Today was a difficult day. Ronan was beginning to show all the signs of Tumor Lysis Syndrome (TLS) -- eg, rising phosphate, creatinine and BUN (urea) levels, increased nausea and lethargy -- despite being on 2x maintenance fluids to encourage the kidneys to naturally filter the blood. Rasburicase (an even stronger med for removing Uric Acid) was subbed in yesterday for the Allopurinol he was previously taking, but his body was still struggling. The PICU team did an excellent job of escalating quickly to the Nephrology team, let by Dr. Scott Sutherland. The team was well aware of the risk of Acute Renal Failure (ARF), also known as Acute Kidney Injury (AKI) associated with early treatment of Burkitt Lymphoma given the number of / size of the masses that break down quickly from chemo, leaving a bunch of "gunk in the blood" in their wake. This is a "superhuman task" the doctors said, that would challenge even the healthiest of kidneys. Aminophylline (to assist his kidney function) and Sevelamer (a phosphate binder) were added to his rapidly growing list of medications. Labs were "Q6" (every 6 hours) and the team would make a decision by the EOD if we needed to prepare for dialysis to assist the kidneys in fighting TLS.
Additionally, a recent chest X-Ray showed pleural effusions on both sides of his lungs now, resulting in further respiratory impairment. His heart rate was in the 70-80s v. the targeted 40s. The team decided to put him on high flow oxygen to get his oxygen level up from 92-94% the low flow oxygen was getting him to a target of >95%. Note here: Part of the collateral damage from the chemo is to kill (in addition to cancer cells) the red blood cells with the hemoglobin that oxygenates the blood.
The third complication he was struggling with was cardiac arrhythmia. There was a "couplet" episode of tachycardia overnight that was concerning. Some of the medications Ronan was receiving were associated with cardiac arrhythmias (eg, steroids with chemo and Ativan for nausea) but the team wanted to ensure it was not something physical caused by his mediastinal mass. An ECHO was ordered to further evaluation his heart form and function.
As the day went on, with all the fluids being pumped in him to support his kidney function, Ronan was developing pedal edema (swelling in his feet) and walking was becoming increasingly uncomfortable. Ironically, the best thing for this was walking, so we rallied for a mid-afternoon adventure out to the PICU porch. We couldn't have asked for a nicer day. It was high 70s, perfect in the shaded porch with a light breeze. Ronan lay down on the outside loveseat, enjoying the view and snacking on a fresh fruit platter from the LCPH Room Service Menu, followed by a delicious milkshake delivered by his dad from The Melt. Ronan is a champion pill taker -- and that has never been a more valuable skill than it was today. "Ready, aim, fire!" he would declare emphatically before summoning the courage to swallow each of the two 1 inch Sevelamer pills he had to take BID (twice daily). And, never a child to complain, despite this being a miserable day for Ronan, whenever a doctor or nurse would ask how he was, he would put on a cheerful face and respond, "Good enough." OMG what a trooper.
We had a long conversation that afternoon with Dr. Marks from Hematology-Oncology, who was on the Children's Oncology Group (COG) committee that set the treatment protocols for Burkitt Lymphoma. We discussed the nature of Burkitts -- how it is characterized by the translocation of the MYC gene and how aggressive it is, with the expectation that it all began just a few months ago. But the good news is it is super treatable by chemo with a >95% cure rate (ie, never comes back after first chemo treatment). Lucky timing for us too as the COG recently added Rituximab to the treatment protocol, based on a study released in the New England Journal of Medicine June of 2020. Dr. Marks provided an update from Pathology, who determined that there are insufficient cells in the CSF to run a Flow Cytometry test to confirm cancer in the CSF but it is visually comparing the cells in the CSF to the tumor cells from the lymph nodes (the "old school" way) and said they look very similar. So they suspect it is Phase IV, but they are not done with their report. When we asked for a second opinion on the Pathology, I asked about specialists in Burkitts, since it is such a rare pediatric cancer. Dr. Marks took the action to find the Pathologist on the COG committee who reviewed all the questionable slides for that study. She also recommended, given Ronan's complications, to delay the LPs (lumbar punctures for IT chemo) that are typical in the Pre-Phase until he is more stable / there is less risk for the anesthesia required for those procedures. She assured us that it would not effect the efficacy of treatment. I was impressed by her knowledge, her empathetic communication style, and her supportive response to our request for a second opinion on the key Pathology question.
That evening, after 6 days in intensive care, I took a much needed break and went for a run on the Stanford Campus. Turning the corner on Palm Drive with a view of Hoover Tower, I reflected on how much my perspective had changed over the ~20 years since I was an undergrad. I remember feeling so intimidated -- the tall buildings, the incredibly smart faculty, the students who were all Type A overachievers like myself, and all the pressure that came with it as an undergrad. As I looked around campus on my run, all I felt was the purest, deepest gratitude that I believe any person has ever felt. This was the institution that would save my son's life. Stanford, my alma mater to whom I already owe so much, has now fully captured my heart and soul.
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