From our anonymous insider…
Those of us who passed the last block’s exams on our first try are back from a week of vacation. Wildflower Willow, a free-spirited outdoors enthusiast from Oregon and founder of our school’s wilderness club, went on a three-night solo backpacking trip. Pinterest Penelope spent the week in Banff with her family. Gigolo Giorgio crashed his parents’ trip to Europe. “I was planning to go home, but my father had a last-minute business trip to London and Brussels. He called me up to cancel my trip. I asked if I could come along for the ride. He reluctantly agreed. I think he had been excited to spend quality time with my mother.”
Jane and I skipped the Monday morning lecture, so our GI pathology week begins with a new 8-person “small group.” “You look too happy to be medical students” remarked a gentleman in a wheelchair as Jane and I take the elevator to the third floor. The 57-year-old retired orthopedist is our facilitator. Five years ago he had a bike accident that left him paralyzed from the waist down. “I expect a comprehensive differential. Don’t just blurt out syndromes. Tell me why you are thinking that. Do not expect to be leaving early with me.” Geezer George, a 32-year-old Boston native who is our oldest classmate, commented “It is refreshing to have someone hold us to high standards. Most of the facilitators have been more casual.”
Our group discussed celiac disease and common GI pathogens. Celiac disease, an autoimmune disease due to hypersensitivity reaction against gliadin (component of gluten), is most associated with Northern European ancestry. Type-A Anita: “White people have to pay somehow.” The immune reaction produces IgA that frequently cross-reacts with proteins in the dermal papillae (junction of dermis and epidermis) creating the characteristic dermatitis herpetiformis (grouped fluid-filled sacs, named after the similar appearance to a herpes outbreak). The IgA antibodies do not lead to GI pathology, but serve as a useful biomarker for diagnosis.
Geezer George brought up a norovirus outbreak while discussing common GI pathogens: “I was at ground zero in Boston. I lived across the street from the Chipotle where half our school got lunch.” (the illness was traced to a sick employee and it was unrelated to an earlier E. coli outbreak at Chipotle) A student replied, “Chipotle gets a bad wrap… no pun intended. You do not have an increased risk of getting a GI bug at Chipotle compared to any other restaurant, just so many people get meals there. It’s like the Toyota brake scandal.” A student described getting a Staphylococcus aureus enteritis characterized by profuse vomiting and diarrhea: “It’s like you don’t know whether to sit on the toilet or to stand next to it.”
A 45-year-old gastroenterologist specializing in hepatology (liver) gave Tuesday’s lecture on GI pathology: gastroesophageal reflux disease (GERD), peptic ulcer disease, Boerhaave syndrome, and inflammatory bowel disease (IBD).
She explained that “36 – 77 percent of Americans experience GERD throughout their life. The severity of the symptoms do not correlate with the severity of GERD. Patients are not faking the pain. Some just have more sensitive mucosa than others. Avoid caffeine, smoking and late night meals.” She detailed how the use of proton pump inhibitors (PPI), such as Prilosec (omeprazole) has gone through cycles. “Patients and providers have become skeptical about the use of PPI. The problem is that we overprescribed them for some time and they started to be linked to everything without evidence. I had a patient post-MI [heart attack] with a peptic ulcer. The CCU staff took him off the PPI out of fear of reinfarction. [Once off the PPI] The ulcer bled so much he required transfusion. The link has been proven false.”
Peptic ulcer disease, ulcers that form in the stomach and duodenum (proximal small intestine), is associated with nonsteroidal anti-inflammatory (NSAID; aspirin and ibuprofen are examples) use and chronic Helicobacter pylori infection. “20 million people take NSAIDs daily including 70 percent of people over 65. As long as people use NSAIDs, I have a job.” Why do doctors ask if the abdominal pain gets better or worse after eating? “Gastric ulcers worsen after eating. Eating stimulates acid production in the stomach. Duodenal ulcers become better after eating. Eating causes release of bicarbonate in the duodenum that neutralizes irritants.”
Boerhaave syndrome, a condition where intense vomiting leads to esophageal rupture, is caused by binge drinking. “Chronic vomiting such as in alcoholics and bulimics typically does not rupture through the esophagus,” she explained. Boerhaave syndrome is associated with a 35 percent mortality, “the most of any GI perforation.” Gigolo Giorgio: “I’m surprised that none of my college friends got Boerhaave syndrome.”
“Do not get IBD [inflammatory bowel disease] confused with IBS [irritable bowel syndrome]. Much different. IBS comes and goes and is not as severe as IBD,” she explained. The two most common IBD conditions are Crohn disease and ulcerative colitis (UC).
Nervous Nancy has Crohn disease. “My doctor is convinced I am Jewish. I keep telling him I am not. Infliximab [tnf-alpha inhibitor] has been a Godsend. I usually let my roommate inject it into me every three weeks. It’s like ripping a band-aid – easier if someone else does it quickly. He was trying to impress his new female friend by winding up before stabbing me. He ejected prematurely, wasting half the dose. I am freaking out. That’s like $4,000. My insurance won’t give me another prescription so I am going to try to make the next few doses last longer. I can already feel my hands and legs swelling and getting hot.”
Crohn disease, a transmural (entire thickness of gut tube) granulomatous inflammation of the GI system, usually occurs in the ileum [terminal small intestine]. Because Crohn Disease is transmural inflammation it can lead to performation and fistualization (connection between two tubes). If the colon ruptures it can create a connection to the bladder, called a colovesical fistula. Gigolo Giorgio: “Could you imagine peeing feces?”
