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Countdown until WORD Day 2019


Children and Young People Get Rheumatic Diseases Too!
WOrld Young Rheumatic Disease Day (WORD Day)

It was the morning of our son’s 10th birthday. Our preparations were cut short when cries of distress from our youngest child—then almost two—brought me to his bed. I went to check on him and still remember that moment of confusion: Something was clearly wrong. I lifted him from his bed and tried standing him up, only to see him crumple up in pain on the floor. Always busy and on the go, running from the age of 10 months, this morning our youngest seemed quite unable to stand, never mind run or walk. What could possibly be wrong? We quickly undressed him and saw: both his knees had swelled to the size of tennis balls. Our 10 year old celebrated his birthday without us, and we headed to the hospital. Later that day in the hospital, all the tests were clear, and over the next few weeks his knees improved and went back to normal – until the next flare up. Knees, ankles, neck, fingers, jaw—any joint could suddenly become inflamed, sore and painful to use. Because his flare-ups were so sporadic, it took a while to receive a final diagnosis: our son has JIA, Juvenile Idiopathic Arthritis.

What are Pediatric Rheumatic Illnesses?

JIA belongs to a group of Pediatric Rheumatic Illnesses: rare chronic inflammatory conditions of the musculoskeletal system. JIA affects approximately 1 in 1,000 children. These illnesses range from the relatively common to the extremely rare, including: Juvenile Idiopathic Arthritis (JIA), of which there are several subtypes; Juvenile Systemic Lupus Erythematosus (jSLE); autoinflammatory disorders such as Familial Mediterranean Fever (FMF); Rheumatic fever, Kawasaki disease and more. Many of these illnesses are chronic, which means that they persist for many years or indefinitely and need to be managed to minimize their effect on the children’s quality of life.

Rheumatic disorders affect children in many ways. Some systemic disorders, such as SLE and the systemic subtype of JIA, severely impair children’s overall health and wellbeing. SLE, for example, causes damage to internal organs e.g. kidneys, heart and neurologic system. Others are more localized and primarily affect the joints or skin, causing pain and restricted joint movement, hampering daily activities and physical development.

How are they diagnosed?

Because they are so rare, pediatric rheumatologic disorders are often difficult to diagnose and are sometimes unknown to health care providers. This can cause a significant delay before a correct diagnosis is made. Some family physicians do not expect children to suffer from illnesses mostly associated with older people and consequently make erroneous diagnoses, attributing swelling and pain to unreported injuries, growing or psychosomatic pain. I recall the sad story of a young child who went to see an orthopedist with a sore swollen elbow. The doctor placed the child’s elbow in a cast, explaining that it must be fractured (though his mother insisted that her young child had not fallen). When no improvement was seen weeks later, the doctor recast the arm. Eventually the child was referred to a pediatric rheumatologist, who diagnosed JIA and began administering the correct anti-inflammatory treatment. Sadly, it took months to repair the damage done to the elbow by being placed in a cast. The most important treatment for inflamed joints is movement, to keep the joints from becoming fused, which can cause lifelong damage and disability. Another mother told me how she went from doctor to doctor seeking help for her young daughter who cried in pain and refused to walk, only to be accused by doctors of spoiling her daughter, who, they claimed, had nothing wrong with her and must have simply been seeking attention.

Who treats pediatric rheumatic illnesses?

Children must be referred to pediatric rheumatologists to receive optimal care. When it comes to pediatric illnesses, children are not just small adults. The arthritis which affects them is different from adult rheumatism. It needs different treatment, runs a different course and has different complications. One of the most severe complications to look out for in children suffering from JIA is uveitis, an inflammation of the eye which can lead to blindness. This complication does not occur in adults. A pediatric rheumatologist recently recounted the story of a young boy who had been erroneously diagnosed, and subsequently treated with intravenous antibiotics for almost a year to treat his painful swollen joints. By the time he was finally referred to the pediatric rheumatologist, one of his legs was almost immobile from the continued inflammation, and his eyesight in one eye was badly damaged by uveitis.

