Due to a side effect of a medication, we have gone from 90% oral feeds and 10% g-tube feeds, to 90% g-tube feeds and 10% oral feeds.
Because he is much older now, his nutritionist helped me determine the amount of calories, vitamins and nutrients he needs daily to heal, gain and grow.
Though physical activities levels vary from person to person, those with EB tend to have very high Metabolism because the body is in a constant stay of healing and regenerating skin. That requires so much energy, calories and nutrition.
This is the calculator the nutritionist used for us: DRI Calculator
While Joey is a very active child, she recommended choosing the very active option from kids with EB since there is no specific calculator for those with EB.
Here is the recommendation for a pre-teen male with generalized severe EB Simplex. For the Vitamin, Mineral and Nutrients, she recommended making sure he gets at least the minimum amounts thru food but no more than the maximum amounts per day. We will do blood work in 6 months to see if these recommendations are working for him.
**Again, please check with your child's doctor before changing their diet, because what is best for one child, isn't best for all kids with EB.**
Vitamin D: In recent studies have identified Vitamin D receptors in a variety of tissues where their presence was previously unknown. Cathelicidin, an antimicrobial peptide induced by vitamin D, promotes wound healing. These researchers concluded that vitamin D and its receptor signaling regulates structural integrity and transport functions of epithelial barriers with implications for wound healing.
Protein: Protein’s most important use is production of collagen that serves as scaffolding for scar in healed wounds. The proteins are also necessary for forming new blood vessels and encouraging healing cells to migrate to the wound surface.
Amino acids: Protein is made of amnio acids. Among amino-acids, those that play an important role in wound healing, are arginine and glutamine. Arginine is essential for the inflammatory process and synthesis of collagen. Arginine also stimulates the immune system to begin the healing process. Glutamine plays several roles via its metabolic, enzymatic, antioxidant, and immune properties. In wounds, it protects against the risk of infections by stimulating production of immune fighting proteins. Glutamine is also a precursor of glutathione—an antioxidant and an essential cofactor in stabilizing cells. The recommendation is to have some arginine and glutamine included in the protein supplements.
Vitamin C: Vitamin C, also known as ascorbic acid (AA), is involved in all phases of wound healing. In the inflammatory phase it is required for neutrophil apoptosis and clearance. During the proliferative phase, AA contributes towards synthesis, maturation, secretion and degradation of collagen. The role of vitamin C in wound healing is believed to be due to an influence on collagen formation, immunomodulation, and antioxidant functions. Deficiency results in impaired immune response during the inflammatory phase with increased capillary fragility and reduced collagen tensile strength and synthesis during the proliferative and remodeling phases,It also stimulates collagen and elastin formation. It is essential for stabilizing the structure of collagen with strong bonds and crosslinks to strengthen the healing tissue. Without this stabilization, the structure becomes fragile. Vitamin C also provides tensile strength to newly built collagen; otherwise, new tissue could not stretch without tearing. Tensile strength is important in pressure ulcer healing because healed pressure ulcers are susceptible to future skin breakdown. Vitamin C also is required for proper immune system function, a consideration in patients with open wounds.
Zinc: Zinc plays a major role in regulating every phase of the wound healing process; ranging from membrane repair, oxidative stress, coagulation, inflammation and immune defense, tissue re-epithelialization, angiogenesis, to fibrosis/scar formation. Decreased zinc levels result in decreased cytotoxicity of natural killer cells, impaired phagocytosis in macrophages and neutrophils, and decreased number of granulocytes. Also with zinc deficiency, B-cell precursors and mature B-cells are reduced. Zinc deficiency affects all phases of wound healing. In the inflammatory phase, there is decreased immunity and increased susceptibility to infections. In the proliferative phase, there is impaired collagen synthesis and tensile strength. Finally, in the remodeling phase, there is a dampening of fibroblast proliferation, collagen synthesis, and epithelialization, therefore the body needs adequate Zinc stores. However, excess zinc supplementation can interfere with the absorption of other cations, specifically iron and copper. Therefore supplementation should be based on individual need.
Vitamin A: It’s one of the most essential nutrients for wound healing since it helps “Control Inflammatory Response”. Vitamin A is required for epithelial and bone tissue development, cellular differentiation, and immune system function.Vitamin A is known for its ability to stimulate epithelial growth, fibroblasts, granulation tissue, angiogenesis, collagen synthesis, epithelialization, and fibroplasia. Vitamin A also has the unique ability to reverse the inhibitory effects of steroids on wound healing. Steroids reduce the ability of healing cells to migrate to the wound and build strong collagen bonds. Vitamin A can reverse this effect! In addition to its role in the inflammatory phase of wound healing, Vitamin A will also strengthen the collagen bonds and reduce the ability of wound inhibitory enzymes to counteract the healing process.
Iron: Iron was previously postulated as beneficial in wound healing, due to its status as a cofactor in collagen synthesis. In wound healing, iron deficiency results in impaired T cell and phagocyte function in the inflammatory phase and decreased tensile strength and collagen synthesis in the proliferative phase However, iron supplementation has been shown to prolong inflammation, and no evidence exists that iron supplementation benefits wound healing. Patients with iron deficiency anemia, which is known to inhibit wound healing due to decreased oxygen transport to proliferating tissues, may benefit from iron supplementation; however, more data is required to establish treatment guidelines.
Blood sugars: Though not a nutritional supplement, blood sugar control is essential for healing. Though glucose is the fuel for cells, when the blood stream is saturated with sugar, the healing cells become sluggish and stop healing. They also block the path for important nutrients and oxygen to pass into the wound. Unfortunately, bacteria use this excess sugar to wreak havoc on wounds. This leads to an infection with no healing cells able to fight the bacteria. We often see normally benign bacteria cause severe wounds due to elevated blood sugar.
