for the variation in Malaysian prevalence of HD.

The interim results of the Malaysian HD registry have shown that HD exists in all major racial groups in Malaysia, with all cases exhibiting an excess of CCG repeats. The theory that Caucasian migration led to genetic mixing and a resulting low HD prevalence in Malaysia is less plausible according to the data collected by the registry; more likely, cases of HD in Malaysia arose due to a separate mutation of the HD gene. Nevertheless, because only seven HD patients were identified, these conclusions should be considered with caution to avoid errors in assumption and generalization.

The Global HD research and articles received partial support from the Bingham Fund for Innovation in the Program in Human Biology.

Resources in Malaysia^

University of Malaya Medical Centre in Kuala Lumpur

In 1995, a nationwide HD registry was established at the University of Malaya Medical Centre, Kuala Lumpur. The Medical Centre is one of the major public tertiary referral hospitals. It keeps reports of clinical and genetic studies of the patients seen since the setting up of the Registry.

For more information regarding the association, please visit: http://www.ummc.edu.my/view/index.php

Japan^

An Overview^

Huntington’s disease (HD) is notably rare in Japan, with a prevalence of 1-4 cases per million people – about one-tenth of the prevalence in most European and European-origin populations. Even among those who do not have HD, studies consistently report that the average CAG (Perhaps briefly state around here what CAG repeats are) repeat size in western populations is larger than that found in Japanese populations. Research from Chinese Medical Journal shows that the mean CAG repeat length is 18.4 ± 3.7 in western populations, but only 16.6 ± 1.5 in Japan. It is known that larger CAG counts in the trinucleotide expansion (Perhaps briefly explain the trinucleotide expansion a little more between two commas. Maybe not. That’s a style call.) characteristic of HD diagnosis are associated with the course and severity of illness, such as the progression and magnitude of motor and cognitive decline.

Figure 1. HD is notably rare in Japan

Figure 1. HD is notably rare in Japan

HD populations in Japan experience typical symptom onset. Kageyama et al. reported that, similar to observations in Caucasian populations, adult-onset of HD in Japanese patients presents spasticity (unusual stiffness) and cerebellar ataxia (lack of muscle coordination).

It has been hypothesized that the Huntingtin mutation in Japan has a separate origin from the HD mutation in Europe or Africa. Researchers came to this conclusion after comparing the genetic sequences of the Huntingtin gene (HTT) that there are significant differences between the European, Japanese, and African HTT genes. If one HTT allele were responsible for all the HD cases in the world, there would be strikingly similar HTT alleles in all persons with HD – not the distinct varieties that are actually found. The European, African and Japanese general population chromosomes can be grouped into three different major haplogroups (defined as group of genes within an organism that was inherited together from a single parent). The majority of HD chromosomes in Europe, African and Japanese are found on haplogroup A, B and C respectively. The highest risk HD haplotypes (A1 and A2), are absent from the general and HD populations of Japan, and therefore provide an explanation for why HD prevalence is low in Japan. Such differences, therefore, suggest that the HTT allele originated independently in these three regions.

hd-in-japan-quote-2

Figure 2. Japan has a separate origin

Resources in Japan^

Japanese Huntington’s Disease Network (JHDN). The main goal of JHDN is to use online activities to provide and share information about the latest HD research and resources. Its more specific aims are: 1) to liaison with other HD societies, such as the World Federation of Neurology and International Huntington Association, to gain access the latest HD research; 2) to translate and distribute documents and publications for Japanese readers; 3) to distribute newsletters to HD families and those who are interested; and 4) to organize HD family meetings for informal talks and discussions. For more information, please visit http://www.jhdn.org/ejhdn.html or e-mail jhdn@mbd.nifty.com

The Movement Disorder Society of Japan (MDSJ). The main goal of MDSJ is to promote clinical and basic studies on movement disorders, including chorea and Huntington’s disease. They host seminars, educational lectures, symposiums, video sessions, forums to discuss controversies and poster presentations about genetic studies, biomarkers, recent advances in drug therapies, and regenerative medicine. For more information, please visit http://mdsj.umin.jp/en/index.html

Source:

Xu, M., & Wu, Z.-Y. (2015). Huntington Disease in Asia. Chinese Medical Journal, 128(13), 1815–1819. https://doi.org/10.4103/0366-6999.159359

Other Related HOPES Articles^

  1. HD Spotlight: Rehabilitation Physician in Thailand
  2. HD Spotlight: Founder of the Parkinson’s and Movement Disorder Society in India
  3. HD Spotlight: Founder and Director of Movement Disorders Center in Thailand
  4. A Case Study: Pregnancy and HD in India
  5. Exorcism and Mental Illness Across Different Cultures: A Case Study of North Thailand Hill tribes

More

HD Spotlight in Asia (Rehabilitation Physician in Thailand)

HD Spotlight in Asia

Professor Areerat Suputtitada, M.D. — Leading Rehabilitation Physician in Thailand

AreeratShort Biography:

Dr. Areerat Suputtitada is Professor of Rehabilitation Medicine, full time working faculty at Chulalongkorn University and King Chulalongkorn Memorial Hospital in Bangkok, advisor of the editorial board of Thai Rehabilitation Medicine Journal, and an educational board member of Exercise and Health Promotion in the Ministry of Public health. She received 15 national awards, 3 international awards, and published more than 20 international and 30 national articles in the areas of her experts including neurological rehabilitation, spasticity and dystonia, gait and motion, and pain. She has also been invited to lecture and act as a chairman for over 70 international conferences. Dr. Suputtitada has been elected and appointed to important positions in the International Society of Physical and Rehabilitation Medicine (ISPRM) such as the Chair of Women and Health Task Force and International Exchange Committee. Moreover, she is the youngest physician to have won the Fund Award for Professorship in Thailand.

Medical Degree: Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand

Thai Board of Physical Medicine and Rehabilitation: Chulalongkorn University, Bangkok, Thailand

Training Certificates: Mount Sinai Medical Center, Colorado Neurological Institute, Vienna University Hospital, Western IRB, Gait and Clinical Movement Analysis Society in London, the Association of Pain Therapy in Switzerland, Australian Institute of Ultrasound, American Academy of Physical Medicine and Rehabilitation, Rehabilitation Engineering and Assistive Technology Society of North America, and Collaborative Institutional Training Initiative at the University of Miami.

Email: prof.areerat@gmail.com, sareerat1111@gmail.com

Primary Research Interest/project:

Her areas of expertise include neurological rehabilitation, spasticity and dystonia, clinical neurophysiology, pain, Botulinum toxin, therapeutic exercise and rehabilitation engineering. She treats a variety of patients, including patients with motor disorders like Parkinson’s disease and Huntington’s disease. Examples of her research includes:

  1. Cueing Device For Improving Gait Ability in Parkinson’s disease and Other Motor Disorders
  2. Extracorporeal Shock Wave Therapy (ESWT) For HD Patients Rehabilitation
More

Maladies of My Mind – Ch. 4 Invisible Girl

Maladies of My Mind

Ch.4 Invisible Girl

The first time Awut tried to kill himself was when his genetic test came back positive. I found him lying on the bathroom floor with sleeping pills scattered all over. I took him to the emergency room and he threw up a bit, before a doctor made him drink activated charcoal. He was then hooked up to a heart monitor and the doctors put a line in him to draw blood and give him saline. After they concluded that Awut wasn’t going to die, an ambulance transferred him to a specialized facility, where he was put on a three-day mental hold. Awut told me that he spent most of his days hanging out in the lounge and talking with other suicide and self-harm survivors. A guy told him that people hurt themselves because of anger and that he needed to learn healthy ways to address it. So when Awut was released, he tried to teach me a game called “knuckles,” where we put our knuckles on a table and flick coins as hard as we can across the table at the other person to make them bleed. The loser is whoever flinches first.

Awut flinches when he sees me approaching. There are wires glued to his chest and coming up from the neck of his hospital gown. The gown has no back and lets everyone see the fat parts my brother might want to hide. It is the most embarrassing garment ever invented. Both his hands and legs are also strapped to the hospital bed as if he’s a cow about to be slaughtered. The doctors finally decided to restrain him after his countless attempts to hurt himself and others. His pupils dilate, black and with adrenaline, like rabid animal’s eyes– wild, unfocused, and full of rage. His wretched eyes are glued to my face because there is nothing else to do but lie in his bed, emaciated, and possessed. He begs me to kill him with desperate eyes.

I say quietly, “Did the voices in your head tell you to drown yourself in the tub?”

Awut opens his mouth to say something but hesitates. He then opens it again and says, “T-they said that if I don’t, you’ll come and dr-drown me yourself.” His voice is more staggering than the last time we spoke. It takes him more effort to finish his sentences. “J-just do it already! Kill me and get it o-over with!” He spits at me.

Paranoid schizophrenia-like symptoms occur in 6-25% of Huntington’s disease cases and my brother is one of them. Other than paranoia, Awut also suffers from auditory hallucinations. There are many types of hallucinations, including visual, auditory, gustatory, olfactory and tactile.

I slowly put my hand on his arm and notice how unusually stiff he is. He tries to pull away but has no control over his movement. His body twitches and his muscles spasm. I say calmly, “Why would I hurt you, Awut?”

“Because y-you’re just like him. You’re like f-father.” His limbs jerk involuntarily and his face grimaces, “Y-you want to get rid of this d-demon inside me like father did to mother.”

I touch the scar that runs across my forehead and licks away my brow. The memories of the shaman my father hired to whip my mom with the dried stingray tail rush back to me. I tried to protect her and was marred as a result. Despite my efforts, we ended up losing my mother anyway. My knees weaken and I kneel down beside Awut’s bed. I place my forehead on his arm and he struggles beneath my touch. “I’m not like him.” I feel tears trickle down my face and I fight to keep my voice straight, “If anything, I could turn out just like you and mom.”

Awut’s breathing becomes less erratic and his body relaxes slightly. When I lift my head and look at him, I notice that his facial expression softens. I say, “Awut, I think I’m ready to take the test.”

***

I wake up beside Sujin. My cheeks are covered with a cocktail of sweat and tears. I’m breathing heavily. Sujin leans over me and brushes a wet strand of hair out of my face. I asked him to be my counselor for the genetic test but he refused. He said he’s already attached to me and it is unethical for him to be my physician.

I shouldn’t get involved with him. But waiting for my test result has been so stressful that I often find myself getting drunk and ending up in his bed. He gently grabs my hand and whispers, “Are you okay?” That’s become his constant question. Almost recited.

It’s been almost two weeks since my genetic test and I have one day left until the result is out. Although specific procedures vary among testing centers, genetic testing for Huntington’s disease generally involves several sessions, including pre-test genetic counseling, neurological examination and psychiatric interview, that can last for months. The genetic test itself is a simple blood test. The blood sample is sent to a laboratory where DNA testing is performed to check for the expanded CAG repeat within the Huntington gene. My fate depends on these three little letters. If the CAG repeat size is less than 35 repeats, I do not carry the genetic mutation for the disease. But if I carry more than 40 CAG repeats, I test positive and am cursed with the devastating illness.

I often wake up gasping, my eyes hunting frantically for the family I’ve lost and the family I have decided not to have. This cursed disease can extend its claws and prey on the next, innocent generation. Even though I’m not religious, I often find myself praying to all the gods in every religion in hope that one of them can overturn this curse. I try not to show my concerns but my eyes always betray me. Sujin often tells me that I can still have a family if I want to. He says that I can get what he calls ‘Test tubes babies’ through pre-implantation genetic diagnosis. I respect those who choose that path but I still don’t want my children to witness my mental and physical deterioration like Awut and I went through with our mother. I stare at the wall across from us, lost in my nightmare, and Sujin has to tap my foot with his to snap me out of it.

“I’m fine,” I say as I look away. Sujin develops the habit of stroking my hair and kissing my tears away. He runs his hand down my scarred back. Even though I received this unwanted art piece years ago during the fire my brother started to rescue our mom from the shaman, the smell of my burning flesh still haunts me. My tender skin has toughened into an unnatural patch of quilt work. I despise the pattern but Sujin always reassures me that I’m beautiful. A labored breath escapes my lips and I snap at him. “Stop.” The lump in my throat threatens to choke me. I say coldly, “I can’t be bothered with menial displays of affection right now when I have so much at stake.”

