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Stories of HOPES: “Bryan’s Dad Visits Dr. Kealy”

“Bryan’s Dad Visits Dr. Kealy”

Lila made an appointment for her husband to visit the family physician. It is difficult for Ben to confront his feelings and the truth, but during a “physical,” Dr. Kealy’s charm and listening skills manages to open Ben up. Ben talks about the changes he has dealt with and speaks openly about his mother’s mysterious condition. Dr. Kealy tells Ben that his preliminary thoughts are that he has HD, but Ben should undergo genetic testing to find out the truth. Ben takes the information bravely and Dr. Kealy suggests he should talk to a patient of his who has undergone genetic testing already. Ben realizes more than ever how much his wife and family mean to him. This story attempts to underscore the difficulty and the necessity of coming to terms, both medically and emotionally, with the potential of an HD diagnosis.

“Ben! Lila! Good morning and it’s so wonderful to see you!”

Doctor Kealy had been Ben’s family physician for many years now. The man had a contagious exuberance and it spread to his patients like wildfire, no matter their situation. Today, the wildfire had consumed Dr. Kealy’s face, his reddened cheeks commanding attention – not from embarrassment, but from sheer energy.

“We are having such revitalizing weather recently! Don’t you think? How’s Bryan? I imagine his earache went away without too much trouble. Such a healthy young boy – still playing baseball like a champ, I’m sure! Oh yes, just follow me back here. We’ll talk in my office – I apologize in advance for the clutter!

Dr. Kealy was a charmer – friendly and engaging. As difficult as it might be to imagine, he was an excellent listener. Not just to what was said, but more importantly, to things that he knew his patients could not say. When Lila called for the meeting, she didn’t tell him very much about the “problem.” But Dr. Kealy heard what Lila could not put into words – her worry, the relief in talking with him, the entire lingering ache of unknowns he could hear from her without the words. Now that she was here, he could see the sparks of hope all over her face – trying her best to smile enough for herself and her husband. At first glance, he had also read Ben – whose face remained flat like the surface of a deep pond. Something was happening underneath, that he could tell, but what was his responsibility to discover.

They made their way to Dr. Kealy’s office. He hadn’t lied, his office was a mess; the “every-man” qualities of imperfection in Dr. Kealy were strangely comforting to Ben and Lila.

“Oh, I’m so sorry! I forgot to bring another chair in here – doctors can be forgetful too, you know. Lila, please take my chair.”

“Oh, Dr. Kealy, that’s not necce—”

“Lila, I insist.” He encouraged her with a broad smile.

Dr. Kealy brought around his leather padded chair, and brought a foldout chair for himself from the hall. He crossed his legs and kept his hands in his lap. Dr. Kealy felt the tension in the two people who sat across from him: The wife who had so much to say – too much to explain clearly, and unsure where to begin. The husband who had the most to say, but was certain to do the least talking.

“I hope the two of you are comfortable. I just want us to have an informal discussion here; to open up a little to one another. I know I don’t eat dinner at your house, but I do want to say I care for the two of you like family.”

“Thank you, Doctor,” Lila replied, all too happy to break the silence, “I’m sure Ben and I will have no problem at all speaking with you…right honey?”

Ben shifted around in his chair and produced an almost inaudible grunt; a sound that spoke to his uncertainty, but also to his uneasiness about doctor’s offices. Whether or not it was an agreement or a struggling effort to respond, Lila couldn’t tell.

“Well, to begin – Ben. Your wife called me a few days ago and was a little concerned about a few things. As I understand, you haven’t been feeling too well lately. Of course all of us want you to feel at your best, so if you could explain to me about—”

“I’ve been a little off, that’s about it,” said Ben, rushed syllables shooting like a machine gun.

“I understand, we all have our ‘off-days.’ How long have you been feeling like-”

“I don’t know, um, well, maybe a couple of months.”

“Honey, please just let the doctor finish. He’s only trying to help! Dr. Kealy, I’m sorry, my husband has been rather…unpredictable with his moods?”

“Oh geez! Lila!”

“Shh shh shh,” cooed Dr. Kealy, “let’s calm down a little. Ben, your wife and I really just want to help. Lila, let Ben speak up when he’s ready.”

Lila sighed a little. She looked visibly disappointed – the office visit wasn’t playing out the way she had expected. After talking with her mother and making the appointment, she was certain that everything would go ‘up’ from here. Now, it seemed like Ben was shutting himself in – she felt the feelings of helplessness creep back in. She loved Ben, she truly did – and she would do everything in her power to help him. In all honesty, talking about the last several months were in his, and her family’s, best interest.

“Well, Dr. Kealy – my husband has been a bit off lately. Like I said, he’s had some mood swings. Sometimes he gets angry about the strangest things – or doesn’t seem to care about things he normally gets very involved in. Like work – he forgets his briefcase, or forgets to pick up Bryan from baseball practice? He doesn’t seem to want to take our dog out like he once—”

“I can’t, honey! I can’t – you know I would love to take Auf out more, but I just can’t!” Ben hunched over, his hands on his knees – veins beginning to become visible on his strained neck.

Lila leaned back, obviously saddened by Ben’s outburst. She looked appealingly to Dr. Kealy, who reclined and looked over the scene. His expression was knowing, and his brows furrowed over his good-natured face. He popped his knuckles slowly, and got up out of his seat.

“Well, I believe we may need to take this a little more slowly; one step at a time. Ben, do you know when your last physical was?”

Ben stared blankly at him, his forehead glistening with the beginnings of a nervous sweat. He shook his head meekly.

“Yes, I don’t believe you’ve been checked out in a while. Why don’t we go to another room and just run a quick check-up. Okay?”

Lila seemed defeated – as if Dr. Kealy was evading the real question, but relieved that her husband was at least in the doctor’s hands. The truth was, Dr. Kealy knew exactly what Ben needed. It was not as if Ben could not stand to be around his wife, but his guilt and embarrassment had taken over. Dr. Kealy knew that if he was going to be of any help here, he’d need to speak with Ben alone. Before he and Ben left the office, Dr. Kealy turned to Lila and gave her a comforting nod.

“We’ll be back in a few moments. Help yourself to some coffee in the lounge if you’d like.”

Ben had inherited many things from his mother: An intense affection for long Sunday afternoons outdoors, her blue eyes, her distaste for mayonnaise, and unfortunately, a fear of doctors. Dr. Kealy knew this without having met Ben’s mother. Out of the family, Ben came to the office far fewer times than Lila or Bryan. He also knew that Ben needed, no wanted his help. Through the aggravation and the strain he knew that Ben was afraid, and had so much to say.

“Ben, why don’t you take a seat on the table. It’s not the best seat in the house, but it’ll have to do.”

Ben chuckled a little – the first break in his downward turned face that morning. Dr. Kealy set to work, checking Ben’s reflexes, blood pressure, eye movements, and so forth. All the while, Dr. Kealy talked and talked. He ran the gamut: baseball, fly fishing, his own family life and the outrageous cost of car repair. The man had a way about him – he made everything into a joke: “Who knew that running over a curb would flatten my pocket book!? I killed that car worse than I killed my wife’s African violets!”

Ben had visibly loosened up. He was nervous though, his thumbs swirling around one another like a wind vane. Dr. Kealy had completed his exam, and was not too pleased with the results. He had noticed a slight twitching in Ben’s movement and slow responses to stimuli. At one point, Dr. Kealy had thrown Ben a small bean-bag shaped like a baseball; “Who’s your bet for the World’s Series?” As the ball sailed in a wide lob, Ben reached up awkwardly and missed catching it by a long-shot. He was obviously embarrassed by the miss, and Dr. Kealy glossed over the moment as if nothing had happened at all.

“So tell me, what’s been on your mind, Ben?”

Ben hesitated, weighing some invisible balance in his mind.

“Well, doctor – my wife’s not too far from the truth. It’s hard for me to talk about it, but I feel like I’m falling apart.”

“Hmm. Well, I definitely feel like that time to time Ben – but it seems like this has been happening pretty consistently for a while.”

“Yeah. I guess you could say things are slowly getting worse. I mean, I feel stressed all the time now, and really defeated by things I can’t do too well anymore.”

“Like what, Ben? You mentioned you can’t take the dog out like you did before. Is that it?”

Dr. Kealy sat back and listened. He heard about Ben’s inability to stay active with his dog and his struggles to deal with the rigors of his workplace. More importantly, Ben spoke about how all these changes were frightening to him – how he sometimes doesn’t feel like himself. Like somebody who wasn’t anything like him was taking over his body and his emotions. Dr. Kealy sat back serenely with an encouraging expression – inserting comments where necessary to keep the conversation flowing. He noticed how difficult it was for Ben to explain how that last several months had transformed him, at first slightly, and later, more perceptibly. The difficulty was not only in the sensitivity of the subject, but also in Ben’s ability to accurately express himself. He paused every now and again, exerting much effort to find the right phrases or the right word to describe how he once acted, and what behavior now replaced it. Dr. Kealy heard every word Ben said, sorting out the facts in his head and piecing together a puzzle.

After Ben had told everything and looked to Dr. Kealy with a longing expression, hoping something,anything, could be prescribed to bring his old self back. After a few ponderous moments, the good doctor spoke:

“Ben, do you have any brothers or sisters?”

“No. Mom always wanted to have more children…but I never minded being an only child.”

“Were either of your parents ever hospitalized for any reason? I’m afraid I never had the pleasure of meeting either of your parents.”

Ben pressed his lips together – hard. His face muscles stood out and his lips turned white – as if he had seen a ghost. Looking away from his doctor, Ben remembered looking through his mother’s small house, searching for the knitting needles that had once defined her. He shook his head.

“My mother got sick when she was about fifty years old. She was afraid of doctors, and as much as we – I mean – the family, tried to get her to go, she never would. We got a full time nurse for her, though.”

“So, she was never diagnosed?”

“No.”

“I know this is probably very painful for you, Ben – but could you tell me about what your mother was experiencing?”

Ben caught on his own breath and closed his eyes. When he looked up, the pools of his eyes had deepened.

“I don’t know. No one ever knew. I don’t know.” The machine gun edge to his voice had returned. Dr. Kealy, softened his expression and nodded his head. He rose and placed a hand on Ben’s back – it was hot and damp.

“You realize Ben, your family is only trying to help you the way you wanted to help your mother? I don’t like talking about this any more than you do, but was she having some of the same problems you’re starting to have? Difficulty getting around? Not being able to do things she really liked to do?”

Ben felt as if knitting needles were working away in his brain, knitting a startling and horrible picture together. He knew what the doctor was saying, but wanted desperately not to hear him. He felt like dissolving in the presence of this reality, the truth that had been exhumed from memories past. Ben sat silent for a long time. Dr. Kealy couldn’t read him, but knew that what Ben would have to say next would be one of the most difficult tasks ever.

“Yeah, I mean, yes. Mom was having the same problems…” Ben looked up to the ceiling, feeling at once a huge weight lifted from his shoulders and a heavy cloud forming to replace the vacancy.

Dr. Kealy let out a small sigh – empathetic but deliberate; “Ben, what I will tell you now will probably be something you won’t want to hear, but something you definitely need to. I can’t say with absolute certainty what’s causing all the changes you’ve experienced. I’ll refer you to a neurologist, but you’ll have to take a trip to the city to speak with a specialist.”

Ben felt numb, but tried his best to listen attentively. Dr. Kealy seemed to be wrestling with himself for his next words. In a moment of courage, Ben finally took charge:

“Dr. Kealy, if you know what’s wrong with me, you can tell me.”

Dr. Kealy looked steadily at Ben, “Like I said, I can’t say with absolute certainty…But I think you may have HD…Huntington’s disease. It’s a neurodegenerative condition.”

Neurodegenerative? You mean?”

“Ben, we can’t know for a fact until you get more thorough tests. You’ll need an MRI and the neurologists at the city hospital will be able to tell you more. But our testing today has revealed some of the primary symptoms associated with the disease. Your loss of motor control, the slight shaking… What you told me about working, driving your car, forgetting things – they all might be some of the behavioral or cognitive symptoms beginning their onset.”

These moments don’t often happen. Those times where an inconvenient, yet already noticed truth make their appearance. Not just any truth, but one that will change your outlook on the future, your understanding on what is currently happening, and forces the past move into a new perspective. It can be, and often is – a tragedy. But, it is in such moments that we can see what someone is truly made of. In the face of an oncoming 18-wheeler, do I scream and cover my eyes, or do I take control of the wheel and save myself from impending destruction?

Ben kept his hands on the wheel.

“I understand. What can I do? Can I get better?”

“We can’t do anything until we get better test results. HD is not something I have had a lot of experience with – it’s not very common. But I know it’s genetic and is inherited from one’s parents. I believe this is what your mother had… But today the field of medicine is far more advanced. If HD is what you have, there is no cure – yet, there are ways we can deal with the symptoms. There are medications you can take to relieve them.”

Ben retained his strong posture, soaking in the information Dr. Kealy had to offer and trying to organize his thoughts.

“Okay. So I need to meet with a neurologist you say?”

“Of course, I can help you get an appointment. I’ll also find some information; there are some incredibly helpful websites you can visit to prepare for your appointment. Since HD is genetic, you will have to get tested to confirm the results… It is not an easy decision to make, and you will need your family’s support.”

“I understand.”

“I’m not sure if there are any support groups in the area, but I had a patient some time ago that was tested for the disease, genetically tested. I’ll get in touch with her and see if she’d mind talking to you about the process. She was tested before any symptoms emerged…”

Ben asked a few more questions, and then readied himself to go back to Dr. Kealy’s office. He realized more than ever how much he needed his wife… and how dearly she needed to know what was happening. They were a unit. They loved one another more than anything, and she needed to know everything.

Dr. Kealy followed Ben out the door, one hand on his shoulder. They made their way back to his office and opened the door. Lila looked up from a magazine, and tried her best to smile. Dr. Kealy sat down at his chair, his hands placed lightly behind his head. Ben bit his lip.

“Honey, there’s something I have to tell you…”

For further reading on the topics discussed:

  1. Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  2. HD and Family: For greater understanding of family interpersonal dynamics and HD, click here .
  3. Physical Symptoms of HD: This links to the “HD Basic” page on the website. It provides useful knowledge on the physical symptoms associated with HD, primarily chorea.
  4. Cognitive Symptoms of HD: This links to the “Cognitive Symptoms” page of the website, which provides information on what cognitive abilities could be compromised by HD, what causes these symptoms, individual variability with respect to symptoms, and treatment options available.
  5. Family History: This links to information on the inheritance of HD on the HOPES site. This provides the genetic background of inheritance and approaches practical family questions concerning the potential inheritance of HD. It may be insightful to visit the “Genetic Testing” part of the site as well.
  6. Genetic Testing: This link explores a variety of topics on genetic testing, such as: testing guidelines, what takes place during genetic testing, what the test actually does/means, accuracy of the test, confidentiality, and the benefits/consequences of a genetic test for HD.
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HOPES Stories: “Bryan’s Dad Wants Ordinary Back”

“Bryan’s Dad Wants Ordinary Back”

In this story, we learn that Bryan’s Dad is neglecting the things he once loved: his pet dog, his son’s baseball games, and his job. Ben is dealing with the onset of physical and cognitive symptoms from HD and doesn’t know it yet. He feels that he is losing the grip on his old and comfortable life. This story touches on the day-to-day stresses associated with HD symptoms at home, in the workplace, and even while driving. Ben is in denial about these changes and we learn information about Ben’s family history that psychologically impacts him. This story seeks to set the symptoms of HD in a perspective outside of a text book.

There are those particular sounds we do not hear very often, but when we do our hearts quicken, our eyes open just a little wider, and our very synapses fire with understanding. It is truly an ageless phenomenon. Take for instance the discordant clang of an alarm in the middle of algebra class – A fire drill!The first gathered breath and spastic lifting of one’s head off the less-than-comfortable desk, a gut-fear. Will my locker burn down? Then the calm – Oh, it’s just a drill, no more class this period! The elation. Years later, that special collection of consonants and vowels, said by that special person, over a special dinner and clink of champagne glasses – “I love you.” Few words, or sounds for that matter, can really say all there is to say about what the body feels and the brain struggles to define about such moments – ecstasy.

