Diet and Nutrition
Although there is no specific PSC diet, ensuring adequate nutrition along the entire path of a chronic and progressive liver disease is complex, especially where other factors such as diabetes or inflammatory bowel disease co-exist. Below are some principals, however, you should always talk with your doctor or dietician before making any significant dietary changes.
If you are early in your liver disease and still have good liver function, you may not need to adjust your diet. It is still wise to have your micronutrients (see below) checked regularly and add supplements where needed. Maintaining a healthy weight in early PSC will help you manage your disease as it progresses. Sticking to the Australian Healthy Eating pyramid that encourages fruits and vegetables as the highest intake source, followed by grains and then dairy (or dairy alternatives) and meat, with smaller amounts of good fats and sugary treats to be kept to a minimum, should see you manage your diet well.
If you are advanced in your PSC it is important that you monitor and try to maintain a healthy weight. Body mass index (BMI) is a score calculated by dividing your weight by your height squared. This gives a rough guide to an appropriate weight for your height but does not allow for other factors such as increased muscle tone (for example, in an athlete), for someone who is retaining additional fluid weight (for example in the liver or heart patient who may have oedema or ascites) or for those whose natural tendency is to be very lean. Some caution should be used in calculating this, and you should not necessarily make it your aim to be within this, but it can give you and your doctor a guide to your healthy weight.
One of the liver’s many functions is to store and release glycogen, a chemical in the body that provides us with our energy. As the liver deteriorates in function, the body is forced to rely on other sources of energy, such as protein (eg. Muscle) and fat stores. A high protein diet can provide some protection from muscle loss. The concern of the past was that the high protein may increase the risks of hepatic encephalopathy, but that is no longer considered to be the case.
Another way to minimise muscle breakdown is to consume foods high in energy. You may not be able to tolerate high-fat foods well, so opting for carbohydrate-rich foods such as bread, rice, and pasta will also assist in providing higher energy amounts. Full cream dairy products, or soy products such as soy milk, are also a good nutritional source.
High energy and protein drinks such as Sustagen and Fortsip may also be useful as they are nutritionally complete and provide high nutritional value in quantities more manageable for someone who is not able to tolerate a full diet.
Micronutrients refer to the vitamins, minerals and trace elements that your body needs in order to function well. There are many of these, but a few are worth a special mention in liver disease.
Fat-soluble vitamins: Vitamins A, D, E and K form the group known as the fat-soluble vitamins. The liver produces bile for these vitamins to be metabolised and used in the body. If the liver is not able to do this adequately nutritional deficiencies occur and require supplementation to reduce the risk of complications. It is important to have these levels checked and supplement them in some form if they are low. However, supplements should only be used in the setting of medical advice and where blood levels have been measured, as they can cause toxicity if they are too high. Supplements may in the form of an oral tablet, injectable into the muscle or via an intravenous infusion. Talk to your doctor about the best form for you.
- Low vitamin A can lead to impaired immunity, issues with the body’s ability to produce blood cells, and certain eye disorders including night blindness.
- Low vitamin D can affect bone health, resulting in lower bone density, osteoporosis and potentially some growth issues in children.
- Low Vitamin E can result in muscle weakness and effects on the central nervous system.
- Low vitamin K can lead to poor blood clotting.
Calcium: This is a mineral we have all heard of, and it works alongside Vit D to help strengthen bones. A calcium supplement may be required in liver disease due to reduced liver capacity or due to its interaction with some medications.
Magnesium: Another common mineral that requires a supplementation due to poor liver function and interaction with some medications. Lower magnesium can lead to muscle cramping and/or restless leg syndrome. This can be supplemented with an infusion, tablet or even by having an epsom salt bath.
Iron: This can be low in liver disease and often found to be low where there is inflammatory bowel disease (IBD) – particularly at times when the IBD is active. Infusions are available as well as tablets. Low iron can lead to worsening fatigue.
