In July 2008, HOPES researcher Tiffany Wang visited the Huntington’s Disease Society of America Center of Excellence located at the Hennepin County Medical Center (HCMC) in Minneapolis, Minnesota. The Center of Excellence serves approximately four hundred Huntington’s Disease (HD) patients and families from Minnesota, North Dakota, South Dakota, Northern Iowa and Western Wisconsin. Although it was not formally designated as a Center of Excellence at its start, the HD clinic at HCMC has provided care to HD patients since it was founded in 1978. For more about HDSA Centers of Excellence please click here. Today, under the direction of Medical Director and neurologist Dr. Martha Nance, the HD clinic continues to provide patient care through an expanded program. The clinic is staffed by a multidisciplinary team of specialists, some of whom have worked with the HD clinic for over a decade. HOPES had the opportunity to speak with several of the staff members:
- Susan Braun-Johnson, P.T. Physical Therapist
- Sally Gorski, M.A. Speech-Language Pathologist
- Carol Ludowese, M.S. Genetic Counselor
- Mary Morgan LaGorio O.T.R. Occupational Therapist
- Stacey Payerl, R.D. Clinical Dietician
- Dawn Radtke, R.N. Clinical Research Coordinator
- Lena Ross, M.S.W. Social Worker
- David Tupper, Ph.D. Neuropsychologist
The HCMC Huntington’s Disease clinic is located in the Center’s Neurology department and provides patient care through weekly in-house admittance, monthly HD clinics and visits to long-term care facilities. Some of the clinic’s HD patients represent families that have been with the HCMC clinic for three generations. Generational patient care facilitates strong understanding of patient histories and good patient-physician relations.
Every Wednesday, neurologists Dr. Martha Nance and Dr. Scott Bundlie provide care to HD patients through in-house admittance at the medical center. These appointments with the neurologists are generally for newly diagnosed HD patients, genetic testing consultations and medication changes.
Patients who require other services are advised to visit the clinic during the monthly HD clinic days which occur one Wednesday per month. On these days, a multidisciplinary team of specialists offers services including consultations in neurology, physical therapy, speech-language pathology, genetic counseling, occupational therapy, nutrition, neuropsychology, and social services. Dr. Nance lauds the clinic days as being particularly helpful because patients and their families can see several different physicians and specialists without the hassle of scheduling several appointments or coming to the hospital for multiple visits. HOPES members were able to visit Minneapolis on one of the HD clinic days to see how they are run.
Before the HD clinic opens, the staff holds a meeting to determine the needs of incoming patients. Dr. Nance reviews relevant medical history and highlights concerns that may arise for each of the approximately ten patients who visit on clinic days. By holding the pre-clinic meeting, the staff members are prepared for more meaningful interactions with patients. Following the meeting, the specialists disperse to tend to the patients. Dr. Nance greets most patients personally, inquiring about how the patient has been since his or her last visit. Check-ups for the HD patients are usually scheduled for every three months, so most patients have news to tell Dr. Nance whether it is about serious new symptoms or simply a recent family vacation.
In many instances, the patient’s family and caregivers will accompany them to the clinic and can give insight into how the patient is coping with HD. Family members, particularly siblings and children of HD patients, may want to consider genetic testing. Genetic counselor Carol Ludowese talks with family members about their options for genetic testing. She can also give advice to younger family members about options like pre-implantation genetic diagnosis that would enable them to have children without passing on the genes for HD. For more information about genetic testing click here.
Following each of her consultations, Dr. Nance will report back to the rest of the HD clinic team who will go into the rooms to see the patients. The other specialists will then tailor visits and consultations to the needs of the patients and their families. In addition to finding out how the patients are doing holistically, Dr. Nance also asks more specific questions depending on the condition of the patient. Many of these questions are about topics that can be more thoroughly addressed by one of the specialists on the team.
Dr. Nance often asks about the patient’s weight and eating habits. One of the common symptoms of HD is weight loss, and in the later stages of the disease patients’ weights can fall significantly below healthy levels. If patients are showing drastic weight loss clinical dietician Stacey Payerl offers advice on how to maintain a healthy weight. Her recommendations often extend beyond what kinds of foods to eat to how caregivers can encourage food intake by making eating more enjoyable for the patient.
