Issues for Pediatric PSCers

After a number of tests and excruciating waits, you have found out that your child has PSC. What now? What does this mean? Here is a survival list for those first few months—and longer term.

First and foremost, do not get overwhelmed. I think the first thing that happens to most parents is to become overwhelmed. The information on the Internet can be scary and focuses on the harsher outcomes. The key to dealing with any chronic illness is to take it a day at a time and remain focused on the well being of your child. Although not always easy, it is key to facing this journey—and again as with any chronic illness, this is a long journey.

Become informed. As a parent the ultimate responsibility for your child’s wellbeing rests with you. Get informed on the illness, treatments, research and liver transplants. Having the right information helps deal with the illness and allows you to make informed decisions and take a pro-active role in your child’s treatment. This includes understanding any medications your child will take, how they work and any symptoms that your child may experience.

The PubMed.gov literature for "PSC in children" is available here.
Similarily, "Pediatric PSC" is available here.

Understand tests. Some of the tests you may face as part of the diagnosis and follow up are as follows:

• Blood work—liver enzymes and other tests are one of the first markers the doctors will look at to determine if additional more invasive testing is required. Blood work will continue to be repeated as part of regular follow up for your child – the frequency depends on the doctor and how your child is doing.
• MRCP—effectively an image of the bile ducts (this is a non invasive version of ERCP), this is the most definitive diagnostic test, and due to its non-invasive nature is often used in diagnosing children.
• Liver biopsy—this is an invasive test that can determine if there is damage to the liver, but it is not as conclusive for PSC in children as MRCP; however it can help determine if there is an overlap syndrome and help stage liver damage.
• Ultrasound—although not useful for diagnosing PSC it is often used as part of follow up due to its non-invasive nature to assess overall well being of the liver.
• Colonoscopy—this test is not related to PSC, but to ulcerative colitis diagnosis/follow up. UC can be present in patients with PSC and hence if your child has any symptoms of UC you may face this diagnostic test.

Click here to download our brochure Diagnosing PSC.

Understand medications. Overlaps with other autoimmune diseases are thought to be more prevalent in children than in adults and as a result your child may face additional medications as compared to adults. Here is a list of the most common ones:

• Ursodeoxycholic acid (also known by its brand name Ursodiol or Actigall) is the most common medication currently taken by many PSC patients, including children and is thought to reduce elevated liver enzymes (often present in patients with PSC) by facilitating bile flow through the liver.
• Prednisone is a corticosteroid, which may be used in cases where overlap with autoimmune hepatitis is thought to exist (also can be used to treat ulcerative colitis).
• Imuran/Azathioprine is an immunosuppressant that may be used in cases where overlap with autoimmune hepatitis is thought to exist (also can be used to treat ulcerative colitis).

Click here to download our brochure on PSC Medications.
Click here to learn more about PSC medications.

Be an advocate for your child. Depending on the age of your child, he or she may not be in a position to take an active role in their treatment and follow-ups. As a parent you know your child best; be an advocate for your child and help guide him or her so at an older age, advocacy for themselves will be routine.

Find the right doctor. PSC is a rare disease, and even rarer in children. It is key to have someone who not only understands the disease but is up-to-date on the latest research and developments to look after your child. It is worth the drive to go to a pediatric hepatologist! Click here to learn how to find a specialist in PSC.

The illness in children does not necessarily follow the same patterns as in adults, not to mention having a doctor adept at dealing with kids helps tremendously. The other key aspect, as with any long-term relationship, is to find a doctor that you feel comfortable with. This may not always be a choice, but having a good relationship with someone who looks after your child, someone you feel comfortable asking questions of, will obviously make life easier.

Take responsibility or allow responsibility. Depending on the age of your child you will need to decide who takes the responsibility for medications, appointments etc. Obviously with a younger child most of that responsibility will rest on you, but the key is to instill some of it in the child early on, so that they accept it more easily as they grow up.

Provide support. Being a caregiver is about providing support in both physical and mental aspects. Depending on the age and well being of your child you may be doing one or both of these at the same time. Chronic illness should not change the basic fact of parenting: children need support and guidance as they learn to go through life on their own and deal with their own issues. Provide support throughout and if you need help, you can seek it through your doctor’s office.

Get support. The second aspect of being a caregiver is about remembering yourself. Parents bear a huge burden of dealing with their child’s illness – often because the children are not yet ready to make their own decisions. We often forget that caregivers need support too – each person needs different type of support – make sure you know yours and don’t be shy to ask for help or a listening ear.

Deal with the external world. How you decide to deal with your child’s illness external to your home and immediate family is a personal decision, but you may want to consider the following factors:

• Does your child have any symptoms? If no symptoms are present should you alert the school or friends to the condition of your child? If your child does have symptoms, such as fatigue, will it be easier to explain to the school their condition and why running in gym may be an issue?
• Support to you: will it be easier for you if you share with others and can look to them for support?
• Privacy: no matter what age your child is, how you disclose (or not disclose) their illness to the external world will ultimately impact on their privacy. Whereas it is near impossible to determine how a young child might react in a few years time, with an older child you may want to have a discussion around their preferences.

Prepared by Eve J.