To dogs, especially Auf, no sound resonates as clearly or so strongly as the one his beloved owner makes; ‘I love you’ pales by comparison. Most assuredly, the soft clinking and sensuous shape of a folded and dangling dog leash makes Auf go buck-raging mad. He skittles around the kitchen tile floor, each doggy nail scratching the sound of rain falling on a car windshield. Auf lives up to his name, aufing and playful whines, encouraging his owner to click on the long length of blue chord and metal attachment faster than the speed of sound. When the door opens and Auf trots out into the free expanse of sunshine, he can reasonably expect several things dramatically important to life: 1. The existence of other dogs to sniff. 2. The existence of plenty of squirrels to chase. 3. All the trees in the world to mark his unquestioned territory. To be quite honest, it’s as close to a spiritual experience as the canine species has yet to achieve. It’s true.

Auf occupies his downtime by guarding the backyard. It’s not a terribly large place, but it’s his, and he takes it upon himself to make sure nothing is amiss. He knows every nook and cranny, and this pleases him. When the new family of loud magpies took residence in the largest tree in the yard, this inspired some brow-furrowing and entertainment for Auf. These birds seemed to enjoy his dog bowl as much as he did. When he wouldn’t look, they’d take the liberty of snatching a piece of kibble – his kibble, and fly up out of reach. Such rudeness was persistently met with chorals of barking and a racetrack of activity around the base of the tree. Auf knew one day he’d catch the cursed magpies and end their rein of terror. However thrilling this pursuit has been for him, Auf has become increasingly antsy. It’s been far too long since the last time he heard his favorite sound – and this distresses him far more than a lost mouthful of kibble. Ben, his owner, has neglected his post of Auf-walker.

Truth be told, Ben hasn’t been up to par with the Auf-walking in recent months. As far as Auf can remember, Ben hasn’t taken him for a walk in a good month. Lila, his owner’s wife, has been pretty good at taking him out for a spin – but by no means does she ever take him to his favorite places, nor does she throw the tennis ball. Oh, the tennis ball! That’s the icing on the walking cake! Nothing beats the bouncing silhouette of a tennis ball in the forefront of a setting sun, the lake in the background, and plenty of crunchy grass under-paw. Nothing! It’s true, the last few times Ben took him out for one of their long walks, the ones that have made both Auf and Ben the closest canine-man relationship ever, had been less than spectacular. Ben couldn’t keep up with Auf – sometimes he even let the leash go. Auf, not realizing this, would run up ahead of Ben. When Ben caught up or Auf noticed and ran back, Ben wasn’t in his typical and universal good mood. Instead, he seemed aggravated – holding the leash more tightly, and yanking back when Auf got a little too excited by a carousing squirrel on the path. Not only had the walks been less free-flowing as they once were, they got steadily shorter as well. The tennis ball made fewer and fewer appearances. Auf was confused by all this. Ben was always a good throw – he could probably throw the ball clear across the lake if he wanted too! But Ben’s throwing skills had taken a nosedive. The ball wound up in the lake, or in a bank of shrubbery, denying Auf his long runs to and from the bouncing beacon. Auf didn’t really mind swimming to get the ball, or going on search and rescue missions to recover the ball – but his owner’s frustration at not throwing the ball where it should go was palpable. Frustrated, Ben would stop throwing the ball after a few moments and would take a seat next to the lake, sit, and just stare for the longest time. Not that Auf really minded that either, snuggling up against his owner by the lake was one of his favorite activities.

Although Auf was certainly willing to let a few sour ball-throws go by, he was keenly aware that his job was to support his owner. If Ben wasn’t feeling well, he would encourage him to try, and maybe make him just a little happier in the process. Auf would pick up the stagnant tennis ball and drop it on the ground next to Ben’s hand as he sat staring out at the blue. Wagging like a maniac, and jaws wide open in a slobbery grin, he’d wait for Ben to pick up the ball. After a few impatient moments, Auf would up the ante – and use his stubby nose to push the ball into Ben’s hand. Ben picked up the ball and pocketed it; then gave Auf a short scratch behind the ears, and continued to stare out into nothingness. Auf, feeling that he had failed to encourage and electrify Ben as he used to, picked a spot close by, and laid his head on his outstretched paws – occasionally looking to his owner with wide-set eyes.

Now, as Auf scouts the backyard, he forces himself to be uncomfortable. The next walk could come today, right? And if it did, he would be ready with as much territory marker as possible. Imagine for a moment that Auf was a cow. What do cows do if their owners don’t milk them? Can cows milk themselves? Probably not – but if they could, they wouldn’t waste the milk on a patch of grass in the middle of their pasture, would they? Auf lived by the same philosophy – he would hold it all in until he couldn’t bear it further. Needless to say, when dusk came slowly and his food was set out for him on the porch, he waited anxiously for his favorite sound of the dangling leash. It never came – and as Auf ate, all he could think was, “What a waste.”

Ben went to work, came home, went to sleep, went to work and maintained the beats that sounded the drum of daily life. He was a busy man – he had a job, a family, and a life. As ordinary as ordinary days can be, they are nonetheless busy. Although he had become accustomed to the routine-ness of a breadwinner, his mind had wandered from the busy that is going to meetings, changing the car’s oil, and mowing the lawn. He was busy trying to understand what was happening to him; to his body and his mind. Ben never grew bored of the busy metronome clickings of all the parts of his life – in other words, he never minded being this comfortable. What distracted him now was his inability to keep up with the metronome. He was chronically late; late to work, late to respond to his coworkers during lunch, and late to pick up his son, Bryan, from baseball practice. He was off beat – but what was worse, the beat was changing daily – ever so slowly, but perceptibly.

They started out small – almost silly in a way. One morning on the way to work, he started noticing how hard it was to concentrate while driving. Suddenly, he was aware of the cognitive acrobatics involved in navigating a 1500 pound hunk of churning metal. You had the accelerator and the brakes – all which had to be pressed in a certain way at a certain time. The vision too – you had to check the mirrors and the odometer. There are speed limits to consider, and fellow yawning employees scurrying like ants to get to their positions and maintain their drums for the remainder of the day. Changing lanes involved a small turn of the steering wheel, right-hand turns were a bit sharp, whereas left-turns were more arched and meandering. The road almost never stays straight the whole time; sometimes lanes appear, then disappear as they merge into larger streams of highways or ebb down to one-lane driveways, or pool into the wide expanse of parking lot. The thought hit Ben as he nearly side-wiped a car when changing lanes. The sudden jerking to maintain the road, get comfortably back into his lane, and resume the speed of traffic nearly gave him a headache. He had to be careful – he was almost passing stop signs he had stopped at faithfully for years. Nothing needs to be said about the perils of highway driving and making sure to get off at the right exit – changing speed, merging, and knowing where to go and how to go next.

Work was no better. He made a habit of stopping by the bagel shop to pick up a baker’s dozen for his buds at work and the secretaries that made his life so much easier. When he had time, he went through the Java Joe’s drive-through to pick up some juice. Appreciation was never lost on anyone, but in all honesty, people at the office had developed the bad habit of assuming that Ben would always bring in these amenities. Now, as Ben’s ability to drive began to suffer, the 15 mile commute to work seemed so much more daunting, and eventually became a 20, 25, 30 minute careful expedition. He often missed the beginning of morning meetings. Still stressed and adrenaline pumped from his commute, he forgot to maintain his up-beat attitude; wishing everyone a good morning, remembering to ask how Tom’s house renovation was coming along, and congratulating Diane on her new nephew. When these inconsistencies within himself became apparent to Ben, he drew within himself – upset that he couldn’t maintain his cocktail party mannerisms. The off-hand comments about the bit of lather left under his ear from shaving didn’t help either. People could see that he was different. It wasn’t some abstract concept; suddenly Ben became all too aware of the nakedness of his failing physical, cognitive, and emotional well-being. Honestly, Ben couldn’t wait to retreat to the confines of his office.

Although the quiet interior of his office relaxed him, Ben could not retreat from the signs that something was wrong. Daily reports were part of his daily work-beat. He reviewed the materials from accounting and an assignment from his boss – and, if all went well, typed up a memo to return to management by lunch. Ben would now sweat over the papers – all the orbiting acronyms and symbols looked like a steamy bowl of Alphabet soup.

“What did AcctP mean again? Accounts payable, or Actual Percentile, yeah. I think so – let me just ask? No, I can’t talk to anyone right now, not just this minute. I’ll figure it out, I will.”

Ben recognized that it took him much longer to process information than it used to. He would eventually figure it out, but ‘eventually’ became a euphemism. He began to pattern his desk and computer monitor with a quilting of sticky pad notes, reminding him of things that used to come to mind without help. Writing the memos after he’d organized his thoughts were a problem too. Ben would stop to ponder, sometimes minutes at a time, for a particular word to use.

“Annual reviews show an – Annual reviews show an? something, an increase. Yes, definitely an increase, but what type of increase was it? External? Expositiory? No no no no no!”

It would take a few minutes before “exponential” would come to mind. When it did, Ben would sigh with relief, but it was a rather unimpressive relief. Fine, he could get the reports done when he put his mind to it, but why did he have to exert so much energy to find one word in his mind? Sooner than later, Ben began to compile a list of words he was commonly having difficulty locating when he needed – but to his frustration, the list became so long, it took him almost as long to find it on paper.

The off-beats of Ben’s mind and body made him feel like an unreliable metronome. He felt that no one could trust him with anything. Lila, bless her, had reminded him time and time again to bring the briefcase before he headed out the door, to pick up Bryan after practice, and to take Auf out. He tried his best, but the stress of doing just about anything overrode Lila’s best efforts. Auf was another matter – he never forgot that he had to take him out to the lake. Even though Auf was the second love of his life, he couldn’t bear to take him out anymore. He just couldn’t keep up with him anymore, and this worried him in a way that work couldn’t. Ben was failing his dog, a creature that really couldn’t do much without him. Auf was his responsibility – and he was steadily becoming unable to perform those things that make dogs happy. Much to his own surprise, Ben didn’t feel as guilty as he thought he would be when he stopped taking Auf out to the lake. Lila took up the task willingly, but Ben, as much as he knew he loved his dog, began to withdraw himself from the post of Auf-walker, and more to Auf-scratcher – and even that became an occasion.

Ben knew he should be upset by all of this – work, Auf, Lila, Bryan – all the things that once made his life turn – the things that used to beat rhythmically, like heart-beats. But, he really wasn’t. The lack of concern felt far better than constantly reflecting on his mounting list of inadequacies. He couldn’t play baseball all that well with Bryan anymore, so why bother? Ben had difficulty sorting through the mail and writing out the proper checks and envelopes for the right addresses – so why not ignore them altogether? For Ben, this felt like a good proposition. Although Ben graduated from novice to proficient in lying to himself about what mattered and what didn’t, he couldn’t stop himself from getting bogged down by his collection of failures. His attitude vacillated from apathetic, to incensed, to downtrodden, to frustrated, and back again – thesefeeling began to sound the beats of his daily life. Persistent and off-kilter, the sound began to deafen him, and for much of this, Ben could only try to hold his hands over his ears, and hope.

Denial is everyone’s friend we hate to love and love to hate. There’s no better way for describing it. Denial will give us a backrub, tell us what we want to hear, and suggest we go out for ice cream. Then again, denial becomes the person we wish would move as far away as possible, so we can change our phone number and never hear from again. Ben had become two people over the past several months. He became a man who could see what was happening to himself while driving to work and leaving the blue dog leash on the kitchen counter. He also became the man who saw nothing, heard nothing, and in turn, felt nothing. Lila told him one evening at dinner that she had met with the family doctor, and that he suggested Ben come in soon to talk with him. Lila did her best to sound upbeat and supportive – to point out that she knew something was wrong without actually saying anything of the sort. She used phrases like “just for a checkup,” or, “just a chat, you know, if anything is on your mind.” Ben didn’t know how to feel about this. Ben #1 wanted to say, “What do you mean – a chat with the doctor? I don’t need a check up! Stop heckling me about everything, let’s just eat already.” Ben #2 wanted to let the tears finally out, to expel the frustration and confusion that was polluting his being. This Ben wanted to open up to his wife, to be able to keep up with his son, his dog, and with his old rhythm of life and say, “Thank you honey, I’ll see him tomorrow. I love you. The food smells wonderful.”

Instead, Ben took a new road, recently traveled, and said nothing. He pretended not to care because he couldn’t reconcile the Ben imposter and the Ben who was real, but horribly lost in himself. It’s not to say that he had no idea what was happening to him. He did – he just didn’t recognize it yet. Ben had snapshots of his teenage years, where his mother began to change. She was an expert mother, she ran a leak-tight household and had a passion for knitting. After Ben left for college, he would come and visit during breaks and see the relationship between his parents deteriorating. The house became dusty – running his finger over the living room lamp, he collected an earplug-sized formation of fuzzy grey. Dad left, and Mom moved closer to be with Ben after he graduated, got married, and bought a house. She aged quickly, eventually needing a full-time nurse. His mother was incredulous about seeing a doctor – she had a patently unshakable fear of doctors because her father went to an institution and never returned. She would call Ben to take her to the fabric store to buy yarn, sometimes three of four times a week. Ben remembered her strange mood swings, her shaking hands, and wondered if she could even knit anymore. More importantly, he wondered if she was still the woman who raised him – Ben couldn’t see that woman any longer, but felt obligated to indulge her. Each time they went to the store, Ben’s mother relaxed, picking a rainbow of different yarns, which Ben happily paid for. Unlike past years, Ben never received his Christmas bulky sweater, or multi-colored scarf. When she died, Ben cleaned up her house, and found closets full of unused yarn – covered with dust. The knitting needles were nowhere to be found, and Ben could only conclude that someone had thrown them away.

So, answering the question of “what is happening to me,” was more delicate and far too personal to approach with any steadfast rationale. To say Ben saw his deteriorating mother within him would be inaccurate. That Ben had been quieted by the convincing speech of denial – he was fine. If Ben couldn’t lie to himself – like when he forgot his briefcase for the third time this week, he would decide it didn’t matter at all – he had missed the meeting anyway and nothing he could do would change that. But all this was not lost on Lila, the family physician – or Ben, completely. Although Ben said nothing about his wife’s meeting with the family doctor, he said nothing about the appointment she had promised to make for Tuesday, 9:00AM sharp. He would go, and as apathetic or irritable as he might be that morning to have his wife drive him – “just to make sure he got there on time.” He went for a short walk with Auf that evening – which his dog was all too happy to be on, and went to bed. He didn’t thank his wife, nor did he bring the tennis ball on his short walk around the block; but the most internal piece of Ben, the piece that really mattered, was ready to get back some hope that would bring the beat of his life, back to ordinary.

For further reading on the topics discussed:

  1. Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  2. HD and Family: For greater understanding of family interpersonal dynamics and HD, click here
  3. This links to the “Cognitive Symptoms” page of the website, which provides information on what cognitive abilities could be compromised by HD, what causes these symptoms, individual variability with respect to symptoms, and treatment options available.
  4. HD and Driving: For detailed information about HD and driving, please link here.
  5. Family History: This links to information on the inheritance of HD on the HOPES site. This provides the genetic background of inheritance and approaches practical family questions concerning the potential inheritance of HD. It may be insightful to visit the Genetic Testing part of the site as well.
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Stories of HOPES: “Bryan’s Dad Isn’t Who Lila Remembers”

“Bryan’s Dad Isn’t Who Lila Remembers”

In this story, we meet Lila, a loving wife who is concerned for the wellbeing of her husband. She no longer recognizes him as the man she once knew. Unbeknownst to her, Lila’s husband has HD and is becoming symptomatic. Through her experience, we may understand the complexity of the family experience when encountering the signs of HD. Ben’s change in attitude represents how HD can alter behavior and cause stress on the family.

Lila breathed across a clouded coffee cup, drumming her fingers to the tune of a cold morning’s chorus of birds. The family’s dog, a rather spry Australian Shepard arched back, front paws splayed out and jaws opened wide, yawned a welcome to another day. Though the backyard was just a small rectangle, it was full and green with bright colorful patches of Nerf guns, a plastic bat, and an array of sports balls. Lila had her busy morning schedule down to a science: wake Bryan up and get his lunch made while he showered, make sure Ben got ready to go to work on time, prepare their breakfasts, ready the grocery shopping list, pass around kisses and “good morning, honey(s)!” She drove Bryan to school, grabbed the groceries, and was back at home with time to spare before Good Morning America. Lila should have prepared the marinade for this evening’s dinner, but instead she stared out over the backyard with a cup of instant coffee. She never drank coffee until she got the notion to pick up a can this morning. She winced at every hot bitter sip, calling attention to newly furrowed wrinkles on her young face. Close up, you could see her hands tremble weakly – she looked down at the rippling surface of her cup, startled at herself. They weren’t coffee jitters, the cup still almost full, releasing delicate spindles of steam into the creeping sunlight.

