NILES - Each day that passes for Chasity Sampson, 41, of Niles, she learns to recognize and adapt to the relentless disease that has consumed her and her family. She inherited the mutated gene from her father, who died of the disease she has inherited. Her children and their children are also at risk.
Chasity Sampson has Huntington's disease (HD). She is the first female in her family to show symptoms.
HD is a hereditary, degenerative brain disorder that affects people both mentally and physically. Those who suffer from it can become irritable, depressed, anxious and have mood swings. Physically, HD patients can have shaky hands, involuntary movements and poor coordination, according to the Huntington's Disease Society of America. About one out of every 10,000 people are affected by HD. Most people learn they have the disease between the ages of 30 and 45, but children can start showing symptoms as well.
From left to right, Ryan Hager, Chasity Sampson, holding Donovan Cravalho-Hager, and Mark Sampson are shown. Chasity Sampson was diagnosed more than 12 years ago with Huntington’s Disease, a hereditary disease. Her son, Hager, has a 50 percent chance of developing the disease himself, and if he has it, his son would also have a 50 percent chance of having Huntington’s. If Hager does not develop the disease, his son will not either.
As for Sampson, she found out more than 12 years ago.
"It's just a blood test," Sampson said. "You have to go through counseling before, during and after. There is certain protocol that is followed. I had to wait six weeks after the test results came back because, I mean, that is a life or death situation."
She then decided that she needed to start planning the rest of her life.
What is Huntington's disease?
HD is a "neurodegenerative disease," which means that it causes the progressive death of nerve cells in the brain. Symptoms typically appear in mid-life, between the ages of 30 and 50, and progress for 10-25 years. However, the disease can also strike young children, adolescents and the elderly.
What are the symptoms?
HD is a complicated disease that affects the body, the mind and the emotions. Symptoms of HD vary widely from person to person, even within the same family. In addition, symptoms change over time as the disease progresses.
Abnormal movements are the most visible symptoms of HD. Early signs of HD may include clumsiness, loss of balance and fidgeting. Problems with movements may include quick involuntary movements, known as chorea, twisting postures known as dystonia, and reduced speed and accuracy of fine movements.
HD also affects the ability of the brain to understand, organize and retain information. The disease progressively affects cognitive functions such as organizing and prioritizing, controlling impulses, beginning and ending activities, creative thinking and problem solving. The person with HD may become forgetful, distracted or reckless.
HD causes progressive damage to the nerve cells in the brain that regulate thoughts and feelings. These unregulated emotions, caused by the disease, may cause mood swings and irritability. Depression is also common in HD.
Who is at risk?
HD is a disease of families. Though everyone is born with the HD gene, the disease is caused by an abnormal copy of the gene that is passed from parent to child. It is not contagious in any way. Only a person who is born with the abnormal gene can ever get the illness or pass it on to their children. Every person who carries the abnormal copy of the gene will eventually develop symptoms, if they live long enough.
Every child born to an affected parent has a 50 percent chance of being born with the gene that causes the disease. Males and females have the same chance of inheriting the abnormal gene. Those who have not inherited the gene will not get the disease and they cannot pass it on to their children. HD does not "skip" generations.
Source:?The Huntington's Disease Society of America
"I put my life insurance in order before I got my results because then I could honestly say that I didn't have it," Sampson said. "Now, I have to say that I do have it. After the shock wore off, I decided that I was going to start doing case studies. Within two weeks, I started checking it out. There is no cure or anything, but we do a lot of case studies. We go to Rochester, Cleveland and all over."
When she participates in the studies, she goes to medical centers and gives copious amounts of blood. Her husband, Mark Sampson, said that it usually is around nine vials.
"They take a lot of blood work and use that," Sampson said. "It just depends on which study."
Sampson is so dedicated to helping scientists to find a cure for HD that she is donating something out of the ordinary.
"I'm also donating my brain,"?she said. "I actually had to call around about it. I think that people thought I was prank phone calling them. I had to ask specific questions to the funeral home, like if they had a freezer. I had to know the answers to these questions and they needed to be aware of my preferences. I have to carry a brain donation card along with my license. People have to be aware of that. There is only a certain amount of time to do it afterward."
"Pretty much anything, if she wants to do case studies or donate time or whatever, I'm just here to support her," Mark Sampson said. "I'm not going to tell her that she can't or has to. These are her decisions to make."
"When they first realized her symptoms were starting, that was a hard trip,"?Mark Sampson said. "When we first got back together, they hadn't started yet. Now she does a lot of shaking and has bad mood swings. But I knew that before the doctor told me."
Sampson takes medicine for her mood swings. Her son, Ryan Hager, said that she calls it her "be nice" medicine.
Along with her son Ryan, she also has a daughter, Christiana. They have a 50 percent chance of showing symptoms of Huntington's. Hager's infant son, Donovan, also will have a 50 percent chance of carrying it if Hager has it. If Hager does not have the disease, then Donovan will not either.
Neither one of Sampson's children have gotten tested for it yet. The test is not covered by insurance.
Along with participating in the case studies, Sampson, her family and friends entered to participate in the Huntington's Disease Society of America's Team Hope Walk on April 5.
The Team Walk is a day for families to come and show support and spread awareness about HD. A 5k run, sponsored by Hermes Sports in Cleveland, will also take place.
Mackenzie Platten, walk chair, is responsible for the entries of those who want to take part in the day's activities.
"This is Chasity's first year participating in our Team Hope walk," Platten said. "She has shown tremendous effort and her enthusiasm has been inspiring. One of most rewarding parts of attending the Team Hope Walk is meeting others like yourself that are affected by this terrible disease. We can use each other for support."
To Chasity, one part of the disease that she would like to make people aware about is home care and how to take care of people.
"I think that people with Huntington's need more attention in this area," Sampson said. "Huntington's isn't a popular thing like Alzheimer's and Parkinson's disease. People need to be educated about it."
In the meantime, she is doing everything she can to raise awareness and work toward better treatments and a cure.
"I may not help me, but I'm going to help somebody," Sampson said.