If you or a loved one is newly diagnosed with primary sclerosing cholangitis. . . . . . you’re not alone. We’re together in the fight, whatever it takes.
Because PSC is a rare disease and because it can be related to other diseases, many of us have been misdiagnosed or undiagnosed, often for a number of years. Many of us have had the disease for a while before it was identified because the symptoms can be minor for some patients. Now that you know what you have, you want information and answers. Becoming educated is the number one way to start dealing with the disease.
Survival time for those who have PSC is increasing each year. There is hope. Thousands of people with PSC live active and full lives. Find out how they do it; read their stories; join a support group; attend our annual conference.
You can download our educational brochures for your caregiver and your medical team. Check them out, Living with PSC, Diagnosing PSC, PSC Medications, and Pediatric PSC are all available by clicking here.
I’ve taken good health for granted. I've always been very active, participating in numerous sports (and fortunate to maintain those activities over the years). I was diagnosed with ulcerative colitis (UC) in late 1977. With medication, my UC has been well controlled since then. In April of 2000, I was diagnosed with PSC. I’ll never forget the words I heard in the ERCP recovery room (or the shockingly dispassionate delivery): “You have primary sclerosing cholangitis and you’re going to need a liver transplant.” Oh great. Now what? Take Charge.
You are the patient. You are the doctor’s customer. You are in command. Although PSC can be a life-threatening disease, one of the world’s foremost PSC researchers has frequently stated: “You're more likely to die with PSC than from PSC."
There are people who have had PSC for 30+ years and are still doing well. You can help yourself!
PSC is incurable—that’s the bad news. How many of us will die from PSC? It’s far more likely that many of us will die from other causes. The CDC lists the following leading causes of death (US data, 2009 final):
One of our members greatly feared dying of PSC and died of something else. One of the early members fought PSC tooth and nail and died after being hit by a car when he was training for a master’s bicycle race (!). Watch out for that bus!
I have a family history of heart disease (my father died at 48) and diabetes (grandmother). I have asthma (respiratory). I work on a farm (accidents). Am I worried about PSC? Sure, but to be fair, shouldn’t I also worry about driving to work, baling hay, canoeing in the wilderness, and diet? Oh yeah – and bicycling!
Have you ever seen those cartoons where Wile E. Coyote is running along as fast as he can and suddenly he’s suspended in mid-air and ready to drop a good couple thousand feet? Well, that’s what a diagnosis of PSC can feel like; you’re running along and then the bottom all but drops out.
The good news is it doesn’t have to be that way. I am sorry to tell you that some of what you read is true. PSC is rare. PSC currently has no cure. PSC is now a part of your daily life. However, some of what you read is simply untrue or outdated. PSC doesn’t mean you will drop dead tomorrow. It doesn’t mean that you only have ten years to live, like the gods are checking off your life on some giant calendar in the sky. And it doesn’t mean that you will immediately turn yellow and start resembling your favorite Chiquita banana. In fact, there are ways to help you turn this Pretty Sucky Condition into a Personal Survival Code.
For many, the worst part of diagnosis is the feeling of isolation that comes along with it. Many doctors and medical staff have never heard of PSC and have no idea how to treat it. But knowledge is power. So keep yourself informed and inform those around you. Here’s a listing of some of the things I sure wish I’d known in the beginning when the word “transplant” was looming in the air and the doctors at the ER were all saying “PS what?”
First and foremost, PSC is not a death sentence. So don’t sit idly by and wait for the worst. Speak up! Don’t let a busy doctor give you your diagnosis and walk away, and don’t let a fabulous doctor off the hook if there’s something you don’t understand. Ask questions; don’t be afraid to request clarification on your concerns.
Many PSCers have a hospital buddy, someone who comes with us to appointments and ERs, and can be a second set of eyes, ears, and an extra mouth for asking questions. When your head’s spinning, your “advocate” can take the lead and vice versa.
Find a good doctor. There’s a bit of a debate over hepatologists versus gastroenterologists. Bottom line: find a doctor who is familiar with PSC if at all possible. If that’s not possible, be prepared to educate your doctor. Information is paramount. Support groups and other PSCers can be invaluable in this regard. Symptoms get shared, new research uncovered, medications discussed, questions broached.
Check this link for help in finding a physician.
Get yourself a notebook. It doesn’t matter if it has sparkles and Hello Kitty on it or is covered in your son’s GI Joe stickers. Get that notebook and keep it regularly updated. In it, put all of your vital information: insurance info, name of diseases, medication lists, doctors’ contact info, emergency contact, living will, advanced directives, all your lab and test results, procedure notes—you name it. A brochure describing your diseases is another excellent idea to include; find one at:
Think of this notebook as your lifeline. In the event that you can’t speak for yourself or you’re just too darn tired to, handing that puppy over to your advocate and/or doctor can be a lifesaver and keep you from unnecessary procedures, needle pokes, and the like.
Find others like you! PSC is a rare disease and many of us are told that we’re incredibly isolated, which leaves us believing that we’ll never meet another PSCer, never be able to use anyone else as a base check to see if what we’re feeling is normal, and never have anyone to commiserate with who really, truly gets it. That is absolutely 100 percent true bologna! Yes, we’re rare. So what—all the more reason for us to bond through a great support system.
Find others online and in person here.
You might start by joining our Facebook group (click to join).
You can find more support and useful information about our conferences, fund-raising, current research, trials, and more by clicking here.
Consider building your own network, but don’t forget to talk to those around you, too. There is simply this undeniable pleasure of talking to somebody or sitting next to somebody who knows what it’s like to itch; to be in a room where you’re not the only scratcher there—heavenly! With your PSC you have, at no extra charge, a whole new vocabulary. You’re now fluent (or soon will be) in Urso, LFTs, CA 19-9s, and the like.
