A new study on patients with cystic fibrosis (CF) who have had abnormal bleeding from ruptured blood vessels in the esophagus (variceal hemorrhage) as a result of liver disease found that transplant may not be indicated if there are no other indications of advanced liver disease.
The results of this study appear in the December 2005 issue of Liver Transplantation, the official journal of the American Association for the Study of Liver Diseases (AASLD) and the International Liver Transplantation Society (ILTS). The journal is published on behalf of the societies by John Wiley & Sons, Inc. and is available online via Wiley InterScience (http://www.interscience.wiley.com/journal/livertransplantation).
Cystic fibrosis, a genetic disease that affects the respiratory and digestive systems, can sometimes affect the liver. The most common complications are due to blocked blood flow in the liver (portal hypertension) which can result in variceal hemorrhage. Variceal hemorrhages normally carry a high death rate and so CF patients with this condition have generally been considered to be poor candidates for liver transplants, although this group has previously not been well studied.
Led by David Westaby of the Department of Gastroenterology at the Chelsea and Westminster Hospital in London, researchers studied a group of 18 CF patients from a pool of 1,154 at a referral center at the Royal Brompton Hospital in London from 1981 onward. The 18 patients had each suffered at least one episode of variceal bleeding. Almost all of them (17) had a liver appearance indicative of cirrhosis when an ultrasound was performed, although the majority either had no clinical indications of liver disease (other than the bleeding) or suffered only temporarily from ascites (an accumulation of fluid in the abdomen). These patients were treated with a variety of non-transplant measures, such as band ligation therapy. The median survival for the group was 8.4 years, which was not significantly shorter than the control group of 36 CF patients who had not suffered variceal hemorrhage, and longer than normally seen in patients without CF who have had variceal bleeding.
The study represents the largest series of adult CF patients with variceal bleeding reported to date. The authors hypothesize that since liver disease in CF patients may involve damaged bile ducts, "variceal bleeding in patients with cystic fibrosis does not necessarily indicate a significant deficit of hepatocellular function," the authors state. This is important because the poor prognosis seen after variceal bleeding in patients with other liver diseases does not apply to CF patients, although this type of bleeding has been used as a basis for non-eligibility for lung transplantation. The authors suggest that the natural history of cystic fibrosis liver disease is such that if liver function is good, a patient's survival depends on the progression of respiratory disease. They conclude: "Our data suggest that a history of variceal bleeding in the absence of decompensated cirrhosis can usually be managed without liver replacement."
In an accompanying editorial in the same issue, Maureen Jonas, M.D., of the Harvard Medical School notes that because patients with CF are living longer, recognition of liver disease in these patients seems to be increasing and that its management depends on clinical indications. When cirrhosis is present, liver transplant is an option, since it may lead to an improvement in both pulmonary function and nutritional status, but the author notes that the timing of the transplant requires careful thought. Some studies have shown favorable outcomes for liver transplants performed early due to complications of portal hypertension (such as variceal bleeding), which was presumably contributing to decreased lung function. The current study examines more closely the need for liver transplants in these patients. "There is no doubt that liver transplantation has a role in the management of end-stage liver disease due to CF," Dr. Jonas concludes. "However, the data presented by Gooding et al. suggest that perhaps it should be limited to those patients who have evidence of true hepatocellular dysfunction and failure, and not simply for the management of portal hypertension."
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