Some disease processes shout at you- dramatic symptoms bring you immediately to medical care and treatment. However other unhealthy biological processes are more subtle and are likely to go undetected while damage is being done. For Tina, it was a routine blood test for a life insurance physical which revealed elevated liver enzymes that led down a long road to diagnoses. What was wrong? A rare disease: Primary Biliary Cholangistis.
“Let’s watch them” – said the doctor. And together they watched them rise with time. Tina’s advice to others? “If they’re high, find out why”. Sometimes Advanced PBC can lead to the necessity of a liver transplant and there are never enough donated organs, with most going to people with NASH and HEP C.
What is PBC and who does it affect? PBC is thought to be an autoimmune disease which causes the bile ducts to become damaged. It has a highly variable course with some people progressing rapidly and some remaining quite stable for years. Symptoms include itching, fatigue, accumulation of white spots under the skin, etc. They are not related to the stage of the disease. Some individuals with mild, early stage disease may face distressing complaints, while other may be close to needing a liver transplant, and find it hard to believe that something so serious is going on. Approximately 90% of those diagnosed are women. It is auto-immune disease that may or may not be triggered by an environmental exposure. Left untreated advanced liver disease and osteoporosis may result.
For about 20 years there has only been one medication for PBC – ursodeoxycholic acid (URSO). More recently, the FDA approved obeticholic acid for those who are non-responders or only partially respond to URSO. Obeticholic acid- trade name Ocaliva™, has a different mechanism of action and helps reduce inflammation. It is produced by Intercept Pharmaceuticals. It is used I conjunction with URSO (UDCA).
Tina was on URSO for 12 years, and it brought two of the three major liver enzymes down. She was one of the first in the country to add Ocaliva to her treatment plan and it brought her other number into line, with it dropping more than 25% in the first three months.
How has PBC affected Tina’s life? While this disease led her to retire early from a stressful position in finance and wealth management, Tina lives a full life of service. Tina became an advocate for PBC helping to define the disease process and educate others on PBC. She works in partnership with her liver specialists, even coordinating speakers for a national conference on PBC.
But her health advocacy goes beyond her own condition. She is a baby “cuddler “in the Neonatal ICU, helping tiny premature infants to thrive. She helps bewildered and anxious hospital patrons find their way to the correct offices of a complex hospital system that can bring adults to tears. She has headed the hospital auxiliary and helped to raise funds. She is able to travel overseas and does, PBC or not. Being diagnosed with a serious chronic disease is alarming, but Tina has not let it define her.
For the newly diagnosed she recommends three sources of information:
- NIH: just the facts
- American Liver Foundation: information in detail, research regarding potential new developments for treatment.
- The PBC’s web page and Facebook group: advice for daily living and support