Salt: Even without liver disease many of us eat too much salt to maintain a healthy cardiovascular system. However, in liver disease, the additional issue of fluid build-up, either oedema or ascites, is compounded by too much salt. Avoiding processed foods that contain salt, and not adding salt to your food at the table will help reduce your salt intake. However, for those who have had their colon removed, remember that this is where salt is absorbed, so you may not need to be quite as wary. Check with your specialist or dietician to determine what your salt intake should be.
Especially if you have IBD, you will find resources for a wide variety of diets claiming to be specific to IBD. If these diets have a good nutritional base then it is safe to try them for yourself to see if your symptoms improve. There is no scientifically proven diet for PSC or IBD that will reverse the condition or put it into remission, though some diets are being more heavily studied with promise. Always talk to your specialist or dietician to determine if a specific diet is safe for you to try.
The amount of exercise that is ideal for you will depend on factors such as previous fitness and health levels, degree of liver damage, other conditions you may have and any surgeries that you may have had, particularly recently. We encourage people to find the level of activity that optimises their current health and fitness levels. Specialist physiotherapists may provide some useful individual guidelines. Some Medicare funding may be available to you via your GP.
A PSC patient may do anything from running a marathon, to in bed strengthening exercises. If you can, just keep moving.
Mental Health and Wellbeing
Being diagnosed with a chronic and often progressive illness can be devastating and take time to come to terms with. There are many natural and healthy responses to this. Always be kind to yourself as you learn about your new diagnosis. Don’t expect to know everything straight away. Seek out support – family, friends and health professionals. Try to identify what help you need from them and accept that help when it is offered. Learn as much as you’re able to about PSC and develop and understanding how the overall view fits in your scenario. Ask your doctor questions and find reputable sources of information online. There has been a lot of development in our understanding of PSC over the last 10 years, so be mindful of how old information is too. Importantly, find strategies to deal with the situations you face, and implement them as much as you are able.
Some research suggests that 28% of people with a chronic illness will also suffer from a mental health condition, with chronic pain increasing it’s likelihood fourfold. It is not surprising then that many people with PSC have a co-existing mental health diagnosis. PSC may threaten your independence, ability to study or work, place financial strain on you and put strain on a relationship or even make a relationship difficult to start. Having a rare disease with sometimes invisible symptoms can lead you to feel misunderstood and disconnected from family, friends and even health professionals. Whether you have PSC in early stages and no other complications or you have multiple health issues and severe or end-stage liver disease, there are some things you can do to help you manage these conditions.
While these things are bound to cause a level of stress, anxiety is a condition that goes beyond that expected feeling of stress or worry and becomes more relentless and affects your quality of life and even ability to do things. People express their anxiety in a variety of ways – phobias or panic attacks, distressing dreams, fixating on issues and worrying obsessively about them.
If you think you are experiencing anxiety it is important you seek help as there are therapies and treatments that can improve anxiety significantly. Your GP is a good place to start, or websites such as Beyond Blue have helpful tips. You cannot make your condition go away, but you can manage your response to it.
Like anxiety, depression goes far beyond being sad. Depression affects how you feel about yourself, how you view the world and it’s situations. It causes you to look at any problem you have through a magnifying glass. PSC is big enough on its own, we don’t need a magnifying glass!
If you have lost interest or no longer find pleasure in the things you used to, or are unable to find new things that engage you, you may be depressed. Having PSC can have a real impact on your physical ability to do things, or it may be the thought of what might be to come, that has triggered depression in you. Whatever the cause, reach out to professional help and friends and family. Depression can be successfully managed in most cases. Building coping strategies and connecting with others who understand all form part of a potential framework for your management for mental health issues.
