A sick baby gets a liver transplant overcoming multiple hurdles during emerging covid times
How soon after transfer can you do the liver transplant? Thus, came the query from the treating hepatologist of baby N from Malaysia. Within 24 hours in an emergency, perhaps a little longer for international patients depending on the paperwork and permissions from the embassy, was our prompt assurance.
Baby N had been jaundiced since her early neonatal period and when it didn’t resolve over the next few weeks, detailed evaluations led to a diagnosis of biliary atresia. She had the most severe syndromic form with dextrocardia, heterotaxy with midline liver, malrotation and polysplenia. A Kasai portoenterostomy was performed on day 55 of life along with corrective surgery for the malrotation. Despite the timely Kasai procedure that entails a hepaticojejunostomy with the aim to establish biliary drainage, her disease continued to progress rapidly and she had decompensated liver disease by the time she was 6 months of age and had an episode of upper GI bleed due to advanced portal hypertension. She needed a liver transplant, the only definitive curative treatment for her condition that remains the leading indication for pediatric liver transplant in most parts of the world.
We were sent the clinical details and with a donor identified, by end February all preparations to travel to us had been made by the family. Tickets booked and ready to fly but baby N’s route was on a different tangent. She developed severe pneumonia and encephalopathy landing her into intensive care. Sir Henri Bismuth, one of the pioneers of liver transplant had very poignantly and precisely said “The worst complication of liver transplant is to die without receiving liver transplant”. Every life deserves a chance that innumerable babies who die in similar situations never get. As though refusing the flight to Heaven, baby N improved and once stabilized was put on a flight to India only after the precondition of an urgent transplant was accepted by the receiving team.
The covid pandemic had erupted and was increasingly engulfing the world when baby N reached India in the second week of March. Travel restrictions were not yet in place but quarantine requirements for selected nationals were mandatory. Malaysia was not amongst them. The dawn after the night they landed was to throw all plans haywire with the advisory coming from the Govt of India to quarantine for 2 weeks all travelers from Malaysia as well, as quite a few coronavirus cases had been reported in the country.
The family was devastated, the medical team in deep dilemma. Baby N was presently stable but could deteriorate any time considering her end stage liver failure. To deny a life saving surgery for fear of an infection that she may never be carrying in the first place, how ethical was that? Nevertheless, she could very well be in the incubation period and what outcome could be expected if she received a transplant and then manifested covid disease? How could her donor, her mother, a healthy beautiful young woman be subjected to a surgery that would place her at grave risk if she was infected? How could the medical staff be exposed to the risk? Covid testing was under government regulation and not yet available in private hospitals. It was only offered to symptomatic individuals in select government hospitals as testing kits were limited. Request for covid testing for baby N and her parents was denied by the requisite authorities as most surgeries had been put on hold. Also, a negative test would still not forego the mandated quarantine. The disease was very new to India, very little was known of the virus and treatments were just being researched. Fears were high, the thin line between being brave and being reckless was obscure. The transplant would have to wait. Would baby N be a covid mortality regardless of her covid status?
The time bought was used as an opportunity to build her nutritional status and she inched closer to her new life as she continued to remain stable and gain weight with each passing day despite her alarmingly high bilirubin levels of about 45 mg/dl. As she neared the end of 2 weeks, covid testing also became available to us and both mother and daughter tested negative. Her transplant was finally scheduled to happen, or was it?
Exactly 14 days after her arrival, the effect of the dangerously high bilirubin levels on the SA node of a dextrocardiac heart led her to develop heart block. With heart rates between 40-45/minute, she was again in intensive care with medical therapies instituted for the heart block. Doses were hiked and titrated with no response. Would a plasmapheresis to reduce the bilirubin help? With its attendant risks in this scenario, the option was not chosen. She would require a pacemaker. Temporary pacing was started before she was taken up for the complicated transplant surgery considering her abnormal anatomy. Perhaps as a concession for all her trials and tribulations, the transplant surgery was accomplished without any major intraoperative and postoperative complications. Death had in fact chosen to quarantine itself from baby N and let her bloom into a lovely radiant little girl, a marvel of human resilience, modern medicine and divine grace.