Scleroderma

The two main types of scleroderma are:

• Localised scleroderma – this type of scleroderma causes hard skin, which may gradually begin to occur in one place and then, perhaps, in other areas of the body. There are different subtypes of localised scleroderma too, which are defined by the areas of skin that are affected, and how. For example, plaque morphoea scleroderma causes patches (or ‘plaques’) of skin to become reddish brown. Localised scleroderma does not usually lead to complications, although the skin may be uncomfortable, unsightly and painful

• Limited scleroderma (also called systemic scleroderma) and diffuse scleroderma – these differ from localised scleroderma because additional symptoms may occur. Both may also affect the functioning of internal organs, leading to other health conditions developing, or long-term health problems. Limited scleroderma affects certain areas of the body, whereas diffuse scleroderma affects the whole body. Read more information on systemic sclerosis

The two main types of scleroderma are localised or systemic scleroderma.

Localised scleroderma symptoms include:

• Patches of skin that may be itchy, look shiny, or discoloured, which may get better over time

• Areas of thickened skin on the face, head, legs or arms, which sometimes improve, but may cause growth or mobility problems if bone and muscle is affected

Systemic scleroderma is generally considered to be a more serious form of the disease. Its symptoms may include:

• Thickening of the skin anywhere on the body, as well as odd spots and lumps

• Infections of the skin

• Skin ulcers

• Organs and digestive system more likely to be affected

• Swallowing difficulties

• Heartburn

• Raynaud’s disease – which may occur first

• Fatigue

• Joint pain, inflammation, swelling, stiffness due to the skin and tissue around joints becoming thickened and hard

• Weight loss

• High blood pressure

• Pulmonary hypertension

When skin thickens, it becomes very tight and may feel uncomfortable and painful. This may restrict movement of the affected part of the body. For some people living with scleroderma, the hands and feet may be affected, which sometimes affects a person’s ability to pick things up, grab, walk, etc. Some people also find they have scleroderma of the face, which can physically change their appearance, and may be a difficult symptom to deal with.

Scleroderma can be difficult to diagnose. Some GPs rarely see the condition, but they are likely to refer you to a consultant. You are likely to see a rheumatologist initially. The thickening of skin is often the key to a diagnosis, but other tests may be performed such as a blood test for autoantibodies, which may suggest the person has an overactive immune system, as seen in scleroderma. Read more information on the process of scleroderma diagnosis.

Scleroderma is likely to affect a person’s life in various ways, depending on their symptoms, and which type of scleroderma they have. Here, we list some of the challenges that a person living with scleroderma may face and ways they can help to combat these head-on.

• Symptoms – scleroderma symptoms may be difficult to deal with, causing visible, and sometimes invisible, issues. Getting the right treatments for scleroderma are important, and we cover these in the next section. Talking about your symptoms may also help, and Scleroderma & Raynaud’s UK provide a helpline for those affected by the condition to talk about their experiences and ask for advice. Similarly, talking to others who have scleroderma may help, and the NHS provides an online scleroderma forum

• Physical appearance – because scleroderma causes changes to the skin, such as tightening and thickening, the skin can change appearance and some people with the condition may be conscious of this if the skin is visible to other people, e.g. if their face is affected. There are creams and treatments to help skin look and feel better, which we talk about in the next section. There are also ways to cover areas of skin that people are conscious of, using camouflaging make-up and other products. Read more about scleroderma and skin camouflage

• Lifestyle – people living with scleroderma can do most of the things that other people do, such as go on holidays, work and enjoy a social life, but scleroderma symptoms may affect these activities. It is advisable for people living with scleroderma to have a healthy lifestyle as much as possible, to retain their general health. They should try to eat a healthy diet. Some people may develop coeliac disease, so may require a gluten-free diet. Getting regular exercise is also important. Quitting smoking and reducing high blood pressure is also really important

• Fatigue – a common symptom for several reasons, scleroderma causes fatigue which can be difficult to manage and may affect everyday life. Fatigue may be due to medication side effects, using lots of energy due to having weakened muscles, difficulties sleeping at night, and pain. Generally, people experiencing fatigue are advised to spread activities out across the day and week, ensuring they have frequent periods of rest, rather than doing too much one day and feeling exhausted the next. Read more about scleroderma and fatigue

• Sex – most men and women living with scleroderma are able to have sex and enjoy sex, but some may have issues if scleroderma affects their genitals, or if they develop depression, anxiety or health conditions. There are lots of ways to help with sexual health difficulties though and these should be discussed with your GP in the first instance

• Pregnancy – lots of women living with scleroderma are able to have children but some may have increased risks to themselves or their baby. Read more about pregnancy and scleroderma

• Prognosis and survival – living with a chronic and life-limiting health condition inevitably brings anxiety about the future and what this will hold. Most people live a full life and are able to do all the things that people without the condition can do. Scleroderma is unlikely to be life-limiting for them. However, for others with systemic scleroderma, which affects internal organs, they may be living with the prospect that their condition may progress more severely and affect how long they live. This can be a difficult thing to deal with, but there are lots of support services that can provide help for scleroderma patients in dealing with the unknown aspects of their condition. Talk to your GP or scleroderma specialist if you are finding it hard to manage concerns about prognosis and survival

For some people, scleroderma may get better on its own, depending on the type of scleroderma they have, but systemic scleroderma may progress over time. There are a variety of treatments for scleroderma which may be available, depending on a person’s symptoms, such as drug medications, localised or topical treatments, therapeutic programmes such as physiotherapy, and many self-care treatments.

The scleroderma treatments listed below are for some of the more common symptoms – this is not an exhaustive list, and if you are finding any of your symptoms difficult to manage, it is advisable to talk to your GP or specialist team.

