Huntington’s Disease: Filing for Disability
By Sharon McClellan Thomason
Filing for disability and navigating the system of SSDI, SSI, Medicare, and Medicaid can be both challenging and frustrating. It’s a world we usually know little about until we plunge headlong into the journey of Huntington’s and Juvenile Huntington’s disease.
I consider myself to be a well-educated and reasonably intelligent woman, but I can tell you that helping my son file for disability and associated benefits stretched my knowledge and problem-solving skills to the limit.
Here are some things I learned along the way.
First, some simple definitions you will need to keep in mind—make these a part of your new vocabulary:
- SSDI stands for Social Security Disability Income. This is administered by the federal government, once it’s determined that you have a disability.
- SSI stands for Supplemental Security Income. This is administered by the Social Security Administration and is income-based, providing cash assistance and healthcare benefits (Medicaid—see below) to people with limited income and resources.
- Medicare is a federal healthcare program. Once you’ve been on disability for two years, you are automatically eligible for Medicare. People who have worked and paid into the system are also eligible for disability upon turning 65. Medicare has Parts A, B, C, and D. Part A is hospital/hospice insurance; Part B is medical insurance that covers doctors’ visits; Part C is an optional Medicare Advantage plan; Part D is a prescription drug plan.
- Medicaid is a state-run program for people with limited income and resources. It also covers things not normally covered by Medicare, like nursing home care and personal care services. It is funded jointly by the state and federal government, but eligibility varies greatly from one state to another. States that have granted Medicaid expansion under the Affordable Care Act are more likely to grant eligibility for Medicaid. The map here (https://commons.wikimedia.org/wiki/File:ACA_Medicaid_expansion_by_state.svg#/media/File:ACA_Medicaid_expansion_by_state.svg) shows which states have Medicaid expansion. Medicaid also includes optional dental services for people over age 21.
As a basic review, SSDI and Medicare are federal programs that are not income based; SSI is a federal program that is income based; Medicaid is a state-run program that is income based.
Compassionate Allowance List and Appealing a Denial
When applying for SSDI, it is helpful to know that Huntington’s and Juvenile Huntington’s disease are included under something called the “Compassionate Allowances List,” or CAL. (https://www.ssa.gov/compassionateallowances/). According to the Social Security Administration, “The CAL initiative helps us reduce waiting time to reach a disability determination for individuals with the most serious disabilities. The Compassionate Allowances program identifies claims where the applicant’s disease or condition clearly meets Social Security’s statutory standard for disability.”
So this should make the awarding of disability benefits automatic, right? Not necessarily. The trick to claiming this allowance is knowing where it is listed and alerting the claim worker that HD is on the CAL. Interestingly, HD can be found in two places: under ‘A,’ as Adult Onset Huntington Disease, and under ‘M,’ as Mixed Dementias, where Huntington’s dementia is listed under alternate names. It is NOT listed under ‘H.’ Juvenile Huntington’s disease isn’t so hard to find—it’s under ‘J,’ as Juvenile Onset Huntington Disease. The complete list, with links to the above named, can be found here: https://www.ssa.gov/compassionateallowances/conditions.htm. The listing for each includes the necessary documentation to include with your application.
When I helped my son apply for SSDI, HD was not listed on the CAL. I used the guide developed by Phil Hardt, available through the Huntington’s Disease Lighthouse Foundation (http://hdlf.org/node/210), which many families have used successfully. After a few years of severe psychiatric symptoms, a positive genetic test with a CAG of 45, and a diagnosis by a neurologist, I started the application in the summer, after school was out (I was still teaching full time). Despite following the guide precisely, the application was denied. We then had the option of appealing. By then, I was back at work and was afraid I didn’t have the time to do the appeal properly, so I hired an attorney who specializes in Social Security law. This seemed like the ideal solution since they, by law, do not require any payment up front, and receive a set payment only upon a successful appeal. I should’ve done my homework, though, as I learned that all Social Security law firms are definitely not created equal! The one I hired had me do all the work (gathering and mailing paperwork, filling out forms), and I only met the attorney once—to sign the contract. After that, I dealt with a paralegal who sometimes returned my phone calls, most often, not. I even found out that the appeal had been approved before they knew it!
My hope is that with the Compassionate Allowances, and with organizations that help you with applying for disability, you will not experience the difficulties we experienced. At one time, the Caring Voice Coalition helped people apply for disability; however a statement on their website (http://www.caringvoice.org/disability-assistance/) says, “CVC’s disability program supports patients through the various stages of the Supplemental Security Income and Social Security Disability Insurance application process. Our patient advocates coordinate supporting evidence, assess eligibility, submit claims for benefits and draft arguments. The disability program is temporarily at capacity. We hope to begin accepting new applicants for this service soon.” Instead, they are referring people to the Huntington’s Disease Assistance Foundation and the National Organization for Rare Disorders (NORD).
Another thing I learned that evidently is not generally known is that my son, who had very limited work experience before the onset of HD, qualified for additional benefits as the “disabled adult child of a disabled adult.” The window is very limited, as defined below:
An adult disabled before age 22 may be eligible for child’s benefits if a parent is deceased or starts receiving retirement or disability benefits. We consider this a “child’s” benefit because it is paid on a parent’s Social Security earnings record.
The “adult child”—including an adopted child, or, in some cases, a stepchild, grandchild, or step grandchild—must be unmarried, age 18 or older, and have a disability that started before age 22. (https://www.ssa.gov/planners/disability/qualify.html#anchor8)
Since my son’s father had passed away from Huntington’s disease, and he had a strong work record, Randy was able to qualify for about $250 extra in SSDI benefits, more than he would have received under his own work record.
Social Security also provides something called “Extra Help” with the Medicare prescription drug plan. To see if you qualify and to apply, go here: https://secure.ssa.gov/i1020/start. Note: if you already receive Medicare and Medicaid or Medicare and SSI, you do not need to apply as the extra help is automatic.
As daunting as it may seem, don’t give up on applying for these benefits! If you are a person with HD, get an advocate to help you with applying. The benefits are ones to which you have a right, and they can make a huge difference in your quality of life!
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Hypersexuality and Huntington’s Disease
Hypersexuality and Huntington’s Disease
By Sharon McClellan Thomason
Of the troubling behavioral changes that occur with the onset of Huntington’s disease (HD) and Juvenile Huntington’s disease (JHD), and there are many, one of the most troublesome can be hypersexuality. It’s important to realize that this is a part of the disease, that there is no shame or embarrassment in talking about it, and that there are treatments that can help resolve the problem.