Our patient case is Rebecca, a high-school swimmer who began seeing our gastroenterologist/hepatologist lecturer when she was 15. Rebecca presented for bloody diarrhea with mucous, fatigue, and a seven-month history of crampy abdominal pain. Over the preceding week she has experienced sharp right-upper quadrant (RUQ) pain. On physical exam, Rebecca appears pale with an enlarged liver palpable six centimeters below the costal margin and a palpable spleen. No scleral icterus (yellowing of the sclera) is noted. CBC shows pancytopenia (low red and white blood cell count) with a normocytic anemia (normal red blood cells, but not enough of them) and high reticulocyte count. Stool sample tests positive for white blood cells, red blood cells, but negative for pathogens. After a colonoscopy, Rebecca is diagnosed with ulcerative colitis.
What is causing her enlarged liver and spleen? Ten percent of patients with UC develop primary sclerosing cholangitis (PSC). PSC is an inflammatory reaction that causes fibrosis of the biliary tree connecting the liver to the duodenum. Over time this causes incurable cirrhosis (hardening of the liver), which clogs portal circulation of blood returning to the liver.
“The treatment of PSC is liver transplantation. That is how serious a disease it is. Liver transplant is not even a cure,” explains her doctor (our lecturer). Rebecca is placed on the liver transplant list.
Patients on the liver transplant list are ranked according to the Model for End-Stage Liver Disease (MELD) score, which predicts three-month mortality among liver failure patients based on three lab values: creatinine (kidney function), bilirubin (liver’s ability to breakdown and excrete heme), and the international normalized ratio (liver’s ability to synthesize clotting factors). Rebecca was at 12 out of 40. “PSC patients are screwed over by the MELD score,” explained our hepatologist. “Their lab values do not reflect their deterioration. I told Rebecca’s family that she would not make it to the expected donation time.” Her family and doctor petitioned the UNOS (United Network for Organ Sharing) to no avail. Pinterest Penelope whispered, “This story reminds me of Denny from Grey’s Anatomy losing the heart transplant by 17 seconds.”
Her mother described searching for a living donor. Live donor liver transplant (LDLT) is a procedure where a liver section from a living donor is removed for transplantation. The liver is able to regrow to normal function over time. LVLT has several ethical dilemmas. Who gives consent for a pediatric donor? A cousin or uncle who matches may experience immense family pressure to donate, compounded by the fact that many liver transplants require immediate decisions. Pinterest Penelope whispered again, “This is just like Grey’s Anatomy! Remember that episode where the son of an abusive father has to decide to give him part of his liver?” Rebecca’s real-life situation was more serious, but less dramatic. There was no abusive father and nobody in her immediate family was a match.
Rebecca waited three years for a liver while enduring serious complications such as hyperammonemia (high serum ammonia causing mental status changes). One evening she presented to the ED for severe hematemesis (vomiting blood). The dilated veins in her esophagus ruptured. (Esophageal hemorrhage is the most frequent cause of death in liver cirrhosis patients.) Rebecca underwent banding endoscopy (put rubber bands around the veins) to stop the bleeding. After these episodes, the family and doctor petitioned UNOS, who increased her MELD score.
Rebecca underwent a domino liver transplant the summer before her freshman year of college . The first domino was a cadaver (dead person) whose liver is transplanted into a patient with a genetic disease such as familial amyloidotic polyneuropathy (FAP) or Maple Syrup Urine Disease (MSUD). The second domino is the liver removed from that patient, which can be installed in Rebecca’s body and then function normally. We saw a picture of the domino family smiling next to each other: the widowed wife of the cadaveric donor, the mother holding an 8-year-old daughter with MSUD, and Rebecca.
Rebecca’s PSC returned three years later. Her mother said, “We knew the system better the second time around. We listed at a transplant center that did not have a national reputation and in a state with high donation rates.” Rebecca showed us her scars. The scar from the first transplant was roughly 4 inches long on her right side. The scar from the second liver transplant went across her entire abdomen. Her transplanted liver had enlarged to cover her spleen. The extensive fibrosis also adhered parts of her liver to the diaphragm making it difficult to remove. As a result, she experienced pain for several months requiring high dose IV opioid painkillers and neurontin. Two years out she is dealing with opioid tolerance and withdrawal symptoms as she tapers off. Rebecca, now a rising senior at college studying chemistry, plans to return to school after a semester break. “I hope to get back in the water next month. It symbolizes, sort of, returning to normalcy.”
After Rebecca and her mother left, a student asked the hepatologist, “Given that there is such a long waiting list for transplants, what are your thoughts on a single patient receiving two livers?” She passionately responded: “Rebecca deserved this liver. I just came back from the AASLD [American Association for the Study of Liver Diseases] conference. UNOS just approved liver transplants for alcoholics who are three months sober [Hepatitis C from IV drug use is another common reason for requiring a transplant]. I have never met someone who is more motivated and wants to be a productive member of society. Throughout her first transplant recovery she kept going to college. Can you imagine the drive that requires? A lot of potential liver transplant patients just sit at home on disability. What do they do after the transplant. They continue to sit at home on disability. No, she deserved this second liver.”
For each of the next six weeks we will write a two-page single-spaced ethics essay. “I am really excited about doing this ethics course with you,” explained our
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