For People With HD/JHD & Their Caregivers
By Lauren Holder
Figuring out what to get someone who has Huntington’s disease can become more and more difficult as the disease progresses.As promised, here is a list of great gift ideas for a person with HD/JHD, as well as some ideas for the caregivers! Happy Holidays!
- Subscription to Netflix or Hulu or Audible
- Weighted blanket
- iPad or tablet
- Mighty Mug – https://themightymug.com/
- Gift certificates for getting nails or hair done, etc.
- Gift certificates for ice cream or lunch
- Weighted pens/pencils
- Adult coloring books with markers (Osborne Big Color by Numbers Book, Books Dream Big, Draw Bigger, Draw, Doodle Design & Doodle Imagine, Draw) You can find these on Amazon.
- “Tickets of Love”
This is a personally-made booklet of coupons that is filled with little gifts the giver (child or adult) wants to give the recipient. For example:
- This coupon is good for a free foot rub, or
- A free hair wash and style,
- A free nail-coloring session,
- An afternoon walk,
- A picnic lunch,
- A reading from a favorite book, out loud,
- An afternoon ride to the ocean (lake, park, etc.)
Other Great Ideas
- Chapstick (a good stocking stuffer item!)
- Slipper socks with ‘treads’ on the bottoms
- Funny, colorful socks
- Gloves, winter hat, and/or scarf
- Purifying Salt Lamp
- Essential oil diffuser
- Motion detector lights inside and outside the home
- Electric toothbrush
- Memorabilia from favorite sports team(s) (i.e., jersey, t-shirts)
For the Caregiver
- Gift certificate to a local cleaning service
- Hire a Virtual Assistant for an hour or two every month to help with staying organized.
- Create a break for the caregiver! Schedule it, make the plans for them, oversee the help (i.e., getting a manicure or having lunch with a friend).
- “Host the holidays” – Decorate the inside and outside of the home, take care of the planning or the cooking, etc.
- “Tickets of Love” (See above.)
***Please check out https://dailycaring.com/the-11-best-holiday-gifts-for-caregivers/ for these and other ideas for caregivers!***
You can also listen to Help 4 HD’s archived program about gift giving on BlogTalkRadio. Listen to Katie Jackson and Lauren Holder’s discussion at http://www.blogtalkradio.com/help4hd/2018/12/12/gift-giving.
Gastrointestinal Symptoms in Huntington’s Disease
Gastrointestinal Symptoms in Huntington’s Disease
By Katrina Hamel, Katie Jackson, and Sharon Thomason
Let’s talk about one of the “untalked about” symptoms of Huntington’s disease: gastrointestinal (GI) issues. According to Dr. LaVonne Goodman’s article “Gastrointestinal Problems in Huntington’s Disease,” “Human study shows that inflammation in the esophagus (swallowing tube) and stomach is common and occurs more frequently in Huntington’s disease (HD) as it progresses. Study also shows that individuals often do not complain. Lower GI function has not been investigated in individuals with HD, but if mouse models of HD are predictive, we can expect problems there, too. But the biggest contributor to gut distress in HD is likely due to medication side effects, less intake of water and more sedentary lifestyle as the disease progresses.” More details, including suggested treatments, can be found here: http://hddrugworks.org/dr-goodmans-blog/gastrointestinal-problems-in-huntingtons.
Anecdotal evidence suggests that lower GI function is indeed a problem for many with HD, just as upper GI function is. Three of the members of Help 4 HD International’s Executive Board (Katrina Hamel, Vice President and CFO; Katie Jackson, President and CEO; Sharon Thomason, Executive Secretary and Director of Education) decided to share their stories.
Sometimes, people with Huntington’s Disease experience gastrointestinal complications due to medications, which is common in people outside of the HD community as well. In my mother’s case, she took zero medications. Believe me when I say she needed them, because she truly did, but medications were not the reason for her gastro discomfort.
As my mom’s disease progressed, she had many body systems change and slowly fail her. Before she had psychiatric issues, before she had behavioral issues, and before her chorea, she seemed to have an “upset stomach”; we ALWAYS had Imodium and Pepto in our medicine cabinet. As time moved on, and she continued to physically change, so did her bowels. Some days, my mom would go to the bathroom several times with no results. This would frustrate her to no end. Her stomach would become distended, and she would have trouble eating. Can you imagine? This must have been so uncomfortable and was also something that triggered perseveration.
This wasn’t always the case, though; she would also have days where she would go too frequently. On these days, we needed to stay close to home because her bowel movements were constant. Her skin would become raw. We needed to clean the bathroom several times a day, and I could always hear her growling, upset stomach. Some days, she wouldn’t be able to get from her room to the toilet quickly enough, and she would leak or have full blown accidents on the way there. This caused her so much shame, even though we tried hard to avoid her feeling that way. My mom ate a balanced diet, and despite our efforts, we never did find any type of food that made the GI issues better or worse.
Dr. Goodman suggests that gut motility and absorption of nutrients are both impaired in patients with HD because of a compromised lower GI function. This was the case with Katrina’s mother. Dr. Goodman also cites a 2009 study which shows that 32 percent of HD patients had an inflammation of the esophagus (esophagitis), and almost an equal number had inflammation of the stomach (gastritis). Many experienced both. This is the case with Katie’s husband.
I remember when I first found out my husband was at risk of inheriting Huntington’s disease I went to town, looking up as much as I could to learn more about HD. What I read was terrifying. To think my husband was going to have to endure the suffering I was reading was an indescribable feeling. My husband now has been living with Huntington’s disease for over 13 years. Little did I know upon that first google search that all the symptoms I was reading about weren’t going to be some of the most challenging symptoms we would have to deal with.
One of the most extreme struggles we’ve dealt with is issues with my husband’s stomach. That first google search, I didn’t see anything about gastrointestinal problems. Even when my husband started experiencing severe stomach pains and excessive diarrhea, I could still find very little information about this in the searches I did. The one place I was able to find a lot of information about GI issues associated with Huntington’s disease was in the support groups on social media. It was a common topic. Caregivers are constantly talking about how their loved ones are suffering with GI problems.
The symptom my husband was experiencing was not only incredibly uncomfortable and sometimes painful for him, but it also scared me. I was constantly wondering if he was dehydrated, and keeping my husband at a stable weight once the GI problems really kicked in was impossible.
