VOL: 101, ISSUE: 08, PAGE NO: 30
Anne H. Mawdsley, MBE, is chief executive, Raynaud’s and Scleroderma Association, Alsager, Cheshire
Susan J. Brown, MSc, RGN, EN(G), is clinical nurse specialist in connective tissue diseases, Royal National Hospital for Rheumatic Diseases NHS Trust, Bath, Somerset.
The 2005 Raynaud’s and scleroderma awareness campaign aims to increase detection rates. If a person often has cold hands, he or she could have Raynaud’s phenomenon. Anyone with severe Raynaud’s should ask their GP for a blood test to confirm or eliminate the possibility of an underlying condition, such as scleroderma.
Raynaud’s phenomenon is an episodic circulatory disorder in which the small blood vessels in the extremities are oversensitive to changes in temperature. It affects between 3-20 per cent of adults worldwide and there may be as many as 10 million people affected in the UK. The condition can affect children, adolescents and adults. Raynaud’s is most commonly found in women, with about 10 per cent of women in the UK suffering from it to some degree (Denton and Black, 2004; Cherkas et al, 2003; Jones et al, 2003; Ziegler et al, 2003).
For a small number of people, Raynaud’s is one of the earliest symptoms of impending scleroderma, so prompt assessment and diagnosis are essential.
The hallmark of Raynaud’s is biphasic or triphasic colour changes (white and/or blue and red) in the extremities on exposure to the cold, or to sudden but slight temperature changes or stress. The symptoms usually occur in the fingers and toes, but the ears, lips, nose, nipples and penis may also be affected. The patient may complain of pain, numbness or tingling.
The symptoms of Raynaud’s, whether isolated (primary Raynaud’s phenomenon) or secondary to another condition, may cause severe pain, discomfort and problems with hand function. For the vast majority of sufferers, Raynaud’s is a benign primary condition that may interfere with daily activities but does not cause any long-term damage to the extremities. However, people who have Raynaud’s phenomenon that is secondary to an underlying disease such as scleroderma will often suffer more acute symptoms and, in severe cases, may develop persistent finger ulcers, infection and, in extreme cases, gangrene.
Practical measures such as using hand warmers, thermal gloves and hats often help alleviate symptoms. An even ambient temperature is as important as keeping warm because often it is not the absolute temperature but a small change in ambient environment that precipitates an attack. Cold draughty places should be avoided.
All patients should be strongly encouraged to stop smoking as this causes narrowing of the blood vessels, and to avoid excessive intakes of caffeine, which may cause peripheral vasoconstriction.
The term scleroderma is a generic or umbrella term that covers a number of related diseases involving the overproduction of collagen. Although uncommon, scleroderma is the most deadly of the connective tissue diseases. It is associated with chronic, often disabling, symptoms and its impact on patients should not be underestimated.
Like many other rheumatic disorders, scleroderma is believed to be an autoimmune disease. The immune system becomes dysfunctional and stimulates cells called fibroblasts to produce excess collagen, which is then laid down in the connective tissues, causing them to become thickened, hard and fibrous. The exact cause of scleroderma is unknown, but female hormones probably play a role. There may also be genetic risk factors.
There are several types of scleroderma, from localised forms where hard, tight skin is the extent of the problem, to systemic forms such as diffuse scleroderma and limited scleroderma, where the internal organs are affected.
The most common sub-type of scleroderma is the systemic form of the disease. This can be subdivided into diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis. These two types have different disease progression and organ involvement. The symptoms of diffuse scleroderma include:
- Skin changes within one year of onset of Raynaud’s;
- Skin thickening and tightening all over the body, often causing reduction in range of movement;
- Significant incidence of lung, kidney, gastrointestinal and myocardial involvement;
- Joint and muscle pain.
The symptoms of limited scleroderma include:
- Long-standing Raynaud’s phenomenon;
- Skin involvement limited to hands, face, feet, forearms and lower part of legs;
- A significant late (10-15 years) incidence of pulmonary arterial hypertension, skin calcifications, telangiectasia and gastrointestinal involvement;
- Some lung fibrosis.
Other types include localised scleroderma, linear scleroderma and morphoea. In these conditions, only the skin and underlying tissues, muscles and sometimes bones are involved - the organs are not usually affected. This is the type more commonly found in children.
In addition to the fibrosis caused by excess collagen, scleroderma causes damage to both the small and large vasculatures, resulting in circulatory problems. This means that any organ of the body can potentially be affected by either fibrosis (scarring) or vascular damage.
Almost all patients with scleroderma suffer from problems in the gastrointestinal tract, including difficulty swallowing, acid reflux, diarrhoea, constipation, malabsorption, malnutrition and faecal incontinence.
Lung disease is also a feature and can take two forms: pulmonary fibrosis, which is replacement of the airspaces in the lung with scar tissue; and pulmonary arterial hypertension, in which damage to or stiffening of the vessel walls lead to high pressure in the pulmonary artery. This puts strain on the heart, eventually resulting in right-sided heart failure.
Scleroderma causes vascular damage where the small blood vessels in the kidneys lose their ability to filter the blood and remove waste.
A renal crisis can develop very quickly in a patient, so it is important nurses are aware of the signs. These are high blood pressure (above 150/90) resulting in shortness of breath, headaches and blurred vision.
Although there is still no cure for scleroderma, there are treatments that aim to slow down the disease process.
Immunosuppressive drugs are used in patients with diffuse scleroderma. Organ-specific therapies are given to reduce symptoms, including:
- Motility drugs to help with difficulty swallowing;
- Proton pump inhibitors to alleviate acid reflux;
- Motility drugs to help with dysphagia;
- Antibiotics to help control bacterial overgrowth in the bowel;
- ACE inhibitors to prevent and treat vascular damage in the kidneys and help reduce symptoms of Raynaud’s phenomenon;
- Iloprost (a potent intravenous vasodilator) to improve blood supply to ulcerated fingers;
- Bosentan and prostacyclin analogues (for example, iloprost) for pulmonary arterial hypertension.
In limited scleroderma, treatment focuses on the vascular problems and organ-based complications such as oesophageal disease, pulmonary hypertension or lung fibrosis. Immunosuppression is reserved for problems such as lung or muscle inflammation, or arthritis that occurs in about one in five patients. Not every patient will be affected by every problem and the course of the disease will vary.
Role of the nurse
Living with a rare chronic disease such as scleroderma can result in stigma, which creates special challenges for the patient (Joachim and Acorn, 2003). Education programmes have been developed for the group setting where the patient is helped to take control of the condition by setting achievable goals for the future (Brown et al, 2004).
Patients with scleroderma may experience a variety of non-specific symptoms, including fatigue, lack of energy, generalised weakness, weight loss, and aching muscles, joints or bones. The role of the nurse is to help patients manage symptoms as well as providing education, support and advice to patients and their families. By becoming the patient’s advocate the scleroderma nurse specialist helps them come to terms with a potentially fatal diagnosis.
Symptoms and severity vary, so it is vital for the nurse specialist to be able to assess patients according to their individual symptoms.
Discussion in the clinic or inpatient setting or on a telephone helpline about managing symptoms, coping with physical changes, skin care and interpretation of investigations is an integral part of the nurse specialist’s role.
Nurse are well placed to discuss diarrhoea, constipation or faecal incontinence. Constipation is a common feature of scleroderma due to the stiffening of the bowel and, although laxatives are often prescribed, the nurse should advise the patient on non-medical approaches, such as diet, exercise and increasing fluid intake.
This article has been double-blind peer-reviewed. For related articles on this subject and links to relevant websites see www.nursingtimes.net