Hi, I’m Peri Pakroo, Jila’s mom. About a month before her 7th birthday, in late April 2013, Jila was diagnosed with Severe Aplastic Anemia. A month later she had not responded to immunosuppressive therapy, and the doctors said she would likely need a bone marrow transplant within the next month. Thankfully in the following two weeks her condition improved, and continued to improve until her counts hit normal in late 2013. It is now March 2015 and she is still doing well, still on immunosuppressive medication (cyclosporine) but otherwise leading a totally normal life.
We are of course hoping she never relapses, but relapses do happen and it could mean that Jila would need a bone marrow transplant—but a bone marrow match has not been found for Jila. This is likely because she is an unusual ethnic mix of primarily Irish and Persian (aka Iranian). Sadly, I’ve learned that non-European white populations and people of mixed ethnic backgrounds are much less likely to find bone marrow donor matches.
Now that Jila is in remission and our lives are stable, I want to be proactive and do everything I can do to help get more people to join the bone marrow registry, both for Jila and for other patients in need. That’s why I created this site: To try to reach as many people as possible — especially people of Irish-Persian heritage — and encourage them to join a bone marrow donor registry: here in the US, the main one is the Be The Match registry. Even if Jila ends up not needing a transplant I would love to help bring more diversity into the international bone marrow registries and potentially help others having trouble finding a donor match.
On a fine summer day at the end of June 2012 I noticed Jila’s eyes looked yellow. That’s when this all started. She had just turned 6 and been a strong, healthy kid all those 6 years. I called our family doctor in Albuquerque (where we live), who said it sounded like a symptom of hepatitis, so they did a blood test and sure enough she had fairly severe hepatitis. That’s when I learned that hepatitis simply means the liver is inflamed. Typically you’ll test positive for a specific virus like Hep A, Hep B, Hep C, etc. and be diagnosed with a specific type of hepatitis — but Jila tested negative for everything. So it was a very weird mystery. She was also jaundiced, meaning her skin and eyes were turning yellow from an accumulation of bilirubin in the blood which is normally filtered out by the liver.
We were referred to a pediatric gastroenterology (GI) specialist who said the hepatitis was likely caused by an unknown virus that would resolve on its own, so we watched it all summer. Her inflammation levels — measured by levels of enzymes in the blood that are released by the liver when it is inflamed — slowly went down, but so slowly the specialist was concerned. Then in October the enzyme levels started going up again and in November they were spiking — plus, her platelets (a specific type of blood cell) started dropping.
I liked Jila’s pediatric GI specialist well enough but her office was was terrible at calling me back (in fact the only way to contact her office was to leave a voicemail message; they had no live phone receptionist) and communication was very frustrating in general, and as Jila’s numbers went south I waited three whole days for a referral to a hematologist. So I Googled “best pediatric gastroenterologist” and Cincinnati Children’s hospital came up near the top. My husband/Jila’s dad Turtle O’Toole’s mom and brother live in Cincinnati, so it looked like a good option for us. I emailed about five docs at Cincinnati Children’s whose profiles looked appropriate, along with a spreadsheet I’d been keeping of all Jila’s lab numbers, and they all emailed me back within eight hours saying they’d see her! Dr. John Bucuvalas was the first to contact me and he scheduled a phone meeting the following morning with himself and his team. After that conversation Turtle and I quickly made the decision to transfer Jila’s care to Dr. Bucuvalas. We flew to Cincinnati a few days later, on Thanksgiving Day 2012.
She was admitted for about six days to start her on steroids, and slowly her liver numbers improved. So did her blood counts (platelets, white cells, etc.) — but at that point it wasn’t her blood we were worried about, it was all about her liver. The improvement was fairly slow and we stayed in Cincinnati till early January with many visits and blood tests each week to carefully track her progress. Back in Albuquerque, she continued to get weekly blood tests and her numbers continued to improve. By March the liver numbers were practically normal so they started to wean her off the steroids.
Then her platelets started dropping again. We had an appointment for a follow-up liver and bone marrow biopsy on April 23, and the docs said we could just stick with that. So we came back, they did the biopsy and found that her bone marrow was severely depleted, down to about 5-10%. The diagnosis was Severe Aplastic Anemia, which means her bone marrow is failing and not producing enough blood cells. There’s something called Hepatitis-Associated Aplastic Anemia which fits her pattern. They don’t know what causes it but it starts with unexplained hepatitis, then a few months later whatever evil agent was attacking the liver moves on to the bone marrow.
Statistically the best treatment for this condition is bone marrow transplant from an HLA-matched sibling. Jila’s brother Jasper doesn’t match. Next on the list is ATG/cyclosporine therapy, which is an intense 10-day inpatient drug regime that kills off a lot of blood cells where they believe the bad agent is lurking; this is supposed to allow the remaining stem cells in the bone marrow to regenerate healthy cells. If ATG/cyclosporine fails the next option is a bone marrow transplant from an unrelated (but HLA-matched) donor.
We started ATG at the end of April, were discharged about 10 days later, and her counts did not improve for a whole month. They don’t really consider it a success or failure until two to three months have passed, but they typically hope to see some upward trends by 40 days or so. Since her numbers were so stuck they did a repeat bone marrow biopsy right around day 30, which showed even lower cellularity (below 5%) in her marrow than from the previous biopsy. The day they gave us those results at the end of May, they said we’d have to move forward with a bone marrow transplant unless she showed a “convincing” response to the ATG therapy (as in, a clear upward trend in her blood counts) within the next 1-2 weeks.
Two days later her counts started improving robustly. Yesterday, (June 11, 2013) after 10+ days of continuous improvement our doc said we could be discharged within a day or so if the upward trend continued. He also said the transplant is off the table … for now. Jila’s counts need to continually improve or we’ll be back to the transplant plan.
Update: June 12, 2013
Today (June 12, 2013), we had a dip in Jila’s counts, the first dip in 11 days. So we were not discharged from the hospital as was hoped. All along, our doctor has said that dips are normal, and today he said not to feel negative; not to feel the sky is falling. So I’m hoping for the best tomorrow.
Another Update: March 2015
It’s March 2015 and Jila has been in remission since her counts hit normal in late 2013. 🙂 She is still on cyclosporine and we hope she will be able to wean off the medication eventually.