“What is wrong with me?” she whispered to Auf, who sniffed at her sandals. Lila laughed lightly to herself, more for the sound of laughter than anything else. Auf smiled broadly, ears pressed back and tail swishing slowly. She bent over to scratch his head, careful not to spill any coffee. Auf ran to investigate the yard more thoroughly, and Lila was again left with herself. Taking a few more harsh sips, she moved back into the small, yet impeccably clean kitchen.

“I guess that’s not the right question at all, now is it?” she thought to herself.

Cleaning off the table from breakfast, she saw that Ben had left his briefcase again. One of the clasps lay undone, and a few documents slid through the slim open space. This wasn’t the first time Ben had forgotten his briefcase – it had been happening more and more frequently over the past several months. Briefcases were a small matter compared to bills left months over due, the unshaven patch of sloppily groomed beard, and missed baseball game practices. Ben, the man she had married, was never late, always clean shaven, and an incredible father. Now, Lila, who couldn’t bring herself to admit it, didn’t even recognize him.

It wasn’t just the odd forgetfulness and slipping attention to facial hair that concerned Lila, but Ben’s attitude. All married couples have their squabbles – bouts of financial difficulty, differing theories of child rearing, and day to day stress. But Ben, the eternal optimist and compromising pacifist never raised his voice or disrespected his family, or anyone for that matter. His warm manner and cool-headedness served him well at his company. He was a popular guy, no doubt – dropping off bagels and cream cheese for his coworkers and cups of coveted Java Joe’s before straightening his tie for the early business meeting. Lila already knew something was wrong when Art, a close family friend and coworker of Ben’s called to ask if everything was alright at home. Ben had been missing his meetings and arriving late in such unexpected moods; sometimes distant and apathetic, other times nitpicky, aggravated, and just plain sad – never mind the lack of Java Joe’s and poppy seed bagels. Lila and Art bonded over their worry; Lila recounted the recently escalating arguments at home, and how Ben didn’t play baseball with Bryan or care to see his games anymore. Art asked if Lila had talked to Ben:

“Art! Of course I’ve tried – but he’s like a Mad Hatter recently. I never know what’s bothering him, or what’s on his mind. He comes, he goes and I just try to keep up. He doesn’t seem to care about the things he once couldn’t go a day without! I’ve had to take Auf for his walks – the dog gets so antsy without his walks to the lake?”

“Well Lila, I’m sure everything will pass. But maybe you need to talk to a counselor, or somebody. Ya Know? I mean, it helped Nancy and I – helped figure out some problems with our marriage we never even thought to talk about?” Lila was taken aback. Of course she was a good wife! It couldn’t be her fault, could it? Was Ben acting strangely because of her? She hoped not.

“Art, I don’t know if you’re right – I hope you’re not right about that. Of course I pray this is just a weird funk, and Ben will pull himself out of it. I just -”

“Yeah?”

“Well, nothing – thanks for calling Art.”

“Lila? You know you can always talk to me or Nancy about it. We’ve been friends for a long time? Just know that everybody at work is worried and just wants the best for you and Ben. Alright?”

“Thanks, Art – I appreciate it. I really do.”

Good Morning America droned in the background. Lila still held the coffee cup in thought. The murky liquid had gotten cold in her hands – the creamer starting to clump up in little islands on the rim of the mug. She set down the cup, thinking about what she needed to do. Whose fault was it? Had she done something wrong? What about Bryan? She couldn’t go another day defending his daddy to him:

“Of course daddy wants to see your game, honey! He’s just not feeling that good, that’s all. You know what? I’ll go with you – you can teach mommy all about it!”
“But MOM,” he dragged the word out in annoyance, “Dad always went to my games! You don’t even likebaseball.”

Lila breathed out a long sigh. She thought to herself, “Just admit it. Alright? Just admit it.” She shook her head, angry and confused. She held the bedraggled briefcase, pushed the papers back in neatly and clicked down the clasp. “I don’t understand, though. I just don’t.” She sifted through the mail, sure to pick out all the bills so the electricity didn’t get shut off again. Lila put on her reading glasses to read all the mail. She even read the junk advertising to avoid the question. The phone rang dully. Thankful for the distraction, she picked up.

“Hey honey.”

“Ben? How are you – sweetie, I saw that you left the briefcase this morning?”

“Oh, yeah. Well, I guess I don’t really need it all that much. I missed the presentation this morning anyway.”

“How, honey? You left when I did.” The silence rang in Lila’s head like a dial tone. “Honey?”

She could hear him breathing still – the phone hadn’t gone dead. “What’s going on sweetie, you seem a little out of it. You know you can talk to me about—”

“It’s nothing! Okay? Jeez! I just was a little tired this morning and missed the exit. Alright? Why is everybody bothering me!” Ben took on an eerie sarcastic tone. “Beeeeen, are you feeling all right? Ben, you look tired. Ben, do you need to take the rest of the day off, Ben Ben Ben Ben Ben!”

A little stunned, Lila choke a little on her own words. A vein of fear seemed to crack open inside of her, washing her whole slight frame with renewed trembles and worry. Missed the exit? How could he have missed the exit? He had been working there for years, taking the same roads day in and day out. Something was wrong.

“It’s all right honey, I promise. I’m just a little worried. I love you – you know that. That’s about it. You’ve been acting a little funny, and I just want to be here for you.”

“Nothing’s wrong!” his last burst of anger seemed to do Ben in. He was quiet for a second. “I’m sorry, darling.  It’s just – I’m just – well, I don’t know. I’m sorry. I’ll be home for dinner at five. Okay?”

Still unable to understand this person on the phone, Lila managed an “Uh huh.” Just as Ben was about to hang up, she remembered,

“Oh honey! Honey?”

“Yeah?”

“Remember, you need to pick up Bryan from baseball practice at 4:45.”

“Oh, right. Of course, sorry. I’ll remember this time.”

Click.

The phone conversation was the last straw to break the camel’s back. Lila just stared off into the distance – her hand clutching the phone, the spiraled chord dangling. Closing her eyes she felt the swell percolating somewhere beneath. She felt the blood knock at her temples and the ache of her hand on the phone. She wished she could crush it. The homely appliance that had forced her to wake up – to think about the last several months – forced her to answer the question: “What is wrong with me?” Hunching over and catching back the tears. She hated soap operas – she hated the horribly acted raw emotions that her girlfriends seemed to drink like the ether of life. They were just bored with their perfect lives – but Lila, felt on the verge of something deep, and was losing the battle. The phone began to yell at her, the squawking bleep of an unhappy appliance – wanting desperately to go back to the safety of its hook. Lila, as unhooked as she was, felt sorry for herself. “Just admit it!” She felt like calling her mother – a thing that she never used to look forward to.

“Mom?”

“Hey honey! I’m sorry, but I’m with the girls now, can I call you later?”

“Mom? um, okay – I guess.”

“Pumpkin? Is everything alright? You sound upset.”

“No, I’m okay Mom, promise. Just call me later.”

“Darling, I wasn’t born yesterday. First off, you never call your lonely mother, and I never taught you how to lie. Talk to me, pumpkin.”

Lila let it spill – the whole string of strange changes with Ben. The mounting collection of concerns she never had to deal with before. Will Ben get fired? Does he love me? Will Bryan be all right? Does he love me? Is he really sick? Does he love me? If Lila’s Mom knew anything, it was that life was hard. Her parents were immigrants – she worked in their grocery store for long hours and dealt with her father’s early death. The woman could wash dishes in scalding hot water – her leathery hands immune to a light touch and well acquainted with hard knocks and pinpricks.

“Now Lila – you’ve got to recognize how lucky you’ve been. You married a wonderful man who I knowloves you very much. You have a beautiful son, and you’re healthy and strong as a mule.”

“Mom, I know – but I need him back. I need him back!”

“I know this is a frightful thing you’ve got going, but you come from pretty strong stock. You got this one in the bag! You need to get over your fear, and call a doctor. Talk to someone about this, and get it off of your own chest. You need some breathing room, Pumpkin. If Ben is sick, it’s better to know what it is than to keep wondering. Do it for him, do it for Bryan, and mostly – do it for yourself?”

Lila loved her mother – she always knew what to say. She gave her the clarity she needed through all the tears and the confusion.

“Pumpkin – you go look up some numbers right now, and set up an appointment. And who knows, maybe Ben will start shaving up nice and pretty again in no time!”

Lila let go a healthy laugh, one that shook her slightly and relaxed her reddened face. She thanked her mother, promised to keep taking the garlic supplements, and hung up the phone. Wiping off her drippy nose, she set out to complete her daily tasks – enveloped in a new security – one that makes no guarantees, but one of hope that the fibers of her family’s life will come back together again. She wrote down the number of her family doctor on a note pad – she’d make an appointment tomorrow and get some advice. She didn’t know if she needed a marriage counselor, a psychologist, or what. But the doctor would know, and the mere fact that someone else would shoulder some of her burden and give guidance under these circumstances was what she really needed.

Dinner was made and she waited in nervous anticipation for her husband to return. She didn’t know what was wrong with Ben – but she would hold on to the belief that she knew who he was, and that the unquestioned truth of their mutual love for one another wasn’t going anywhere fast…not if she could help it. The clock slid past five, and she told herself to be patient. The phone rang.

“Hey, Lila? Hi, it’s Mrs. Rolfes. I’m bringing Bryan home.”

“Um, thank you Mrs. Rolfes. I’m so sorry, I guess Ben forgot about Bryan’s practice. Thank you so much.” Lila felt the old pulse of dread come back – the worry that Ben was forgetting about those things that he used to wake up for. Mrs. Rolfes and her preening tone told her that she was not especially pleased to watch after other children. Lila knew that Mrs. Rolfes thought that she and Ben were bad parents – or at least uninvolved ones.

She finished the pleasantries with Mrs. Rolfes and called Ben’s cell phone. She tried to push down her anger at him. Lila told herself that Ben wasn’t well – it’s not his fault that he was acting this way. He didn’t pick up on the first call, but answered on the second.

“Hey honey. Sorry – I just lost my way a little. I’ll be home soon. Can’t talk now, I’ll run a light or something.”

Bryan was home before he was – sullen that Dad forgot to pick him up again. He clomped upstairs to put up his equipment and wash up for dinner. Half and hour rolled by, and finally Ben pulled into the drive way. Leaving the car door ajar, he walked into the side door. All Lila could do is muscle out a supportive and loving smile, give her husband a kiss on the cheek, and usher him in before the re-heated food got cold again.

For further reading on the topics discussed:

  • Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  • HD and Family: For greater understanding of family interpersonal dynamics and HD, click here
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Companion Animals and Health

When most people consider therapies, they often think of prescriptions and side effects. However, animal companion therapy is proving to be an effective means of improving well-being among patients. Many of the benefits of animal companion therapy can extend to patients and family members living with Huntington’s disease. This article highlights the physical effects caused by companion animals, as well as opportunities for taking advantage of this type of therapy.

The Physical Effect of Companion Animals^

Stress can have harmful effects on the body’s ability to cope with various health issues. Research consistently shows that exercise and meditation can help manage stress levels. More recently, the scientific community has begun to collect growing evidence that animal companions might have the same effect on the health of patients.

The presence of an animal alone can affect our emotions. Animals are often able to focus people’s attention in a way that is calming or de-arousing (Cirulli et al., p. 342). Since animals, especially dogs, respond with affection and generally pro-social behaviors, they can potentially serve as an “emotional bridge” within therapeutic contexts.

The physical health effects of a companion pet can range from everyday benefits to life-saving changes. Within the first few months of acquiring a pet, patients tend to have lowered risk for cardiovascular disease, increased chances of surviving myocardial infarctions, decreased need of physician services during stressful life events and a reduction in everyday minor health problems.

Many HD patients cite lack of familial support as a major problem. Companion animals could help mediate this gap as icebreakers, bridging people with the outside world and jumpstarting communication and social exchanges that can promote feelings of social integration. Research in nonhuman mammals suggests that oxytocin, a signaling molecule in the brain, helps to increase one’s feeling of reward during social interactions while also increasing bonding between individuals. Oxytocin also assists in responding to social stress for humans. In fact, interacting with a dog caused a significant increase in levels of oxytocin within the human, improving his or her ability to forge new social bonds.

Creating an animal companion relationship^

In the study, Animal-assisted Interventions as Innovative Tools for Mental Health, researchers state that dogs are the ideal animal companions. Over thousands of years of domestication, dogs have been “selected for characteristics that enhance their sensitivity to a wide range of human communicative signals, both visual and acoustic” (Cirulli, p. 341). Dogs develop complex communication systems with humans and are highly interactive. Additionally, dogs provide opportunities for physical, recreational, and social activities. They are easily trained to constructively work in different settings, which explain their use as Seeing Eye and rescue dogs.

HD patients who live in nursing homes are often under great duress, as institutionalization can result in a decreased quality of life and stress due to separation from loved ones. Dog-mediated interventions could improve communication and reduce loneliness and depression.

Furthermore, animal companions could also help children of families experiencing traumatic life occurrences. Animal companions have been shown to influence social, emotional, and cognitive development in children. Parents often report that an animal helps teach children about life events. Children who grow up with pets have an enhanced sense of empathy and responsibility, social status within the peer group, and higher self-esteem and self-confidence.

While the positive aspects of animal companionship seem numerous, there are studies that raise questions about the extent of this impact. Visiting dog programs do not consistently “improve mood, cognitive abilities or social interactions” (Cirulli, p. 344). This might indicate that perhaps longer-term, matched interaction is needed between animal and human to see any effects. In fact, saliva spits revealed that there is a time-dependent increase in behavioral results such as improvement in mood or social bonding, as measured by mood changes and cortisol levels. (Cortisol is a hormone often associated with stress. Long term interaction with animals has shown to decrease levels of this hormone, improving well-being.)

Adding Pets to the Home^

In summary, adopting a companion animal into a Huntington’s disease family or an institution housing HD patients might have marked effects on the well being of various participants. However, there are certain aspects to take into account when making the decision to add a family member to the home. To find more information about the logistics of adopting a pet, visit http://www.aspca.org/adopt/adoption-tips.

If you are concerned with integrating a pet into family experiencing health difficulties, please contact Pet Partners, an organization dedicated to “improving lives through positive human-animal interaction.” Visit their website at http://www.petpartners.org/.

For Further Reading:^

1. Cirulli, Francesca, Marta Borgi, Alessandra Berry, Nadia Francia, and Enrico Alleva. “Animal-assisted Interventions as Innovative Tools for Mental Health.” N.p., n.d. Web. 14 Oct. 2013.
2. Bekoff, Marc, Ph.D. “Pets Are Good for Us: Where Science and Common Sense Meet.”Psychology Today. N.p., 23 July 2010. Web. 14 Oct. 2013
3. Allen, Karen M., Jim Blascovich, Joe Tomaka, and Robert M. Kelsey. “Presence of Human Friends and Pet Dogs as Moderators of Autonomic Responses to Stress in Women.” Journal of Personality and Social Psychology 61.4 (1991): 582-89. Print.
4. Health Benefits of Animals. Pet Partners, n.d. Web. 14 Oct. 2013. .

K. Powers
10/31/2013

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HOPES is hiring! Apply now!

The Huntington’s Outreach Project for Education, at Stanford (HOPES) is an educational service project working to build a web resource on Huntington’s disease (HD).  Our mission is to make scientific information about HD more readily accessible to patients, their families, and the general public. We are currently hiring student researchers (writers), graphic designers, and web developers for the 2013-2014 school year. You must be a current Stanford undergraduate to apply.

Student researcher positions:

As a researcher, you will be responsible for researching a specific HD-related topic, writing articles based on your research, and planning the graphics to go along with your article. You are also expected to play a big role in the editorial process for both your own work and the work of other group members. Applicants should have a strong background in biology, human biology, or anthropological sciences, including a good working knowledge of genetics. Strong writing, editing, and communication skills are also necessary.

Graphic designer positions:

Graphic designers work on the most popular parts of the site including illustrated books, articles and interactive tutorials. Responsibilities include collaborating with researchers to visually enhance the educational text, creating interactive tutorials, and brainstorming new projects for the HOPES website. Although not required, college-level biology background is a strong asset. Other skills we are looking for include: experience with Adobe Photoshop or another graphics editing program, Illustrator, Flash, vector or 3D graphics, digital video editing, and web design.

Web developer positions:

We are hiring a web designer to improve our WordPress site. This is a great opportunity for someone with an interest in web design to learn and improve as a web designer, and develop a competitive portfolio. Experience preferred, but not necessary.