My husband has been on anti-diarrhea medication now for over six years, and even with that medication on board, he still has problems daily. We have had him see our primary care doctor as well as a GI specialist, and no one can figure out what is wrong. They thought he may have Crohn’s disease, but they ruled that out. When they couldn’t figure it out, his primary care doctor said he was probably dealing with IBS (Irritable Bowel Syndrome). I accepted that diagnosis until I started seeing things pop up all over the internet from HD families living with the same problem I was seeing.
A little over a year ago, we placed Mike in a care home, partially because of the severity of GI symptoms. Around Christmas time this year, we noticed that my husband started throwing up. Not only was he throwing up, but he was intentionally making himself throw up. I brought my husband home for Christmas to make sure he was here when the kids woke up Christmas morning. Right when we woke up, Mike started throwing up everywhere. I decided to send my kids to my mom’s for the day. They deal with HD already, and I wasn’t willing to let the memory of Christmas with their dad throwing up all day be a memory they had of him. My mom came and picked my kids up, and I spent all day in a dark room holding my husband as he made himself throw up all day. He is pretty much nonverbal at this point, but he did express to me that his stomach hurt, and when I asked him how he felt, he responded, “like crap.” That is a Christmas I will never forget. In order to protect our children, my husband and I weren’t able to spend Christmas with them that year. Was it hard spending Christmas isolated away from our children and family? Of course, it was. The isolation wasn’t the hardest part, though; the hardest part was watching my husband suffer the way he was with stomach pains all day.
The throwing up and stomach pains continued for about a month. We talked to our specialist about what was going on. One doctor thought it may be a psychiatric symptom, kind of like an OCD behavior. I still, to this day, believe that may have been part of it. When I asked Mike why he was making himself throw up, he told me it was because his stomach hurt all the time, and it was the only thing that made him feel better. So now, myself, my mother-in-law, and the nursing staff at the facility my husband is at have really started watching him closely. We are concerned about dehydration and weight loss, but our biggest concern is Mike’s comfort. Can you imagine having to make yourself throw up multiple times a day to try to get some relief from the stomach pains you’re experiencing? It’s awful. We put Mike on Protonix as well as anti-nausea medication to see if that would help. After a while, we think it helped because we don’t see him making himself throw up anymore. I am not sure if this is because of the medication or because he has really cut back his eating to hardly anything now.
If your loved one living with HD is experiencing GI problems, please know you are not alone. There are a lot of us out there trying to help our loved ones on our own with this symptom because it is rarely talked about, and no one really knows how to help our loved ones.
Like any of the untalked about symptoms of HD (hypersexuality, suicide, psychiatric), GI falls into this category as well. It is hard to talk about these things, but you are not alone, and we need more research about HD and the gut. Like all HD symptoms, your loved ones may not experience GI problems, just like your loved one may never manifest chorea over the course of the disease. I am just noticing there are a lot of common stories out there, and I think we need to talk about this issue.
My husband and two of his brothers suffered from HD. Paul and his youngest brother both had severe problems with vomiting. No matter what they ate, or when, chances were, it was coming back up. Their mother, Gwen, was the caregiver for both, and she was from the old school South where we believe that one of the ways you show love for people is by feeding them. It was so frustrating for her not to be able to feed “her boys” the way she wanted. Paul stopped eating because he either choked on or vomited up his food. Paul was still mobile at that time, so the decision was made to insert a feeding tube so that he wouldn’t choke or vomit anymore. Unfortunately, a feeding tube does NOT prevent vomiting, and with vomiting comes aspiration. With aspiration comes pneumonia. Paul continued to vomit and aspirate, even with the feeding tube and not eating. The same was true of his brother, who also had a feeding tube and went through a continuous cycle of vomiting, aspiration, and pneumonia. Both ultimately passed away from pneumonia, a direct result of the GI symptoms.
As always, if your loved one is experiencing GI issues, contact your care team right away. It must be terribly uncomfortable for our loved ones to have to deal with these issues, and if there is anything we can do to relieve some of the pain, we should try. Until then, families will continue to help each other through these symptoms, giving advice to each other like we always do!
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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 */
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.
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Community talks about intimacy, education with HIPE panel
**Mature material has been changed for our audience**
The following is a transcript of the HIPE (Highly Interactive Participant Education) questions and answers at our education day in Sacramento, California. The panel of experts included Dr. Vicki Wheelock, neurologist; Dr. Sasha Duffy, neurologist; and Lisa Mooney, LCSW, all from UC Davis.
Question 1: I love my wife, but every time there is intimacy involved, I feel as though it isn’t consensual; that’s how I’m feeling – though it is consensual; why do I feel like this? Why do I feel as if it is not wanted, but it is?
Response: I’m going to go ahead and broaden that question a little bit, if it’s ok. So, intimacy is a big area that changes, first of all, with any of us, but maybe a little bit moreso with Huntington’s. Remember that part of that is that people with Huntington’s can’t necessarily pick up on the emotions of their partners and the people around them; they can’t read the signs and the signals, and there is no bigger place that’s happening than when you’re being intimate. You’re not usually doing words for everything … some people do words for everything, but it’s a lot of non-verbal, right? It’s a lot of non-verbal communication. So, if you just take it back a little bit, your intimacy changes through the life cycle very much, and it changes with illness – an illness that affects the mind and the body, and this one does both. It (HD) does change people’s responses, their interest level, the way that they show caring, the way that they don’t show caring, the way that they express their needs in every possible way. There is nothing more unique than the way that we are when we are with our partners. So, I think acknowledging, understanding that that’s going on. Letting the person know – ‘cause if you’re feeling something, they might also be feeling something. So, I think it’s important to try to talk about it. Maybe not at that time, but pick a time to talk about it, and then if there are physical issues, or if there are emotional issues – I’ll just pick one – depression can affect intimacy in a huge way – then this is something you can bring to your doctor, you can bring to a therapist or counselor to talk to. Sometimes it’s individual – you just want to talk to your own provider – and sometimes you should go as a couple and talk about it, but don’t just suffer silently, don’t suffer alone. (Dr. Vicki Wheelock)
Question 2: I can’t stand when people stare at my son. What should I do? I want to get angry and literally, sometimes, want to get physical with somebody, but I know I can’t, so what do I do?