He removes his hand from me and pretends it doesn’t bother him. “If that’s what you want, Orasa.” His words, meant to be mature and comforting, instead sound faker the more he says them. “Well, I’m here if you want to talk about it, birdie.” His cheeks flush when he uses my nickname. The hint of a smile appears on his face, pulling me in with its irresistible warmth. But this time, he doesn’t try to hug me. He doesn’t reach for me or scoot closer to let our shoulders touch. He doesn’t stroke my hair, whisper reassuringly into my ear or rest his head against mine. I hadn’t realized how much I’ve grown to crave these little things he does.

I hear humming on the bed and I turn to Sujin. “Do you hear that?”

He looks at me, confused. “Uhh…I don’t think so.”

I grunt, “I swear I heard something.” I search through the sheets and finally find my vibrating phone.

I pick it up and hear a girl’s panic voice. I recognize it immediately. It’s the daughter of the acid assault patient I met in the emergency room. “D-doctor,” The girl sniffles. “Are you free right now?”

I look at the clock. It’s 4 in the morning. I have to perform a surgery in a couple of hours but the girl’s shaky voice prompts me to tell her otherwise. I reply, “Yes I’m free, kiddo. Where are you?”

“At the hospital,” the girl pants. It sounds like she’s running away from something and I can sense fear in her voice when she adds, “My father’s here. Please help me!” I hear the girl screams before the line goes quiet.

“Hey, kiddo! Hey!” I yell into the speaker but I hear no response. I try to redial but I go straight to voice mail. I immediately jump off the bed and scramble to get dressed.

Sujin asks, “What’s going on, birdie?”

I fumble to put on my buttoned clothes. I reply, “I just need to go somewhere.” My hands tremble slightly. It must be from my lack of sleep. “Can I borrow your car?” I pull out the drawer and grab his car keys before Sujin can reply.

He gets up from the bed. “What’s going on?”

“I don’t have time to explain!” I respond a little too aggressively and Sujin seems taken back by my outburst. I pace towards the door and mumble. “It’s just something I have to do.” I look at his befuddled face one last time before rushing out of the apartment.

I get inside the car and hit the pedal. I hit the pedal harder, wanting to go faster and wanting to scream, break something, rip out my hair, scratch my skin off or throw a punch through the window. But, I’m stuck. Bangkok traffic is betraying me. The girl’s scream still rings in my ears. Why is she running? Is her father abusing her? I’m so angry at the injustice the girl is facing. Why isn’t anyone at the hospital helping her? Why doesn’t anyone care? I try to redial the girl again but I still get no response. The burning rage inside me is tearing through me. I feel possessed, a puppet strung to the sadistic hand of a devil.

I finally arrive at the hospital’s underground parking lot. It’s mostly empty, except for a few cars, and it reeks of rotten sewage smell. There’s a glass door at the end that leads me to an elevator and a set of stairs. I run towards the door and swing it open. I bang my finger against the elevator’s button repeatedly. I tap my foot, impatient at its snail pace. It’s taking too long. I decide to use the stairs instead.

As I rush upstairs, I misstep and descend downward. I skid down a couple of steps on my back before I have the presence of mind to stop myself with my palms. I ease myself up to sitting, and I can already feel a nasty series of bruises blossoming on the knobs of my spine. I twist around to examine the stairs, expecting to blame some kind of obstruction. There’s nothing.

When I look up to the top of the stairs, I find the girl I’ve been looking for running towards me. How did she get here? Did she know I’d be here? Logic tries to reason with me but it quickly vanishes when I hear the girl screams.

“Help me!” The girl whimpers, “Please, doctor!”

I lift each arm up and notice that both my elbows are scraped and bleeding. But I don’t have time to examine it. I stand up and grab the girl’s hand. We need to get out of here before her father catches up.

“Where are you taking my daughter?” I hear a chilling voice echoing through the parking lot. I turn around to find a man eyeing the girl like a prized piece of prey. The man’s voice turns eerily calm as he walks towards us and politely says. “Doctor, this is a family affair. Please turn my daughter over. I just need to teach her a lesson that’s all.”

I take a deep breathe and say, “Sir, I’m sure whatever conflict you have with your daughter, we can resolve it over coffee.” I try to distract the man while keeping my chin down and eyeing the girl beside me. But, the first punch glance my chin. I notice too late it is a feint though and the second punch doubles me over and expels the last bit of choked air from my abdomen.

“Please don’t hurt the girl,” I say, lying on the concrete floor. I feel another blow to my guts. My eyes plead for the man to leave his daughter alone. Instead, the man grabs the girl by her hair and starts dragging her away from me. The girl screams again and again as he tugs her away. As if to mock the dire playing inside my head, the girl’s scream plays a familiar dance tune that reminds me of the times I screamed for help when my father punished me as a child.

I cough out blood. “Stop!”

The man stops walking and he turns around. He lets go of the girl’s hair. His face turns red at first but then his lips curl into a vicious, animalistic smile. I can’t decide whether to let my eyes rest on that wicked smile or on the girl’s teary face. Another blow throws me across the floor and my abdomen blazes with pain. I slump against a wall but bear the punishment silently. It is just another bruise. I have dealt with them before. “I heard you’re a surgeon,” the man finally speaks up. “I wonder what kind of trash you’ll be if you can’t operate anymore.”

He eyes my hands with a predatory gaze. Understanding comes later. “No! Please No! Not my hands! I beg you!” I scream but here, in the empty parking lot, no one can hear me.

***

I limp across the hospital lobby, cringing at the smell of puss leaking out of my bruised hands. I hide my hands behind my back when hospital staff members greet me. I don’t have time to explain my condition as I drag my feet across the floor. I need to get to the security room. There must be a camera in the parking lot. If I’m able to obtain the footage, I can use it as evidence to charge the girl’s father with domestic abuse. Even if the man bribes the police, hard evidence like video footage of him beating the girl up will surely get him arrested.

I’m so close to my destination when I see one of my surgical residents runs towards me. “Doctor, you’re here! We’ve been paging you all morning. You have a surgery to go to.” When he approaches me, he gapes. “Doctor…Did you get into an accident?”

He tries to examine the wound on my face but I push him away. I wince while doing so and the resident notices my bruised hands. His eyes shift between my hands and my face. He finally says, “Doctor, I don’t think you can operate in this condition. Don’t worry about the surgery. I’ll call in another attending surgeon while you get your injuries check out–”

“Just prep the OR!” I yell and I notice that everyone in the hospital lobby turns towards me. No one speaks but just stares at me. They’re all mocking me. They’re just like the elders in my village, who think all girls are worthless. Do they think that because I’m a woman, I can’t do my job as a surgeon? I despise the patriarchy in my country. White-hot rage consumes me, the pressure building up and pressing against every cell in my body. I’m sure I’ll physically explode if the rage doesn’t leave me. I turn to the resident and growl, “I said prep the OR! I’ll be there in a minute!”

I don’t bother to see his reaction but continue limping down the corridor. I find myself in the dark security room with the only source of light coming from sets of computer monitors hanging above the long metallic table. I point my finger to the screen and demand, “Check the underground parking lot about half an hour ago. A man was abusing my patient’s daughter.”

The two slightly obese guards stare at me. They’re holding grilled meatballs sticks in their hands and their mouths gape in confusion. They seem inexperienced and unsure of what to do. I roll my eyes. I ram one of the guards against the wall with my elbow and press him deeper into the wall. He drops his food on the ground and I yell, “Check it now!” I eye the other guard. He quickly throws his meatballs away and types something on the keyboard. When I see a screen pop up, I let the fat guard off my grip. He rubs his neck with wide eyes, scared out of his mind.

I pull a chair over and sit on it. My elbows rest on the table as I examine the footage on the screen. I point, “There! That’s the man I was talking about.”

The security guards exchange weird glances. “D-doctor…I think there’s a misunderstanding.”

I growl, “What are you talking about?” The footage is clear. It shows a middle aged man beating me senseless and pulling the girl by her hair away from me.

The guards blink confusedly. One of them finally speaks up, “The video…There’s just you in it….” He pauses for a long moment before he mutters, “You’re throwing your fists against the wall.”

What are they saying? That’s absurd. The girl’s father must have bribed these pigs to say all this nonsense. “How much did he pay you?” Heat rushes through me. “Is that why you’re turning a blind eye?”

“No doctor,” The guards raise both their hands defensively. “Please, doctor. Please calm down.”

I push back from the table to stand, but the push is dramatic and forceful, and the chair crashes to the crappy linoleum floor. I stumble over the upturned legs as I back up, and now both the chair and I are on the ground.

I hear mocking laughs leak out of the guard’s mouths. “How dare you make fun of me?!” I bellow. The guards quickly shake their heads and tell me they didn’t. Liars.

Now riding the back of humiliation, my rage accelerates. Ignoring the throbbing pain of my bruised hands, I stand up, lift the chair by two legs, and smash it against the floor. The legs crack off and several of the back spindles break apart. I toss the legless chair to the ground and march towards the guards.

I look at them and grunt, “What’s your name? I have to get to surgery but once I’m done, I’ll make sure I get incompetent fools fired.”

Both of them look at me in horror. They won’t answer me so I look at their nametags. I pull out my phone to jot down their names. But my hands suddenly feel weak. My fingers flick, as if they are doing their tap dance over my phone screen and ignoring my command. I’m concentrating on my fingers as if I’m performing the most difficult surgery in the world. I try to will my thumbs to type down the names of the security guards onto my phone, but no amount of focus will stop them from trembling.

Heat is building inside me, and I’m holding my breath, losing patience, about to throw the stupid phone to the ground. Even though the room has an air conditioner, the air suddenly feels thick with moisture and humidity. The heat is unbearable and I feel a bead of sweat slowly drip down my neck and sacrifice itself to the cotton confines of my already damp shirt. The entire room seems to be slowly melting. I could swear those security guards were a few inches fatter a couple minutes ago.

When I look up, I’m surprised to find the girl standing beside my surgical resident. She has a gash across her forehead. Instead of helping the wounded girl, the resident rushes to me and says, “Please doctor…Please pull yourself together.”

I point my finger at the girl, “Why aren’t you helping her?” Apparently the girl’s father must have bribed the resident too. I spit in his face. “Can you even call yourself a doctor? How much did the acid assault patient’s husband pay you to turn your eyes away from their abused daughter?”

“Doctor…” He’s sweating, frazzled and directing his eyes towards where I’m pointing. He takes a deep breath before whispering, “I can’t see the girl, doctor. Besides, the acid assault patient doesn’t have a daughter.”

My eyes widen. What is he saying? Of course she has a daughter. She is standing right in front of me. Before I can retort, I feel a pair of small arms wrap around me. I look down to find the girl hugging me. She pleads, “Please doctor. Don’t listen to them. To them, I’m just a poor girl. No one cares about a poor, abused girl.” She cries, “To them, I’m invisible. But you care about me. To you, I’m real.”

I look at the girl’s wound across her forehead. It singes her eyebrow and slightly misses her eye. I frown. “Did your father give you that?”

The girl shakes her head. “No.” The girl takes few steps away from me and says quietly, “The shaman did…with a dried stingray tail.” She pauses before speaking in a somber tone. “I tried to stop him from hurting my mom and I got hurt as a result.”

I hear my heartbeat pulsing in my ears, blocking out all other sound except the breath that is raggedly moving in and out of my mouth at irregular, gasping intervals. I cannot tear my eyes away from the girl. The wound across the girl’s forehead has captured me. I cannot comprehend what is happening to me. The connection has to hold. If it breaks, I fear something within me might break as well. I step towards the girl but she takes another step back. I grab her arm and twirl her tiny body around. I pull her shirt up and stumble back when I see a burnt scar running its tongue across her back. I trip over my feet and fall to the ground. I raise my trembling finger towards her and ask, “W-who are you?”

The girl turns around and heads towards me. I push myself backward until I’m trapped against the wall. She looks down to me and puts her palms together in a Thai prayer-like position as if she is greeting me for the first time. She smiles and says, “I’m you.”

For more information on the association of HD and schizophrenia-like psychosis in HD pedigree, please refer to this article.

More

Coping Strategies For Children Living With a HD Parent

The people most affected by Huntington’s disease are not only the patients with the condition but also the people around the person with the disease. This article focuses on the issues of children living with a HD patient.

Difficulties for Children

The majority of children who have a parent with a neurodegenerative disease, including HD, find it difficult to cope because they do not have the maturity and coping skills to deal with this complex situation. Often children are faced with

  1. Feeling Embarrassed

Having an illness in the family can make the child feel embarrassed or ashamed. The involuntary movements caused by HD tend to be distinct and patients with HD tend to be clumsy. This behavior can draw public attention because it is not immediately clear why the person is acting this way. Unwanted public attention and hearing upsetting things about their ill parent can cause a lot of confusion and anger.