Compensation:

Units or pay (units through Anthropology or HumBio; starting salary of $15 per hour)

Commitment:

Full time or part-time during the summer and part-time throughout the school year. During the school year, weekly hours are flexible and most work is independent, but you must be able to average 6-10 hours of work per week.  There will also be group workshops, outreach events, and weekly meetings, all of which will be scheduled according to the availability of the team.

Faculty Coordinator:

Prof. Bill Durham

HOW TO APPLY:

Applications for all positions are due on Sunday, October 20 by noon. Please send a current resume, letter of application, and unofficial transcript to HOPES Project Leader Ravali Reddy (ravreddy@stanford.edu). The letter should include a candid discussion of your qualifications for the position, your other time commitments, your leadership skills, and your reasons for interest in the position.

Student researchers: Please attach two writing samples, science-related and/or research-based in nature.

Graphic designers: Please send in 3 recent design samples with a brief description about each (tools used, time spent, purpose/client, etc).

Web developers: Please send links to any web-design work you may have done.

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Stories of HOPES

Welcome to Stories of HOPES! The short stories of this section of HOPES are fictional accounts inspired by the experiences of real families living with HD. They are not meant to be instructive or prescriptive, but to serve as artistic impressions of the social and familial impact of the disease. We have found them a useful way to stimulate discussion of HD and its implications with others. As always, we invite your comments on these stories.

– The Staff of HOPES

“Bryan’s Dad Isn’t Who Lila Remembers”^

In this story, we meet Lila, a loving wife who is concerned for the wellbeing of her husband. She no longer recognizes him as the man she once knew. Unbeknownst to her, Lila’s husband has HD and is becoming symptomatic. Through her experience, we may understand the complexity of the family experience when encountering the signs of HD. Ben’s change in attitude represents how HD can alter behavior and cause stress on the family.

Lila breathed across a clouded coffee cup, drumming her fingers to the tune of a cold morning’s chorus of birds. The family’s dog, a rather spry Australian Shepard arched back, front paws splayed out and jaws opened wide, yawned a welcome to another day. Though the backyard was just a small rectangle, it was full and green with bright colorful patches of Nerf guns, a plastic bat, and an array of sports balls. Lila had her busy morning schedule down to a science: wake Bryan up and get his lunch made while he showered, make sure Ben got ready to go to work on time, prepare their breakfasts, ready the grocery shopping list, pass around kisses and “good morning, honey(s)!” She drove Bryan to school, grabbed the groceries, and was back at home with time to spare before Good Morning America. Lila should have prepared the marinade for this evening’s dinner, but instead she stared out over the backyard with a cup of instant coffee. She never drank coffee until she got the notion to pick up a can this morning. She winced at every hot bitter sip, calling attention to newly furrowed wrinkles on her young face. Close up, you could see her hands tremble weakly – she looked down at the rippling surface of her cup, startled at herself. They weren’t coffee jitters, the cup still almost full, releasing delicate spindles of steam into the creeping sunlight.

“What is wrong with me?” she whispered to Auf, who sniffed at her sandals. Lila laughed lightly to herself, more for the sound of laughter than anything else. Auf smiled broadly, ears pressed back and tail swishing slowly. She bent over to scratch his head, careful not to spill any coffee. Auf ran to investigate the yard more thoroughly, and Lila was again left with herself. Taking a few more harsh sips, she moved back into the small, yet impeccably clean kitchen.

“I guess that’s not the right question at all, now is it?” she thought to herself.

Cleaning off the table from breakfast, she saw that Ben had left his briefcase again. One of the clasps lay undone, and a few documents slid through the slim open space. This wasn’t the first time Ben had forgotten his briefcase – it had been happening more and more frequently over the past several months. Briefcases were a small matter compared to bills left months over due, the unshaven patch of sloppily groomed beard, and missed baseball game practices. Ben, the man she had married, was never late, always clean shaven, and an incredible father. Now, Lila, who couldn’t bring herself to admit it, didn’t even recognize him.

It wasn’t just the odd forgetfulness and slipping attention to facial hair that concerned Lila, but Ben’s attitude. All married couples have their squabbles – bouts of financial difficulty, differing theories of child rearing, and day to day stress. But Ben, the eternal optimist and compromising pacifist never raised his voice or disrespected his family, or anyone for that matter. His warm manner and cool-headedness served him well at his company. He was a popular guy, no doubt – dropping off bagels and cream cheese for his coworkers and cups of coveted Java Joe’s before straightening his tie for the early business meeting. Lila already knew something was wrong when Art, a close family friend and coworker of Ben’s called to ask if everything was alright at home. Ben had been missing his meetings and arriving late in such unexpected moods; sometimes distant and apathetic, other times nitpicky, aggravated, and just plain sad – never mind the lack of Java Joe’s and poppy seed bagels. Lila and Art bonded over their worry; Lila recounted the recently escalating arguments at home, and how Ben didn’t play baseball with Bryan or care to see his games anymore. Art asked if Lila had talked to Ben:

“Art! Of course I’ve tried – but he’s like a Mad Hatter recently. I never know what’s bothering him, or what’s on his mind. He comes, he goes and I just try to keep up. He doesn’t seem to care about the things he once couldn’t go a day without! I’ve had to take Auf for his walks – the dog gets so antsy without his walks to the lake?”

“Well Lila, I’m sure everything will pass. But maybe you need to talk to a counselor, or somebody. Ya Know? I mean, it helped Nancy and I – helped figure out some problems with our marriage we never even thought to talk about?” Lila was taken aback. Of course she was a good wife! It couldn’t be her fault, could it? Was Ben acting strangely because of her? She hoped not.

“Art, I don’t know if you’re right – I hope you’re not right about that. Of course I pray this is just a weird funk, and Ben will pull himself out of it. I just -”

“Yeah?”

“Well, nothing – thanks for calling Art.”

“Lila? You know you can always talk to me or Nancy about it. We’ve been friends for a long time? Just know that everybody at work is worried and just wants the best for you and Ben. Alright?”

“Thanks, Art – I appreciate it. I really do.”

Good Morning America droned in the background. Lila still held the coffee cup in thought. The murky liquid had gotten cold in her hands – the creamer starting to clump up in little islands on the rim of the mug. She set down the cup, thinking about what she needed to do. Whose fault was it? Had she done something wrong? What about Bryan? She couldn’t go another day defending his daddy to him:

“Of course daddy wants to see your game, honey! He’s just not feeling that good, that’s all. You know what? I’ll go with you – you can teach mommy all about it!”
“But MOM,” he dragged the word out in annoyance, “Dad always went to my games! You don’t even likebaseball.”

Lila breathed out a long sigh. She thought to herself, “Just admit it. Alright? Just admit it.” She shook her head, angry and confused. She held the bedraggled briefcase, pushed the papers back in neatly and clicked down the clasp. “I don’t understand, though. I just don’t.” She sifted through the mail, sure to pick out all the bills so the electricity didn’t get shut off again. Lila put on her reading glasses to read all the mail. She even read the junk advertising to avoid the question. The phone rang dully. Thankful for the distraction, she picked up.

“Hey honey.”

“Ben? How are you – sweetie, I saw that you left the briefcase this morning?”

“Oh, yeah. Well, I guess I don’t really need it all that much. I missed the presentation this morning anyway.”

“How, honey? You left when I did.” The silence rang in Lila’s head like a dial tone. “Honey?”

She could hear him breathing still – the phone hadn’t gone dead. “What’s going on sweetie, you seem a little out of it. You know you can talk to me about—”

“It’s nothing! Okay? Jeez! I just was a little tired this morning and missed the exit. Alright? Why is everybody bothering me!” Ben took on an eerie sarcastic tone. “Beeeeen, are you feeling all right? Ben, you look tired. Ben, do you need to take the rest of the day off, Ben Ben Ben Ben Ben!”

A little stunned, Lila choke a little on her own words. A vein of fear seemed to crack open inside of her, washing her whole slight frame with renewed trembles and worry. Missed the exit? How could he have missed the exit? He had been working there for years, taking the same roads day in and day out. Something was wrong.

“It’s all right honey, I promise. I’m just a little worried. I love you – you know that. That’s about it. You’ve been acting a little funny, and I just want to be here for you.”

“Nothing’s wrong!” his last burst of anger seemed to do Ben in. He was quiet for a second. “I’m sorry, darling.  It’s just – I’m just – well, I don’t know. I’m sorry. I’ll be home for dinner at five. Okay?”

Still unable to understand this person on the phone, Lila managed an “Uh huh.” Just as Ben was about to hang up, she remembered,

“Oh honey! Honey?”

“Yeah?”

“Remember, you need to pick up Bryan from baseball practice at 4:45.”

“Oh, right. Of course, sorry. I’ll remember this time.”

Click.

The phone conversation was the last straw to break the camel’s back. Lila just stared off into the distance – her hand clutching the phone, the spiraled chord dangling. Closing her eyes she felt the swell percolating somewhere beneath. She felt the blood knock at her temples and the ache of her hand on the phone. She wished she could crush it. The homely appliance that had forced her to wake up – to think about the last several months – forced her to answer the question: “What is wrong with me?” Hunching over and catching back the tears. She hated soap operas – she hated the horribly acted raw emotions that her girlfriends seemed to drink like the ether of life. They were just bored with their perfect lives – but Lila, felt on the verge of something deep, and was losing the battle. The phone began to yell at her, the squawking bleep of an unhappy appliance – wanting desperately to go back to the safety of its hook. Lila, as unhooked as she was, felt sorry for herself. “Just admit it!” She felt like calling her mother – a thing that she never used to look forward to.

“Mom?”

“Hey honey! I’m sorry, but I’m with the girls now, can I call you later?”

“Mom? um, okay – I guess.”

“Pumpkin? Is everything alright? You sound upset.”

“No, I’m okay Mom, promise. Just call me later.”

“Darling, I wasn’t born yesterday. First off, you never call your lonely mother, and I never taught you how to lie. Talk to me, pumpkin.”

Lila let it spill – the whole string of strange changes with Ben. The mounting collection of concerns she never had to deal with before. Will Ben get fired? Does he love me? Will Bryan be all right? Does he love me? Is he really sick? Does he love me? If Lila’s Mom knew anything, it was that life was hard. Her parents were immigrants – she worked in their grocery store for long hours and dealt with her father’s early death. The woman could wash dishes in scalding hot water – her leathery hands immune to a light touch and well acquainted with hard knocks and pinpricks.

“Now Lila – you’ve got to recognize how lucky you’ve been. You married a wonderful man who I knowloves you very much. You have a beautiful son, and you’re healthy and strong as a mule.”

“Mom, I know – but I need him back. I need him back!”

“I know this is a frightful thing you’ve got going, but you come from pretty strong stock. You got this one in the bag! You need to get over your fear, and call a doctor. Talk to someone about this, and get it off of your own chest. You need some breathing room, Pumpkin. If Ben is sick, it’s better to know what it is than to keep wondering. Do it for him, do it for Bryan, and mostly – do it for yourself?”

Lila loved her mother – she always knew what to say. She gave her the clarity she needed through all the tears and the confusion.

“Pumpkin – you go look up some numbers right now, and set up an appointment. And who knows, maybe Ben will start shaving up nice and pretty again in no time!”

Lila let go a healthy laugh, one that shook her slightly and relaxed her reddened face. She thanked her mother, promised to keep taking the garlic supplements, and hung up the phone. Wiping off her drippy nose, she set out to complete her daily tasks – enveloped in a new security – one that makes no guarantees, but one of hope that the fibers of her family’s life will come back together again. She wrote down the number of her family doctor on a note pad – she’d make an appointment tomorrow and get some advice. She didn’t know if she needed a marriage counselor, a psychologist, or what. But the doctor would know, and the mere fact that someone else would shoulder some of her burden and give guidance under these circumstances was what she really needed.

Dinner was made and she waited in nervous anticipation for her husband to return. She didn’t know what was wrong with Ben – but she would hold on to the belief that she knew who he was, and that the unquestioned truth of their mutual love for one another wasn’t going anywhere fast…not if she could help it. The clock slid past five, and she told herself to be patient. The phone rang.

“Hey, Lila? Hi, it’s Mrs. Rolfes. I’m bringing Bryan home.”

“Um, thank you Mrs. Rolfes. I’m so sorry, I guess Ben forgot about Bryan’s practice. Thank you so much.” Lila felt the old pulse of dread come back – the worry that Ben was forgetting about those things that he used to wake up for. Mrs. Rolfes and her preening tone told her that she was not especially pleased to watch after other children. Lila knew that Mrs. Rolfes thought that she and Ben were bad parents – or at least uninvolved ones.

She finished the pleasantries with Mrs. Rolfes and called Ben’s cell phone. She tried to push down her anger at him. Lila told herself that Ben wasn’t well – it’s not his fault that he was acting this way. He didn’t pick up on the first call, but answered on the second.

“Hey honey. Sorry – I just lost my way a little. I’ll be home soon. Can’t talk now, I’ll run a light or something.”

Bryan was home before he was – sullen that Dad forgot to pick him up again. He clomped upstairs to put up his equipment and wash up for dinner. Half and hour rolled by, and finally Ben pulled into the drive way. Leaving the car door ajar, he walked into the side door. All Lila could do is muscle out a supportive and loving smile, give her husband a kiss on the cheek, and usher him in before the re-heated food got cold again.

For further reading on the topics discussed:

  • Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  • HD and Family: For greater understanding of family interpersonal dynamics and HD, click here

“Bryan’s Dad Wants Ordinary Back”^

In this story, we learn that Bryan’s Dad is neglecting the things he once loved: his pet dog, his son’s baseball games, and his job. Ben is dealing with the onset of physical and cognitive symptoms from HD and doesn’t know it yet. He feels that he is losing the grip on his old and comfortable life. This story touches on the day-to-day stresses associated with HD symptoms at home, in the workplace, and even while driving. Ben is in denial about these changes and we learn information about Ben’s family history that psychologically impacts him. This story seeks to set the symptoms of HD in a perspective outside of a text book.

There are those particular sounds we do not hear very often, but when we do our hearts quicken, our eyes open just a little wider, and our very synapses fire with understanding. It is truly an ageless phenomenon. Take for instance the discordant clang of an alarm in the middle of algebra class – A fire drill!The first gathered breath and spastic lifting of one’s head off the less-than-comfortable desk, a gut-fear. Will my locker burn down? Then the calm – Oh, it’s just a drill, no more class this period! The elation. Years later, that special collection of consonants and vowels, said by that special person, over a special dinner and clink of champagne glasses – “I love you.” Few words, or sounds for that matter, can really say all there is to say about what the body feels and the brain struggles to define about such moments – ecstasy.

To dogs, especially Auf, no sound resonates as clearly or so strongly as the one his beloved owner makes; ‘I love you’ pales by comparison. Most assuredly, the soft clinking and sensuous shape of a folded and dangling dog leash makes Auf go buck-raging mad. He skittles around the kitchen tile floor, each doggy nail scratching the sound of rain falling on a car windshield. Auf lives up to his name, aufing and playful whines, encouraging his owner to click on the long length of blue chord and metal attachment faster than the speed of sound. When the door opens and Auf trots out into the free expanse of sunshine, he can reasonably expect several things dramatically important to life: 1. The existence of other dogs to sniff. 2. The existence of plenty of squirrels to chase. 3. All the trees in the world to mark his unquestioned territory. To be quite honest, it’s as close to a spiritual experience as the canine species has yet to achieve. It’s true.

Auf occupies his downtime by guarding the backyard. It’s not a terribly large place, but it’s his, and he takes it upon himself to make sure nothing is amiss. He knows every nook and cranny, and this pleases him. When the new family of loud magpies took residence in the largest tree in the yard, this inspired some brow-furrowing and entertainment for Auf. These birds seemed to enjoy his dog bowl as much as he did. When he wouldn’t look, they’d take the liberty of snatching a piece of kibble – his kibble, and fly up out of reach. Such rudeness was persistently met with chorals of barking and a racetrack of activity around the base of the tree. Auf knew one day he’d catch the cursed magpies and end their rein of terror. However thrilling this pursuit has been for him, Auf has become increasingly antsy. It’s been far too long since the last time he heard his favorite sound – and this distresses him far more than a lost mouthful of kibble. Ben, his owner, has neglected his post of Auf-walker.

