Evan MacNeil's Personal Profile
June 2008: Transplant begins; chemo takes out Evan's hair
Evan was admitted to SickKids on Wednesday, June 4, 2008, after having had a central line (Broviac) placed into his chest earlier in the day at McMaster Children's Hospital, Hamilton. The conditioning began that night and followed the order of daily doses of fludarabine x 4, cyclophosphamide daily x 4 and rabbit ATG daily x 3. Mesna, steroids, antivirals, antibiotics, antifungals and antinauseants were added into the mix as necessary to keep Evan comfortable and safe. Evan became very sick from the chemotherapies from about Day-4 until transplant day on June 12, 2008. It was so hard to see him suffer with so much nausea and vomiting, I felt so helpless. Evan was so stoic through those rough days. He coped amazingly well. He never complained or asked "why me?"...not once. He just managed the best way he could, and for him that meant just lying very still and quiet with his eyes open much of the time. It didn't take me long to realize that he wasn't ignoring me when I would talk to him or ask him questions. He simply couldn't. He was too sick and too exhausted and he was doing what he needed to do to get through the hours. I was so proud but also very scared. It was a sight that disturbed me to my core. Once transplant day arrived and we started to see glimpses of our silly boy again, we were overjoyed to say the least. Evan's donor's marrow arrived to his bedside and it was a beautiful sight. The marrow had been collected the day before from his hip bones and it hung on the IV hook, all 500 mL of it, such a glorious shade of crimson. It occurred to me as the cells travelled down the IV tubing inching closer to Evan's chest, that much like the bag of marrow was hanging on that hook, so too was Evan's future. Evan slept throughout the transfusion, which took four hours. His Dad and I sat quietly and surprisingly peacefully in the room, believing with everything that we had that this was going to be Evan's cure. It had to be.
Evan remained in the laminar air flow room for 26 days. During that time he would have two evenings of fevers, making stronger antibiotics necessary. There would be some concern about his liver with each of four doses of methotrexate, given on Day+1, Day+3, Day+6 and Day+9. This drug would cause a tripling of the liver enzymes, but fortunately the numbers would settle back down after a few days. With each morning came an anxious wait for counts when finally, on Day+26, engraftment was declared! Evan was able to move to a regular room and was discharged home, after an uncomplicated course, on Day+36, Friday, July 18, 2008, the day he had his last ever platelet transfusion.
August 2008: Evan felt great but a drop
in HGB proved to be microangiopathy
In the months after transplant, Evan returned to his home clinic in Hamilton for twice weekly and then once weekly blood work and clinic visits. While in transplant, some small cysts were found in one of Evan's kidneys on CT during a routine fungal work-up after his second fever. The transplant team initially suspected a fungal infection and began caspofungin which Evan continued on intravenously at home. As it turned out, because there were no changes to the cysts after weeks on caspofungin, the team ruled out a fungal infection and called the cysts simple and insignificant, suggesting ultrasound follow up every 6 months and then yearly if there is no change.
The only worrisome complication Evan encountered was approximately two months post transplant when, all of a sudden, his hemoglobin began dropping significantly, making the need for transfusion more frequent than the normal course post transplant. We would go to clinic once a week and Evan would need a bag of blood each visit. I would express to his team my concern that there was something more going on but each time I did, I was made to feel like the 'silly mom who has never had a post BMT child before and therefore couldn't possibly know that this wasn't normal for Evan'. I was so uneasy about the situation that I emailed Evan's BMT doctor in Toronto and provided the details that were of such concern. Long story short, Evan was experiencing a rare and serious side effect of the immunosuppressant tacrolimus or Prograf. The drug was damaging the lining of the tinniest blood vessels, causing fibrin and platelets to adhere to the sites of injury. The fibrin would mesh together, creating hair-like strands that would cross the interior lining of the vessels and as the red blood cells would travel through, they would get ripped in half, causing the rapid destruction of the red cells, leading to the dramatic drops in hemoglobin that we were witnessing. The condition is called microangiopathy and it could have killed Evan if it had not been caught early enough. In fact, around the same time I began emailing the transplant doctor, Evan was experiencing a day of extreme lethargy. He was due to attend clinic the next day where we knew he would need some red cells so we weren't overly concerned about how tired he was. At one point he sat up to say he needed a bucket as he felt sick and after some retching, Evan relaxed back onto the couch with a noticeable facial droop affecting the right side of his mouth and right eye. We got on the phone to clinic and during the five minute call, Evan's apparent stroke-like symptoms dissipated and a neurological check revealed nothing worrisome. I watched Evan like a hawk until clinic the next day when we found his hemoglobin to be 41. That was an all time low and we knew something was really wrong.
January 2009: Evan returns to school
7 months post-transplant
We were instructed by Evan's transplant doctor to begin a quick taper of the Tacrolimus and introduction of MMF (CellCept). Within a few weeks, Evan's need for red cell transfusions had slowed right down.
Evan had only one hospital admission post-transplant at the end of October 2008 for a fever of unknown origin. He had about three days of IV antibiotics and his last ever red cell transfusion, which took place on October 31, 2008.
Evan came off of CellCept in December 2008 and returned to school after the Christmas break. Several months later, Evan's doctor gave the OK to stop his prophylactic antibiotic and antiviral. Evan had his last ever dose of IVIG (which is, in essence, an immune system booster collected from thousands of blood donors) in February of 2009, just prior to leaving for his Make-A-Wish trip to Florida's Walt Disney World, where we celebrated Evan's full recovery.
Evan began his re-immunizations just after his one year BMT anniversary in July 2009. From that time on, Evan has had simple every day viral colds too numerous to count, and November 2009 was a particularly difficult month as Evan had to deal with the flu and shingles simultaneously. He had high fevers and felt pretty lousy but we were able to stay calm through all of that with the knowledge that these viral assaults are very common and are to be expected in the recovery from BMT. Evan continues to get the odd cold which results in a few days of fevers, but his wonderfully restored immune system takes it all in stride!
Next: Update and Reflections