engleski

Nutritional assessment of patients with Epidermolysis Bullosa

The aims of the study are: 1. to determine quality of food intake 2. to asses nutritional status of patients with EB Patients and methods According to the information from DEBRA Croatia, 34 individuals are known to have been suffering from Epidermolysis Bullosa. The prevalence of all types of EB in Croatia is calculated at 7, 7/per million (population in Croatia 2002, 4.437, 460). It is important to mention that, according to the estimated frequency of EB in the world literature, quoted data about the sick in Croatia is for above the real figure. Namely, the number of patients with “mild forms” of the disease is probably higher, but they in Croatia, as is often the case in other parts of the world, aren’t registered at the doctors’ offices. Among above mentioned 34 patients there are 16 male patients and 18 female. Registered affected individuals are aged from 1 to37, which mean that the average age is 19. The majority of patients (70%) are aged between 1 and 18. Sixteen patients (50% affected individuals in Croatia) with Epidermolysis Bullosa (9 female, 7 male ; 12 with dystrophic (DEB), 4 with simplex (EBS) ; age range 2-34) were assessed in the study (Table 1). Each patient underwent the nutritional assessment, which included anthropometrics measurements (weight and height) and dietary intake. The patients were weighed on the scales with weights (100g precision), the height of the patients was measured by meter (0, 5 cm precision).The same person did all measurements. Weights and heights were plotted on National Centre of Health Statistics growth charts. Weight: age, height: age, BMI (body mass index): age ratios were calculated according to these charts for each patient. Food intake and dietary habits were assessed by 3-day food records and through the questions about quantity, number of daily meals, consistence of meals, high energy drinks and supplements consumption. Patients were instructed how to record their food consumption and the parents were told how to record all the food and drink consumed by their children. Results were analysed using computer program at Children’s Hospital Zagreb. The presence of clinical features such as oral ulceration, dental caries, dysphagia and constipation was also registered. Discussion Chronic malnutrition in patients with DEB and the influence of inadequate nutrition on growth and progress of patients were documented in the study. All patients with DEB didn’t reach their normal weight and BMI for their age and sex. Results show that insufficient weight gain was more point out than stature failure (12 patients had weights on or below fifth centile and 5 patients had height on or below fifth centile) (Table 1). Patients with EBS mostly caught up normal weight and height values for their sex and age (Table1.). The relationship between inadequate food intake and clinical manifestation of illnesses was found out. The presence of oral ulceration (11 patients), dental caries (7 patients) and dysphagia (10 patients) make eating painful and tedious. Because of their eating difficults, most patients don’t usually enjoy eating so after certain period of time they lose their interest for food and they have inadequate food and energy intake. Due to these facts patients get bad dietary habits, which are very difficult to influence, because they eat according to their abilities and not according to their needs and recommendations. It has been estimated that 12 patients (75%) take less than six meals a day while their consummation period lasts between 30 and 45 minutes on average. They mostly consume soft and mixed food and they prefer certain groceries. The favourite food in all age groups are milk and dairy products, pudding, eggs and fruit juice (Picture 1.). It is interesting to note that one patient consumes 3 beers a day despite the irritation. Since they all have serious problems with swallowing very few patients consume meat. These dietary habits reflected on the patients` nutritional status. High consummation of milk, dairy products and eggs results in acceptable intake of proteins and calcium, which is also shown in the data that 10 patients have adequate protein intake (Picture 2.) while osteoporosis has been registered in only 2 patients. It is important to note that their total energy daily intake (12 patients take 1000- 2000 kcal less than they should according to their age, sex and illness) has an unfavourable influence on their nutritional status (Picture 3.). According to the data in the literature constipation is present in most patients with DEB. Although a low fruit and vegetables intake has been noted only 6 patients suffer from constipation, while 4 patients take laxatives. The monotony of daily meals (low meat, fruit and vegetables intake) doesn’t satisfy daily needs for vitamins and minerals. It is satisfactory that 13 patients take supplements. Since the patients have enormous problems which influence their appetite and the possibility to eat, insufficient energy intake is deteriorated by the fact that only 4 patients sometimes consume high-energy drinks (Picture 4.). Despite the willingness and the level of being informed about the importance of high-energy drinks consummation, the reason for low consummation lies in inaccessibility of these drinks and in the social and economic situation of patients. It is necessary to mention that, despite their wounds, arm deformation and apathy, patients try to participate in the daily activities. They spend most of their time in front of computers, watching TV, reading while some of them even paint. Very few patients spend their free time walking or doing some sports. Conclusion The study presents the population suffering from the effects of chronic malnutrition. The results provide a strong argument for the value of nutritional assessment and intervention. Thus, we currently have no therapy that enables us to influence the natural course of the disease, we do have to opportunity to improve patients` quality of life by improving their nutrition. Improved nutrition status might accelerate healing, reduce predisposition to infection, encourage growth, increase activity and enhance the patients` general well-being. The prevention of malnutrition will depend on more active and continuous nutritional support, starting at very early age. This may require the use of many more procedures, such as nasogastric or gastrostomy feeding.

Epidermolysis Bullosa ; malnutrition ; nutritional status ; nutritional assessment

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