"JDM has impacted on our family life but we try to not let it rule us"



Charlotte is our first child and we were living in Scotland when she was born.  My husband was in the Royal Navy and was based up there; I joined him after graduating from university.  We married in 2004 and at the end of 2005 we were overjoyed when Charlotte joined our family. She wasn’t an easy baby, having a cow’s milk protein intolerance and gastroesophageal reflux made things tough initially but after the first few months things got easier.  Aside from these issues, which are fairly common in babies and an issue with reduced hearing in one of her ears, she was a bouncing and healthy baby.  She had a strong personality, something which remains evident now.

In the spring of 2008, we went to Chicago to visit my husband’s sister and her family.  Around this time Charlotte started to show some signs of a rash on her arms. It was fairly mild until the end of June, when whilst visiting my best friend in England, I noticed that the rash on her arms and hands had got much worse and her face was really quite red.  This coincided with a spell of very hot weather.

We had been to our GP and practice nurse on a few occasions about the rash; we’d been given some steroid cream and advised to use oral antihistamines to try to settle it. Nothing worked though and I was concerned; the GP agreed to refer us to Dermatology at Yorkhill Hospital for Sick Children, although I later found out the wait would be six months.

In the middle of July, when Charlotte was two and a half, the rash was worse than it had ever been, with what looked like blisters over her finger knuckles. It was around this time that Charlotte started asking to be carried when we were out walking and taking longer than normal to climb stairs. I knew this wasn’t right – I knew in my heart that the rash and the weakness were likely to be tied in and as a children’s nurse, I found people were expecting me to have answers. We had another frustrating visit to the GP around this time, with no helpful suggestions.

I was at a friend’s house, a colleague, who was on maternity leave from her job in paediatric A&E at Yorkhill one day.  Charlotte was utterly miserable; clingy, weak and the rash was awful.  She seemed puffy too, on her face and arms.  It was this friend who urged me to bypass the GP and take her to A&E.  I needed that push; to tell me that I wasn’t over exaggerating the problem. We went home, I packed a small bag and we went to A&E later that afternoon. My husband was in London, at a job interview. We were due to relocate back home to Surrey as he was retiring from the Navy after twenty two years service. I remember not wanting to worry him.

I remember it being quiet that day in A&E.  I’d not been to Yorkhill before as a parent but had transferred children there from the ward I worked in Paisley. We were clerked and shown to a bed space. There were toys but Charlotte was not interested in playing. She was tired, just wanting to sleep and struggled to walk across the room when I was demonstrating to the junior doctor what was wrong.

By the time they did bloods, it was early evening.  Charlotte slept and I sent my mother a message, which I found so upsetting to do as I knew how worried she would be. The doctor came to speak to me a while later, to say that Charlotte’s blood results were abnormal. Her Creatine Kinase was very elevated at 4000 and other inflammatory markers were high. I was upset but didn’t want to wake Charlotte.  I have a clear memory of asking what she thought it could be but she didn’t know.

We were transferred later that evening to ward 7B, a medical receiving ward. We were put in a side room and Charlotte continued to sleep. I sobbed down the phone to my husband, who was flying back to Scotland, giving him a list of some things to bring in for us.  He came late that night and stayed for a short while. I was exhausted and just wanted to sleep.

The next morning we were seen by Dr Davidson, a rheumatologist and her colleague, Dr Galea.  Very quickly they told us they thought Charlotte had Juvenile Dermatomyositis.  I was given some information from the Muscular Dystrophy UK website and told about treatment – pulsed IV steroids, followed by oral steroids and then methotrexate.  We were transferred across to ward 7A, which was a medical ward with overflow from the oncology department and had children who required longer stays.  Cannulating Charlotte proved to be an ongoing issue – she was puffy on all the areas generally used and the cannula did not last long when there was success with getting it in.

We were allowed home after the second night, on the proviso we attended the day ward each day for review, completion of the IV steroids and then to start the methotrexate.

Unfortunately it became fairly quickly apparent that Charlotte was not improving like the doctors had hoped. She developed swallowing issues, with fluids she swallowed dripping out of her nose, was increasingly weak and was having a tough time with constipation – all related to the muscle weakness. She was readmitted to 7A and had a nasogastric feeding tube passed.  She was not able to support herself in a sitting position, to roll or even lift her head off the bed. I found myself handling her little body like I would the children with special needs where I worked: turning her regularly, changing her position so she didn’t get stiff and keeping her muscles gently active.

