Nutrition: Study of long-term gastrostomy use in adults with RDEB

Lynne Hubbard: Specialist Dietician in EB, St Thomas' Hospital, UK

How are grown-up patients coping with gastrostomy on the long term? A lot of them have problems swallowing food, and medication that has to be taken orally. And in that case gastrostomy stays a steady and safe solution. In this way chronic patients get enough vitamins, proteins, fibres and minerals.

Yet it was important to make an analysis of the effects of this approach on the long run.
Therefore data of 12 patients were collected, based on growth and weight; these patients had an experience with gastrostomy from 10-19 years. The analysis showed that with 58% of the patients gastrostomy had a positive influence according to growth, and with 50% it had a positive effect on their weight.


Of course gastrostomy has his problems; for instance; a leaking stoma, or painful experiences when the tube is taken in.

To prevent this problems:

  • One has tried to use better endoscopic methods related to the placing of the stoma.
  • To attend to the whole process as good as possible by giving advice to the patients, who are based on practical experience.

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Nutritional challenges in a baby with Herlitz Junctional EB

Rosie Jones, Specialist Dietitian in EB, Birmingham Children's Hospital, UK

Rosie Jones describes for the audience a case of problems with nutrition in a social deprived family, where a little girl was born with JEB. The physical condition of the child was severe; anemia, inflammations, constipation and a lot of blisters in the oral cavity. The dental care was neglected, brushing teeth was no option. On top of this the girl was a little deaf and staying behind in her mental and physical development. In the overcrowded household one could not expect that the mother of the family could spend a lot of energy and time to improve the child's condition.

Still dieticians and other specialists and assistants tried to improve the quality of the girls' life; energy-drinks and special nutrition-supplements were prescribed, accompanied with practical advices and encouraging support for the whole family. The girl did become speech training, and was offered help with her eat- and swallow problems.

But despite this approach the situation stayed difficult; the family did not cooperate very well. But the decision to let her have a gastrostomy turned out to be a disaster; the wound, caused by the introduction of the tube into the stomach, would not heal, and the tube had to be removed. The parents refused a second gastrostomy.

But, although the situation seemed to be hopeless the family assistants suggested a special adapted form of gastrostomy; another failure was of course out of the question. And this time, with much more precautions like special anti bacterial gauzes to take care of the little stomach wound, this approach succeeded .De then 4-year old girl could go home, where she also got some extra nutrition.

She was visibly getting better, had a lot more energy, started to move and walk again, and was being educated at a special school for children with hearing disabilities. Meanwhile another baby with JEB was born in this family; but luckily the parents agreed with gastrostomy when the baby was 13 months old.

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Nutrition And Dental Health In Eb: A Team Approach

Dr Susanne Kramer, Lecturer in Paediatric Dentistry, University of Chile, Santiago. Dentist for Debra Chile

Preventing dental caries is a common goal for patients, parents, dentist and dieticians. The presentation started by discussing the multiple risk factors involved in the development of caries and analyzing the factors that can and cannot be addressed in people with EB. Some caries risk assessment tools were presented and hints on how to brush the teeth in a gentle but effective manner were presented. Other preventative strategies such as topical fluoride, fissure sealants and chlorhexidine were also discussed.

Carcinogenic potential of food was discussed and the AAPD (American Association of Paediatric Dentistry) recommendations were presented:

  1. Breast-feeding of infants to ensure the best possible health and developmental and psychosocial outcomes, with cessation of ad libitum breast-feeding as the first primary tooth begins to erupt and other dietary carbohydrates are introduced;
  2. Educating about the association between frequent consumption of carbohydrates and caries;
  3. Educating the public about other health risks associated with excess consumption of simple carbohydrates, fat, saturated fat, and sodium.

It was concluded that a dietary caries-prevention programme should be instigated at an early age and that dentists and nutritionists have to collaborate on an appropriate program.

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