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.

Click here to download our Pediatric PSC Brochure.

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.

Note: The online support group for parents of children with PSC can be reached by clicking here.

• http://health.groups.yahoo.com/group/pscmoms/

Pre-teen kids and teens with PSC can get acquainted with others through a new online board.

• http://health.groups.yahoo.com/group/psckids-support/

Click here to download our Pediatric PSC Brochure.

Personal Stories

PSC moms

Day after day I open the messages that PSC moms exchange with each other, each of us rising to the occasion upon each crisis, with unmatched fortitude and resilience. Moms—we are the true heroes. We battle doctors and find ways of solving unfathomable complications for our kids. Often the correspondence is between two moms, with short messages of support, approval, or additional information from other moms interspersed. But these virtual friends know that quietly, many ears are listening, praying, crying, and applauding. Moms are bound by unique ties, no matter where we are, no matter how little we may have to say.

So how can I advise moms who need no prodding to turn into fierce lions to protect their young adult children with PSC, who strive to maintain their independence? At this point, I live miles away from my son. We don’t talk much about health except when we need to exchange pertinent information. It is a tacit understanding between us so he can lead his life as normally as he can, without me being a noose around his neck. He knows I’m the bookkeeper and sends me all his test results. He knows I will interpret them and formulate questions and seek answers. He knows no one will keep me away from his doctors’ appointments or important tests. I send him and his siblings any positive information I read about PSC research and treatment. I try to keep my other children well informed on PSC so they can participate in our quest. As is my nature and my husband’s, we communicate the conviction that together we can find solutions and that there is always a “best way” awaiting us.

And it will take all of us to find the “best way” to eradicate PSC: devoted moms, dads, kids, caregivers, researchers, and volunteers. We truly are “together in the fight, whatever it takes.”

Rachel G.

From the minds of a couple of caregivers

In 2001, in early spring, we became aware that our 13-year-old son Nicklas suffered from some uncommon stomach problems. At first we thought it was an “ordinary” gastritis, but since it tended to hang in too long and he had diarrhoea and gradually worsening pain, we took him to the doctor. From there the ball started rolling.

He didn’t have correct liver status and his intestines seemed to be in a bad state too. The first doctor realized that he didn’t understand what was going on so he sent Nicklas to the specialists at the Astrid Lindgren pediatric hospital in Stockholm.

To make a long story a little shorter we learned, after the specialists extended analyzing of his health, that Nicklas suffered from: celiac disease, lactose intolerance, Crohn's disease and/or ulcerative colitis (combined nowadays as PSC-IBD) and, after some time, he was diagnosed with primary sclerosing cholangitis. The first doctor’s honesty and quite correct reaction on the liver status he didn’t understand made Nicklas come under the scrutiny of specialists in a very early stage and for this we are very thankful!

Shocking News. Then—what was this? Something never heard of had struck Nicklas and us. We had heard about all the other diseases but of course not anything about PSC. I (Peter) did have a colleague whose daughter has Crohn's, I was diagnosed myself as lactose intolerant and “probably sensitive” to gluten, but this PSC thing? When we started to understand and got the full picture we were not the happiest people around.

When a disease like PSC strikes one of your closest relatives—a child, spouse, parent or sibling—it comes as a shock. But, and this is essential, it doesn’t help a bit to fall into a state of self-pity or feeling that it’s the end of the world. These kinds of reactions don’t help yourself or the patient—in this case a young person.

To cope with this situation we learned as much as possible about PSC and also had an honest, open and positive dialog about it in the family. This depends, of course, on how old your child is when the diagnosis is established—in our case Nicklas had just turned 14. It’s individual how one would react to such a diagnosis, but we felt that we would better be totally open with him about all that concerned this. The medical aspects, the possible, or rather expected, progress of the disease and what would most likely be needed in the future: a liver transplant. The way to find information, to start learning, is, to begin talking to the specialists who have done the diagnosis. Thereafter, reading the patient’s medical journals, then searching for medical and scientific papers on the internet, which could be a good way to find more information (as you may have done already, by finding PSC Partners). An excellent repository of information is the PSC-Literature homepage. Click here to go to the literature page.