Dr Galea and his team started Charlotte on IV cyclosporine that day.  The day following that, they changed it to cyclophosphamide and intravenous immunoglobulin (IVIG), telling us that these were the hardest drugs and would hopefully hit the JDM with as much force as possible – I had read that was thought of as the best way to treat it, to hit it hard.  I was scared though, so scared.  I could see how concerned the staff were and remember that first night Dr Galea quietly saying to Charlotte’s nurse to keep a very close eye on her, should she need to be transferred.

At this point it was decided that due to it being almost impossible to get a peripheral cannula into Charlotte, it would be best to have a central venous catheter placed.  The operation to place her Hickman line was straightforward but emotionally for me it was one of the hardest moments.  I struggle to remember that day without crying; remembering leaving her in the anaesthetic room, walking along the corridor alone and crying so much, at how unfair it was that my baby was so poorly.
The Hickman line was fantastic. It meant no more pinning a screaming Charlotte down to try to get venous access and no needles at all.

Within a week of starting the IVIG and cyclophosphamide, Charlotte was showing signs of slight improvement.  We were in Yorkhill for around four weeks that time and we were so, so well looked after.

I left the hospital once, to visit my GP for a sick line for work and couldn’t get back to Charlotte quickly enough.  Of course she was ok.  During these weeks we had visitors.  My husband was on terminal leave from the Navy – a period of transitional leave, before he completed his service – this meant he was able to visit us most days which kept us going.  Of course there were still practical things that needed doing and those jobs fell to him – along with a bag of washing he took home daily!  My mum spent a while visiting and that took some of the pressure off – especially by entertaining Charlotte when I was too exhausted to do so.  These visits meant I could go to the canteen knowing Charlotte wasn’t alone, make telephone calls and share the load a little.    My best friend visited for a night – we went out for lunch to a café down the road and I could barely relax, constantly checking my phone.  Of course Charlotte was fine then too.

Time passed slowly and by early September Charlotte was able to be discharged.  By now she was slowly remobilising, shuffling along on her feet again.  The skills came back quickly, quicker than I thought they would.  The bigger, more central muscles like in her thighs, tummy and neck were the last to get back to normal- they took quite a long time.  It was good to be home, despite the need for frequent visits back to Glasgow for her infusions.  Whilst her Hickman line (nicknamed wiggly) wasn’t in use, she had a ‘wiggly bag’ which hung around her neck under her clothing. We could pop the end of her line in the bag and it would stay clean and out of the way.

Charlotte did well during the six months of cyclophosphamide and IVIG.  Unfortunately, after they had finished, she slowly flared.  During the six months we had moved house from Scotland to Surrey and also I became pregnant with Charlotte’s sibling.  The new consultant was Dr. Pilkington at Great Ormond Street Hospital.  By the August of 2009 it was clear we needed something other than the oral steroids and methotrexate.  Charlotte’s skin rash was worse, having got considerably better and she was having some muscle weakness again.  She was started on infliximab, which was given intravenously and four weekly, on Penguin Ward.  The infliximab worked, although it took a little while to show.  Dr. Pilkington also started Charlotte on hydroxychloroquine around this time, as it can help the skin rash side of things.

Since then she’s gone from strength to strength, quite literally. The infliximab was changed to adalimumab (Humira) in early 2011 as Charlotte had developed a needle phobia and we were having long days trying to get her cannulated each month (the Hickman line had been taken out in February, after she stopped the cyclophosphamide and IVIG).  Adalimumab is given as a subcutaneous injection fortnightly, so our community nurses were able to do that alongside the weekly methotrexate injections she has. She is almost off the steroids now and we pray that she manages to reach remission some time not too far off. Her energy levels are amazing, she doesn’t fatigue easily and I’m thankful for how well she has done.

JDM has impacted on our family life but we try to not let it rule us.  We are very, very careful with sun exposure, as I’m sure it is a contributing factor for Charlotte’s JDM.  We make sure she’s well-covered with clothing and apply sunscreen regularly throughout the day, year round.  Her school are very understanding and apply it there too. We tailor our family holidays and days out to factor in the issue with UV exposure but it’s easily manageable. The hospital visits aren’t as frequent or as long now, which is helpful as I have a toddler in tow too. She takes her medication very well and the injections we are making progress with. No longer do I have to extract her from under her bed, when the community nurse rings the doorbell.  It’s been so hard at times but I’m proud of how Charlotte has coped and of how she’s become such a lovely little girl, despite having had a tough four years. She doesn’t really have any memories of a time before JDM and we hope soon the memories of the harder years will be fading in the distance also.
Kathryn, June 2012

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