Truth be told, Ben hasn’t been up to par with the Auf-walking in recent months. As far as Auf can remember, Ben hasn’t taken him for a walk in a good month. Lila, his owner’s wife, has been pretty good at taking him out for a spin – but by no means does she ever take him to his favorite places, nor does she throw the tennis ball. Oh, the tennis ball! That’s the icing on the walking cake! Nothing beats the bouncing silhouette of a tennis ball in the forefront of a setting sun, the lake in the background, and plenty of crunchy grass under-paw. Nothing! It’s true, the last few times Ben took him out for one of their long walks, the ones that have made both Auf and Ben the closest canine-man relationship ever, had been less than spectacular. Ben couldn’t keep up with Auf – sometimes he even let the leash go. Auf, not realizing this, would run up ahead of Ben. When Ben caught up or Auf noticed and ran back, Ben wasn’t in his typical and universal good mood. Instead, he seemed aggravated – holding the leash more tightly, and yanking back when Auf got a little too excited by a carousing squirrel on the path. Not only had the walks been less free-flowing as they once were, they got steadily shorter as well. The tennis ball made fewer and fewer appearances. Auf was confused by all this. Ben was always a good throw – he could probably throw the ball clear across the lake if he wanted too! But Ben’s throwing skills had taken a nosedive. The ball wound up in the lake, or in a bank of shrubbery, denying Auf his long runs to and from the bouncing beacon. Auf didn’t really mind swimming to get the ball, or going on search and rescue missions to recover the ball – but his owner’s frustration at not throwing the ball where it should go was palpable. Frustrated, Ben would stop throwing the ball after a few moments and would take a seat next to the lake, sit, and just stare for the longest time. Not that Auf really minded that either, snuggling up against his owner by the lake was one of his favorite activities.

Although Auf was certainly willing to let a few sour ball-throws go by, he was keenly aware that his job was to support his owner. If Ben wasn’t feeling well, he would encourage him to try, and maybe make him just a little happier in the process. Auf would pick up the stagnant tennis ball and drop it on the ground next to Ben’s hand as he sat staring out at the blue. Wagging like a maniac, and jaws wide open in a slobbery grin, he’d wait for Ben to pick up the ball. After a few impatient moments, Auf would up the ante – and use his stubby nose to push the ball into Ben’s hand. Ben picked up the ball and pocketed it; then gave Auf a short scratch behind the ears, and continued to stare out into nothingness. Auf, feeling that he had failed to encourage and electrify Ben as he used to, picked a spot close by, and laid his head on his outstretched paws – occasionally looking to his owner with wide-set eyes.

Now, as Auf scouts the backyard, he forces himself to be uncomfortable. The next walk could come today, right? And if it did, he would be ready with as much territory marker as possible. Imagine for a moment that Auf was a cow. What do cows do if their owners don’t milk them? Can cows milk themselves? Probably not – but if they could, they wouldn’t waste the milk on a patch of grass in the middle of their pasture, would they? Auf lived by the same philosophy – he would hold it all in until he couldn’t bear it further. Needless to say, when dusk came slowly and his food was set out for him on the porch, he waited anxiously for his favorite sound of the dangling leash. It never came – and as Auf ate, all he could think was, “What a waste.”

Ben went to work, came home, went to sleep, went to work and maintained the beats that sounded the drum of daily life. He was a busy man – he had a job, a family, and a life. As ordinary as ordinary days can be, they are nonetheless busy. Although he had become accustomed to the routine-ness of a breadwinner, his mind had wandered from the busy that is going to meetings, changing the car’s oil, and mowing the lawn. He was busy trying to understand what was happening to him; to his body and his mind. Ben never grew bored of the busy metronome clickings of all the parts of his life – in other words, he never minded being this comfortable. What distracted him now was his inability to keep up with the metronome. He was chronically late; late to work, late to respond to his coworkers during lunch, and late to pick up his son, Bryan, from baseball practice. He was off beat – but what was worse, the beat was changing daily – ever so slowly, but perceptibly.

They started out small – almost silly in a way. One morning on the way to work, he started noticing how hard it was to concentrate while driving. Suddenly, he was aware of the cognitive acrobatics involved in navigating a 1500 pound hunk of churning metal. You had the accelerator and the brakes – all which had to be pressed in a certain way at a certain time. The vision too – you had to check the mirrors and the odometer. There are speed limits to consider, and fellow yawning employees scurrying like ants to get to their positions and maintain their drums for the remainder of the day. Changing lanes involved a small turn of the steering wheel, right-hand turns were a bit sharp, whereas left-turns were more arched and meandering. The road almost never stays straight the whole time; sometimes lanes appear, then disappear as they merge into larger streams of highways or ebb down to one-lane driveways, or pool into the wide expanse of parking lot. The thought hit Ben as he nearly side-wiped a car when changing lanes. The sudden jerking to maintain the road, get comfortably back into his lane, and resume the speed of traffic nearly gave him a headache. He had to be careful – he was almost passing stop signs he had stopped at faithfully for years. Nothing needs to be said about the perils of highway driving and making sure to get off at the right exit – changing speed, merging, and knowing where to go and how to go next.

Work was no better. He made a habit of stopping by the bagel shop to pick up a baker’s dozen for his buds at work and the secretaries that made his life so much easier. When he had time, he went through the Java Joe’s drive-through to pick up some juice. Appreciation was never lost on anyone, but in all honesty, people at the office had developed the bad habit of assuming that Ben would always bring in these amenities. Now, as Ben’s ability to drive began to suffer, the 15 mile commute to work seemed so much more daunting, and eventually became a 20, 25, 30 minute careful expedition. He often missed the beginning of morning meetings. Still stressed and adrenaline pumped from his commute, he forgot to maintain his up-beat attitude; wishing everyone a good morning, remembering to ask how Tom’s house renovation was coming along, and congratulating Diane on her new nephew. When these inconsistencies within himself became apparent to Ben, he drew within himself – upset that he couldn’t maintain his cocktail party mannerisms. The off-hand comments about the bit of lather left under his ear from shaving didn’t help either. People could see that he was different. It wasn’t some abstract concept; suddenly Ben became all too aware of the nakedness of his failing physical, cognitive, and emotional well-being. Honestly, Ben couldn’t wait to retreat to the confines of his office.

Although the quiet interior of his office relaxed him, Ben could not retreat from the signs that something was wrong. Daily reports were part of his daily work-beat. He reviewed the materials from accounting and an assignment from his boss – and, if all went well, typed up a memo to return to management by lunch. Ben would now sweat over the papers – all the orbiting acronyms and symbols looked like a steamy bowl of Alphabet soup.

“What did AcctP mean again? Accounts payable, or Actual Percentile, yeah. I think so – let me just ask? No, I can’t talk to anyone right now, not just this minute. I’ll figure it out, I will.”

Ben recognized that it took him much longer to process information than it used to. He would eventually figure it out, but ‘eventually’ became a euphemism. He began to pattern his desk and computer monitor with a quilting of sticky pad notes, reminding him of things that used to come to mind without help. Writing the memos after he’d organized his thoughts were a problem too. Ben would stop to ponder, sometimes minutes at a time, for a particular word to use.

“Annual reviews show an – Annual reviews show an? something, an increase. Yes, definitely an increase, but what type of increase was it? External? Expositiory? No no no no no!”

It would take a few minutes before “exponential” would come to mind. When it did, Ben would sigh with relief, but it was a rather unimpressive relief. Fine, he could get the reports done when he put his mind to it, but why did he have to exert so much energy to find one word in his mind? Sooner than later, Ben began to compile a list of words he was commonly having difficulty locating when he needed – but to his frustration, the list became so long, it took him almost as long to find it on paper.

The off-beats of Ben’s mind and body made him feel like an unreliable metronome. He felt that no one could trust him with anything. Lila, bless her, had reminded him time and time again to bring the briefcase before he headed out the door, to pick up Bryan after practice, and to take Auf out. He tried his best, but the stress of doing just about anything overrode Lila’s best efforts. Auf was another matter – he never forgot that he had to take him out to the lake. Even though Auf was the second love of his life, he couldn’t bear to take him out anymore. He just couldn’t keep up with him anymore, and this worried him in a way that work couldn’t. Ben was failing his dog, a creature that really couldn’t do much without him. Auf was his responsibility – and he was steadily becoming unable to perform those things that make dogs happy. Much to his own surprise, Ben didn’t feel as guilty as he thought he would be when he stopped taking Auf out to the lake. Lila took up the task willingly, but Ben, as much as he knew he loved his dog, began to withdraw himself from the post of Auf-walker, and more to Auf-scratcher – and even that became an occasion.

Ben knew he should be upset by all of this – work, Auf, Lila, Bryan – all the things that once made his life turn – the things that used to beat rhythmically, like heart-beats. But, he really wasn’t. The lack of concern felt far better than constantly reflecting on his mounting list of inadequacies. He couldn’t play baseball all that well with Bryan anymore, so why bother? Ben had difficulty sorting through the mail and writing out the proper checks and envelopes for the right addresses – so why not ignore them altogether? For Ben, this felt like a good proposition. Although Ben graduated from novice to proficient in lying to himself about what mattered and what didn’t, he couldn’t stop himself from getting bogged down by his collection of failures. His attitude vacillated from apathetic, to incensed, to downtrodden, to frustrated, and back again – thesefeeling began to sound the beats of his daily life. Persistent and off-kilter, the sound began to deafen him, and for much of this, Ben could only try to hold his hands over his ears, and hope.

Denial is everyone’s friend we hate to love and love to hate. There’s no better way for describing it. Denial will give us a backrub, tell us what we want to hear, and suggest we go out for ice cream. Then again, denial becomes the person we wish would move as far away as possible, so we can change our phone number and never hear from again. Ben had become two people over the past several months. He became a man who could see what was happening to himself while driving to work and leaving the blue dog leash on the kitchen counter. He also became the man who saw nothing, heard nothing, and in turn, felt nothing. Lila told him one evening at dinner that she had met with the family doctor, and that he suggested Ben come in soon to talk with him. Lila did her best to sound upbeat and supportive – to point out that she knew something was wrong without actually saying anything of the sort. She used phrases like “just for a checkup,” or, “just a chat, you know, if anything is on your mind.” Ben didn’t know how to feel about this. Ben #1 wanted to say, “What do you mean – a chat with the doctor? I don’t need a check up! Stop heckling me about everything, let’s just eat already.” Ben #2 wanted to let the tears finally out, to expel the frustration and confusion that was polluting his being. This Ben wanted to open up to his wife, to be able to keep up with his son, his dog, and with his old rhythm of life and say, “Thank you honey, I’ll see him tomorrow. I love you. The food smells wonderful.”

Instead, Ben took a new road, recently traveled, and said nothing. He pretended not to care because he couldn’t reconcile the Ben imposter and the Ben who was real, but horribly lost in himself. It’s not to say that he had no idea what was happening to him. He did – he just didn’t recognize it yet. Ben had snapshots of his teenage years, where his mother began to change. She was an expert mother, she ran a leak-tight household and had a passion for knitting. After Ben left for college, he would come and visit during breaks and see the relationship between his parents deteriorating. The house became dusty – running his finger over the living room lamp, he collected an earplug-sized formation of fuzzy grey. Dad left, and Mom moved closer to be with Ben after he graduated, got married, and bought a house. She aged quickly, eventually needing a full-time nurse. His mother was incredulous about seeing a doctor – she had a patently unshakable fear of doctors because her father went to an institution and never returned. She would call Ben to take her to the fabric store to buy yarn, sometimes three of four times a week. Ben remembered her strange mood swings, her shaking hands, and wondered if she could even knit anymore. More importantly, he wondered if she was still the woman who raised him – Ben couldn’t see that woman any longer, but felt obligated to indulge her. Each time they went to the store, Ben’s mother relaxed, picking a rainbow of different yarns, which Ben happily paid for. Unlike past years, Ben never received his Christmas bulky sweater, or multi-colored scarf. When she died, Ben cleaned up her house, and found closets full of unused yarn – covered with dust. The knitting needles were nowhere to be found, and Ben could only conclude that someone had thrown them away.

So, answering the question of “what is happening to me,” was more delicate and far too personal to approach with any steadfast rationale. To say Ben saw his deteriorating mother within him would be inaccurate. That Ben had been quieted by the convincing speech of denial – he was fine. If Ben couldn’t lie to himself – like when he forgot his briefcase for the third time this week, he would decide it didn’t matter at all – he had missed the meeting anyway and nothing he could do would change that. But all this was not lost on Lila, the family physician – or Ben, completely. Although Ben said nothing about his wife’s meeting with the family doctor, he said nothing about the appointment she had promised to make for Tuesday, 9:00AM sharp. He would go, and as apathetic or irritable as he might be that morning to have his wife drive him – “just to make sure he got there on time.” He went for a short walk with Auf that evening – which his dog was all too happy to be on, and went to bed. He didn’t thank his wife, nor did he bring the tennis ball on his short walk around the block; but the most internal piece of Ben, the piece that really mattered, was ready to get back some hope that would bring the beat of his life, back to ordinary.

For further reading on the topics discussed:

  1. Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  2. HD and Family: For greater understanding of family interpersonal dynamics and HD, click here
  3. This links to the “Cognitive Symptoms” page of the website, which provides information on what cognitive abilities could be compromised by HD, what causes these symptoms, individual variability with respect to symptoms, and treatment options available.
  4. HD and Driving: For detailed information about HD and driving, please link here.
  5. Family History: This links to information on the inheritance of HD on the HOPES site. This provides the genetic background of inheritance and approaches practical family questions concerning the potential inheritance of HD. It may be insightful to visit the Genetic Testing part of the site as well.

“Bryan’s Dad Visits Dr. Kealy”^

Lila made an appointment for her husband to visit the family physician. It is difficult for Ben to confront his feelings and the truth, but during a “physical,” Dr. Kealy’s charm and listening skills manages to open Ben up. Ben talks about the changes he has dealt with and speaks openly about his mother’s mysterious condition. Dr. Kealy tells Ben that his preliminary thoughts are that he has HD, but Ben should undergo genetic testing to find out the truth. Ben takes the information bravely and Dr. Kealy suggests he should talk to a patient of his who has undergone genetic testing already. Ben realizes more than ever how much his wife and family mean to him. This story attempts to underscore the difficulty and the necessity of coming to terms, both medically and emotionally, with the potential of an HD diagnosis.

“Ben! Lila! Good morning and it’s so wonderful to see you!”

Doctor Kealy had been Ben’s family physician for many years now. The man had a contagious exuberance and it spread to his patients like wildfire, no matter their situation. Today, the wildfire had consumed Dr. Kealy’s face, his reddened cheeks commanding attention – not from embarrassment, but from sheer energy.

“We are having such revitalizing weather recently! Don’t you think? How’s Bryan? I imagine his earache went away without too much trouble. Such a healthy young boy – still playing baseball like a champ, I’m sure! Oh yes, just follow me back here. We’ll talk in my office – I apologize in advance for the clutter!

Dr. Kealy was a charmer – friendly and engaging. As difficult as it might be to imagine, he was an excellent listener. Not just to what was said, but more importantly, to things that he knew his patients could not say. When Lila called for the meeting, she didn’t tell him very much about the “problem.” But Dr. Kealy heard what Lila could not put into words – her worry, the relief in talking with him, the entire lingering ache of unknowns he could hear from her without the words. Now that she was here, he could see the sparks of hope all over her face – trying her best to smile enough for herself and her husband. At first glance, he had also read Ben – whose face remained flat like the surface of a deep pond. Something was happening underneath, that he could tell, but what was his responsibility to discover.

They made their way to Dr. Kealy’s office. He hadn’t lied, his office was a mess; the “every-man” qualities of imperfection in Dr. Kealy were strangely comforting to Ben and Lila.

“Oh, I’m so sorry! I forgot to bring another chair in here – doctors can be forgetful too, you know. Lila, please take my chair.”

“Oh, Dr. Kealy, that’s not necce—”

“Lila, I insist.” He encouraged her with a broad smile.

Dr. Kealy brought around his leather padded chair, and brought a foldout chair for himself from the hall. He crossed his legs and kept his hands in his lap. Dr. Kealy felt the tension in the two people who sat across from him: The wife who had so much to say – too much to explain clearly, and unsure where to begin. The husband who had the most to say, but was certain to do the least talking.

“I hope the two of you are comfortable. I just want us to have an informal discussion here; to open up a little to one another. I know I don’t eat dinner at your house, but I do want to say I care for the two of you like family.”

“Thank you, Doctor,” Lila replied, all too happy to break the silence, “I’m sure Ben and I will have no problem at all speaking with you…right honey?”

Ben shifted around in his chair and produced an almost inaudible grunt; a sound that spoke to his uncertainty, but also to his uneasiness about doctor’s offices. Whether or not it was an agreement or a struggling effort to respond, Lila couldn’t tell.

