Family Stories

Stephane: APS & CAPS Galore

null APS & CAPS Galore
One day at work, I was experiencing muscular pain reminiscent of a hernia. At the beginning I did not get too worried and kept on working for about week. By the week’s end I was trying to sleep and the pain had gotten so bad that I decided to go to the emergency of a nearby hospital. There, I got much worse. The doctors thought I either had pneumonia or a lung embolism. At the time I was only 36 years old. The emergency doctor had consulted a pneumologist who thought I was too young for a lung embolism. I could hear them argue over the phone and they opted to give me antibiotics as well as heparin just in case. The pain was excruciating. Morphine did not help in the least. The next day I was ambulanced to another hospital for a more detailed scan. I was greeted by the same doctor who had argued for a diagnosis of pneumonia the night before. To his astonishment, the scan clearly showed a lung embolism. Had the emergency doctor not given me heparin the night before, I would surely have died for I had waited too long to seek assistance. I was hospitalized for three weeks and thanks to heparin, the clot slowly shrunk and I was given Coumadin for the next six months.
At the end of the six months, I had to stop taking Coumadin so that I could be tested for the antiphospholipid antibody. It came back highly positive. It was clear that because of the extremely high level of antiphospholipid they found, I was required to go back on Coumadin right away. At the time, three weeks had gone by without having taken any anticoagulants. The next day I started having a familiar chest pain. Before my blood had a chance to thin out to a therapeutic level, I got another lung embolism. Since I already knew what that felt like, I didn’t wait long before rushing to the emergency room. Once again, that brought me another two weeks hospitalization.
A couple of years later on my wife’s birthday, I got a third lung embolism. At the time my INR was therapeutic at 2.7, which meant that under normal circumstances my blood should have been sufficiently thin to avoid another clotting event. It became clear to everyone that I was unusually susceptible to clotting. As a result, the hematologist decided to install a filter in my vena cava to prevent clots from forming in the lower extremities and gravitating towards my lungs. The operation seems to have done the trick for I did not have another lung embolism since.
In the next ten years I had a number of relatively mild complications. I started having kidney issues, ischemia in the foot and other immune related issues. On the 12th of January 2009 I went to the emergency suffering from strong abdominal pain and nausea. For two weeks the doctors were unable to give me a clear cut diagnosis saying that I probably had some kind of lymphatic cancer. My lymph nodes were swollen throughout my body. The doctors were overwhelmed and decided to transfer me by ambulance to a research and teaching hospital. I was handed over to the internal medicine department. After a week of tests, I was told that I was suffering from catastrophic antiphospholipid syndrome and I was rushed to the ICU and given plasma exchange. At the time, several of my vital organs had started to show some kind of damage. Maybe an hour after the procedure I already felt a whole lot better at last.
Unfortunately the story did not end there. I had no less than 13 more relapses during that same year. Complications piled-up and I remained hospitalized for a total of 7 months, I visited the ICU three more times, I returned to the emergency at least 4 times and I had so many plasma exchange sessions that I can’t even count them. Before I got the plasma exchange, my lungs, kidneys, liver, heart, spleen and adrenal gland all were taking a beating. It ended up being too late for my kidneys but all other organs eventually recovered.
Today, at 48, I have to follow dialysis treatments every second day; I take a ludicrous amount of pills, injections and other products but I am alive and able to resume many of my normal activities. I opted for home hemodialysis. It grants me more autonomy. I am now in my 18th relapse. As of yet there doesn’t seem to be an end to those CAPS episodes. Yet I am hopeful that my doctors will find a solution to this pernicious disease. At times I feel overwhelmed but it helps to talk about it and share my experiences with people like you who may have had similar experiences. Unfortunately, it looks like I will not be able to return to work this time around. My new full time job will be to manage my disease and learn all I can about CAPS. The more I learn the more I regain control over my life. I guess I am lucky. I have a fantastic spouse who continues to support me throughout these ordeals and I have a superb team of doctors, nurses and staff who look after me as if I were family. The warmth I felt from the hospital personnel is without a doubt instrumental to my getting through the rough patches.