PBC is the oddest disorder

The story of Anwen

For years I was told I might have a fatty liver. That I needed to lose weight. That I needed to eat less meath, Then eat more meat. Then some assumed I was using drugs or alcohol. But it was always just that there was the liver level that was just not right. But no diagnosis ws forthcoming and i didnt have any symptoms. 

Then I was given a round of hormones for my endometriosis. 

When my skin and eyes turned yellow and I was itchy and tired- I just assumed that it was a side effect of a treatment for my endometriosis, since that was what the nurses had said it was- a bad reaction, maybe an allergy. I didn’t feel like a major organ was in failure. I tried to convince my GP who had run the tests that I could just come in for testing as needed for the next few days. She insisted I go to the ER. I comprimised and told I would go, but after i finished my work. 6 hours in the ER later I was admitted as in acute liver failure and 3 days later I was told I was in chronic liver failure. As a 35 year old non-smoker, non-drinker, this was a complete shock.  

A few weeks, some imaging studies, blood tests and biopsies later I had PBC. A year later it was confirmed. The medicine helped remove some of the bile, my skin started to get closer to normal, some days my old makeup is even the right shade, but it seems like my other symptoms- fatigue, difficulty digesting, pain, dehydration- all got worse. Then my hepatolgist and pathologist and radioligist coudn’t agree on the disease progression. So I was told I was at stage 1-2, maybe stage 3. There are only 4 stages.

I moved to seek more expert care, then a plague decended and suddenly life became even harder. I am waiting for my current care team to decide which tests they can run safely. 

My disease makes my life very difficult, particularly when you balance a full time job, kids and life. I don’t share my disease with everyone, particularly work. I am afraid of being out of work beause then I couldn’t pay for my healthcare, but I also would miss a huge part of my life. Also, as a person with a rare disease will tell you, you have to explain the disease to people when you reveal your diagnosis. Itching, fatigue, pain, bone density loss, dehydration and dizziness from the medications is such a vague and seemingly very low-key list. But when it’s constant it takes a toll that is hard to describe to poeple. Then ithey learn the progression, then you have to emptionally carry people through. It’s exhausting and when your disease already makes you so tired, that is harder.

Then there is the low-level dread of knowing that I will likely need a liver transplant, and before that happens I will get worse. It’s difficult to get family and friends to understand that the worse will happen by inches and the transplant will only happen when my body gets to a nadir. I wish the disease was better understood, but especially the the mechanisms of the symptoms. perhaps manament of those would help wquality of life and if blood tests didn’t have to be drawn all the time, if there was another to track the disease, that would also help.

I learned this through both my endo treatment and my PBC diagnosis- demand more. Demand information, demand care, demand to be taken seriously. If a doctor doesn’t- get a new doctor. An informed patient is one who can take better care and thus receives better care.