Another illness classified as a rheumatic disease is FMF (Familial Mediterranean Fever). This condition affects mostly people from around the Mediterranean. Symptoms include (but are not limited to) recurring fevers, joint pain, abdominal pain (due to inflammation of the stomach lining) and a (less common) characteristic rash. A young man, well beyond his childhood years, remarked to a pediatric rheumatologist after years of suffering: “I suffered all these years without knowing what was wrong, and not knowing that one of the world experts in my condition lived across the road from me!” Luckily, more and more doctors are becoming aware of these illnesses.

New medical opportunities for sick children

The last 25 years have opened many new treatment options for rheumatological patients. Previously, medicine had little to offer children, and many spent their childhood in pain, accumulating disabilities and misshapen joints. Just a year ago I spoke with a young woman who showed me her misshapen arm and fingers. She said to me that if she had to be sick, she wishes it would have been now, when medicine could actually help her and keep her joints in full mobility. Today, due to pediatric-focused medical research and the advent of biologic medicines, tested specifically on children, the majority of children who receive medical and other supportive treatment can reach symptomatic remission (but not cure), with little or no permanent damage.

Since damage to joints, muscles and tendons is cumulative, early diagnosis is crucial. Early detection usually provides a better prognosis, while delay, misdiagnosis and subsequent mistreatment can aggravate damage caused by the illness.

What can be done to help children with rheumatic disease?

First and foremost—awareness. If your child exhibits swollen joints, muscle weakness, rash, recurring fever that peaks in the evening, or complains of sore fingers or stiff joints, especially in the morning or after rest, don’t assume that he is acting up. Make certain that he sees his doctor, and if necessary is referred to a pediatric rheumatologist.

Secondly, if your child is already diagnosed with a rheumatological disorder, take full advantage of the excellent medical opportunities your child has to maintain his health. Make sure that your child takes his medicine on time and visit your child’s physiotherapist regularly and keep up with the prescribed exercises. Those exercises will keep your child strong, and make sure that his body has a chance to compensate and recover from the damages of the battle with the illness. Most importantly, don’t forget to take your child to see her doctors regularly—both the ophthalmologist (to rule out the possibility of newly developing uveitis), and the rheumatologist (to make sure the illness is properly managed, and no flare ups are taking place). Finally, remember that you are not alone. Reach out to parent organizations (see https://www.enca.org/) to get the support you need for living with a chronic illness in the house. Above all, remember: you are key to your child’s wellbeing and health!

If you are a teacher with a student who has a Pediatric Rheumatic Illnesses, make sure to speak with the child and his parents, to see if he is managing with his everyday tasks. He might have difficulty writing or carrying his bag. Many children with Pediatric Rheumatic Illnesses find that sitting still for a whole lesson leaves their joints stiff and sore, and they need to get up to stretch during class.

Spread the WORD

Because early diagnosis and treatment are so important, the European Society for Pediatric Rheumatologists, PReS and the European parent/patient association ENCA (www.ENCA.org) are jointly inaugurating WORD Day, “WOrld Young Rheumatic Disease Day”. The objectives of this day are to raise parental and professional awareness to these illnesses, by giving parents and doctors tools to recognize symptoms which require rheumatological attention.

Raising awareness of pediatric rheumatic diseases will help to aid the process of diagnosis, especially of rarer diseases. Information about these illnesses has been updated on a special site (EU project SHARE www.printo.it/pediatric-rheumatology).

International WORD Day will be an annual event that will take place on the 18th of March from 2019 onwards. It is up to us, parents, teachers and healthcare professionals to make sure that they get optimal care as early as possible to ensure that they have the best chance of leading a healthy full life, with minimum pain.