“Well, to begin – Ben. Your wife called me a few days ago and was a little concerned about a few things. As I understand, you haven’t been feeling too well lately. Of course all of us want you to feel at your best, so if you could explain to me about—”

“I’ve been a little off, that’s about it,” said Ben, rushed syllables shooting like a machine gun.

“I understand, we all have our ‘off-days.’ How long have you been feeling like-”

“I don’t know, um, well, maybe a couple of months.”

“Honey, please just let the doctor finish. He’s only trying to help! Dr. Kealy, I’m sorry, my husband has been rather…unpredictable with his moods?”

“Oh geez! Lila!”

“Shh shh shh,” cooed Dr. Kealy, “let’s calm down a little. Ben, your wife and I really just want to help. Lila, let Ben speak up when he’s ready.”

Lila sighed a little. She looked visibly disappointed – the office visit wasn’t playing out the way she had expected. After talking with her mother and making the appointment, she was certain that everything would go ‘up’ from here. Now, it seemed like Ben was shutting himself in – she felt the feelings of helplessness creep back in. She loved Ben, she truly did – and she would do everything in her power to help him. In all honesty, talking about the last several months were in his, and her family’s, best interest.

“Well, Dr. Kealy – my husband has been a bit off lately. Like I said, he’s had some mood swings. Sometimes he gets angry about the strangest things – or doesn’t seem to care about things he normally gets very involved in. Like work – he forgets his briefcase, or forgets to pick up Bryan from baseball practice? He doesn’t seem to want to take our dog out like he once—”

“I can’t, honey! I can’t – you know I would love to take Auf out more, but I just can’t!” Ben hunched over, his hands on his knees – veins beginning to become visible on his strained neck.

Lila leaned back, obviously saddened by Ben’s outburst. She looked appealingly to Dr. Kealy, who reclined and looked over the scene. His expression was knowing, and his brows furrowed over his good-natured face. He popped his knuckles slowly, and got up out of his seat.

“Well, I believe we may need to take this a little more slowly; one step at a time. Ben, do you know when your last physical was?”

Ben stared blankly at him, his forehead glistening with the beginnings of a nervous sweat. He shook his head meekly.

“Yes, I don’t believe you’ve been checked out in a while. Why don’t we go to another room and just run a quick check-up. Okay?”

Lila seemed defeated – as if Dr. Kealy was evading the real question, but relieved that her husband was at least in the doctor’s hands. The truth was, Dr. Kealy knew exactly what Ben needed. It was not as if Ben could not stand to be around his wife, but his guilt and embarrassment had taken over. Dr. Kealy knew that if he was going to be of any help here, he’d need to speak with Ben alone. Before he and Ben left the office, Dr. Kealy turned to Lila and gave her a comforting nod.

“We’ll be back in a few moments. Help yourself to some coffee in the lounge if you’d like.”

Ben had inherited many things from his mother: An intense affection for long Sunday afternoons outdoors, her blue eyes, her distaste for mayonnaise, and unfortunately, a fear of doctors. Dr. Kealy knew this without having met Ben’s mother. Out of the family, Ben came to the office far fewer times than Lila or Bryan. He also knew that Ben needed, no wanted his help. Through the aggravation and the strain he knew that Ben was afraid, and had so much to say.

“Ben, why don’t you take a seat on the table. It’s not the best seat in the house, but it’ll have to do.”

Ben chuckled a little – the first break in his downward turned face that morning. Dr. Kealy set to work, checking Ben’s reflexes, blood pressure, eye movements, and so forth. All the while, Dr. Kealy talked and talked. He ran the gamut: baseball, fly fishing, his own family life and the outrageous cost of car repair. The man had a way about him – he made everything into a joke: “Who knew that running over a curb would flatten my pocket book!? I killed that car worse than I killed my wife’s African violets!”

Ben had visibly loosened up. He was nervous though, his thumbs swirling around one another like a wind vane. Dr. Kealy had completed his exam, and was not too pleased with the results. He had noticed a slight twitching in Ben’s movement and slow responses to stimuli. At one point, Dr. Kealy had thrown Ben a small bean-bag shaped like a baseball; “Who’s your bet for the World’s Series?” As the ball sailed in a wide lob, Ben reached up awkwardly and missed catching it by a long-shot. He was obviously embarrassed by the miss, and Dr. Kealy glossed over the moment as if nothing had happened at all.

“So tell me, what’s been on your mind, Ben?”

Ben hesitated, weighing some invisible balance in his mind.

“Well, doctor – my wife’s not too far from the truth. It’s hard for me to talk about it, but I feel like I’m falling apart.”

“Hmm. Well, I definitely feel like that time to time Ben – but it seems like this has been happening pretty consistently for a while.”

“Yeah. I guess you could say things are slowly getting worse. I mean, I feel stressed all the time now, and really defeated by things I can’t do too well anymore.”

“Like what, Ben? You mentioned you can’t take the dog out like you did before. Is that it?”

Dr. Kealy sat back and listened. He heard about Ben’s inability to stay active with his dog and his struggles to deal with the rigors of his workplace. More importantly, Ben spoke about how all these changes were frightening to him – how he sometimes doesn’t feel like himself. Like somebody who wasn’t anything like him was taking over his body and his emotions. Dr. Kealy sat back serenely with an encouraging expression – inserting comments where necessary to keep the conversation flowing. He noticed how difficult it was for Ben to explain how that last several months had transformed him, at first slightly, and later, more perceptibly. The difficulty was not only in the sensitivity of the subject, but also in Ben’s ability to accurately express himself. He paused every now and again, exerting much effort to find the right phrases or the right word to describe how he once acted, and what behavior now replaced it. Dr. Kealy heard every word Ben said, sorting out the facts in his head and piecing together a puzzle.

After Ben had told everything and looked to Dr. Kealy with a longing expression, hoping something,anything, could be prescribed to bring his old self back. After a few ponderous moments, the good doctor spoke:

“Ben, do you have any brothers or sisters?”

“No. Mom always wanted to have more children…but I never minded being an only child.”

“Were either of your parents ever hospitalized for any reason? I’m afraid I never had the pleasure of meeting either of your parents.”

Ben pressed his lips together – hard. His face muscles stood out and his lips turned white – as if he had seen a ghost. Looking away from his doctor, Ben remembered looking through his mother’s small house, searching for the knitting needles that had once defined her. He shook his head.

“My mother got sick when she was about fifty years old. She was afraid of doctors, and as much as we – I mean – the family, tried to get her to go, she never would. We got a full time nurse for her, though.”

“So, she was never diagnosed?”

“No.”

“I know this is probably very painful for you, Ben – but could you tell me about what your mother was experiencing?”

Ben caught on his own breath and closed his eyes. When he looked up, the pools of his eyes had deepened.

“I don’t know. No one ever knew. I don’t know.” The machine gun edge to his voice had returned. Dr. Kealy, softened his expression and nodded his head. He rose and placed a hand on Ben’s back – it was hot and damp.

“You realize Ben, your family is only trying to help you the way you wanted to help your mother? I don’t like talking about this any more than you do, but was she having some of the same problems you’re starting to have? Difficulty getting around? Not being able to do things she really liked to do?”

Ben felt as if knitting needles were working away in his brain, knitting a startling and horrible picture together. He knew what the doctor was saying, but wanted desperately not to hear him. He felt like dissolving in the presence of this reality, the truth that had been exhumed from memories past. Ben sat silent for a long time. Dr. Kealy couldn’t read him, but knew that what Ben would have to say next would be one of the most difficult tasks ever.

“Yeah, I mean, yes. Mom was having the same problems…” Ben looked up to the ceiling, feeling at once a huge weight lifted from his shoulders and a heavy cloud forming to replace the vacancy.

Dr. Kealy let out a small sigh – empathetic but deliberate; “Ben, what I will tell you now will probably be something you won’t want to hear, but something you definitely need to. I can’t say with absolute certainty what’s causing all the changes you’ve experienced. I’ll refer you to a neurologist, but you’ll have to take a trip to the city to speak with a specialist.”

Ben felt numb, but tried his best to listen attentively. Dr. Kealy seemed to be wrestling with himself for his next words. In a moment of courage, Ben finally took charge:

“Dr. Kealy, if you know what’s wrong with me, you can tell me.”

Dr. Kealy looked steadily at Ben, “Like I said, I can’t say with absolute certainty…But I think you may have HD…Huntington’s disease. It’s a neurodegenerative condition.”

Neurodegenerative? You mean?”

“Ben, we can’t know for a fact until you get more thorough tests. You’ll need an MRI and the neurologists at the city hospital will be able to tell you more. But our testing today has revealed some of the primary symptoms associated with the disease. Your loss of motor control, the slight shaking… What you told me about working, driving your car, forgetting things – they all might be some of the behavioral or cognitive symptoms beginning their onset.”

These moments don’t often happen. Those times where an inconvenient, yet already noticed truth make their appearance. Not just any truth, but one that will change your outlook on the future, your understanding on what is currently happening, and forces the past move into a new perspective. It can be, and often is – a tragedy. But, it is in such moments that we can see what someone is truly made of. In the face of an oncoming 18-wheeler, do I scream and cover my eyes, or do I take control of the wheel and save myself from impending destruction?

Ben kept his hands on the wheel.

“I understand. What can I do? Can I get better?”

“We can’t do anything until we get better test results. HD is not something I have had a lot of experience with – it’s not very common. But I know it’s genetic and is inherited from one’s parents. I believe this is what your mother had… But today the field of medicine is far more advanced. If HD is what you have, there is no cure – yet, there are ways we can deal with the symptoms. There are medications you can take to relieve them.”

Ben retained his strong posture, soaking in the information Dr. Kealy had to offer and trying to organize his thoughts.

“Okay. So I need to meet with a neurologist you say?”

“Of course, I can help you get an appointment. I’ll also find some information; there are some incredibly helpful websites you can visit to prepare for your appointment. Since HD is genetic, you will have to get tested to confirm the results… It is not an easy decision to make, and you will need your family’s support.”

“I understand.”

“I’m not sure if there are any support groups in the area, but I had a patient some time ago that was tested for the disease, genetically tested. I’ll get in touch with her and see if she’d mind talking to you about the process. She was tested before any symptoms emerged…”

Ben asked a few more questions, and then readied himself to go back to Dr. Kealy’s office. He realized more than ever how much he needed his wife… and how dearly she needed to know what was happening. They were a unit. They loved one another more than anything, and she needed to know everything.

Dr. Kealy followed Ben out the door, one hand on his shoulder. They made their way back to his office and opened the door. Lila looked up from a magazine, and tried her best to smile. Dr. Kealy sat down at his chair, his hands placed lightly behind his head. Ben bit his lip.

“Honey, there’s something I have to tell you…”

For further reading on the topics discussed:

  1. Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  2. HD and Family: For greater understanding of family interpersonal dynamics and HD, click here .
  3. Physical Symptoms of HD: This links to the “HD Basic” page on the website. It provides useful knowledge on the physical symptoms associated with HD, primarily chorea.
  4. Cognitive Symptoms of HD: This links to the “Cognitive Symptoms” page of the website, which provides information on what cognitive abilities could be compromised by HD, what causes these symptoms, individual variability with respect to symptoms, and treatment options available.
  5. Family History: This links to information on the inheritance of HD on the HOPES site. This provides the genetic background of inheritance and approaches practical family questions concerning the potential inheritance of HD. It may be insightful to visit the “Genetic Testing” part of the site as well.
  6. Genetic Testing: This link explores a variety of topics on genetic testing, such as: testing guidelines, what takes place during genetic testing, what the test actually does/means, accuracy of the test, confidentiality, and the benefits/consequences of a genetic test for HD.

“Bryan’s Dad Plays the Game”^

In this story, Bryan’s Dad decides that life is a game, and no matter how bad the odds are, it’s worth playing. He begins to confront his fears about having HD and decides to meet with Dr. Kealy’s patient, Trisha, to get more information about living with HD and genetic testing. They become fast friends but spend most of their conversation talking about their histories with HD. Trisha gives Ben encouragement and advice, but also raises the question in Ben’s mind if his son, Bryan, will eventually develop symptoms for HD. Notably with higher spirits, Ben and Trisha plan on having a family dinner at Ben’s house, strawberries included.

“Ben! Come on down! You’re the next contestant on The Price is Right!”

Bob Barker had been around ever since Ben could remember. He was a symbol of America, but more importantly, a symbol of Ben’s weekend mornings with his mother. He had always wanted to be on the show. Not because he was an expert shopper, but because of the exhilaration of seeing his face surrounded by flashing lights on television. Every Saturday, like this one, he woke up to Bob’s perpetually smiling face and dogged public reminder to spade and neuter all the pets of the world.

As Ben sat in front of the big screen, feet propped up on the ottoman and a plate of eggs and bacon on his chest, he wondered what he should do next. Lila had cleaned up the breakfast table already, and was busily scouring the internet, studying up on HD. She had taken up the reigns – all the nervous energy and worry she felt about her husband was conducted over to the keyboard. Bryan was out in the backyard, shooting Nerf guns with a friend from school. Lisa squealed as Bryan shouted, and of course, Auf added to the clamor. Ben finished the last of his breakfast during a commercial break. He knew he should call her – he owed it to his family and to himself to talk to Trisha. She knew what he was going through and could talk to him outside the sterile confines of a hospital or doctor’s office. Dr. Kealy had called and said that one of his patients, Trisha, had agreed to speak with Ben if he desired. Dr. Kealy explained that Trisha had been genetically tested before she had any symptoms of HD and that she was reallynice – extraordinary even. Despite his doctor’s enthusiasm, Ben was confronted with a wash of conflicting emotion. Calling Trisha would make things unrelentingly real. It was as if Ben saw HD in a distant world, like the people on TV. Now, it was as if he was thinking about calling Bob Barker himself!

Lila took Ben’s hesitation in stride. Her internet sleuthing had given her an understanding of what people with HD go through – the emotional strain imposed by the progression of the disease and the uncertainty of Ben’s situation in general. She resisted diagnosing Ben’s behavioral symptoms as she saw them, knowing full well that her husband might be “depressed,” “apathetic,” or in “denial” – but also that her role was to provide a stress-free environment. If she couldn’t go to work to make things easier for him, she would definitely make sure her “house” was a “home”. If that meant she left the sticky note with Trisha’s number on Ben’s desk and allowed his Price is Right fix, so be it.

Ben stared at the screen as if in a trance – but his mind was boiling over. As Ben thought about the show, he imagined that in many ways, he was already living in The Price is Right… with one exception – everyonewanted to be on the show, not him. The odds of having HD were pretty low – Lila and Dr. Kealy had told him as much. The odds of getting called down by Bob Barker were abysmally small too. However, Ben had been one of the rare individuals confronted with the issue of getting tested for HD – “Come on down!” indeed. It was difficult not to feel sorry for himself. Although Ben didn’t really know if he had HD, he felt confronted by so many facts that hope seemed to become a hopeless option.

An old woman just lost Plinko and a man was called down from the undulating crowd. He rushed down as if all the jumping bodies and waving hands catapulted him to the stage. It wasn’t as if Ben was forced to get tested. He wasn’t forced to call Trisha – he would decide when he would come on down – not HD. But still…confronting the problem or running away and never knowing for sure…which was worse?

A middle-aged woman from Phoenix guessed closest to the price of an elliptical exercise machine. She jumped up on stage, her mommy jeans cinched up past her belly-button. Bob Barker unveiled one of the gates to the next prize – “Doris! You can be the next owner of? A brand new BOAT!” The woman was from Phoenix – Phoenix! What on earth did she need a boat for? From the look on poor Doris’ face, you could tell she didn’t have a swimming pool let alone a lake, and was asking herself the exact same question. Ben understood that getting “lucky” was most certainly relative. People get struck by lightning? that’s pretty “lucky” in a perverse twisting of the word, right? Doris played the game anyway – it didn’t matter if she wanted the boat, she played the game nonetheless. Ben thought about this; life’s a game too, you just have to play the game. He may have not got lucky the way he wanted, but even if he – if Doris – didn’t like their prize, there was always the “Showcase showdown.” There’s always something to look forward to if you know how to play, and believe the game could be won. Ben sat up slowly and turned the TV off before the game ended. He figured it was about time to give Trisha a call.

Ben washed off his plate and looked out to the backyard from the kitchen window. Bryan was throwing a tennis ball back and forth with Lisa – teasing Auf who thought all tennis balls were his and his only. Auf leaped into the air to intercept the ball. Bryan was smiling.

