HIPE Focuses on the Kids

HIPE Focuses on the Kids

by Sharon McClellan Thomason

Kids were a big focus for part of the questions asked of the HIPE panel in Sacramento, at our Highly Interactive Participant Education (HIPE) Day. Our panel of experts, Lisa Mooney (social worker at UC Davis), Dr. Sasha Duffy (neurologist at UC Davis), and Dr. Vicki Wheelock (neurologist and director of the Center of Excellence at UC Davis) fielded questions that included telling kids about Huntington’s disease and setting boundaries for kids with Juvenile Huntington’s disease.

The following is a transcript of the questions and answers. Questions are always de-identified for the privacy of HD/JHD families.

Question 1: What suggestions do you have for telling children? What age, and how?

Response 1: (Lisa Mooney) In general, kids know a lot more than we give them credit for. They are probably aware that something is going on, and they are probably aware that something strange is happening to Mom, Dad, Grandpa, Grandma, whoever. It is important to name it for them, and not just say, “Oh, Mom is sick, or Dad is sick.” Remember, we all get sick, right? We all get colds; we get sick. That can be very confusing to kids, so it is important to have a label for it. Whether you want to call it Huntington’s disease or just call it HD, or call it something else, that’s fine. But you need to label it for kids to make it less confusing for them. Of course, how you tell your kids will be totally dependent on what age they are and what developmental level they are…you can label it and leave it at that. Tell them if they ever have any questions or concerns, they can come to you. Of course, if they’re older, teenager years, and they’re able to understand a little bit more, you give them a little bit more information. Give them safe places to go to get information outside of you, like Huntington’s disease youth organization (HDYO), or they can call [the social worker], or they can come to you to ask you questions. It is important to tell kids. Usually, it doesn’t end well if it is a family secret because eventually it does come out, and there is usually a lot of anger, resentment, guilt, and things of that nature if it’s not talked about. It’s not something you have to talk about every single meal. It doesn’t have to consume every single day. But it is something you need to let kids know about so that they know what is going on, and they aren’t scared because a lot of times, if they think something is going on, or if they see Mom crying or see Dad crying, they’re worried, and they need an outlet to sort of talk to a person about it.

*HDSA has a resource packet with “How to talk to kids about HD,” available on their website.

Response 2: (Dr. Sasha Duffy) A couple of situations that I can think of are at time of diagnosis, so when some people come to us for predictive testing, and they find out they have the gene, and they will get Huntington’s disease at some point in their life, and where they come to us and get a new diagnosis of Huntington’s disease, and there is a question of, “What do I tell my kids?” I will often say, “Just take a moment and let it penetrate you, ok? Just let it sink in for you; let it be a little bit clearer for yourself before you are ready to pass that information on. It’s ok to just take that moment before letting your children know.”

Question 2: With a child with JHD, what limits are acceptable? So if they don’t get their way and they throw a tantrum, what should you do? What are the limitations with a child with JHD? She wants to take her clothes off inappropriately, or she wants to hug and kiss strangers, things of that sort. What limitations should we put on a child with JHD?

Response 1: (Lisa Mooney) Kids are still kids, sick or not. With Huntington’s or not, it is important to give them responsibilities that are appropriate for their developmental level. It is important to make sure that they follow the rules that you set forth. It is ok to give them time-outs or to have consequences for what has been talked about as not appropriate behavior or something of that nature. That being said, kids are still going to push their boundaries, and still try and do everything. Consistent routine is key, really for all of us, definitely for a person with Huntington’s. Adult or juvenile, routine is key. Some of the kissing and hugging strangers and some of that is probably more HD-related and a little bit more challenging to sort of manage. I would say, just try and have conversations that are developmentally appropriate for that child, and see if you can’t talk about it, or maybe try and avoid situations where they may be overstimulated, if you will. There could be other triggers; there could be things going on that could be triggering this behavior. Maybe it’s grocery stores, maybe they’re scared, maybe that’s their defense mechanism for that particular situation because they’re anxious. It’s hard; it’s not easy, as we know parenting is not easy in general, and parenting is even harder if you are parenting a child with Juvenile Huntington’s disease. You guys are all amazing. You are doing your best, and your best is all that anyone can ask for.

Response 2: (Dr. Vicki Wheelock) The other side of that, though, I think is that you want to always make sure the person with Huntington’s is safe, and I think you are touching on something where sometimes there is a safety issue, and it’s about understanding who is a stranger and who is a friend that you know, that type of thing. I want to say that this goes to teens and young adults where sometimes, because of some of the brain changes, they aren’t judging danger adequately, and that’s one of the hardest things—you want your child to mature, be as independent and to follow their own dreams as much as you can, but you also have to watch out for them; you want to try and help them understand. So I think it has to be developmentally appropriate; something for a six year old is different than a 12 year old and an 18 year old, but if you can do that consistently and get into a plan with them, it will be easier for them to accept guidance. The hardest thing about this is that the disease is progressing, and that’s why we have to adjust how we’re maintaining their safety. For every person, it’s always going to be different, but I do think that creating a culture of safety and safe boundaries and talking to them about that, not to scare them, just to help them understand that sometimes things can happen that you wouldn’t expect and that there is a reason I don’t want you to do that [is important], and then try to redirect.

The Huntington’s Post is made possible by grants from Teva Pharmaceuticals and The Griffin Foundation.