  1. Stressful Household

Having HD in the family can cause a lot of stress as a result of increasing demands on the rest of the family members as the symptoms progress. The stress can also arise from the children being repeatedly separated from a parent that needs to be hospitalized for treatment or that is unable to provide consistent care. The eldest child may also have to grow up faster than other children because he or she has to act as a parent to the ill parent and younger siblings (This last point seems a little too circumstantial). An increase in stress may cause the children to feel insecure and anxious.

  1. Maltreatment and Abuse

In some cases, HD patients can become emotionally or physically abusive to family members as a result of the patients experiencing the behavioral symptoms of the condition. Some of the behavior changes as a result of HD that could manifest into abusive behaviors include aggression, disinhibition, hallucinations and mania. For more information these behavior changes, please refer to HOPES article on ‘The Behavioral Symptoms of Huntington’s Disease’: http://web.stanford.edu/group/hopes/cgi-bin/hopes_test/the-behavioral-symptoms-of-huntingtons-disease/#aggression

When children of HD patients are faced with abusive situations a number of problems may arise. Often children will withdraw and isolate themselves as they develop feelings of anxiety faced with an unpredictable environment. These children may also find it difficult to concentrate on a task due to their anxiety levels. Children can also develop behavior problems when faced with situations and feelings that they are not prepared for. Children will often learn maladaptive behaviors and coping tools from their ill parent like temper tantrums, hitting, lying, bullying, and manipulation. Children may also feel anger or frustration due to their family situation, which can be exhibited in physical or verbal aggression.

How to decrease risk factors for the children?

  • Provide Knowledge

The most important thing a family member can do for a child that has HD parent is to educate the child about the disease. Children develop anxiety and worry when they observe unstable behaviors. Explaining to a child that a parent has these behaviors due to an illness may help the child empathize with rather than resent the ill parent. There are many resources for parents on how to talk to your children about HD. For more resources please refer to HOPES article on “Talking to Children About Huntington’s Disease”: http://web.stanford.edu/group/hopes/cgi-bin/hopes_test/talking-to-children-about-huntingtons-disease/

  • Provide a Stable Environment

It is often very difficult to provide a stable environment when one parent is unpredictable and schedules are continually being challenged and changed. However, it is important to try to provide predictability for a child and to commit to a routine. Children need a sense of routine to feel secure.

  • Seek Psychotherapy

Seeing a professional on a regular basis can be quite helpful to the child and all family members. Having a designated, impartial person to talk to and work out the issues with can be extremely beneficial. This can help children feel more supported and understood as they work through difficult feelings associated with having a parent with a neurodegenerative disease.

  • Nurture the Relationship With the Parents

It is important for children to have a positive connection with their parents. Often when a HD parent is unable to properly care for their child due to their symptoms, the relationship becomes strained. Children can become fearful or anxious around their ill parent and even feel unloved. It is important for the caregivers to make extra efforts to maintain the relationship between parent and child so that the child can grow up feeling secure and loved. When a child experiences instability due to a parent’s mental illness, it becomes important for the child to have appropriate role models. If a parent is unable to provide a sense of security for their child or to attend to their emotional needs appropriately, having a stable and secure relationship with another adult can help the child more easily be able to separate a parent’s behaviors due to the illness from negative feelings towards the child.

  • Healthy Peer Relationships

It is helpful for children to have healthy friendships with their peers. This allows them to develop trusting bonds and negotiation skills that will help them to cope with difficult times in the future. Interacting with their friends can help children of HD parents develop a more encompassing view of the world than they would have if kept isolated.

  • Foster Healthy Interests Outside of the Home

Often children of HD parents are not adequately socialized with peers and rarely have the opportunity to partake in sporting events or cultural activities on a regular basis due to lack of organization or chaos in family functioning. It is always important for children to develop their personal interests outside of the family in order to learn how to properly separate and develop a strong sense of identity and self. Children can also learn tools to cope with their daily environment and the stresses of living with a HD parent through the extracurricular activities.

In conclusion, as with any difficulty in life, having an HD parent is much easier to deal with once we have the tools to understand it and deal with it appropriately. With education and coping strategies, children with an HD parent can develop nurturing relationships with people in their lives.

 

Further Reading:

Brown University Child & Adolescent Behavior Letter, 18(7), 1. Leschied, A. W., Chiodo, D., Whitehead, P. C., & Hurley, D. (2005).

The relationship between maternal depression and child outcomes in a child welfare sample: implications for treatment and policy. Child & Family Social Work, 10(4), 281-291. Orel, N. A., Groves, P. A. & Shannon L. (2003).

Positive Connections: a programme for children who have a parent with a mental illness. Child and Family Social Work, 8, 113-122.

More

Eye Tracking Device for HD Patients

Eye tracking devices measure our eye behavior and predict our gaze points or the locations of the objects in space that we look at. The Eye Gaze System is an eye-operated communication and control system that empowers people with Huntington’s disease and other disabilities to communicate and interact with the world. By looking at control keys or cells displayed on a screen, a user can generate speech by visually typing a message or selecting pre-programmed phrases. Currently, this technology is being used to write books, assist education and enhance quality of life of people with Huntington’s and other conditions. Patients with Huntington’s Disease (HD) have poor muscle coordination and mental decline and behavioral symptoms. The eye-tracking device is especially useful for later stage HD patients when communication is a challenge.

Generally, the Eye Gaze System has a specialized video camera mounted below the screen. The video camera observes one of the user’s eyes and the image processing software analyzes the images sixty times per second to determine where the user is looking on the screen. A user operates the system by looking at rectangular keys or cells that are displayed on the control screen, which then activate. Through visual activation, the array of menu keys and exit keys allow the user to navigate the software independently. Through this eye tracking technology, users can operate lights and appliances remotely, control infrared devices such as televisions and stereos, surf the web and send emails, store and play music, organize and view photos and home movies, read books reading tablet, watch online videos, update social media statuses, and use a word processor to write down their thoughts.

For more sophisticated computer access there are several of the above-mentioned “eye gaze” computer systems on the market. Insurance will only fund an eye gaze system if the individual’s speech therapist can document that it is strictly necessary. Most insurance companies, including Medicare and some private insurance providers, will fund a significant amount of the cost of this device (http://www.infinitec.org/insurance-medicare-and-medicaid-funding-for-assistive-technology). Producers of eye gaze systems include DynaVox (www.dynavoxsys.com), LC Technologies (www.eyegaze.com), Prentke Romich Company, (www.prentrom.com), and Tobii (www.tobii.com). However, following Medicare guidelines, people enrolled in hospice or living at an assisted living facility are not eligible for communication devices.

Nevertheless, there are now other options on the market. Most are not fundable via insurance but some options can be found at a lower price. The Eye Tribe (http://theeyetribe.com) costs $99.00. This software enables eye control on mobile devices, allowing hands-free navigations of websites and apps. (It seems like this software has a different target audience – i.e. mobile phone users? Maybe you could say more about this?) The EyeWriter Project (www.eyewriter.org) is a consumer assembled do-it- yourself kit with free blueprints and material costs at $100. This low cost, open source eye-tracking system allows patients to draw on a tablet using just their eyes. PCEye by Tobii (www.tobii.com/pceye) costs 3,900. The technology replaces the standard mouse, allowing the users to navigate and control a desktop or laptop computer using only their eyes. Vision Key (www.eyecan.ca) costs $4,000 and is the latest eye controlled communication that enables users to type and talk with their eyes. The system gives the users a voice by enabling them to control a speech synthesizer in the VisionKey unit or on the computer by looking at the screen. Users look at a specific word, letter or character on the chart in front of their eye and ‘type’ by holding their gaze until a selection is confirmed by a green highlight and a beep.

In conclusion, eye-tracking technology can be helpful for patients with HD or other disabilities. Eye tracking devices are especially useful for patients who have trouble communicating due to their conditions. The research into these devices continues to develop and as new technology emerges additions will be made to this list.

More

Maladies of My Mind – Ch. 3 Drowning

Maladies of My Mind

Chapter 3. Drowning

The cold collar chafes my neck and makes the shivering hard to control. At least I am no longer in the claustrophobic tube, while the machines click and whir around me, listening to a disembodied voice telling me to hold still while I try to convince myself I can still breathe. Even now, when I’ve been assured there will be no permanent damage, I hunger for air. The residents are scheduled to remove the collar around my neck today but it takes them a lot longer than I expected. My patience is wearing thin. I’m not sure if their reluctance is out of their fear of hurting me or just of me, their superior physician.

After I change into my normal clothes and put on my white doctor coat, I slide the curtains that surround my hospital bed open. I am not surprised to find a shapely woman in a voguish red dress and black heels. Kris is my best friend and at one point, even more. But I broke things off with her because I believe serious commitment isn’t an option for me. Kris greets my residents with a traditional Thai wai. She slightly bows, with her palms pressed together in a lotus position and says sweetly, “Thank you for taking care of Orasa.”

The young doctors swoon over her. They quickly press their palms together to accept her wai. A resident adjusts his glasses and scratches his trimmed pepper beard nervously, “Oh– yes, of course. Umm…With pleasure, miss.”

I roll my eyes. I send a dismissal hand signal to my residents. They won’t pay any attention to me but continue to stare at Kris’ smiling syrup lips. I finally find my voice, “Shoo.” The residents turn to me. I growl slightly, “You’re all dismissed.” The minions scurry out of the room.

I turn to Kris and ask, “How’s Awut?” I straighten, tensing. Doctors and nurses nearby were able to subdue him after he strangled me the last time we met. Even though he has just begun to show subtle signs of motor disabilities, my brother’s cognitive and behavioral symptoms are growing rapidly. Coupled with his existing anger issues, the aggression and paranoia symptoms from Huntington’s disease exacerbate my brother’s violence.

It’s been a while since I’ve heard any real updates on Awut’s condition. My eyes scan Kris’ face, searching for clues. I notice that her pupils slightly dilate and her face conveys anxiousness. What is she going to tell me? Or perhaps it’s no news at all and she’s simply going to tell me what she usually does. ‘Not much change, today, I’m afraid, but at least he’s still stable.’ I’ve grown so used to hearing that.

“Is he lucid?” I ask.

“Yes,” Kris says quietly. My pulse quickens. The news is so sudden. One minute my brother is being strapped in bed, losing control of his identity day by day, and now he’s awake and himself. Just like that.

I break into a smile, and before I can stop myself, I pull Kris into a tight hug. She stiffens. I haven’t held her this closely in a long time. She smiles and pats my head awkwardly, but I don’t care. I want to see my brother. “I’m going to the psychiatric ward.”

Just as I am about to head out, Kris grabs my hand and her smile wavers. She says, “Awut told me he doesn’t want any visitor.” My heart twists until it threatens to break. “He just felt really guilty for hurting you. He’s not eating much and has trouble sleeping.” Her voice trails off quietly, “He said he wants some time alone to rest.”

“Oh,” I say lamely. I take in Kris’ words and my fist tightens

“I’m sorry,” Kris replies guiltily. Now that I take a closer look at her face, I notice how stressed out she looks. Her under eyes blacken from the lack of sleep.

Over the course of my brother’s hospitalization, Kris has always been so supportive. I try to come up with ways to thank her but even now that she is standing in front of me; all my thoughts go to mush. I manage, “Don’t apologize. You’ve done all you can for my brother and me. I don’t know what I’ve done to deserve such a great friend like you.” I unclasp my hand from hers. “I have a lot of work to catch up with anyway,” I mumble. I know I’m supposed to take a few days off from work but I need to distract myself right now.

I give Kris a weak smile and numbly walk out of the room. As I exit, I find a surgical resident leaning against the wall. He is holding a red clipboard with notes on it, which I am assuming are lists of patients’ stats from his round this morning. He is also sweating profusely and fidgeting.

“What do you want?” I deadpan. I’m dumping my frustration on the resident.

The resident jumps lightly. He adjusts his glasses and stammers, “I’m waiting for your friend, sir… I mean ma’am…doctor. I-I’d like to ask for her number.”

His stammer annoys me. Typically, I would let it slide. But for a second, I want to smack him in the face with the stupid clipboard just to shut him up. “Do whatever you want to do,” I mutter and grab his red clipboard. I quickly scan through the list of patients’ stats. My eyes stop at the name Sang Boonsawang. I swivel my head towards the resident and raise my voice, “Here it says that the acid assault patient is conscious but still unable to speak.”