A lot of us PSCers have guilt, especially those of us with symptoms. We don’t like asking for help; we want to be able to do everything on our own. However, sometimes we can’t and just need that helping hand, so bring people in. I’m not telling you to be a Debby Downer or start every new conversation with “Guess what, I have PSC.” What I am saying is, let them know what you’re going through. Say “yes” if someone offers to watch the kids or cook dinner so you can take a nap. Don’t be shy if you’re freaked out by a procedure and need a hand to hold, tangible or not. Let those who love you, help. I’ll even let you in on a little secret. Those who offer help, want to help. They want to feel like a part of a solution, to ease your day.
Maintain a sense of humor and a positive attitude. It’s not just an adage. Laughter is the best medicine. Make a game of your PSC when it’s at its worst or you’re feeling low. Take P, S, and C and see how many other things you can come up with. (Hint: there are two in the opening paragraph of this article). Watch a comedy, laugh at old “Three’s Company” reruns, take a stab at morbid health humor (refer to your siblings as spare parts; your jaundice as a golden tan).
There’s no doubt about it, some days your PSC symptoms will drive you bonkers, almost literally around the bend and back. The itching and fatigue alone can be enough to make anyone cry for mercy, but there are some things you can do to help your doctors help you.
Keep track of your symptoms. Even if you need to resort to writing things down, do it! Be specific. Be graphic. Make your doctor realize that even though you’re saying the words “itch” and “fatigue,” or explaining that you feel like you have a severe flu with a brick lodged under your ribs, that those words don’t really cover it. Be proactive in getting the help you need. Do not fear being called a drug seeker if you need pain meds. Be open to any alternative therapies offered. Do not give up on yourself and let the symptoms win!
Lots of PSCers find that one autoimmune disease leads to another, so pay attention to your body. If something doesn’t feel right, do not feel silly asking about it. Do remember that not every ill feeling or cough or sneeze is related to PSC. Sometimes, a cold is just a cold. It’s hard not to be paranoid, and if you really do think something is wrong, by all means go to the doctor. But, think, don’t freak. Fevers are usually a good reason to call the doctor, especially when accompanied by vomiting, shaking, and chills. A headache or a runny nose is usually not enough of a reason to call.
If possible, use one pharmacy; preferably one with branches that share info in more than one state. That way, when any new medications are prescribed, the pharmacist can check for conflicts. Be prepared—some medications for PSCers are not overly common (Urso for one) and may take a day or so to come in at your local pharmacy. Don’t leave refills to the last minute. Keep an updated medication card with you at all times. Type up your medications and dosages, put your insurance ID and doctors’ numbers on it, allergies, emergency contacts, any major medical conditions (like the fact that you have PSC). Shrink it down with a copier and laminate it if you need to and stick it in your wallet, purse, wherever, and keep it with you. Think of it as a really, really efficient medical alert bracelet.
Watch out for your nutrition and follow the doctors’ advice. Everyone with PSC is different. However, there are a few things most of us have in common. We all need to keep hydrated. Dehydration is definitely the enemy on, like, a defcon 1 level. We should be eating healthy foods. Low fat seems to be the top recommendation. Ask about low- or high fiber in your case. Make sure you’re getting enough potassium, vitamin D, vitamin A, and vitamin K. Avoid alcohol or any other item that can stress your liver and has no proven benefit, such as some herbs. Take your medications! You may feel like you’re climbing an uphill battle tantamount to Mt. Everest, but good nutrition and exercise is critical! Even if you can only walk ten minutes a day, squeeze it in.
Read about PSC and nutrition here.
Keep your insurance and manage your finances wisely. Be aware of insurance policy timelines. If you have insurance, do whatever you can to keep it. It’s important. PSCers have terrible trouble getting insured. If you don’t have insurance, look into options: Medicare, private companies, etc. Be vigilant and protect yourself. Many PSCers find themselves in need of some assistance as they become too physically tired or too mentally exhausted to work. You can apply for SSDI (PSC is a recognized disease) or SSI. Check out what your state offers and read through what your personal disability insurance covers. Unfortunately, having a chronic illness isn’t cheap. Spend your money wisely. Make sure you have enough to cover yourself for not only regular expenses but also for hospitalizations, medications, doctor visits, co-pays, etc.
Many of us hear the Big T word during our “Welcome to the World of PSC” initiation, but we don’t get too much information on the actualities. Chances are you may, indeed, need a transplant. But you probably won’t need it tomorrow and you shouldn’t go into it blind. Ask your doctors, use the support groups, research, ask fellow PSCers. Find out about the stages of liver disease. Learn about MELDs and FISH. Don’t be shy; plug yourself in!
Click here to learn more about transplant.
How and when do you tell somebody you have PSC? Some of us blurt it out in the first ten minutes, some of us wait weeks. It’s a personal judgment call. Think about what you want to say and whom you want to say it to. Do you want to go into details or just say that you have an autoimmune disease of the liver and bile ducts? Know what you want to say if someone offers you alcohol, asks how you’re feeling, or worries you’re contagious (you’re not). For those of you who are symptomatic—don’t hide away. Share what you’re comfortable sharing and leave it at that. Don’t get bullied into over-sharing and don’t feel bad if you simply need to pull the “having a bad day” card.
Become an advocate. Know the best way to find a cure for PSC? Become one of the soldiers against it. Hand out pamphlets to your doctors, throw a fund-raiser for PSC Partners, inform your doctors about new treatments or alternative therapies you want to try. Some of us swear by tanning. Something about those tanning booths (or the actual sun if you have access to warm enough weather for it) makes the itching more bearable. Acupuncture helps some people. Others have tried Reiki or Sam-e, different diets, different lotions. Be an explorer. It’s your life, so jump into that driver’s seat and get the show on the road.