Primary Sclerosing Cholangitis (PSC) impacts every aspect of a patient’s life. This patient will be referred to as R.G. R.G. is a 62-year-old Caucasian male. He was diagnosed ten years ago with Primary Sclerosing Cholangitis. R.G. lives with his wife. They have three boys and a girl who died in a car accident 11 years ago. The boys are all healthy and attend college or have graduated and now live on their own. R.G. works in a college library an hour and a half away from his home and has for the past 40 years. Besides Primary Sclerosing Cholangitis, he has Ulcerative Colitis and hypertension. He is otherwise healthy. The medications he takes include Mesalamine, Furosemide, Omeprazole, Rifaximin, and Laculose. R.G. has had a very active, and healthy life. During the summer, he was always found out on his boat, water skiing or sailing competitively. Now he cannot partake in any of these activities, or even many simple life activities due to severe impairment of his strength and energy. This essay will discuss the pathophysiology behind Primary Sclerosing Cholangitis, the medications R.G. takes, how he is emotionally coping with his disease and physically managing it, and how his disease impacts his life.
Hypertension is when a person has a systolic blood pressure of 140 mm Hg or more and a diastolic blood pressure of 90 mg Hg or more. This occurs when the cardiac output (CO) or systemic vascular resistance (SVR) are increased. Treatment and management include antihypertensive medications, nutrition therapy, increased activity, weight loss, stress management, cessation of tobacco use and moderation of alcohol intake (Lewis, 2017).
“PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction. The fibrosis causes diffuse narrowing of the intrahepatic and extrahepatic bile ducts, and the resulting persistent biliary stasis leads to hepatic cirrhosis and a poor prognosis” (Nakazawa, 2014, p. 3246). A breakdown of the disease’s name best explains what it causes: ‘primary’- meaning that it is not caused by something else (unknown etiology), ‘Sclerosing’- meaning hardening of tissue, and ‘Cholangitis’- meaning inflammation of the bile ducts. This inflammation occurs in the intra-hepatic and extra-hepatic ducts. Fibrosis occurs due to hardening and necrosis of the duct. Other sections that do not undergo fibrosis dilate, causing an irregular lumen of the bile duct. Because of the necrosis of the duct cells, bile can leak into the blood and cause increased levels of conjugated bilirubin and liver enzymes in the blood. The treatment for Primary Sclerosing Cholangitis focuses on reducing biliary complications, screening for bile duct and colorectal cancer (Lewis, 2017), and if the disease is advanced a liver transplant (Nazazawa, 2014). This, however, is not a cure because “posttransplant disease recurrence is not uncommon” (Razzak, 2016, p. 158).
Primary Sclerosing Cholangitis is affected by both environmental and genetic factors. It progresses gradually and has many different outcomes (Lazaridis, 2016). “PSC has a significant association with inflammatory bowel disease (IBD) with an estimated 70%-80% of affected individuals suffering concomitant ulcerative colitis (UC) or Crohn׳s disease” (Razzak, 2016, p. 158). IBD is autoimmune, meaning there is an immune reaction that targets the person’s cells. IBD can be caused by “environmental factors, genetic predisposition and alterations in immune function. Environmental factors such as diet, exposure to air pollution, stress and smoking” (Lewis, 2017, p. 944). Ulcerative Colitis affects the rectum and spreads into the colon and is an inflammatory disease. It causes bloody diarrhea and abdominal pain that subsides after a few days or can remain for months. Relapse occurs after an asymptomatic period (Porth, 2015). “The goal of treatment of IBD are to rest the bowel, control the inflammation, combat infection, correct malnutrition, alleviate stress, provide symptomatic relief and improve quality of life” (Lewis, 2017, p. 946). Surgical treatment includes a total proctocolectomy with ileal pouch/anal anastomosis: a two-surgery procedure ending with the anal sphincter controlling defecation, or total proctocolectomy with permanent ileostomy: a surgery that leaves the patient with a permanent ileostomy (Lewis, 2017).
R.G. must take many medications to manage his diagnosis and stay healthy. R.G. takes Mesalamine four 1.2 g tab P.O. daily with a meal. It is an anti-inflammatory to prevent irritable bowel syndrome. It’s an active metabolite of sulfasalazine and acts topically by hindering prostaglandin creation in the colon. Common side effects include Headache, dizziness, fever, fatigue, abdominal cramps, diarrhea, nausea, vomiting, constipation, hemorrhage, nephrotoxicity, and a skin rash (Karch, 2017). The desired response is to decrease diarrhea and abdominal pain, return to normal bowel pattern, and to preserve remission from IBS (Vallerand, 2017). Nursing implications include monitoring: renal function studies, blood cell counts, patients who are sensitive to sulfites for a hypersensitive reaction, and signs and symptoms of intolerance syndrome often like an uncreative colitis exacerbation. Important teaching topics include that the tablet must be swallowed whole, not chewed or crushed, avoid taking with antacids, report all adverse effects they experience and stop using the drug if a fever or rash occurs. Patient should remove the foil wrapping before insertion and follow the directions on how to appropriately use retention enemas (Karch, 2017).