According to an article in Huntington’s Disease News, “Studies have found that up to 75 percent of women and 85 percent of men with Huntington’s report difficulties in their sexual relations.” (https://huntingtonsdiseasenews.com/sexual-problems-huntingtons-disease/)
While a loss of interest in sexual relations is openly discussed in the literature, hypersexuality and inappropriate sexual behavior are not frequently talked about. In fact, when they are discussed, they are downplayed as something that doesn’t occur very often. The same article in Huntington’s Disease News says,
Less frequently, Huntington’s disease may also cause an increased sex drive and inappropriate sexual behavior. Such disinhibition is thought to be due to damage to the caudate nucleus, a deep area of the brain that controls behavior.
Some patients may be more likely to engage in risky sexual behavior, such as one-night stands or sex under the influence of alcohol or illicit substances. It is important to always ensure that safe sexual practices are upheld, including using condoms and taking oral contraceptive pills to avoid sexually transmitted diseases and unexpected pregnancies.
Families will tell you, though, that an increased sex drive and inappropriate sexual behavior are NOT rare; it’s just rarely discussed openly, perhaps because of embarrassment.
In fact, the Journal of Neuropsychiatry and Clinical Neurosciences reports, “Psychiatric syndromes (present in up to 79% of patients) most commonly include impulse control disorders, depression, personality changes, and, more rarely, psychosis or mania. Symptoms include disinhibition, irritability, aggression, apathy, and neurovegetative markers of depression. The suicide rate has been reported to be up to 20 times that of the general population over age 50.1 Increased criminal behavior and hypersexuality have often been reported in HD patients.” (https://neuro.psychiatryonline.org/doi/10.1176/jnp.11.2.173)
Recently, one woman posted in a Facebook caregivers’ group, “I’ve (yet again) been approached by a Facebook friend, and my brother has been harassing women. Young, old, married, or single. He begs for dates and tells them all the same lines about how attractive they are. So far, all I know it that he compliments them ‘sexy or beautiful’ and how he dreams about them. I would be uncomfortable if a man sent these messages to me. He has been doing his for years, and it is getting more frequent. Can I report him to Facebook? He won’t listen to me. I’ve begged, yelled, and threatened, to no avail. Maybe therapy? He’s so lonely. But he’s losing the few contacts that he still has!”
Several people responded with empathy. One said, “Dealing with the same issue with my son. Like many others dealing with this disease, the impulsive behavior is out of control. I have been dealing with this for years. Sometimes it is worse than others. We deal with it as we go. I will send messages and explain the circumstances, and most people understand. As the disease progresses, there will be different things you have to deal with. This is just a stage. Look at it as unfortunately at some point they will not be able to do it at all … deal as it comes is my outlook. It’s temporary, unfortunately.”
Another commented, “I reported my ex (we have two daughters). It’s sad, but I did not want them to see it.”
One woman wrote about the potential consequences. “My son has definitely changed. His mind has taken him to all kinds of stuff … porn from women, men, transvestites, gay, anything he can watch …. very scary to think what’s going on in his mind. However, I have to remember he’s 24, trapped in a body that is not functioning properly. He is not living a normal life, and that is his outlet, I would assume–so it’s definitely a fine line. He’s usually good about keeping within his bedroom and closing the door, so I don’t bother him too much unless he starts texting and sending inappropriate pictures to people. I explain to him that some people will not understand or do not care; it’s inappropriate, and you will go to jail, and at that point, I will not be able to help you … with Huntington’s and especially Juvenile Huntington’s it doesn’t matter how many times you say it; it’s in and out of the brain. It’s a constant battle [because] they cannot manage thoughts like they used to.”
Another woman shared, “My husband is starting to be the same way. He sends messages to my friends all the time, even if he hasn’t met them. He has no concept of it being awkward and weird. He even got blocked too many times on a dating app.”
I went through similar problems with my ex, back before we had Internet and dating sites. He once picked up a strange woman and brought her to our home, even gave her some of my clothes, and sent our young four-year-old son off in her care, not knowing her last name or how to contact her. Another time, he went to my son’s first grade Christmas party and gave the teacher a very sexy piece of lingerie, which she opened in front of students and parents, something that was incredibly embarrassing for her and for the parents and the students who realized something inappropriate was happening.
The problem with hypersexuality and inappropriate sexual behavior does not occur only with males affected by HD. One man shared, “I have been having a similar issue with my wife. She claims that she has no sex drive due to all the medications that she is on for her mood, and her doctors verify her claims of reduced libido. Yet she keeps reaching out to her ex-boyfriend from almost 20 years ago, trying to hook up with him. She claims it was a one-time thing and apologized, yet I have caught her calling him or messaging him many times now. I even checked the GPS history on her phone and found out that she left the house one night after I fell asleep. Sure enough, when I checked the call history on that day, her ex called her, and within minutes, she left the house and went to a local park for about an hour. When confronted with all this, she confessed to meeting him, yet she SWEARS that they just met up to ‘talk,’ yet according to the phone records they talk on the phone all the time (even though she keeps telling me that she doesn’t really talk to him anymore). I just don’t understand how if she supposedly has no sex drive, then why does she keep trying to hook up with this guy?? I finally called his wife and told her what was going on. Now I have seen messages between her and another guy (her friend’s 27-year-old son) that were VERY sexually explicit. This caused a huge argument and nearly ended our marriage.”
The problem, when it’s a woman sending sexually explicit messages and invitations, seems to be quite different than when it’s a man doing the same thing. The same poster added, “If a man with HD sends a woman a sexually explicit message, or flat out asks for sex, 99% of women would be very offended and even get angry. But if a woman offers a man sex or a ‘sexual favor,’ a LOT of men will actually entertain it to see if she is serious and possibly even take her up on it. I have heard of MANY female HD patients being taken advantage of by men because they are hypersexual and being VERY flirty with men.”
In another caregivers’ group, someone shared how his wife enjoyed masturbation with a vibrator so much that when the batteries ran out, she went to the neighbors’ house, naked, and threatened to set their house on fire if they didn’t give her new batteries for the vibrator.
