A Parents’ Experience Getting their Daughter on the Right Treatment for JRA-Associated Uveitis

We finally asked the pediatric ophthalmologist a very difficult question, but one all parents of children with JRA-associated uveitis should ask.” How many patients with JRA-associated uveitis have you treated till it ‘burned out’?”
Submitted: May 2001

Demi was diagnosed with pauciarticular JRA (ANA+) when she was 2 years and four months old (1997).Only her left knee (swollen, painless with a limp) was involved at that time and it quickly resolved.The rheumatologist told us that she should have her eyes checked every 3 months for uveitis (“what is THAT”,I wondered) by apediatric ophthalmologist.We were fortunate that the pediatric ophthalmologistwas very patient with Demi so that she became accustomed to having the slit lamp examination done.

I was away on a skiing trip 23 months later (1999) when I called my wife and she told me the doctor had found some inflammation (1+) in DemiÃs right eye and that DEMI had to start pred forte(PF) drops and mydriacyl right away.I told my wife I would do everything and anything to prevent Demi from losing her vision.

When I spoke with the pediatric ophthalmologist he said hers was a mild case and she would be fine.I then went to the internet to find out what this disease was and it’s treatment.I was quite surprised when I read about many children losing their vision from this disease and that the mainstay of treatment was steroid.I was fortunate to come across an article by C. Stephen Foster, MD, in the Journal of the American Medical Association titled “Saving the Vision of Children with Juvenile Rheumatoid Arthritis”.When I read that I realized how serious this disease was and that other drugs might be necessary to avoid the potentially blinding complications often caused by a steroid only approach.

I found the MEEI website, http://www.uveitis.org/, and printed out everything written by Dr. Foster and brought it to the pediatric ophthalmologist who told me DemiÃs case was not that severe.At that time Demi was inflammation free on 4 drops of pred forte a day and the plan was to taper the drops. Unfortunately when the drops were tapered she flared.So off we went to Disney World with Demi on 6 drops per day.When we came back the inflammation was down so we tapered to 4 and then the plan was to taper again.However the same thing happened; DemiÃs eye ûflared” again.

I brought up Dr. Foster’s treatment algorithm again, and possibly seeing him for a consult, but was told that Demi’s case was “too mild for Dr. Foster to treat”, and if we tapered her more slowly off prednisone she would be “fine”.We took Demi for a consult with a uveitis doctor at Wills Eye Hospital who did not seem to be overly concerned with tolerating low grade inflammation.They advised us that . . . long term use of steroids was O.K. and that surgery could “fix” a cataract.Methotrexate (MTX) was to be used “rarely if ever” on children . . .That was enough for me.When we told him we were going to see Dr. Foster, he wanted to know what he thought.

At that point we took Demi to see Dr. Foster for a consult. Dr. Foster wrote a letter to the pediatric ophthalmologist and rheumatologist to start her on Naprosyn and hopefully that would prevent her uveitis from flaring as the steroids were withdrawn; if that didn’t work then Tolectin, and if that didn’t work then MTX, but he would like to evaluate her before that would take place.Dr. Foster was more than willing to collaborate in my daughter’s care. So, back on pred forte drops 4X a day.

As I spoke wth the rheumatologist and pediatric ophthalmologist they did not seem to want to move forward according to the treatment algorithm advocated by Dr. Foster.And, the pressure in Demi’s right eye had risen into the mid 20’s.What concerned me was that the pediatric ophthalmologist said … if the pressure continues to rise we’ll just add another drop to bring it down.  I had read that the leading cause of permanent visual loss in children with JRA-associated uveitis is glaucoma.And, I read that cataract surgery on children is not nearly as successful as on adults.  I had the feeling that this doctor was only comfortable with a steroid only approach and any complications from such would be handled with more drops or surgery.When he recommended that Demi be checked again in 8 weeks I knew Demi had to see Dr. Foster.

