Living With PBC

Typically, PBC advances slowly. People with PBC may lead active and productive lives for many years after diagnosis. Those who show no symptoms at the time of diagnosis often remain symptom-free for years. 

Jaundice appears to be a sign of diminishing liver reserve and may be an important indication regarding the progression of the disease. The illness is chronic and may lead to life-threatening complications, especially after cirrhosis develops.

When medical treatment no longer controls the disease and the patient has severe liver failure, transplantation is indicated. Signs of liver failure include accumulation of fluid in the abdomen (ascites), malnutrition, malabsorption, gastrointestinal bleeding, intractable itching, jaundice, and bone fractures. Transplantation may be recommended before all these events occur. 

The outcome for patients with PBC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent. The use of new drugs to suppress rejection has made transplantation even more successful. The disease’s slow progress makes it possible to plan elective transplant surgery.

A transplant center, also referred to as a transplant hospital, is where transplants are performed. Every transplant hospital in the United States is a member of the Organ and Procurement Transplantation Network (OPTN), and must meet specific requirements that promote safe and effective transplants performed by experienced health professionals. There are 124 liver transplant programs in the United States and other centers worldwide.

Patients may choose to list at a different transplant hospital and transfer their waiting time to that hospital. For liver transplants, the waiting time is used only as a tiebreaker when the MELD (Model for End Stage Liver Disease) scores and other criteria are identical.

The transplant teams at the original hospital and the new hospital will be responsible for coordinating the exchange of information and notifying UNOS of the waiting time transfer. Patients should ask each hospital if they accept waiting time transfers.

Organ Procurement Organizations (OPOs) are the vital link between the organ donor and the recipient. They are responsible for recovering the organ, ensuring the viability of the organ until it is transplanted and transporting the organ to the recipient’s transplant team. Each OPO provides its services to the transplant programs in its area. This can be a large city, a whole state or a region. To locate the OPO in your area, call UNOS at 888-894-6361.

When a hospital identifies a patient as a possible organ donor, the OPO is contacted. The organ recovery coordinator from the OPO accesses the OPTN database to match the donated organs with patients waiting for a transplant. All OPOs allocate donated organs according to the OPTN policy that ensures that the organ goes to the best candidate at the time for that particular organ. Livers are allocated on the basis of the MELD system.

You should try to stay as active as possible while waiting for your transplant. This will aid in your recovery. Even if you become weak or bedridden, you should still try to breathe deeply, tighten and relax different muscles, stretch your arms and lift your legs.

While you are waiting for your transplant and not confined to the hospital, you need to supply your transplant team with all possible contact numbers. The transplant team will need to contact you quickly once a suitable liver has been found for you. Once you are offered a liver, your transplant center has only one hour to accept the organ or turn it down. If you are not available or are unable to have the transplant at that time, the next potential recipient on the list will be offered the liver. You will stay on the list for future livers.

When your transplant hospital calls you with the good news, you will be asked a series of questions to determine if you have any signs of infection. These signs include a cough, fever or burning sensation when you urinate. If you meet the health criteria, you will be asked to go to the hospital to prepare for the surgery. Try to stay calm and have someone drive you. If you live far away from the hospital, you should already have your transportation planned. Start making your phone calls for transportation, babysitters and pet-sitters right away.

Tips:

• Always keep your transplant coordinator’s phone number with you.
• Keep your cell phone charged and with you at all times.

To reduce the chances of contacting a cold, flu or other infection:

• Avoid crowds during cold and flu season
• Don’t share utensils or drinking glasses
• Wash your hands frequently
• Avoid sick children
• Ask family and friends not to visit when they are sick
• Use bacterial cleaning solutions
• Ask your team about precautions to take while cleaning up after pets
• Do not share cosmetics or nail files with anyone (including nail salons)
• Have your laboratory tests done as ordered, even if you are feeling well.
• Yearly flu and pneumonia shots. It is also advised to have Hepatitis A and B shots.
• Keep an extra request for your lab tests with you in case you are out of town and need to have them done.