Another woman shared, “My Phd sister-in-law (now passed) was very sexually promiscuous, to the extreme of bringing home men from the bar with her husband and son right there in the house and also taking off with men she met for days at a time.”
The fear that a loved one will go to jail because of inappropriate sexual behavior is real. The tragic story of Joseph Heverin in Delaware serves as a reminder of just how dangerous it can be. Joseph was convicted twice of unlawful sexual contact, something that his family believes stemmed from the effects of Juvenile Huntington’s disease—hypersexuality and poor impulse control. His mother, Dianne says she had reached out to mobile crisis and mental health before the police became involved. Because he had just turned 18, Joseph was considered an adult, even though he was disabled, and he spent about nine months in jail, waiting for sentencing. At 21, he was admitted to Dover Health Behavioral Systems for depression. Dover Behavioral, a short-stay psychiatric facility, tried for a year to move Joseph but was unable to find a place that would accept him because he’d been labeled a sex offender. At the age of 22, Joseph choked to death on a grilled cheese sandwich while eating without supervision at Dover Behavioral. Although his mother fought hard for justice, no one was ever found culpable for his death.
So what can we do about hypersexuality and inappropriate sexual behavior with our loved ones? First, we must recognize that it’s not their fault; this is the HD/JHD brain acting. While it involves problems with executive functioning (lack of judgement, poor impulse control), Dr. LaVonne Goodman considers it a part of OCB (Obsessive Compulsive Behaviors). Goodman, an internal medicine doctor in Washington state who lost her first husband to HD and is the founder of Huntington’s Disease Drug Works, notes that, “These behaviors rarely occur in isolation and are associated with anxiety, depression, agitation and can occasional [sic] be associated with delusional thoughts.” (http://hddrugworks.org/treatments/obsessive-compulsive-behavior)
Her approach to treatment includes both behavioral and pharmacologic approaches. Behavioral strategies include both a “do” and “don’t” list:
“Do” behavioral strategies include modifying the environment by simplifying life:
- Regular schedules/routines. If change is necessary give advance warning
- Allow more time for accomplishing activities
- Identify triggers of overstimulation like noise, too rapid requests/demands
- Non judgemental response
- Give time to cool down
Don’t behavioral strategies:
- Don’t try to rationalize or convince
- Don’t respond with anger
First line treatments are SSRI antidepressant drugs, several of which have been FDA approved for obsessive compulsive symptoms. These include fluoxetine (Prozac®), paroxetine (Paxil®), sertraline (Zoloft®), and fluvoxamine (Luvox®). The NSRI antidepressant drug venlafaxine (Effexor®) has been approved for severe anxiety and is also used for obsessive compulsive symptoms. Duloxetine (Cymbalta®) is a good alterntative NSRI. Often higher doses of these drugs are needed than those used for depression. Clomipramine (Anafranil®) that has combined SSRI and tricyclic that can be used in more severe presentation, but is not used first due to greater side effects than SSRI or NSRI drugs. Mirtazepine (Remeron®) is an atypical antidepressant that is FDA approved for obsessive compulsive disorders.
Antipsychotic are not the drugs of choice for obsessive compulsive symptoms, but are often added if this symptom causes agitation.
Another article in Huntington’s Disease News concurs:
Disinhibition can be one of the most troublesome symptoms in Huntington’s disease, particularly for the family and caregivers. Milder symptoms can include speaking out of turn, embarrassing remarks, and childish behavior. More socially compromising behaviors, such as inappropriate sexual remarks, hypersexual behavior or exhibitionism can be particularly troubling.
Studies also show that disinhibition is closely linked to delusions, irritability, and agitation, suggesting that the symptoms may be on a spectrum of more classical psychotic symptoms, also commonly seen in Huntington’s.
The problems may also be more severe in younger Huntingtin gene carriers. The reviewers noted that disinhibition can be treated both with behavioral interventions and with selective serotonin reuptake inhibitors (SSRIs). Drugs used for behavioral problems in frontotemporal dementia might also be valuable. (https://huntingtonsdiseasenews.com/2016/09/29/psychiatric-and-behavioral-symptoms-in-huntingtons-ignored-in-research-review-finds/)
Others report having their loved ones put on a medication that lowers the sex drive. As always, consult with your loved one’s doctor before making any medication changes.
Most importantly, recognize that it’s okay to talk about this problem, that it’s a symptom that, if left unchecked, can lead to legal problems, and that it IS treatable! Keep talking because this subject is no longer taboo!/* Custom Archives Functions Go Below this line */ /* Custom Archives Functions Go Above this line */
Navigating Juvenile Huntington’s Disease
Navigating Juvenile Huntington’s Disease
By Denise Hudgell
“Sometimes superheroes reside in the hearts of small children fighting big battles.” – Author Unknown
Any parents who have or had a child with a terminal illness know the heartache of watching their child suffer and the heartache that it brings. Navigating through the world of Huntington’s disease (HD) is especially difficult at times, due to the lack of knowledge, awareness, and providers that are familiar with the disease. The Juvenile Huntington’s disease (JHD) world has its own set of challenges, from difficulty getting a diagnosis to even fewer providers that know about JHD to unrecognized symptoms that appear in JHD. The inability to receive a diagnosis of Juvenile Huntington’s disease in a timely manner can make a big difference in the way a child is treated. For example, children with JHD are often misdiagnosed with ADHD and treated with medications that can actually make JHD progress more rapidly. It can also make day-to-day activities difficult for families when a potential diagnosis is looming over their heads. When a child is at risk for HD, and he or she begins to have symptoms that could be HD-related, it can be very difficult for the parent to not wonder if it could be JHD. Every little misstep, fall, change in behavior can trigger worries in the parent.
Being the parent of a child who is at risk for HD is scary, especially when symptoms appear in childhood or adolescence. I’ve been navigating the JHD world for over eight years, beginning when my son Aidan started having symptoms at the age of four. It is both frustrating and heart wrenching. I know the fear of JHD and the anxiety of waiting for two years to receive a diagnosis. Aidan started to show mental health symptoms such as aggression, agitation, and obsessive-compulsive behaviors at the age of four. His physical symptoms were only a few months behind. Aidan began falling more frequently, walking on his tiptoes, and having difficulty with balance. He also started to have staring spells, which were suspicious for petit mal seizures. I spoke with his preschool teacher and asked her to keep monthly handwriting samples so that I would have those to take with me to our neurology appointment that was still nine months away, since handwriting difficulty is one of the symptoms in JHD. Being an RN (registered nurse), I was very involved in our local medical community, so I started to research providers that I could reach out to. The hours I spent researching, making phone calls, and worrying were all-consuming. Once we received a diagnosis, even though it wasn’t the outcome we had hoped for, we were able to move forward with finding a specialist and focusing on how my son’s JHD was going to be treated.
