While on a week-long camping trip in 2010, Abbotsford, B.C. resident Brian Tjepkema began to experience several sudden and concerning symptoms. Formerly a cabinet maker, the 39-year-old father of two endured terrible headaches, and his feet were so swollen that his shoes wouldn’t fit. Upon returning home, Brian went to see his doctor, who immediately ordered blood work. Brian was shocked to learn that his platelet count was so low that his doctor thought he had only 24 hours to live if his blood wasn’t stabilized quickly. After being misdiagnosed with TTP (thrombotic thrombocytopenic purpura), Brian was referred to a specialist who determined it was aHUS.
By the time Brian received an accurate diagnosis, he could barely function. In addition to experiencing the same debilitating headaches, he also endured chronic fatigue, nausea and high blood pressure, while the disease ravaged his kidneys and other internal organs. In an attempt to stabilize him, Brian started receiving daily plasma exchange – a supportive therapy which has not been proven safe or effective in aHUS. In fact, studies show that within the first year after diagnosis, 65 per cent of all aHUS patients die, require dialysis or have permanent kidney damage, despite plasma exchange. Though plasma exchange did stabilize Brian’s condition, he was forced to discontinue therapy due to life-threatening allergic reactions caused by the plasma. He could not work, help raise his children, or do the things he once enjoyed – his life revolved around trying to manage his disease and staying alive.
Fortunately, in 2012, Brian began to receive compassionate access to eculizumab (Soliris), the first and only pharmaceutical treatment approved for aHUS. Experts believe that eculizumab is on its way to becoming a new standard of care for aHUS, as it has shown greater efficacy than plasma therapy in the prevention and treatment of the condition. Since starting treatment, Brian’s blood pressure, platelet levels and kidney function were restored to near-normal levels, and he was soon able to return to his usual routine. Today, he continues to receive one eculizumab infusion every two weeks, grows stronger every day, and hopes to return to the workforce.
Brian knows he is one of the lucky ones. Diagnosed and started on eculizumab before he experienced irreparable damage that could have taken his life, he knows that this life-altering medication is out of reach for other adults and children in British Columbia living with aHUS. Frustratingly, besides Quebec, all provincial governments in Canada – including B.C. – had refused to provide access to eculizumab for aHUS through public funding.
Adamant that eculizumab saved his life, Brian committed himself to advocating for nationwide publicly-funded access to eculizumab for aHUS patients, before other Canadians living with aHUS fall victim to the deadly consequences of this disease. Brian has met with his Member of the Legislative Assembly (MLA) and has participated in interviews with his local media to increase public awareness of this urgent issue. He joined aHUS Canada’s board of directors in 2013.
Thank you to Brian for sharing his story. If you are interested in sharing your story of how aHUS has impacted your life, please contact us at firstname.lastname@example.org.