Living with ALS
It began with his fingers, a gradual weakening in his hands. He noticed he had some difficulties handling his car keys.
Active and athletic, Brian Heaney thought he may have a pinched nerve.
“I made an appointment for a physical,” says the Waterloo resident. “They ran a number of tests over a one month period, X-rays, MRI etcetera. Then I was sent to a neurologist.”
The neurologist put on an electromyography (EMG) to check nerve pulses and determined that Brian may have amyotrophic lateral sclerosis (commonly known as ALS). That was in 2009 before the ice bucket challenge raised the public’s awareness about ALS, and Brian had no idea of the gravity of the diagnosis.
“Since I didn't know how bad it was, I went back to work, looked it up there and it said an ALS patient has about two to three years to live. It was quite a shock. I told Tammie that night after the kids were asleep. It was a hard day,” says Brian. “Tammie and I were devastated.”
Brian was then referred to the Motor Neuron Disease (MND) Clinic at London Health Sciences Centre. The official diagnosis was made after further testing and a nine-month monitoring period to watch his progression to determine the definitive diagnosis. Brian was 41 years old.
“There is unfortunately no specific test for ALS. As a consequence, when symptoms begin they can be quite mild and may be initially dismissed by the patient, or even the family doctor. When the symptoms evolve to be more noticeable, the initial symptoms may not be specific for ALS and several potential diagnoses need to be considered,” says neurologist Dr. Christen Shoesmith, Medical Director of the MND clinic.
“When all treatable conditions have been excluded by the results of investigations, and the patient progresses in their neurological deficit, we can then make a diagnosis of ALS.”
Before his definitive diagnosis in September 2010, Brian ran three marathons -- Toronto in 2005, Boston in 2006 and New York in 2007. He continued running the first two years after his diagnosis, stopping only when he began feeling uncomfortable running in the snow. Around that time he also stopped playing hockey and later swimming due to increasing difficulties with balance and stamina.
In the early years of his diagnosis Brian was still going in to work every day. Then, as his reliance on a walker and then a wheelchair increased, he cut back his time in the office and now works from home as a computer analyst.
The impact of ALS is personal, private and extends to every member of the family when a loved one is diagnosed with the disease.
And this is certainly true for Brian, husband of Tammie and father of Mollie, 16, Emma, 14, and Megan, 12.
“I’ve found ALS to be very constant and relentless. For anyone who has just been diagnosed with ALS, I would recommend living life to the fullest, especially in the first year or so when you still have muscles. You start losing abilities and what you can do decreases with time. My wife and I took our three daughters on a trip to Disneyworld after I found out I had ALS,” says Brian.
The Heaneys told their daughters about ALS when Brian was first diagnosed, but say it didn’t really sink in until they saw the changes, such as doing less on the home front.
“We could no longer split the household chores and the running of errands, and when Brian stopped driving it had a big impact because we have three active daughters in sports,” says Tammie.
About three years ago when stairs became increasingly difficult, the family moved from their multi-story home into a new fully accessible home. It has made a difference for them.
“One of the biggest adjustments is the time it takes to get anywhere, go places and see people. You need to think through every aspect of the activity beforehand to make sure everything is accessible and that Brian will feel comfortable,” says Tammie.
A trip to a Blue Jays game in Toronto in August seemed like a perfect family outing. Tammie purchased accessible seats and planned to spend a bit of time back-to-school shopping beforehand at Yorkdale Mall.
What they hadn’t counted on was that the Yorkdale subway station would not be accessible. It left them scrambling and eventually taking the next station stop along the line.
“We just did not consider that every subway stop wouldn’t be accessible,” says Tammie. “It was very frustrating. Luckily we made it in time for the game.”
Progression of the disease has been steady, moving from the fingers and hands to the upper body and the legs. Now Brian finds his voice is affected and swallowing has become more difficult.
“While it is a gradual weakening, you notice it more when you suddenly can’t do something,” says Brian.
The family has daily help in the home. “For one or two hours a day I have a Personal Support Worker, because there is so much that I cannot do on my own anymore.”
Treatment and research
Brian and Tammie come to London every four to six months, and Brian has a five-hour period of appointments at the ALS clinic. These include blood tests, pulmonary function tests for oxygen levels and breathing, occupational therapy, physiotherapy, speech language pathology, social work, and dietary.
“The team advises the patients of potential modifications that may improve their safety, function, or quality of life. This might include suggesting an ankle foot orthotic, or suggesting avoidance of certain food textures that might reduce the risk of coughing with eating, or advising of changes that may help with symptoms of shortness of breath,” says Dr. Shoesmith.
“Patients will be seen in the ALS clinic every three to six months, depending on their rate of progression. Patients with average progressive ALS will be seen every three months. Patients with slowly progressive disease such as Brian will be seen every six months or so.”
There is no cure for ALS, although there is one medication available - Riluzole - which, Brian says with a wry laugh, “is supposed to prolong my life by three months.”
Contributing to the effort to find a cure, Brian has participated in two research studies over the past few years. The first study for the drug Ceftriaxone did not prove to make significant change or slow ALS in the final study results.
“The first trial for the drug was pretty intense. I had a catheter in the chest with which to pump drugs into my body twice a day,” says Brian. This took about 20 minutes each time. Brian participated for 18 months, including monthly visits for drugs and tests at LHSC.
Brian then participated in the Cytokinetics Phase II oral drug study for ALS. While Brian himself didn’t see any benefits, the results of this eight-week study were very promising for some of the participants, slowing the rate of their respiratory and muscle weakness.
Phase III of this drug study will begin shortly, and if that trial shows positive results it will be because of participants like Brian that this drug may make it to market for treatment to slow ALS.
In addition to participating in research trials, Brian and Tammie are active at their local ALS Support Group giving back to others. The ALS ice bucket challenge brought Brian joy and hope as people learned of this disease and their donations allowed a lot more research to be done.
Brian created the Heaney's Heroes team and invited all his family and friends to join him at the Walk for ALS to raise money for support services and research.
“Every year we do the Walk for ALS in Kitchener Waterloo. This year we had over 100 people, our largest team ever,” says Brian. “It is a lot of fun to have family and friends out for the walk and Heaney’s Heroes has raised between $20,000-$30,000 in each of the past five years.”
Organizing the team for the ALS walk is one of the ways Brian keeps positive.
“A lot of it has to do with my children. It’s easy to stay positive when they’re around. I’m keeping pretty busy and don’t have time to be sad, such as organizing the team for the ALS walk, I have to have something to look forward to,” says Brian.
“ALS has made my life and my family's life much harder but I'm still making the most of the time I have left. ALS gradually takes away your abilities until you die. This disease needs a cure and soon. ALS robs you of everything. I've been blessed that my ALS journey has been longer than most allowing me and my family time together.”