Life will get better again one day

Emma was 10 when she woke us up one morning complaining of pain in her upper arms and thighs.

A few weeks back she had played a song on her recorder at my father’s funeral in the Netherlands, and in retrospect I had noticed the high colour on her cheeks, but it had not registered. And that morning I wasn’t too worried either: she was a very active boisterous child, who played in the school netball team, so I told her it must be muscle ache and she would feel better soon.

A few days later I walked up the stairs with her, and noticed she had trouble lifting her legs and was out of breath when we reached the top of the stairs. I made an appointment with one of the GPs at the small country practice in our Welsh village. The relatively new young doctor who saw us joked that other than Emma’s vaccination records her file was empty – he knew Emma, but she had never visited the surgery before. Maybe that was why he took the seemingly innocuous symptoms quite seriously. He noticed the rash on her face and a slight lack of strength in her arms and legs. He consulted some books and made an appointment for us to see the paediatrician in the nearest county hospital as soon as possible. He mentioned the word dermatomyositis, but to me that sounded like a skin complaint, so I was not overly alarmed.

The paediatrician told us she suspected Emma had juvenile dermatomyositis and explained it was rare condition and hard to diagnose. Blood tests were taken, and appointments planned in for more tests, to be taken after our short holiday in Spain. As we ran a very busy B&B, we usually planned our holidays in February, the quietest month of the year.

In Spain it quickly became clear that Emma was in fact quite sick – one day, when she was tired of walking and my husband gave her a piggyback she screamed out in pain. We had to find a wheelchair as walking became increasingly hard for her. The rash on her face was now very noticeable. It was around this point I really started to worry about her and just wanted to go home. By the time we got back to Wales it was clear that Emma no longer had the strength to manage the stairs to her classroom (no politically correct disabled ramps in this century-old farmhouse that was our local village school), and from then on she stayed home, for the rest of the school year.

Despite Emma’s very rapid decline the hospital was still reluctant to start treatment until the official diagnosis was made. Understandable on the one hand, as treatment consisted of a hefty dose of steroids to aggressively attack the inflammation, but on the other hand the progression of the illness was so quick that I feel we lost precious time in getting her started on medication, causing her to be on steroids for longer and on a higher dosage.

Emma had to spend a week in hospital to complete all the tests (an MRI scan and a biopsy of her thigh muscle), after which she was diagnosed with juvenile dermatomyositis. While still in hospital she received a few rounds of intravenous prednisone, which made her feel very sick and vomit. She was sent home on a high dose of oral prednisone, which, the Rheumatologist had warned us, would make her feel ravenously hungry and change her appearance.

Despite the medication Emma went downhill for a while longer. She spent a lot of time in bed with fatigue and moved around in a wheelchair. I remember her trying to get up from the sofa to get something from the table and her legs giving away from under her. Devastating to see: from a fit healthy sporty little girl to an invalid in a few months.

The next very worrying symptom was that her voice changed and she found it increasingly difficult to speak and swallow. Eating became a scary experience for her and me as she regularly choked on her food. She developed a very deliberate way of swallowing, which I still catch her doing now sometimes.

Over the next weeks and months she put on a lot of weight and her appearance changed: the moon face, a coarsening of her features, black body hair. Around her hips her skin popped like a sausage, which resulted in painful sores which had to be cleaned and bandaged. Luckily Emma was still at that age where she was childishly unaware of her appearance, but I remember walking in a shopping centre and a man hissing in passing: ‘Ugly’! Emma, stoic as she was, shrugged it off, but I had to fight back tears.

Once a month we saw the Rheumatologist and every time I hoped to hear that we could start cutting back on the steroids. In my recollection that took forever, but eventually we were allowed to slowly lower the doses of prednisone and, after a relapse a few months later Emma was referred to Great Ormond Street Hospital where there was a special JDMS unit. Here she was to take part in clinical trials and was given a combination of Methotrexate and prednisone. She responded well to this, and thankfully has never had a serious relapse since.

In the very beginning we were told that the progress of the illness could go in three ways: it could become chronic, resulting in real physical malfunction; it could come back over her lifetime in relapses; or the patient would recover completely.

Very slowly Emma’s body returned to normal proportions. She had started school again and was able to join in PE and sports. Eventually the only outward signs of her illness were very noticeable calcinosis in her neck and upper arms, and a myriad of stretchmarks on her thighs. After the few relapses in the first two years Emma did remarkably well, and her medication kept the illness at bay. She would never become as sporty and fearless again (she was a good swimmer before her illness, but completely lost that ability), but she joined in at school sports and apart from having to inject herself weekly (and not being allowed alcohol!) she was just ‘normal’ again.

Slowly she was weaned off the Methotrexate, until that momentous day when Emma was 19 and she was off medication altogether. I spent some uneasy months fearing the disease might come back, but so far (she is 25 this year) she has been as healthy as any other young woman.

For a mother it is devastating to see your healthy child decline into illness. How fragile life is. Illness is just around the corner, and why should it affect other children and not yours? Well, that would be the rational approach, one I certainly didn’t have back then. Why did this happen to us, to our beautiful daughter, to me? I was scared and desperate, and the only thing that kept me sane was having three other young children to look after.

Looking back JDMS has made Emma a very strong and confident young adult. Compassionate and resilient. A fighter and a positive person. A joy to be around, and probably tougher than her three brothers. The stretchmarks on her thighs, which will never go away, are her battle scars and have never deterred her from wearing short skirts or bikinis.

And retrospectively there were good times too during her illness. Having four children close together in age and running a busy B&B meant life was hectic. When Emma got ill I suddenly got to spend a lot of quality time with my daughter. We went to aquarobics and physiotherapy. We shopped a lot because every few weeks she had grown another size. We had fun cooking and baking together as she was forever hungry.

But I realise it’s easy to look back in gratitude when your child has made a full recovery. Not all JDMS patients are so lucky. I hope Emma’s story can give hope to patients who are now suffering with this condition and to their parents: life will get better again one day.

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