The sealed envelope held life-changing news for a Royal Newfoundland Constabulary officer and his wife; the results of a genetic test done to determine whether Sgt. Rich Wheeler’s wife Ruby had a devastating, and always fatal, brain disorder.
The couple decided they needed to know if early symptoms of Huntington disease were causing Ruby to stumble often and cut her fingers frequently when preparing food.
“Ruby’s gait was off stride… and I know it’s not uncommon for anybody working around the kitchen to nick your finger but it seemed like that was happening more frequently,” Rich said during a telephone interview.
Ruby had a 50-50 chance of inheriting the gene that causes Huntington’s. The testing confirmed that she did have the disease.
Rich now spends as much time as possible educating police officers about Huntington’s, which he knows will one day take his wife’s life, just as it took her father and sister. Her brother is also battling the disease.
According to the Huntington Society of Canada, the disease causes cells in parts of the brain to die, resulting in less control of movements and a reduced ability to recall events, make decisions and control emotions.
People don’t die from Huntington’s itself, however, but from complications such as choking, heart failure, infection or aspiration pneumonia.
Ruby’s symptoms have worsened over time. Her mobility is limited, Rich said. She uses a walker at home and a wheelchair when Rich takes her out. Her long-term memory is good, Rich said, but the disease has affected her short-term memory.
The Wheelers 24-year-old son Jordan has chosen not to undergo genetic testing at this time.
“Jordan is doing great. He has a physical education degree and is working on an education degree now. It will be his choice down the road, if he decides to have a child, if he gets the genetic testing done to see if he’s carrying the gene,” Rich said.
An early diagnosis comes with added pressures such as not being eligible for insurance, Rich said. There is currently proposed legislation before the Senate to prohibit and prevent genetic discrimination.
“Right now there is no protection for anybody with a genetic disease, so people will put off getting testing knowing if they have the gene – even if (symptoms) don’t surface for 20 or 30 years – insurance companies in Canada can say they are not going to insure you,” Rich said.
{Recognizing the signs}
Rich, who is with the RNC’s criminal intelligence unit in Corner Brook, Newfoundland, says people with Huntington’s can sometimes appear intoxicated. He gives presentations to law enforcement officers to help them recognize the signs and symptoms.
The Wheelers have also created a Facebook page (Ruby’s Battle) to help people understand Huntington’s.
The couple and their extended family and friends are great supporters of the Huntington Society of Canada. They raise money through several yearly fundraisers to help fund services and support research.
Rich said they are grateful for support from family and friends and from his comrades within the RNC.
Knowing the disease will eventually be fatal, Rich said the couple treasures their time together and take one day at a time.
“We had all those plans that when we both retired we would do some travelling, visit family. We are trying to do those things now but it is getting difficult because it’s very hard to travel with somebody who has Huntington disease.”
About one in every 7,000 Canadians has Huntington’s and approximately one in every 5,500 is at-risk of developing it.
While Huntington’s cannot be cured there are medications to treat the symptoms, Rich said.
“Ruby is on a medication to control her movements. It works reasonably well but it’s not stopping them. The researchers we have here in Canada have made great strides and are breaking new ground on trying to find a cure (for Huntington’s).”
As Mary Tyler Moore once said, “You can’t be brave if you’ve only have wonderful things happen to you.”
Ruby and Rich draw courage from each other while overcoming adversity and uncertainty every day.
It’s unlikely that a cure will be found in time to save his wife’s life, Rich admits, but adds that a cure could change the life of future generations.
“I think about our son and any children that he might have in the future. By that time they may find a cure.”
BIO
Visit huntingtonsociety.ca for more information. dooley@blueline.ca
Sidebar
Symptoms of Huntington disease (Source: Huntington Society of Canada)
• Emotional turmoil (depression, apathy, irritability, anxiety, obsessive behaviour)
• Cognitive loss (inability to focus, plan, recall or make decisions; impaired insight)
• Physical deterioration (weight loss, involuntary movements, diminished coordination; difficulty walking, talking, swallowing)
There are significant variations in symptoms, and not every person will have all of them to the same degree.
{Early stages}
• Early symptoms of the disease often include subtle cognitive changes.
• May have difficulty organizing routine matters or coping effectively with new situations
• May have difficulty recalling information, appearing forgetful
• Work activities may become more time-consuming
• Decision making and attention to details may be impaired
• May include irritability
Slight physical changes may also develop at this stage. There can be involuntary movements which may initially consist of “nervous” activity, fidgeting, a twitching of the hands or feet or excessive restlessness. Individuals may also notice a little awkwardness, changes in handwriting or difficulty with daily tasks such as driving. At this stage, people with Huntington’s can function quite well at work and at home.
{Intermediate stages}
As the disease progresses, the symptoms become worse. The initial physical symptoms will gradually develop into more obvious involuntary movements such as jerking and twitching of the head, neck and arms and legs. These movements may interfere with walking, speaking and swallowing. People at this stage often stagger when they walk and their speech may become slurred. They may have increasing difficulty working or managing a household, but can still deal with most activities of daily living.
{Advanced stages}
The advanced stages of Huntington’s typically involve fewer involuntary movements and more rigidity. People in these stages can no longer manage the activities of daily living and usually require professional nursing. Difficulties with swallowing, communication, and weight loss are common. Death usually occurs 15 to 25 years after the onset of the disease.
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