The following is relevant to all inflammatory rheumatological diseases although the focus is on treatments for Juvenile Idiopathic Arthritis (JIA).
Clinical 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, family life and encouraging the child or young person to achieve their full potential. Too often illness or having and illness can diminish expectations of what the child or young person is capable of as their parents/carers and others including teachers can have a tolerance of non-engagement /achievement.
Monitoring of disease activity and damage is an important aspect of management and involves clinical status, laboratory measures and patient / parent perspectives. There are many tools that are used - some are generic (e.g., Child Health Assessment Questionnaire - CHAQ) and some are disease specific (e.g., Juvenile Arthritis Disease Activity Score - JADAS) and the Systemic Lupus Erythematosus Disease Activity Index Damage Activity Score (SLEDAI) and the Childhood Myositis Activity Score (CMAS). More Information about these scores is available.
Transitional care is important to support young people with chronic rheumatic disease through adolescence and into young adulthood.
Medical treatments include pain relief and for many chronic rheumatic diseases, includes the use of 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. Regular blood tests to monitor the efficacy and safety of immunosuppressive agents are essential.
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 the 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. 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. 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.
- Non-steroidal Anti-Inflammatory Drugs (NSAIDS) and Pain Relief.
- Disease Modifying Anti-Rheumatic Drugs (DMARDS).
- Biological therapies and Biosimilars.
- Tests before immunosuppression.
- Vaccines and immunosuppression - Immunosuppression has an impact on vaccination schedules; live vaccines are contraindicated and specialist advice is often needed depending on the case scenario.
Autologous stem cell transplantation and bone marrow transplantation are used for children with severe resistant JIA.