Dr James Ferguson talks about symptom management in PSC:
Fatigue is the most commonly reported symptom in people with PSC and affects quality of life. Fatigue in PSC does not relate to severity of the liver disease 115. We all experience tiredness at times, which can be relieved by sleep and rest. Fatigue is overwhelming tiredness that isn't relieved by sleep or rest.
The exact mechanism by which fatigue is caused in PSC is unclear 115 although studies have found that fatigue in PSC can be related to autonomic dysfunction 116, psychological distress and physical pain (rather than associated IBD or PSC severity) 117 and depression 115.
People with PSC often report that they have disturbed sleep patterns and insomnia, all of which can contribute to the feeling of tiredness and fatigue.
Fatigue can also be explained by other causes such as thyroid issues, anaemia and diabetes.
Why assess? While there is no treatment for fatigue in PSC 117, graduated exercise is thought to help in patients with the chronic fatigue syndrome 119. There are other potential treatable causes of fatigue that should be considered.
Initial assessment: Your doctor should ask you about fatigue. Alternative causes of fatigue such as hypothyroidism, anaemia, diabetes and depression should be considered.
Frequency: at each review appointment
Evidence: EASL Cholestatic Guidelines Section 11.2 4
Itch (also called pruritus) is one of the most commonly reported symptoms by people with PSC. Itch can seriously impact on your quality of life.
Why assess? The cause of itch in PSC is not clear and several mechanisms have been proposed, including increased levels of bile salts in the blood 120,121. Sudden onset of itch is cause for concern and you should contact your PSC doctor as soon as possible to let them know. A range of medicines are used to control itch, however in certain cases this may be a very difficult-to-control symptom.
Assessment and management: Your doctor should ask you about itch and consider medicines outlined in the EASL guidelines. If a medicine doesn’t resolve your itch, you should let your doctor know, so that a medicine that works in a different way can be considered. In the UK, cholestyramine (Questran) is considered first, with rifampicin and naltrexone as second line therapies. The consistency of cholestyramine makes it somewhat unpleasant to take, although there are different ways you can take it and it is also occasionally available in tablet form. You can also disguise the texture by adding it to food.
There have been concerns about the use of rifampicin in liver disease patients in the past. A 2018 study looked at 105 adults with PSC and PBC and found that 95%, including those with jaundice and advanced liver disease took rifampicin safely. The researchers conclude, ‘In the absence of alternative safe, licensed and equally effective agents, clinicians may consider, the use of rifampicin in cholestatic pruritus’ 122. In some cases, sertraline is also attempted.
Pain is a common symptom in PSC and affects quality of life.
People often feel pain in the right upper quadrant (RUQ) or upper right quadrant (URQ). This is in the upper right part of the abdomen just below the rib cage but it can extend to the middle of the abdomen, back and even the right shoulder 123,124. The pain is described as a ‘pain under the ribs’ or a ‘pinching pain in upper right abdomen’ and it can come and go. The cause of pain could be because the liver is slightly enlarged and is stretching the liver capsule which contains many nerve fibres (whereas the liver itself does not). If you experience sudden pain in your right upper quadrant that does not go away, you should let your doctor know.
Sometimes pain is associated with fevers and shakes, and in some patients, and this might be associated with choledocholithiasis (gallstones) or infection in the bile ducts (bacterial cholangitis) which may respond well to antibiotics 1,22. Other people get pain with no obvious cause. This can be frustrating, but this pain is real.
What pain relief can people with PSC take?
We also experience everyday pain just like people without PSC. What medicines can someone with PSC take? Here’s some information from Professor Gideon Hirschfield on pain management for PSC, given to PSC Support on 9 March 2018 (personal communication)125:
We all get headaches, sprained ankles, abdominal pain etc from time to time, and need to take a painkiller!
For patients with liver disease there can be added anxiety about what medicines they can use, and what they can't.
It is always the case that medical advice needs to be tailored to the individual, and the following applies to people with stable established liver disease, rather than those who may have an acute hepatitis still under investigation:
- If you don't have cirrhosis then Paracetamol remains the first line pain killer, in normal dose;
- If you do have cirrhosis, then Paracetamol remains a good painkiller but most liver doctors would recommend that the dose of Paracetamol is dropped to a maximum of 2 or 3g per day;
- Anyone taking Paracetamol is advised against continued use without medical input;
- If you have cirrhosis, particularly if you have varices, then drugs like ibuprofen, voltarol, naproxen etc are not usually recommended (in case they make you bleed or upset your kidney function).
- If you don't have cirrhosis, then occasional ibuprofen or related drugs, is generally fine. The only exception is if you have colitis, because some (not all) patients find that if they have colitis and take ibuprofen, or similar drugs, other than sporadically, that there colitis gets worse;
- If simple oral painkillers don't work, and topical relievers are no help, then sometimes more powerful drugs like codeine, tramadol, and other morphine drugs are needed. These always need to be prescribed. They can be used in patients with liver disease, but your prescriber will be careful to match the dose to the pain, so you don't get unwanted side effects (constipation, confusion).
- For patients post-transplant usually once they are back home the best pain killers are paracetamol to start, and if needed drugs like codeine. Drugs like ibuprofen are usually always avoided in case it upsets your kidney, and if used, are only given after a pain specialist has been involved in your care.
- Some pain responds to drugs like Gabapentin and Amitriptyline- these are usually fine to use in patients with liver disease.
The impact of having PSC can affect your quality of life, future plans and life goals 35,123. The uncertainty associated with the unpredictable nature of PSC can understandably lead to feelings of anxiety and low mood.
Do I Need Formal Psychological Support?
Many people manage perfectly well without formal psychological support but there may be times when you may need to speak to a professional.
When to get help
Psychological support for patients and carers can be helpful at all stages of the illness journey. Seek help if:
- you are finding it difficult to cope with your thoughts and feelings
- your thoughts and feelings are having a negative impact on your day to day life e.g. you are so anxious you can’t concentrate
- your thoughts and feelings are stopping you from engaging with the management of your PSC
How to get help
Speak to your GP about accessing psychological support for dealing with PSC (which is a long-term condition). There may be a wait, but they will be able to refer you for this service.
Your liver unit: your liver unit may have access to a psychology/counselling team, although at the moment this tends to be restricted to liver transplant patients only.
PSC Support Helpline: 01235 25 35 45
Private Sector - Seeking help privately can be expensive and it is important to see someone who is appropriately qualified. The British Association for Counselling and Psychotherapy (BACP) website has information on how to find a therapist and a therapist directory. You can also find register of Accredited BABCP Cognitive Behavioural Therapists on the British Association for Behavioural and Cognitive Psychotherapists (BABCP) website, has a register of accredited CBT therapists.
How to live positively with PSC
It is possible to live positively with PSC! Check out our tips and strategies for living a happy and healthy life with PSC.