Tsipi Egert, tsipi.egert@gmail.com based on an article published in The Lancet Child & Adolescent Care, online Nov 23.https://www.sciencedirect.com/science/article/pii/S2352464218303742?via%3Dihub

Growing up with JIA – Niamh Costello, Ireland

My name is Niamh Costello, I am 19 years old and I have had Juvenile Idiopathic Arthritis since I was 3 years old. It only seems like yesterday when I crawled into my parent’s bedroom on my hands and knees. I was screaming in agony and I could not walk. I was this tiny frail body with a huge swollen knee that had been bothering me for weeks now.

Looking back, my Mam thinks that I was born with JIA. I was always slow to walk, I would get tired easily and when I could I would crawl and drag my left leg behind me. I was always napping and would be extremely tired after playschool. Although it was my knee that was the ongoing problem I would always point to and hold my leg when it was sore and I would complain of it feeling tired, I suppose I was too young to articulate what pain was.

I was always picking up infections and viruses so when my parents took me to my local GP that morning I was told that it was just another virus, or that I had fallen on it and to go home and rest it. It all went downhill from there.

A couple of weeks later I was admitted to hospital with a rash and a temperature and this swollen knee that was getting bigger by the day. Lots of tests and scans were done and one day, a doctor who had done some of his training under Paediatric Rheumatology, recognised my symptoms and referred me straight to Our Lady’s Children’s Hospital in Crumlin, Dublin. That is where my lifelong journey of JIA began.

I was diagnosed a couple of weeks later and was put on a high course of steroids. At the time there was no paediatric rheumatologist in the whole of Ireland so little was known about JIA and its prognosis. It was going to be a case of trial and error with my treatment. The oral steroids didn’t work so I was given a steroid injection directly into the knee joint and the fluid was drained. And believe it or not that was it for 4 years! I went into remission and I thought I would never have to see a hospital again. Unfortunately I was wrong.

I remember the day that it came back, I was about to go on a family holiday to England. I remember sitting on a picnic rug and screaming because I couldn’t get up off of it and I looked down to see a familiar swollen red knee back again to haunt me. We went on the holiday which was a disaster. I spent most of the day in my 4 year old sister’s buggy as I physically could not walk. I couldn’t stand for long periods of time, I was drained and in agony 24/7. No pain relief was helping and I just wanted to go home. I felt sorrier for my family as I felt like I had ruined the holiday. The trips to hospitals started again and this time I wouldn’t get a 4 year break.

My illness soon started to spread to other joints which made my day to day life that bit harder. Getting up for school every morning was a struggle. I would have to get up a half an hour earlier during flare up times so that my mam could lift me into the bath and help me loosen out my joints sol that I could attend school. At the start, I would stay off school if I was having a flare up and my mam and I would lie on the couch and cry. Why me I would keep asking; when will this pain go away? Life seemed so cruel. My Mam decided that we couldn’t go on like this. From then on, she would make me go to school during flare ups, if it was the case that I would have to use my wheelchair or crutches I was still made to go in! I hated my Mam for that at the time, but looking back now I was a very quiet child so I would have definitely suffered socially and probably fallen way behind in school work. It also made me hardier and nowadays I tend to push through the bad days and try to continue on with my daily routine as best as I can. Meeting my friends and going to school was also a distraction from the pain.

My friends grew up with my life with JIA so they were always very understanding and a great help to me in school. They would sit inside with me during lunch break if I was too sore to go out and play, they would push me in my wheelchair, take down notes for me if I couldn’t write and little things like that made a huge difference to my wellbeing. They will never understand fully what it’s like to have JIA and my how my illness can fluctuate daily, but as long as they are there for me and willing to help me when I need them, that means the world.

Growing up with JIA did bring its challenges. I missed out on lots of things growing up that were important at the time such as school tours, play dates and birthday parties. I started to realise maybe I am a bit different. I hated being the child that had to sit out of PE class or was not able to go to my best friend’s birthday party or go on a school trip. I hated being the only one in the class that had to use a special chair or crutches.

