From the National Magazine

Not Another Diagnosis?

Presentation by Sister Sue Brown, Clinical Nurse Specialist in Connective Tissue Diseases at the LUPUS UK Convention, Plymouth May 07

This presentation concentrated on conditions associated with lupus and discussed the role of the lupus nurse specialist. The three autoimmune conditions discussed in this presentation were Raynaud's Phenomenon, Antiphospholipid Syndrome and Sjogren's Syndrome. All these conditions share some common features:

1. They are classified as disorders of the immune system, autoimmune diseases
2. They commonly affect more women than men
3. They carry specific auto-antibodies (blood proteins) which aid diagnosis
4. They can be either a primary diagnosis or secondary to a condition such as lupus

Raynaud's Phenomenon

Raynaud's phenomenon was first described by Dr. Maurice Raynaud in 1862, when he observed 25 patients with peripheral vascular problems, describing this as 'asphyxia of the extremities.' This means people suffer with a decreased blood flow to the body's tissues, especially the extremities such as the hands, feet and nose. Classic descriptions include a 'triphasic' colour response, white, and blue then bright red. The order of the colour change can vary between people and duration of attacks from minutes to hours. Attacks can be provoked by cold (or even slight temperature change) or stress and are worsened by smoking as nicotine decreases blood flow.

Raynaud's can develop at any age and it affects between 3 and 20% of adults worldwide. It is more commonly seen in females and can affect all ages. It is occasionally seen as a side effect of taking beta-blockers, used to treat high blood pressure. Diagnosis is usually made through detailed patient history and thermographic imaging of the hands following cold stress test. It is estimated there are up to 10 million people in the UK who suffer with Raynaud's (Raynaud's and Scleroderma Association, 2006).

The Role of the Clinical Nurse Specialist in Raynaud's Phenomenon
The type of information and advice a nurse specialist would offer is as follows:

• Avoiding cold exposure, dressing warmly especially in hands and feet
• Stop smoking
• Provide written information sheets
  Medication advice including vasodilators (such as Nifedipine) and vitamin regime (including Evening Primrose Oil, Fish Oils, Vitamin E, Vitamin C and Gingko Biloba)
• Skin care advice
  Join a patient support group such as LUPUS UK or Raynaud's and Scleroderma Association (www.rayanuds.org.uk)

Antiphospholipid Syndrome

Antiphospholipid Syndrome (APLS) is an autoimmune disorder where the blood tends to clot more quickly due to an excess of Antiphospholipid antibodies. This condition is also called 'sticky' blood and was observed by Dr Graham Hughes in the 1980s at St Thomas' Hospital in London, when he was studying lupus patients with brain disorders. These blood clots can affect any artery or vein and there is significant chance of miscarriages in women. The most common presentation is as a DVT (deep vein thrombosis) in the leg or a PE (pulmonary embolus in the lung) or in frequent miscarriage. Some patients develop a characteristic 'blotchy' rash, often seen on the arms and legs, resembling a lacy pattern (livedo reticularis). ApLS can affect different parts of the body and can lead to a low platelet count, kidney problems (renal artery stenosis) and strokes. Indeed APLS is responsible for CVAs (strokes) in up to 20% of those under 40 years of age. Many with APLS also suffer with Raynaud's.

Specific antibody tests will be positive for anticardiolipin (or Antiphospholipid) antibodies in 80% of cases. Another test called a Lupus Anticoagulant is positive in 30-40% of cases. The aim of treatment is to thin the blood, thereby reducing the risk of clots, using drugs such as Aspirin, Warfarin and Heparin. Warfarin is only used for those who require long term anticoagulation. Pregnancy needs to be planned as it can lead to a slight increase in blood 'stickiness' which is unable to cross the small blood vessels in the placenta. This can lead to spontaneous abortion and other pregnancy complications such as pre-eclampsia, growth restriction and prematurity.

The Role of the Clinical Nurse Specialist in Antiphospholipid Syndrome
type of information and advice a nurse specialist would offer is as follows:

• Increasing exercise where possible as immobility can increase risks of clots
• Review contraception and pregnancy planning in women
• Pre-pregnancy counselling in those women with Ro/La antibodies and Antiphospholipid Syndrome
• Support following pregnancy loss
• Provide written information sheets
• Stop smoking
• Discuss air travel including medication, support stockings, regularly exercise during flight, drinking plenty of water and avoiding alcohol
• Join a patient support group such as LUPUS UK

Sjogren's Syndrome

Sjogren's Syndrome is an autoimmune disorder that is associated with inflammation of the salivary (found in the mouth) and lachrymal (found in the eye) glands. First recognised by a Swedish eye specialist, Henrik Sjogren, he reported initially 5 cases of dry eyes and mouth in 1930 and subsequently published his doctoral thesis describing 19 patients with dryness plus arthritis in 1933. This syndrome is seen in 10-20% of patients with longstanding Rheumatoid Arthritis and Lupus. Symptoms may be 'non-specific' but often patients report 'grittiness' of the eyes, with an associated dry mouth.

Joint aches and pains and fatigue are common. Treatments include lubricants to replace lost moisture and in severe cases, eye plugs (punctual occlusion) and a drug called Pilocarpine can be started. Lubricants need to be preservative free as frequent use can lead to allergies. Saliva replacements can be helpful, as can chewing sugar free chewing gum, with lozenges and pastilles if needed. A useful tip is to try a spoonful of natural (sugar free) yoghurt before retiring at night which helps to trap any residual saliva in the mouth.

The Role of the Clinical Nurse Specialist in Sjogren's Syndrome
The type of information and advice a nurse specialist would offer is as follows:

• General advice to avoid dry atmospheres and to humidify rooms
• Chew sugar free chewing gum to stimulate saliva flow
• Shower rather than bath
• Avoid soap
• Wear glasses with side arms to protect eyes
• Provide written information sheets
• 6 monthly dental checks with 3 monthly dental hygienist review
• Vigilant tooth care to prevent dental caries
• Pre-pregnancy counselling if Ro/La positive
• Join a patient support group such as BSSA (British Sjogren's Syndrome Association www.bssa.uk.net) or LUPUS UK

The Lupus Nurse Specialist

The lupus nurse specialist is able to see lupus patients in order to give advice, education, information, support and counselling. This role is essential to help patients understand more about lupus, helping them to recognise any particular complications that they need to be responsive to and in supporting them throughout their journey with lupus. This is a senior nursing role that is available only in certain parts of the country, often only possible due to the allocation of funds by LUPUS UK. The nurse specialist acts as the patient's advocate in situations such as receiving a new diagnosis of lupus, interpreting medical treatment plans, giving information in order to enable individuals to manage their disease more effectively and encouraging patients to make lifestyle choices in order to maximise limited resources.

Examples of specific types of advice for lupus patients include the following:

Lupus Nurse Specialists are able to dedicate time to support lupus patients and their families. Building up partnerships with patients enables a more comprehensive co-ordinated rehabilitation treatment package where patients are encouraged to make positive lifestyle choices to influence their future life. Patients are enabled to make informed choices and appropriate education enriches the individual's experience. Lupus Nurse Specialists need time to continue to research the patient's experience of lupus and how best to tailor the support each patient needs.

Sister Sue Brown Clinical Nurse Specialist in Connective Tissue Diseases, Chair of LUPUS UK Nurses Network Group, Royal National Hospital for Rheumatic diseases NHS Foundation Trust, Bath - 20 July 2007

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