Response 1: Educate; that’s your moment. That’s your moment to educate someone to what JHD and HD is. I get it, I get what it feels like to get angry and defensive, and that’s okay for you to feel that way. – But take a pause, turn it around, and see it as an opportunity. (Sasha Duffy)
Response 2: I think that something that happens with HD is that people who don’t know about it – and for us, we all know about it – but Huntington’s is a rare disease – it’s considered a rare disease, so a lot of people don’t know about it. One of the common misperceptions about the person with Huntington’s is they’re intoxicated, or they’re using drugs or something like that. So that’s a very common picture that we see, a scenario that we see. (Someone in this room has some awesome T-shirts that’s like—why don’t you ask about it?) Sometimes we recommend – I’ve had people say that those medic alert bracelets really make a difference, that when someone sees that you have a medical alert bracelet, that’s kind of identifying that way, people might take a step back. I do think that a lot of people just don’t know and judge, cause we humans judge. (Dr. Vicki Wheelock)
Response 3: So it’s important to remember if you have Huntington’s disease, or anything else, that you are still a person, and you should be proud of yourself. If you’re going through something hard, it’s okay to tell people. It’s also important to remember that there are people in this world that are just not compassionate and not caring – those people don’t matter. Okay? It’s the people who care and are compassionate that matter. So, Raul, our beautiful model here, the front says the same thing as the back (showing his T-shirt). The back says, “I’m not drunk!!! I have Huntington’s disease.” He wears this all the time. You can tell he loves this shirt, right? His wife made them. It’s just a way for him, when he’s walking down to get his soda from the local 7-11 or Circle K, people will know what’s going on with him, and it’s also an opportunity for people to talk about it. There was an event we had, the JHD walk (referencing Help 4 HD’s JHD walk) and another shirt that his wife, Gracie, made, said “I have a broken heart because of Huntington’s,” and I was wearing it, and I went and got my family after the walk, and some lady was like, “What is that about?” So it was an opportunity for me to talk about it – of course, she talked to me in the bathroom – so it was an opportunity for me, though, to talk about Huntington’s and what it’s about. So I educated one more person, and now that person is going to be a little bit more caring and compassionate and think about things when she sees someone that may be a little bit different. (Lisa Mooney, with Raul, HD patient)
Response 4: I like to wear this shirt because when I’m at a sport game and they ask, “Do you got drugs?” or “Are you drunk?” or something…they make you mad ‘cause everywhere you go, they ask you that. But with this shirt, it’s better ‘cause they already know what’s wrong with you. (Raul)
Response 5: So, I just have to say when we found these shirts, we called Gracie, and I asked, ‘How many of those shirts can we buy?” ‘cause when Mike wears his shirt, people actually come up to us and ask, “Well, what is Huntington’s? Explain it to us.” So, people want to learn, so then you’re educating, and you’re cutting the whole stigma of what’s going on, people staring – so they are definitely my favorite shirts and are a part of my husband’s wardrobe. (Katie Jackson, president/CEO of Help 4 HD International)
The Huntington’s Post is made possible by grants from Teva Pharmaceuticals and the Griffin Foundation.
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First HIPE Day Answers Questions from Huntington’s Disease Community
Sacramento HIPE Panel: Dr. Vicki Wheelock, Dr. Sasha Duffy, and Lisa Mooney, LCSW
First HIPE Day Answers Questions from Huntington’s Disease Community
by Sharon McClellan Thomason
Have you ever wanted to sit down and just talk to a healthcare professional who is actually knowledgeable about Huntington’s and Juvenile Huntington’s disease? Maybe you’ve wanted to ask, “How do I know when it’s time for my loved one to stop driving, and how do I tell him/her?” Or maybe you have questions about feeding tubes or physical therapy or medications for psychiatric and behavioral symptoms. Maybe you want to know more about hospice or the difference between palliative care and hospice. How do you know when it’s time for those services?
People in the HD/JHD community have so many questions, but often have nowhere to go for answers. We end up frustrated with the lack of resources and the dearth of credible information. We feel isolated and alone, burdened with many unmet needs.
That’s why Help 4 HD International began offering our HIPE (Highly Interactive Participant Education) Days in March of this year, in Sacramento, California. The brainchild of Katie Jackson, president and CEO of Help 4 HD, HIPE brings a panel of experts to an audience of HD/JHD families and others interested in the disease. The participants come up with fictitious crisis scenarios like the questions above that might come up (and may have actually occurred) in HD/JHD households. The scenarios are written down, handed to Jackson or another facilitator, and then read one by one to the panel. The experts on the panel problem-solve, often identifying local resources that can provide help, and sometimes participants offer answers as well. It’s a way to get questions answered without identifying the people who are asking the questions, while also respecting and protecting the anonymity of the HD/JHD patients.
The sessions are presented live on Facebook, with as many as 2,000 viewers, and are also posted on our Vimeo channel. In addition to the Sacramento HIPE Day, we’ve hosted HIPE Days in Kirkland, Washington, and Atlanta, Georgia. Our next one is in Cedar Rapids, Iowa (August 26), followed by Houston, Texas (September 30) and our annual symposium in Tampa, Florida (October 21).
This series of articles brings to you the questions and answers from all of the HIPE Days, as transcribed by Katrina Hamel, vice president of operations at Help 4 HD. We hope you find this information beneficial!
This particular article includes the first three questions and answers from the Sacramento HIPE Day.
Question #1: What do you do when your loved one with HD loses their job from HD and cannot afford to pay rent in the community where the rent is very high?
Are there any options or resources for rent assistance?
First thing, if they (your loved one) were working and got fired from their job or got laid off or they quit because they weren’t meeting expectations, you should look into disability. [Look into] whether they have supplemental disability that they maybe got through their employer or state disability, if they paid into that system, or Social Security Disability [SSDI] or Supplemental Security Income [SSI]. So that’s another way to get some more income into the home; all of those things are not super easy to navigate, so if you’re in that situation, of course call me, and I have lots of information, and I can give you all of the details of all that.—Lisa Mooney, social worker at UC Davis HDSA Center of Excellence, Sacramento
Note: SSDI and Medicare are federal programs; SSI and Medicaid or MediCal are state programs, and laws for those will vary from state to state.
