Scleroderma and your healthcare profession

OTs can provide splints, both to assist with functional activities and to rest in at night. OTs can also show the patient hand exercises to maintain muscle strength and range of movement.

Digital ulcers have been mentioned previously as being a problem for people with scleroderma. OTs canprovide Silipad digital caps to assist with the healing of the ulcers by keeping them moisturised and soft. The grade mineral oil contained in the proprietary polymer gel helps with this, as well as relieving pressure andfriction. However, the ulcer must be dry and scabbed prior to wearing.

For patients experiencing symptoms of Raynaud’s, silver gloves can be provided to regulate body temperature in the hands. It is the natural pure silver contained in the gloves that helps with this.

Isotoner gloves, which contain nylon and spandexmaterial, can ease painful and swollen joints byproviding gentle compression. Precautions must be given if the patient has Raynaud’s regarding circulation.

It is useful to give advice on joint protection to help reduce the risk further strain on the joints whencompleting tasks.

Occupational Therapists Most patients with scleroderma will have beendiagnosed before being assessed by an Occupational Therapist (OT). For the few that have not, they areusually an inpatient being investigated to have thecondition confirmed.

OTs assess how patients with scleroderma aremanaging with daily living at home and promote theirindependence. If a patient identifies that they arestruggling with a particular activity (transfer) they can be provided with advice and equipment that can help make these tasks easier. This can include rails, toilet frames, perching stools as well as advice on smalleraids, such as for the kitchen or dressing.

An OT can also advise patients on managing at work and signpost them to Occupational Health services andAccess to Work for further assistance.

Splints can help with reducing pain, stiffness and the risk of further deformities/contractures

developing.

The Scleroderma Society supports people withscleroderma and their families by providing:

leducational literaturela telephone helpline

la comprehensive website & forumla newsletter with research information

lmember contactlinformal group meetingslan annual conference

We also work to promote awareness of scleroderma among the medical profession and general public in order to improve early diagnosis and prognosis. We fund medical & scientific research in the UK and are a founder member of FESCA (Federation of EuropeanScleroderma Associations), working to forward the cause of people with scleroderma throughout Europe. To join the Society, visit our website or phone the office on the number below.

The Scleroderma SocietyBSR House, 18-20 Bride Lane

London EC4Y 8EE

020 7000 1925info@sclerodermasociety.org.uk

Helpline 0800 311 2756www.sclerodermasociety.org.uk

UK Registered Charity No. 286736

Written May 2013 / ©2013Next Revision May 2015

Who develops scleroderma? lScleroderma can affect anyone from anybackground and any age group, including youngchildren and older people.

lOnset is most frequent between the ages of 30 to 50.

What are the symptoms of scleroderma? There are different types of scleroderma. lSymptoms can vary widely from person to person.

lThe cardiovascular system, kidneys, lungs, anddigestive system can all be affected – some symptoms can be life threatening or cause physical disability.

lOther possible symptoms are joint pain and stiffness, blood vessel damage leading to a physical over-reaction to cold or stress, changes to facial and physicalappearance, swelling of the hands and feet andtightening of the skin.

lIn children, scleroderma may affect their growth –resulting in, for instance, different length legs and arms.

PhysiotherapistsYou may only ever see a few patients with scleroderma over the course of your career. However, as clinicians managing patients with musculoskeletal conditions,having an awareness of scleroderma means that you may spot a previously undiagnosed case. Working closely with patients and on regular follow-ups, physios are well placed to pick up on conditions a GP may miss.

Most patients who have scleroderma will havepreviously developed Raynaud’s Phenomenon. Seeing a patient with Raynaud’s who presents to you with non-specific range of movement limitations, may raise suspicions of underlying inflammatory causes. The skin and underlying tissue tighteningcharacteristic of scleroderma most often presentsinitially at the hands, but could appear anywhere on the body. A full range of movement assessment is useful

patients are susceptible to digital ulcers. Encouraging patients to use emollients and moisturising regularly is highly recommended.

