Monday, 26 December 2011

On blistering babies, disappearing doctors and useless dermatologists

Isn't he gorgeous?
All pictures have been shown with his Mother's permission
It has been a hard couple of weeks and I am glad that we are away on our Christmas break.  I was rostered to lecture the second year EADV (diploma students) on Epidermolysis bullosa (EB).  I had volunteered myself as this is a very rare genetic blistering disease, and I was the doctor that had seen the most cases.  As a dermatology registrar I was fortunate to organise an attachment at the Birmingham Childrens’ Hospital with Celia Moss an international expert in paediatric dermatology.  She has established Birmingham as one of the two EB centres in the UK.  Birmingham has a high Asian population where first cousin consanguineous marriages are very common so unfortunately EB is seen quite frequently. 

So there I was last Thursday at 8.15am sitting in the big lecture theatre waiting for something to happen.  As Kelvin and Esther were away there was no picture session where recently seen patients are discussed.  I therefore suggested to the second year EADV students that I gave my lecture scheduled for that afternoon.  I explained that the lecture was on EB – a rare blistering disease and I invited everyone to stay: medical students, first year EADV students and residents.  True to form however everyone else left apart from Michael the Dutch dermatologist and the 2nd year EADV students.  The first year EADV students sat outside around some tables and talked very loudly and irritatingly until I went outside and invited them to either come in or join the lecture or to shut up and go away.  They of course chose to go away.

After the ward round Alice took us to see the ward referrals and we were asked to see a baby who was on IV antibiotics with “congenital syphilis”.  I undressed the baby to see extensive blistering, erosions, scarring and dystrophic (abnormal )nails.   This wasn’t congenital syphilis but was Epidermolysis bullosa; a dreadful diagnosis for a baby here.  EB is not a curable condition as there is an abnormality in the skin causing permanent fragility and blister formation on minimal trauma.  In the UK with multi-disciplinary care, soft non-stick specialist dressings and amazing EB nursing input babies are now living to adulthood. Obviously it is a different picture here and babies often succumb early to sepsis from the multiple skin erosions.  It was so bizarre to have given a lecture on the condition in the morning and to see a baby later on that day with EB.

This boy is a little fighter and I scoured the cupboards and drawers of the RDTC for appropriate dressings.  We found some allevyn which is a non-adhesive foam dressing and Alice, Sebastian and I cleaned the eroded areas, tried to explain to Mum about the condition and did a Blue Peter attempt; cutting the allevyn to size and trying to stick it circumferentially whilst covered in Vaseline from the Vaseline gauze and then tying soft bandage around trying not to dislodge the allevyn.  Our first attempts were pretty dismal but I am pleased to say we have improved and on the third dressing change all areas were healing apart from his thumbs where he has lost both nails and keeps rubbing the areas with his fingers so they are dreadfully eroded.  His poor mum was distraught as we tried to dress the thumbs, which was very hard and they kept bleeding.  However once everything was covered he settled down, as eroded areas open to air are very painful.

Our 3rd dressing  - a much better Blue Peter attempt!

I asked for advice from the UK and Celia was amazingly helpful again and thought the baby had Herlitz Junctional EB.  This is still a very difficult diagnosis to have here and I have e-mailed drug reps in the UK who are kindly donating specialised EB dressings for the baby and dermasilk (silk) suits for him to wear.  My colleague Claire Fuller is coming out for the CME meeting in January and is kindly going to bring them although in her experience these babies do poorly.
Friday was another tough day as it was quite a big paediatric clinic and as per usual Sebastian and I together with 2 residents were the last to leave.  The attitude of many of the doctors, diploma students and medical students is that as volunteers are here the work will get done so it is fine to disappear as early as possible.  And by early I mean at 11.30am and this is after having gone off to breakfast between 9-10am!  One who shall remain nameless has disappeared early 4 Fridays in a row and Professor Grossman yesterday told the Diploma students firmly that they were expected back on 3rd January for the new term.  He said “ I have heard it all before– the bus broke down, I lost my money, my Aunt died ......”  It remains to be seen how many will return on this date.