Can one get unemployment in that situation? (Asked by Dr. Vicki Wheelock, UC Davis HDSA Center of Excellence)
Part of that is yes, if you paid into the system; if it’s where they’re unable to return to work, then it would be disability. It’s basically the same pot of money, unemployment and disability. As far as rent assistance, each county has a Housing and Urban Development [HUD] branch of their county government. Now, I’ll tell you a lot of them have a long waiting list – that doesn’t mean it’s impossible; there are ways to sort of negotiate. If you’re in a rented space, and you just lost your job, always talk to the landlord and/or the home supervisor to see if you can strike a deal because of financial hardships at the moment in time. Things are always negotiable; most things are not set in stone, so always ask questions; the worst they can ever tell you is “no,” and then you are in the same situation you were in before you asked. No harm, no foul. As far as rental assistance, there’s really not a lot of resources. There is like Section-8 housing for low-income persons and things of that nature. Most counties have two- or three-year waiting lists, which are not helpful, but what I would say is if you’re in that situation, definitely reach out for help. Start calling people, seeing what options are available to you. I of course can be one of these persons that you call for help and support and [I can] help sort of problem solve the details of your situation.—Lisa Mooney
Note: Unemployment benefits also vary from state to state. HUD is a federal program administered by each state.
Question #3: How can a caregiver have a getaway? What if something happens to me, and I can’t care for my loved one anymore?
So what we tell families is whether you have Huntington’s disease in your family or whether you don’t, we all need to do future planning. We never know what tomorrow holds for any of us, so it’s important that you have plans in place to take care of your family members if you are sort of are the primary breadwinner in your family or you are the primary care provider, so there is a lot of future planning that sort of needs to go in process, especially with Huntington’s disease. Caregivers aren’t saved from death; just like Huntington’s disease, or me or Dr. Duffy or Dr. Wheelock, we’re all going to die. So we all need to prepare for that, so you need to do things like advanced health care directives, and that’s for everybody, whether you have Huntington’s disease or not. We need to make health directives so that we have a power of attorney in place so that that person can make decisions for you in the event that you are unable to [make decisions for yourself]. You need to look at your finances, make sure that you have a second person, a person you trust, on all of your finances, your bank accounts, and things of that nature. Make sure your loved ones maybe have a binder on a shelf that has all of your information about what accounts you have, where they can get access to them, what your passwords are – I know you’re not supposed to share those – but you should with your loved ones . . . those types of things ’cause in the event of an emergency, people will need those. If you are in an accident, it’s important to have a plan B. You always need to have a plan B, so if you get in an accident, or if you have surgery, or something of that nature, you need to make sure that you have arranged for family and/or friends to come help you out and support you in that situation and help care for your loved one if you’re a primary caregiver. Those all need to be done beforehand so hopefully you have some leeway like in your surgery situation. If it’s an emergent situation, sometimes if you’re at a hospital, hospital members can help, but definitely always have a family member that you can call or a friend (it doesn’t have to be family) or a friend that you can call that can sort of support you in that situation.—Lisa Mooney
Note: My attorney, Victoria Heuler, in Tallahassee, Florida, advised me to have an alternate guardian assigned for my son, since I am his plenary guardian. There are forms that have to be filled out by the plenary guardian and by the alternate guardian, then filed with the court. Guardianship laws are also going to vary from state to state./* Custom Archives Functions Go Below this line */ /* Custom Archives Functions Go Above this line */
The Patient Advocate: Celebrating the Season of Giving
The Patient Advocate: Celebrating the Season of Giving
by Katie Jackson, President/CEO, Help 4 HD
I would like to begin this article with some of my favorite quotes about being a patient advocate:
- “Advocacy: To change ‘what is’ to ‘what should be’”
- “Strong people stand up for themselves. But the strongest people stand up for others.”
- My final quote is one that was introduced to me by one of the strongest patient advocates I know, Judy Roberson. This quote is used by Judy every time she speaks publicly, and because of her, it is now used by many HD advocacy groups. “Never doubt that a small group of thoughtful, committed people can change the world. Indeed, it is the only thing that ever has.”—Margaret Mead
Whenever I think of the power of the patient advocate, I think about the polio epidemic. This is an event that I have talked about for years when I speak about what a group of patient advocates can accomplish. What Franklin D. Roosevelt, Basil O’Connor, and Eddie Cantor, along with all the powerful advocates, did to combat the polio virus was astounding. A vaccination for the polio virus was the result of their efforts. The world was forever changed because of the March of Dimes advocates.
I started my journey as a patient advocate over ten years ago. I quickly learned that to be a patient advocate, you have to find a cause you are deeply passionate about. One that you can feel deep in your soul that brings out a power in you that you didn’t know you had. You must be passionate, compassionate, persistent, patient, resilient, and strong. You will endure disappointment and pain when your countless hours of work don’t go the way you planned.
The patient advocate knows what I am talking about, but the passion and strength we have make it so that we “won’t back down,” and we continue to fight and push on, even when things don’t go our way. We don’t have time to cry over a blow; there is too much work that needs to be done and too many people that are counting on us, so we push on.
There are those brilliant moments when you work so hard on a project, and it succeeds, and the change happens right before your eyes. These moments are what it is all about—to know all the work, blood, sweat, and tears have made a difference for the families you are serving. There is no better feeling in the world for a patient advocate than when you get to see the world change a little because of your efforts.
The patient advocacy spirit within the Huntington’s disease community is strong. This is a family disease, and families have been advocating for years and years and generation after generation. I have had the honor of meeting HD patient advocates who have been fighting for over 30 years. Being a patient advocate myself, I am here to tell you that at times it is exhausting. That is why I am always in awe when I get to meet the “pioneer” patient advocates. Their persistence and strength to keep going after so many years is truly incredible. Although there are still no therapies and no cure for our loved ones living with HD/JHD, can you imagine where we would be if it weren’t for those early advocates who fought so hard for our families to get support, care, and to find the gene that has affected families for hundreds of years?
Without the patient advocate, change wouldn’t happen.
I am so excited that we are using the month of December for “Help 4 HD Live!” to highlight the patient advocate. They call this holiday season the “season of giving,” and I can’t imagine a person in this world who is more giving than a patient advocate. They are truly the most selfless people walking this earth, fighting hard, not for themselves, but for those who can no longer fight for themselves.
We have decided to name the series of shows we’re doing in December “Celebrating the Season of Giving.” I would like to share with you a little about the four advocates we will be interviewing and a little about what they have done and are currently doing to advocate for our HD community.
Lauren Holder, North Carolina
Lauren is an incredible advocate and person. Lauren has been advocating for our Huntington’s disease community for 10 years now. She has hosted many Huntington’s disease fundraisers. She started her advocacy journey by going to Washington, DC, twice a year to speak on behalf of the Huntington’s Disease Parity Act. She started educating law enforcement after her father went missing for 12 hours, and she was unable to get any help, due to the lack of knowledge about HD.
