It’s been several weeks since our last official update, and so much has happened. Thank you for patiently waiting and understanding how difficult it has been. We’re actually not quite sure how we are functioning as a family, but we wake up each morning and start again.
Our appointment with the Hospital for SickKids on Hillary’s birthday was difficult. Their transplant team went over the various treatment options, risks, and possible outcomes. We also received some news that Hillary had developed a peculiar Paroxysmal Nocturnal Hemoglobinuria (PNH) clone, which now makes her condition even more rare. It’s not “classical” PNH, but a clone. As her PNH clone changes (in either direction) — and her Aplastic Anemia fluctuates between severe and moderate — different treatment options come into play. Her condition remains idiopathic (meaning for an unknown reason) with different signs leading both towards and against it as a immune-related response. More tests are out all over the world. Our doctor said it best — we are in no man’s land. We now know it’s okay to be emotional, unbalanced, and exhausted.
The experts at CHEO and SickKids have been incredible, and they fully support our decision to welcome another opinion from the transplant centre at Chu Sainte-Justine Hospital in Montréal, Québec. They want to see Hillary. And so, we are off to Montréal this week to help us make the most educated, informed decision we can regarding the survival of our daughter.
Here are our possibilities as they exist at this moment.
Watch and wait. This is where we have been for the last two months. This means frequent blood tests to check her hemoglobin, platelets, and neutrophils, and giving her blood transfusions when needed. Hillary remains neutropenic, meaning she can’t fight off infection, so we are staying at home in isolation. It’s tough. But if anyone gets it, Hillary does. She’s not sad. Short term pain for long term gain. We can handle that, if it means we get to keep her. But, each blood transfusion adds new antibodies to her system which can change her chance of success for a bone marrow transplant, and transfusions over the long term can cause organ damage. So, we watch and wait, but are planning for imminent treatment.
Immunosuppressive therapy. This treatment involves bringing down her immune system with anti-thymocyte globulin (ATG) for five days in hospital, and then trying to kick start her bone marrow with strong drugs for a year at home (cyclosporine). But, it only works half of the time, and many go on to a bone marrow transplant anyways. While it is less risky than a transplant, she can still develop a deadly infection during treatment. And, the disease may come back later in life, in a more serious form. Calculating the odds.
Going to transplant. If we had access to a 10/10 sibling stem cell match, we would have gone immediately to transplant. If Hillary dips into the “very severe” category, we go right to transplant. Hence, the desperate quest to find a 10/10 unrelated donor match. But, the dangers of proceeding with a bone marrow transplant are serious and life-threatening, particularly with an unrelated donor. First, is finding that perfect match. While we still remain confident that her match is out there due to her common HLA type, and that a potential match will follow through when called upon to donate (God, please), it remains unconfirmed at the moment. A transplant will mean relocating to either Toronto or Montreal for at least 100 days. Praying for her survival will be the most time-consuming part.
Clinical trials. Half-match (haploid) transplant. Immunosuppressive therapy variations. And of course, there is still the chance of a miracle….a full unexplained recovery. Still.
We will continue to encourage the world over to join the global stem cell registry to increase Hillary’s chances of having a match available when she needs it. We are in contact with so many families who have found their “one” match in the world, so many people waiting for it to happen, and so many whose children have died waiting. We simply can not fall in the latter category, and we will forever advocate for everyone to have an equal chance at survival. In the words of Hillary’s favourite Princess Jasmine in the new Aladdin, we will not go speechless.
We have always been very cautious parents when it comes to caring for our children. Making this decision will be the single hardest thing we ever do. Your compassion and support is all that is needed. The rest has no meaning.