A fifty year-old man entered the Emergency Department with severe abdominal pain. Three years earlier he had undergone liver transplantation from a donor on life support who had collapsed suddenly with subsequent brain death. A year after his transplant he had a blood clot on his lung after knee surgery. He was on blood thinners for three months. Shortly thereafter he developed a blood clot in a large vein in his pelvis resulting in massive leg swelling. He was advised to be on blood thinners for life. He was never evaluated as to a cause for these clots.
Ten days prior to this ED visit he was admitted to the hospital for rectal bleeding. His blood thinners were stopped and he was given Vitamin K as an antidote to Warfarin. Colonoscopy revealed bleeding hemorrhoids. The bleeding stopped spontaneously and he was discharged. The hospitalist physician caring for him on the day of discharge told him on his way out the door to check with his family doctor about resuming his blood thinners. He called that office and spoke with the receptionist who, after putting him on hold for two minutes, told him not to resume the blood thinners until he saw the doctor. After receiving Vitamin K his anticoagulation had been completely reversed and he had no blood thinner in his system. His family doctor did not know this.
Upon admission to the hospital he was found to have a blood clot in the main vein draining the small intestine (superior mesenteric vein). He was given blood thinners and then taken to surgery. Most of his small bowel was considered non-viable by the surgeon despite the rapid reinstitution of anticoagulants. He underwent removal of most of his small intestine and has been unable to take in enough calories to survive without developing life-threatening diarrhea. He will be on intravenous feedings for life at a cost of over a hundred thousand dollars per year.
He has filed suit against the hospitalist who discharged him and his primary care physician for the advice given over the phone. The suit is ongoing.
My Take on the Case
His attorney called me to ask my thoughts on the case. I was surprised that his tendency to clot (thrombophilia) had not been studied.
At my suggestion, further evaluation was conducted and revealed the likelihood that along with his transplanted liver our patient was given an inherited error of blood clotting, the Factor V Leiden mutation. Many clotting proteins are made in the liver. About five percent of otherwise normal people have a liver that makes an abnormal factor five, “Factor V Leiden” named for the university in the Netherlands where it was first described. That abnormal clotting factor causes thrombophilia resulting in occasional catastrophic events such as fatal pulmonary embolism or intestinal infarction, as seen herein. In the absence of prior clotting events and with a negative family history of abnormal clotting, it is highly likely that our patient acquired the Factor V Leiden mutation from his transplanted liver.
There have been other cases reported in the literature where this has happened, so the hypothesis is not far-fetched. The sudden collapse of the donor with subsequent brain damage is strong indirect evidence of a vascular calamity of some sort such as a pulmonary embolism or heart attack. Because of medical privacy issues, I was unable to learn more about his terminal illness.
What can we learn from this catastrophic event?
As with many medical disasters there was not just one error. First of all, liver transplant centers do not screen for inherited blood clotting disorders. Only one center in Japan has studied this issue and screens organ donors for thrombophilia routinely (Ogawa et al., Clinical Transplantation 25: 277-82, 2011). In their hands about five percent of potential donors have major clotting abnormalities; when they discover this the surgeons prepare the recipients with appropriate use of blood thinners and the transplantation surgery is done without clotting complications. Not stated in the article is whether those recipients were put on blood thinners for life, but they should have been. The number of donors with clotting disorders is likely higher in the US because Factor V Leiden, the most common inherited predisposition to clot, is not seen in ethnic Japanese.
In the US potential donors are screened for HIV, hepatitis and cancer (by history, not CT scans or the like) but not for other conditions routinely. Given that up to ten percent of the US population has thrombophilia of varying severity, clotting evaluation by American transplant centers of their donor pool is too casual. Furthermore, thrombophilic individuals would not necessarily be excluded from donation. The organ recipients could be put on blood thinners after the surgery. Most recipients would take this trade-off, since end-stage liver disease is otherwise a death sentence and donor livers are in short supply.
Our patient was never evaluated as to a cause of his hypercoagulability. Had his physicians known that he had the Factor V Leiden mutation their future attitude towards stopping his anticoagulation, in this case for a minor bleeding episode, would likely have been different.
Next, the hospitalist who discharged our patient told him to call his family doctor for instructions regarding anticoagulation. In the meantime, he could have been given injectable heparin for the several days it would have taken for Warfarin to work properly again. Arguably the hospitalist should have called the family doctor to make detailed plans for the hand-off of care. The family doctor, in turn, gave short shrift to the problem by refusing to deal with it without an office visit. He knew of the patient’s prior propensity to clot and failed to appreciate the risk associated with leaving his patient without the protection of blood thinners, even if he was not aware of the acquired Factor V mutation.
Most medical errors never make it to the courtroom. This case is instructive of what can go wrong for lack of attention to detail.
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Dr. James Stark is the founder of StarkOncology, where he practiced Oncology in Hampton Roads for thirty-four years. He is now a health-care consultant specializing in topics such as breast cancer, colon cancer, lung cancer, chemotherapy complications, and failure to screen. In addition to this new venture he continues to serve as Professor of Medicine at Eastern Virginia Medical School.