I ran the surgical procedures I performed on the patient through my mind. Maybe the surgery wasn’t a success like I thought it was. Maybe the damages to her vocal cords are more extensive than I suspected. Maybe I missed something.

Unsure of what to say, the resident just stares at me. I feel a boiling rage seeps through my dry demeanor. This useless brat is a waste of our government’s resource. “Uhh…I’m not sure…It could be a complication.” He finally clears his throat and says, “There’s another attending physician looking at her case right now.”

I glance over the patient’s room number. 202. Perfect. That’s just two rooms down the hall. I shove the clipboard back to the resident and head to my destination. As I enter, I notice that the room has a low light on, and there are cords hanging down by the patient’s side for the nurses’ call button and the IV solutions. The patient is sleeping; but there is an empty aqua colored glass with a bent straw in it and a half eaten tray of food with the big metal cover that is on the plate.

“Hello, doctor.” I hear a small, squeaky voice. It’s not coming from the patient. Following the source, I bend down to find a little girl with a bob hair cut and uneven bangs. It’s the patient’s daughter I met in the emergency room.

I hear the door propped open and the sound of sensible shoes squeaking on the pristine tiles approach me. The girl raises her index finger to her mouth and whispers, “Shh…” I’m confused at her gesture but I decide to play along. She’s probably just shy.

When I stand up from my crouched position and turn around, I bump into a well-built figure. I inhale his overly strong cologne and recognize the scent immediately. The man speaks with a weird accent, “Birdie, it’s good to see you again.”

I bite my lips, trying to hide my frustration. I didn’t expect to find a psychiatrist; especially the one I had a one-night stand with, as the consulting physician for my surgical case. “What makes you think this is a psych case, doctor?” I ask.

Sujin runs his hand through his hair and says, “The patient has gone through tremendous emotional trauma. It is possible that her loss of speech may be due to psychological reasons.”

Despite our different specialties, he makes a valid point. However, physiological causes should still be considered. The patient could have had a stroke during the surgery that affects her brain’s Broca’s area in the left hemisphere, which typically disrupts the patient’s ability to speak or comprehend language.

I turn my attention back to the patient. There is an electronic machine sitting on a cart with wires leading to it, and a curtain hanging from a track on the ceiling. The patient’s chest is moving steadily and her heart monitor indicates that she is stable. Sujin walks towards me. “I have another consult but feel free to contact me when the patient is awake,” he hands me a small clean-edge business card with his name and number on it.

“You can also call me for other reasons, birdie.” His lips curl into a crooked smile, “I’ll be going to a Thai boxing match this weekend if you want to come along. It will be Champion Veera’s first return to the ring since his leave of absence.”

Nostalgia of my days when I practiced Thai boxing rushes back to me. I started practicing it because I couldn’t take my father’s abuse anymore and I wanted to fight back. I miss the feelings of wrapping my hands with ropes and dipping them in water to harden them. Because ropes are abrasive and able to cut, they’re used to increase blocking and striking power. The elders in my village claim that females are not worthy enough to practice this sacred sport. However, I was able to practice it because I had a hideous boy cut so people mistook me for my brother. As an avid fan, I’m tempted to go with Sujin just to witness how Champion Veera’s first return to the ring will turn out.

But I can’t. Dating leads to attachment and I can’t afford any attachment. It wouldn’t be fair for either of us. I answer professionally, “I’ll contact you if I want another consult, doctor.”

Sujin’s smile falters slightly but he immediately returns to his cavalier tone. “Well, if you change your mind about my latter proposal, you have my number, birdie.” He winks at me before heading out of the room.

At the sound of the door shutting, I hear shuffling noises of skin and clothes rubbing against the floor tiles. The small girl props her head from under the patient’s bed and crawls out of her hiding spot. “He sounds cute,” she says innocently. “I like his accent.”

I ignore the girl’s observation and ask, “What were you doing down there anyway, kiddo?”

The girl rubs her neck sheepishly, “I was hiding.”

“From what?”

“Not what but who,” she corrects me.

“So who were you hiding from then?” I rephrase my question.

The girl sucks in her round cheeks and stares at the ground. Avoiding my eye contact, she mumbles, barely audible, “My father.”

My heart drops. According to the reports, the patient’s husband is the main suspect for the acid assault. However, there isn’t enough evidence and because victims of domestic violence rarely come forward to testify, the husband is still free.

I notice the bruise on the girl’s cheek and say, “Did your father hurt you too?”

The girl doesn’t reply and continues to look down at her feet. I understand her reluctance to talk to me. When I was younger, I avoided answering any question regarding my father’s abuse like a plague because I didn’t want to face more punishments.

I walk towards the table by the patient’s bed and grab the empty glass of water. I pour water into it and return to where the girl is standing. “You should tell the authorities,” I hand the girl a glass of water. “Here, have a drink and then we can talk more about it.”

Her eyes harden and her cheeks turn red. “The authorities don’t care. No one cares. They’ll just say it’s none of their business and that it’s a family affair. They’ll say that my father is just teaching a disobedient daughter like me a lesson. To them, I’m invisible.” The girl raises her voice and pushes me away.

I lose my balance and drop the glass of water. The glass shatters and water spills all over. Immediately, I reach for the napkins and start wiping the floor. I look at the shattered pieces of glass and suddenly stop cleaning. I remember what the elders in my village taught me: how to carry beverage without spilling, how to cleanup after someone else makes a mess, how to be a good girl, how to be a virtuous daughter, how to respect even if the person doesn’t deserve it simply because he is a man and your father, yet still, how not to respect your own self worth. I can relate to the girl’s pain and burden.

I feel a prick on my index finger and notice blood flowing out of it. A broken glass must have cut me. The girl’s eyes widen and she snaps out of her anger. She scoots closer to me so she can look at my wound. She says quickly, “I’m so sorry, doctor. I didn’t mean to push you. I’m so sorry.”

“Don’t’ worry about it, kiddo. It’s just a little cut.” I smile. I know that if I continue to probe the girl about her father, she will just shut me out. I need to gain her trust first. I reach into my pockets to search for my business cards but I only find the business card Sujin gave me. So I grab a small stack of neon sticky notes and a cheap hospital pen I found on the nightstand near the patient’s bed.

I write my number on it and hand the sticky note to the girl. I say, “Feel free to contact me if you have questions about your mother or just want a friend to talk to.”

The girl reluctantly takes the sticky note. She looks up from it and I notice that her face is filled with concern. “I was so caught up with my own problems that I forget to ask.” She says, “Who’s the man that hurt you the other day?”

I tilt my head slightly, trying to figure out how she knows about Awut’s attack. Then, I remember that the girl was there when it happened. I reply calmly, “He’s my brother. He’s just sick, that’s all.” I keep the tone light. “Don’t worry about it, kiddo. Siblings quarrel all the time.”

The girl giggles, “I think I can relate to that.” She nudges me lightly, “But y’know… if your brother is sick, maybe you can ask that cute doctor for a consult. His accent makes him sounds like a knowledgeable British knight.” The girl reaches into my pocket and pulls out Sujin’s business card. She places it on my palm and beams, “Here. Call him. If you end up going out with him, I want to hear all the details.”

I look at the business card and then at the girl. Even though it’s obvious that the girl is playing cupid, it doesn’t hurt to call Sujin since I have to contact him about the patient’s psychiatric consult anyway. Maybe the girl will open up more if we talk about boys and knights in shining armor or whatever. I smile and say, “Alright, kiddo.”


I walk into the hospital’s café, distinguished from the other parts of the hospital not by walls or a door but merely a change in the pattern of the floor tiles from plain white to different shades of beige. As soon as I step into the café, warmth embraces me. I approach the glass display glittering with meals and desserts. I scan the treats then look up to the chalk menu hanging on the wall above the coffee bar.

“What can I get you?” asks the barista in a bored, drone-like voice. I gesture with my index finger and give him an amiable, apologetic, and closed-lips smile. I’ve been so busy with surgeries lately that I barely have time to go to cafés. Occasionally, my residents get me coffees in hopes of getting on my good side, but I actually don’t drink coffee. I’m going to cherish this moment, where I can place my own order.

“It’s just a drink, birdie,” Sujin whispers, leaning towards my ear. He turns to the barista and chirps, “Double espresso for here please.”

Ugh. Double espresso? Disgusting.

“Coconut Thai Tea Latte for here, please,” I finally place my order.

Sujin and I saunter towards the drink-delivery end of the counter. “Double Espresso,” the barista calls out, leaning over slightly as he places the ceramic cup on the counter. Sujin takes the cup, taking a sip immediately without a thought. That disgusting hot water probably burnt the tip of his tongue.

After I receive my order, I take in the perfect image and aroma of my beverage. Its orange color and sweet taste come from crushed tamarind seeds and condensed milk. When I turn the cup at certain angles, I notice that the reflective sheens from my coconut Thai tea latte catch light and play tricks with anything and everything that screams ‘paradise.’

We head towards the corner table that is squeezed into the small space, shoved amidst the shelves with selections of coffee beans and tea bags. We sit there, taking off our white coats. Sujin’s brown eyes look at me as he excitedly devours his espresso like an addict. When he finally places his drink on the table, he says, “It’s too bad that this isn’t a date. I’ve managed to get us front seats at the boxing ring.”

My ears perk and before my logic can stop me, I ask curiously, “How did you manage to get that?” Darn it. He successfully reels me in.

“Champion Veera is a close friend of mine,” he replies smugly as he returns to his nasty drink. “If you want, I can get you his autograph.”

Interesting. I’ve heard rumors that Champion Veera had to take a leave of absence because he was suffering from hallucinations in the middle of a boxing match. I can’t help but ask, “Are you the psychiatrist that treated his Schizophrenia?”

Sujin stops drinking and says quietly, “You know that’s confidential.” His gaze shifts from my eyes to his espresso and then back to me. “But even if he does have Schizophrenia like the rumors say, schizophrenic patients, like other patients with any illnesses, can live a relatively normal life with proper treatment and support. I have patients with mental disorders who go on to have families and successful careers of their own,” he adds protectively.

I take a couple sips of my coconut Thai tea latte and look to the right to avoid meeting Sujin’s gaze. Suddenly, I feel a vibration in my pant pocket. “Excuse me,” I say quickly and move my fingers downward to pull out my mobile phone.

When I answer it, I hear panic seeping through the speaker. All I can manage are a few “ahuh” and “I’ll be there soon.”

“What is it, birdie?” Sujin asks.

I try to compose myself but somehow the air in the café seems thin. I can’t breath and I feel like I want to vomit. The coconut Thai tea latte is betraying me and is climbing its way up my throat. Restroom is the first thing that pops into my head. I tell Sujin I have to go. I almost run when I see the restroom sign. Forcing my way through the crowd, I lack the time and motivation to throw out redundant apologies but simply use my palms to push people off my way, unable to focus on anything but the growling queasiness rising inside my stomach.

There’s no one in line so I lunge at the stall. Without bothering to shut the door, I lean over the toilet stall and move my head closer to the water. A loathing sound escapes through my lips. I taste the acid, battling its way up my throat. I bring my face closer to the water, arching my saliva between the liquid and me, as if bringing two star-crossed lovers together, our lips longing for a desperate wet kiss. I wonder what will happen if my face unites with the water and I just allow myself to drown in it. I wonder if it will be the happy ending to my tragic story. I used to love happy endings but then I grew up and realized that they are all lies.

I pop my eyes open to find Sujin, kneeling beside me. A string of saliva pathetically hangs from the corner of my lips. My chest heaves heavily and I try to take in as much air as possible. Sujin hands me a brown paper bag with the café logo on it and says calmly, “You’re having a panic attack. Just breath into this.”

I take the bag and put my face into it as I breath in and out. When I finally manage to control the dizzying sensation, I straighten myself up and take some toilet paper to dry my mouth. I push the door open and attempt to get out of the stall only to stumble and hold onto Sujin’s arm for support. I push him away and take two steps away from him but then turn back as if a string has attached me to him.

Maybe I’m emotionally vulnerable right now or maybe it’s the lack of oxygen that impairs my judgment. But I pull Sujin towards me, and my arms wrap around him. I’m so unused to touch but instead of pushing him away, I close my eyes and collapse against his chest. His shirt is rumpled and soft against my skin, and beneath it, his warm body pulses with the faint beating of his heart and it calms me down. A labored breath escapes me. “I-it’s my brother.” I bury my head against Sujin’s shoulder and finally let myself cry. “He tried to kill himself.”

