Regression of Activities - JIA
Katie is a 3 year old girl who attends the GP with a history of reluctance to walk. This has been going on for 8 weeks or so. Mum says that Katie used to walk to nursery, but they now have to carry her as she complains of pains in her knees. She has stopped going down stairs in the mornings, and wants to be carried. She cries when her parents dress her at breakfast time. Nursery nurses report that she is less active and is not keen on going outside during playtimes. She occasionally complains of pains in her knees, but has no other pain. Mum has not noticed any redness or swelling.
She has had no recent infections, apart from a mild cold which lasted a few days, and there is no history of recurrent temperatures or rashes. Katie is normally fit and well, and has had all of her immunisations as scheduled.
The GP notes a swollen left knee, with reduced range of movement. She notes that Katie’s right ankle seems quite stiff, although she is not sure if it is swollen. She speaks to the on-call paediatrician, who recommends referral to the paediatric outpatient unit. Katie is seen on the outpatient unit. She has normal observations, and is afebrile with a temperature of 36.8. Katie is found to have an effusion at her left knee, and at her right ankle and right subtalar joint. She has blood tests done (full blood count, acute phase, autoantibodies), which were normal, and normal x-rays. She is referred to the paediatric rheumatology clinic.
A diagnosis of Juvenile Idiopathic Arthritis (JIA) is made, and Katie is listed for intra-articular steroid injections under general anaesthetic to her affected joints. She is referred to the ophthalmologists to have her eyes screened for anterior uveitis. A physiotherapy review is arranged to follow her joint injections. Blood tests show that Katie is non-immune to varicella (chicken pox) and vaccination is advised.
The nurse specialist liaises with the nursery to support them in their understanding about JIA and how this impacts on Katie's daily routine.
Red flags suggest serious illness.
The red flags in this story that suggest a diagnosis of Juvenile Idiopathic Arthritis (JIA) are
- Morning stiffness
- Behaviour change
- Regression of achieved developmental milestones (e.g., walking down the stairs).
Symptoms of pain and stiffness that are worse in the mornings or after periods of rest, with variability in symptoms throughout the day.
Varicella vaccination is advised because Katie is varicella non-immune. Children with JIA may need systemic immunosuppression (Methotrexate [MTX] and potentially Biologics) and the risk of complications with varicella infection are considerable. Katie has oligoJIA and may well settle just with joint injections - however 1/3 of children with this form of JIA will develop a more widespread form of JIA and need MTX and or Biologics - in such case live vaccines would be contraindicated.
There is therefore a window of opportunity for the vaccination to be done at the same time as she is admitted for joint injections.
Impact of diagnosis upon the family
- Provide family with disease information in varying formats to improve understanding; signpost to further information (e.g., websites).
- Discuss the role of the specialist team and the family know how and who to contact for advice.
Explanation about disease process and management
- Encourage attendance at appointments and aid understanding as to why review is necessary.
- Aim to rationalise appointments if possible to minimise disruption to family and nursery life.
- Warm bath in the morning can assist with improving joint movement.
- Regular pain relief and anti-inflammatory medication (non-steroid anti-inflammatory drugs NSAIDS) may help relieve symptoms.
- An admission to hospital will be required for joint injections at diagnosis
- Liaise with key members of the extended multidisciplinary team - health visitor, primary care team, nursery workers.