It started out as a fever during flu season in January. My teenage son, Tim, complained of aches and pains — nothing out of the ordinary for a fever.
After four days with no change, we made an appointment to see his primary care doctor at the local clinic. Two days later, and all of his respiratory viral tests returned negative, but his fever continued.
By day seven his mom and I began to worry that he might have a serious bacterial infection, since he showed no flu symptoms. The three of us drove to the urgent care clinic in Sacramento, California where the doctor directed us to take him directly to the emergency room at the UC Davis Medical Center about 15 minutes away.
Biopsies, Tests, and Scans
The emergency room doctors quickly realized they did not have the resources to diagnose or treat Tim. The ER doctor, in consultation with the on-call pediatric team, admitted him to the hospital.
His fever continued over the next 10 days, despite continual full doses of acetaminophen every four to six hours. During that time he underwent numerous tests, biopsies, and scans, which showed lesions and nodules in his liver, right lung, and skin. His spleen and liver were swollen, and his blood counts were erratic.
We hit a particularly rough patch as Tim prepared for an ultrasound-guided liver biopsy. He couldn’t have any food or fluid by mouth after midnight. What was supposed to be an early morning procedure didn’t happen until mid-afternoon. By then Tim’s fever had spiked to over 105 degrees. He looked pale and despondent, desperately parched. His mom and I soaked wash towels in ice water in an attempt to cool him down. Mercifully, the surgical team rolled him to the operating area, and gave him anesthesia and a dose of acetaminophen through his intravenous line. I heaved a sigh of relief once they finally took him in for the procedure.
It didn’t take a doctor to know that something was terribly wrong — but what?
Cancer, Infection, or Autoimmune Disorder?
Various teams of doctors — including infectious disease, dermatology, general pediatrics, and immunology specialists — made their rounds. The pediatric hematology and oncology team took the lead. We were asked questions about sudden child deaths in the family, travel to foreign countries, or possible exposure to harmful substances or diseases. Every “no” answer led to more questions and confusion. One morning they informed us that Tim either had cancer, a systemic infection, or an autoimmune disorder. A thick fog fell over our family as we contemplated the seriousness of his condition.
During the entire hospitalization, my wife, Lori, stayed with Tim. My asthma, psoriasis, and eczema only worsened in the dry, heated, sterile air of the hospital. The stress of Tim’s care, lack of sleep, and driving 30 minutes from home to hospital took its toll as well. I took two full weeks off from work. My full-time job became taking care of my two daughters, helping out at the hospital, and transporting clothes, food, and other things my son had requested. Friends from our church helped with meals and shopping. We were in full crisis mode.
The doctors eventually eliminated cancer as a possible diagnosis, which was a huge relief. However, they could not rule out a systemic fungal infection. Meanwhile, they sent vials of Tim’s blood to Cincinnati Children’s Hospital for tests. Liver biopsy slides went to UCLA Medical Center. And an immunologist from the UC San Francisco Medical Center was consulted. Tim got anti-fungal infusions every night for good measure.
A Frightening Diagnosis: HLH
Almost three weeks after Tim’s fever started, an immunologist told us the multidisciplinary team had made a tentative diagnosis: hemophagocytic lymphohistiocystosis (HLH). He told my wife to Google HLH, and look at the more reputable medical websites. One of the first pages we visited was for Cincinnati Children’s Hospital’s HLH Center of Excellence. According to the hospital:
“Hemophagocytic lymphohistiocytosis (HLH) is a disorder of the immune system in which too many infection-fighting cells are produced and activated, causing damage to organs. While rare, HLH is rapidly fatal. However, with prompt and accurate diagnosis and timely treatment, a cure is possible.”
Some of the articles I read on HLH did not inspire confidence. Words like “life-threatening” and “fatal” rang in my head. Parents who lost children to HLH spoke of how they were told cancer is more treatable than HLH. Fortunately, our doctors told us that Tim had a relatively mild case. I began to believe them when, two days after starting a high-dose systemic steroid treatment, his fever finally broke.
After 18 days in the hospital, Tim finally got his discharge papers. The day before discharge he received his first chemotherapy infusion and a list of medications he’d need to take as part of his treatment plan. In addition to his medications, a home healthcare nurse would come to our house twice a week to draw blood. And we would need to drive Tim to the pediatric cancer center for infusions every Tuesday, and follow-up appointments with his hematological oncologist every two weeks.
‘One Day, One Step at a Time’
Tim’s journey is far from finished. His blood counts are slowly normalizing, thanks to a number of blood transfusions along the way. A recent CT scan showed amazing shrinkage of the nodules in his right lung. With this phase of treatment nearing an end, my question is, what’s next? What are the chances of HLH recurring? Will Tim develop leukemia or lymphoma from the treatments or the disease?
Our family has settled into a new normal. We freely use medical jargon and terms such as “fibrinogen” and “ANC “(absolute neutrophil count). Then there’s the twice-daily medication regimen. We watch to see if side effects such as tremors, bruising, low blood counts, hair loss, and weight gain improve or worsen. We wonder if life will ever be the same; four out of five members of our family have either a chronic or serious medical condition.
I’ll never forget what Tim’s pediatric nurse told me as I peppered her with questions: “One day, one step at a time, Mr. Chang.” Every parent on that floor no doubt needed to hear those words as we worried endlessly about the future. Her words remain with me to this day. If today is what God gives me with my son, then that is what I will cherish while I pray for many more days to come.