For more information on the coping strategies for HD patients going through depression and suicidal thoughts, please refer to our article.

Next Chapter

More

Maladies of My Mind – Ch. 2 Sticks and Stones

Maladies of My Mind

20 Years Later

Ch. 2 Sticks and Stones

Screams pierce the air as the smoke engulfs my father’s praying room and the collapsing wooden roof mows down a group of monks in orange gown. A strangled cry comes from my throat but no words follow. I lost grip of my icy demeanor, when I see the fire creeping towards me. I inch my hands to my ears, trying to block out the angry sounds of the crackling flames. When I straddle the corner, I swing my right foot up over the side. Suddenly, I feel something catch my feet, dragging me across the floor. Panting and trembling, I try to crawl away from the grasp and grab the edge of the wall for anchor. The image of my mom rushes back to me. I remember her anguish cry and her shaking body as the shaman whips her repeatedly with a dried stingray tail.

“Mom…” I croak into the abyss, heedless of being recognized. “Mom…”

“You need to get out of here!” I turn my head towards the source of wilderment and catch a glimpse of a pair of eyes that scare me to my bones. Father.

I smell the leaking gas nearby and the next thing I know is that my father is carrying my body as he limps towards the door. We are so close…for a moment my father looks at me.

Then, he flings my body and I stumble to the outside world. I open my mouth to call after him but that is when I see an explosive shockwave consuming everything and everyone in its path.

There are ringing in my ears. My head throbs and I want to vomit. But soon the nauseous feeling is replaced by a single sensation—agony. Flame runs its tongue up the back of my body and I can’t feel anything else but the unrelenting burning of my flesh. I open my eyes to see my brother, Awut.

‘You’re not supposed to be here,’ I want to say. Father will be upset if he finds out Awut comes to his praying room. So what is he doing here and why on earth is my older brother holding an oil container and a lighter in his hands?

I jolt awake in bed, gasping. I can barely breathe as my lungs heave in an attempt to suck in air. ‘It’s just another dream,’ I remind myself. I look around frantically. The unwelcoming white walls blind me, and the smell of harsh cleaning solvents fill my nostrils. The floors are black and white tiles arranged in checked patterns, and the walls are interrupted by sets of glass, revealing views of the bustling Bangkok city outside.

I turn to the side and notice a bright neon sticky note on the nightstand with a neat cursive handwriting on it. I pull the note off and take a closer look at it. “Your clothes are folded in the closet,” I read quietly. That’s odd. When I wake up at a stranger’s place, I usually find my clothes scattered across the floor.

After I am dressed, I find the man I came back with last night sitting by the kitchen table. Noticing that the man is reading a newspaper, I try to walk out of the apartment as discreetly as possible. I stop when he says, “Good morning, birdie.”

He has a weird accent and is a lot paler than most people I’ve seen in Thailand. I can’t tell his ethnicity, which is unusual around here, considering how few foreigners there are since the military coup in Bangkok a couple months ago. He looks Caucasian. No wait, Thai. A mix maybe. I don’t know but he’s attractive in a way that distracts me.

Before I could utter a response, the man walks up to me and says, “Are you free this weekend, Orasa? I have an extra ticket to a Thai boxing match this weekend.”

I’m slightly taken back. The man with the weird accent just replaced ‘birdie’ with my actual name and asked me out on a date. I thought I told him not to get attached.

“Look…” I try to remember his name.

“I’m Sujin,” he beats me to it.

“Oh,” I nod slightly, biting my lips as my cheeks color. “Look…Sujin. You seem like a nice guy but I’m just not looking for a relationship right now,” I use the same line I’ve rehearsed many times before to men and occasionally women I hooked up with. I roll my hair back and confine it with a clip I found in my pant pocket. I’m all business now, taking refuge in the formality of my professionalism. “I have to go to work if you’ll excuse me– ”

Sujin pops a grape into my mouth, “Aye, Orasa. You’re one rude birdie. First, not remembering my name and now you’re going to leave without eating my home cooked breakfast.” Taking advantage of my befuddlement, he grabs my hand and tugs me to the kitchen table. He places me on a seat and heads to the kitchen. He returns with two plates of breakfast and says, “I couldn’t recognize you at first but now that you roll your hair back like that, I think I’ve seen you at the hospital before.”

I flinch. Is he a patient? Or is he a hospital staff member? This is bad. What if he’s one of my co-workers? I gulp. I tend to forget a lot of faces at work; and I don’t want to break my personal rule of not getting involved with someone I work with. I speak casually, “So, are you a patient?”

“No, I’m a doctor,” he replies.

I pretend to act cool. “I see. So, what department do you work in?” Please not surgery. Please not surgery. I chant mentally.

“I’m the new attending psychiatrist,” he adds enthusiastically. “I just moved here from the States. I’m originally from the UK though. My father’s British and my mother’s Thai. To be honest, I’m quite excited to back in my mother’s home country.”

He gives out too much familial information for my taste. Nevertheless, I’m relieved. At least psychiatrists and surgeons in Thailand do not interact that much and I probably won’t see him again. Soon my relief turns into confusion. I look around the room and notice Sujin has a nice apartment. From what I recall last night, he has a fancy sports car too. I blurt, “How much money do you make?” A long silence follows and I break it, “I mean, this isn’t the States. Thai people don’t use shrinks. Shouldn’t you be broke or something?”

He stares at me. I didn’t mean to offend him because I was actually curious. But it’s probably better this way. No strings attached.

Finally, he smiles and pushes a plate of breakfast towards me. Thai people tend to eat porridge for breakfast but I notice that Sujin has prepared a Western style breakfast. He made me bacon and scrambled eggs with grapes in one corner of the plate and steamed vegetables in another. I look up and notice that he isn’t eating his breakfast but using his fork to separate the corns, carrots and broccoli from the vegetables mix into their own smaller piles based on the types of vegetables. Without looking up from his plate, he says, “Oh, don’t worry about it, birdie. I have Obsessive Compulsive Disorder. I’m better now though. I used to pop tons of pills.”

I’m surprised at how casually he reveals this private information. Finally, I manage a question. “How can you admit it so easily?”

He looks up from his plate and returns my gaze. “Would you hide the fact that you can’t produce insulin if you have diabetes?” He explains, adding quietly and sitting straight, “Psychological actions can result from of physiological causes; and I think we shouldn’t treat mental illness differently from other diseases.”

I take in his words but don’t say anything. I look at my breakfast and take a spoonful of steamed vegetables into my mouth.


The hospital corridor is stale and the air has a tinge of bleach. The walls are white and are dented in places from the patient trolleys that have countlessly bumped into them. The portraits on the walls are prints of the Thai royal family and above the sliding glass doors is a slim red plastic sign with bolded white letters that say ‘Emergency Room.’

Wearing my blue surgical scrubs and surgical cap, I am standing by the Emergency Room’s entrance and waiting for my patient to arrive. The patient is being transferred from the outskirts of Bangkok to a city hospital. The patient is a thirty-seven year-old female victim of an acid attack. According to the police reports, the jealous husband is a suspect. However, there are no witnesses other than the victim, who is in critical condition. The medical effects of sulfuric acid to the face are extensive. The severity of the damage depends on the concentration of the acid and the time before the acid is thoroughly washed off. However, according to the paramedics’ reports, the acid has rapidly eaten away the patient’s skin, layers of fat beneath the skin and underlying bone. The patient’s skull is partially deformed and her nostrils are closed off completely due to destroyed cartilage. She has also inhaled the acid vapor, which exacerbates the restriction of her airway.

I close my eyes for a moment, trying to conjure and mentally prepare for all the complications that could arise during the surgical procedure. Not everyone appreciates the attractions of surgery like I do. When I was a medical student in the operating room for the first time, and I saw a surgeon press the scalpel to someone’s body and open it like a juicy peach, there were two types of reaction. You either gagged in disgust or gaped in awe. I elicited the latter reaction. It is not just the blood and guts that entice me. It is the idea that a mere human being could have so much control over someone’s life with just a scalpel in her hand. Maybe I have no control over my own life and that’s why I am so addicted to the control of over someone else’s.

I open my eyes when I feel a tug on the hem of my surgical scrub. I look down to find a small girl with a bob haircut and uneven bangs, which look like she may have cut them herself. She’s carrying a leather bag pack that seems too heavy for her spine. There’s also a cotton tag on her public school uniform that says she is a seventh grader. However, her malnourished appearance and small stature make the girl looks like she’s no more than ten years old. “Doctor,” she tugs the hem of my surgical scrub again. The bruise on her round cheek and the cut mark across her forehead grab my attention.

“Hey kiddo.” I crouch to the girl’s level and say, “Let me take a look at your forehead.”

She flinches away at my touch. “Please help my mother first, Doctor.”

I cock my head curiously, “Where’s your mother?

The girl raises her tiny hand and points her finger at the Emergency Room’s sliding door as a patient trolley rushes through it. The paramedics shout, “Incoming!” I’m not sure how the girl managed to get into the hospital before her mother but she is the daughter of the patient I have been waiting for. I rush to the trolley and a group of surgical residents flock after me.

“Who’s the attending surgeon in charge?” the paramedic calls out and looks at the surgical residents, who happen to be all male. Even though medical schools are trying to recruit more women to create diversity, trauma surgery is still a heavily male dominated field.

“I am,” I announce.

The paramedic turns to me, surprised to hear a female voice. He regains his composure and speaks professionally, “This is Mrs. Sang Boonsawang. 37-year-old female. Victim of an acid attack.”

I look at the patient and realize that the damage may have been more extensive than the report. Her ear cartilage is partly destroyed and deafness may occur. Her eyelids are burned off, leaving the eyes extremely dry and prone to blindness. Her mouth becomes shrunken and her lips are partly damaged, exposing her teeth. She may lose her mouth’s full range of motion. Eating and speaking can also become difficult. In addition, she faces the possibility of septicemia, renal failure, skin depigmentation, and even death.

I look at one of the residents and say, “You.” The resident turns his attention to me. “Prep OR 7 and scrub in.”

I turn to another resident. “And you.” I look around the Emergency Room, trying to find the patient’s daughter but have no luck. I turn back to the resident, “Find a 13-year old girl who looks more like she’s 10. She has a laceration to her forehead. Make sure she’s all stitched up.”

The patient is wheeled into the pre operative area of the theatre suite. She is convulsing in pain and trying to cry out but no voice follows. It is possible that her vocal cord is damaged as well. Her arms and legs are secured to the operating table with bonds that are strong but padded so she won’t hurt herself as she struggles.

“Relax, I’m here to help you through this.” The anesthesiologist says. Because the patient’s nostrils have been damaged, giving her an anesthesia through a gas mask isn’t optimal. Instead, the anesthesiologist inserts a small plastic tube into one of the veins on the patient’s backhand. He then puts a blood pressure cuff around the patient’s arm and attaches ECG electrodes on her chest.

The patient is then wheeled into the operating theatre and slides onto the metallic table. She is still awake at this stage. “We’re putting you to sleep now so you won’t feel any pain,” says the anesthesiologist. It will take about two minutes to get the equipment plugged in and for the patient to fall asleep.

Her breathing finally slows down, indicating that the anesthesia worked. I turn to one of the scrub nurses and stretch out my gloved hand, “Scalpel, please.” The nurse hands the sharp object to me. I press the instrument to the patient’s body and open her up like a juicy peach. I am in control now.


The surgery is a success. The patient is transferred to the recovery area for a short while, and then returns to her room on the ward. However, she is still in critical condition and complications may arise during her recovery. Even if the patient recovers physically, acid assault survivors usually face social implications. Such attacks typically leave victims handicapped in some ways, rendering them dependent on either their spouse or family for everyday activities, such as eating and running errands. In some cases, acid victims are psychologically persecuted. Despite having operated on a few acid assault cases myself, I rarely see the issues covered in the news. The media overwhelmingly avoids reporting acid attack related violence. If covered, the description of the attack is minimized and sometimes the media blames the victims, omits women’s voices, and treats husbands who commit these crimes sympathetically.

I enter the waiting room, which is empty except for the victim’s daughter sitting on the hospital bench. She appears to be engrossed in a hardcover government issued biology textbook. I remember reading an earlier edition of the textbook when I was in middle school. When the girl hears me approaching, she asks without tearing her eyes away from the text, “Is my mother going to be alright?”

“The surgery was successful but we still have to watch out for any sign of complications,” I say. The girl nods but continues to read. Usually, I would leave the room at this point. I’ve done my job as a physician by telling a relative about the patient’s condition.

