My blood smear showed schistocytes. I had a central line placed in my femoral vein with great difficulty and pain. Then I had a plasma exchange that I reacted to. My legs kept jumping; I could not even hold them down! After that, the hydrocortisone was dropped from my regimen. The next day a central line into my jugular vein was placed by a doctor in radiology. There was a tiny prick and nothing else. What a difference! I had plasma exchanges daily for the next six days, then again every so often as my hospitalization dragged on. My platelets were up and down and my creatinine blood test result seemed to lag behind improvements in hemoglobin and platelet count but I had no obvious kidney problems. I was in the hospital over Christmas and New Year with reduced staff. Once I had the diagnosis of aHUS, on December 24th my treating physician applied for special funding for eculizumab (Soliris) which is not covered by Alberta Health.
I was told that it was approved (they understood it to be only four treatments), then cancelled, then approved again on January 6, 2017. I received my first infusion Monday, January 9, 2017. I had no reaction. I was discharged the following Thursday. I had the Meningococcal vaccines only the Thursday before so I had to avoid public places for another week. I was put on Cipro until the vaccines had been in my system the full two weeks. I was also put on Prednisone (on a decreasing dose) and accompanying Septra for five weeks.
I am going back to the University of Alberta Hospital as an outpatient for the weekly, and now bimonthly eculizumab infusions. I went back to my part-time job at the beginning of February 2017. I have had headaches that come and go which may be a result of the infusions increasing to 1200mg. They are worse early in the morning. I will ask to have the infusions done here in Sherwood Park when my hematologist applies for another six months’ funding of the Short Term Exceptional Drug Therapy program. I go for weekly bloodwork. I had a Meningococcal booster a month after the first. It was done at the public health clinic here.
I wrote to the Minister of Health here in Alberta but so far they have no intention of publicly funding eculizumab because it is so ridiculously expensive. The Canadian Drug Expert Committee reviewed it in 2013 and 2015 (so I am hoping for another review in 2017!). At that time, “CDEC recommended it not be listed on publicly funded drug plans as the clinical benefit could not be adequately established.” Based on the current situation, our government considers funding of eculizumab for aHUS on a case-by-case basis under the Short Term Exceptional Drug Therapy program.
But bloodwork shows quite clearly that the drug works. Hemoglobin goes up, platelets go up (because they are not elsewhere making clots), creatinine goes down as kidney function improves and LDH goes down as the damaged cells are reduced and then eliminated completely. On eculizumab, the patient returns to full health.
Thank you to Robyn Tansey for sharing your story. If you are interested in sharing your story of how aHUS has impacted your life, please contact us at info@ahuscanada.org.