Paediatrics: Juvenile arthritis - Treatment and support

       

Foley, Judith,  Juvenile arthritis - Treatment and support, World of Irish Nursing, Vol 10 (2),
February 2002, pp 27-28.

This month in part two of the Continuing Education model on Paediatrics, we tackle the issue of juvenile arthritis. Previously in this module, part one dealt with assessment and intervention for gastroenteritis in children. Subsequent articles will deal with a range of common paediatric illnesses and conditions including diagnosis and management of type 1 diabetes in children; the asthma emergency; treatment of UTIs; palliative care of the dying child; epilepsy in children; severe constipation in infants and young children; febrile convulsions; eating disorders; and the management of burns in children. Module co-ordinator: Moira Cassidy,. RGN, staff writer with the World of Irish Nursing

Juvenile arthritis is a chronic illness of childhood that is characterised by remission and exacerbation of manifestations. A chronic inflammatory disease of childhood, it has unknown aetiology but many contributory factors have been identified such as physical and psychological trauma, hereditary factors, and deficiency of gamma globulin.  Other theories that have been suggested include autoimmune and infection processes.1

Pathophysiology

Inflammation occurs in the joints, initially localised in the joint capsule. The tissue becomes thickened from congestion and oedema. Inflammatory response follows which invades the interior of the joint along the surface of articular cartilage. This in turn deprives the cartilage of nutrients and the articular cartilage is slowly destroyed. Growth plates may fuse prematurely. Inflammatory changes may occur in tendons and sheaths and inflammation of muscles may also occur.

Classification

George Still first described juvenile arthritis in 1896 and initially his name was attributed to juvenile arthritis in children. Later juvenile arthritis was described as having certain subgroups and the name Stills disease was assigned to the subgroup of systemic onset juvenile arthritis only.  The term juvenile chronic arthritis is now the preferred term in the UK, while in the US the term used is juvenile rheumatoid arthritis. The latter term within the UK only refers to those children who are positive for the rheumatoid factor (RF).2

Some have identified that the process of the disease in children may present in one of three subsets, namely acute systematic onset also known as Stills disease, pauciarticular (oligoarticular) onset and polyarticular onset. All of these subsets have subgroups, with varying joint involvement and manifestations (see Table). Juvenile arthritis can also be classified as follows, systemic arthritis, oligoarthritis (persistent), oligoarthritis (extended), polyarticular arthritis (rheumatoid-factor positive), polyarticular arthritis (rheumatoid-factor negative), enthesitis arthritis, and psoriatic arthritis. Within each classification, the manifestations and joint involvement differs.3

Clinical features

The primary clinical manifestations are arthritis, stiffness and pain. The onset may be particularly rapid or insidious. Pain can affect how the child accepts the disease. Other systems within the body can be affected depending on the type of juvenile arthritis present, which leads to certain systematic manifestations occurring in children (see Table).

Management

There is no specific cure for juvenile arthritis and therefore treatment is supportive rather than curative. Treatment and care are multidisciplinary and family orientated in nature, with an holistic approach involving the relief of pain, preservation of joint function and the prevention of joint deformity. The nursing assessment includes the establishment of the childs general health, the joints involved, the physical restrictions because of joint involvement and the severity of pain. 

Nursing care needs to be related to certain goals, which include the management of systematic manifestations, the relief of pain, the reduction of joint inflammation, the performance of activities of living, and knowledge of the treatment, interventions and prognosis of the disease process. 

Paediatric nurses need to work in partnership with the multidisciplinary team and specialists nurses in order to assess, plan and implement nursing care so that the child and family can learn to deal with the condition. Treatment should include parent and child education in relation to management of the disease, and the promotion of good health.

Management includes physiotherapy, occupational therapy, drug therapy, periodic visual examination and surgery.  Ideally the child is treated at home and only hospitalised during severe exacerbations. 

Drug therapy

The first group of drugs prescribed for juvenile rheumatoid arthritis are the nonsteroidal anti-inflammatory drugs (NSAIDs), namely ibuprofen. If this regime is not successful other drug regimes maybe needed.  The second group of drugs used are the slow acting antirheumatic drugs (SAARDs) and include gold, D-penicillamine and hydroxychloroquine. 

Other drugs include cytotoxic agents but are only used for those children with severe form of the disease and who have not responded to the above drugs. Other anti-inflammatory drugs include the corticosteroids. The use of cytotoxic and corticosteroid drugs are limited because of the long term effects. Any of the above drug regimes need careful monitoring because of potentially severe side effects. 

