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 saturation, ferritin)

Signs of Anemia

Low hematocrit(HCT) – Low hematocrit may indicate Anemia, malnutrition and blood loss among other things.

Low Hemoglobin (Hb) (Hgb) – The average hemoglobin level in adult women is 12.1 to 15.1 and in adult men it’s 13.8 to 17.2. Although it is often considerably lower in RDEB patients. I myself have dropped to a 4 hemoglobin once. I am usually around an 8 hemoglobin but some EB patients can get up to normal levels with proper treatment.

Small red blood cells

Low serum ferritin (serum iron) level – The serum ferritin level is directly proportional to the amount of iron stored in the body.

High iron binding capacity (TIBC) in the blood

Treatments

Inflammation Reducing Supplements – To improve the ability to absorb nutrients, a supplement can be taken to help heal and reduce inflammation in the digestive tract.

Eat Healthy – Those with Anemia should eat foods high in iron and other nutrients.

Oral iron supplements – Oral iron can be taken, however in EB the degree of deficiency is too great for it to have much effect. Oral iron can also cause constipation. A good alternative would be an herbal iron supplement (an all natural supplement that contains herbs known to be high in iron).

Supplemental vitamins – Some important vitamins to take are:
~Vitamin B12 (100mg/day + other B complex)
~Folic Acid (1mg/day)
~Vitamin E (400 – 800 U/day)
~Vitamin C (30mg/kg/day)
~Vitamin K (1-5mg/day)

Vitamins come in chewable, powder and liquid which makes it easier for EB patients to swallow. Keep in mind that whole food vitamins absorb better than regular vitamins.

 Wound care and Treatment of infection

It is extremely important to take care of all wounds and treat any infection as best you can to reduce inflammation.If the above methods do not work, or if the patient is already severely anemic the following treatments can be done:

Intravenous iron – IV iron can help a lot and is more effective when also paired with EPO shots. Frequency of iron infusions depends on how severe the anemia is. Iron must be infused slowly and a test dose is always given first to monitor any allergic reactions. Getting IV iron is an easy procedure and usually takes a couple hours. Please ask your doctor about the risks and benefits of IV iron.

Erythropoietin (EPO) shots – Erythropoietin is a naturally occurring hormone, produced by the kidneys, which stimulates the body to produce more red blood cells. It is generally given as an injection under the skin. It usually needs to be given one to three times a week. Common names for EPO shots are Aranesp (Darbepoetin Alfa), Procrit (Epoetin Alfa). Please ask your doctor about the risks and benefits of Epo shots.

Blood Transfusion – If the iron and epo are not effective, a blood transfusion is another option. This is another simple procedure that takes a few hours. Please ask your doctor about the risks and benefits of blood transfusions.

 Terms and Definitions

Anemia – Anemia is a lower than normal number of red blood cells (erythrocytes) in the blood, usually measured by a decrease in the amount of hemoglobin. Hemoglobin is the red pigment in red blood cells that transports oxygen.

Anemia of Inflammation – This type of anemia develops as a result of extended infection or inflammation.

Chronic – A chronic condition is continuous or persistent over an extended period of time. A chronic condition is one that is long-standing, not easily or quickly resolved.

Copper – Copper is an essential trace mineral present in all body tissues. Copper, along with iron, helps in the formation of red blood cells. It also helps in keeping the blood vessels, nerves, immune system, and bones healthy.

Differential – The blood differential test measures the relative numbers of white blood cells (WBCs) in the blood. It also includes information about abnormal cell structure and the presence of immature cells.

Erythropoietin – a hormonal substance that is formed especially in the kidney and stimulates red blood cell formation.

Ferritin – a crystalline iron-containing protein that functions in the storage of iron and is found especially in the liver and spleen.

Folate (folic acid) – Folic acid is a water-soluble vitamin in the B-complex group. Folic acid works along with vitamin B12 and vitamin C to help the body digest and utilize proteins and to synthesize new proteins when they are needed. It is necessary for the production of red blood cells and for the synthesis of DNA (which controls heredity and is used to guide the cell in its daily activities). Folic acid also helps with tissue growth and cell function. In addition, it helps to increase appetite when needed and stimulates the formation of digestive acids.

Hemoglobin – Hemoglobin is the most important component of red blood cells. It is composed of a protein called heme, which binds oxygen. In the lungs, oxygen is exchanged for carbon dioxide.

Hematocrit – The hematocrit is the percent of whole blood that is comprised of red blood cells. The hematocrit is a measure of both the number of red blood cells and the size of red blood cells.

Inflammation – It occurs when tissues are injured by bacteria, trauma, toxins, heat, or any other cause. Chemicals including histamine, bradykinin, serotonin, and others are released by damaged tissue. These chemicals cause blood vessels to leak fluid into the tissues, resulting in localized swelling. This helps isolate the foreign substance from further contact with body tissues.

