I just DoJo's press conference on
Facebook Live (starts around the 30 minute mark). He certainly does play it down as no big deal, and gives no reason to believe that it's having a significant detrimental effect on him... but for the first time, he mentions the name of his condition,
primary sclerosing cholangitis. It actually sounds VERY serious. Hopefully he's one of the "many" patients who are asymptomatic, but primary symptoms include:
- Pruritus (i.e., itching), which may be severe.
- Severe fatigue (a non-specific symptom often present in liver disease)
- Jaundice and scleral icterus (i.e., yellowing of the white of the eyes)
- Episodes of acute cholangitis (infection within the bile ducts), which can be life-threatening[4]
- Dark urine due to excess conjugated bilirubin, which is water-soluble and excreted by the kidneys (i.e. choluria)
- Malabsorption, especially of fat, and steatorrhea (fatty stool), due to an inadequate amount of bile reaching the small intestine, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.
- Hepatomegaly (i.e., enlarged liver), due to portal hypertension caused by compression of portal veins by the proximate sclerosed intrahepatic bile ducts, and right upper quadrant abdominal pain
- Portal hypertension, a complication of cirrhosis, which can manifest with esophageal and parastomal varices[5] as well as hepatic encephalopathy (mental status alteration/disturbance caused by liver dysfunction and shunting of blood away from the scarred liver; such that ammonia detoxification is reduced with concomitant encephalopathy)
When DoJo says that his "enzyme levels are high", he's probably referring to this primary basis for clinical diagnosis:
- serum alkaline phosphatase (ALP) > 1.5x the upper limit of normal
It really gets serious when you get into the Management and Prognosis sections:
- No pharmacologic treatment has been approved by the U.S. Food and Drug Administration for PSC.
- Liver transplantation is the only proven long-term treatment of PSC
- Although there is no curative treatment, several clinical trials are underway that aim to slow progression of this liver disease.
- Estimated median survival from diagnosis until liver transplant or PSC-related death is 21.3 years.
Hopefully his case is as mild as, by all indications, it seems to be!
...dave