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“To
heighten awareness of EB and |
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“To
heighten awareness of EB and |
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Lesley Haynes, Specialist Paediatric Dietitian Lesley works solely with EB patients at Great Ormond Street Hospital for Children (GOSH), London, England as part of a large multi-disciplinary team which is committed to improving the lives of EB children. She is passionate about the role of nutrition support in EB and, in this article, outlines her team’s management of EB and asks DEBRA Canada members to contact her with their experiences of novel approaches to nutrition support. The Beginning About 16 years ago, although it was widely recognised that severely-affected EB children consumed inadequate nutrition for their needs, there was little information in the medical literature documenting this and no one had researched the effectiveness of dietary intervention. DEBRA UK awarded a research grant to my predecessor, Sue Allman, to allow her to look further at the nature of the nutritional deficiencies in 15 DEB children and to evaluate the benefits of dietary advice. Sue's findings hold few surprises for parents of EB children. Basically, eating and swallowing are painful, tiring and tedious; indeed frightening if food sticks in the oesophagus needing to be regurgitated. Extremely painful bowel movements are typical and guaranteed to reduce appetite. Poor food intake cannot support the dual needs of normal growth and continual skin healing and so, both suffer. The child is small and thin with poor immunity to infection. Unfortunately, despite dietary prescriptions tailored to each child, Sue found that long-lasting improvements in intake and growth could not be sustained. Although this seems a disappointing outcome, it was not unexpected considering the many factors which conspire against nutrition in the severely-affected EB child. It was also a crucial step in the learning curve for dieticians and doctors dealing with such a rare condition. The main conclusion of Sue's study was that avoidance of malnutrition depends on active and continuous nutritional support, ideally from birth, with the aim of preventing, or at least minimising nutritional problems. Realistically, this meant the use of naso-gastric and gastrostomy feeding at an early stage. The Middle At GOSH, we have based our current approach to nutritional support in EB on these conclusions, and nutrition support in EB is seen as a pivotal aspect of management. I work as part of a dedicated multi-disciplinary team headed by a paediatric dermatologist and paediatrician, with three EB Clinical Nurse Specialists. Nutritional assessments and reviews take place either during a short admission to hospital, or in the outpatient clinic when the child's progress is reviewed by other members of the EB team. As well as blood tests, children may undergo such investigations as barium swallow and/or video-fluoroscopy to determine the extent of swallowing problems, a dye study to illustrate their rate of bowel emptying, echo cardiogram if heart damage related to chronic poor diet is suspected and bone scans to indicate bone thinning or osteoporosis. In children with recessive dystrophic EB, whose intake is severely limited by mouth, swallowing and oesophageal problems, a feeding gastrostomy (often combined with oesophageal dilatation) is usually the management of choice, often in conjunction with oesophageal dilatation. Gastrostomy placement is a reversible procedure, well-accepted by children especially if it is carried out well before adolescence. Over 50 children have had gastrostomy buttons inserted at GOSH, through which they receive supplementary feeds. Feeds are generally given overnight, but not always – the regimen depends on the child’s and family’s lifestyle. For it to be successful, the regimen must be structured to fit the family, not the other way round, and it should be reviewed regularly to make sure it is still appropriate. With gastrostomy plavement, the children can eat as much as is comfortable and be “topped –up” with feeds via the gastrostomy. Family stress levels decrease as fast as the child’s nutritional status improves ! Of course, no single aspect of EB operates alone; good nutritional status affects, and is affected by, other features of the condition. The improvements in nutrition that our patients have achieved would not have been possible if aspects of EB care e.g. improved dressings, better management of constipation and painful bowel movements, improved management of gastro-oesophageal reflux etc, were not being addressed too. The End? No way - there’s a long way to go yet! The goal-posts keep moving and fourteen years into my job, I still find it challenging and hugely rewarding. We all know that nutrition alone cannot cure EB, but making sure that sufferers are as well-nourished as possible is of paramount importance to optimise quality of life for sufferers and carers alike, and to promote wound healing and resistance to infection. Novel approaches to nutrition support keep appearing and one such is the use of glyconutrients, which are said to belong to a group of substances called nutraceuticals. The manufacturers of these products claim that they support the process that our individual tissue cells communicate with each other and somehow promote health. I’ve been told that some EB patients in Canada are finding these helpful. If you’ve tried them, please contact me and tell me about your experiences – good, bad and indifferent. I’d also like to hear from anyone who’s taking pro-biotics such as live bacterial cultures which are believed to have a beneficial effect on human gut by counteracting the adverse affects of anti-biotics so often prescribed to treat skin infections. Please don’t be shy; e-mail me or write to me (contact details below) and help me not to needlessly re-invent the wheel or worse, subject my patients to treatment you’ve already found to be useless. I’m looking forward to hearing from you ! Lesley
Haynes,Specialist
Paediatric Dietitian Phone: 020-7405-9200 extension 5761 |
