HLH: What Five Years and 700 Miles Means to Me
Tomorrow I’ll be starting a journey that sounds almost ridiculous. On September 12, I will join 20 others as we set off on a 700-mile bike ride, pedaling about 100 miles a day for seven days, all the way from Natchez, Mississippi, to Cincinnati, Ohio.
The ride is called 700 Miles to Hope. When I tell people “seven days, 700 miles,” it sounds physically grueling, mentally challenging, maybe a little crazy. But it’s nothing compared to what my daughter Maya went through six years ago. She’s the reason I’m riding. She and everyone else who has been touched by the immune deficiency disease called hemophagocytic lymphohistiocytosis, or HLH.
Maya had the genetic form of HLH, which is a life-threatening disease where the immune system attacks the body’s organs. My wife Mina and I now know that Maya had symptoms of the disease long before Maya was diagnosed. The path this devastating disease took us on started in our hometown of Torrance, California, and eventually led us to Ohio and the HLH Center of Excellence at Cincinnati Children’s.
A Warm Welcome
When we showed up in Cincinnati, another set of parents, Justin and Kristin Akin, were in the middle of two battles with HLH. They took precious time to welcome and help us. We became friends immediately. Justin and Kristin guided us through the mad world of bone marrow transplant, HLH, and patient advocacy. It was so incredibly helpful to be able to talk to other parents who understood what we were going through.
Justin and Kristin later lost both of their sons to HLH. They started The Matthew and Andrew Akin Foundation to raise awareness and funds in support of HLH. We helped along the way with donations, but we always wanted to do more.
Then five years ago, Justin and Kristin started 700 Miles to Hope in honor of families who have been affected by HLH — both those who have survived it, and those who haven’t. Mina and I have talked about it time and again. What can we do for people who have faced this disease, or who will face this disease? How can we help? How can we show our gratitude for the care Maya received?
I finally decided this year was the year for the bike ride. I chose to challenge myself to test my limits physically, the way Maya’s body tested her limits while she was battling HLH. That’s when I signed up for this ride. I wanted to help bring awareness to HLH, which is still not well known, even in the medical community. And I wanted to help the Akins raise money to support the core priorities of the HLH Center of Excellence, which are education, research, and family support.
Maya had a normal birth and early childhood, though it took her several days more than normal to resolve fevers. She was hospitalized at 10 months for a severe reaction to a virus. Her bone marrow was analyzed then and her oncologist said all was good. That is the conundrum of HLH — it is very hard to diagnose. It turns out that virus was a symptom that none of Maya’s doctors picked up on.
When she was 4 years old, Maya came down with what we thought was the flu. After her pediatrician felt her swollen spleen, she had us go straight to the emergency room. The next day she was transferred to the intensive care unit. Her organs quickly began to fail. Very soon the medical team determined Maya had HLH and she was put on a treatment protocol that required chemotherapy. As she stabilized it became clear: A bone marrow transplant would be Maya’s only chance at a cure.
When we researched marrow transplant centers, there were many factors, but being at the best was the most important to my wife and me. During our research, we found a table that showed Cincinnati Children’s had at least three times more HLH case experience than anyone else. We had to get Maya there. We will never forget our first discussion with Dr. Michael Jordan at Cincinnati Children’s. He was so engaged and immediately helpful.
We arrived in Cincinnati in December of 2009, and Maya soon began preparation for her marrow transplant. The transplant began in March of 2010. Shortly after it became clear that the transplant was failing. We were stunned. Maya struggled with new viruses with nearly no immune system. We endured so many painful procedures, long nights, volumes of medications, rashes, diarrhea, feeding tubes. It was very tough on Maya. It was tough on all of us, especially my wife who rarely left Maya’s room.
Fortunately, a second marrow donor was found. In May 2010, a second transplant was initiated, this time with much harsher chemotherapy and with that, potential for more severe complications. This time it was a success. We were beyond overjoyed. Six months later Maya was discharged and she began life as a survivor. She is now a five-year survivor, and we are so very grateful.
Maya just made the jump from elementary school to middle school. She is now in fifth grade and is enjoying making new friends in both band and soccer. Maya also takes swimming lessons and sings in a local children’s choir. In her free time, she loves to read, draw, paint, hang out with her brother Jaden, and play with friends in the neighborhood. It fills us with joy to watch her experience life and see how healthy she is today.
Maybe Justin picked this route and length to inspire people to overcome an enormous challenge, much like many families have to overcome thousands of challenges when their child is fighting HLH and struggling to survive a bone marrow transplant. Maybe witnessing these 21 riders pushing ourselves far beyond our comfort zones will inspire more people to learn about HLH and support the fundraising effort. What I hope for the ride is that it will continue to inspire excellence from the hospital that saved Maya, Cincinnati Children’s.
I would tell other families battling HLH, never to stop advocating for your child. This is one of the first lessons the Akin family taught us, that so much can be done for your child during these extremely fragile times by just watching, noting, and speaking up with the medical team. It takes all hands on deck to give your child the best chance at survival.
Our family was at the precipice a couple of times while Maya was very ill. For her to have come back so far, to the point that you can’t tell anything happened to her, it fills our hearts with pure joy. It has not been an easy road to travel, but it’s been well worth it. Maya is very proud of her survival. She wears it like a badge. Each year post transplant is another big celebration. In May of 2015 we celebrated her fifth year, and we look forward to celebrating many, many more.