An physically active twelve-year old girl felled by almost continuous fever for a month; an 11-year old boy whose feet and heels began to hurt, then swell, along with his knees. A 2 year old toddler who was often ill with fever, and then, began limping even before she could really even toddle.
Disparate symptoms of a varied and multi-faceted disease, known as Juvenile Idiopathic Arthritis, or, JIA. As is often the case in medical practice, the label is nothing more than a description of symptoms of the illness.
This chronic disease is called “Arthritis” because it is characterised by persistent joint inflammation, and “Idiopathic” is used because it means that we don’t know the cause of the disease. It is called “Juvenile”, because symptoms appear before 16 years of age. Within this broad category, there are sub-categories which differentiate the disease according to which part of the body is affected and for how long.
Although JIA is considered the most common form of arthritis in children, it is in itself a rare disease that affects about 80-90 per 100,000 children, and this rarity results in confusion and delays in diagnosis when symptoms arise. The illness now more widely known as JIA was previously referred to Juvenile Chronic Arthritis, or Juvenile Rheumatoid Arthritis. JIA is presents symptoms that are differ from arthritis experienced by adults.
JIA is now an umbrella term which covers oligoarthritis, polyarthritis, systemic, juvenile psoriatic, enthesitis related arthritis and undifferentiated type.
Doctors diagnose someone as having JIA when the onset of the disease is before the age of 16, arthritis lasts for more than six weeks and the causes are unknown (which means that all other diseases responsible for arthritis have been ruled out).
The arthritis must be present for more than six weeks in order to exclude forms of temporary arthritis that have been known to follow viral infections.
The diagnosis of JIA is, therefore, based on the presence and persistence of arthritis and the careful exclusion of any other disease by medical history, physical examination and laboratory tests.
Aliah’s story :
Aliah Amirah’s journey to the hospital which finally diagnosed her as having Systemic JIA at age 12 years began with a series of visits to general practitioners to treat almost continuous bouts of fever over the period of one month, before a visit to the hospital led to a two-week hospitalisation, where a battery of tests ruled out other possible illnesses, like leukaemia. Steroid treatment was started and there was steady improvement, but no miracle cure – she continued to feel unwell, only less so.
Now, more than six years after her initial diagnosis, Aliah has the upper hand on her illness. Consultant paediatric rheumatologist Dr Tang Swee Ping, who has been treating Aliah holds her out as a model patient, for the responsibility she holds over her own treatment regime. “The medicines have reduced over time. My daily dose of tablets has gone down from 10 to 4 now,” she says with a degree of satisfaction.
Aliah’s independence stems from the support and encouragement of her parents. She practices what her father taught her : “As far as possible, I do things for myself, and only when I find I am unable to, do I ask for help.”
With this, Aliah managed to take over the responsibility of her own injections when she switched medications two years after her diagnosis. Etanercept required twice weekly injections, and it was impossible to work hospital visits into her school schedule, so Aliah learnt to self-administer her injections when she was 14, and went on to boarding school at the age of 16.
She counts her parents as providing the greatest support at the most critical moments. Taking steroids, with the resulting weight gain and acne, added greater social pressure on what is normally a difficult period for any teenager. “I do remember once, feeling terribly depressed by the medication and seemingly endless problems of the disease. I locked myself in my room to cry, I felt like stopping all treatment. My mom found out and sat me down, and gave me a pep talk. She listed all the reasons why I should not give up, she encouraged me. Since then, I have tried never to have negative thoughts and just focused on trying to improve my situation,” she says resolutely.
Sasi : From bench warmer to Hockey Captain……………
Sasidharan, or Sasi to those who know him, spent his early teens watching sports from the sidelines. “I would sit there, just hoping, one day, I could do the same,” says this bubbly and cheerful 18-year old.
For years, Sasi suffered from swollen ankles and knees, unable to walk more than a hundred metres before having to sit down for a rest, because of the intense pain he experienced almost constantly.
“It began suddenly when I was 11, first my feet and heels began to hurt, and then my knees swelled really large and I could barely walk,” he recalls. Extensive tests, including taking a sample of bone marrow, at the General Hospital in Seremban yielded no conclusions. Further consultations at the paediatric clinic were also futile.
For the next three years, Sasi endured the almost constant pain in his legs, and learnt to live with the limitations presented by his condition. “My parents spent a lot over that time, on traditional healers, and I did not get better,” he says. Despite which, he tried to keep things as normal as possible, with the help of his parents, and younger siblings. “We still did normal things, like taking family holidays, I would just rest a lot in between walking.”
Sasi and his parents never gave up, and they continued to seek medical advice, but it was not until three years after the onset of his symptoms that he finally was diagnosed with JIA. His responded positively to a regime of Methothrexate, although it was not easy taking the 6-tablet weekly dose, “I felt very nauseous,” he says, “but my mom helped me.”
A year of medication and treatment cleared up Sasi’s illness and he was, as he’d hoped to be, normal. Indeed, he managed to be made captain his school’s hockey team by the time he was 17. And today, other than a six-monthly blood test, he is like any other 18-year old, pursuing a diploma in medical lab technology, with hopes of taking a further degree overseas.
Liberty : Aged 2 years
Liberty’s story of parental persistence mirrors that of Sasi’s. Terence Beck, Liberty’s father recalls five months of frustrating to-ing a fro-ing, where “We tried almost everything family and friends suggested: bone specialist, neurologist, Chinese doctors. Sometimes the opinions are so contradictory, it was very frustrating. We felt that we were no closer to getting to the bottom of our daughter’s problem. ”
All they had to go on was Liberty’s frequent fevers, and a limp caused by a stiff knee.
She was only 2 years old then, and would cry in the mornings when she woke with a stiff knee which would gradually loosen to allow her to walk later in the day. So it was with great relief that a full diagnostic test run in Pantai Hospital led them to Selayang Hospital, and Dr Tang, who began treating Liberty with steroid injections to the affected knee, and pain medication in the form of ibuprofen. Now, Liberty has moved on to Methothrexate and despite the concerns of toxic build-ups and resulting blood tests to monitor this, Terence says, “We feel it was a good move. Liberty is 4 and half years old now, it has been more than 2 years. Her legs are now straighter. She walks without a limp and doesn’t tire easily as before. She looks like any other child in kindergarten, very active and playful. No one would have guess that she has this condition.”
In the case of very young patients, parental involvement is at its highest, and it is often their keen observation which leads to an early diagnosis. They also have the painful responsibility of putting a child through treatment which is hard to explain to a young child.
Says dad Terence : “(Liberty’s illness) put a lot of mental stress on us. The entire family was very worried that whatever she has might handicap her for life. My wife applied a lot of long leave from work just to care for her. She and my mother were emotionally affected the most during that difficult period. More so when we found out that not only was her left knee, but her right wrist, ankle and toes were also showing symptoms of JIA.”
What has helped is the progress that Liberty has shown under treatment, and the hope that she will one day outgrow the disease. In Terence’s words : “It was painful to put her through all those diagnosis, but I think our persistence in finding a solution paid off. All we wanted was for her to have a normal healthy childhood and we believe that we’ve somewhat achieved that for her. “
Resources for JIA :