Iron – Iron is an important trace mineral that is found in every cell of the body, usually combined with protein. Iron is an essential mineral for humans because it is part of blood cells. About 30% of the iron in the human body is in storage to be readily available to replace any that is lost. Iron is essential to the formation of hemoglobin and myoglobin, which carry oxygen in the blood and muscles. It also makes up part of many proteins and enzymes in the body.

Iron Binding/Transferrin Saturation (TIBC = total iron binding capacity) – A test that measures indirectly the transferrin level in the bloodstream

Iron-Deficiency Anemia – Iron deficiency anemia is a decrease in the number of red cells in the blood caused by too little iron.

Malnutrition – Malnutrition means a person’s body is not getting enough nutrients. The condition may result from an inadequate or unbalanced diet, digestive difficulties, absorption problems, or other medical conditions.

Mean Cell Hemoglobin – Hemoglobin amount per red blood cell (MCH)

Mean Cell Volume – Average red blood cell size (MCV)

Nutritional Anemia – anemia (as hypochromic anemia) that results from inadequate intake or assimilation of materials essential for the production of red blood cells and hemoglobin — called also deficiency anemia

Plasma – Plasma is the liquid portion of your blood. Plasma transports water and nutrients to your body’s tissues. Plasma also contains many proteins that help the blood to clot and fight disease. Plasma is a type of lymphocyte that produces immunoglobulin (antibody) that is necessary for normal immune system function.

Platelets – Platelets are necessary for normal blood clotting (hemostasis). Most important, they aggregate (clump together) to plug small holes in damaged blood vessels. They also activate factor VIII (a component of the coagulation cascade) and release phospholipids necessary for coagulation.

Red Blood Cells – RBCs transport hemoglobin. Hemoglobin transports oxygen. The amount of oxygen body tissues receive depends on the amount and function of RBCs and hemoglobin. RBCs normally survive about 120 days in the blood. They are then removed by specialized “clean-up” cells in the spleen and liver.

Retic Count – This is a test that measures the percentage of reticulocytes (slightly immature red blood cells) in blood.

Sedimentation Rate (ESR) – ESR (erythrocyte sedimentation rate) is a nonspecific screening test for various diseases. This 1-hour test measures the distance (in millimeters) that red blood cells settle in unclotted blood toward the bottom of a specially marked test tube.

Selenium – Selenium is an essential trace element. It is an integral part of enzymes, which are critical for control of the numerous chemical reactions involved in brain and body functions.

Serum Ferritin/Iron – A test that measures the amount of iron in the blood.

TIBC – Total iron binding capacity. A blood test that measures the total iron binding capacity (TIBC) as an indirect measure of transferrin.

Transferrin – Iron ions are delivered in the blood by the protein transferrin. Each transferrin molecule can carry two iron ions.

Vitamin B6 – Vitamin B6 is a water-soluble vitamin and is part of the vitamin B complex. Vitamin B6 plays a role in the synthesis of antibodies by the immune system, which are needed to fight many diseases. It helps maintain normal nerve function and also acts in the formation of red blood cells. Vitamin B6 is also required for the chemical reactions needed to digest proteins. The higher the protein intake, the more the need for vitamin B6.

Vitamin B12 – Vitamin B12 is a water-soluble vitamin that is part of the vitamin B complex. Vitamin B12, like the other B vitamins, is important for metabolism. It helps in the formation of red blood cells and in the maintenance of the central nervous system.

Vitamin C – Vitamin C is a water-soluble vitamin that is necessary for normal growth and development. Vitamin C promotes healthy teeth and gums, helps in the absorption of iron, aids in the maintenance of normal connective tissue, and promotes wound healing. It also helps the body’s immune system.

Vitamin E – Vitamin E is a fat-soluble vitamin that acts as an antioxidant. Vitamin E is an antioxidant that protects body tissue from damage caused by unstable substances called free radicals. Free radicals can harm cells, tissues, and organs, and they are believed to be one of the causes of the degenerative processes seen in aging.Vitamin E is also important in the formation of red blood cells and it helps the body to use vitamin K.

Vitamin K – a fat-soluble vitamin that plays an important role in blood clotting.

White Blood Cells – White corpuscles in the blood. They are spherical, colorless, and nucleated masses involved with host defenses. Elevated counts can be seen in cases of inflammation and infection.

Zinc – Zinc is an important trace mineral. This element is second only to iron in its concentration in the body. Zinc plays an important role in the proper functioning of the immune system in the body. It is required for the enzyme activities necessary for cell division, cell growth, and wound healing. It plays a role in the acuity of the senses of smell and taste. Zinc is also involved in the metabolism of carbohydrates.

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