However, I find myself trying to strike up a conversation with this girl. I ask, “How did you manage to get to the hospital before the paramedics came in with your mother?”

Once again, the girl speaks without looking up from her textbook, “I was at school when it happened. I heard that it was severe so I rushed to the nearest city hospital because I knew the local clinics wouldn’t be able to handle it.”

Impressed, I can’t help but compliment her. “You’re one smart girl.”

The girl finally looks up to give me an appreciative smile. That’s when I notice the gash across her forehead. I thought I told the resident to stitch her up. The sight of the girl’s bleeding forehead sets off my rage. I’m going to make that resident’s life a living hell. I look down to the girl and say quietly, “Wait right here. I’ll go get a suture kit to patch you up real quick.”

I smile reassuringly and turn around. I look up to find a brawny man wearing a hospital gown. He resembles a bear more than a man. Despite the patchy, unkempt beard and thick glaze over his eyes, his eyes bear a striking similarity to mine. His hand traces the hospital wall slowly and he limps towards me. Suddenly, he trips and I immediately rush to his side. Just when my lips are forming his name, his fingers lock around my throat and he says in a deep voice, “It’s good to see you again, sister.” He spits out the last word like venom. His grip tightens. Oxygen is sucked out of my lungs and I am losing the battle to hold onto my consciousness.

“Sticks and stones will break my bones

But words will never hurt me”

I first heard of the Sticks and Stones children’s nursery rhyme when a group of English missionaries came to our village when I was in pre-school. We call these towering, pale foreigners ‘Farang,’ which is also a Thai word for the guava fruit. I was curious as to why we call them guava because the two bear no similarities at all. One is green and delicious. The other is white, at least the ones I have seen when I was younger. Most importantly, I doubt that a foreigner tastes as a good as a guava. After days of burying myself in the public library, I finally learn that foreigners are called Farang because the French were the first Europeans to establish ties with Thailand. I guess the words Francais and Farang sound close enough that they become interchangeable.

Even though it has been years since those Farang missionaries have taught me the meaning behind the Stick and Stones nursery rhyme, I disagree with the lesson behind it. The rhyme persuades the victim of verbal assaults to ignore the pain that comes from words, to refrain from retaliation, and to remain calm and good-natured because words can’t break your bones.

I have been broken physically countless times at the hands of my drunken father. But those broken bones never hurt me like words do.

There were two times words destroyed me.

The first time was when I learned about the devastating impact my mother’s illness had done to our family and what it could continue to do.

I was lying on the hospital with my back exposed. The nurses had just finished rinsing my burned skin with cool water and left to retrieve antibiotic ointments. I recalled crying in pain at each contact to the open blisters on my back.

I saw my brother, Awut, walked into the room. He approached me and I noticed the dirt stains on his round cheeks. He kept a straight face and said, “I just gave the police a statement about the fire accident.”

The memories of him holding an oil container and lighter crept back to me. I avoided his eye contacts. I said quietly, “An accident huh?” Maybe I was mistaken. Maybe I was imagining things when pain from the fire consumed me.

“That’s what I told the police,” Awut replied stoically. I sighed in relief. Of course, my sweet older brother who cried at the most mundane things wouldn’t do the unimaginable. “But just between you and me,” My train of thought suddenly stopped when Awut continued in a cold, distant voice, “Father shouldn’t have left his lighter within a child’s reach.”

My face, the once blank canvas, broke – first into confusion, then shock and finally realization. My mind witnessed wave after wave of emotion; as it crashed down, my lips trembled slightly. Before I could utter a word, I heard the door propped open. My brother’s back straightened and he asked, “Doctor, do you have news about our mother?”

The doctor led Awut to a seat and cleared his throat, “You might want to sit down for this.”

I blocked out all the noises in the room and just stared at the glass of water on the table stand nearby my hospital bed. Through the water in the glass, I saw a distorted image of myself. My eyes traveled up to where a wound licked across my forehead, singeing away my brows but missing my eyes. The scent of blood curled into my nose, down into my throat, squeezing it tight with despair. I was brought back to the memory when a shaman whipped my mother repeatedly with the dried stingray tail. I tried to stop him and I was hurt as a result. But it didn’t matter at that time because my mother was alive. All those years that I thought she ran off with another family was a lie. She was actually sick. My father had hidden her away from the world because he was ashamed of her mental illness. When her conditions worsened, he paid an undertaker to fake her death so he could hire a shaman to drive my mother’s illness away without suspicions.

But by the time the hospital ambulance arrived after the fire, my mother’s health had deteriorated due to constant beating and starvation. Not long after her hospitalization, she passed away. I also found out from the doctors what mental illness my mother was suffering from. I had to ask the doctor to repeat the name of the disease twice because it was in a foreign language. The disease was named after a Farang physician, George Huntington, who wrote about the illness in the late 1800s.

The doctor concluded that my mom had Huntington’s disease; a rare mental illness often described as a deadly combination of Alzheimer’s, schizophrenia, and Parkinson’s. The doctor explained that in Thailand, there has only been one reported case in the past twenty years. However, this low number may result from under reporting and the extrapolated statistics of Huntington’s disease prevalence in Thailand is approximately 2,000 people.

A few key words seared into my memory regarding this disease:

  1. 50 % chances of inheritance
  2. Symptoms begin at age 30 to 50
  3. Losing control of mind and body
  4. Incurable

Early signs of the disease vary greatly from person to person. Behavioral symptoms may include mood swing, anxiety, memory loss or hallucinations. Physical symptoms progress from clumsiness and loss of coordination to the point where speech is slurred and vital functions, such as swallowing, eating, speaking, and walking, are abysmal.

I took in the doctor’s description of the symptoms. My first response was denial. From what I remembered, my mom was an adventurous woman who loved to take Awut and me to swim in the lake. But then I recalled how her body twitched uncontrollably when I encountered her in father’s praying room. Coupled with Awut’s account of her incorrigible speech, the doctor’s description of the disease my mom was suffering from sounded accurate.

My second response was anger—anger of what my mom had to go through and anger towards my father for putting her through more suffering. But selfishly, I was most angry at the fact that my brother and I could inherit this devastating curse at just a coin flip away.

I recalled the doctor saying there’s a genetic test I could take to see if I carry the gene for the disease. Since there is no cure anyway, I didn’t want to know. But, my brother believed that knowledge is power.

The second time words destroyed me was two weeks after Awut’s eighteenth birthday when he told me his genetic test came back positive.

For more information on Huntington’s Disease and its symptoms, please refer to this our articles.

Next Chapter

More

Table of Contents: Maladies of My Mind

hd monster2

Disclaimer: Even though the image above depicts the monster holding a U.S. coin, Huntington’s disease affects families around the world. It is just not often talked about. Maladies of My Mind is inspired by interviews of Thai individuals affected by different mental illnesses, but it does not cover the entirety of a Huntington’s disease experience in Thailand. Significantly, the main character and her brother suffer from paranoid schizophrenia-like symptoms, which occur in a minority of Huntington’s disease patients. The story aims to explore themes of abuse and domestic violence in hope to shed light on the negative consequences of what mental illness stigma can do to a family. Nevertheless, these experiences are not necessarily synonymous with all mental illness or Huntington’s disease experience.

More

Exorcism and Mental Illness Across Different Cultures

Huntington’s Disease (HD) is a rare inherited disorder that breaks down nerve cells in the brain over time, affecting the patient’s behavior and movement. In its early stages, HD symptoms can vary among patients. Mood swings are a common early symptom and HD patients may become depressed, irritable, apathetic, or angry. In some cultures, psychological disorders that also have a motor component, such as Huntington’s Disease (HD), Tourette syndrome and schizophrenia, can result in the individual being labeled as possessed. The notion of possession, in which a person becomes demonized, possessed, or controlled by a demon, was present in many ancient religions and is still present in many beliefs today. To drive out the demons or evil spirits, family members or religious figures turn to exorcism. An exorcist is typically someone of religious faith who is thought to be able to channel benevolent powers and cast out evil spirits from otherwise good people. The concept and practice of exorcism crosses cultural and historical boundaries.

In Catholicism, priests undertake prayers for repentance, divine intervention and protection before engaging in ceremony. The patients then undergo counseling to enhance their spiritual life after the ritual. Buddhist exorcism involves prayer and meditation to persuade the spirit to leave the body. Taoists mainly use chanting, praying, and physical movements to drive away the evil spirits. Among Muslims, the belief of demon possession is not universal. Mr Nazirudin Mohd Nasir, head of the Office of the Mufti at the Islamic Religious Council of Singapore, said, “We believe there are unseen creations of God such as the ‘jinn’ or spirits.” However, he added that although, “One could possibly be possessed by the unseen, but one should not simply blame everything on this. In fact, some people do not believe in possession, although they believe that the unseen exist.” For Muslims who do believe, however, he said that there were those in the community who specialized in the exorcism ritual.

As a case study for practiced exorcism, this paper will examine North Thailand Hill tribe Shamans. There are Lahu, Lisu, Karen, Hmong and Lawa hill tribes. Among these hill tribes of Thailand that migrated from southern China, Laos and Burma, Lisu hill tribal villagers believe in exorcism and possession. According to their belief, there are two beings that can take possession of a person, including the ‘phi pheu’ or tigers as well as ‘phu seu’ or vampires. The hill tribe believes that ‘tai’ or black magic that is implanted in the body of the afflicted person by another person causes many of the illnesses they experience. This object must then be sucked out by the shaman in order for the person to be cured.

While the process of exorcism varies across many cultures, the act of restraining the patient is common; and these restraints can sometimes lead to injuries. The illusion that exorcism works on people with a disease experiencing supposed symptoms of possession can be attributed to the placebo effect. However, there are 370,000 reported cases of people killed during exorcism and another 310,000 reported injuries. These numbers are an underestimate as there are still unreported fatal cases in many rural areas of developing countries where mental illness is stigmatized. Scientifically, demonic possession is not a valid psychiatric or medical diagnosis recognized by either the DSM-V or ICD-10. Patients with symptoms associated with physical or mental illness can be mistaken for victims of demon possession. For example, a shaman beat a mother of two from Thailand with a dried stingray tail because the family had suggested it as a way to rid of her mental illness (later revealed to be a form of motor disorder). Despite her efforts to flee, the shaman abducted her and continued until she died. Even though the shaman was later on charged with murder, this case study shows that the process of exorcism can be fatal and victims can been bound, beaten, burned, starved, and even tortured.

In conclusion, it is difficult to come by documentation of any outcomes of official exorcisms, harmful or beneficial. Exorcisms are supposed to be low-key. Although they are not necessarily a secret, exorcisms are not performed in public or in front of press representatives. However there have been reported cases that victims of exorcisms, whom the officials believe to have mental illnesses, had past away from the rigorous ritual. Therefore, it is imperative to raise awareness about treatments for mental disorder in areas where exorcism is prevalent and mental illness is a social stigma.

 

More information:

http://whatstheharm.net/exorcisms.html

http://science.howstuffworks.com/science-vs-myth/afterlife/exorcism5.htm

http://pittsburgh.cbslocal.com/2014/02/12/demonic-possession-real-or-mental-illness/

http://exorcistinrealasiasingapore.blogspot.com/

http://innerjourneys.com.au/north-thailand-hilltribe-healers-tour/north-thailand-hilltribe-healers-shaman

 

More

Maladies of My Mind – Ch. 1 Father’s Praying Room

Maladies of My Mind

Ch. 1 Father’s Praying Room

I see sacred white strings tied around my mom’s ankles and wrists. Although I haven’t seen her since father told us that she left us for another family, I want to see her one last time. The charred body lies before me, burned beyond recognition, but the necklace father gave her years ago is still recognizable. I wouldn’t have believed that the burnt corpse was my mother if it was not for that necklace and my trust in the elders. They say my mom died in a fire accident. She is laid out on a table with her hands held together in a prayer-like gesture, holding a lotus flower and incense sticks. I look down at her blackened face and graze my hands over her necklace. Bits of ash flake off onto my hands. The act alone almost causes me to break mentally. The smell makes me turn my head and gag. My hands start trembling so hard that I push them against my black dress in an attempt to steady them. The undertaker pulls me aside and gently puts a coin in my mother’s mouth. I don’t know what sets off my anger, maybe it’s the ash stains on my hands that I can’t seem to rub off or the fact that my dead mother has broken her promise to never abandon me for the second time. First was when she left me for another family and now she has left me forever.