If patients have digital ulcers they need to be treated quickly, as they can get rapidly infected. Ulcers are very painful, and take a long time to heal, so the quicker they are treated the better, as they can easily affect the patient’s quality of life.

There is no special diet for patients with scleroderma, however they should be encouraged to have a healthy balanced diet and also to recognise and avoid foods that give them problems. It is easy for scleroderma patients to lose weight rather than put it back on. Patients should be encouraged to stop smoking, as this can have an impact on their blood circulation problems, and to drink alcohol in moderation. Stretching exercises (hands and feet) should be strongly recommended. These need to be started as soon as possible after diagnosis. Scleroderma patient care plans should be individualised because the disease severity and progression varies from person to person and different approaches may be required.

How many people have scleroderma? lThere are about 6000 – 8000 people with scleroderma in the UK

lWomen are affected four times as often as men

lThe complex nature of the condition can makediagnosis difficult, so the true number of people affected may be higher

Nurses Nurses have an important role in providing holistic care, in whichever department or unit a patient withscleroderma is seen. As scleroderma is a rare condition, local support may be limited, however. These basic recommendations could help patients feel less isolated.

Around 95% of patients with scleroderma also have Raynaud’s Phenomenon, so keeping warm is essential. Some patients go through many changes, including facial changes and contractures, which can result in low self esteem. Newly diagnosed patients, in particular, need a lot of emotional support. Fatigue is a common

symptom, so they and their families frequently require daily support.

Patients with scleroderma can often experience significant, widespread musculoskeletal pain, so a basic knowledge

of analgesics is essential. The use of NSAIDs is discouraged,

however, as they can affect the stomach lining and

patients withscleroderma are susceptible to reflux and associated gut disease.

Mobility can be aproblem for some patients because of skin tightness and stiffness. Other patients may have difficulty with self-care, due to contractures,

stiffness and ulcers.Appropriate skincare is very important withscleroderma, as the skin

tends to get dry easily and

What is scleroderma?Scleroderma is an auto-immune condition that affects the connective tissues. It has many symptoms and can be life threatening. Due to the variety of symptoms and because the progression of the disease varies from patient to patient, it can be hard to diagnose.

AHPs vital roleAs an AHP, you may see patients who have beendiagnosed with scleroderma. You may also see patients who are apparently presenting with other problems, but who in fact have undiagnosed scleroderma.

The involvement of AHPs is vital in helping people with scleroderma manage their condition and have the best quality of life possible. This leaflet explains how nurses, occupational therapists and physiotherapists can help.

If you suspect a patient is undiagnosed forscleroderma, please raise your concerns with an appropriate health care colleague. Most people with scleroderma are diagnosed byrheumatologists, or sometimesdermatologists.

What causes scleroderma? lIt is not known what causesscleroderma and there iscurrently no cure. Proper treatment and management can make itpossible for many people with scleroderma to lead full and productive lives.

lEarly treatment is vital, as the most serious problems, such as renal failure, tend to occur within the first two or three years.

in patients whom you suspect may have the condition. Palpation and visualisation of tightened areas is also recommended.

During the first year or so of someone developingscleroderma, it is thought that over aggressive stretches can be detrimental to the patients’ long-term outcome. But advice on gentle stretches and the use of heat can be vital in limiting the rampant initial stages of the disease to minimise longer term effects and potential disabilities

Once the condition is established and diagnosed, the patient will remain under regular review of arheumatology team. They may see a physiotherapistassociated with this team.

However, should your patient list include people with scleroderma there is a lot you can do to ensure they have a high level of care. Regular, gentle stretches are important. Specific guidelines will soon beavailable. The use of wax treatment, hydrotherapy, mouth stretches with tongue depressors, manualtherapy and general cardiovascular work have all been shown in RCTs to aid these patients. The treatment techniques are not complex and patients willappreciate the convenience of seeing their localphysiotherapist, should you be involved in theirtreatment.

People with scleroderma can of course have othermusculoskeletal problems, which an MSKphysiotherapist would be very well placed to treat. As with all inflammatory conditions, close liaison with the medical team is recommended.