E’s Story

Our granddaughter E aged 4 lives in central BC with our daughter S and son in law M who are both veterinarians and her big sister C aged 7. In early March 2009 she developed a rash and sore throat, was seen at the local walk in clinic and given antibiotics for a suspected strep throat. 48 hours later the rash was much worse, a visit to ER brought a confused diagnosis HSP? Glandular Fever? Scarlett Fever? probably Scarlett Fever. Back home for another 24 hours. By now the strawberry tongue and pale face mask associated with Scarlett fever had developed along with swollen joints and abdominal pain. Back to ER this time to be admitted. That was when the full set of HSP symptoms hit hard. Arthritis, edema, severe abdominal pain (IV morphine couldn’t deal with it), bruising wherever touched and blood in stools urine and vomit. They got her stabilised and through this first crisis.

Eleven days later, Sunday 22
nd March, she went home under the care of her parents who as I mentioned are both vets and so have a high level of medical skill. She was back in hospital every day with various problems including ear pain (two burst eardrums) and a “dry” or sterile meningitis. On Wednesday evening she was admitted again with uncontrollable vomiting and renewed abdominal pain. On Thursday morning the hospital needed to set up an IV drip to get her rehydrated and that is when the real problems began. E had developed severe vasculitis, her veins were so inflamed and fragile that getting a line in proved to be easier said than done. In fact it couldn’t be done and after many attempts under sedation they gave her subcutaneous fluids and morphine, very painful. That was Thursday. By Friday morning E was starting to drift away. She suffers from a condition called Ketotic Hypoglycaemia which basically means that if she doesn’t eat she goes into a coma. This is not normally a major problem but by now she hadn’t eaten since Wednesday. S had difficulty getting through to the nurses that E was not sleeping peacefully but was unconscious. There were now several more attempts to set up a drip, all failed. The hospital now realised that this case was beyond them and E needed to be at “Sick Kids”, the children’s hospital in Vancouver. The problem was that by now she was so far gone that the journey itself could be too much. It was at this stage that my wife had a phone call from our daughter with a very flat voice saying “she is only little and she has had enough”. That is something that believe me you really don’t want to hear. One last ditch attempt was made to get a line in, this time under general anaesthetic into a deep vein. She was rushed to theatre, where the anaesthetist said she was too dehydrated to survive an anaesthetic. That seemed to be that, but an anaesthetic technician had one last attempt to get an IV line in. This one was successful. Ten minutes later when S phoned us again there was already a marked improvement, with some response though not yet full consciousness. From then on things moved really fast. 20 minutes later they were in the air on their way to Vancouver by Medivac Lear Jet to be met by a full emergency paediatric team. The line had come out on the way to the airport, but they quickly put another one in. It was easy to count the 27 previous attempts as each left a large bruise.

The level of care then from then on was just superb. E had a dedicated team from each of the disciplines ending in ology, about five of those, and a paediatric team coordinating the whole thing. She was soon up and about though still a very long way from well. At one time there were 8 doctors in her room, but the paediatric consultant told them all to leave her for a few days of R&R to recover from both the physical and the emotional trauma before starting any more tests and investigations, of which there were to be many. This is a world class children’s hospital, they treated the whole family. M & C flew to Vancouver on Saturday and stayed in hospital sponsored accommodation. C was seen by the hospital social and psychiatric staff and attended the hospital school.

By Wednesday things had improved considerably. E was up and about and playing happily but still had sudden bouts of extreme pain. These seemed to strike random parts of the body needing morphine to control them. Between these attacks she was so bright that one of the paediatricians wanted to discharge her. This was fortunately vetoed by her paediatric consultant, once again showing how essential it is to have someone with experience and the big picture in charge of the case. The various “ology” teams also seemed happy with her, in particular nephrology. There was minimal kidney involvement so far, they think the blood in her urine was from her vasculitis rather than kidney damage.