It’s been seven days since my mother passed away but the monks continue to chant and people in black continue to weep. They take turns pouring scented water over her hands. Most make blessings or ask for forgiveness for past misdeeds. The line to my dead mother is endless and I am waiting for my older brother, who is almost fourteen and a year older than I am but his squeaky, stupid sobs put me in a bloody temper. “Stop crying, Awut. Father will be displeased if he finds his eldest son crying like a little wuss.”

“But that drunken fool isn’t even here, Orasa.” My brother stops crying and those angry brown eyes are now playing tricks in the light coming from the candles situated around the temple. “He won’t even come to Mom’s funeral.” His dirt marked face wears a painful expression and while I am not crying like he is, his face mirrors my own. My dark short hair makes me look more like a boy than a girl; and more often than not, people mistake my brother and I for twins. My train of thought is interrupted when I feel drops of water falling onto my face.

“It’s raining. Let’s go home before father knows we are here,” I mutter. When Awut gives me an accusatory look, I deadpan, “Or would you rather face father’s wrath tonight? I’m not in the mood to get kicked out of the house again because of your stupidity.” My brother looks like he wants to beat the shit out of me. Instead, he sucks in his round cheeks and stares past me. Even though he’s my older brother, I’m the scarier of the two of us and he follows me around like he’s the younger one.

We find ourselves rushing to our house on silent feet. I nearly slip. It has begun to rain and everything turns slick. We make our way to the wooden fence that circumvents our house, and I climb on top of it. As I crouch on the apex of the fence, I look down to my brother and tilt my head to signal for him to follow my lead, “Come on over, you coward.”

He ardently shakes his head. He’s too scared that our old man will be waiting for us on the other side. I roll my eyes. I dangle on the edge of the fence for a few moments before my trembling hands finally let go. My legs crumple beneath me when I land. The impact knocks the breath from my chest, and for a moment I can only lie there on the muddy ground, drenched in rain, muscles aching, fighting for air. Strands of my hair cling to my face. I wipe them out of my way and crawl onto my hands and knees. The rain adds a reflective sheen to everything around me. My focus narrows. Awut and I need to get back into our house before father discovers we went to mom’s funeral. I scramble to my feet and just when I am about to call out for my brother, I hear something. I freeze in my tracks. At first the familiar deep voice seems distant, almost entirely muted by the rain, but an instant later it turns deafening, “ORASA!”

I tremble where I stand. Father. Before I can think of anything else, I see him, a sight that sends terror rushing through my blood –my father, his eyes flashing, materializing through the fog of a wet midnight. “Where were you coming from, Orasa?” he asks, his voice suddenly turns eerily calm.

I try in vain to escape his grasp, but his hands only grip tighter until I gasp from the pain. My father pulls hard – I stumble, lose my balance, and fall against him. Mud splashes my face. All I can hear is the roar of rain, the darkness of his voice. I peer through the fences to see if my older brother is still on the other side. Of course, he is nowhere in sight.

“Stand up, you ungrateful brat,” my father hisses in my ear, yanking me forcefully up. I smell alcohol on his breath. Then his voice turns soothing. “Come now, dear. You’re making a mess of yourself. Let me take you home.”

I glare at him and pull my arm away with all my strength. His grip slips against the slick of rain. My skin twists painfully against his, and for an instant, I am free.

But then I feel his hand close around a fistful of my hair. I shriek, my hands grasping at the empty air. “So rebellious. Why can’t you be more like your brother?” he murmurs, shaking his head and hauling me off away from the wooden fence. “Where did you come from? Did you run off to the funeral? Answer me!”

I scream. I scream with all the air I have in my lungs, hoping that my cries will alert the people in the village, and they will witness this scene unfolding. Will they care? I doubt that. I’m just a daughter after all. Girls are expendable. My father tightens his grip on my hair and pulls harder.

“Come home with me now,” he says, pausing for a moment to stare at me. Rain runs down his cheeks. “Why don’t you be daddy’s little girl? You know daddy knows what’s best.”

I grit my teeth and stare back. “I hate you,” I whisper.

My father strikes me viciously across the face. I stumble, and then collapse in the mud. My father still clings to my hair. He pulls so hard that I feel strands being torn from my scalp. “You look so much like her,” he whispers in my ear, filling it with his smooth, icy rage. “You look so much like your mother.” My father slowly pulls out his belt. I immediately recognize that predatory look on his face. I shut my eyes and pray for the beating to be over soon.

 


 

Later that night, I wake up to the sound of a thunderstorm. Water drips from the leaky ceiling, running in streams and splattering over my head. The electricity is cut short but I can still hear the echo of footsteps heading towards me. “Orasa! Orasa, wake up!” It’s my backstabbing brother.

I mumble, “Go back to sleep, Awut. It’s just a thunderstorm.” My body aches from the beating I received from father. I roll to the side and try to cover my body with the ragged blanket. However, the blanket is too small to cover my feet and I can feel my toes freezing as the wind blows through the cracked windows. As if the wooden floor I’m sleeping on and the water dripping onto my head are not making me cold enough, my brother has to put his cold hands over my face in an attempt to wake me up. Frustrated, I finally turn towards him. “For Buddha’s sake, what do you wa—”

I stop midsentence when I see the look on his face. Despite the darkness, I can see how pale he is. His voice is shaking, “Orasa…I think I did something I don’t think I’m supposed to do. I think…”

I roll my eyes. “Spill it out already.”

He fidgets and rubs his hands together nervously. “I went to father’s praying room.”

“You did what?!” I sit up and grab my brother’s shoulder tightly. I wince slightly at the throbbing pain that runs through my body. My brother looks at me with concern. I know he’s sorry that he didn’t stand up for me; but of course, my cowardly older brother would never do anything about it. I regain my composure. “You fool. You know what father will do if he finds out you went into his praying room.” I grab his shoulder tighter as if I’m trying to force a confession out of him.

“I didn’t go in though,” he continues weakly. “I followed the monks from Mom’s funeral. I saw them heading towards father’s wooden hut in the forest so I thought they were going to pray for mom. I was about to follow them in but then I heard something.” At this point, Awut’s entire body is shaking uncontrollably and his puffy red eyes are starring into empty space. He whispers, “Her voice wasn’t soft and smooth like I remember. But I heard her, Orasa. I heard Mom calling out for us.”

I sit still and stare past my brother’s face. He looks at me and his hands reach to grab mine as if to comfort me. My body is shaking and I think he is expecting me to cry. Instead, I give him a small chuckle. “You fool. I think you ate too much of that free intestines soup at the temple today.” I push my brother away and slump back onto the ground.

“But, Orasa. I – “

“Go back to sleep.” I cover my face with the blanket and mumble. “If you talk about this nonsense again, I’ll beat the crap out of you.”

I hear my brother sighs in defeat as he scuffles back to his sleeping spot. I hear him shifts uncomfortably at first but eventually his breathing levels, indicating that he has fallen asleep. I try to go back to sleep as well but my brother’s words keep haunting me. A fragmented image of my mother comes back. I can barely remember what she looks like. All I can see is that charred face with none of mom’s resemblance at the funeral. Despite the cold weather, my brow is covered with sweat, and my cheeks are wet with tears. I’m breathing heavily. I sit up, brush a wet strand of hair out of my face and rub a hand wearily across my eyes. I feel dumb. Crying doesn’t solve anything. It’s pointless now to go back to sleep so I stand up and take one last look at my brother before I walk out of our room. As I exit, I make sure that father isn’t around. I look first at the stacks of crates in one dark corner, then at the burlap lining the floor, and lastly the little sack of jasmine rice and water sitting between them. Good. He’s nowhere in sight. It takes me a minute to reorient myself and direct where I am heading.

Father’s praying room is situated in a hut in the middle of the woods away from where we actually sleep. He says that the praying room is too sacred to have little brats like Awut and me running around. As I take a step out of our house, the cool air washes over my face and the moonlight drenches the whole night in dark silver. My heart pounds erratically in the way it does after running away from my drunken father when Awut or I have disappointed him. My train of thought halts when I reach a lake our family used to swim in— when we were still a family that is. I hesitate but quickly shake the happy memories out of my head. I turn the corner and head towards a tiny, boarded-up hut with faded inscrutable Thai characters carved on its door.

Gingerly I climb my way over the rusty aluminum roof, and then listen intently for any sound from within. Through the roof’s gaps, I see and hear bald men in orange gowns chanting prayers. I still can’t see the entire view but these men must be the monks my brother told me about. They are sitting in lotus positions and forming a circle but I can’t see what is in the middle of that circle. I hear shuffling noises inside but they are soon subdued when I hear father’s shaky voice, “Please. Please. Please.” I can’t believe my ears. The last time I heard him say such a polite word was when mom was still with us. My pulse quickens. I need to see more. I go from one spot on the roof to another until I finally find one that has a crack between two of its aluminum boards. Father is standing furtively in the corner of his prayer room with his head in his hands. I see him reach for a small family portrait. The moonlight shining through the roof allows me to make out the image of father smiling in the picture. “Please release her,” he mutters. My father suddenly swings our family portrait across the room. His breath comes in ragged sobs, and he claws his scalp in agony. Blood trickles down his face. The sight of it alone triggers the smell of iron filling my nose. My father puts both his hands to his face and I hear him cry in despair.

It’s too dark inside for me to immediately make out the figure in the middle of the monks’ circle. However, I can see that one of the men who was sitting among the monks stands up and walks towards the figure in the middle. He appears to be wearing a different outfit from the monks. Instead of having his head shaved and wearing an orange robe, this man is in a white robe and he grows his hair and beard wildly. He holds out a long sharp object and after squinting my eyes, I realize that it’s a dried stingray tail. I gasp. I have never seen one up close before but the man in front of my eyes is the shaman. The elders said that a person’s illness or drastic change in behaviors is caused by black magic called “tai”. Specifically, it is an object that is magically implanted in the body of the afflicted person by another person. The shaman must then suck this object out before a cure can happen.

In one swift movement, the shaman whips the figure in the middle of the circle with the dried stingray tail and the silhouette shakes wildly. The sound the figure makes is incoherent but I can see the anguish etched into every single crumpled, devastated muscle of the uncontrollable body. It does not take long for me to realize what, or more specifically whom, that body belongs to. The sound of her crying is so foreign that it tears my heart. I’ve seen my mother cry before, but never like this.

 

For more information on Exorcism in Mental Illness Across Different Cultures, please take a look at this article.

Next Chapter

More

Potentials of Telehealth Devices for Speech Therapy in Motor Disorder

Introduction:

Rapidly evolving technology influences our daily lives. In particular information and communication technologies allow us to communicate faster and more globally than ever before. In this article, the possibilities of technological developments in healthcare, particularly for patients with Huntington’s disease (HD), will be explored. Patients with HD often struggle with speech intelligibility, a measure of how comprehensible speech is, or the degree to which speech can be understood., because as brain cells deteriorate in HD, problems may develop in the following three areas: motor control (movement); cognitive (thinking); and behavior. Speech and swallowing problems, due to muscle weakness and loss of coordination, arise when the motor control centers are affected. Cognitive problems include memory, sequencing, new learning ability and problem solving.

Telehealth and Speech Language Pathology

Telehealth is defined as the use of telecommunication technologies both to provide health care services and to enable access to medical information for training and educating health care professionals and consumers. Telehealth applications, could provide solutions to overcome barriers of access to therapy services caused by factors such as decreasing financial resources, shortage of professionals and increasing numbers of patients. Thus, telehealth concerns both mobile and computer’s applications of information and communication technologies, enabling the retrieval, recording, management and transmission of information to support health care.

Studies have suggested that online speech training for patients with motor disorder, specifically patients with Parkinson’s disease, turned out to be effective. In one study, ten patients used videoconferences during a four-week program of intensive training, involving 16 therapy sessions. Patients’ conversational monologue and prompted reading was recorded both before and after treatment Comparison of sound pressure level, pitch measurements and perceptual ratings from audio recordings revealed significant improvements. This study shows that remote diagnosis and treatment of speech in patients with motor disorder has vital benefits, in particular for patients who are less mobile and easily fatigued due to their deteriorated physical condition.

 

E-learning based Speech Therapy

Another study developed an E-learning based Speech Therapy (EST) in 2010. The therapy aims at patients with speech disorders resulting from acquired neurological impairment such as stroke or Huntington’s disease. Particularly in the chronic phase of the patients’ HD, once in-clinic therapy sessions are no longer an option, the lack of speech practice results in diminished speech. An important benefit of EST is to enable the patient to follow a specialized speech-training program in the patients’ home environment. Not only does E-learning reduce time energy and costs normally involved with speech training, the convenience the EST offers allows the patient to attend more therapy and practice speech could often lead to better speech outcomes.