Daily Routines. In daily living we had to change quite a few things when the diagnoses were at hand. Due to food allergies we were most often “forced” to make two sets of food for all cooked meals. Bread and all other foods were scrutinized for flour. All needed to be gluten free. Nicklas did cope well with the change over from the earlier diet to the new. Even if the other three at the table ate like before he got his own and there was no big deal. Hand in hand with the new diet came the medications for all his diseases: “Pentasa” (5 ASA) and “Omega 3” (fish liver oil) for UC/CD (to start with he also got “Imurel” for a couple of years) and the UCDA (URSO) for the PSC.

There’s a quite big portion of pills, both in the morning and in the evening. This is something that there’s no escape from taking the medication if one wants to stay as healthy as possible. As the caregiver you have the obligation to assure that your patient really does take the medication. For some young patients this is something that could be troublesome and also traumatic and hard to accept: to be locked up for life with a bag of pills. For our son this was never a problem—knowing his diseases and the results that could be an effect of not following the prescriptions. But for others there could be a bit of a struggle to really have the patient take the medicine. In such cases there is no other way than firmly tell that this is actually a must. You can’t let go and say “OK, but you must take it tomorrow.” Taking the medicine has to become a habit, even if it’s not a very pleasant one.

Value of a Specialist. It’s of great importance that the patient has a knowledgeable doctor, a specialist, who he/she relies upon—also for the caregivers this is essential. A good established contact with the doctor and the hospital gives a feeling of relative safety, to have somebody to turn to with questions when they do occur. Through the years you will also develop a sensitive ear, feeling the mood of the patient and be able to put the right questions to him/her. Growing up there are things other than the disease that can be interpreted as problems. It’s good to be able to put things in the right perspective, not always connected to the disease. Unnecessary reactions, always believing the worst, are not healthy for either the patient or the caregiver. Be observant but not always on edge.

Still being asymptomatic regardless of PSC, Nicklas, at 22, goes on pretty much as before. The medicines keep him at the right levels. If you didn’t know he has these diseases you couldn’t tell he was sick! This is very positive, but sometimes it could also be very hard for others to really understand that he actually is quite ill and very sensitive to what he eats.

Reality of PSC. A concealed handicap is often difficult to explain, even to relatives and friends, to give the full picture and severity of it. If you don’t look ill, you couldn’t be very ill. There’s not much you can do about this, apart from giving the best information you possibly can find. Hopefully your family, relatives, and closest friends do understand this, and it’s supportive if they do. It’s always good to be able to share your thoughts with your nearest if and when you feel it’s needed.

PSC has, among other causes, a genetic predisposition as one link in the chain to develop the disease. This genetic aspect could lead to you or your spouse (if it’s a child having got PSC) to start thinking “is it my fault, does it depend on me?” The thing is, yes, of course, this genetic predisposition would be either from one or the other parent. But, when you met each other some 5, 10, 20, or more years ago, married and had children, neither of you knew anything about a genetic influenced disease called PSC. No one should be blamed for this. It’s a very dangerous way to start thinking about what ifs, or, we should never have—. Even if you did know this disease existed in the family and with open eyes made the decision to go ahead and have children, the risk for your child getting the disease is so extremely low you mustn’t blame yourself anyway!

In close to 100 percent of the cases you couldn’t have a chance to know this beforehand. It’s a rare disease! Never blame yourself or your spouse. It doesn’t help or lead to anything good, and rest assured your child, or young adult, doesn’t blame you for it!

One advice we would give is that you do need to come to terms with is that the disease is present in your life. You have to live with this knowledge and with this you’ll learn to appreciate all what’s positive in life, because there’s so much more to life than this! You should, however, not accept that a disease like PSC exists. It must be defeated and eradicated. This “fight” could actually help you to think positively. It works for us!

Peter and Helena H.