Since I began keeping this journal, I’ve received a number of messages via  email and Facebook from other parents who have a child with HLH. Each time I find myself broken up by the experience of hearing about their child, so closely can I relate to what they are going or have gone through, yet I value each chance I get to hear from other parents.

I received another such message recently from Jun, and with his permission I’m posting the story he wrote to me about his son, Jeremy. I’ve edited it slightly for length and clarity, but the words are otherwise his. I greatly appreciate his and his wife’s willingness to let me share this, and his kind wishes and prayers for Zoe.

Jeremy’s Story

Our second baby was born on March 4, 2010 via a emergency c section. We named him Jeremy Daniel.

At the time he was born he was only 7 months old, they had to do a c-section because they noticed that his growth had slowed, and the amniotic fluid was decreasing. When Jeremy was born he had a full body rash that looked much like a German Measles rash. My wife was never sick throughout her pregnancy, except for the expected nausea and vomiting.

At first the doctors thought it was some sort of an infection. They did every test they could think of to rule it out, and all of the neonatologists and pediatricians were baffled with his condition. In addition to the rash, his liver and spleen were enlarged, and his lab levels were all low. He required a blood and platelet transfusion nearly every day.

By the third day after the c-section, his belly had become so big that they had to intubate him to assist his breathing. He had xrays, MRI, CT scans and ECG, all of which came back negative.

We were happy, and at the same time sad. Happy because his tests were negative, sad because they still couldn’t diagnose him.

Finally after a week, on March 11th, they came to a working diagnosis. A Hematologist/Oncologist took a look at the biopsy of the rash, and he noticed some Histiocytes. Based on that, and the symptoms, signs and the timing, he was almost certain it was FHL, the familial and primary form of HLH.

My wife and I are both nurses, but in our careers we had never seen this disease. The Hematologist explained the process to us and the treatment options. When the genetic markers were sent off to CCH, he personally handed them off to the FedEx guy. He was very optimistic throughout, and said he would expect Jeremy to improve after the first or second dose of chemotherapy.

We were working with several doctors during this process. The doctor who diagnosed Jeremy said this was only his 3rd case of HLH, and of them, only one survived. A second doctor, who had more experience, said this was her sixth case, but again, only one survived. I suspect they may have been talking about the same patient.
She was very hopeful though. She said as years have gone by, they’ve come to understand more about the disease and the treatment option. Now they know when to be aggressive, and when to be gentle.

We started HLA typing to determine who would be the most likely donor for the bone marrow transplant, and the stories from the other nurses kept us going. Being a nurse has it’s advantages and disadvantages. We hear more from the other staff, but at the same time we weren’t able to attend Jeremy’s first chemotherapy session because we were both sick.

The night of his first chemo session, he was still in the NICU. This was a first for them, and they didn’t have a certified chemo nurse. They asked a PICU certified nurse to oversee his treatment, but when they found out how small and how sick Jeremy was, all of them refused. Their reason was that they were trained to give chemo in pediatrics, not neonatal.

An older nurse was kind enough to volunteer to oversee his treatment finally. She even changed her work schedule to ensure that she would be working when his chemo doses were due. We met her the day after his first session, and we hugged and thanked her. It was then that we found out from her that she was herself a cancer survivor.

As time went on, Jeremy wasn’t improving as expected. After his thrid dose of chemo, the doctor informed us that all of his organs were failing, with the exception of his lungs. He even suggested weaning him off the ventilator. The determination was that he was unable to tolerate the chemotherapy. They stopped his Cyclosporine at that time, but continued his Dexamethasone and Etoposide to continue fighting the disease.

Jeremy’s doctor told us he was giving him a week or two to get better, and then would arrange a family meeting to discuss other options. He felt then that if he doesn’t get better, he wouldn’t get better at all.

The night of his fourth chemo session, we noticed Jeremy’s eyes were open for hours. This was the first time that had happened, normally he would have them open for a few minutes, then fall asleep again. He was looking at us, eyes turning side to side, looking at Mommy and Daddy. We noticed then that he was unable to move his arms or legs, and that he wouldn’t make a face or move when pinched. We talked to him, singing him songs. We asked him to give us a sign if he wanted to give up, or if he wanted to go on with his treatment.

After the chemo was complete, Jeremy’s heart rate and oxygen levels started to go down. They continued to drop until they were at zero.

My wife and I were in the room as it happened. They started compressions, but when the doctor yelled for a medication to restart his heart, I found the courage to say “Stop”, crying as I did so. This was the sign we had been looking for. My wife was in agreement — no more pain, no more suffering.

Jeremy Daniel gained his angel wings on March 24th, 2010 at 1:48am. He had a tough fight, and we always wonder had he been born full term if the outcome might have been different. We continue to grieve, and our first born child, who is now almost 2 years old, is our inspiration to keep going.

– Jun