It is common to see symptoms of Post Traumatic Stress Disorder (PTSD) in patients (and parents in the case of children) who are transplanted. Although not usually seen as a comprehensive disorder, these symptoms are important to treat for the obvious reasons of wellbeing as well as having some special considerations in transplant patients. One key feature of PTSD is the avoidance of things that remind you of the traumatic event, for example, taking your immunosuppressive mediations may remind you of the event so you don’t take them. This of course brings it’s own dire consequences.
A liver transplant for most people with PSC is a relatively anticipated event. While we may not know when we receive a transplant, we do know that it is imminent, giving a period of time to develop strategies to help in preventing PTSD. Research has also explored the concept that some level of stress in this setting actually provides space for growth and resilience.
Overall PTSD is a complex disorder, but if you feel you are experiencing any symptoms seek help early to enable you to manage it most effectively.
The key message about looking after your mental health is to seek help. At first from family or friends and support groups, but do not feel you cannot seek professional help. Finding a psychologist who specialises in the mental health of patients with chronic illness should give you a solid starting point for recovery.
For those with PSC who are still trying to study, whether you are in primary school, high school, TAFE or university, if you need some additional assistance or allowances there are some avenues to follow.
Most institutions have education support who can help you navigate through the process, however, depending on your needs, you will need to actively engage these people and work closely with them to ensure your educational opportunities are maximised.
Like claiming disability pension, your diagnosis alone will not be enough to secure support. You will need to identify how your illness is affecting your ability to learn and what assistance or modifications you require to optimise your education.
With perhaps a few exceptions, a diagnosis of PSC itself shouldn’t stop you from doing any job you want. However, depending on disease progression and what other illnesses you may have, you may find you need to temporarily or possibly permanently, modify your workload.
For some, even in the earlier stages, fatigue can demand the need for work modifications. More manual jobs that are physically demanding may put additional pressure on you, though there are many who can manage very demanding jobs. If you are affected by fatigue you may need to reconsider your hours or style of work.
Some jobs will require you to produce medical documentation to state that your disease in its current state and foreseeable future, is not expected to limit your ability to perform the job. Entering the police force may require something like this for example.
If you are employed or seeking new employment, it is the employer’s obligation to make reasonable adjustments or to work with you to find a balance in a position you can manage. Both employer and employee have responsibility here, so consider the job you are being employed to do and if that can be reasonably adjusted to allow you to continue, or if it has become too much.
Work-Life balance becomes tighter with a medical condition. If well you will still need to attend medical appointments, and if you are more affected by symptoms, working long hours may take from your home life more than could previously be accommodated for. Finding your new, often ever-changing normal will bring you the best outcomes at work and at home.
For assistance on work modifications and entitlements contact Fair work Australia.
People with PSC travel all the time. The key to traveling with any chronic illness is planning! While spontaneous trips may be a thing of the past, they don’t have to be completely lost.
Things to consider when traveling include:
- Ensure you have a good supply for the trip or easy access to top up while you’re away.
- Consider taking a letter from your doctor outlining your current condition (your last specialist letter may be enough), in case you need to present to an emergency room somewhere.
- If you are flying you will need a letter to support your need to carry your medications as carry on luggage. And ALWAYS take your medications with you on carry on. Any requirement for needles should also be stipulated in the letter.
- If you have any special needs for your flight, for example, wheelchair assistance or medical devices such as oxygen etc, let the airline know early.
- Consider where you’re going and your state of health. What medical care might you need, and how will you get it.
- Discuss immunisations with your GP, and/or specialist travel doctor. Make informed decisions on the safety of locations in this regard, and which immunisations are safe and allowable for you to have. Do this well ahead of your trip especially if you are going to areas of high risk where multiple immunisations are recommended.
- Make lists! Plan, plan and plan a little more. Taking everything you need will help you enjoy your time away.
- Travel insurance can be tricky. Different people have had different experiences obtaining travel insurance. You might like to join our Facebook group and ask around for current success stories in obtaining travel insurance. Also, check out our blogs for possible tips.
