Huntington's Disease Awareness Month

It is May. The month that I post more than any other month. Why, you ask? Because it is Huntington's Disease Awareness Month. That month where the HD community works to raise awareness of this dreaded disease we call Huntington's. I like to do my part and tend to write more this month than any other time of the year.

So, I always start the month explaining what Huntington's Disease. In the simplest terms, it is an inherited condition in which nerve cells in the brain break down over time, Think of ALS, Alzheimer's and Parkinson's Disease all rolled in to one. That is the easiest way to communicate the devastation this disease has since people are more familiar with the three of them as compared to HD. There is presently no cure for this disease but there are ways to manage the symptoms of the disease to make it easier for the person suffering. While great advancements are being made, there is still nothing that will stop the affects of the disease.

Huntington's Disease is inherited. That means, the only way a person get the disease is if a parent had it. There is a 50/50 chance of passing the affected chromosome on to a child. Here is a simple info graphic to breakdown the randomness of how it might spread though the generations.

I could get all technical on you and explain that it has to do with the number of CAG repeats on the 4th chromosome and tell you that the higher the number, the greater chance of getting the disease and the higher the severity of it. But, that isn't what really matters.  To understand what the disease is about, you want to know what it does to a person and why it matters to my family that you understand what this disease is all about. 

Here are some quick facts about the disease. 

While there are cases of Juvenile Huntington's Disease, they are very few and far between. The most common cases are adult on-set. The life span from on-set to the end of life is usually 25 - 30 years. However, since most people are not diagnosed until about midway through the onset, there is usually a much shorter life span once a diagnosis has been made. 

The delay in a diagnosis usually comes because a person familiar with the disease is living in denial that HD is the issue for the symptoms. For a person who is not familiar with the disease, for whatever reason, it takes a lot longer because Huntington's Disease is not the first thing that somebody considers when looking for the cause of the many symptoms a person is experiencing. 

Some of the symptoms:

Awareness matters. I hope that the information that I have shared today will help you in your understanding of the disease. Without awareness, we cannot find a cure. Without a cure, families will continue to lose people to the disease. Help raise awareness and find a cure!

A Fictional Story about Huntington's Disease?

The story opens with something that anybody with exposure to Huntington's Disease can understand...

 A police officer cannot find his gun. He swears that he took it out of the safe and put it in the holster but it isn't there. Why not? What happened? His wife moved it on him. He knows she did. And she has been doing things like that a lot lately. Finally, he finds it, makes accusations at his wife and even looses his temper in the process. But he doesn't understand why and the family is scared. He is even more scared when he looks and sees his mother's glossed over eyes looking back at him. His mother was in the mental hospital because she was a drunk. However, he never drank so it must be his imagination. His mom died when he was just 11 years old so he had no idea what was really wrong with her. 

Fast forward seven years. The forgetfulness has gotten worse, his temper is more erratic he has a hard time sitting still. He has problems walking a lot of the time and his mind seems all jumbled and uncoordinated at times. Others have noticed it, too. Even his friends have started to wonder if he has drinking problem or something. He finally decides to humor his wife and goes to the doctor. What he learns at the doctor will forever change the lives of his entire family.

And so begins the story of Inside the O'Briens by Lisa Genova. 

Lisa Genova is a graduate of Harvard with a doctorate in Neuroscience. She already has three critically acclaimed books about the brain. One about a lady with a traumatic brain injury - Left Neglected, the story of a boy with non-verbal autism - Love Anthony, and the story of a family as it deals with the diagnosis of Alzheimer's Disease - Still Alice. Still Alice was made into a movie, too. I haven't read any of these but have heard that Still Alice is a wonderful true to life story of what families go through - from the diagnosed person to the people caring for that person. Her latest book - Inside the O'Briens - has been said to be transcendent in its story of a family dealing with the diagnosis of Huntington's Disease. 

I approached this book with some skepticism. In the past, the exposure of Huntington's Disease in the main stream world hasn't been the best. It is usually glossed over or gets all of 5 minutes mention in a TV show. Not necessarily the best representation of what Huntington's Disease really is. So, I was really apprehensive about this book.

One page in, I was hooked.

I could tell that it was going to be a wonderful story and from the get go, it was obvious that Ms. Genova had spent some serious time with a family dealing with Huntington's Disease. She took time to understand the intricacies of the disease and how it affects not only the person diagnosed but the family, too. The story is told from the perspective of Joe and his daughter, Katie. Lisa has done a very good job of showing the struggles of the entire family. There is the workings of Joe's mind before the disease and the concerns of his wife who can tell that something is not right. Then, what happens after the diagnosis has been made - from telling the family to knowing that you have possibly passed on this awful disease to your children.  From Katie's side, you get to see the struggle of a someone who is "At Risk" and whether or not to get tested and if you really want to find out the results of the test.

I'm not going to lie. This story is sad at times and you will probably shed a few tears, but it is full of happy moments, the love of a family as they all come together and triumph for everybody in the family as the begin to accept the inevitable but refuse to let a diagnosis rule their lives. It is a definite must read.

The Parity Act: What It Is and Why It Matters

Dealing with a diagnosis of Huntington’s Disease is challenging enough. Especially if you have reached a stage in your life where you are exhibiting symptoms of the disease prompting you to get tested. So, when you reach that point where you can no longer work because of the disease and are not old enough to retire, you need an easy approval process for both Social Security Disability and Medicare.

As it stands today, the definition of Huntington’s Disease as defined by Social Security is inaccurate. The only symptom of the disease that they recognize as being a disability is the presence of chorea. If that was the only problem with the disease, it would not be a big deal. Unfortunately, that is not the case.

