Go ahead: Just ask. Don't worry if you think your question is silly or stupid. If you're wondering, ask. It's likely someone else has already asked that question and the doctors have an answer for you. When you are open and communicative, you will develop a better relationship with the medical team.
Be sure to get the name of the person and his/her contact information so that if you think of something later on you can re-connect with your question. Ask how long you should expect to wait until you get a response, an answer to your question. PSCers recommend that you bring someone with you to medical appointments, perhaps to ask questions, and to hear what the doctor is saying. That person can take notes for you, too.
Check our brochure Diagnosing PSC by clicking here.
Write your questions down ahead of time; don't be rushed during your visit. You need the information to maintain your health. Here are some questions experienced PSCers developed that you will want to ask your team if you are newly diagnosed with PSC.
Working with your physician
Recommendations on tests
Recommendations on tests
Upon diagnosis, it is important to sit down with a family member or close friend and outline questions to ask your physician. Having someone to brainstorm with helps ensure that you don't miss something pertinent. Consider asking one or two people close to you to be your advocate(s). Your advocate(s) becomes and stays educated about your prognosis and the progression of your PSC, since PSC presents itself in different ways and progresses at differing rates. Due to the degree of variance in progress and stages, your questions and concerns will change throughout the course of PSC.
After 25+ years of PSC, I found these issues to be important:
Ask your physician to perform a colonoscopy to determine whether or not you have ulcerative colitis or Crohn's disease (forms of inflammatory bowel disease or IBD), in addition to PSC. In the majority of PSC patients IBD is also detected.
What stage is your PSC? In 1984 when I was diagnosed, stage was not a concern for my physician. However, I have learned from other PSC patients that staging may be helpful to know, allowing your physician to have a baseline for comparison with possible future biopsies, in order to note the rate of progression. Staging may also be used to help determine the length of time until transplant. Remember, "may" is the key word here, because PSC has been known to progress at different rates at different times, within a single patient.
It is important to know how often your doctor anticipates an exam, either via an ERCP or an MRCP (specific MRI that focuses on the bile ducts). For the first few years I was expected to undergo an ERCP every six months. Later, my bile ducts were viewed every six months, alternating between an ERCP with brushings to look for signs of cholangiocarcinoma, and an MRCP.
Will there be other ways in which you are monitored for signs of cholangiocarcinoma? Often a CA-19-9 and/or an Alpha-Fetoprotein are checked through a simple blood test. These are strictly cancer indicators; an elevation in either or both of these numbers does not conclude that cancer is present. It is often done, again, to have a baseline for comparison as the disease progresses.
Will you be prescribed Urso (Ursodiol/Actigall)? If so, is the dose considered "high dose," and what are the pros and cons of the medication? For those who have IBD, please note that in one percent of patients there will be a worsening of IBD symptoms.
Is there a support group in your area? Stay connected to other PSC patients when possible.
Click here to learn about local and online support groups.
Can your physician recommend a health psychologist? A psychologist who has experience in working with patients and their families will assist in equipping participants with coping and communication skills.
What transplant facility does your physician recommend, when transplant becomes an issue? Upon your physician's recommendation, research the hospital, hepatologists, and transplant team. If you are comfortable with what you learn, making an appointment sooner rather than later can simulate an interview of sorts. It's better to choose your hepatologist and transplant team before you are too sick to make the best decision.
Advocating for yourself is of even greater importance than finding a competent, involved advocate to intervene on your behalf. Self-advocacy begins with locating one, or more person(s) to get involved in your care. Get familiar with your physician-whether consulting with a new physician or one that you've seen in the past. Research his/her expertise in this area. Create a list of well thought-out questions-you'll become educated while taking control of your care.
Strongly consider connecting with a support group in your area and online. However, allow yourself to take a break if, or when, you feel overwhelmed. There is a lot of stress involved with a diagnosis of this type, obviously physically, but mentally and emotionally, as well. Information is priceless, but determine your own pace at which to learn and process. Be honest with your doctor regarding your symptoms, and listen to your advocate and family when they claim you are undergoing changes. Patients tend to get accustomed to their deteriorated state, and changes can go unrecognized.