Lila called from across the living room, “Honey! Is the show over yet? Remember we were going to the farmer’s market today.” Lila poked out her head from their bedroom. Her lips turned up in a smile, the apples of her cheeks seemingly glossed like she was in a toothpaste commercial. “I’ll remember to bring the sun-block this time!” She winked.

For everyone, there are countless reasons to live. For Ben, there were two big reasons to start living the best he could. Ben returned the enthusiasm of his wife, and said he’d be ready in about an hour. Lila returned to the computer and Ben picked up the bright yellow sticky note with Trisha’s number. He paused before dialing the number, rubbing around the edges of the post-it. Then, as if Ben decided not to think too much about it, he dialed.

“Hello?” A woman answered in a Southern accent, a little out of breath.

“Um, hi. Trisha?”

“Yeeees, this is she” Although she seemed in a rush, Trisha’s voice was loud and friendly.

“Yeah? My name is Ben – did Dr. Kealy—”

“Oh yes! Of course, Dr. Kealy told me you might call. How are you doing?” Her Southern drawl became immediately welcoming, but Ben’s heart was racing. He felt like he was in high school, asking someone he never met out to the prom. In reality, the situation had much higher stakes; he gathered himself and tried to match the brightness of Trisha’s voice.

“Oh, I’m doing alright I guess. This is a little awkward, but I was wondering if I could talk to you about some things? If you have the time of course.”

“I understand, Ben. But I was just running out the door with my son, Todd – he’s such the handful! I would love to talk with you though.”

“Well if you’re busy…maybe another time.”

“Hmmm. I’m heading over to the park with the lake to tire Todd out some. I’m not a huge fan of phone calls anyway – do you live near the park? Maybe we could have a chat out in this beautiful weather we’re having today! It’d be a shame to pass up all this sunshine.”

Ben liked Trisha. He didn’t like talking on the phone all that much either, especially not about things like this.

“Sure, Trisha! That’d be nice. I have a twelve year-old son, Bryan. I bet he’d love to come along and give Todd some company.”

“Well that’s mighty fine – Todd’s ten, I’m sure they’d love to play around some. Okay then, we’ll be near the lake. Todd’s always trying to catch some fish with his little fishing pole. He never catches anything, but what can I say – like father like son!”

Ben and Trisha laughed a little more over the phone, their goodbyes interrupted by a loud crash in the background. Trisha sighed and said she had to go and see what Todd was “up to this time.” When Ben put the phone back on its hook, he looked up to the ceiling. He had felt a strange sense of camaraderie with Trisha. They hadn’t talked about HD at all, but the mere existence of someone who could identify with the “game” Ben was presented with was comforting.

He walked to the backyard door and yelled for Ben and Lisa. Lisa said she had to go home soon, but Bryan and Auf were more than ready to head over to the park. Ben felt the strange sensation of inevitable change on the way to the bedroom. Lila was getting ready to head out to the market, a light summer dress of butter yellow and flip flops.

“Hey honey.”

“Hey darling, are we about ready to head out?”

“Well, I just got off the phone with Trisha and…”

“Oh did you? Everything go well?”

“Yeah, it did. I’m heading over to the park to have a chat with her. I know I said we’d go to the market, but can we go tomorrow?” Lila’s smile held the underpinnings of the pride she had for her husband. She understood what guts it took to call Trisha – to confront his fears and to take action.

“Sweetie. Don’t worry about it – go to the park and take Bryan and Auf if you can. I’ll head over to the market to get some strawberries for tonight. Okay?

“Okay.” Ben leaned over to kiss Lila.

“I love you.”

He raced to get his shoes on and to ready the leash before he heard a reply. There wasn’t a need for one at all.

On the way to the lake, it was difficult to say who led who. Ben let Bryan walk Auf, who was tugging the boy along. Ben meandered a little behind, keeping Bryan in sight.

When the lake jumped into view, he saw a skinny boy with blond curly hair on the small dock jutting out into the lake. He was barefoot and leaning over the edge of the railing to cast the lure. On a bench nearby he heard Trisha’s familiar voice warning Todd that if he fell in snapping turtles would “bite his little toes clear off.” Ben was slightly shocked to see that Trisha looked exactly like she sounded; a slightly rounded woman with soft big Southern hair and a stack of magazines, most likely Martha Stewart Living. Ben was shocked because Trisha looked so ordinary. He wasn’t sure what he had expected to see, but it was surprising to see that HD could touch anyone – even a southern-born house wife watching her young child from a bench in the park.

Bryan was dragged by Auf to the clearing near the lake – the dog leaping and mouth agape waiting for a tennis ball to chase down. Somewhat shyly, Ben approached Trisha who had already seen him and waved him over to her side. She slid the magazines away in a gigantic tote bag while she shouted at Todd.

“Tooooooooodd! Look who’s here to see you. You see the boy with the dog over there, that’s Bryan – why don’t you say hi.” Todd dropped the fishing pole, which almost teetered over the edge into the lake, and bounded barefoot toward Bryan and Auf.

“Well, Ben – it’s nice to meet you! It looks like Todd and Bryan are going to get along just fine.”

“Looks like it. Mind if I take a seat?” Ben motioned to the space that Trisha had cleared.

“Of course! Of course!”

They looked out at the boys and the dog quietly for a while, the sun was high and the lake glistened in response. Trisha looked over at Ben.

“There’s really no easy way to start talking about this, but I’d like to help you any way I can.”

“Yeah, I know. I haven’t gone to the doctors – the specialists I mean – about anything yet, but I already feel like it’s been a long road. I really do appreciate you coming out to talk with me.”

“A long road is a way to put it, no doubt. But one that you don’t have to let get the best of you. I was tested a few years back – after my father was diagnosed in his mid-fifties. I remember being so worried about him! No one knew what was happening, everyone just saw him changing steadily, little by little. I had moved by then so it was really hard not being able to be around him all the time – not being able to help. When we found out it was HD and that it was hereditary…Definitely a long road.”

“I’m so sorry to hear about that…My mother was never diagnosed, but I think that she had HD – or at least that’s what I’m told. I just don’t know all that much about HD or testing, or what have you. I’ve already started to see some changes in myself—”

“Well, let me just tell you that getting tested is one of the hardest but one of the best decisions I ever made…” Trisha looked out over the lake and then craned her neck to make sure Todd wasn’t causing havoc. She looked into Ben’s face, “I have a family – a loving husband and Todd. I have things I want to do. I had to know so I could prepare. You know what I mean? I had to stop just sitting and wondering if I would be next, if my life would just change out of nowhere. I had to be in control, to start thinking about what I could do to live my life to the fullest, to the healthiest, so I could push off the symptoms for as long as possible.”

“But doesn’t getting tested make things too real? I mean, I’m just not sure I want to know everything – it’s just too much.”

“Honey,” Trisha took on a rather stern and motherly Southern drawl, “we’re in the real world. We got a lot to deal with, but if you love your life and your family, pretending that nothing’s happening is gonna rip you up worse than knowing the truth.”

“I guess, you’re right.” Ben sat with his back pitched forward, looking at the ground.

“Look, I know it’s hard – and I’ll tell you everything I can about my experience – but you have to make the decision yourself. But if you’re already seeing changes, you should make an appointment with an HD Center of Excellence as soon as you’re ready. I can give you all that info whenever you want it…”

“No, you’re right. I do want to get some answers, I really do. Can you tell me anything about HD, I haven’t really read up on it yet.”

Trisha was quiet for a second, and assumed her loud friendly manner again. “Well, I don’t know toomuch about genetics and things like that, but I do make the best banana blueberry pancakes you have ever tasted!” She laughed at herself for a moment, and went on. “But I’ll tell you what I understand – doctors are better for this sort of thing.”

“I’m not too great with doctors to be honest. I get pretty nervous.”

“Well, rest assured. The Centers of Excellence are genetic testing centers that specialize in dealing with HD patients. They’re really good at what they do, and the whole process is designed to make sure you know everything about getting tested before you make the decision.” Ben nodded while Trisha spoke, encouraging her to continue.

“Anyway, I’m getting ahead of myself. I’m what doctors call “pre-symptomatic.” I underwent the genetic test before I noticed any symptoms. From what I understand, the genetic test looks at a certain part of your DNA that shows whether or not you’ll end up getting symptoms for HD. There’s a geneticmarker” that traces inheritance of the Huntington gene. Since Daddy had HD, they said I had a 50% chance of also having HD. It’s hereditary, and since you get one copy from your mom and your dad, you have a 50% chance…”

Ben bit his lip while he listened. He understood so far, but he wondered about the test itself. “So, I think I understand that hereditary thing, but what do the doctors do for the test?”

“The doctors will send a lab a small amount of blood and take a closer look at the Huntington gene that you have. Now here’s a part that’s kind of difficult to explain, but the doctors check if you have a Huntington gene that is a little out of whack. When your body turns the Huntington gene into a protein, a person that has HD will have a protein that doesn’t work the right way. It doesn’t work right because it has some extra pieces that make the protein not do all the things it’s supposed to do in your brain. The doctors call it an ‘expanded C-A-G repeat’. Like I said, I’m not a genetics expert and you’ll have to talk to the doctor about it, but if you have too many of these C-A-Gs, then you will eventually develop symptoms of HD. I found out that I had too many and that I’ll likely start getting symptoms before I turn 50…” Her voice toned down a little – Trisha was still grappling with the reality of knowing she’ll get a disease before she feels anything. Ben was in a different boat – one that he didn’t feel all that great being in either, but he understood that it must be difficult just “waiting for the day…”

“Trisha? I mean, how do you deal with all this…waiting? Doesn’t it scare you that one day you’ll start changing too?”

“Ben, darlin’ – the thing is I knew all about that before I got tested at all. Like I said, I went to an HD Center of Excellence. Before you can even go into a doctor’s office and get a genetic test you get counseling first. I spoke to someone who explained (far better than I did just now) about genetics, HD inheritance, and especially how the test and HD will impact me physically, psychologically, and especially emotionally. They made sure I understood about the risks I was taking in going through with the test – about how much knowing that I either had or didn’t have HD…let alone the possibility of Todd…” Trisha choked a little on her words, her folded hand covered her mouth and eyes squinted, staving back any tears that might come.

Ben was startled, he hadn’t even thought about Bryan having HD too. “Wait, do you know if your son has HD?”

“No, Ben, I don’t. I think it’s too early to think about something like that – plus, he’ll make the decision himself to get tested when he’s old enough. I just worry, you know? I don’t want my baby staring the same problems in the face when he’s my age…But I guess that’s one of the best reasons for people who are at-risk to go out and get tested…to make sure that this disease doesn’t get any farther and affects more people than it already has.”

Ben felt like he was just punched in the chest. He looked over to the lake. Bryan and Todd had their shoes off, slopping around in the grassy mud surrounding the lake’s edge. He bit his lip again – the mere thought of Bryan…Ben wouldn’t, couldn’t think about it.

Trisha saw Ben’s gaze over to the boys. She reached over and grabbed Ben’s hand and held it tightly. She waited till Ben looked her in the face, “I’m sorry to worry you darlin’, but this is just part of the reality, part of the game of life.” Ben shook his head, not saying anything. He saw himself on The Price is Right with Bryan in the crowd. He understood, but it hurt terribly. Trisha let Ben be with himself for a moment. Ben eventually looked over to her, a sign to continue their conversation.

Trisha was a little quieter now, as if reverence must be paid to something. “After they counsel you about getting a genetic test, you’ll get a neurological exam to see if you’re showing symptoms of HD. If you are, you’ll get details about them to see what they can do to ease the symptoms.”

Ben cleared his throat a little, “What exactly happens in one of these exams?”

“Well, as I recall, they tested me for things like my reflexes, my eye and body movement, hearing, balance…that sort of thing.” Ben remembered Dr. Kealy’s friendly conversations as he conducted Ben’s “physical.” Trisha continued, “Then the doctors will do some brain imaging to see if there are any structural changes…which could explain better some symptoms that are already showing up. The doctors also asked me about my family’s medical history. That helps them ‘finalize’ a diagnosis I guess…After the doctors examined me, they said I wasn’t showing any symptoms quite yet, but that my family history put me “at risk.” This is when they told me I could choose to go through with the genetic test if I wanted.”

“And you did?”

“Well, not really…not at first.”

“Why not?”

“I guess I was concerned about getting the tests done. Maybe like what you were saying earlier – it made things too ‘real’.”

“But I thought you said that it was the best decision you ever made.” Trisha looked at Ben with a broad knowing smile. She seemed to relive the entire waiting process – to say whether or not she smiled about it or in spite of it was a mystery.

“Now Ben, I said it was the best decision I ever made, but also the hardest.” Ben looked at Trisha empathetically, but also with hard squinted eyes. He was trying to figure out from Trisha’s expression what had happened. Trisha didn’t seem to want to discourage Ben from getting the test, but also seemed to understand why someone wouldn’t want to go through with it.

“Trisha, what were you worried about?” Ben paused for a moment, “Sorry, that was a dumb question, but is there anything I should know about – a reason why there is a “wait” period before the genetic testing stuff?”

“Well, if you’re worried about your confidentiality, you really don’t have to. All the test results are strictly confidential – the doctors at the HD genetic testing place I went to wouldn’t talk to anyone about me, not even my family. It was a decision for me to make, and me alone.”

“Then what were you worried about?”

“A lot of things, really. I didn’t know if I wanted to know – not because I couldn’t deal with the truth, but because I didn’t know if others could…” Trisha squinted her eyes, the sun was blazing. She ruffled around in her tote bag and pulled out black round sunglasses. Ben thought Trisha looked like she was putting on a disguise.

“Should I tell my co-workers I have HD? What about my health insurance? What about my husband – I kept thinking; if that man only knew what he was getting himself into? I just kept thinking about my mother dealing with daddy…it didn’t look like a cake walk. I guess I was worried if I could trust my family, friends, and coworkers to still treat Trisha like Trisha, not like a ticking time bomb.”

“I’m sorry Trish – may I call you Trish?”

“Sure, why not, that’s what all my friends call me.”

“Did anything happen? How did you decide to get tested with all those thoughts running through your head?”

“Ben, I think you’ll understand when you get there. You just have to take some things on faith…I didn’t want to have anyone see me so afraid, lacking the faith. I just dove straight in…I loved and still love my husband. I have my family and my friends…I got tested and I told them. They all still come to Thanksgiving, now don’t they?” Trisha cracked a smile – it was bittersweet.

“What about where you worked?”

“I decided to stay home after I found out – I figured I needed to be closer to my family as long as I could. I never told anyone who I didn’t really think should know. It’s really personal for me.”

“I understand.” A silence passed. Trisha breathed out a heavy sigh and began to fan herself with her hands.

“So I took the genetic test. Found out I would probably get HD. I guess I was kind of ready for the result, but no one ever truly expects to get news like that…you always have hope, you know?” Ben nodded.

“After you come in to get your results – if you want your results, the testing centers have psychologists and psychiatrists interview you. To see if you’re taking things well and to help guide your “coming to terms,” that’s what they call it anyway…It was rough, but I have to say – knowing was way better than wondering.”

“Really?”

“Absolutely.”

“Why?”

Trisha grabbed Ben’s hand again, took off her sunglasses to look directly into his face. “Because I make the best banana blueberry pancakes known to man and go to the park with Todd – and love every second of it. I have learned not to take the day to day for granted. I spend as much time as possible with my family, do the things I want to do, you know?” Ben nodded in understanding.

“There’s only one way to fight back. You live your life to the best you can, and start taking care of yourself so you can live that way as long as possible. I have my “plan.” I take care of my body – eat foods that keep me in tip-top shape, exercise…that’s all I know to do – to win the game. Trisha smiled again and slipped the sunglasses up on the bridge of her nose.

A moment later, Ben and Trisha heard shouting. Bryan and Todd came bounding up, covered in mud and grass stains, laughing.

“Momma!”

“Dad!”

The boys squealed in unison, “Can Bryan/Todd come over tomorrow!?”

Trisha looked at Ben, a signal that she’d have no problem with it. Ben mouthed a “Thank you,” and looked at his son.

“Bryan, as long as you get cleaned up and help Mom with the dishes.”

“Daaaaaaad!” Bryan said, pretending to be annoyed.

“Oh come on Ben, cut the kid some slack.” Trisha winked at Ben.

The group said their goodbyes and Trisha said she’d ask her husband if they could make it for dinner tomorrow.

“I know it may be inappropriate, but maybe we’ll have some pancakes for dessert?”

“As long as you think strawberries would go well on top.”

