Nutritions
Hsin-Chin Lu
(Dietitian, Nutrition Dept. of NCKU Hospital)
Malnutrition is a common care problem for some EB patients, especially the Herlitz type of patients with recessive dystrophic epidermolysis bullosa (RDEB) and junctional EB (JEB).
Reasons behind malnutrition include oral/esophageal blisters, esophageal stenosis, dental caries, gastroesophageal reflux, tongue inflexibility, etc. which all lead to obstruction of chewing and swallowing, as well as difficulty in taking food due to poor hands. When the above-mentioned reasons lead to insufficient food intake, it will cause nutrient deficiency, which hampers the speed of wound healing, the wound will then not heal and lead to insufficient food intake. Many patients with large wounds are often in such a vicious circle.
In terms of calorie intake, one can start by adjusting the texture of the food to increase eating. Using food scissors or a food processor can transform the general texture of food into a more edible state, and can be adjusted to the patients according to the individual’s swallowing or chewing ability. One can also add commercially available nutrients supplements to increase calorie intake. The calorie and protein requirements of EB patients are higher than those of people of the same age and sex. The research suggests:
-
The correction age of children is defined as the age when the actual height corresponds to the 50th percentile on the growth curve.
-
The recommended target for calorie intake is 1-1.5 times more than the correction age calories. 3. Protein intake should be 1-2 times more than the recommended intake of the correction age.
Besides, if the eating status is poor, it is suggested to take in multi-vitamins, calcium, and vitamin D3 on a regular basis. One should check iron and zinc blood levels based on blood draws to determine whether additional supplements are needed. Many patients also suffer from constipation due to insufficient food and dietary fiber intake, coupled with low activity levels. One should increase intake of vegetables, fruits, unrefined whole grains, and nuts to obtain appropriate dietary fiber.
Furthermore, bear in mind that sufficient water intake and increased activity levels are the keys to improve constipation. The most common problems among mineral deficiencies are iron and zinc deficiency. More than 90% of patients with RDEB suffer from anemia. The iron in food is absorbed by animal-derived blood substrates, such as red meat, offal, and clams, which can be taken with fruits to increase the absorption rate. But on the whole, if the anemia is very serious, it is easier to improve with medication. About 55% of RDEB patients and about 35% of JEB patients have low zinc. This may be related to low body albumin and high inflammation response protein. One can take more oysters, beef, and internal organs to supplement their diet.
In brief, increasing food intake can enhance nutrient intake and assist wound healing. Therefore, increasing overall intake can improve the quality of life of EB patients.