Be proactive. Again, due to the nature of the disease and its prognosis, you should consider seeking counseling from a health psychologist for you and your family as soon as possible. Counseling helps the patient and caregivers tolerate the changes as they come. Certainly, if hepatic encephalopathy becomes a condition, mental capacity and personality changes will occur and can be very difficult to deal with.
Prepare a list of your conditions, dates of diagnosis, medications (dosage and frequency), and allergies. Making a comprehensive list and keeping copies in accessible places, including purse and wallet, as well as at home, will answer many questions in situations when you may not be able to recall everything. This list is a priceless tool when visiting both your physician's office and the hospital; it reduces the stress for everyone involved in your care. Prior to traveling, locate the nearest hospital to your accommodations and, if possible, print directions from maps.google.com. Many patients find peace of mind from preparing a medical power of attorney and medical directive, as well.
Endoscopy (specifically ERCPs) will mostly likely-and should-play a role in your treatment and cancer screening. There are risks involved with an ERCP so it is best to seek out an endoscopist with a good reputation among his/her clients. My suggestion is to get a referral from other patients, if at all possible. The greatest complication of an ERCP is the possible induction of pancreatitis. Endoscopists with good reputations typically will be those who are able to avoid causing pancreatitis the majority of the time.
If you are unhappy with your treatment by a physician, the staff, or the hospital, do not hesitate to say so. Of course, there are several ways to handle these situations, but I have found that more progress is made when you sit down and discuss the issues at hand, calmly and matter-of-factly.
However, if you do not get the response you need or deserve, do not be reluctant to begin searching for a physician who makes you comfortable as well as treats your disease properly. You're entrusting your care, and possibly your life, to this facility and you have to know that you're in good hands. You deserve the best.
Support is of the utmost importance. In addition to all of these suggestions, I have discovered that building a support network is crucial. This network will often get you through the days that you feel too fatigued, ill, or overwhelmed to carry the load alone. Support can be found through your physician, local transplant facility, family and friends, as well as online support groups listed here. You'll find amazing people with a plethora of information and lots of heart.
Use these social networks as an avenue to enlighten friends and family, getting them more involved in your daily life experiences, PSC fund-raising, and organ donation.
Talking to friends and family about your disease can be complex. My daughter was only four months old when I was diagnosed, so she has been involved in the life of a PSC patient all of her life. We have discussed in great detail, what it has been like for her and what she can share with patients and their loved ones that may help them on their journey.
Most likely there is a wide range of ages and relationships to consider when finding the best way to tell others of the disease. Regardless of diversity, we maintain that honesty is essential. In dealing with children, explain details which are appropriate for their age group-you will reduce their fear of the unknown, and the feeling that they aren't important or worthy enough to be a part of your life. Children, like adults, feel most secure when they have a sense of what to expect-knowledge is power and leads to a sense of control. Teenagers (mine and teens of other PSC patients) seem to have the most challenges dealing with your situation.
As a parent, the natural reaction is to protect your children, regardless of his or her age, but teens see themselves closer to adulthood than childhood. They want to be informed, they want to help, and frequently they will ask to be the patient's live donor. Sit down and discuss these issues with your teen rather than disregarding their requests. Dismissing your teen leaves them feeling helpless, insulted, and frightened. Allow them to help in ways that are appropriate for their age; suggest that they help you remember things, do some of your chores when you are too tired, and include them in decision making whenever possible. While dealing with an older teen or an adult, explain PSC and then provide a brochure on Living with PSC.
The one mistake that is best to avoid is putting up a front that "all is well." Pride may suit you at the time, but there will come a point when you'll need assistance, and it will be more difficult for people to understand this need. Children will be caught off guard and often resort to anger in response to your potentially frightening "surprise."
Work on getting a network of people to help you get through the bad days; a large "entourage" of support helps prevent any one person from feeling overly burdened when and if things deteriorate. PSC does not have to control lives, but patients must learn to work with, or around it. You can help pave the way for a healthy, full life regardless of your diagnosis, and there's no time like the present to begin.