Speech-language pathologist Sally Gorski can also help patients who are having trouble maintaining a healthy weight. Patients find swallowing becomes more difficult as HD progresses, so she can administer a swallow exam to determine what kinds of foods are safe for a patient to eat without choking. To increase calorie intake and overcome swallowing difficulties, physicians often recommend that a feeding tube be inserted into the stomach. Dr. Nance emphasizes that whether or not a patient wants to have a feeding tube is an important issue to discuss early on because when the time comes to make the decision, the progression of HD may make it too difficult for patients to make the decision or convey their wishes to their families. Gorski can help patients and their families learn more information to help decide whether a feeding tube is right for them. Making the decision to have a feeding tube falls under the category of advanced directives, which Dr. Nance thinks are important to bring up to patients even during beginning stages of the disease.
While engaging the patients and families in conversation, Dr. Nance often asks patients to complete several motor tasks. Some of these actions include sticking the tongue out, walking a short distance, tapping the index and middle fingers against the thumb, and looking in different directions without moving the head. Watching the patients’ performances helps Dr. Nance evaluate which stage of HD they are in according to the Unified Huntington’s Disease Rating Scale (UHDRS). Physical therapist Susan Braun-Johnson and occupational therapist Mary Morgan LaGorio can provide more extensive advice to patients about their physical and motor symptoms, as well as ways to cope with these symptoms. For example, assistive devices can help in daily activity and changes within the home, such as installing additional bathroom equipment, can maximize safety. For more information about assistive devices and physical therapy click here.
Patients are also asked about their mental status and, if they are still formally employed, whether they are experiencing any difficulties at the workplace. These questions can help determine how behavioral and cognitive symptoms are progressing. If more thorough examination is needed, neuropsychologist Dr. David Tupper can administer several neuropsychological tests to determine how a patient’s brain is functioning. Results from these tests not only help members of the HD clinic better understand their patients, but they can also be important for determining qualifications for social security disability.
Questions about disability determination and other topics related to social services can be answered by the social worker Lena Ross on the HD clinic team. She can advise HD patients about resources within the community. One of the recurring topics that Lena receives questions about is health insurance. Given the complex nature of health insurance, patients along with families and caregivers often find it difficult to understand how HD patients can manage their healthcare costs. Lena can also answer questions regarding care for the patients who are progressing into the later stages of HD. Many family members of HD patients find it difficult to balance their busy lives with caring for a loved one with HD. It is important for families to recognize that HD patients can receive meticulous care without becoming a burden for their family at home. If a patient begins showing more severe symptoms, it may be safer to consider other options, such as a long term care facility. In this regard, Lena helps HD families learn about and weigh the options that are available to them.
At the end of the day, the HD clinic team meets again to discuss the status of the patients. Many patients are scheduled for another visit in three months or, if necessary, follow-up visits within a shorter amount of time. Although official reports are documented for each patient, the post-clinic session helps the HD clinic staff familiarize themselves with the patients for more individualized care.
For patients who are in the later stages of HD and cannot go to the clinic in Minneapolis, staff from the clinic visit local long-term care facilities to provide care to HD patients. The HCMC Center of Excellence is affiliated with the Good Samaritan Society – University Specialty Center also located in Minneapolis. The facility provides long-term care to patients with several chronic diseases and has a unit for HD patients that can care for up to fifty patients. Dr. Nance organizes monthly visits to the Good Samaritan Society patients. Whether at the HCMC or the Good Samaritan Society facilities, the HD clinic continues to provide comprehensive and personal care to its patients.
Dawn Radtke, Clinical Research Coordinator, spoke with HOPES about the research that is conducted at the HCMC Center of Excellence in Minneapolis. The Center has been directly involved with several clinical studies including some drug trials. Although the clinic is not engaged in HD research on the molecular level, the HCMC is affiliated with the University of Minnesota where many groups are researching HD-related biological mechanisms. Several factors are considered when the clinic chooses which studies in which to participate: time involvement for participants and medical staff as well as whether the clinic has an appropriate patient population for the study. Research is mainly focused on relieving the symptoms of HD.