She has worked with NAMI (National Alliance for Mental Illness) to get CIT (Crisis Intervention Training) in North Carolina for law enforcement. Lauren was the HDSA North Carolina president for many years before she had to step down to help care for her father. In 2004, she was honored with the HDSA Person of the Year award. She has written a book about living with Huntington’s disease and is in the process of composing her second novel.
Recently, Lauren and her father’s story was featured in an article in Good Housekeeping. Lauren is a powerhouse, and we are lucky to have her voice telling her story and educating people about what it is like to live with Huntington’s disease.
Havanna Lowes, Missouri
Havanna is one of the most brilliant 17-year-olds I have ever met. Havanna visited Washington, DC, for the first time in June of last year. There, she was personally able to speak to four Congressional staff members about HD and the parity act. Havanna spoke to her senator from Missouri, and he signed the parity act in July.
Havanna has spoken at many education days. Just recently, she spoke at the Huntington Study Group meeting in Nashville in November. She is passionate about Huntington’s disease youth programs like NYA (National Youth Alliance) and HDYO (Huntington’s Disease Youth Organization). She has held fundraisers and has given talks about HD to her community.
Havanna says, “Advocacy is about telling your story to your community and getting people involved and informed.” Havanna is an amazing young lady who I believe will go so far in this world and will continue to be a champion for HD.
Marie Clay, New York
Marie Clay has been a Huntington’s disease advocate for 20 years. Marie has educated many people about HD over the years. She has done law enforcement HD education, CIT, firefighter and first responder training, hospital training, and much more. She has spoken to many community organizations, like the Rotary Club, about HD.
Marie has been on Capitol Hill many times to speak up for the parity act, and recently, in September 2015, Marie spoke on a panel at the FDA (Food and Drug Administration) Patient Focus Meeting. Marie was able to tell members of the FDA about the challenges living with Huntington’s disease.
In 2008, Marie started a support group in Virginia Beach and has held many fundraising events. Marie worked very closely with Sitrin, a care facility in New York state that now has a unit dedicated to HD/ALS. She has introduced many families to Sitrin, and everyone’s hope is that one day we will have more options like Sitrin available to our HD community.
Marie is one of only two employees that have been honored twice with the Volunteer of the Year award by GEICO Insurance. Marie has been fighting for HD families for many years, and we are thankful for all she has done for the HD/JHD community.
Kinser Cancelmo, Massachusetts
Kinser became more involved with both HD and JHD after her daughter Meg passed from JHD after spending four months in the hospital, with doctors unsure about how to help her. Kinser lost her husband shortly after she lost her daughter.
While Meaghan was in the hospital, Kinser enlisted local news teams and radio stations to do programs to raise awareness for JHD. The local radio station had Kinser on their morning talk show to speak about JHD.
When Kinser lost her daughter and her husband just a couple of months apart from each other, she decided it was time to work on starting a nonprofit business in memory of her daughter, “Meg’s Fight 4 a Cure, Juvenile Huntington’s Disease Foundation, Inc.” Kinser says, “I was appalled at the lack of medical services and places available for children/teens her age that had illnesses that the medical community couldn’t handle. Especially JHD. I wanted to raise money for researchers to continue working on medicines and hopefully a cure for this horrible disease. A group of my friends, who are now Board members of my business, began working on a fundraiser to raise these funds to send out to UC Davis in California. The fundraiser was held in September 2016, raising more than $20,000.”
Kinser is a board member of her local Massachusetts Chapter of HDSA. She is involved in attending meetings quarterly and helping with the HD walks around the state, Education Days, as well as many other events that the chapter sponsors.
Kinser recently took Meaghan’s service dog, Dixie, and finished putting her through a pediatric training therapy class. Dixie passed the test so that she is now an official K-9’s for Kids therapy dog.
Kinser is an incredible woman. One can only imagine how immensely hard it would be to lose your husband and your little girl only two months apart from each other. Kinser is a survivor and is taking what she has learned to help others. We can’t wait to see what the future holds and the changes that will be made because of the “Meg’s Fight 4 a Cure, Juvenile Huntington’s Disease Foundation.”
Celebrating All the Patient Advocates
Help 4 HD International would like to thank all the Huntington’s disease patient advocates out there. Without you, change will not happen. So keep fighting, my friends, for the next generation and all the generations to come. Let’s hope that one day our efforts become a paragraph in a history book of “how they used to live before they found the cure for Huntington’s disease.”
Sacramento Symposium Brings Hope
Jimmy Pollard, Lorenza Estandia, Margaret D’Aiuto de Gallardo, Katie Jackson, Katrina Hamel, and Sharon Thomason enjoy networking at the Sacramento Symposium. Lorenza and Margaret traveled all the way from Mexico to attend!
Sacramento Symposium Brings Hope
By Sharon McClellan Thomason
“The symposium in Sacramento was the best HD [Huntington’s disease] gathering we have ever attended,” Bob Lohse wrote for his wife, Gail, who has HD. “We expected lunch sandwiches and sodas and were blown away with the spread. Your organization was a mix of progress, ‘fun,’ and Q/A with speakers available for questions during breaks. We thought Kyle [Fink] did a great job of explaining how all this gene/DNA stuff ties together for someone not a DNA engineer.”
Dr. Kyle Fink, from UC Davis, was one of seven keynote speakers who presented hope and understanding to attendees at Help 4 HD International’s third annual symposium on April 9, 2016, in Sacramento, California. In addition to speakers’ presentations, the symposium featured hands-on activities, a silent auction to raise money for JHD (Juvenile Huntington’s disease) research, and recognition of some very special people in the HD community.
Dr. Jan Nolta, “Bench to Bedside”
After opening remarks by Katie Jackson (president) and Katrina Hamel (vice president), Dr. Jan Nolta, Director of the UC Davis Stem Cell Program and Institute for Regenerative Cures, opened the day’s presentations with “Bench to Bedside,” a talk about PRE-CELL, her stem cell research that she hopes will produce a therapy for HD. PRE-CELL is funded by CIRM (California Institute of Regenerative Medicine), and Nolta’s team has applied for bridging funds for additional short-term studies that the FDA (Food and Drug Administration) has requested before approving human clinical trials.
Nolta’s lab is focusing on producing “paramedic” mesenchymal (MSC) stem cells that will deliver Brain Derived Neurotrophic Factor (BDNF) to the brain. BDNF is very low in people with HD, and so the paramedic cells, which are mature cells derived from healthy bone marrow donors, are engineered to deliver BDNF to the brain. The goal of the research, said Nolta, is to “slow down striatal degeneration and hopefully coax the new striatal neurons to replace those that are dying.” Nolta noted that in her research, they’ve been able to measure in the HD mice that receive BDNF a reduction in anxiety, a regeneration of striatal volume, and a 15 percent increase in lifespan.