Characteristics and clinical manifestations of juvenile arthritis related to mode of onset1
  Systemic onset Pauciarticular (Two or three subtypes) Polyarticular  (Two subtypes)
Percentage of patients 30% 45% 25%
Age at onset  Bimodal distribution 
1-3 years of age
Type 1: Less than 10 years
Type 2: Over 10 years
Throughout childhood and adolescence
Sex ratio (female:male) 1.5:1 Type 1: Almost all female
Type 2: 1:9
Mostly female
Joint involved Any
Only 20% have joint involvement at time of diagnosis
Usually confined to lower extremities knee, ankle and eventually sacroiliac; sometimes elbow Any joints, usually symmetric involvement of small joints. Hip involvement in 50% Spine involvement in 50%
Extraarticular manifestations Fever, malaise, myalgia, rash pleuritis or pericarditis, adenomegaly splenomegaly, hepatomegaly Type 1: Chronic iridocyclitis; mucocutaneous lesions
Type 2: Acute iridocyclitis, sacroiliitits common; eventually ankylosing spondylitis in many
Type 3: Arthritis only
Systemic signs minimal. Possible low grade fever, malaise, weight loss rheumatoid nodules, and/or vasculitis
Laboratory tests Elevated ESR, RF negative, ANA* rarely positive, anaemia, leukocytosis Elevated ESR, ANA positive Elevated ESR
Type 1: RF positive
Type 2: RF negative
Long term prognosis Mortality: 1%-2% of all JRA patients Joint destruction in 40% Continuous disease eventually remission in 60% 
Type 1: Ocular damage; functional blindness in 10% 
Type 2: Ankylosing spondylitis
Type 3: Best outlook for recovery
Longer duration, more crippling Remission in 25%
Type 1: High incidence of disabling arthri

Physical Therapy

Physiotherapy and occupational therapy are aimed at the preservation of joint function and the prevention of deformity of joints.  Each of these therapists have specific aims. Physiotherapy focuses on the strengthening of muscles, mobilising of joints and correcting and preventing deformity.  In contrast occupational therapy involves general mobility and the performance of daily living activities.  Splinting and positioning are recommended because deformity and pain may be reduced.  In addition, there needs to be a balance between rest and activity.  Surgery in young children is questionable but joint replacement has proven helpful in older children who are fully grown. ref. 1, 4, 5.

Long term care of the child and family with arthritis

Because of the chronic nature of the disease and its characteristic remission and exacerbation cycle, the child may have difficulties in relation to physical, psychological, emotional and social development.  Many children experience nutrition related problems due to the disease process itself because of treatment involved with the disease.  Schooling may be affected and the child may become dependent on the family unit.

Consequently, the role of the nurse as part of the multidisciplinary team is to help the child and family to adapt to the disease and the restrictions it causes.  Education, counselling, guidance and support are important aspects of the long term care of the child with arthritis.  The nurse plays an important educational role for the child and family regarding the management of the disease.

The child needs to lead as normal a life as possible within the limits of the illness.  Problems in relation to family functioning, schooling and long term care prospects, need to be addressed with the child and family by the multidisciplinary team on a long term basis.  Parents can be made aware of the Arthritis Foundation of Ireland which they may find of benefit, ref 26-28.

 


 

Judith Foley is a nurse tutor at the Children's Hospital, Temple Street, Dublin

References

1. Wong, D. Nursing care of infants and children. 6th ed. St. Louis; Mosby, 1999

2. Leach M. Juvenille chronic arthritis: epidemiology and genetics. Nursing Times 1997: 93 (18): 46-48.

3. Woo P, Weederburn L. Juvenile Chronic Artrithis, The Lancet 1998; (351): 969-973.

4. Moules T, Ramsey J. The textbook of children's nursing. Cheltenham: Shanley Thornes, 1998.

5. Belkengren R, Sapala S., Pediatric management problems. Ped Nursing 1996; 22 (6): 522-523.

6. Maycock J. Juvenile chronic arthritis. Br J Nursing 1992; 1 (5): 223-234.

7. Ryan S. How rheumatoid arthritis affects patients and families. Nursing Times 1997, 93 (18): 48-49.

8. Purdy K, Dwyer J, Holland M, Goldberg D, Dinardo J. You are what you eat: health food choices, nutrition and the child with juvenile rheumatoid arthritis.  Pediatric Nursing 1996; 22 (5): 392-398.

 


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