Anemia

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Most patients with Recessive Dystrophic Epidermolysis Bullosa are also Anemic. Anemia in EB is caused by many factors and can become very severe if not treated.

Anemia means not having enough red cells and hemoglobin. This can occur because they’re not produced, or are being lost and destroyed. Anemia can lead to low oxygen levels, which can lead to damage to organs, including the heart.

There are three types of Anemia that effect EB patients. They are Iron Deficiency, Chronic Inflammatory and Nutritional.

 Causes of Anemia in EB patients:

Blood and iron loss from wounds – Obviously with so many wounds there is blood loss and fluid loss which can worsen anemia.

Chronic infection/inflammation – Because of chronic infections there is inflammation. Inflammation inhibits red cell production and diverts iron away from the bone marrow to the liver and spleen.

Problems absorbing iron – Due to the effects EB has on the gastrointestinal tract.

Malnutrition/Malabsorbtion of nutrients – such as B-complex vitamins, B12, B6, vitamin C, vitamin E, vitamin K, folic acid, zinc, copper and selenium which are all needed for cell formation. Due to the effects EB has on the gastrointestinal tract, nutrients are not easily absorbed.

Loss of transferrin and other proteins through wounds – Iron is transported by a protein called transferrin. Non-transferrin bound iron, known as free iron, is very toxic and can cause low blood pressure, nausea, rash, facial reddening and eventually may cause heart failure and other damage. Iron is stored in a part on a protein called ferritin.

Symptoms of Anemia

Pale skin color, weakness, decreased appetite, fatigue, shortness of breath, rapid heartbeat, palpitation, headache, irritability, lethargy, dizziness, weight loss, lowered immunity, depression, slow healing, bruising.

What to Check

First a complete blood count (CBC) must be taken. It is important to check levels regularly. A CBC checks the White cells (WBC), Red cells (RBC), Hemoglobin (Hb), Hematocrit (HCT), Mean cell volume (MVC), Mean cell hemoglobin (MCH), Differential (percent of different kinds of white cells), Platelets, and Retic count (new red cells). Also check the Sedimentation rate (ESR) for a crude measure of inflammation. And check the Iron Status (iron, iron binding/transferrin s