The doctors at UC Davis Medical Center in Sacramento puzzled over Tim’s diagnosis for a full week. By then, the mysterious disease he was fighting had impacted multiple organs and threatened his life. My wife, Lori, and I were terrified. On an evening walk around the hospital, we contemplated losing our son. Lori asked me if our marriage would survive. I assured her we’d make it, not knowing if I’d survive. Soon after, the pediatrician pulled us aside to tell us Tim had a rare immune disorder: hemophagocytic lymphohistiocytosis (HLH). The HLH treatment protocol of chemotherapy and systemic steroids worked quickly to lower Tim’s fever and calm the raging immune system response overwhelming his body. After he was discharged from the hospital, we followed up for a few months with at-home nursing care and treatments at the pediatric oncology infusion center. Tim’s recovery, though, took much longer than we expected. He continued to feel too fatigued to go back to his regular life. Eventually he left his public high school for a private school where he could receive individual attention. Lori and I thank God that Tim made a full recovery while he was attending his new school. It’s amazing to look back at how he found friends, captained the soccer team his senior year, and graduated with plans to attend community college locally. This is his third year in college. I see Tim’s story in a different light a year into the pandemic. Just as my family faced potential loss from an aggressive, sudden-onset illness, countless others have walked through a similar dark valley with COVID-19. I lament alongside all those devastated by the trauma of what we’ve collectively experienced. The silver lining of the COVID-19 lockdowns in California was having Tim move back home last spring. I’ve enjoyed the time with him, including playing frisbee and video games, grilling, and talking about his future. I cherish every moment all the more knowing we almost lost him to HLH six years ago. Below is my original blog post about Tim’s illness. RELATED: Facing My Anxieties During the Coronavirus Crisis After four days with no change, we made an appointment to see his primary care doctor at the local clinic. Two days later, with all his respiratory viral tests returned negative, 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.
Biopsies, Tests, and Scans
The emergency room doctors quickly realized they didn’t 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 midafternoon. By then Tim’s fever had spiked to over 105 degrees. He looked pale and despondent, desperately parched. His mom and I soaked towels in ice water in an attempt to cool him down. Finally, 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 took him in for the procedure at last. 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 specialists in infectious disease, dermatology, general pediatrics, and immunology — 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 the 30-minute drive from home to hospital took a toll as well. RELATED: Overcoming a Psoriasis Flare I asked for two 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. But 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 antifungal 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 lymphohistiocytosis, or 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. We read: “Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the immune system causing uncontrolled inflammation that damages organs of the body leading to rapid death. A cure is possible but an accurate and quick diagnosis needs to be made.” Some of the other 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 acquired case, which most likely was triggered by a viral infection. I began to believe them when, two days after Tim started a high-dose systemic steroid treatment, his fever finally broke. After 18 days in the hospital, Tim received his discharge paperwork. The day before, he’d received his first chemotherapy infusion and a list of medications. The plan was for a home healthcare nurse to come to our house twice a week to draw blood. We would also need to drive Tim to the pediatric cancer center at UC Davis Medical Center for infusions every Tuesday, and to follow-up appointments with his pediatric hematologist oncologist every two weeks.
‘One Day, One Step at a Time’
Tim’s journey was far from finished. His blood counts were slowly normalizing, thanks to a number of blood transfusions along the way. A CT scan showed amazing shrinkage of the nodules in his right lung. But I couldn’t help but wonder, what was next? What were the chances of HLH recurring? Would Tim develop leukemia or lymphoma from the treatments or the disease? Our family settled into a new routine. We freely used medical jargon and terms such as “fibrinogen” and “ANC” (absolute neutrophil count). Then there was the twice-daily medication regimen. We watched to see if side effects such as tremors, bruising, low blood counts, hair loss, and weight gain would improve or worsen. We considered whether life would ever be the same: Including Tim’s illness, four out of five members of our family now had 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 that. Those words remain with me. 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. You can read more about my experiences in my blog for Everyday Health and on my website.