R.G. takes Furosemide 40mg tab P.O daily in the morning. It is an antihypertension medication. It works to inhibit “sodium and chloride reabsorption at the proximal and distal tubules and the ascending loop of Henle.” Common side effects include transient deafness, tinnitus, abdominal discomfort and pain, diarrhea, constipation, pancreatitis, frequent urination, agranulocytosis, aplastic anemia, leukopenia, thrombocytopenia, jaundice, hypokalemia, hypocalcemia, hypomagnesemia, muscle spasms and cramps, epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiform (Karch, 2017). The desired response is to decrease: edema, abdominal girth, weight, blood pressure and increase urine output (Vallerand, 2017). Important nursing implications are monitoring: weight, blood pressure, pulse, hydration status, electrolytes, fluid intake and output, glucose level, uric acid level and symptoms of severe urine retention. Important teaching for patients taking this medication are: to take it in the morning before meals, the possible need for potassium or magnesium supplements, slowly standing to avoid dizziness, limiting alcohol intake, report all adverse reactions (especially tinnitus, severe abdominal pain, sore throat or fever) and to avoid direct sunlight (Karch, 2017).
R.G. takes Omeprazole 20 mg tab per day one hour before breakfast. It is used as a short-term treatment for duodenal ulcers. Its action is binding to hydrogen-potassium adnosinetriphosphates, inhibiting proton pumps, suppressing gastric acid secretions. Common adverse reactions include headache, abdominal pain, nausea, vomiting, fever, and flu like symptoms (Karch, 2017). The desired response is to decrease abdominal pain, prevent gastric irritation and bleeding, reduce GERD symptoms and erosive esophagitis (Vallerand, 2017). Nursing implications include to monitor for increased risk of CDAD, monitor for malabsorption of vitamin B12 in long-term use, monitor for osteoporosis, and monitor for hypomagnesia. Important teaching for patients beginning the drug use is that the tablet must be swallowed whole, not crushed, opened or chewed, to take the drug a minimum of one hour before meals, the risk of becoming dizzy, that it may be taken up to four times a day to reach the full drug effect, and to report signs and symptoms of low magnesium (Karch, 2017).
R.G. takes Rifaximin 550 mg tab P.O. b.i.d. It is an antibiotic that kills E. coli and prevents bacterial RNA synthesis. The action is to bind to beta-subunits for bacterial DNA-dependent RNA polymers, inhibiting bacterial RNA synthesis and kills E. coli. Common side effects include dizziness, fatigue, headache, peripheral edema, ascites, abdominal pain, constipation, defecation urgency, nausea, and vomiting (Karch, 2017). The desired response is to decrease severity of travelers’ diarrhea, reduce the risk of overt hepatic encephalopathy reoccurrence, and reduce symptoms of IBS diarrhea (Vallerand, 2017). Important nursing implications include monitoring for overgrowth of nonsusceptible organisms and stopping taking the medication if diagnosed with Campylobacter jejuni, Shiglla, or Salmonella, has bloody stool, diarrhea with fever, or diarrhea caused by anything other than E. coli. Importing teaching includes that the drug can be taken with or without food, explaining the importance of continuing to take all prescribed drugs even if they begin to feel better, to contact their prescriber if diarrhea worsens, they have bloody stool, or a fever, and that it is to treat diarrhea caused by contaminated foods and beverages and not for other infection types (Karch, 2017).
R.G. also takes Laculose 20 g or 2 tablespoons P.O. 3-5 times daily. It is used to prevent constipation. The action is to cause an osmotic effect in the colon, resulting distention promotes peristalsis it can be used to decrease ammonia. Adverse effects include nausea, vomiting, diarrhea, and abdominal cramps (Karch, 2017). The desired response is to promote passage of soft, formed stool, decreasing confusion, apathy, irritation and improving mental status (Vallerand, 2017). Nursing implications include monitoring sodium level, potassium level, mental status, and replacing lost fluid. Important teaching topics include reporting bothersome reactions and diarrhea, showing proper use and mixing of the drug, and to avoid taking this drug with other laxatives (Karch, 2017).
Below is a table showing a cost analysis of the above-mentioned medications. The prices represent the amount of a month’s supply of medication from Walmart and the price after Medicare, the insurance R.G. has. Each drug has a lower generic price, and all except Rifaximan are covered by R.G.’s insurance. Without Medicare, R.G. would not be able to afford all of his medications even if he did buy them only in their generic form (Prescription, 2017).
Initially, R.G. had few symptoms. As the disease progressed, R.G. experienced increasing exhaustion. He also has a loss of strength, cramping of his extremities, including his legs, feet and hands, and a loss of appetite in the past three months. He reports that he is nowhere near as productive as he was five years ago. R.G believes that having a set daily schedule benefits his wellbeing, supporting his ability to stay awake. Luckily at work, he has very supportive coworkers who understand his falling asleep during meetings and taking notes for him. Adapting to his new very sedentary lifestyle has been very hard because he was an active man, involved in Boy Scouts, his community, and his job, he now feels unproductive and unable to partake in activities that have been a source of pleasure and fulfillment for him. He shows signs of depression, which are understandable due to a bleak prospect. He had a chance at a liver transplant, drove to Cleveland Clinic, prepared for surgery, only to be told there was something wrong with the donor’s liver. He continues to wait, continuing to try to manage his symptoms and remain as engaged in his life as possible.