Agony, Trust and Hope

"That really stuck with me [that many suffering from the rare Stiff Person Syndrome commit suicide]. Their lives are really affected in ways you can't imagine."
"We approach these cases very carefully and are always aware that there have just been a few patients treated and followed for a short time. Seeing these two patients return to their normal lives is really every physician's dream."
"We went in with the feeling that the patients made a very good case about how devastating this disease was on their lives and they were willing to trust us with our technology. We thought if nothing else had worked, this might."
"It is not a cure. We don't know how long these remissions will last; it is just a few patients with a short follow. And there are risks associated with this procedure, it is not like taking a pill."
"It should only be considered where nothing more conventional is working … the symptoms have to be severe and the impact on their life very, very profound because there are risks associated with this. [One in 20 patients receiving a bone marrow transplant for the treatment of leukemia or lymphoma will die of a complication.] And we don’t think stiff person syndrome patients … would have a lower risk [of complications from chemotherapy, including infertility, premature disorders like heart disease and diabetes.]"
“I think for the right patient it might offer some benefit in helping them improve, but it’s too early to say that this is the answer to Stiff Person Syndrome. It’s a pretty dramatic treatment and we need to keep thinking about better ways to do what we’re doing — getting rid of the immune system perhaps with less toxicity and being able to grow back a new one more quickly.
“So I think there are a lot of unanswered questions yet.”
Dr. Harold Atkins, bone-marrow transplant specialist, Ottawa Hospital Research Institute

Dr. Harold Atkins had the results of his treatment of Stiff Person Syndrome published in the Journal of the American Medical Association on Monday.    Photo Credit: CBC / Laurie Fagan

"It is tremendously difficult. Your hope for a positive future is so bleak, the disease starts to chip away at you."
"Now I feel like the possibilities are endless. I appreciate life so much and everything it has to offer."
"I went to visit Dr. Atkins and meet the team [of scientists from the Ottawa Hospital Research Institute] and I was able to thank them. My gratitude for them can't be expressed in words. What they are doing to change peoples' lives is extraordinary. I wouldn't be thriving today without Dr. Atkins and The Ottawa Hospital."
Tina Ceroni, Stiff Person Syndrome sufferer in remission, Toronto

Tina Ceroni is seen in this undated handout photo. Canadian doctors have begun using stem cell transplants to treat "stiff person syndrome," a rare neurological condition in which a patient's leg and other muscles suddenly contract painfully, often leaving them immobilized like a tin soldier. THE CANADIAN PRESS/ HO, Ottawa Hospital Foundation

Tina Ceroni was the second individual to have been treated with the medical pioneering stem-cell transplant developed by by Dr. Atkins and his research team at the Ottawa Hospital (using her own purified cells). A 36-year-old kinesiologist and talented triathlete, her symptoms began in 2008 as she was training for an Ironman half-triathlon. As the disease progressed she realized it was not an injury on the way to healing. When her condition was diagnosed she began to deteriorate, losing muscle control, losing mobility, losing her business, losing her ability to drive, losing her independence.

She had to return to living with her parents and several  times monthly she would be rushed to hospital by ambulance when something inconsequential led to a consequential episode of severe muscle stiffness in her legs, her torso, her abdomen, a disability that could promote itself to threaten her very life. She underwent a stem-cell transplant in 2011, and that event caused her life to take an unbelievable turn for the better; a miracle, she calls it. Her experience and that of another patient preceding her represent case studies Dr. Atkins and his team published in the prestigious JAMA Neurology journal.

She is once again taking her life up where she was forced to leave it; incapable of doing anything physical. She is now cycling again and competing in triathlons. She has also embarked on a fund-raising venture to help raise funds for Dr. Atkins and his team at the Ottawa Hospital Research Institute. Dr. Atkins has been involved with stem cell transplants for quite awhile in its more traditional use to treat leukemia. The procedure has been modified enabling doctors to treat people with severe autoimmune disease, including multiple sclerosis and scleroderma.

Stiff Person Syndrome is a rare and painful disease, a condition that can leave people erect like tin soldiers, incapable of moving their bodies normally. Ms. Ceroni and the previous patient treated by Dr. Atkins have been symptom free for two years. Originally, a patient with the disease had contacted Dr. Atkins beseeching him to consider performing a stem-cell transplant on her. Dr. Atkins did some research on the disease, and then decided to proceed with the request. He was terribly moved when the patient told him how horrible life is with the syndrome.

The procedure developed by Dr. Atkins requires removing blood-forming cells from a person's blood (autologous stem cells), purifying them to remove misdirected immune cells, knocking out the patient's immune system using chemotherapy, and then returning the treated stem cells with the purpose in mind to regenerate the body's immune system with a now-properly operating immune system. The stem cells are young new immature cells capable of maturing to become various types of specialized body cells.

This procedure requires a month in isolation while the risky business of a rebuilt immune system slowly proceeds. A one-in-20 chance of death resulting from the procedure is certainly a daunting feature of the process. Researchers, emphasizes Dr. Atkins, are uncertain what they can expect down the road with this treatment of Stiff Person Syndrome. But patients whose lives have been placed in suspension while their bodies gradually deteriorate and they become other than what they were, horribly suffering, now have the choice to turn their symptoms completely around.

For however long that health-rescue situation lasts, a valuable asset for people whose suffering is so great they see suicide as a release.