EB Tips..Dowling Meara

Please note: The following are mearly suggestions please take them as ideas rather than do's and don'ts.

Diagnosis

We all have our stories of the tramas of getting a correct diagnosis, my advice is go to the top, go and find those that really know, for instance we went to Sydney Childrens Hospital, if you are in America go to Stanford University, California and if you are in England go to Great Ormond Street Childrens Hospital, London. If anyone knows of other good EB clinics I would love to hear of them. Dont mess with those who dont quite know, as there is a high risk of an incorrect diagnosis. Have qualified EB specialists instruct instruct a local medical team on how to perform the biopsy. Remember EB is rare and few people in the medical world have seen it let alone dealt with it.

Bathing

Using an antibacterial liquid soap as a bubble bath is a great way to kill any germs, and let a child enjoy a bath with a brother or sister or even a friend. Cutting finger nails in the bath gives you a soft nail to cut.

Nappies

I used disposable and lined them with a soft disposable nappy liner to stop the tapes rubbing or elastic causing friction you can cut the elastic out but this becomes tedious as the days go on, and as if we dont all have enough to do.

Climate control

We have discovered that keeping Luke in a regulated temperature proves to be easier on his blisters, he just gets the usual number not a sudden increase due to a sudden heat or humidity change. Air Conditioning for those summer months is a must.

Sea Salt and Sun

I cant say I have the magic cure but its pretty close to the best remedy I have found yet. A dip in the sea and some time playing in the sand with lots of fresh sea air, takes the angry reddness out of his blisters. I even pop on the beach and place wet sand on the site, it has almost healed up by the end of the day. The sand helps him remove scabs and sort out the itchy spots as he rubs his feet around. The sun is definitely good for Luke, amazingly he tans like no one else in the family. We have a sun shade tent for him which he sits under at the beach so that I dont have to apply lotions, as I think they would sting. Just keep the sea water in a bucket so that he/she can play with it, hense giving their hands a good soaking and the splashing helping any other spots.....most of all have fun, it really is a cure for all the stress too.

Out and About

Getting out and about is probably the hardest thing of all, facing those stares answering the questions and hearing people make accusations or diagnosises themselves. Be strong, dont ever feel that you have to answer, yet 7 out of 10 people will be concerned and interested, but those others are ignorant and will annoy you so just hang in there and say whatever you need to. I have had so many people give me their magic natural cure ideas, from new diet to drinking dead sea water. They all find it hard to believe its Genetic and there is nothing that can be done yet....Steriods is the latest word that comes out in the advice line. It does get easier and it is important to give these children a life so dont hide them away, go out and enjoy if you can.

Eating Habits and Diet

Luke did not eat solid food until he was about 14mths, he just would not put it in his mouth, he still wont take anything from a spoon, so everything is finger food. He will try anything now, yet chocolate remains the favorite. He does eat junk food as that is the only way we could get him interested. However with the constant picking and nibbling all daymeans he does not get enough fibre etc. and he tends to not eat too much, whether it is because he finds it boring or it hurts his mouth I dont yet know. We do give him a high fibre high calorie drink mixed with his milk, he has about 400mls a day of this (Ultracal) this takes a lot of the stress out of feeding him if hes not in the mood and it assures me he is getting the right vitamins etc.
  • Constipation
    has and is a never ending battle, Luke drinks pear juice and I try hard to get fibre into him, but he cant seem to get it sorted out, we have to give him liquid parafin when things get bad, and the warmer the weather the worse it seems to be. I am open to suggestions.
  • Iron
    Luke looses a lot of iron like most if not all EB kids, it is due to the blood loss and the constant healing, it is worth having the iron levels in their blood checked regularily and if your child becomes lethargic pale and their palms of their hands are white then chances are their iron levels are down, this can lead to lots of problems including slow healing. There are oral iron supplements, but be ready to battle the constipation.

EB Tips..Debra UK.

Jacqueline Denyer, the Specialist Nurse for children with EB answers some common questions about EB Simplex.

Q. "Do all types of EB have the same dressings?
A. Dressings used for the Dystrophic and Junctional forms of EB are generally highly unsuitable for those with EB Simplex as they tend to be bulky and therefore generate a lot of heat. For example, those with the Dowling Meara form of EB Simplex should try to use a minimal amount of light dressings and only dress actual wounds, rather than blistered areas. Many people prefer to avoid dressings wherever possible.
Q: Are EB Simplex blisters painful?
A: Yes! Advice can be given regarding pain relief after careful consultation to find the correct drug to suit the individual life-style.
Q. Should children with EB Simplex attend a specialist centre?
A: Although there is yet no product to stop the blisters from forming, being able to talk openly to a doctor from a centre with knowledge of such problems and who will believe the history must be of enormous benefit to some families. Advice can be given regarding footwear and friction relieving products as well as information on mobility, benefits and allowances.

From a new DEBRA publication, Epidermolysis Bullosa: an Outline for Professionals, which is now arailable from DEBRA. This new publication has been adapted by Dr. Dauid Atherton and Jacqueline Denyer from a chapter written for "Textbook of Paediatric Dermatology" published by Blackwell science. Further details of DEBRA funded projects are available from iKI9A House, Wellington Business Park, Dukes Ride, Crowthor Telephone: 01344 771961 Registered Charity 284754