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Understanding Irrational Behavior in Huntington’s Disease

Understanding Irrational Behavior in Huntington’s Disease

by Debra E. Andrew   

It happens out of the blue. There you are, going along with what appears to be a somewhat normal moment in time, when suddenly the irrational behavior rears its ugly head! It isn’t that you have never seen this before. It’s that even though you have seen it, you are still caught off guard. One second, your loved who has Huntington’s disease seems rational, and then suddenly, he or she is anything but. How does one cope with irrational behavior?

First, let’s talk about the why. Knowing the why won’t change the behaviors you see, but it may help you to wrap your head around what is going on. The brain of the person with HD is being attacked. We could go into all kinds of medical and scientific descriptions about that, but rather than do that, let’s understand it in simple terms. The brain is being attacked, injured, damaged, and brain cells are being murdered. When that happens in the frontal lobe of the brain, it impacts behaviors.

Some factors that trigger irrational behaviors are:

Impulse control

Frontal lobe damage erodes impulse control. At times, we may all have some irrational thoughts, but our impulse control allows us to get rational again and to get control of any irrational thoughts. That is an impossibility for people with Huntington’s disease who are experiencing irrational thoughts or irrational understanding. The brain has taken off on its own direction of thought, and what the brain believes to be true is true to that person. To someone with HD, every one of those thoughts is real and true. And they behave accordingly.


People with Huntington’s disease can feel very anxious when their brains aren’t working for them like they realize they should be. Their ability to cope is undermined, they feel a loss of control, and their anxiety begins to rise. This isn’t the basic anxiety that a person without Huntington’s disease faces. This is an intense, all-consuming anxiety that begins to overwhelm them completely.

Being overwhelmed

With so many emotions swirling around all at once, it is confusing and extremely overwhelming. There is no way for people with Huntington’s disease to sort through all of those extreme emotions. It becomes so overpowering that it removes rational thought from them. Just coping with those emotions is more than they can do; forget adding rational thought to that. Even if the processing of the brain allowed rational thought at that point, this extreme mixture of emotions would hijack it.

Confusion of thoughts and emotions

There’s a part of the person with HD that will fight to find what is true, what is going on, and even what is rational. Much like being in a room full of mirrors with hundreds of reflections, people with HD are seeing all of these thoughts and emotions, and they’re trying to figure out which is real. They may doubt if any of them are real, yet then believe all of them are real. Imagine how overwhelming that would be. The only survival available is to choose, to decide what is real and hang on to it. Unfortunately, that often can be the irrational thoughts that take over.


Things just aren’t adding up. Things aren’t working like they should work. And there is no way to understand why, or to sift through things and get them lined up again like they should be. That is where the frustration begins. A loss of control. A loss of understanding that is frightening and overwhelming. It often comes out as frustration because acknowledging the fear that they truly are “losing their mind” is too much to process or accept.

Unmet Needs

Being hungry, thirsty, or having pain or other unmet needs isn’t something that people with HD can always express or process. Their bodies may hurt, but their minds may not tell them what they are feeling is pain. They may be hungry but can’t express their hunger. Gnawing at them is some feeling they can’t communicate, process, or meet for themselves. And yet, the feeling is relentless. They are at a loss of what to do. Remember, HD erodes the ability to know how to choose or how to do an act. The desire to act is there, yet all that comes out is to be frozen, unable to act on what they want to act on or unable to choose how to do it.

Perception, Unawareness, Lack of Emotional Recognition

Adding to this terrifying scenario is the inability to perceive exactly the responses around them. People with HD may be unaware of others’ responses, emotions, and much more. Although the facial cues that we normally would see and understand are there, they can’t pick up on those cues. They are left without understanding of any response, or they become extremely confused at the responses being received. When responses are negative or unexpected (and they are all unexpected), it’s like being hit in the head with a two by four. They are caught off guard, and now, added to all the above extreme confusion they are going through, they are baffled and often irrational. Reality is fractured.


Although it is natural to attempt to rationalize with a person who is behaving irrationally, all these factors make a rational discussion or reasoning with an irrational person with HD futile. This may sound hopeless, but it isn’t. Thankfully, there are ways to manage and to cope with irrational behaviors. Those will be discussed in a follow-up article.

About the Author

Debra E. Andrew lives in Utah, is happily married, has seven children, 23 grandchildren, and one soon-to-be great grandchild. Her love of health and wellness has led her to empower others in all eight areas of health and wellness in her daily life and businesses.

Debra is the creator of Business Hands, a non-profit serving those who are disabled and their caregivers; the founder of Power HC, PWR HC – Preventative Wellness Resource Health Community; a Huntington’s Disease Regional Advocate; and she has established several Facebook groups supporting those with Huntington’s disease, their caregivers, and families. She also has a blog, http://hdinsider.weebly.com, where you can find more of her articles.

Debra’s educational background includes Business Management, Marketing, and e-Commerce, and she holds a B.S. in Community Health and a minor in Community Health Education. She is also a Certified Brain Health Coach.

Debra’s husband, Allen, has Huntington’s disease, and Debra is his full-time caregiver. The Huntington’s disease incidence rate in Allen’s family is 80 percent.


The Huntington’s Post is made possible by grants from Teva Pharmaceutical and The Griffin Foundation.

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