In October, the FDA required that Nolta’s team try the therapy in pigs, so they are looking for additional funding to purchase the pigs and are hoping they will then be ready to move into HD-CELL, the human clinical trials later this year. According to Dr. Nolta’s website (www.jannolta.com), her team is seeking an industry partner to help take the MSC/BDNF platform to even more patients, outside of their planned initial Phase 1 clinical trial.
Jimmy Pollard, “You Are a Part of the Change”
Jimmy Pollard, a popular speaker for CHDI who lives in Lowell, Massachusetts, opened his morning presentation with the theme of “Families Keep Telling Their Stories.” His brief history of HD began in East Hampton, New York, where a young doctor named George Huntington saw families with this “curiosity” and continued to Oklahoma in 1888, where Nora Ball and Charly Guthrie had four kids, one of whom was famed singer-songwriter Woody Guthrie. Nora Guthrie developed HD, and Woody moved to Brooklyn, where he married dancer Marjorie. As Woody developed symptoms of HD, Marjorie began to tell her story. Doctors told her to find other families, so she put an ad in the newspaper, found other families who were living with HD, and founded CCHD, the Committee to Combat Huntington’s Disease, which is now known as HDSA, or Huntington’s Disease Society of America.
Pollard said, “Families told stories, organized, partnered up with doctors and researchers, and now we have pharmaceutical and biotech companies interested in HD.”
He noted that Marjorie always talked about the ripple effect of the pebble being tossed into the pond and said that’s what happened and continues to happen in the HD community because, “Families keep telling their stories.” Without those stories, he said, there would be no change. Equally important, according to Pollard, is that families continue to participate in clinical trials.
Participant Lisa Mooney said, “I loved Jimmy Pollard’s presentation. It was such a thoughtful addition to celebrate families moving science forward with participation in studies.”
Morning Panel: “Update on Clinical Trials and Studies in HD”
After a short break, a three-person panel presented updates on the progress of current clinical trials and studies. Dr. Ben Cadieux, Senior Director of Clinical Development at Raptor Pharmaceuticals; Dr. Victor Abler, neurologist and Global Medical Director of Teva Pharmaceuticals; and Dr. Peg Nopoulos, professor of psychiatry and pediatric neurology at the University of Iowa shared research and results with an audience that ranged from scientists and medical professionals to HD patients and their caregivers.
First up, Abler talked about three drugs that Teva currently has in the pipeline. The first clinical trial he spoke of is Pride-HD, a Phase 2 clinical study of pridopidine to see what effect it has on movement, thinking, and behavior, compared to placebo, in people with HD after 26 weeks of use, one tablet per day. Abler pointed out that it is not for chorea, but is for other physical motor symptoms. Researchers believe that the drug may have an effect on some of the symptoms of HD that depend on dopamine. He also spoke about the Legato-HD trial, which seeks to measure the effects of laquinimod, an immunomodulator that has already been in clinical trials for multiple sclerosis. Legato-HD is currently enrolling and is for people with little or no motor symptoms. The third study, SD-809, often referred to as “the next generation” of tetrabenazine, is currently waiting for FDA approval for the treatment of chorea. According to Teva’s website, SD-809, or deutetrabenazine, reduces dystonia as well as chorea and has fewer side effects than tetrabenazine.
Cadieux spoke about Raptor’s drug RP103, a cysteamine bitartrate that was approved for another disease (cystinosis), not HD, but is believed to be a potential drug therapy for HD. Cadieux explained that the drug has antioxidant properties and that HD mice improve motor symptoms and survive longer when placed on RP103. He said patients are currently enrolled in long-term trials in France. Using the total motor scale for HD to measure the effectiveness of the drug, those receiving early treatment do 25 percent better than those receiving later treatment, and completers who are on the drug for a total of 36 months do 35 percent better than non-completers. He said they are currently developing Phase 3, a multinational clinical trial in which they hope to include patients in the U.S., something that Huntington Study Group (HSG) is working on with the FDA. Raptor’s website says that, “The potential clinical benefit of RP103 in Huntington’s disease is reinforced by preclinical studies supporting three proposed mechanisms of action: 1) increased synthesis and mobilization of cysteine resulting in increased levels of glutathione and reduced oxidative stress, 2) inhibition of transglutaminase and induction of a heat shock protein (HSP) response, which assists in promoting proper protein folding and reduced proteolysis, and 3) enhanced gene transcription and increased expression and secretion of brain-derived neurotrophic factor (BDNF).”
Nopoulos, who is the director of Kids-HD and Kids-JHD studies at the University of Iowa, shared some of the goals and findings of her research. She said that brain development in humans continues through the age of 25, and in Kids-HD, they are trying to determine if there may be subtle signs of HD even in childhood. Thus far, research has shown that the higher the CAG repeat, the lower the striatum development, and so the cerebellum compensates by enlarging; however, with CAG repeats between 45 and 59, there are subtle symptoms in childhood because the cerebellum is not quite compensating. Nopoulos said that Htt (the Huntingtin gene) appears to be evolutionary because the more repeats, the better the brain development as evidenced by a higher IQ and a bigger brain. Nopoulos’s studies are funded by grants that pay all travel costs for the family of a child participating in either study, plus there is monetary compensation for the child. As a caveat, she added, “Kids have to want to do it and to understand about HD and that they’re at risk.”
Nopoulos noted that the DeNovo study has shown that a CAG repeat of 30+ is more unstable, particularly in males, and that in rare cases, their kids can develop HD.
Several attendees noted that it was good to hear about the programs at University of Iowa because they really didn’t know much about JHD research.
Dr. Vicki Wheelock, “Pre-Cell: The Path Forward and Findings Along the Way”
Wheelock, director of the UC Davis Center of Excellence, talked about Pre-Cell, an observational study at UC Davis that is managed by Terry Tempkin, ARNP. The trial to develop “the best therapy possible” began five years ago and studies adults at six-month intervals. Wheelock said 42 patients were screened, and 32 were admitted to the trial which includes a study of behavior and movement, brain imaging, and biomarkers.
Wheelock said the MRIs have shown slow, subtle changes, and the Unified HD Rating Scale has shown a slow progression in members of the trial. The trial also identifies problem behaviors, does an E-Cog rating, and measures quality of life. Spinal fluid that’s drawn is sent to Mass General for analysis and has shown plasma BDNF to be very low.