Fatigue in PSC can occur at any stage of the disease. Optimising any possible medical management can help, such as ensuring your vitamin and mineral levels are within good range, including your iron levels, and ensuring any treatable condition is treated, particularly any inflammatory or autoimmune condition.
Beyond that some more practical tips that might help you manage your fatigue include:
- Listening to your body: finding a balance between rest and busyness or exercise can be tricky. Knowing when to push a little and when to rest is a skill you can develop over time. Exercise and rest are both vital. Don’t feel guilty about finding time for either.
- Diet: a healthy diet and maintaining good hydration may boost your energy and ability to concentrate.
- Knowing your limits: if you need to adapt your work hours see if your doctor can write a supportive letter to your employer. Take time off when you need to. And “budget” your energy so you don’t miss out on the things that are most important to you.
- Accepting help: if your friends or family offer to help you out with things, it’s ok to accept. Think about how you feel when you are able to help someone else, and allow someone to feel that way when they help you.
1063 children and 4827 adults received a liver transplant between the first Australian transplant in 1985 and the end of 2017. As a rare disease, PSC makes up only small numbers of our national transplant cases. 8 children have received a liver transplant for PSC and 468 adults. Of the adults, around 30 have experienced recurrent PSC and required re-transplantation. The data collected to date shows our centres as world-class in their transplants with excellent survival rates.
The Australia and New Zealand liver transplant organisation collect data on each of our transplant centres. People are geographically allocated to their centres, of which most states have one adult centre, and some have an affiliated paediatric centre. In general, each centre accepts donor organs from its own region, however, in some extreme instances, a donor organ from another state may be allocated to someone. A region generally covers a whole state and may include a second state.
The decision and work up:
The decision for transplant is not one made lightly and is an important stage of the PSC journey for many people. Mixed emotions are common, fear, anxiety, joy, relief, among a few. If your doctor refers you to the transplant team in your region it is because they feel your liver is no longer functioning well enough and a transplant is a good option.
The transplant team is a multi-disciplinary team of hepatologists, surgeons, specialist nurses, radiologists, dieticians, psychologists, physiotherapists to name a few. Your “work up” will include meetings with some of these people as well we appointments with a number of other specialty doctors such as cardiologist and renal physician, who will order a further series of tests to fully evaluate your health and pre-empt and treat any foreseeable issues to transplant.
The preparation time:
I prefer not to use the term waiting….because waiting implies that you’re sitting around doing nothing.
Once you’re listed for transplant you have the opportunity and responsibility to be in the best possible shape you can be in, both physically and mentally.
Ensure you attend your appointments and manage your medications as prescribed. If your care is being shared with your regular hepatologist and the transplant unit check they each know what is happening in your case.
If you’re not able to tolerate much exercise, find something you can tolerate. Finding an enjoyable physical activity, even if very light weight, will help you stay in better physical shape and improve your mental state. Even walking out to the letterbox can be an activity!
Keep living your life. You may have some physical restrictions that you have to work with but keep doing the things you love if you’re able. Don’t give anything up just because you’re on the transplant list. Go out for dinners and shows……even short holidays, so long as you stay within the parameters outlined by your transplant centre.
Be ready for a “dry run”. You might get a call only to later discover that the liver is not viable, or not the best match for you. As heartbreaking as this is, try to focus on the fact that you were called, your time is nearing.
Once called in you will be prepped for surgery. The transplant team will have gone through this with you as part of your workup. Liver transplant surgery is a huge operation, but one that we have highly experienced and capable surgeons for.
The transplant is facilitated most often by the generous gift of organ donation from a deceased donor. Where someone has passed away under very specific circumstances and their family has bravely made the decision that their loved one’s organs can be donated. Live donation, that is where a person donates a portion of their liver for someone, is sometimes considered, however it is not always suitable for a variety of reasons. It has most commonly been performed on children in Australia but is not a common occurrence in itself. If you wish to explore this option talk to your transplant team.