After Aidan’s diagnosis, I became very involved in the JHD community, raising money for research, advocating, spreading awareness, networking with providers across the country, and working for Help 4 HD International, a non-profit that is committed to the HD/JHD community. Working so closely in the community was cathartic, even as we lived through some of the worst days of our lives. The amount of love and support that I received from the HD/JHD community, both families and professionals, local and worldwide, has been wonderful. It’s a club that none of us want to belong to, but I have met some of the most loving and caring people along our journey.
Over the last eight years, I have experienced care for my son through several different providers and specialists. There are a couple that stand out in the care that Aidan received. University of Iowa’s Center of Excellence played a pivotal role in Aidan’s care. Their medical and professional staff are very knowledgeable about JHD. They are compassionate, caring, and understand that a diagnosis of JHD encompasses caring for the family as a whole. The support and care that we received was second to none, and I am grateful for their time and passion for treating children with JHD and their families. Unfortunately, since we live four hours away, having continuous care with them was almost impossible, especially as Aidan became more symptomatic, so we only had the opportunity to see them once a year. Aidan had been getting his regular JHD care from a local HD provider, but we didn’t feel that it was the right fit, so about three years ago, we switched the rest of Aidan’s care to our local Children’s Hospital. While none of the specialists had specific knowledge and experience with JHD, all were more than happy to research and gain knowledge on how to best treat Aidan. They listened to my concerns, and we established a wonderful team for Aidan. I went with my gut, and Aidan received the best care these last three years.
In 2015, I had the pleasure of meeting the regenerative medicine team from Dr. Jan Nolta’s lab at UC Davis in California. I was able to tour the lab where regenerative medicine research is being done for JHD. It was a wonderful experience, meeting with their team. Seeing and hearing their passion, compassion, and overall love for the HD/JHD community was humbling. They are truly warriors in this fight against HD/JHD, and I feel very fortunate to have been able to get to know all of them and also to have had the pleasure of interacting with them throughout the HD/JHD community.
Unfortunately, on March 7, 2018, I became a member of another club no parent wants to be a part of. Aidan lost his battle with JHD at the young age of 12. We thought we had another year or so, but JHD had other plans. The last two months of his life were spent in and out of the hospital. Without the support of my family and the HD/JHD community, I wouldn’t have been able to make it through the worst experience of my life. Two months later, I still have so much love and support from the community, and I know that will continue. I feel very lucky to be a part of this community. I encourage everyone who has a family member with HD or JHD to become involved in some way. There are many ways to become involved: online, working with non-profits, hosting an event, educating and awareness, research. Being involved and getting to know others who are in similar situations can be very helpful and can make you feel less alone in a world that can be very lonely at times.
Although Aidan left this world way too soon, I still will be involved with the HD/JHD community, and I live in hope that a cure will come soon so that no one else has to suffer at the hands of this awful disease. I will continue to fight in Aidan’s name and hope that no parent has to experience what I and so many other parents of children with JHD have experienced.
Aidan and his family are featured in the award-winning documentary The Warriors: Fighting the Incurable Juvenile Huntington’s Disease. With a suggested donation, the documentary is available for virtual download, or you may order a copy of the DVD. Information can be found here: https://help4hd.org/events/the-warriors-fighting-the-incurable-juvenile-huntingtons-disease-is-now-available/
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Help 4 HD Announces Huntington’s Disease Awareness Month Programs
Help 4 HD Announces Huntington’s Disease Awareness Month Programs
By Sharon McClellan Thomason
“We are excited this year about our programs we will be launching in the month of May for HD Awareness Month,” says Katie Jackson, president and CEO of Help 4 HD International Inc. “This year, we spent a lot of time thinking of things we could offer our community during the month of May.”
Beginning today (May 1), we are making available to the HD/JHD community, free of charge, an “a-WEAR-ness” package. The package includes wristbands, buttons, a keychain, a window decal, and brochures. We are also offering a set of six communication boards. You may order all or any portion of the package. Items will be shipped at no cost, but because of the prohibitive cost of international shipping, the package will be available only in the United States.
“We started creating projects like communication boards and window decals to bring awareness about what Huntington’s disease is to first responders and the general public when approaching a car with one of our loved ones in it,” Jackson said. “We do hope that our community finds value in the items that we came up with.”
The window decal alerts first responders that there is an individual with Huntington’s disease on board and lists some of the symptoms that might occur with that individual.
A-WEAR-ness items can be ordered at https://www.research.net/r/Order_Form_May_2018.
Something we are extremely excited about offering for the first time is our long-anticipated award-winning JHD documentary, The Warriors: Fighting the Incurable Juvenile Huntington’s Disease. In exchange for a donation to JHD research, you may receive a DVD or a download of the documentary. Levels of donations are:
- $15—receive a DVD of the documentary
- $10—download a permanent copy of the documentary
- $3.99—download a copy of the documentary to keep for 48 hours
To make a donation and order your copy of the documentary, go to https://help4hd.org/events/the-warriors-fighting-the-incurable-juvenile-huntingtons-disease-is-now-available/.
Another feature of HD Awareness Month is a special radio program every Wednesday in May. Programs air live at 4 p.m. Eastern/1 p.m. Pacific and may also be heard in our archives. To listen, go to www.blogtalkradio.com/help4hd.
You can also go to our website and download a copy of a Proclamation establishing May as Awareness Month. The Proclamation can be customized for a particular city, county, or state. We encourage as many people as possible to consider doing this for your hometown, county, or state! The template can be found here: https://help4hd.org/events/request-a-proclamation-from-your-city-or-county-for-hd-awareness-month/
Finally, a new article will run in The Huntington’s Post each Friday in May. The articles will offer information and insights into rarely talked about symptoms of HD and JHD, including gastrointestinal issues and hypersexuality.