“We have developed measurement tools to be shared with the world,” said Wheelock. “Good science takes time.”
Dr. Kyle Fink, “Gene Therapy in JHD”
Fink, who is part of Nolta’s lab at UC Davis, explained his research that he hopes will lead to clinical trials for Juvenile Huntington’s disease. He’s working on gene modification or correction, targeting the DNA that is the root of the disease. His research with JHD mice has shown the CAG repeat to collapse down to approximately 16 CAG repeats and has produced mutant gene silencing.
Fink said his study is looking for the best way to deliver the therapy to the DNA. “Htt [the huntingin gene] is expressed throughout the body,” said Fink, “so where does it [the therapy] need to be delivered?”
They’re not sure yet what will happen if one area is treated and not another. Fink added, “Htt is critical for development, so the embryo won’t make it out of utero with a total absence of Htt.”
He said that JHD research may help scientists understand how other genes affect the development of the disease, but, he emphasized, “The bottom line is that we need funding to continue.”
Dr. Peg Nopoulos, “The Neuropsychiatric Disorder”
In addition to directing the Kids-HD and Kids-JHD studies at the University of Iowa, Nopoulos is a psychiatrist. She explained, with the use of slides, that the striatum (a part of the brain known to be affected by HD) is highly connected to the frontal lobe; thus, non-motor symptoms controlled by the frontal lobe often appear years before motor symptoms appear. She explained that while psychiatric and cognitive symptoms are the most disruptive for individuals and for families, they are also highly treatable.
Nopoulos pointed out that depressive symptoms decrease as the disease progresses, and are probably more situational than organic. According to Nopoulos, research has shown that major depressive syndrome has about the same prevalence in the HD population as in the non-HD population.
“The suicide rate,” said Nopoulos, “is higher right before (23.5 percent) and right after (21 percent) diagnosis.”
Nopoulos explained that the striatum helps control frontal lobe symptoms, so impairment of the striatum increases frontal symptoms. An increase in agitation and irritability are probably the most common and often contributes to the difficulty of getting placement into and retention in a care facility. Other problematic frontal lobe symptoms include impulsivity and disinhibition, a lack of insight and unawareness (anosognosia), and apathy.
“Apathy,” said Nopoulos, “is more common than depression and, unlike depression, progresses with the disease. The world becomes very small, and the person with HD becomes isolated.” Nopoulos pointed out that this is often more of an issue for the family than it is for the patient, urging that families and doctors ask the patient, “What is your quality of life?” If the patient is content with his/her quality of life, then the isolation is more of a problem for the family than for the patient.
Nopoulos also explained that the cerebellum is also very connected to the striatum and when affected causes increased chorea, difficulty with balance and swallowing, cognitive impairment, and an increase in psychiatric symptoms. Drugs that modulate the neurotransmitters change the brain chemistry: too much dopamine results in chorea, psychiatric symptoms, mainly agitation and irritability. Nopoulos pointed out that treatment of psychiatric symptoms is the same as treatment of psychiatric symptoms caused by other conditions, such as bipolar disorder and schizophrenia. She said the psychotherapy can be helpful in the early stages and encouraged consultation of The Physician’s Guide to the Management of Huntington’s Disease, published by HDSA.
Terry Tempkin, ARNP at UC Davis, Honored
An emotional tribute to Terry Tempkin, who is loved by many families who attend the clinic at UC Davis Center of Excellence, began with a video of colleagues saying their goodbyes as Tempkin prepares to retire. With barely a dry eye in the audience, Tempkin, who works with over 500 HD families, said, “It is about the families. It’s an extraordinary partnership.”
Judy Roberson, president of the Joseph P. Roberson Foundation, presented a certificate to Tempkin, noting that she is the ONLY privately funded ARNP. Roberson said Tempkin is “a visionary, energetic, has heart, and is compassionate.”
Tempkin, in accepting the award, said, “I can think of no higher calling than to come together to help other people.”
She noted that the clinic began with eight patients and today serves 500.
“I’m leaving UC Davis,” said Tempkin, “but I’m not leaving the HD community.” She challenged everyone to reach out to other parts of the world with far fewer resources.
Joseph P. Roberson Person of the Year Award
Judy Roberson began with Margaret Mead’s famous quote, “Never doubt that a small group of thoughtful, committed citizens can change the world. Indeed, it’s the only thing that ever has.”
Roberson said that when her brother-in-law Joe was diagnosed, there was no one in northern California who knew anything about HD. He started the foundation and contacted Dr. Ira Shoulson, with HSG (Huntington Study Group). Shoulson told Joe he needed to find eight people for HSG to open a Huntington’s Clinic. At that time, there were only two in California. Joe found eight people, a clinic was opened at UC Davis, and Dr. Vicki Wheelock became the director. Now, the clinic cares for 500 people with HD. Terry Tempkin came up with the idea to honor one person each year, someone who showed courage and strength in their battle with HD.
Laura Gagnon, announced Roberson, was chosen as this year’s honoree. Gagnon, who lives in Woodland, California, and is a patient at UC Davis, is active in studies and trials and is the third person in her family to receive the award. She was a registered nurse for 40 years in maternity and lost her dad and brother to HD.
In surveys collected at the end of the symposium, one person answered the question, “What could be improved upon?” with this comment: “Nothing, except for a box of tissues on each table!”
Jimmy Pollard, “Hurry Up and Wait”
The afternoon closed with Jimmy Pollard teaching the audience what it feels like to live with HD, what it feels like to think with HD. Through hands-on activities and the use of volunteers, Pollard demonstrated what it’s like to experience slow thinking, the difference between recognition and recall, the difficulty trying to focus when there’s distraction, the difficulty in trying to plan, organize, and sequence, and the problem with impulsivity—“I can’t wait!”
As the audience tried to write their names ever-so-slowly, Pollard noted that fluency decreases, distractibility increases, and the “inner cheerleader” disappears. These things, he said, are the “seeds of misbehavior.”
In an exercise of trying to draw from memory the picture on the face of a penny, Pollard demonstrated the difference between recognition and recall. Recall, he pointed out, is a much more difficult task than recognition.
Through use of the Stroup Test (color names are printed in different colors; i.e., the word “red” might be printed in green, and the person must read the name rather than saying the color), Pollard demonstrated what happens when there’s cognitive interruption.