• Skin soreness, ulcers, tightening, dryness – many people find it is key to keep the skin moisturised which may provide some relief to skin, and some people find that physiotherapy can help with tight skin

• Raynaud’s (poor circulation, extreme sensitivity to temperature changes) – try to stay as warm as possible by wrapping up in cold weather, wearing extra pairs of gloves/socks, using heat pads, keeping your house warm etc

• Digestion – people who are living with scleroderma may have digestive or gastrointestinal symptoms such as diarrhoea, gas, bloating, constipation, heartburn, weight loss, poor appetite. There are many medications that may be used as treatment for scleroderma symptoms such as these, and lots are available over the counter. However, it’s a good idea to discuss these treatment options with your GP or specialist scleroderma team to ensure they are suitable. Otherwise, many digestive issues can be managed through diet, and we talk more about this in the next section

• High blood pressure – this may be treated with lifestyle changes and medication such as ACE inhibitors

• Joint pain and inflammation – this can be treated with pain relief and anti-inflammatory drugs

• Stress, anxiety, depression – issues such as these are common for people who are living with a health condition, and there are lots of ways to help treat these difficulties, including anti-depressant medication and psychological therapies

In severe cases of scleroderma, drugs that target the immune system may be prescribed.

Read more about scleroderma treatment.

Daily living aids are products designed to help you with mobility, getting around the home, getting around outside, getting ready and doing chores around the home.

At NRS Healthcare, we are experts in daily living aids. If you have scleroderma, you may find the following daily living aids useful.

• Mobility aids – scleroderma can affect a person’s ability to get around, and these products can help you do this more easily and safely:

o Duo Walking Trolley

o 3-Wheel Rollator

• Comfort when sitting – if your feet are affected by scleroderma, you may find comfort from raising your legs when sitting, and some people find a rise and recline chair is useful to help them get in and out of a chair:

o Foot Rest

o Rise and recline chair

• Grip helpers – if scleroderma is causing sore or tight skin on the hands, you may have difficulty gripping a pen or eating utensils, and these aids may help:

o Foam Tubing

o Pen Grippers

o Kura Care Cutlery

o Good Grips Cutlery

o Thermo Mug

o Dressing aids

o Food preparation aids

• Help around the home – you may find that your symptoms affect your ability to carry trays/meals or stand for long periods, and these products for scleroderma may help:

o Household trolley

o Perching stool

• Raynaud’s aids – if your hands and feet are sensitive to the cold or temperature changes, you may find that a wheat warmer provides relief:

o Wheat Warmer

NRS Healthcare has a team of occupational therapist product advisors who can advise what products may be useful to you. Contact them for free on 0345 121 8111 or email [email protected]

Diet for scleroderma

An adequate, nutritional diet for scleroderma patients is important, whatever type of scleroderma they may have. People with systemic sclerosis are likely to have digestive issues and may experience weight loss as a result of their condition, so eating a balanced diet is essential. It is important to get enough calcium, vitamin D, iron and healthy fats. Fibre can help with blood pressure and anti-inflammatory foods such as ginger may help reduce some symptoms of scleroderma. Read more about scleroderma diet.

If a person has coeliac disease as well as scleroderma, they may need to follow a gluten-free diet. Read more about coeliac disease.

Some people take vitamins and minerals as supplements for scleroderma, such as vitamins A, C, D, E, K, folate, B12, zinc and iron. Some people take probiotics to help with digestive issues, or anti-oxidant and anti-inflammatory supplements such as turmeric, selenium and beta-carotene. It is advisable to discuss this with your GP before taking scleroderma supplements.

Exercise for scleroderma

Scleroderma exercise is important but may feel challenging for some people, for example, if their skin is tight and uncomfortable. Any activity that gets the body moving, within the reasonable limits that a person can manage, is beneficial for joints, muscles, lungs, heart and mind. It is important for people living with scleroderma to obtain advice before starting an exercise programme from their specialist team or a physiotherapist. Read more about how exercise may help scleroderma.

Scleroderma and employment

Lots of people who are living with scleroderma are able to work. Some people who have systemic sclerosis that progresses to a more serious stage, may decide to leave work if their scleroderma symptoms make working difficult.

Scleroderma and Raynaud’s UK online community – a forum for people affected by Raynaud’s or the associated condition of scleroderma, to share experiences

Scleroderma Facebook Group – an online support group for scleroderma patients and their families

Coeliac UK – for those living with scleroderma who also have coeliac disease, Coeliac UK provides a wealth of information about the condition

NHS – source of official medical information about causes, symptoms and treatments for Raynaud’s and related conditions

PHA UK – a charity supporting people who have pulmonary hypertension, which may be a complication of systemic sclerosis

Scleroderma and Raynaud’s UK – a charity providing support and information to the millions of people living with scleroderma, Raynaud’s and associated conditions, with an online community and lots of online information about daily life with these conditions

ACE inhibitors

– medication used to treat high blood pressure, which blocks the body producing angiotensin II, a chemical that narrows blood vessels

Autoimmune diseases

– conditions where the immune system attacks the body, mistaking it for an invading virus/bacteria

Coeliac disease

– digestive health condition caused by the immune system reacting to a protein called gluten which is found in wheat, barley and rye, and then attacking the tissues of the gut, causing intestinal damage and an array of unpleasant symptoms

Pulmonary hypertension

– a condition caused by high blood pressure that affects the pulmonary arteries, damaging them and affecting blood supply to the lungs and heart

Rheumatologist

– a doctor specialising in conditions affecting the musculoskeletal system e.g. bones and tissues