The recovery following a transplant begins in the intensive care ward. This can be quite confronting but is a highly specialised and monitored environment to give you the absolutely best first stage of recovery.
The time in ICU and then on a general ward will vary greatly. But each stage of recovery can be celebrated and enjoyed. While on the ward you will be educated about your immunosuppressant medications and any other medications you will need. You will remain closely monitored even once you return home, often having to return for blood tests and examinations in order to detect any issue early and deal with it.
Many people find they can return to their previous job and enjoy physical activities they have not been able to manage in years. Some even choose to participate in the National and International Transplant games.
Transplant is a highly successful treatment for most who undergo it, however, learning of the issues of immunosuppression must also occur. Lifelong medication to allow your body to keep your new liver is necessary. Your medical team will choose what is best for you and adjust to lower levels the further away from your transplant you get.
Unfortunately for a small number, PSC can recur in their new liver. This may or may not behave in the same way as your initial diagnosis, but your medical team will help you through this.
Those who care for people with a chronic illness help carry the load of the illness and are truly remarkable people. By choice, they stand beside a family member or friend to help them through.
Carers Australia has acknowledged that there are literally millions of unpaid carers in Australia, with over 800,000 of these identifying as a primary carer. These primary carers typically have lower incomes than those who are able to work without the addition of caring for someone and are unable to work as many hours as they otherwise would.
Our carers are highly valued and it’s important for the carer to be cared for. There are many supports to help carers, some of which can be found through Carer’s Australia, which has national and stage based programs available. Check their website for details of supports and eligibility.
Tips for carers:
- Find space and time to do something you enjoy on a regular basis. This may not be easy to do, but it’s important when caring for someone with a chronic illness. If the person you care for cannot be left alone, and you don’t have someone who can give you these breaks, connecting with carer’s Australia may help you resource some formal respite.
- Get rest and nutrition. We often talk about the rest and nutrition needed by someone with PSC, but as a carer, you need to eat and sleep well too.
- Accept and ask for help. If help is offered accept, perhaps even if it’s not exactly what you were wanting, every little bit helps, and it builds a relationship with the other person. Consider what you really do need too, and ask for it. If you’re going through a bad patch, reach out and be specific in the ways people can help. Cooking a meal, mowing a lawn, mopping the floors…..people feel good when they help, but often aren’t sure how to help.
- Do enjoyable things together with the person you are caring for. Make sure not all your time is spent on meeting health needs. Take them to their hospital appointment and call into the café for lunch on the way home, or walk through the park together. Don’t lose sight of the things you enjoy together and the reasons you love the person in the first place.
- Be realistic. Don’t expect to prepare a lavish 3-course dinner for the family after caring for someone who has been very unwell all day. Find that pre-prepared frozen meal, or get the occasional takeaway. If your house isn’t spotless it’s OK too. If anyone is unhappy about the state it is in ask them over then hand them a mop and bucket.
- Expect good days and bad days. Some days you’ll get to the end and smile as you drift off to sleep. The day went smoothly and any hiccups were easily adjusted to – you even got that lavish 3-course dinner on the table! But other days you’ll fall into bed late, exhausted and with a sense of defeat. That’s OK too. Know that tomorrow is a new day, and congratulate yourself for getting through a tough one, even if it was by the skin of your teeth and with a few disasters along the way.
- Make sure you have someone you can offload to. Someone to talk through the tough stuff who will not judge or provide an opinion (unless you ask), but who will listen and be your friend.
- Don’t be afraid to seek counseling yourself. Having someone you love diagnosed with PSC is a big deal. Take care of your mental health too, and if that means seeking some professional support then that’s what you should do. Finding strategies to help you through the harder times will help you in the longer term too.
Carers are often the biggest cheerleaders to the person they’re caring for – so to all of you who have one, thank them in advance for the hard stuff they might do tomorrow when you’re also too stressed to thank them.