I loved sports as a child but I was always the last to the ball or the last one over the line, again I was the different one. I was advised to take up swimming, so I did and it was one of the best decisions I made. I took to it like a duck to water (literally!). I wasn’t the different one in the class anymore and I quickly progressed, passing out my peers. I was so happy, I loved the water and it was a sport that was really good for my joints which was a bonus. I started taking part in competitions and I loved going into school on a Monday morning and showing my class my medals. I am so glad I found swimming, it just goes to show that just because you have an invisible illness doesn’t mean that you can’t be just as good as something as someone who doesn’t have an illness.

I remember in 5th class we started school swimming so the 5th and 6th class would go swimming together. We were doing relay races and nobody knew that I was training 3 times a week in the pool as I was in a swimming club. Two 6th class girls were the team captains and I remember one girl saying to them, “don’t pick Niamh, she has arthritis so she will be slow”. Well, I don’t think I’ve ever swam a relay race as fast in all my life! I was going to do anything to prove them wrong.

That was one of the turning points in realising that maybe my peers were also starting to realise that I was different and it wasn’t just myself anymore.

I remember the day that I was told I would have to start injections. I was 8 years old and I remember hearing my consultant say “I think we might have to try the subcutaneous route”. All of these questions were running through my head, I was freaking out! What on earth is subcutaneous, I had awful visions in my head and it was only on the way home in the car that I plucked up the courage to ask my mam what it meant and she said the dreaded word: injections. I hated any sort of change, and this was going to be a big one.

Friday was everyone in my class’s favourite day of the week. It meant no homework and no school until Monday. Not for me, I dreaded Fridays. Friday meant injection day, and a weekend of nausea. My mam would do my injection for me and it could take her 2 hours some days to finally pin me down to receive it. My Granny used to have to come over so that I had somebody to cry into.

Thankfully nowadays I do my injections myself and I don’t need anybody to cling onto! I dreaded going into any hospital and still to this day even the smell of a hospital turns my stomach.

I had to learn to grow up quicker than my peers and start making my own decisions, be it big or small. I was always told when I was younger that I could possibly grow out of JIA, so my family lived in hope. We did everything we could to try and keep my JIA at bay but when I was 12 I remember my consultant telling me that I will probably have this for the rest of my life. When you are 12 that is a shock to the system. All these thoughts were running through my head, how can I deal with this pain for the rest of my life, will I have to take injections forever, will I be able to have children?! I hit a slump and I was very down in myself for a couple of years after that.

I had to learn to adapt the life I wanted to the life that I was given. I had to gain some confidence from somewhere and learn to find my own voice to help my through a life with JIA.

I am now 19 and on a drug that is doing wonders for me. I have started working out in the gym and have completed my first 5km race which 5 years ago I would have never have seen myself doing. I am in University and I am studying to become a primary school teacher, which was my dream.

Don’t let your illness define you, you can still lead the life you want to live just maybe with a few adaptions. I always say that I have JIA, but it doesn’t have me.

MY NAME IS LOU

My name is Lou, I am 19 years old. I was diagnosed with SLE and rheumatoid arthritis in 2015 at the age of 15. I had lots of symptoms going on at that time that included; hair loss, pain, fatigue and just generally feeling unwell. It only took me 5 months to get my diagnosis. It was hard to adjust at 15 to my new life with lupus. I had real trouble with school, friends and family who just did not understand what I was going through. There were lots of appointments, blood tests and medications to get my head around. Once the appointments started to settle and the medication began working I was able to return to some normality in my life. There are so many things I can do despite my conditions and did not have to give up. I’ve learnt to take every opportunity that is given to me and to just try things. There is lots I have done that I wouldn’t have if it weren’t for my diagnosis. I’ve got myself an amazing job with children really doing something I love which I only found because I started looking into different careers because of my conditions. I have met some wonderful people with lupus who have had such a positive impact on my life.

I think it is important to share more awareness of SLE because lots of people don’t even know what it is. I knew nothing before I got diagnosed. If people had a better understanding It would make life so much easier and stress free for everyone especially young people. It is a lot to get your head around without having to keep explaining yourself to others.

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