A couple of years ago, Katie Jackson came up with Help 4 HD International’s motto, “Help 4 HD International is in the trenches with the people because we are the people.” Jackson says, “We are your community, living in homes that are impacted by HD every day. We love our community and want to bring as much support and resources as we possibly can. We are your grassroots organization started by families and continue to be run by families. We hold tight to our grassroots values and know that we are fighting to be the last generation to live with Huntington’s disease and Juvenile Huntington’s disease the way our generations and past generations have been forced to do. We hold tight to the hope that one day soon a viable therapy or the cure will be found. Till that day, we continue to fight beside you, proud to be a part of a community as compassionate, resilient, supportive, and strong as our Huntington’s disease community.”/* Custom Archives Functions Go Below this line */ /* Custom Archives Functions Go Above this line */
Huntington’s Disease: All About the Kids
Huntington’s Disease: All About the Kids
HDYO Announces North American HD Youth Camp
by Sharon McClellan Thomason
Talking to kids about HD can be one of the toughest parts of the Huntington’s disease journey. It’s one thing to explain that Mom or Dad is sick; it’s quite another to explain that it’s genetic and that each child has a 50/50 chance of inheriting the disease. Fortunately, there’s a fabulous resource to help—Huntington’s Disease Youth Organization, or HDYO as it’s commonly known.
Right now, HDYO is gearing up for its annual North American HD Youth Camp at Camp Cedar Glen in Julian, California, August 11-15, 2018. Camp applications are open until April 30, 2018, to anyone ages 15-23 from the United States or Canada who is IMPACTED by Huntington’s disease. Thanks to generous donations, the entire cost of travel, food, and camp is covered for up to 50 young people!
Chandler Swope, Director of Youth Services, says, “We hope that young people will leave camp feeling less isolated, more knowledgeable about HD, and feeling more supported.”
The five-day camp is about an hour and a half from San Diego, California, and includes lots of team-building, paddle boarding, ropes courses, canoeing, archery, and much, much more! Experienced professionals from HDYO, HDSA (Huntington’s Disease Society of America), and HSC (Huntington’s Society of Canada) will be on-site at all times to support the campers. Trained volunteers will also be there to help supervise and to share their own experiences with campers.
This is an amazing opportunity for young people! For more information and how to apply, please visit https://en.hdyo.org/eve/events/569.
HDYO has also partnered with Help 4 HD International again this year to offer youth days at our HIPE (Highly Interactive Participant Education) Day in Gainesville, Florida, on June 30, and at our fifth annual symposium on October 13, in Des Moines, Iowa.
Another tool HDYO offers is an interactive web site (https://en.hdyo.org/) that is rich in resources for kids, teens, young adults, parents, and professionals. There is also a section for friends of young people impacted by HD as well as a section on Juvenile Huntington’s disease (JHD).
HDYO is an international non-profit organization that was launched in 2012. The idea originated in the summer of 2010 when Matt Ellison, the eventual founder, presented it at the Young Adults Working Group for EHDN (European Huntington’s Disease Network). HDYO is run mostly by young people impacted by HD. In its six years of existence, HDYO has developed a team of over 170 volunteers in a variety of roles. The volunteers include a board that oversees the organization, a translation team, forum moderators who respond to questions posted in the HDYO online forum, HDYO reps who spread positive HD awareness globally, and a feedback team made up of professionals who review content.
Please feel free to contact Chandler Swope with any questions: email@example.com or 202-674-4848.
Remember: Enroll at https://en.hdyo.org/eve/events/569 before April 30th.
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Choosing Death with Dignity in Huntington’s Disease
Choosing Death with Dignity in Huntington’s Disease
by Sharon McClellan Thomason
Alan Pfeffer is a retired attorney in New York state whose first wife died from Huntington’s disease (HD) and whose 37-year-old daughter resides in a nursing home, slowly dying from HD. Alan is leading the fight for laws that will allow those like his daughter to choose a peaceful death rather than suffering the ravages that HD inevitably brings in the end stages.
Those of us who have lived with HD have seen the suffering. We’ve seen loved ones who can no longer swallow, talk, sit, walk, or attend to personal hygiene. We’ve seen our loved ones reduced to a decimated body, curled into a fetal position. We’ve seen our loved ones throw up repeatedly, unable to keep down any food or liquids. We’ve seen our loved ones refuse feeding tubes and starve to death, painfully. Perhaps worst of all, we’ve seen our loved ones, like my brother-in-law, resort to suicide, often violently.
While there are currently six states with Death with Dignity laws/statutes (California, Colorado, District of Columbia, Oregon, Vermont, and Washington), none of these laws provide relief for those who are suffering from HD or Juvenile Huntington’s disease (JHD). Based on the Oregon model (the first in the nation to pass Death with Dignity legislation), the law requires standards that are impossible for someone with HD to meet. In Oregon, the following criteria must be met:
- The patient must be at least 18 years of age.
- The patient must be capable (defined as able to make and communicate health care decisions).
- The patient must be diagnosed with a terminal illness and within six months of death, as certified by two physicians.
- The patient must make two oral requests to his or her physician, separated by at least 15 days.
- The patient must provide a written request to his or her physician, signed in the presence of two witnesses.
- The prescribing physician and a consulting physician must confirm the diagnosis and prognosis.
- The prescribing physician and a consulting physician must determine whether the patient is capable.
- If either physician believes the patient’s judgment is impaired by a psychiatric or psychological disorder, the patient must be referred for a psychological examination.
- The prescribing physician must inform the patient of feasible alternatives to DWDA, including comfort care, hospice care, and pain control.
- The prescribing physician must request, but may not require, the patient to notify his or her next-of-kin of the prescription request.
- The patient must be able to self-administer the medication by swallowing; if the pills are dropped or regurgitated, or if the patient regains consciousness, there is not a second chance.
In order to make sure patients with HD may avail themselves of the mercy intended by these laws, Alan is filing this month to present an argument to the NYS assembly at its Public Hearing on Physician Aid in Dying (PAD).
Alan asks the question that strikes at the heart of the matter:
So why do people with Huntington’s refuse feeding tubes when they can extend their life? Because it is life they don’t want to continue to live. My daughter watched her mother slowly die and spend years rigid, unable to move any body part, unable to speak, orally eat, painful muscle contractions, no hope for a miracle drug being researched, placed by the nursing home staff in front of a TV all day. My daughter has a health care directive not to prolong her dying. She was a woman of the world, a Peace Corps volunteer who served her country by teaching the children of Turkmenistan in Central Asia English, who worked and lived in Thailand teaching English to children, who volunteered as an English teacher to foster care children in Israel. She lived for 18 months in Argentina; she speaks five languages; she toured the world; and she has made her choices and wants the same right of choice that is being considered for other people dying of things like cancer. Let’s not leave anyone behind.