The cost of transplantation and follow-up care varies across the country. You will incur costs even before your transplant. These costs may include:

Medical costs:

• Pre-transplant evaluation and testing
• Anti-rejection and other drugs (cost can easily exceed $8,500 per year, depending upon your insurance coverage)
• Hospital stay and surgery
• Fees for surgeons, physicians, radiologist and anesthesiologist
• Follow-up care and testing
• Fees for procurement of the organ from the donor
• Insurance deductibles and copayments
• Physical, occupational and vocational rehabilitation

Non-medical costs:

• Transportation to and from your transplant center
• Food, lodging and long distance phone calls for you and your family
• Child and pet care
• Lost wages for you and/or your family member

You may need to live near the transplant center before and after your transplant. Some centers offer low-cost housing. You may need to make arrangements for air transport to your transplant hospital quickly. You should also make backup travel plans in case of bad weather.

Few patients are able to pay all the costs of transplantation from a single source. Most likely, you will have to rely on a combination of funding sources. The most common funding sources are: 

• Private insurance
• Extended insurance coverage through COBRA
• Medicare and/or Medicaid
• Fundraising campaigns
• Charitable organizations

You may want to ask these questions of your transplant financial team and your insurance provider:

• How much of the transplant is covered by my insurance?
• Specifically, what is covered?
• What financial coverage is accepted by the hospital?
• How much will I have to pay in addition to my insurance?
• Who will pay for my living donor’s testing and surgery?
• How much are the organ recovery costs if I have a living donor?
• Does the hospital have an agreement with my insurance company to accept a lower payment than the normal charge?

One of the goals after transplantation is to return to your normal routine activities, hobbies and work. This goal can be accomplished through a positive attitude, a healthy diet, regular sleep habits and daily exercise. As an individual, your transplant recovery may be different from someone else’s. It all depends on the nature of your illness, how ill you were before transplant, and your individual health status.

Your body protects itself against foreign invaders, such as germs, by using its immune system. Special blood cells attack the invader and destroy it before it can cause an infectious disease. The body may see a transplanted organ as an invader and try to attack (reject) it. To stop this immune response, you will be taking immunosuppressive drugs to keep your immune system from rejecting your new liver.

One of the reasons transplants are so successful today is thanks to these medications. While the risk of rejecting your new organ decreases as time goes by, it never goes away. It is important to know what happens if your body starts to reject your new organ.

Your transplant center will probably have you record your weight, blood pressure and temperature daily. It is important to take all of your medication every day and at the times directed by your physician. You will be taking these or other drugs for the rest of your life. Your dosage and drugs will be adjusted according to your body’s reaction and your lab results. Each drug has its own side effects. You should never change drugs or doses on your own.

Keep a written list of your current medications, dosages, etc., with you in your purse, wallet or pocket at all times.  It is important for you to know:

• What is each medication for?
• What does it look like?
• What is the daily dosage?
• What are the side effects of these medications?
• What side effects should I call about?
• What if I miss a dose of the medication?
• Who should I call for a new prescription?
• Which drugs should I take with food?
• Are generic drugs OK?
• Should I take antibiotics before/after my dental visits?
• Which over-the-counter medicines should I avoid?
• What vitamins should I take?
• What vaccines should I get (flu, pneumonia, etc.)?
• Should I avoid people who have had a vaccine? If so, which ones? For how long?

You need to learn everything possible about your medicines. Your physician, transplant coordinator and pharmacist can help. Medications should never be taken with grapefruit or grapefruit juice. 

You should always check with your transplant coordinator before taking any new medicines, even the ones you buy over the counter, including vitamins and herbal remedies. You should also wear a Medic Alert (or similar brand) bracelet that states you are a transplant patient and take immunosuppressive drugs, in case you are ever in an accident or someone finds you unconscious.

• Always keep extra doses of medicine with you in case you are delayed or miss a plane or train.
• Keep your pharmacy and transplant coordinator’s PHONE numbers with you AT ALL TIMES.
• Never pack your medications in your luggage. Always carry them with you. If possible, have a traveling companion carry your extra doses.
• Carry a letter from your doctor about your medications if you are traveling overseas.
• Prevent infection by washing your hands often and thoroughly, especially before eating.

Data shows that more people receive transplants every year, and people with transplants are living longer. Information on the PBCers transplant webpage was compiled by members of the PBCers Post Transplant Group.

Sharing your decision to be an organ and tissue donor is as important as making the decision itself. At the time of your death, your family may be asked about donation.

Sharing your decision with your family now will prevent confusion or anxiety about your wishes later. Carrying out your wish to save other lives can bring your family members great comfort in their time of grief.