I’ve posted several times about primary biliary cirrhosis, a condition for which the only certain cure is a liver transplant, and recently by chance listened to two audio novels in which liver transplants function as plot devices. How weird is that? In all other ways, Peter James’ Dead Tomorrow and Abraham Verghese’s Cutting for Stone couldn’t be more different.
I knew going in that transplants would figure in Dead Tomorrow since the blurb alludes to human trafficking and the black market in organs. This novel is the fifth in James’s Brighton noir series featuring Detective Superintendent Roy Grace. Two plot strands alternate. Several teenagers’ bodies have been found buried at sea. They show no signs of violent death, but in each case high demand organs have been surgically removed. Meanwhile, a mother becomes increasingly desperate as her teenage daughter’s liver fails and no match has been found.
Sixteen-year-old Caitlin needs a transplant because she has primary sclerosing cholangitis (PSC), which after six years has caused cirrhosis and liver failure.
Primary biliary cirrhosis (PBC) and PSC have some things in common. Both illnesses affect the bile ducts, progress over a number of years, may be autoimmune conditions, and are incurable. But PBC affects only the small bile ducts inside the liver and the nearby liver cells, while PSC affects bile ducts inside and outside the liver. PSC is strongly associated with inflammatory bowel disease and 70 percent affected are middle aged men. PBC affects mostly women.
Now, it would be possible for a 10-year-old girl to be diagnosed with PSC. Possible, but highly unlikely. PSC is a rare disease to start with, and its incidence in children is 20% of that in adults. In the US, only 2% (223 of 11,322) of the liver transplants performed on children between 1988 and 2008 were because of PSC.
While James gives Caitlin convincing symptoms of liver failure: extreme fatigue, pruritus (itching that can’t be controlled), ascites (swollen belly), and encephalopathy, there are some aspects of her history that don’t add up. For example, Caitlin’s mother uses as an excuse for refusing to hospitalize her until a compatible organ might be found other prolonged stays Caitlin has endured, including one of a year’s duration at some point during the six years she’s been ill. But as best I can tell, nothing would be achieved by hospitalizing for a few days, let alone a year, a PSC patient during the early or middle stages of the illness.
Cutting for Stone
The liver transplant in Cutting for Stone came as a surprise to me. This richly imagined novel covers the first 30 or so years in the lives of twins Marion and Shiva Stone, born in 1954 in Addis Ababa, Ethiopia to an Indian nun, who dies in childbirth, and an English surgeon, who abandons the sons whose births have killed his beloved. Raised by an OB/GYN and her GP husband, the boys follow their biological and adoptive parents’ passion, a calling shared by their creator, Dr. Abraham Verghese, Professor for the Theory and Practice of Medicine at the Stanford University, and the author of two nonfiction books, most notably My Own Country, the story of his years spent treating AIDS patients in rural Tennessee.
Autopsies are commonplace elements of crime fiction, but I can think of no other novel that treats procedures on the living human body with the depth and skill of Verghese’s Cutting for Stone. The liver transplant is the culminating such episode in the novel and its climatic event.
Spoiler Alert: stop reading now if you don’t want to know what happens to Shiva and Marion Stone
Verghese’s Marion is just as unusually unlucky as Peter James’ Kaitlin, but once again, here the similarities end. Marion doesn’t have PSC but is one of the ~1% of those infected with hepatitis B who develop fulminant hepatitis and liver failure just weeks after contracting the infection. He is too sick even for the only thing that can save his life — a transplant — because he couldn’t cope with the immunosuppression necessary to prevent organ rejection.
Now, it turns out that Thomas Stone ended up in the US specializing in live donor liver transplant (LDLT) research, and Marion encounters his father during his medical internship. Father, adoptive mother, and twin Shiva are at Marion’s deathbed when Shiva announces that he wishes for their father to give Marion part of his liver. Because they are identical twins, immunosuppressants won’t be needed, nor will Marion have to wait for a donor liver to be found.
I think it would have been possible, just barely, for Marion to have received his living brother’s liver. One disdavantage of listening to a book rather than reading it is the difficulty of going back and verifying facts. Several reviews mention 1954 as date of the twins’ births, but I can’t find the date of Marion’s transplant.
Historically, the first LDLT was in November 1989, when Marion was 35, but this was from a mother to a toddler.
The first adult to adult LDLT was in 1994, and by early 2000, some 275 had been performed in the US.
And what about an LDLT without the need to administer immunosuppressants? Again, it is in the realm of the possible: A 2002 report in Gasteroenterology details “2 cases of LDLT between identical twins wherein perfect haploidentity has allowed these recipients to be transplanted without the need for immunosuppression.”