Alan proposes that people with Huntington’s disease execute an advanced health care directive, while still of “sound mind,” that would request PAD when they reach the point that they no longer have what they have personally predetermined as “quality of life.” Just as people are currently able to execute a DNR (Do Not Resuscitate) order or refuse a feeding tube or a ventilator, they should be able to execute a request for PAD.
My husband would not have chosen PAD. He wanted to live as long as possible, in a nursing home, assisted by a feeding tube, to share in every part of our son’s life possible. His brother was the opposite. He had no children, and he had said from the time he was diagnosed that when it got to the point that he could no longer take care of himself, he would take his own life. Any of us would have taken care of him, but he did not want to live life on those terms.
Another shortcoming of current law is that it automatically eliminates the children who are suffering from Juvenile Huntington’s disease, since they have not yet attained the age of 18.
The whole point to this is that end of life should be a personal and humane choice. To this end, Help 4 HD International has issued an official position statement on Death with Dignity, which states in part:
HELP 4 HD International supports efforts to increase services and support for HD/JHD families, including palliative care from the time of diagnosis through their loved ones’ remaining lifespan. This includes educating the community about what options are available to them at any time in their journey to retain as much control as they can.
HELP 4 HD International is not a lobbying group and thus does not participate in activities to promote legislation on this issue. Neither does HELP 4 HD International have an official position on what families “should” or “should not” do. We believe, though, that Huntington’s patients should have the right to choose death with dignity by a prior health care directive, a prior appointed agent, and, if necessary, physician assistance in order to enable people with HD to access the mercy that Death with Dignity laws intend.
If you wish to read and/or print out our official statement of position, it may be accessed here: https://help4hd.org/wp-content/uploads/2018/04/HELP-4-HD-Death-with-Dignity-Position-Statement.pdf.
To hear an interview with Alan Pfeffer on “Help 4 HD Live!” click here: http://my.blogtalkradio.com/tools/#/my-episodes/edit?episode=9611851
Survivor’s Guilt With Huntington’s Disease
Survivor’s Guilt With Huntington’s Disease
by Ginnievive Patch
When it comes to Huntington’s and Juvenile Huntington’s disease, guilt can play a huge factor in the lives of those who test negative and are parents, children and siblings of someone very ill.
It is also a subject that can get thrown in the face of the ones without the illness. Many times, my own spouse and one of our oldest sons have said, “What are YOU depressed about? You are not sick.” This may be true, but watching your whole family die before you, especially knowing you had children who now have it, carries a burden like no other.
Survivor’s guilt. Imagine having three siblings, and you test negative. Imagine all your children and spouse testing positive. Imagine being the only child without HD. The questions, Why me? Why am I the healthy one? Why me? Why do my kids have to suffer with HD/JHD? Why my family? Why my parent? Why my sister? Those thoughts trample through the brain with hypervigilance. Such thoughts can actually cause severe depression. Wait! What? Testing negative can cause depression?
The answer is yes. Survivor’s guilt can cause depression. Thoughts like, “What gives me the right to be happy when my sibling will die from it?” can race through your mind. These are difficult issues that the family has to deal with. Young children with a sibling who gets all the attention because of JHD may even get really jealous of the sick sibling and wish to be sick, too, because they are in the shadows with no normal around.
Dealing with survivor’s guilt is multifaceted. Counseling, support groups, and journaling can all help. Online support groups are wonderful. Advocacy helps. Help 4 HD, Wehaveavoiceglobal, and HDSA are all groups that need help. Reading and teaching is another way to deal with it. Admitting your feelings is the first step. Tell someone, anyone, how you feel. Seeking proper medical help if depression is taking over is one thing I say really helps. Learn to forgive yourself for testing negative, for not having the disease.
Other family members may act in what seem to be inappropriate ways, such as withdrawing from the person who is ill. It helps to remember that they may be suffering from survivor’s guilt and cannot handle being around the person who is sick. Learn to forgive them, too.
Taking a break from HD can also be beneficial. It is especially important to arrange alone time with a non-JHD child, away from the sick sibling. Time alone with the well parent is important for the kids whose parent has HD. HDYO (Huntington’s Disease Youth Organization) and NYA (National Youth Alliance) also offer great camps for kids in HD families.
Things to remember: YOU did not do this on purpose. YOU did not get to choose to test negative. YOU would never have harmed a child intentionally. YOU are a blessing to the ones who are ill. HD/JHD teaches unconditional love, but it can come with a price of overwhelming guilt. Take a deep breath and stop holding yourself hostage with guilt.
Bioethics in Huntington’s Disease Treatments and Therapies
Katie Jackson is a strong advocate for the Huntington’s and Juvenile Huntington’s disease community and is president/CEO of Help 4 HD International, which has been on the forefront of the struggle to increase HD awareness and generate funds to combat Juvenile HD. In one of her weekly blog radio broadcasts, she hosted Dr. Mark Yarborough on the topic “Bioethics” and discussed the roles bioethics plays in developing treatments and therapies for Huntington’s and Juvenile Huntington’s disease. Dr. Yarborough is a professor of philosophy and dean’s professor of bioethics at the University of California, Davis. He has published several scholarly publications and lectured numerous professional and public organizations on bioethics.
Bioethics is the study of the ethical issues emerging from advances in biology and medicine. It is also moral discernment as it relates to medical policy and practice. Bioethics is basically divided into two areas:
- Healthcare ethics
- Research ethics
There are regulatory bodies that monitor and control ethical issues that might arise in each of these areas. For clinical environments, such as hospitals, there are Hospital Ethics Committees and Institutional Review Boards (IRBs) that review and make decisions about clinical research. IRBs appear not only in academic but also in commercial sectors. This is compulsory for any organization that receives federal funding for research on human subjects, and it also a prerequisite for organizations to gain approval by the FDA (Food and Drug Administration) of an application for any investigational new drug. This means that all clinical research, including that for Huntington’s disease (HD), in the United States goes through an IRB review process.
