Multisystem Diseases Nursing Care

The following highlight issues that arise with exemplar rheumatological conditions.

More information is given in the General Nursing notes and also highlighted in the Cases for Discussion

Juvenile Systemic Lupus Erythematosus

• The diagnosis of JSLE may occur during adolescence and treatment may compound problems with altered body image; e.g., corticosteroids causing weight gain, acne, striae.
• Fatigue and poor sleep are common with impact on normal routines. Families may need support to re-establish bed time, sleep hygiene and appropriate rest. Pacing and building up of stamina need support from the MDT.
• General health education is important; avoiding smoking (due to risk of thrombosis), wearing sunblock (all year round is advocated) as sunlight can trigger disease activity.
• Menstrual cycles can be affected by chronic illness. Specific advice relating to taking oestrogen/progesterone combined contraceptive pill may be needed as oestrogen may trigger disease flares.

Juvenile Dermatomyositis 

• There is a spectrum of disease severity and the features which are most prominent (e.g., skin, muscles) may vary. 
• Muscle weakness can be prolonged and strategies at home may include therapist assessment and community nursing support. It is important to consider mobility and self-care issues.
• Fatigue and tiredness can result in poor stamina and struggling with full time school. Liaison with school for reasonable adjustments may be required (such as phased return to school and a flexible time table).
• Skin lesions and calcinosis (calcium deposits in the skin resulting in hard sometimes protruding lumps) may increase risk of infection (and abscess) including opportunist infections compounded by immunosuppressive treatments and reduced mobility.
• Sunblock is advised all year round as sunlight can trigger disease flare and the skin is more sensitive to sunburn.

Vasculitis 

• Vasculitis can involve blood vessels internally so may not be obvious on skin examination.
• Skin involvement can be painful in the acute and healing phases so adequate analgesia is important.
• Skin emollients may be needed to keep the skin moist and intact.
• Specialist advice is needed if the skin lesions are broken. There is risk of serious infection that may be increased with immunosuppressive effects of treatment. Opportunistic infections are also possible.
• Skin lesions may look unpleasant; issues around acceptance and looking different from peers need to be addressed with the child and family.  

Scleroderma 

• The impact of scleroderma varies depending upon site and size of lesion. Patients may be embarrassed or ashamed of their lesions especially if they are easily visible, or pigmented.
• Referral to camouflage make-up services may be helpful if lesions are on the face or other conspicuous areas.
• Sometimes cosmetic surgery can be offered (including fat pads being used to replace lost subcutaneous fat layers) and needs specialist consultation to consider surgical risk.
• The affected skin is more fragile and sometimes thickened and stiff. Emollients to moisturise the skin and gentle massage can improve the skin flexibility.
• The affected skin may burn more readily in sunlight so sun block is essential all year round.
• The affected skin may crack or split, can be difficult to heal and be prone to infection. Opportunist infections can be increased with immunosuppressive treatments.
• Growth can be affected – such as foot or hand size, limb length or chest wall deformity. Disfiguring lesions of the face can occur. The impact can be severe with distress and poor self-esteem.  
• Families need support, practical advice (such as footwear) and may need psychology input.

 Fever Syndromes 

• These conditions can run in families, as there is a genetic component. It is important to ask about other family members who may have similar symptoms. 
• As part of the disease, fever can be extreme (>39/40 degrees centigrade) and the patient can appear quite unwell. The family need to be aware of measures to reduce fever and keep the patient comfortable.
• Rashes may cause concern particularly when associated with a high fever but often subside as the fever diminishes.