From Ms. Radtke’s experience with patients, families and caregivers are responsive to the idea of participating in clinical trials. Most HD patients are eager to take part in drug studies because receiving a new drug that is not yet FDA approved may alleviate symptoms. Patients are warned, however, that they may receive a placebo rather than the new drug itself.
HCMC researchers face several challenges when conducting clinical trials. Ms. Radtke tells HOPES that the greatest challenge in managing research at the Center of Excellence is time restraints. Some clinical trials require a significant time commitment from participants and all of the studies require time from the medical staff. Another challenge is keeping study participants involved. Although caregivers and family members are usually able to maintain their involvement in studies, it is difficult for HD patients to continue committing to studies as their disease progresses.
PHAROS or the Prospective Huntington At Risk Observational Study monitors individuals who are at risk for HD but have not received genetic testing for the HD allele. By recording characteristics of these participants over time, researchers hope to better understand the progression of HD. At the HCMC Center of Excellence, Dr. Martha Nance is the Primary Investigator and Dr. Scott Bundlie is the independent rater who separately scores participants to ensure that the data is consistent. Ms. Radtke showed HOPES some of the PHAROS tests that are conducted at the HCMC Center of Excellence. Participants are monitored through performance on a verbal fluency test, the Stroop Test, the UHDRS, and questions about frequency and severity of behavioral symptoms.
The PREDICT-HD study is similar to PHAROS, but enrolled subjects are certain that they have the HD allele. The brains of both participants with HD and participants who are at-risk for HD are monitored and compared to better understand the neurological changes associated with HD.
2CARE is a phase III clinical trial investigating the therapeutic effectiveness of the anti-oxidant coenzyme Q10. Enrolled subjects have responded with enthusiasm to this drug trial. To learn more about HD and coenzyme-Q10 click here.
To learn more about clinical trials like the ones conducted at the HCMC and how drugs are developed click here.
Support and Outreach^
The HCMC Center of Excellence works with the Minnesota Chapter of HDSA to offer support for HD families and caregivers. You can visit the HDSA website for the Minnesota Chapter by clicking here. There are monthly support group meetings facilitated by social worker Jessica Hancock. The groups are held in three different regions for the convenience of HD families: the Twin Cities, Northern Minnesota and Northwest Minnesota. In addition to giving HD families an encouraging environment, the support groups will occasionally have guests from the HD clinic to give presentations about health and medical topics. An annual Minnesota HD conference in September gives these groups an opportunity to meet with each other and hear from others who are closely involved with HD, like healthcare workers.
There are also collaborative efforts within the HD community to hold events to raise money for HD. Among these events are an annual Hoop-a-thon started by a local high school basketball player whose mother has HD. The Hoop-a-thon increases HD awareness and raises funds for HD medical research. Events such as the “Hits for Huntington’s” Golf Classic and “Hunt for the Cure” Charity Paintball Big Game also raise money for HD organizations and research.
Several HD families have taken the initiative to reach out to the community and encourage people to learn more about HD. One family whose inspiring story has increased awareness about HD within their community is the Johnson family. The family cares for Mr. Johnson’s childhood friend, Cory Daniels, who was diagnosed with juvenile HD at the age of eighteen, Cory was only given eight months to live when he was put in the care of the Johnsons. HOPES had the opportunity to speak with Mr. Johnson’s wife, Heather, who worked as a registered nurse at a long-term care facility where Cory lived. Heather reconnected the childhood friends who lost touch after high school The Johnsons were aware that community care is more beneficial for patients than institutionalization, so they worked hard to obtain certification so Cory could come to their house to live. Cory has been with the Johnsons for over four years despite physicians’ predictions that he would only live for another eight months. Through organizations like the HCMC center of Excellence and the Minnesota Chapter of HDSA as well as individuals like Cory and the Johnsons, the HD community in Minnesota hopes to continue providing support to one another and increase awareness about HD.
HOPES would like to thank the HDSA Center of Excellence at HCMC and Dr. Nance for allowing us to experience first-hand a clinic that provides quality care to HD patients. We would also like to extend our thanks to Research Coordinator Dawn Radtke for helping to arrange the visit and the Twin Cities Metro Area HD support group members for allowing HOPES to join their July meeting. To visit the website for the HCMC Center of Excellence click here.