How to treat recurrent jaundice after multiple ERCP stents?

Question

Hello doctor,

I am seeking guidance regarding my health condition. I developed jaundice seven years back with clay-colored stool, and my bilirubin level reached a maximum of 50 mg/dl.

I underwent ERCP stenting six years back, which brought my bilirubin down to 21 mg/dl. Since then, I have had multiple stent exchanges, and a Fibroscan indicated a mean of 6.9. I was diagnosed with protein C deficiency.

I had a PSRS with splenectomy. I also underwent several attempts at ERCP with stent exchanges. I was on a stent-free trial after that, maintaining bilirubin levels between 2 to 5 mg/dl. However, I recently developed jaundice again, accompanied by fever. An ERCP was performed, and it showed partial atrophy of the left lobe with hypertrophy of the caudate lobe, mild intrahepatic biliary radical (IHBR) changes, and a distended gallbladder. There was an occlusion of the distal SMA, MPV, and intrahepatic portal vein branches, leading to collateral circulation.

Another ERCP and stent exchange were performed, cultures showed E. coli, and I received upgraded antibiotics. I also faced signs of shock, which were managed, and after resuscitation, I improved. At present, I am afebrile, able to walk, eating normally, and in a stable condition. However, my bilirubin has risen above 50 mg/dl again, with some concerning lab results.

I had a liver biopsy two years ago, and I would like to know what durable treatment options are available for me. Is there a possibility for improvement in my condition?

Please guide.

Answer 1

Hello,

Welcome to icliniq.com.

I have read your query and understand your concerns.

Based on the information provided, you have a complex case of biliary tract disease characterized by multiple interventions and complications.

The current symptoms of jaundice, fever, and signs of shock raise concerns about the possibility of an infection or sepsis.

The liver biopsy report indicates chronic cholestasis, inflammation, and fibrosis (excessive, chronic buildup of scar tissue (collagen) in organs, resulting from persistent injury, inflammation, or dysfunctional healing), which are likely associated with the underlying biliary tract disease.

Additionally, it suggests the possibility of primary biliary cholangitis (PBC), a chronic autoimmune liver disease that predominantly affects women. The presence of Protein C deficiency and Protein S deficiency could further complicate the liver disease and heighten the risk of bleeding during subsequent interventions.

The decision for durable procedures or treatments will depend on several factors, including the underlying cause of the biliary tract disease, the severity of liver damage, and your overall health and preferences.

Various treatment options may be considered, such as liver transplantation, which, although a potentially curative option for end-stage liver disease, is a major surgical procedure that entails significant risks and requires careful evaluation of candidates.

Biliary drainage procedures may also be considered to alleviate symptoms and prevent further liver damage by bypassing or removing obstructed bile ducts, such as

Choledochoduodenostomy (a surgical procedure that creates a direct, side-to-side connection between the common bile duct and the duodenum to bypass obstructions and allow bile to drain directly into the small intestine).
Hepaticojejunostomy (a surgical procedure that creates a new connection (anastomosis) between the hepatic bile duct and the jejunum (small intestine) to bypass obstructions or repair damage, allowing bile to drain directly from the liver into the digestive tract).
Cholangiojejunostomy (a surgical procedure that creates a direct connection between the bile duct (cholangio-) and the jejunum (jejunum), typically used to bypass obstructions or repair injuries in the bile duct system).

While these procedures can provide relief, they come with their own set of risks and complications, including bleeding, infection, and bile leakage.

Medications can help manage symptoms such as pruritus, fatigue, and osteoporosis, and may include antiviral or immunosuppressive therapies for underlying infections or autoimmune diseases.

Although they can improve the quality of life and slow disease progression, these treatments may have side effects and interactions with other medications.

Additionally, lifestyle modifications, such as dietary changes to manage malnutrition or steatorrhea, along with exercise and stress management techniques, can improve health and well-being and enhance outcomes when combined with medical or surgical treatments.

Ultimately, deciding on durable procedures or treatments will necessitate a multidisciplinary approach involving hepatologists, gastroenterologists, surgeons, infectious disease specialists, and other healthcare professionals experienced in managing complicated cases of biliary tract disease.

I hope this clarifies the situation.

If you have any questions or need further assistance, please do not hesitate to ask.

Thank you.