Huntington’s disease occurs when a DNA sequence at the end of the huntingtin gene repeats too many times. The mutant gene then produces a toxic protein that damages neurons. HD is known as the quintessential family disease because every child of a parent with HD has a 50/50 chance of carrying the faulty gene. Today, there are approximately 30,000+ symptomatic Americans and more than 200,000 at risk of inheriting the disease.
Though the cure for Huntington’s disease is yet to be discovered, there has been research into a gene editing system, “CRISPR-Cas9,” which has generated excitement in scientific circles for its potential to cure diseases caused by a single defective gene, including HD. But editing genes with this technology is risky because cutting strands of DNA can lead to unintentional gene edits, causing dangerous off-target effects. Due to reasons such as this, regulatory bodies have been set up to monitor and control risks of ethicality involved in bioresearch.
The major roles of IRB in research oversight are (and are not limited to):
- Ensure the safety of research participants. This is the minimization of risk that volunteers might undergo in the course of the research.
- Ensure that the potential benefits of the research, to both participants and society, far outweigh any risks the research participants will undergo.
- Ensure there is fully informed consent of the research participants.
IRBs ensure ethical application of science in the pursuit of a cure to various diseases for the maximum benefits of every stakeholder involved.
People, especially the families of victims suffering from diseases such as HD and Alzheimer’s, have considered the IRB review process to be slow in approving new methods and treatments, but as Dr. Yarborough stated, “The review process is a very formalized and bureaucratic process that involves investigators producing a very exhaustive application and an awful lot of paperwork that covers the background of the science, that answers the questions about the problem the clinical trial was designed to solve, and also information as to why it’s important to do this study, as well as the process for conveying that information.”
This has been the major drawback in the approval of novel medical developments, but there has been some ongoing conversation about improving the review process.
Juvenile Huntington’s disease (JHD) is a special case of Huntington disease which appears in persons below the age of 20. Most institutions have IRBs that specialize mainly in juvenile cases. Lots of questions have been raised by the JHD community about the restrictions placed on underage treatments and tests for children suffering from JHD. This is a case of whether it is right to place juveniles under undue risk for someone else’s benefit since children do not have the legal rights or intellect to make the decisions for themselves. There are also controversies and complications to consider in experimenting on children.
According to Dr. Yarborough, this concern can be bypassed or overcome by creating a community of patients and researchers on related diseases, thereby having its own oversight and regulations. This might not be possible, primarily due to legal reasons. Sacrifices will also have to be made on the part of the members of these communities to help move the field forward, which entails undergoing life-threatening risks.
Most IRB committees are not knowledgeable about all the different diseases that are being studied at any point in time, which is another limitation of the current system. It is not necessary for IRB members to know much about these diseases or to have scientific expertise on these diseases, let alone be familiar with the communities who are affected by the diseases under study. That’s why it’s very important, and even encouraged, according to Dr. Yarborough, for members of these disease communities to volunteer their services on IRBs which are spread across the country.
Dr. Yarborough concluded with these points:
- Ethics is about making sure you’ve got the right people sitting around the table to deliberate about these really complicated questions.
- Frequently, the case is that there are people who have power but lack foresight and people who have wisdom but have no access to power. The job of ethics is to try to get those people in the same room together at the same time. There’s an abundant amount of wisdom in the patient community that doesn’t necessarily spill over into the research community. Therefore, patient advocates must ensure they are not just a voice but also an equal voice at the table.
- People must be willing to invest a lot of time and to build relationships and stick with processes. This may sound challenging at the outset, but it’s worth it.
Listen to the entire interview on BlogTalkRadio: http://www.blogtalkradio.com/help4hd/2018/03/07/bioethics-and-irbs-for-huntingtons-disease
A CRISPR cure for Huntington’s? Retrieved from https://www.fiercebiotech.com/research/a-crispr-cure-for-huntington’s/* Custom Archives Functions Go Below this line */ /* Custom Archives Functions Go Above this line */
Finding a New Normal
Finding a New Normal
By Sharon McClellan Thomason
“December 15th will be two years since we lost Cory,” says Stacey Sargent, who lost her son to JHD when he was 21. “Every holiday and special occasion feels incomplete; however, Thanksgiving and Christmas are especially hard. I feel anxious this time of year, trying to remain strong for everyone else in the family, when all I want to do is hide under the covers and cry. It’s exhausting, actually. I go somewhere and see Christmas lights or decor, gifts that I would have bought him, and my heart breaks all over again.”
As Stacey so poignantly puts it, the holidays can be an especially challenging time for people who are grieving the loss of a loved one. The holidays can also be daunting for those who are still living with Huntington’s and Juvenile Huntington’s disease. Whether it’s anxiety over being around a lot of people, swallowing and choking issues, having a loved one in a care home, dealing with irrational behavior or aggression, or just plain missing someone, holidays can be anything but jolly for people with HD/JHD and their care partners.
So what do you do with these feelings? How do you cope? Can you do more than just “get through” the holidays? The key to surviving the holidays lies in finding a new “normal.”
Stacey says that since Cory passed, “A few close friends give me angels for Christmas, and the fact that others think of him gives me such joy. At our annual family Christmas party, something always happens that makes us all think of Cory, and that, too, brings me joy, though I usually ride home crying at the heartache of missing him so much. Missing him doesn’t getting any easier. I think of him daily; this time of year, it’s just harder to hold back the tears. My husband asks me every Mother’s Day, birthday, anniversary, Christmas for gift ideas . . . the one thing I want, my son, he can’t buy or make or give me in any way. We place something of his under the tree, a pillow that he didn’t get to open or see two years ago. We make donations in his memory. We (Terry and I) release a balloon on his birthday, Angel Day, and Christmas. These things help a small bit, and my faith. I know he is celebrating in Heaven, and try to imagine the party there……”
Others try to carry on traditions started by the loved ones they have lost.
Katrina Hamel, who lost her mother to Huntington’s five years ago, says, “Thanksgiving and Christmas remind me so much of my mom and her holiday spirit. She dressed up, she decorated from top to bottom, she always invited everyone over and always cooked a feast, for as long as she could. This year, I will channel her. I have invited family and friends, I will cook, I may dress up, but most definitely, I will decorate! I choose to give the best of myself to others because it makes me happy!”
Holidays can be hard not just for caregivers but for those who have Huntington’s, too.