For further reading on the topics discussed:

  1. Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  2. HD and Family: For greater understanding of family interpersonal dynamics and HD, click here.
  3. Physical Symptoms of HD: This links to the “HD Basic” page on the website. It provides useful knowledge on the physical symptoms associated with HD, primarily chorea .
  4. Cognitive Symptoms of HD: This links to the “Cognitive Symptoms” page of the website, which provides information on what cognitive abilities could be compromised by HD, what causes these symptoms, individual variability with respect to symptoms, and treatment options available.
  5. Family History: This links to information on the inheritance of HD on the HOPES site. This provides the genetic background of inheritance and approaches practical family questions concerning the potential inheritance of HD. It may be insightful to visit the “Genetic Testing” part of the site as well.
  6. Genetic Testing: This link explores a variety of topics on genetic testing, such as: testing guidelines, what takes place during genetic testing, what the test actually does/means, accuracy of the test, confidentiality, and the benefits/consequences of a genetic test for HD.

“Like Father Like Son?”^

Ben and Trisha’s family have dinner together and discover that support and camaraderie goes deeper than their mutual concerns about HD. Although the gathering ends happily, Ben is agitated by the thought that his son, who is like him in almost every way, will eventually develop HD symptoms. Ben’s all-consuming worry causes him to shower too much attention on Bryan, who begins to guess that something is wrong. Lila encourages Ben to tell her what’s on his mind, and Ben explains his concerns for Bryan’s future and how he feels that he is to blame for Bryan’s assumed condition. Although Ben wants Bryan to get tested with him, Lila consoles her husband and explains that he was given the time to think about getting tested and that they should give their son the same freedom. Despite their mutual worry, Bryan’s parents decide to preserve their son’s childhood and to not treat their son any differently.

The two families had dinner together. Although the reason for their joining was HD, and the topic made itself known with the after-dinner wine, the dinner felt like a celebration. It was difficult to say why – each person around the dinner table had their own laundry list of concerns, their own desires – but as plates of steaming food traveled around it was clear that they all shared something even more visceral than food. It was unity in every sense of the term.

After the world’s finest banana blueberry pancakes, topped with the farmer’s market strawberries, made an appearance and then a quick disappearance as dessert, Todd and Bryan sped off to play. “Adult time” was just as relaxed as dinner, but as “adult things” go, the tone was more serious. Trisha’s husband, Robert, spoke with Lila about her internet researching. In many ways, Robert had filled the same shoes as Lila when it came to planning for their spouse’s doctor visits. They talked about recent news on the HD boards, something about CoQ10, and finally to the genetic testing process. Trisha and Ben spoke like the best of friends. Trisha thought that the air conditioning unit in her house was broken, but didn’t know if it just needed “more of that Freon junk.” In other words, they stayed away from talking about HD – Ben realized that Trisha was more than a source of knowledge, but a source of true camaraderie and support. Ben did say one thing:

“Yeah, I think I’ll be making an appointment soon, Trish. Do you have the doctor’s number?”

“Sure darlin’ – but let’s get some of that wine first!”

The night didn’t feel like a round-table support group. Not that Ben or Trisha would be opposed to a HD group like that, but it felt personal without being disquieting or overtly emotional. They laughed and everyone helped to clear off the table and do the dishes. As dusk drew closer, Trisha asked Todd to get ready to leave, since tomorrow was a “school night.” This was met with playful wailings from Todd and Bryan both; “Just 10 more minutes – pleeeeease!?”

After Trisha’s family went out the side door to their car, Ben and Lila got Bryan ready for bed. Lila made sure that Bryan took his shower and brushed his teeth. Ben made sure that Bryan finished his homework and when it was finally dark outside, went to tuck his son into bed. He hadn’t done this for a couple of years now. At first, Bryan was a bit confused,

“Hey Dad! I’m a big boy now, I can get into bed by myself.”

“I know, son, I know…”

Truth be told, nothing worried him more about the talk at the park with Trisha than the idea that Bryan, his son, may have HD. The dinner went smoothly, the chatting that followed was warm and comfortable, but the elephant in the room for Ben was how to cope with his son’s risk. But like many topics, there are simply some conversations that are near-to-impossible to have after a wonderful dinner, and whether Todd and Bryan would have HD was one of them. Ben wanted to know more about how Trisha and Robert felt about Todd’s risk. He wanted desperately to speak plainly with Lila about his fears…he just couldn’t.

He understood Trisha’s feelings on the matter – to worry about her son’s risk of HD was incredibly real, but too real for childhood. She wouldn’t want to rob her son of his childhood in any way. Ben also thought that Trisha could bear the weight of her own life with HD, but couldn’t deal with the weight of her child’s too. Ben had to agree…sort of. He knew the strength and maturity that Trisha had on the subject was admirable, but Ben’s fear ran so deep, he couldn’t help but draw conclusions – and diagnoses – for himself.

As Ben tucked Bryan into bed, he stroked his son’s light brown hair until Bryan made him stop, “Daaaaaaaad!” He looked into his son’s eyes, the same color blue as his. When he promised to leave Bryan’s room, he stood up and took a long look at the walls. They were covered with posters of baseball stars and Ben remembered his own room as a child, plastered with colorful images of his own sports heroes. As Bryan grew up, Ben was exceedingly proud how much Bryan was “the spitting image” of himself. No one could say that they didn’t look alike – they liked the same sports and were athletic and boys of the outdoors. Ben even had an Australian Shepard as he grew up. This wasn’t the end of it; even nuances of personality were identical between them. During dinner, Ben watched as Bryan pushed all the peas, mashed potatoes, and pot roast away from one another, like he was herding his food into separate corals. Bryan couldn’t stand when food touched each other. As Ben stared down onto his own segregated plate, he smiled. When Bryan ate his food clockwise, always finishing the vegetables first and leaving the meat (his favorite, and Ben’s too) for last, he almost burst out laughing. Not from the strange similarity of father and son, but because of the intense pride he felt Bryan’s instinctual connection to him.

But things were different now – Ben wasn’t entirely healthy. He wasn’t athletic anymore and sometimes forgot to go to Bryan’s baseball games. Deep down, Ben knew that he had HD – and every day that he woke up and looked himself in the mirror, he grew closer to coming to terms with the idea and making the decision to get tested. But when he looked in the young mirror of his son, the pride he had felt about their nearly identical appearances and personality transformed into a sharp fear and sadness. How could Bryan nothave HD? As far as he was concerned, he had already decided that Bryan had the HD allele, and Ben couldn’t help but feel responsible for it. He couldn’t think about anything else.

A week had past since Bryan and Todd played when his family came for dinner. Bryan might be young, but he was a perceptive kid. Adults often make the mistake that their children don’t know what’s going on, or don’t wonder why their parents start acting differently – well, it’s simply not true. But kids don’t often know whether or not they should let on that they “feel something’s up”. Every night that whole week, Ben came in at night to tuck him into bed. At first, it felt like a novelty, so Bryan responded with a joking indigence. After three days, he waited expectantly for his Dad to come in to see if he would. Ben did. After a whole week, Bryan finally spoke up.

“Hey Dad, what’s up? You know I don’t need to be tucked into bed anymore.”

Ben was quiet for a while. He never assumed that Bryan caught on to the increased attention and thought anything of it, but he struggled to find the right response.

“Dad, I think it’s cool that you came to my last game and come to all my practices, I really do. I also like that you help me with homework and take me out for pizza and ice cream whenever I want it, but I don’t really need you to all the time. It’s okay if all that stuff is just a special thing…you know?”

Bryan loved his dad – he was a great role model. They shared much in common and Ben had given him great support in everything; encouraged him at school, taught him the basics of baseball, and on weekends, would do something special. Sometimes they went to the water park or the baseball stadium to watch his favorite team, he even bought him a new bike one day last year, and it wasn’t even his birthday! But this past week, Ben’s attention and affection became smothering…and a bit unusual too.

Two nights ago, Bryan’s dad helped him with his math homework. Well, he didn’t really help, he pretty much did the whole assignment for him.

“Daaaaaad! Why won’t you let me do this problem?”

“Well, son – you’ve been having some trouble with this subject lately, and I just figured you needed a little help.”

“Dad, I know I make mistakes, but if I don’t make them and learn from them, then I’ll do really bad on the test!”

“Well, let me just finish this one, and I’ll check your answer after you’re done.”

Helping with homework was one thing, but doing the assignment for him was another. Bryan’s dad kept telling him that he wanted Bryan to get into the advanced math class – that taking advanced classes would “give him more opportunities later in life.” Bryan was confused by this; he wasn’t even in junior high yet! School was supposed to be fun, and the stressful “homework helping sessions” were not fun at all. The “help” was stressful too because Ben was quick to temper sometimes – insisting that “Bryan, just try again,try again!” Other times, Ben would seem distant, as if helping with homework wasn’t really such a big deal at all.

This strange reversal of attitude was something Bryan had noticed some time ago – and it scared him a little to see his dad acting so differently. It was as if he had two dads now, one that was overbearing and constantly concerned and other times, tired and ambivalent. Bryan wouldn’t say anything when his dad’s moods changed, and tried his best to adapt to each ‘personality,’ but sometimes he wanted to ask him if everything was okay.

Bryan’s dad had come to all his practices and a couple of baseball games. Sometimes, he would encourage him to “give it his all, and maybe,” he said with a wink, “you’ll get a college scholarship.” Other times, when Bryan was almost hit by a ball, or slid hard onto first base, his dad would jump up and scream if he was alright. The love of the game seemed to be gone for Bryan’s dad; he either worried about Bryan’s distant future or nearly had a heart attack every time Bryan was at risk of hurting himself. Regardless, Bryan felt overwhelmed with his dad’s attention, and decided it was time to find out why.

“Dad?”

Bryan’s dad still hunched over him as he rested his head on the pillow. His dad seemed hurt, but mostly conflicted as to what to say to his son.

“Dad, you don’t need to worry about me so much, or get sad about me. Everything’s going to be fine. Can we just try to have fun now?”

“Yes son, but I just worry about you sometimes, I just want to make sure you grow up big and strong with opportunities I didn’t have, like any parent would want for his child.”

Bryan didn’t really know what to say. He let out a sigh, “Okay dad, okay. I love you, good night.”

Ben closed the door quietly to his son’s bedroom door, and stood on the outside holding the doorknob for a few seconds. He wasn’t sure how he was feeling. As he walked to his bedroom, he saw Lila reading a book in her armchair by a lamp. As he walked past, Lila looked up.

“Honey, is everything alright? You seem troubled. Are you worried about your appointment next week?”

“No,” Ben replied quietly, “it’s nothing.”

Lila was quiet for a moment, “You know you can talk to me about anything, I’m in this with you.”

Ben turned to his loving wife, hands in his pockets, and eyes searching the floor.

“Darling, do you worry about…Bryan at all?”

“Well honey, I do want the best for him, but I don’t think that’s what you mean.”

“Well, I want the best for him too, it’s just…”

Lila looked deeply into her husbands face, and waited for him to continue. Ben changed his tone quickly:

“Bryan thinks I’m giving him too much attention, and I just want him to be healthy and have all the opportunities in the world.”

Lila put down her book, ear-marking her spot and motioned for Ben to sit next to her.

“Honey, Bryan’s fine. He’s healthy, doing well in school, and is a fantastic athlete already.”

“I know, but I’m not sure it will last?”

Lila’s eyes showed a spark of understanding, and lowered her tone as if the walls might repeat what she said later if they heard.

“Are you worried if – if Bryan will get HD?”

Ben was dead silent, as if what Lila said was too loud and frightening to respond to. Lila waited for Ben to say anything, but his silence gave her the answer.

“If it makes you feel any better, I worry about that too. But it’s just too much for us to deal with right now, you getting tested next week and everything. I think we should just take this all in one step at a time. And besides, Bryan may not have HD at all.”

Ben’s face looked up quickly, “But darling! Bryan looks exactly like me, he likes all the same things…we even eat food the same way!”

“Shh shh shh,” Lila tried to calm her husband down, “Yes darling, but HD doesn’t work that way. Children do look like their parents, and sons typically look up to their fathers and do the things they see them do. It’s natural.”

“I know that, but I feel like if Bryan got HD, it would be all my fault…I just can’t bear the thought of him dealing with everything I’m going through. I want him to live life to the fullest, to make sure that he succeeds, in spite of HD. But I worry that he won’t have a full life to live to the fullest – does that mean he should go to college? Get married? Even try to advance a career – if it all will start crumbling down?”

Lila saw how much these thoughts tormented her husband. She also saw how contradictory his feelings were. How could Ben shower Bryan with attention and even do his homework for him if he thought that college and a career might not be necessary for Bryan’s future?

Ben sounded like he was on a roll, all his fears and fatalist musings kept pouring out, and Lila listened patiently to him.

“Lila – I know Bryan will have HD – he has to. I think he should get tested with me…so we know how to deal with the future.”

Lila almost felt like crying, seeing how much her husband blamed himself for his HD – she couldn’t bear it. No, her husband wasn’t crumbling…and even if he was, she wouldn’t let him feel that way. She couldn’t. Lila pulled herself together and knew how important it was to support her husband right now, and forever.

“Honey, we can never know what the future holds. We need to allow Bryan his childhood – he’s a good kid. He’s smart and will go far in this world if we guide him. He will have his desires and goals, and I know we wouldn’t want to deny him the experience of living for the future. Yes. Bryan may have a 50% chance of eventually getting HD, but you can’t blame yourself for that, and you can’t know that our son will ever get HD. You can’t blame your mother and I know you don’t. But honey,” Lila’s eyes started glistening over and she grabbed her husband’s hand tightly, “You grew up to be an amazing man, husband, and father. If Bryan grew up to be the same man you are we would have done a great job raising him.”

Ben bit his lip and small tears began to leak from his eyes, “But…I just feel that we need to know…so we can do our jobs as parents better.”

“Honey, I think we’re doing our jobs just fine already. I know it’ll be hard for both of us – just wondering if Bryan will have to go through the same things. But when he gets old enough, he’ll decide for himself when he’s ready to get tested. You had the freedom to find out when you were ready, and Bryan should have the same. Now we just have to do our part. We can’t treat him differently – we can’t make decisions for him based on something we can’t know for sure – we need to give him space, room to breath, to get in trouble, get a scraped knee now and then, and make mistakes!”

Ben knew Lila was right and knew that he’d have to treat his son with the same openness and possibility before he started getting symptoms – before he had ever even heard of HD. Ben still felt the bruising ache of worry, and didn’t know if he’d ever be able to forget about Bryan’s risk – but Lila’s shared worry and commitment to allow Bryan his choice and childhood ignorance was strengthening for him.

As Ben and Lila tucked themselves into bed that night, Ben made a promise to himself that he wouldn’t tuck his son into bed anymore…well maybe every now and again…just as something special.

For further reading on the topics discussed:

  1. Behavioral Symptoms of HD: This link to the HOPES website includes information on the behavioral changes associated with HD. This includes the causes of behavior change, what behavioral changes commonly occur, and potential treatment to manage symptoms.
  2. HD and Family: For greater understanding of family interpersonal dynamics and HD, click here.
  3. Physical Symptoms of HD: This links to the “HD Basic” page on the website. It provides useful knowledge on the physical symptoms associated with HD, primarily chorea.
  4. Cognitive Symptoms of HD: This links to the “Cognitive Symptoms” page of the website, which provides information on what cognitive abilities could be compromised by HD, what causes these symptoms, individual variability with respect to symptoms, and treatment options available.
  5. Family History: This links to information on the inheritance of HD on the HOPES site. This provides the genetic background of inheritance and approaches practical family questions concerning the potential inheritance of HD. It may be insightful to visit the “Genetic Testing” part of the site as well.
  6. Genetic Testing: This link explores a variety of topics on genetic testing, such as: testing guidelines, what takes place during genetic testing, what the test actually does/means, accuracy of the test, confidentiality, and the benefits/consequences of a genetic test for HD.
  7. Enzyme CoQ10: This link will take you to a report on the site on the exciting research and discovery of this potential HD treatment option.

The short stories of this section of HOPES are fictional accounts inspired by the experiences of families living with HD. They are not meant to be instructive or prescriptive, but to serve as artistic impressions of the social and familial impact of the disease. We have found them a useful way to discuss HD and its implications with others. As always, we invite your comments on these stories.

M. Woloszyn, 8/18/07

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HDSA Honors

HDSA Honors

On June 8th, 2008, the Huntington’s Disease Society of American (HDSA) honored HOPES with the first annual “Giving a Voice to HD” award. According to the HDSA, the award recognizes “an individual or group whose efforts have significantly helped to raise awareness about Huntington’s disease in their local community and beyond.”

The HDSA “Giving a Voice to HD” plaque

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