What happened?
Karen was a private person who chose not to share much information about her medical condition and treatment. With her passing, I felt it would be helpful to describe the past year to help people understand what happened, and to say thank you to the many family and friends that lent us much-needed support.
In July 2015 Karen was diagnosed with an extremely rare blood cancer called myelofibrosis (MF), a non-hereditary disease caused by acquired gene mutations. MF is a serious bone marrow disorder that disrupts the body’s normal production of blood cells. MF affects at most 1 in every 100,000 Canadians. Following the diagnosis, we consulted extensively with MF specialists in Toronto and Chicago. At a session in Chicago Karen met for the first time someone who shared her condition.
MF has a tempo; it can progress slowly or quickly in different people. In Karen’s case, a series of tests – including ones that are in the research phase – confirmed that Karen had a high-risk, rapidly evolving disease. Doctors estimated her survival to be measurable in months, not years. Two options were presented to us. The first was to try to manage Karen’s symptoms with medication, but ultimately there was nothing they could do to slow its progression to inevitable leukemia and death. The second option was an allogeneic stem cell transplant (SCT, also called a bone marrow transplant).
SCT is a high-risk, high reward therapy. It had the potential to be curative, but also the potential to be fatal. SCT is available to perhaps 10% of MF patients – many are deemed not healthy enough to survive the therapy, and others are unable to locate a match. SCT for MF has to be performed using stem cells donated from another person. We were well aware of the risks and made the choice to seek the treatment and a cure.
In August the Ottawa Hospital initiated a worldwide search for a donor while we waited and hoped. In early September they found a donor, anonymous to us to this day, who was scored a 10/10 – the best possible match. In October the transplant was scheduled and we began to notify friends and colleagues as we prepared for what would in the best-case scenario be a two or three-month course of treatment followed by a one or two-year recovery.
My sister Laura moved in with us and I took a leave from work to be Karen’s caregiver. We were fortunate to be so close to the Ottawa Hospital, which has a world-class transplant program and became our home away from home. At the end of October, Karen began ten days of intensive chemotherapy before receiving her new stem cells on November 6th. In December complications arose, and she developed a serious and life-threatening condition called Graft versus Host Disease (GvHD). The new stem cells provoked an immune response from the small amount of Karen’s own immune system that remained, which resulted in severe inflammation of her gastrointestinal tract. GvHD is a life-threatening complication that is impossible to predict – even with a good match it can occur. It was a serious blow.
Karen was hospitalized for three and a half months while she recovered from GvHD. We knew that if it returned as the immune suppression was tapered that it would be harder to treat. April and May were spent at home with regular visits to the General, and we were hopeful that things were on the mend. Laura left us in March and Karen’s mother Joan moved in to help. I returned to work nearly full-time. A second life-threatening complication arose in May; the long-term immune suppression required to treat the GvHD had enabled a virus within Karen to “re-activate” and she developed a secondary cancer called non-Hodgkin’s lymphoma. Lymphoma occurs in 1-2% of transplant patients and can be cured. However, the treatment regimen required additional chemotherapy and Karen was severely deconditioned from the long course of GvHD. Hearing this diagnosis was like climbing onto the beach after swimming across the ocean, to be told you need to swim back. We were very concerned but we prepared to continue on the hard road. The GvHD had abated and treatment started for lymphoma.
In late July, the GvHD returned. Karen was re-admitted and for the first time serious doubts began to enter my mind about whether she could recover. She continued to work hard to take her medication, do her physio, and stay focused on the treatment. We celebrated our wedding anniversary on August 8th in hospital on 5 West while Alex and Lindsay visited my family in Nova Scotia. Karen grew weaker and started to experience pain in her back and belly and on August 10th while Joan was visiting she experienced breathing distress and was rushed to the ICU. The next day doctors explained that she had developed a lung infection. This made three life threatening complications and doctors confirmed our worst fears – recovery was no longer possible. On August 22nd she died peacefully in the ICU with Alex and Lindsay and I at her side.
Throughout this whole process, Karen showed incredible strength, courage, determination and bravery. We must also acknowledge the support from the many people who made it possible to even attempt this journey: our families, especially Laura and Joan who did so much. My employer Guy and my colleagues at ipss and DND. Our friends and neighbours and teachers who made us food, cleared our driveway, drove to countless sporting events and activities, and supported the kids. And of course the staff of the Ottawa Hospital – the dozens and dozens of nurses, doctors, housekeeping staff, clerks, physiotherapists, dieticians, occupational therapists, and others who were always professional and compassionate through these many long months.
In the ICU Lindsay suggested that it would be nice to give money to the hospital, so they could help others to survive. Research in MF and SCT is a worthy cause that has the potential to save lives. Any support to make it safer for those who follow Karen will be greatly appreciated.
Adam Robb, August 25, 2016