“I find that Christmas is the best and worst part of the year for me,” says Cameron Wedge Howard. “I absolutely love the holiday decorations, watching my kids open stockings and presents . . . there’s no better feeling. But at the same time, we’re busy with extended family, and that’s overwhelming. Also, my impulse control is horrible, so I lose myself in buying presents. I’m not fond of stores, so I pretty much shop online. It makes it easy to go overboard.”
Dealing with all the emotions, the excited chaos, and grief can mean it’s time to find a new “normal.” For someone who is struggling with impulse control, that may be as simple as handing over the credit and debit cards to a trusted family member so that spending doesn’t get out of control. Another possibility is to provide the loved one with a pre-paid card that limits what the person with HD can spend.
Since anxiety is such a huge part of Huntington’s disease, anticipating and avoiding the things that cause anxiety can go a long way toward making happier holidays for everyone. This, too, can be part of creating a new “normal.”
“Planning to serve the meal at the regularly scheduled time may be helpful,” says Ginnievive Patch, who has a husband and two sons with HD, “and minimize extra noises (no TV or music), since family is noisy.”
Limiting holiday outings and limiting the number of people sharing holiday meals (immediate family vs. extended family) can also help to ease anxiety. For the caregiver, allowing others to help prepare the holiday meals, ordering food from a restaurant, or even going out to eat at a restaurant are all possible alternatives to wearing yourself out by trying to juggle cooking and caregiving. Again, finding a new “normal,” one with new traditions that can provide everyone with the most enjoyable, stress-free holiday possible, is important.
If your loved one is in a care home, you might consider taking the holiday to the person rather than bringing him/her home. Nursing homes and assisted living facilities are usually willing to let the family have the dining hall or a conference room for a celebration. I can remember when my daddy (non-HD) was in the hospital after suffering a stroke. We took a small tree, presents, and our traditional holiday meal to the hospital and had Christmas there.
Depending on the temperament and care needs of your loved one, you may be able to bring him/her home for the day. When my husband was in a nursing home, a family member would bring him to his mother’s house for the holiday, and then one of us would take him back.
Jacki Harrison, who lost her husband, Ken, two years ago, says, “I was a married widow for ten years, so he was gone way before he left this earth. After a while, it was difficult him going to our friends’ anymore for Thanksgiving. He would sneeze at the Thanksgiving table, and the saliva would reach people’s faces. He would growl, groan; he would need to go to the bathroom and needed help doing so. He would try to talk, and people would get impatient, although [they would] be polite. This was the point we realized it was better for him to stay home. He was much more comfortable being at home, even alone for one or two hours, and then in later years with [his sitter]. The first couple of years, I felt bad for him, but I understood how much better it was for him to be at home where he was comfortable. Also, even though I felt bad, it was much easier on me, too.”
Jacki says that she and her children go to visit Kenny at the cemetery and say prayers during Thanksgiving. Her daughter talks privately to Ken, and she talks to him, telling him her heart. Their son reads prayers from the Jewish prayer book. This has become their new “normal.”
Kevin Jess, whose wife is in a nursing home in end stages, says that as Jehovah’s Witnesses, he and his family do not celebrate the holidays, however, “My wife, Sheila, always celebrated it. In her current condition, it is not possible for her to do things such as gift shopping or baking, etc. She also prided herself on an incredibly large collection of village pieces that took up an entire room. She enjoyed the smell of turkey roasting and pumpkin pies baking, and yes, she enjoyed the music which drove my son and me crazy. However, since I also love turkey and pumpkin pie, as well as giving and receiving of gifts, I usually gave gifts at other times of the year, when I could afford to do so, and when it wasn’t obligatory. I also do cook a turkey and all ‘the fixin’s’ once a month. She always thinks it’s Christmas, and during the holidays, I make her a sock to rummage through, and I turn on her holiday music that she loved as it stirs memories that she cherished.”
Preparing a holiday meal that is both palatable and easy to swallow for those with dysphagia (swallowing disorder), can require some real creativity to find a new “normal.” In the Nov. 14 issue of Today’s Caregiver e-Newsletter, Bobbi Carducci shares the following recipe for “A Dysphagia Thanksgiving”—her creation for her father-in-law who wanted “real” food:
Turkey – I tried pureeing both dark and white meat turkey and found it too grainy so I used a well-known brand of junior baby food and pureed it further to remove all lumps.
1 cup homemade stuffing – Place in food processor with 2 tablespoons of homemade gravy. Puree until smooth, making sure all the lumps are removed. (Add gravy one teaspoon at a time as needed.)
½ cup mashed potatoes – mash or puree to remove all lumps. Add gravy to the potatoes for flavor.
½ cup creamed spinach – puree until very smooth
For dessert – remove crust from one slice of pumpkin pie, top with whipped cream.
The measure of the meal’s success was her father-in-law’s comment that, “This is just like my wife used to make.” (https://caregiver.com/articles/thanksgiving_w_dysphagia/?utm_source=Caregiver+Newsletter&utm_campaign=844894ef8d-Caregiver_Newsletter_10_10_17&utm_medium=email&utm_term=0_8c5d5e6a5e-844894ef8d-94190545&mc_cid=844894ef8d&mc_eid=ce0f1754d1)
Thanksgiving this year was an opportunity for me to practice finding my new “normal.” My son suffers from extreme, isolating anxiety and did not want to be with anyone on Thanksgiving; he emphasized, though, that he wanted me to go and be with people I love and have a good time, that it wasn’t going to bother him to be alone and miss all the festivities. I had to fight back a lot of guilt in order to do this, but since plans had been made a month before my son decided he couldn’t go, I went ahead and joined family and friends. Was it hard knowing that Randy was home alone while I was off having fun? Yes. Was he happier not having to leave his comfort zone to socialize with people? Yes. Would he have enjoyed Thanksgiving if I’d prepared dinner at our house and invited people over? No. This goes against everything I’ve always believed about celebrating holidays, but this is our new “normal.”
I suspect I’ll get to try it out again at Christmas.
Tune in to “Help 4 HD Live!” on Wednesday, Dec. 6, at 4 p.m. Eastern/1 p.m. Pacific, to hear Katie Jackson’s interview with Dr. Bonnie Hennig-Trestman, a clinical therapist providing teletherapy.
The Huntington’s Post is made possible by grants from Teva Pharmaceuticals and the Griffin Foundation.
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.