On Friday E was allowed to go home. Following lengthy discussion with her consultant in Vancouver it was decided that the psychological benefits would be considerable and that M & S were quite able to handle her heavy drug regimen once she was stabilised. Their local hospital was “spoken to” by the Consultant as was the on duty paediatrician. M & S had the Consultant’s pager number and his secretary’s direct line. It was unlikely that the situation would be allowed to develop so far again, if there was any sign that it might, Vancouver is a five hour drive or 50 minute commercial flight away, (20 minutes by Lear Jet!) They would have been on their way very quickly. They are very aware of the potential kidney problems. Ironically S has only one kidney herself as a result of a totally unconnected condition, so will not let E go unmonitored. The first follow up appointment with the Paediatric Nephrology Consultant was booked before leaving Vancouver.

E was far happier at home in familiar surroundings with her whole family including dogs and cats. She still had joint and gut pains but they were soon manageable without morphine. There were still good days and bad days. If she overdid it she would get hives and if she really overdid it the purpura would return. She developed the moon face associated with high doses of steroids, which were so high that they also temporarily stopped her growing. It took a long time before she could be weaned off all her drugs. A month later in early May she was still on blood pressure medication, naproxen for her joint pain, and of course the steroids. Her blood pressure was once again normal having been dangerously high in spite of medication at the height of things and her urine was testing free of blood and protein. By the end of June she was pretty much off her drugs and almost but not quite symptom free. When tired she would walk on tiptoes as this hurt her joints less, also her stamina was greatly reduced from its pre HSP level.

The latest major milestone as I write this in August 2009 was her follow-up visit to the Paediatric Nephrology Consultant in Vancouver. This took place at the end of July and was all good news. In spite of her “dramatic” initial HSP presentation her kidney involvement was actually fairly minor compared with cases they often see. She has no signs of any lasting problems, with however the caveat that this is HSP and may return. In any event she will have regular monitoring, probably for life. Six monthly checkups initially, reducing to annual, with the continual awareness that kidney problems as a result of HSP can crop up many years down the line.

This was a near vertical learning curve for us all. We consider ourselves to be a fairly medically aware family. My wife is a retired dentist and our daughter and son in law are both vets. This came at us from nowhere. None of us had heard of HSP or had the slightest idea just how brutal it can be. So what have we learned? The most important thing and it really is vital in the true meaning of the word is that there is someone in overall control of the case, someone with sufficient experience to know what to expect, and access to the full medical history. What is really needed is what M calls “joined up” medicine. Everyone has to be kept in the picture and work as a team. E’s case was complicated by the difficult diagnosis. Perhaps Scarlett Fever hit at the same time as HSP. We will probably never know. Also nobody factored in the effect of her hypoglycaemia. With 20/20 hindsight she should have been in Vancouver at least 48 hours earlier, but that is now history.

Finding this site was a great help, particularly for me as a non medical member of the family. M and S are fairly recent immigrants to Canada from UK where we still live. The whole business as far as we were concerned unfolded through frequent telephone calls and emails. We did consider hopping on a plane and heading across the Atlantic, but realised that we would be unable to provide much in the way of material help beyond that already being given by their large group of friends, neighbours and work colleagues. We would probably still be getting most of our information via the telephone. Also we had other serious family reasons to be in UK at the time. We did make it to Canada for last month’s follow up. I found that it helped me enormously to let off steam by writing things down and sharing them. HSP, according to a friend here in the UK who is a very experienced nurse and now practice manager for one of the larger GP groups in the area, can be something or nothing. It can be a few days of general unwell with a bit of a rash or the full works. E certainly had the full works. One of the paediatricians in Vancouver told us that he had never heard of burst eardrums or meningitis associated with HSP, but that at the higher levels “weird things happen”.

We consider ourselves extremely lucky that E seems to have come through with no lasting damage. Our hearts go out to anyone who is going through the worst of it or dealing with any lasting side effects.