The following is relevant to all inflammatory rheumatological diseases although the focus is on treatments for Juvenile Idiopathic Arthritis (JIA).
Medical management aims for disease control as soon as possible and to minimise side-effects of treatment or complications of disease. Monitoring includes screening for complications of disease, especially uveitis, effectiveness and safety of medications, physical growth and pubertal status, as well as evaluation of psychosocial functioning, whilst optimising school attendance and family life.
Medical treatments include pain relief and immunomodulatory or immunosuppressive agents that can modify the disease process. It is important to note that many drugs used in adults are not licensed for use in children. The doses are calculated by age / weight and body surface area of the child.
Many of the treatments are administered at home - oral medicines or by subcutaneous injection (Methotrexate and some of the biologic therapies) and parents and children themselves can administer medications. Patients are generally reviewed in the outpatient clinic either in the specialist centre of outreach or shared care setting. Some specialist nurses can provide a nurse-led service addressing specific aspects of care. Day-case facilities are important for delivery of infusions such as biologics as well as joint injections given under general anaesthetic or inhaled analgesia.
In-patient care is necessary for patients who are being assessed or treated; e.g. intravenous corticosteroids as part of induction for disease control, or as a day case for some therapies (joint injections under anaesthetic or using inhaled analgesia) or some biological treatments. This may be delivered at the specialist centre or at local hospitals. Nursing staff will require education and support from the specialist team to deliver treatments safely and effectively.
Links with school, community care and primary care are essential to supporting the child and family.
Patients on immunosuppression can become unwell very quickly. Recognition of the sick child is important especially for those children who are immunosuppressed as classical symptoms and signs may not be apparent. Urgent referral is warranted if infection is suspected and families need to know when and how to seek health care.
Adjuncts to medical treatments include physical and occupational therapies as well as psychological support. Complementary therapies are not recommended in the management of rheumatic diseases and are not included in this module.
The sub-modules give further information about the medical treatments and also the tests that are needed before immunosuppression starts.
Regular blood tests to monitor the efficacy and safety of immunosuppressive agents are essential. Immunosuppression has an impact on vaccination schedules; live vaccines are contraindicated.
Autologous stem cell transplantation and bone marrow transplantation are used for children with severe resistant JIA. A short video is available to describe the procedure and the story of a young boy with severe systemic JIA who had the procedure.