Huntington’s Disease affects the brain in a variety of other ways as well. Many of these other symptoms begin showing up long before the chorea begins to take place. They can be just as debilitating and make it impossible for a person to perform many of the duties that even the most basic of jobs requires. There are many cognitive and behavioral issues that can begin showing as far out as 10 years before any of the uncontrollable movements begin to take place. 

There are many different ways that loss of cognitive function can affect you. For example, say you are a UPS Delivery Driver that has to drive a truck around every day. You have the same route every day. You have several places that you need to stop at every day either to deliver packages or pick some up. Unfortunately, Huntington’s Disease has begun to affect your mind though, and one day you forget a stop on your route – even though you have had the same route for 10 years. This causes you to be reprimanded in some way. It continues to happen at random and you progress along the disciplinary action plan as set forth by your company until they have no choice but to let you go.

The situation really is beyond your control.  You cannot help that your brain has short circuited in such a way. It is not your fault that Huntington’s Disease has begun to wreak it havoc in your life making it impossible to do things that you used to do every single day. The last thing you need is to have difficulty getting the disability benefits you need because of this outdated definition of the disease.

Speaking from experience, it can take years of denials before being approved for Social Security Disability. From the time Robert’s brother first applied to when it was finally approved, it took three denials and nearly two years. Finally, thankfully, he was approved for benefits. It still took two years and caused a lot of hardship on him and us as his caregivers as well. Stress is not a good think for a person dealing with Huntington’s Disease and heaping this undue stress only makes things worse.

Plus, there is a mandatory two-year waiting period before a person can get Medicare after they have been awarded disability benefits. Two years is a very long time in the world of a person who has reached this stage in their condition. There are numerous medications that can help a person living with HD by minimizing some of the symptoms of the disease but that are not cheap and cannot be afforded by someone living on disability alone. There have been many who have died during the two year time frame because they do not have access to the medications that can help them.

Both the definition  of Huntington’s Disease as defined by the Social Security Administration and the two year waiting period for Medicare need to change. Currently, there is an act before Congress called the Huntington’s Disease Parity Act that is aimed at making these changes a reality.  We need them to pass it. Not just for my family, but for every family out there that has to struggle with this dreaded disease.  We need to urge our elected officials to pass this bill for every family out there.  

You can help!

For more information, visit the Huntington’s Disease Society of America’s Advocacy page to learn more about the bill and how you can help. You can also visit Raise Your Voice for Parity to locate your state representatives in Congress and encourage them to take action on this bill.  I hope you will consider doing so.

  

Team Hope Walk 2014

So, five months since my last blog. How does that happen? Factor in a anniversary trip to Disney World to celebrate our 10th wedding anniversary, my graduation from college with a Bachelor's Degree in Business Management, the holidays and a surgery for me that involved a few set backs, it is easy to see how time can get away from you. Add in a severe case of writer's block and time can pass in a blink.

Hopefully, life will return to normal now and I can get back in to the swing of things.

In October of last year, Robert and I did something that has become one of our favorite things to do each year. We participated in the Team Hope Walk that the Great North Texas HDSA affiliate organizes for the area.  I thought I would share some of the pictures from that day.

     

First, we had t-shirts made for everyone to buy and wear. We charged extra for the t-shirts and sold them to family and friends, too. All told, we raised an additional $286 dollars from the t-shirts to donate to the HDSA. Thank you to everyone that bought a t-shirt. 

We got a lot of compliments on our shirts, too. Pretty much every person that we walked by commented on how much they loved our shirts. 

On the back is the name of several people in the family that have Huntington's Disease. If they have lost the fight, we added in the years. They are 

Robert's Great Uncle (His grandfather's brother)

Robert's Grandfather

His Mom

His Uncle (From his Great Uncle)

His brother

His sister

His cousin (daughter of his Uncle)

Robert

For the walk, we had Robert's sister Debbie, her three boys, a friend to one of the boys and Robert's daughter. We even brought Lady along for the ride. 

We had a pre-walk warm up with the shark and lion to the Cha-Cha Slide. 

And then we were off. The actual walk is approximately a mile around a small pond near Les Lacs park in Addison. Here, Debbie is being helped along by a couple of the boys as we walked around the pond.

Afterwards, there were hot dogs and other snacks. There were also several tables set up from the different sponsors for the walk as well as a lot of informational tables. We are hoping this year that we can get Robert's brothers to join us and have an even bigger group and show of support. 

Celebrating the Life of Cheryl Everett

It was four years ago today that Cheryl Everett's body lost the fight to disease. This disease called Huntington's Disease. Four years that she has not been with us. Four years of so many life events she has missed out. Four years of growth in grandchildren and even a new grandchild and some more great grandchildren.

After four years, it has gotten easier to think about her and the many memories that we have of her. But that doesn't mean we aren't sad and that we miss her any less. That couldn't be farther from the truth. We still miss her and still get sad when we think about her. I don't think there will ever be a time that we don't miss her no matter how many years go by. 

The thing is, she wouldn't want us to be sad. She wouldn't want us to dwell on how much we all miss her. Instead, she would want us to celebrate her life and our lives, too. If she were here today, she would tell us all that we have so much to be grateful for and so many better things to do instead of worrying about her. And so, this year, I want to celebrate all that she has given us. If it weren't for her, there would no Everett kids. There would be no Bill, Debbie, Craig, Robert, or Kevin. And I have to say, I am especially partial to Robert. Plus, there are all the grandchildren and some great grandchildren that are here as a result of Cheryl. We have so much to be thankful for and so much to celebrate as a result of her life. 

So, while we do miss her and are sad she is no longer with us, we can celebrate the life that she had and the lives we all have because of her time on this earth. And we can continue to support the effort to find a cure so that her future generations will have a life free from HD. 

Cheryl, I hope you are still enjoying dancing with Elvis.