Happy Sunday, friends! But it’s not just any ordinary Sunday.
Today is PSC Awareness Day!
What’s PSC? you might ask.
Why does it need awareness-spreading?
Why is it on your blog about inflammatory bowel disease?
Is this the same PSC you mentioned in your last post?
Today, I’ll answer all these questions by explaining what PSC is and why it’s crucial for me to do my part and spread awareness about this disease.
What is PSC?
PSC is a rare, progressive, non-alcohol-related liver disease.
It stands for Primary Sclerosing Cholangitis, meaning that the bile ducts inside and outside the liver become inflamed. Like ulcerative colitis and Crohn’s Disease, PSC is chronic. Also like IBD, its cause is unknown.
So, why are inflamed bile ducts dangerous?
Well, your bile ducts transport bile (a fluid that helps digest food) from the liver to the intestines. When the ducts are inflamed, they can’t work properly; this leads to scarring and narrowing of the ducts, which can eventually lead to blockages.
Blockages trap bile inside the liver, which further progresses the disease toward fibrosis (early stages of scar tissue) and cirrhosis (severe scar tissue and impaired functions) of the liver. This leads to the need of a liver transplant.
Bile duct inflammation > narrowing > blockages > fibrosis > cirrhosis > liver transplant
Stages of PSC
There are four stages of PSC. I pulled the descriptions below from PSC Partners Seeking a Cure page called “Diagnosing PSC”:
Stage 1: A small amount of fibrosis limited primarily to regions in the liver called “portal areas”
Stage 2: Fibrosis begins to appear outside the portal areas. The strands of fibrosis are not yet connected to each other
Stage 3: Areas of fibrosis connecting to each other
Stage 4: Widespread, honeycomb like scarring known as cirrhosis.
***Each stage naturally progresses over the course of five years***
Are there treatments or a cure?
A liver transplant is the closest thing to a cure. A liver donor can either be deceased or alive. Fun fact: livers grow back!!
As far as treatments go, there are medications on the market, but they really only help alleviate symptoms rather than slow the progression of the disease.
What are the symptoms & related problems?
- Severe abdominal pain
- Right Upper Quadrant Pain: Known as RUQ pain, can range from dull to sharp pains beneath the rib cage where the liver sits
- Extreme fatigue
- Pruritus: Insatiable itching
- Jaundice: The yellowing of eyes and skin, due to accumulation of bile
- Chills & Fever: A sign of an infection in the bile ducts
- This requires immediate medical attention
- Heptic Encephalopathy: A condition associated with chronic liver disease
- A neurological disorder that alters your level of consciousness and causes extreme brain fog
- PSC patients are at an increased risk of developing a rare bile duct cancer called cholangiocarcinoma
- There are fewer than 50,000 people in the United States with PSC
- Over 700 patients are awaiting a liver transplant in the United States
Jenna, how does PSC relate to IBD?
- Over 75% of PSC patients have ulcerative colitis or Crohn’s Disease
- Only 3% of IBD patients have PSC
- So, most people with PSC have IBD, but only a small percent of people with IBD have PSC
Well, guys, it’s looking like I’m part of that 3%.
My GI has not officially named be a PSC patient because PSC is tricky. It’s an elusive disease. It sometimes presents symptoms, and other times it doesn’t. This makes it difficult to officially diagnose someone. There are medical reports suggesting that I do have the disease and that I do not have the disease.
Suggestions that I do have PSC
- My blood work results came back showing high liver enzymes
- My ultrasound showed high levels of fat in the liver
Suggestions that I may not have PSC
- On November 8th, 2017, I had an MCRP, which stands for Magnetic Resonance Cholangiopancreatography
- It’s a non-invasive imaging that analyzes your liver, gallbladder, bile ducts, and pancreas
- It searches for:
- Obstructions, blockages, or stones
- Swelling and inflammation
- Infections in the bile ducts, cholangitis
*My MRCP came back normal and unremarkable*
- I completed specialized blood work called a FibroSpect
- It tells you what stage liver disease you’re in
- But here’s the catch: It’s not definitive. It tells you one of two things:
- If you’re in Stage 1 OR in Stage 2, 3, or 4
- So if your results are the latter, you don’t know if you’re in Stage 2, 3, or 4
- If your results are the former, you don’t know if you don’t have the disease, or if you have it and are in stage
*My results came back saying I’d be in Stage 1, if I have the disease*
So, as you can see, half the results came back positive and have came back negative, making it very difficult to diagnose one way or the other.
I’m currently working with my doctor and discussing further procedures we can do to give us a definite answer.
But I want to use this blog post to raise awareness for those out there that definitely DO have PSC.
…For those suffering from nausea and pain daily.
…For those who can’t remember the purpose of a TV remote due to Heptic Encephalopathy.
…For those in Stage 4 who are on the liver transplant list and are fearful if a liver donor match will be found in time.
Ways to Get Involved
If this is something that tugs on your heartstrings, there are so many ways to get involved to help people like the ones described in the paragraph above.
PSC Partners Seeking a Cure is an amazing non-profit organization dedicated to researching for a cure, educating the community, and providing support for patients, their families, and their caregivers.
You can volunteer, fundraise, and even become an organ donor. What’s super cool is: Livers grow back! They’re a regenerative organ, so donating half your liver will save someone’s life, and yours will grow back, too. How cool is that?
There are hundreds of people on the liver transplant list, in need of a donor match. Some cases involve kids and teenagers being diagnosed at such a young age, that they need a liver transplant by age thirty.
Registering as a donor is super easy!
Or if you’re sold and ready to save a life and regrow your own liver baby inside of you, you can jump straight to the organ registration site here!
One last thing. Can you help me spread awareness? Maybe one share on social media will reach the right person who will become a life-saving donor.
Thank you for your help in saving lives like mine.
Have an amazing rest of your weekend, and see you on the blog soon!