As a dentist I knew the long-term problems of systemic steroid use and I knew that a joint could only be injected so many times in a year with steroid without causing tissue  damage.And, as a person with psoriasis, I knew injudicious use of potent topical steroids to the skin causes thinning of the skin.Why should the long-term use of topical steroids to the eye be different?

I finally asked the pediatric ophthalmologist a very difficult question but one all parents of children with JRA-associateduveitis should ask.How many patients with JRA-associated  uveitis have you treated until it has ‘burned out’?The doctor replied that he had treated 3 children during his medical training.I appreciated all this physician had done for my daughter but felt that in order to save her vision she needed to be treated by a sub-specialist and a physician who was treating these patients all of the time; Dr. Foster was my first choice.

My wife initially took Demi to Boston by herself every 6 weeks.The next NSAID Dr. Foster suggested we use was Tolectin.Dr. Foster told my wife that if that did not keep the inflammation quite ‘we’ would have to have a discussion about methotrexate.Dr. Foster also told my wife that the next five years would be critical in terms of Demi’s treatment.When I heard that I realized I would have to go to Boston the next time because, more than likely, Dr. Foster would be recommending that Demi start on methotrexate (MTX).

I did PubMed searches on uveitis and methotrexate and JRA and methotrexate.What I found reassured me than MTX was probably bettered tolerated than systemic steroids.And, I found that MTX DOES NOT CAUSE CANCER.I bought medical textbooks and got a hold of journal articles about children taking MTX for autoimmune mediated diseases.I spoke with a colleague who gave me the name of a physician at Wilmer.I e-mailed Dr. Phillip Dunn who stated that they often use MTXin children with uveitis.However, Demi’s rheumatologist, at that time, was against it.When we saw Dr. Foster in late November 1999 I brought a list of questions with me.Dr. Foster answered them all.Demi’s right eye had 1+ inflammation and Dr. Foster suggested that Demi get any vaccinations she needed and start on MTX 3 weeks after those were completed.

As I thought about it, and after I gathered more information, I realized that Dr. Foster’s treatment algorithm for treating uveitis is the same as the algorithm for treating JRA.Why would doctors use MTX for jointdisease and not eye disease?Most joints such as the knees,hips, and shoulders can be replaced.The EYES cannot be replaced!When I discussed this with the rheumatologist he agreed.I then decided we would need physicians locally with extensive knowledge and experience prescribing MTX in case Demi had a problem.So, we scheduled an appointment with the pediatric rheumatologist at Childrens Hospital of Philadelphia.

In December 1999 Demi stared methotrexate.In January, when we went to the pediatric rheumatologist, they stated that many of the patients with uveitis are on MTX and that it is a safe drug if monitored properly.The doctor, who trained a Denver Childrens,was very familiar with Dr. Fosters work and had participated with him in the care of some uveitis patients while in Denver.Finally.Someone who concurred!

Demi has been on methotrexate now for 18 months.She has had to use steroid drops once for 8 weeks due to a flare possibly triggered by one cold after another, or just simply that in that year she had grown.She now takes 10 mg once a week, by injection.She has had no side effects.We look forward to the time when we can start to taper the methotrexate.When we first started MTX we asked Dr. Foster what Demi should avoid doing.Dr. Foster’s response was,”just let her be a kid”. During our most recent visit to MEEI the Fellow/Resident questioned us with regard to any vision loss or complications.He seemed surprised when we said “none”.Clearly, many children who get, finally, to MEEI are seen after the development of cataracts, or glaucoma or other complications.Thankfully, for Demi, all that has been avoided because we followed the treatment advocated by Dr. Foster  TOLERATE NO ACTIVE INFLAMMATION OFF ALL STEROID!

Link to Photo Essay of Demi’s Trip to Boston

Glenn

CLICK AND READ . . .

How often should my child get an eye examination if they have JRA?

Juvenile Rheumatoid Arthritis and Uveitis

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