Finally, Pollard blindfolded a volunteer, turned her round and round, then asked her to find her way across the room to a designated spot, then asked another volunteer to count backwards from 100 by sevens. Both exercises demonstrated the frustration that occurs when there’s difficulty in organizing and planning.
“When skills start to erode,” said Pollard, “you just want to do things; others get judgmental, saying things like, ‘He could do it if he wanted to.’” But as tasks begin to take extra concentration, it becomes fatiguing, and people with HD just don’t want to continue. It takes more effort to recall, to answer hundreds of questions in a day, and it just becomes harder to stay focused.
One survey respondent commented, “I loved Jimmy Pollard because of his delivery style.”
Wrapping It Up
End-of-symposium evaluations reflected excitement about the day’s events: “I enjoyed the interactions between clinicians, scientists, patients, care partners, industry. The set-up of the meeting was conducive to a more intimate dialogue!”
As one respondent summed it up, “I enjoyed the talks, but I especially enjoyed the level of professionalism. From the appearance of the speakers and the coordinators to the wonderful food, it let me know that HDers are cared for and important and valuable.”/* Custom Archives Functions Go Below this line */ /* Custom Archives Functions Go Above this line */
IACP Conference a Huge Success
This article originally appeared in November 2014.
Frances Saldana, Tom Owen, Vicki Owen, and Sharon Thomason represent Help 4 HD International Inc. at the International Association of Chiefs of Police Conference in Orlando, Florida.
IACP Conference a Huge Success
By Sharon McClellan Thomason
A shocking video that went viral in September spurred the Huntington’s Disease community into action. The ten-minute video showed a young man in West Virginia, Jeffrey Bane, being held face down on the ground by four police officers, his face ground into the pavement, screaming for help and choking on his own blood. Bane has HD and had been stopped while taking his two young children to the park because he looked “suspicious” and appeared to be under the influence of alcohol or drugs.
Frances Saldaña, co-founder and president of HD CARE at University of California, Irvine, decided to take action. She first contacted Louise Vetter, CEO of HDSA (Huntington’s Disease Society of America) and asked that HDSA get on the agenda and have a booth at the 121st annual IACP (International Association of Chiefs of Police) conference in Orlando, FL, Oct. 25-28, for the purpose of educating law enforcement officials about HD. Vetter responded to the request by saying that her “advisors” felt it was better to build a strong strategy and relationship with the IACP organization before setting up a booth in October.
Vetter added that HDSA believed personal conversations with the police departments and using the “Tool Kit” (available on www.hdsa.org) would do much more to build relationships than setting up a booth, and that they would certainly work on getting on the agenda for next year. She said people wouldn’t be able to stop by the booth and have a conversation, and it wouldn’t do much good to develop a relationship with the law enforcement community with a booth.
In an email to H4HDI (Help 4 HD International), Saldaña asked if the organization would be interested in stepping in since HDSA would not. Passionate that this was an opportunity that the HD community could not overlook, Saldaña said, “Yes, they [law enforcement] are at fault for lack of education and mishandling situations like this, but we can also take some of the blame for not being pro-active enough to educate our police about HD.”
It was obvious from community response that Saldaña was right; something HAD to be done NOW. The original video, which has been reposted on multiple sites including personal blogs and news media sites, has been viewed over 120,600 times. The enhanced video, which can be viewed here (http://youtu.be/0Nb2ZPXuWIs) has an additional 25,438 views. “Justice for Jeffrey Bane,” a Facebook page set up by Bane’s brother, currently has 3,722 likes. Many members of the HD community expressed outrage and frustration, sharing their own experiences with law enforcement as well as those of their loved ones. A common thread quickly emerged: many of our loved ones with HD were being stopped and picked up because police officers mistakenly thought they were intoxicated or under the influence of drugs.
Melissa Biliardi, president and CEO, Katie Jackson, vice president, of H4HDI decided this was a project the advocacy organization needed to tackle. Biliardi contacted IACP and learned that it was too late to get on the speakers’ agenda this year, but that we could reserve a booth and talk to attendees and distribute materials.
Since the convention was taking place in Orlando, and I live in Florida, they asked me to be the point person. We recruited HD advocates Marsha Miller to write the copy for a trifold pamphlet and Diana Kastelic to help with graphic design, created a donation site to fund the expenses for the convention, and recruited HD community members to help man the booth. Our donation site quickly raised $4,660, and Lundbeck Pharmaceutical generously gave us a grant for $2,000 for the conference, followed by a second grant of $2,000 for educational materials.
Approximately 16,000 people from 84 countries attended the convention. About 250 people over three days stopped to talk with us. Of those people, only a handful knew for sure what HD is; most had never heard of it! We spoke with people from all over the U.S., Canada, Nigeria, the Bahamas, Ireland, England, Iraq, and Brazil, just to name a few of the countries represented there. One of the things that struck me the most was the interest and compassion that people expressed. They were shocked by our stories, and nearly every person we spoke with offered compassion and prayers, expressed a desire to learn more, and wanted more training. I left there feeling like we had expanded knowledge about HD exponentially.
T.W. Baker, Sr., staff sergeant at the Indialantic Police Department in Florida, said that he was so appreciative of the information we’d shared with him. He said that whenever a new family moves into his community, he makes it a point to go meet them and see if they have any special needs or circumstances. He added, “I try to teach my officers to look beyond what they see at first sight.”
Todd Kirchgraber, training director at St. Petersburg College, located in St. Petersburg, FL, expressed an interest in partnering with us to send trainers to advanced law enforcement officer training all over the country.
Vicki Owen, an HD advocate who lives in Sun City, FL, with her husband Tom, who was diagnosed in January 2010, helped hand out pamphlets and talked to attendees. She said the most important thing she came away with from the conference was “how receptive they were to hear our stories and how readily they gave us ideas.”
Other advocates who helped at the booth were Saldaña and Jeannie and Bob Grundborg. Both Frances and Jeannie lost two children to JHD; Frances has a son who is now in the end stages of the disease. My son, Randy Thomason, helped with setup of the booth.
Despite the fact that we had a comparatively small, low-tech booth (think shooting ranges, semi trucks, armored cars, and a helicopter!), interest in our message was high, and we had a much greater attendance than many of the more “advanced” exhibits. People were genuinely interested in the pictures, videos, and stories we had to share.
Our work is just beginning, though. There is much left to do, including filming and production of a training video and following up with the many people who shared their contact information with us. It’s an exciting journey, and one that proves that grassroots efforts DO make a difference!/* Custom Archives Functions Go Below this line */ /* Custom Archives Functions Go Above this line */