As a pediatric rheumatology nurse, my visits typically involve more than just the patient. Including mom and/or dad in the discussion often requires a variety of communication and negotiation tools as we try to do what is best for our patient while making sure that all parties buy into important decisions.

One of our more interesting recent cases involved TP, a 10-year-old male who initially was referred to our office complaining that his right shoulder hurt. He thought it was possibly as a result of “throwing around a football too much with my dad.”

TP had a recent episode of left hip pain and a slight fever 3 months before we first saw him, during which time he claimed he “couldn’t walk.” At that time, he was seen in a local hospital Emergency Department (ED), where he was diagnosed with toxic synovitis based on labs and X-rays. He was treated with an IV dose of an anti-inflammatory and instructed to take ibuprofen as needed. According to TP’s parents, this episode was preceded about a week before with a viral illness accompanied by diarrhea.

After his ED visit, TP’s hip pain improved within a few days and his fever subsided. A week later, however, he complained of intermittent left wrist pain, although that lasted only a day or two before resolving.

TP’s only other notable history was an episode of left ankle pain with swelling and warmth during the fall soccer season, which his parents attributed to a possible on-field injury.

Our initial evaluation revealed tenderness at the right bicep tendon insertion site, but no tenderness or warmth in any joints. TP had full range of motion, and X-rays of his right shoulder did not indicate a fracture or any other abnormalities. Results of a basic laboratory panel, including acute phase reactants, were unremarkable.

Finding nothing of significance in our workup, we suggested that TP continue with the ibuprofen as needed and follow up with an orthopedic surgeon if his shoulder pain did not improve within a week.

Three months later, TP was back in our office for further evaluation after conservative treatment suggested by the orthopedic surgeon failed to resolve his shoulder pain. At this visit, TP also complained of intermittent wrist pain and demonstrated decreased range of motion with irritability on rotation. We performed an MRI, which showed fluid distention of the biceps tendon sheath, large glenohumeral joint effusion, and extensive synovitis. More targeted lab testing showed highly elevated rheumatoid factor, anti-cyclic citrullinated peptide (anti-CCP), and C-reactive protein levels.

Based on these results, we diagnosed TP with rheumatoid factor-positive, CCP-positive polyarticular juvenile idiopathic arthritis (JIA), also known as JRA.

Rheumatoid factor-positive polyarthritis is classified as a JIA, representing the pediatric version of rheumatoid polyarthritis. It has an estimated prevalence of between 1-30 per 300,000 children. Approximately 70% of cases of rheumatoid factor-positive polyarthritis occur in females, with typical onset between ages 10 and 12. It is generally a bilateral and symmetrical joint disease, with a distal prominence involving the joints of the hands (wrists and fingers) and the feet (ankles and metatarsophalangeal joints).1 It is the most aggressive type of arthritis in children and carries the highest risk of joint damage.

“…parents often need a little more time to work through some of their own feelings before helping their child cope with their diagnosis. A parent who is not able to cope well with the diagnosis will often see some of their anxiety transfer to their child.”

After making our diagnosis, we began treatment of TP with a combination of naproxen 10 mg/ kg BID, sulfasalazine 500 mg PO BID, and prednisone 5 mg PO daily. We also referred TP to an ophthalmologist for evaluation of possible uveitis, which was negative.

Six weeks later, seeing no improvement, we introduced weekly methotrexate (MTX) 25 mg SC and increased the prednisone to 7.5 mg PO daily. This too was ineffective and we suggested the introduction of etanercept. TP’s parents were initially hesitant about escalating to use of a biologic therapy, so we sat down as a group to go over the pros and cons of our suggestion.

When evaluating and treating any patient with JIA, it is important to recognize that children do not always present with abrupt onset of RA, as is often the case in adults. Making the proper diagnosis can often take a series of visits due to possible viruses, sports injuries, and lack of clear physical findings. Once a diagnosis is made, however, the approach to treating JRA is similar to adults – intervening quickly and aggressively to give the patient the best chance of achieving a rapid remission and preserving joint function.

Certainly, there is added complexity when dealing with parents of pediatric patients, especially when more unfamiliar medications such as biologics are being suggested. As when dealing with any chronic condition, parents often need a little more time to work through some of their own feelings before helping their child cope with their diagnosis. A parent who is not able to cope well with the diagnosis will often see some of their anxiety transfer to their child, possibly resulting in acting out toward medication and blood draws, nonadherence to medication plans, and poorer overall outcomes.

In TP’s case, we could sense the apprehension and fear of his parents when we suggested the addition of etanercept to his regimen and agreed to give them time to think about our suggestion while seeing if a longer trial of MTX would be effective. This is often when parents will ask, “If this was your child, what would you do?” Parents are looking for validation and approval for the tough decision to start a treatment that can lead to serious health sequelae.

Use of a biologic therapy in a pediatric patient is not a decision that should be taken lightly due to safety risks and the support that is often needed from parents to help the child cope with their treatment. Eventually, TP’s parents acquiesced to the use of etanercept due to their son’s rapidly worsening pain. We will know in a few weeks whether it is effective in reducing TP’s symptoms and allow us to discontinue his steroid.

AUTHOR PROFILE:
Cathy Patty-Resk, MSN, RN, CPNP-PC is a certified pediatric nurse practitioner in the Division of Rheumatology at Children’s Hospital of Michigan in Detroit, MI, where she provides medical services to inpatient and outpatient pediatric rheumatology patients.

Reference
1. Orpha.net. Rheumatoid factor-positive polyarticular juvenile idiopathic arthritis. Available at www.orpha.net/consor/cgi-bin/
OC_Exp.php?Lng=GB&Expert=85435. Accessed May 30, 2017.