Leading up to his initial illness in 2002 at age 37, Michael had no health issues. He worked as an electronics technologist and was an active and healthy man; husband and father of two young kids aged 2 and 5. A few days before Christmas of 2002 he came home from work not feeling well with a flu-like virus which did not clear up. He had severe headaches and was vomiting. Within a few days his eyes and skin turned yellow.
On Christmas Eve he went to his family doctor who rushed him straight to the emergency department of his local hospital. He was admitted and within 24 hours was diagnosed (incorrectly he later found out) with TTP, a rare blood disease. This required immediate plasma exchange (replacement of blood plasma with donor’s plasma), so on Christmas day he was transferred to Princess Margaret Hospital in Toronto. Initially he received plasma exchanges every day and this hospital stay lasted 6 weeks. He experienced severe fluid retention and fatigue. The plasma exchanges were very hard on his body. Eventually his blood levels improved and the illness seemed to go into remission, plasma treatments were slowly stopped and he began to feel better, returning to work part time.
A few months later in June of 2003 he began to feel sick again. He restarted plasma treatments and needed a kidney biopsy to assess his kidney function. Unfortunately, this biopsy led to complications. He had extensive internal bleeding which caused excruciating pain and required admission to hospital again. He was sedated for several days and was extremely sick. A few days later the results of the biopsy showed he had total kidney failure and he immediately needed chronic dialysis. This time he remained in the hospital for 4 months, receiving many more plasma treatments and regular dialysis. While in hospital, he also experienced extreme, dangerously high blood pressure. He ended up in the ICU for several days, experiencing a TIA (Transient Ischemic Attack) which left him temporarily unable to speak. There were several times he was close to death.
His nephrologist who specializes in home hemodialysis said this type of dialysis would be the best for him. So, during his hospital stay he and his wife Margriet were trained to do home hemodialysis. This was a very difficult time as he was very sick and not always able to participate in the training. After spending the entire summer in the hospital, he finally returned home in October and started nocturnal hemodialysis there. He dialyzed 5 nights per week for 8 hours each night while asleep. While his blood disease and subsequent kidney failure caused anemia, blood pressure issues, blood vessel damage, gastrointestinal problems, reduced immunity, neurological issues and mental health challenges and extreme fatigue, his dialysis has further caused osteoporosis, muscle cramps, and sleep disorder. Other risk factors he faces are cardiovascular disease, infections and even death.
Michael finally began physically feeling better and returned to work, however the nocturnal hemodialysis was having a huge impact on his and his family’s lives. Besides it being hard on Michael’s body, it never allowed travel for more than two days at a time. It required a significant amount of time to set up the machine before dialysis, clean and disinfect it after dialysis and manage all aspects surrounding the process (including ordering supplies, taking blood samples, monitoring the water purity, etc). Michael felt like a machine – working during the day and dialyzing at night.
Margriet wanted to donate one of her kidneys to improve the quality of their lives. Testing concluded that she was a match. In November of 2006 the transplant took place. Hopes for improvement were very high. For the first two weeks the transplanted kidney worked very well, but things went very wrong then when the last of three anti-rejection drugs was introduced. This third drug caused a relapse (at that time he was still misdiagnosed with TTP) and he had to have plasma exchange therapy again to stabilize him. The next two years were an incredible emotional and physical roller coaster for their family as he received continuous plasma treatments which were very difficult for his body to endure. He was in and out of hospital constantly with complications and unable to work. In total he has received more than 1600 units of blood plasma. During these two years, he experienced extreme fatigue and significant body swelling. At one point Michael weighed 25 kg more than he should since he was retaining fluid while the transplanted kidney was shutting down. He was on many different medications, at one point over 50 pills a day, and even given a chemotherapy drug (Rituximab) to try to get the transplant to work better.
By October 2008 things had deteriorated to the point that Michael and his doctors had to give up on the transplant. Michael had to go back on hemodialysis. This was a huge disappointment for him and his family. However, the hope was that he would start feeling better and return to work. He continued suffering extreme fatigue which did not make it safe to work. His transplanted kidney was surgically removed a year later with the hope that this would improve his fatigue, but it had no effect. He was told that he would not be eligible for another kidney transplant as long as his disease was not under control. With the wrong diagnosis, the doctors did not understand why he was not feeling well. This disease had led his family on a very lonely journey as they did not know a single other person who had the same rare disease and there was no support for them.
In 2010 Michael met another doctor who did further research and testing. The tests concluded that he actually has aHUS instead of TTP, which turns out to be even more rare. This explained a lot of the unknowns, especially the transplant failure, fatigue and continued sickness. In 2013, Michael was told that a safe and very effective new drug, eculizumab (Soliris), had been approved by Health Canada for the treatment of aHUS. This gave him new hope to become healthy again with a future kidney transplant, vastly improving the quality of his life.
Unfortunately, the Provincial Health Ministries decided to not provide public funding for this extremely expensive drug. He and Margriet became board members of the newly formed patient group, aHUS Canada, to support patients and advocate for access to the drug. Now patients can connect and support each other in Canada so the journey is not so lonely.
While the quest to secure public funding for eculizumab in Canada is still an ongoing issue, aHUS Canada has had success. They have convinced Ontario to make a plan to fund this drug for critically ill aHUS patients and subsequently for patients who have lost kidney function and need a transplant, but many aHUS patients in Canada, like Michael, are still without the doctor prescribed access to eculizumab. He, Margriet and others continue to advocate and educate until all Canadian aHUS patients have access to the treatment they are prescribed.
Michael continues to be on dialysis and has been unable to work for 14 years. He has recently been approved for transplant when he will receive eculizumab at time of surgery. His hope continues to be that one day this will happen in order to regain his health.
Thank you Michael for sharing your story. If you are interested in sharing your story of how aHUS has impacted your life, please contact us at firstname.lastname@example.org.