A case study that evaluated EST included 20 patients with Parkinson’s disease suggests that EST is indeed a powerful web-based speech training device with potential efficacy for patients with movement disorder. The patients had completed face-to-face sessions with speech therapist and were able to conduct the training program that the patient was already familiar with, independently. The patients’ speech intelligibility had significantly improved as measured by the percentage correctly transcribed words given to the patient in a random sequence. After 4 weeks without practice, the patient’s speech intelligibility declined. Thus, while EST is a powerful tool, patients must also practice speech or continue with EST lessons in order to maintain speech quality.

In conclusion, these studies show that Telehealth devices can significantly improves speech intelligibility in patients with Parkinson’s disease. This finding is relevant for patients with HD because patients with HD also face troubles related to speech intelligibility. With this in mind, hopefully there will be more development in Telehealth devices that further can improve the quality of life HD patients.

 

Resources:

If interested, here are some of the resources you can look into for telehealth devices on speech intelligibility.

http://integrisok.com/speech-telepractice

http://presencelearning.com/resources/online-speech-therapy-research-summary/

http://www.med-speech.com/product-category/telehealth-skype-sessions/

 

Sources:

Beijer, Lilian J., Toni C.m. Rietveld, Vera Hoskam, Alexander C.h. Geurts, and Bert J.m. De Swart. “Evaluating the Feasibility and the Potential Efficacy of E-Learning-Based Speech Therapy (EST) as a Web Application for Speech Training in Dysarthric Patients with Parkinson’s Disease: A Case Study.” Telemedicine and E-Health 16.6 (2010): 732-38. Web.

Bilney, B., M. E. Morris, and A. Perry. “Effectiveness of Physiotherapy, Occupational Therapy, and Speech Pathology for People with Huntington’s Disease: A Systematic Review.” Neurorehabilitation and Neural Repair 17.1 (2003): 12-24. Web.

Hill, A., and D. Theodoros. “Research into Telehealth Applications in Speech-language Pathology.” Journal of Telemedicine and Telecare 8.4 (2002): 187-96. Web.

Shelton, R. L. “The Genetics of Huntington’s Disease.” Perspectives on Speech Science and Orofacial Disorders 13.2 (2003): 7-12. Web.

 

More

A Case Study: Pregnancy and HD in India

The family members of the HD patients have to cope with the knowledge that the patient will suffer from mental and physical deterioration while accepting the fact that the HD patients’ children are at risk of getting the disease. The risk of develop­ing HD in an offspring of an affec­ted parent is 50%. One such case of full-term pregnancy is reported. It merits atten­tion because of rarity of HD and lack of reports on it from Central India. Therefore, a careful study of family history is indispensable in the assessment and understanding of HD as it enables the prediction to be made for the fetus during pregnancy in 90% of cases. The case report also emphasizes the need of detecting DNA polymorphism by chorion biopsy or amniocentesis to avoid transmission of HD gene to further generations despite of ethical issues, which may be raised.

A Case Report

A 35-year-old Indian woman with history of amenorrhea or absence of menstruation of 9 months and labor pains was admitted in pri­vate hospital. Her pulse was 80 per min, blood pressure 110/80 mm Hg she had no edema. The abdomen examination showed height of uterus to be 36 weeks, tense, tender with moderate ute­rine contractions. Fetal heart was recorded to be normal. She had characteristic involuntary choric movements. The diagnosis of HD was established due to presence of chorea and history of progres­sive dementia and emotional disturbance supported by a positive family history. Her paternal grandmother suffered and died of the same symptoms. The grand­mother had one daughter and four sons, of which the daughter and one son have no symptoms whereas three sons suffered. The age of onset of the patient’s father was at the age of 38 years and death occurred 12 years after onset. In the second son, on­set was at the age of 35 years and he committed suicide after suffer­ing for 10 years. The third son is 40 years of age and still alive but with same symptoms. The patient has three brothers and one sister all less than thirty years of age without any symptoms. The onset of symptoms in the patient was at the age of 30 years. She has three daughters, 17 years and 15 years and a son 13 years old. She delivered normally a healthy male child. The postpartum period was uneventful. The doctors suc­ceeded in convincing her husband to undergo vasectomy.

Discussion:

As the case denotes, normal pregnancy and delivery is possible in a patient suffering from HD. As the offspring are at a 50% risk of inheriting the disease, prediction of this risk is essential. The identi­fication of a deoxyribonucleic acid (DNA) sequence G-8 on chromo­some 4 showed close genetic linkage to HD. Over the years availability of new DNA markers more tightly linked to the HD locus than the G-8 probes has improved predictive accuracy for both pre-symptomatic and prenatal exclusion testing. Pre­natal exclusion testing can be an ideal option for high-risk indivi­duals wishing to procreate as HD trait shows vertical transmission through successive generations. Non-availability of such predictive tests in India and the added economic restrain are the limita­tions. According to a study by Harper on Genetic Testing and Family Structure shows that only when a specific test for the HD gene itself is available it may be feasible to make a prediction for an isolated subject without studying the family unit. Meanwhile families are likely to benefit from prediction along family structures. In conclusion, pre symptomatic and prenatal ex­clusion testing by genetic counseling definitively predicts the risk of HD. But the family structure will remain crucial for prediction, as the identification of HD gene in Central India seems to be some way away.

Here are some of the resources for genetic counseling in India:

  • Genetic And Mental Retardation Clinic
    of Paediatrics, AIIMS, New Delhi-110029
    Phone number: 26588500
  • Genetics Cell, Sri Ramachandra Medical College (SRMC) Porur, Chennai, India
  • Surendra genetics Laboratory, Lloyd’s road, Royapettah, Chennai,India
  • Genetics department, Manipal Hospital, Karnataka.

Reference

Gusella, James F., Nancy S. Wexler, P. Michael Conneally, Susan L. Naylor, Mary Anne Anderson, Rudolph E. Tanzi, Paul C. Watkins, Kathleen Ottina, Margaret R. Wallace, Alan Y. Sakaguchi, Anne B. Young, Ira Shoulson, Ernesto Bonilla, and Joseph B. Martin. “A Polymorphic DNA Marker Genetically Linked to Huntington’s Disease.” Nature 306.5940 (1983): 234-38. Web.

Harper, P. S., and M. Sarfarazi. “Genetic Prediction and Family Structure in Huntington’s Chorea.” Bmj 290.6486 (1985): 1929-931. Web.

Wasmuth, John J., Jeffrey Hewitt, Barbara Smith, Denis Allard, Jonathan L. Haines, Douglas Skarecky, Eric Partlow, and Michael R. Hayden. “A Highly Polymorphic Locus Very Tightly Linked to the Huntington’s Disease Gene.” Nature 332.6166 (1988): 734-36. Web.

More

A Case Study: Pregnancy and Symptomatic Huntington Disease

Although symptomatic HD patients are rarely encountered during pregnancy due to its late onset, the shift toward older maternal age means doctors will more likely encounter pregnant patients with HD. A search of the entire PubMed literature from January 1966 to August 2007 yielded 203 reports focusing mainly on prenatal diagnosis and counseling among healthy individuals at risk for HD. However, none of these reports focused on care issues relevant to our patient with a pregnancy complicated by advanced HD. This article therefore describes the illustrative complicated course of the patient, whom despite the complications, are able to deliver a successful birth with the support of relatives and hospital care.

Case study: Department of Obstetrics and Gynecology (University of Alabama at Birmingham)

A 31-year-old white female was referred at 25 weeks gestation due to unplanned pregnancy. She had a 5-year history of symptomatic HD and was under the care of a neurologist. She had deteriorated significantly over the preceding year, with impairment of communication, swallowing, ambulation and cognition. She had an uncomplicated term vaginal delivery 2 years prior to the diagnosis of HD. She was wheelchair bound with chorea, appeared malnourished, and was on oral liquid dietary. Nevertheless, fetal growth and anatomy were normal.

At 30 weeks, she was admitted in preterm labor but arrested at a cervical dilation of 4 cm after she received pre mature labor suppression drugs and steroids for pulmonary maturation. Due to her diabetes insipidus, she developed an intense thirst despite drinking large amount of fluids and excreting large amount of urine. As part of her symptoms, she also had acute renal failure, elevated sodium level in blood, a free water deficit and an abnormal increase in the osmolality of the body fluids. After she was treated with desmopressin (DDAVP), she had a positive response. After giving birth, the doctors failed to wean the patient off her DDAVP and she was kept on a maintenance dose.

As a result of intractable dysphagia or discomfort in swallowing, her nutritional status deteriorated, enternal feeding or delivery of a nutritionally complete food directly into her stomach, duodenum or jejunum was recommended. Managing tube feeds prove to be difficult because of recurrent tube dislodgment and the patient’s aspiration pneumonia. Percutaneous endoscopic gastrosomy (PEG), or an endoscopic medical procedure in which a tube is passed into a patient’s stomach through the abnormal wall, was used to provide a means of feeding. However, the PEG was unsuccessful due to the distortion of the patient’s stomach and her inability to maintain gastric distention. Hence, the medical team used computed tomography-guided placement of a Dobhoff tube. Antibiotics and total eternal feeding were also initiated.

The patient also developed kidney inflammation due to bacterial infection. Nevertheless, she responded to appropriate antibiotics. At 33 weeks, she developed an inflammation of the fetal membranes due to a bacterial infection and labor was induced. She received epidural anesthesia and spontaneously delivered a male infant with the Apgar scores for physical condition of 8 out of 10. Her central line was discontinued 1 day after she gave birth because of suspected site infection. The patient responded to a broad-spectrum of antibiotics and the PEG tube was successfully placed 5 days after she gave birth and enteral feeds resumed. She was discharged after 12 days after she gave birth. The infant was discharged in good health at 1 month of age.

Discussion

This patient highlights several issues important to pregnant women with impaired mental, autonomic and physical ability such as symptomatic HD patients. While involuntary movements are manageable, the patient’s difficulty in swallowing indicated terminal disease and significant risk for aspiration syndrome and death. Hence, counseling regarding pregnancy termination early in the pregnancy is reasonable. Although prenatal diagnosis is available, it is highly controversial, as termination of pregnancy is rare due to the late, adult-onset of the disease.

Although not applicable to the case study patient, preconception counseling, preimplantation genetic diagnosis could have reduced the risk of a HD affected offspring.

The challenges experienced with gastric tube placement in the patient made enteral feeding tube the only feasible option during pregnancy. Increased abdominal pressure, gastric deformation in pregnancy, sphincter and muscular dysfunction associated with HD, likely contributed to the risk for aspiration and the failed tube placement attempts.

Another challenge was the patient’s inability to communicate effectively, especially regarding painful contractions. Family support was also vital as they received education on examining contractions and assisted with nutrition, mobilization, and activities of daily living and decubitus ulcer prevention. Social work, nutrition and physical therapy services helped optimize this patient’s care.

Although there is no direct link between diabetes insipidus and HD, reports of reduced levels of hypothalamic neuropeptides in animal models of HD suggest an association. The episode of kidney inflammation may be related to the combination of pregnancy, restricted activity and muscular dysfunction of HD, which posed a higher risk for bacteria infection.

The successful result of the case study patient’s pregnancy was possible due to a multidisciplinary effort and strong family support.

Resources:

Hoskins, K. E., A. T N Tita, J. R. Biggio, and P. S. Ramsey. “Pregnancy and Active Huntington Disease: A Rare Combination.” Journal of Perinatology 28.2 (2008): 156-57. Web.

Kotliarova, Svetlana, Nihar R. Jana, Naoaki Sakamoto, Masaru Kurosawa, Haruko Miyazaki, Munenori Nekooki, Hiroshi Doi, Yoko Machida, Hon Kit Wong, Taishi Suzuki, Chiharu Uchikawa, Yuri Kotliarov, Kazuyo Uchida, Yoshiro Nagao, Utako Nagaoka, Akira Tamaoka, Kiyomitsu Oyanagi, Fumitaka Oyama, and Nobuyuki Nukina. “Decreased Expression of Hypothalamic Neuropeptides in Huntington Disease Transgenic Mice with Expanded Polyglutamine-EGFP Fluorescent Aggregates.” Journal of Neurochemistry 93.3 (2005): 641-53. Web.

 

 

More