Read Hannah's original patient story written by her mother, Kelly. Hannah provides an update on her life:
By Hannah Marsh
Our family’s journey with hemophagocytic lymphohistiocytosis (HLH) began in the summer of 2008 when I had a couple of nosebleeds. My mom didn’t think anything of it at the time – after all, lots of 5 year olds get nosebleeds. But when I woke up one morning with blood on my face, bruising and purple and red blotches all over my body, Mom knew something was wrong, and we went straight to the emergency room in our hometown of Sarasota, Florida.
Some of the initial tests showed that my platelet count was 16. "Normal” levels are between 150 and 400. That meant that my blood couldn’t clot, and that put me at risk for internal bleeding that could have been fatal. That same afternoon, my platelet count fell to 1. My liver was shutting down, and my spleen was enlarged. The medical team managed to get me stable, and did several biopsies of both my bone marrow and my liver. Within a week, I was diagnosed with hemophagocytic lymphohistiocytosis (HLH).
HLH is so rare that even some doctors haven’t heard of it. It primarily affects children — about one in 1.2 million. HLH originates in the bone marrow, the spongy tissue inside some bones that contains stem cells. The disease causes these cells to malfunction and destroy the body’s own red blood cells, white blood cells, and platelets. It can cause permanent damage to the spleen, liver, and central nervous system.
Extensive tests at the hospital showed that I likely had a genetic predisposition to HLH. Other children acquire this disease when their immune system malfunctions in response to a virus. Either way, HLH is fatal without appropriate treatment.
Finding the Best Treatment Available
There weren’t any specialists in our area who knew how to treat HLH, so our doctors in Florida relied on the expertise of Alexandra (Lisa) H. Filipovich, MD. Dr. Filipovich is director of the Immune Deficiency and Histiocytosis Program at Cincinnati Children’s Hospital Medical Center, and world-renowned for her work in treating kids with HLH. Through tests, phone calls and emails, she helped our doctors diagnose Hannah, but their lack of experience and training in caring for kids with HLH made my mom very anxious. They said it had been two years since they had seen another HLH patient.
Meanwhile, I wasn’t getting better. In fact, I was going into liver failure. One Saturday, after I had been in the hospital for almost three weeks, my mom decided to reach out to Dr. Filipovich on her own and she responded to her email within 45 minutes. That spoke volumes to my mom about Dr. Filipovich’s compassion and level of commitment. My mom arranged a three-day visit to Cincinnati to determine the best treatment plan. We didn’t know then that it would be almost six months before we were able to come home.
Treatment of Last Resort
After examining me and running lots of tests, Dr. Filipovich told us that my condition was so serious that the best course of action would be for me to stay at Cincinnati Children’s Hospital for treatment. The news was such a shock to all of us. After all, we had come to Cincinnati thinking that Dr. Filipovich would simply prescribe a treatment plan that our doctors in Florida could carry out. Instead, my mom would have to stay in Cincinnati while my dad went home to take care of my siblings, who at the time were 14, 12, and 4. But it was the only choice we had, and the very best option for me.
The first step would be for me to undergo eight weeks of chemotherapy to put the HLH into remission. But after seven weeks, it became obvious that my body wasn’t responding. In fact, my bone marrow was failing, and had to have blood and platelet transfusions around the clock, and a bone marrow transplant became my only chance for survival. The search for a donor began immediately, as no one in my family was a match for me.
Finally, Good News!
Within a few weeks, our medical team found an unrelated 26-year-old male donor who was a perfect match for me! There are no words to express our family’s gratitude for the life-saving option of a bone marrow transplant, and our gratitude toward that selfless and courageous young man. Without the transplant, I would not be able to tell my story today.
The transplant took place on September 26, 2008, a day before my 6th birthday, and it went very well. The new cells immediately began to produce healthy blood cells and platelets. Three weeks later, I was discharged, but we had to stay in the area for three more months so that Dr. Filipovich and her staff could continue to monitor my recovery. Social workers at the hospital helped us find a furnished apartment nearby and we stayed in Cincinnati until January 2009.
Before I tell you what happened next, I want to take a minute to say that the doctors, nurses, therapists, hospital school teachers and volunteers at Cincinnati Children’s are simply amazing! A hospital over a thousand miles from home is the last place you want to be for three months, but the staff made it as comfortable and homey as they possibly could. We are all still in awe of Dr. Filipovich — she is soft-spoken and compassionate, and her knowledge is second to none. I felt very safe in her hands. Child life specialists brought me board games and movies to help pass the time, and everyone on staff played my favorite game, Sorry!, with me at some point. Hospital School was also a great way for me to stay on top of my reading and math skills, so that I could keep up with everything.
Being discharged from the hospital was an exciting milestone, but the months we spent in the apartment were very hard. My immune system was still compromised, and I really couldn’t go anywhere except to the hospital, for fear that I’d get sick because of it. Finally, on Jan. 8, 2009, after countless check-ups and tests, Dr. Filipovich released me to go home! Stepping into our house and being together under the same roof with my family again, for the first time in six months, was a moment we will never forget.
The New Normal
I had to remain in near-isolation for over a year while my new cells continued to engraft — no school, no play dates, no movies or dinners out. Within two years from transplant, my central line was removed, and I was finally able to return to school. There were many changes that had to be made, but life returned back to normal within a few years. I still have annual checkups, and lots of tests to ensure that I remain healthy. I have also become very involved in learning more about HLH and transplant survivorship. I try and be a source of support for other kids and teens going through a similar situation, and I have become very involved with our local blood bank and marrow donation organizations by being their spokesperson for special events and talking about the value of the gift of life that blood and marrow donations bring.
10 Years Later
I will be turning 17 years old this year and will be a Junior in high school in the fall. I recently celebrated my 10 year transplant anniversary, and I live a completely normal life again. I am very involved in dance and musical theater, and I’m looking into a career in the medical field. I have begun volunteering at the local hospital here in my hometown of Sarasota, Florida. I fully credit Cincinnati Children’s Hospital for being instrumental in saving my life and putting me on the path of survivorship. The future is bright!