Julianne recently asked one of her physio students why they missed her teaching session and they said quite unashamedly “ I went to a party last night and came back late and so was too tired to come in this morning”  !!!!  Our residents/ students etc.  are frequently telling us that they are “feeling tired/ a bit dizzy/ unwell/ feverish” and have to go home.  Quite an eye-opener for us hard bitten work through anything brigade.  

It makes teaching here quite a challenge!  I am trying to understand why people do not want to learn as we are used to treasuring teaching particularly from experts.  Here in Tanzania I am amazed to hear that no doctors, teachers, professionals will attend any courses unless they are paid to go!!  The most effective and useful thing us foreign volunteers can do is to teach so that all these dermatology doctors/ medical officers can use this learning to treat patients in East Africa.  It will be non-sustainable help if we just run the clinics.

So what do you do?  Sebastian and I have started to joke with each other that “we are a bit tired/ had a late night/ can’t come in....”  We did explain the situation to Prof Grossman but I am sure he has heard it all before.

The EB baby story took an unexpected and sad turn as the baby has been labelled as having congenital syphilis.  Due to this diagnosis the baby’s father refused to allow his wife and child to come home.  There is still a huge stigma about syphilis, HIV  and other sexually transmitted diseases and the men tend to conveniently forget that they are the ones that usually do the transmitting!  We kept explaining that the baby did not have congential syphilis but a totally different diagnosis – EB and the mother had tested negative for both syphilis and HIV.  This would have been the end of it in the UK but here the medical director became involved as someone from the family had telephoned someone else important who had telephoned the Medical Director....  So Alice took me as an “international expert” on EB to see the Medical Director who was in his room sat reading the paper.  I explained that the diagnosis was unequivocally EB but it remains to be seen if the poor woman is able to take her baby home for Christmas.  Life is very hard here.
Teaching Mum how to do her baby's dressings

So now I must admit that the useless dermatologist is me.  On Wednesday Aaron took the boys and Noga and Amy swimming before the Hanukah party.  When he was drying Josh he noticed that he had a red and blotchy rash.  Josh seemed fine and the rash looked urticarial to me so I wondered if he had eaten something/ reacted to something at the swimming pool.  The next day we got up early to fly to Zanzibar and the rash got redder, more extensive and itchier and in the middle of the afternoon, after I had woken up as I was feeling poorly and a bit tired, Josh had a raging temperature and a terrible rash.  I dosed him up with anti-histamines, Calpol and Brufen but it kept him and Aaron up most of the night.  Apparently though at one point when Aaron was asleep Josh woke him to say softly and sadly “Daddy will you please stop snoring?”

In the morning Josh looked dreadful with a very extensive rash, a temperature and it took Aaron to notice that his face was swollen!  He was lethargic too and even his horrible doctor mum was worried!  I thought he might have malaria so we took him to the Zanzibar health clinic in town. 
Zac said “who will I play with if Josh dies?!!!” but I am sure he was just concerned.

Josh's very impressive urticarial rash - and this is after a lot of anti-histamine!

Luckily we saw a very competant doctor and after a traumatic finger prick test malaria was excluded.  The doctor thought it was just a severe urticarial reaction and despite the fact that I had never seen a temperature with urticaria I was at a loss to know what else it was.  I had started Josh on azithromycin (antibiotics) that morning.  It is a nightmare having an ill child but much worse to have an ill child on an island in the middle of the Indian Ocean just before Christmas.  The doctor suggested a 4mg IM dexamethasone injection into Josh’s bottom, which was not fun but miraculously his rash all settled and he felt much better after pizza and ice-cream.  He was fine all day but the rash started up slightly in the evening so I have given the prednisolone 15mg and promethiazine syrup as prescribed.  I hope it settles now and have posted some pictures.

We are hoping he is well enough for our planned Safari Blue dolphin spotting, snorkelling trip tomorrow and will keep you posted.......

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