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

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1 Anemia Mst patients with Recessive Dystrphic Epidermlysis Bullsa are als Anemic. Anemia in EB is caused by many factrs and can becme very severe if nt treated. Anemia means nt having enugh red cells and hemglbin. This can ccur because they're nt prduced, r are being lst and destryed. Anemia can lead t lw xygen levels, which can lead t damage t rgans, including the heart. There are three types f Anemia that effect EB patients. They are Irn Deficiency, Chrnic Inflammatry and Nutritinal. Causes f Anemia in EB patients: Bld and irn lss frm wunds - Obviusly with s many wunds there is bld lss and fluid lss which can wrsen anemia. Chrnic infectin/inflammatin - Because f chrnic infectins there is inflammatin. Inflammatin inhibits red cell prductin and diverts irn away frm the bne marrw t the liver and spleen. Prblems absrbing irn - Due t the effects EB has n the gastrintestinal tract. Malnutritin/Malabsrbtin f nutrients - such as B-cmplex vitamins, B12, B6, vitamin C, vitamin E, vitamin K, flic acid, zinc, cpper and selenium which are all needed fr cell frmatin. Due t the effects EB has n the gastrintestinal tract, nutrients are nt easily absrbed. Lss f transferrin and ther prteins thrugh wunds - Irn is transprted by a prtein called transferrin. Nn-transferrin bund irn, knwn as free irn, is very txic and can cause lw bld pressure, nausea, rash, facial reddening and eventually may cause heart failure and ther damage. Irn is stred in a part n a prtein called ferritin. Symptms f Anemia Pale skin clr, weakness, decreased appetite, fatigue, shrtness f breath, rapid heartbeat, palpitatin, headache, irritability, lethargy, dizziness, weight lss, lwered immunity, depressin, slw healing, bruising. What t Check First a cmplete bld cunt (CBC) must be taken. It is imprtant t check levels regularly. A CBC checks the White cells (WBC), Red cells (RBC), Hemglbin (Hb), Hematcrit (HCT), Mean cell vlume (MVC), Mean cell hemglbin (MCH), Differential (percent f different kinds f white cells), Platelets, and Retic cunt (new red cells). Als check the Sedimentatin rate (ESR) fr a crude measure f inflammatin. And check the Irn Status (irn, irn binding/transferrin saturatin, ferritin) Signs f Anemia Lw hematcrit(hct) - Lw hematcrit may indicate Anemia, malnutritin and bld lss amng ther things. Lw Hemglbin (Hb) (Hgb) - The average hemglbin level in adult wmen is 12.1 t 15.1 and in adult men it's 13.8 t Althugh it is ften cnsiderably lwer in RDEB patients. I myself have drpped t a 4

2 hemglbin nce. I am usually arund an 8 hemglbin but sme EB patients can get up t nrmal levels with prper treatment. Small red bld cells Lw serum ferritin (serum irn) level - The serum ferritin level is directly prprtinal t the amunt f irn stred in the bdy. High irn binding capacity (TIBC) in the bld Treatments Inflammatin Reducing Supplements - T imprve the ability t absrb nutrients, a supplement can be taken t help heal and reduce inflammatin in the digestive tract. Eat Healthy - Thse with Anemia shuld eat fds high in irn and ther nutrients. Oral irn supplements - Oral irn can be taken, hwever in EB the degree f deficiency is t great fr it t have much effect. Oral irn can als cause cnstipatin. A gd alternative wuld be an herbal irn supplement (an all natural supplement that cntains herbs knwn t be high in irn). Supplemental vitamins - Sme imprtant vitamins t take are: ~Vitamin B12 (100mg/day + ther B cmplex) ~Flic Acid (1mg/day) ~Vitamin E ( U/day) ~Vitamin C (30mg/kg/day) ~Vitamin K (1-5mg/day) Vitamins cme in chewable, pwder and liquid which makes it easier fr EB patients t swallw. Keep in mind that whle fd vitamins absrb better than regular vitamins. Wund care and Treatment f infectin It is extremely imprtant t take care f all wunds and treat any infectin as best yu can t reduce inflammatin. If the abve methds d nt wrk, r if the patient is already severely anemic the fllwing treatments can be dne: Intravenus irn - IV irn can help a lt and is mre effective when als paired with EPO shts. Frequency f irn infusins depends n hw severe the anemia is. Irn must be infused slwly and a test dse is always given first t mnitr any allergic reactins. Getting IV irn is an easy prcedure and usually takes a cuple hurs. Please ask yur dctr abut the risks and benefits f IV irn. Erythrpietin (EPO) shts - Erythrpietin is a naturally ccurring hrmne, prduced by the kidneys, which stimulates the bdy t prduce mre red bld cells. It is generally given as an injectin under the skin. It usually needs t be given ne t three times a week. Cmmn names fr EPO shts are Aranesp (Darbepetin Alfa), Prcrit (Epetin Alfa). Please ask yur dctr abut the risks and benefits f Ep shts. Bld Transfusin - If the irn and ep are nt effective, a bld transfusin is anther ptin. This is anther simple prcedure that takes a few hurs. Please ask yur dctr abut the risks and benefits f bld transfusins. Terms and Definitins:

3 Anemia - Anemia is a lwer than nrmal number f red bld cells (erythrcytes) in the bld, usually measured by a decrease in the amunt f hemglbin. Hemglbin is the red pigment in red bld cells that transprts xygen. Anemia f Inflammatin - This type f anemia develps as a result f extended infectin r inflammatin. Chrnic - A chrnic cnditin is cntinuus r persistent ver an extended perid f time. A chrnic cnditin is ne that is lng-standing, nt easily r quickly reslved. Cpper - Cpper is an essential trace mineral present in all bdy tissues. Cpper, alng with irn, helps in the frmatin f red bld cells. It als helps in keeping the bld vessels, nerves, immune system, and bnes healthy. Differential - The bld differential test measures the relative numbers f white bld cells (WBCs) in the bld. It als includes infrmatin abut abnrmal cell structure and the presence f immature cells. Erythrpietin - a hrmnal substance that is frmed especially in the kidney and stimulates red bld cell frmatin. Ferritin - a crystalline irn-cntaining prtein that functins in the strage f irn and is fund especially in the liver and spleen. Flate (flic acid) - Flic acid is a water-sluble vitamin in the B-cmplex grup. Flic acid wrks alng with vitamin B12 and vitamin C t help the bdy digest and utilize prteins and t synthesize new prteins when they are needed. It is necessary fr the prductin f red bld cells and fr the synthesis f DNA (which cntrls heredity and is used t guide the cell in its daily activities). Flic acid als helps with tissue grwth and cell functin. In additin, it helps t increase appetite when needed and stimulates the frmatin f digestive acids. Hemglbin - Hemglbin is the mst imprtant cmpnent f red bld cells. It is cmpsed f a prtein called heme, which binds xygen. In the lungs, xygen is exchanged fr carbn dixide. Hematcrit - The hematcrit is the percent f whle bld that is cmprised f red bld cells. The hematcrit is a measure f bth the number f red bld cells and the size f red bld cells. Inflammatin - It ccurs when tissues are injured by bacteria, trauma, txins, heat, r any ther cause. Chemicals including histamine, bradykinin, sertnin, and thers are released by damaged tissue. These chemicals cause bld vessels t leak fluid int the tissues, resulting in lcalized swelling. This helps islate the freign substance frm further cntact with bdy tissues. Irn - Irn is an imprtant trace mineral that is fund in every cell f the bdy, usually cmbined with prtein. Irn is an essential mineral fr humans because it is part f bld cells. Abut 30% f the irn in the human bdy is in strage t be readily available t replace any that is lst. Irn is essential t the frmatin f hemglbin and myglbin, which carry xygen in the bld and muscles. It als makes up part f many prteins and enzymes in the bdy. Irn Binding/Transferrin Saturatin (TIBC = ttal irn binding capacity) - A test that measures indirectly the transferrin level in the bldstream Irn-Deficiency Anemia - Irn deficiency anemia is a decrease in the number f red cells in the bld caused by t little irn. Malnutritin - Malnutritin means a persn's bdy is nt getting enugh nutrients. The cnditin may result frm an inadequate r unbalanced diet, digestive difficulties, absrptin prblems, r ther medical cnditins. Mean Cell Hemglbin - Hemglbin amunt per red bld cell (MCH) Mean Cell Vlume - Average red bld cell size (MCV)

4 Nutritinal Anemia - anemia (as hypchrmic anemia) that results frm inadequate intake r assimilatin f materials essential fr the prductin f red bld cells and hemglbin -- called als deficiency anemia Plasma - Plasma is the liquid prtin f yur bld. Plasma transprts water and nutrients t yur bdy's tissues. Plasma als cntains many prteins that help the bld t clt and fight disease. Plasma is a type f lymphcyte that prduces immunglbulin (antibdy) that is necessary fr nrmal immune system functin. Platelets - Platelets are necessary fr nrmal bld cltting (hemstasis). Mst imprtant, they aggregate (clump tgether) t plug small hles in damaged bld vessels. They als activate factr VIII (a cmpnent f the cagulatin cascade) and release phsphlipids necessary fr cagulatin. Red Bld Cells - RBCs transprt hemglbin. Hemglbin transprts xygen. The amunt f xygen bdy tissues receive depends n the amunt and functin f RBCs and hemglbin. RBCs nrmally survive abut 120 days in the bld. They are then remved by specialized "cleanup" cells in the spleen and liver. Retic Cunt - This is a test that measures the percentage f reticulcytes (slightly immature red bld cells) in bld. Sedimentatin Rate (ESR) - ESR (erythrcyte sedimentatin rate) is a nnspecific screening test fr varius diseases. This 1-hur test measures the distance (in millimeters) that red bld cells settle in uncltted bld tward the bttm f a specially marked test tube. Selenium - Selenium is an essential trace element. It is an integral part f enzymes, which are critical fr cntrl f the numerus chemical reactins invlved in brain and bdy functins. Serum Ferritin/Irn - A test that measures the amunt f irn in the bld. TIBC - Ttal irn binding capacity. A bld test that measures the ttal irn binding capacity (TIBC) as an indirect measure f transferrin. Transferrin - Irn ins are delivered in the bld by the prtein transferrin. Each transferrin mlecule can carry tw irn ins. Vitamin B6 - Vitamin B6 is a water-sluble vitamin and is part f the vitamin B cmplex. Vitamin B6 plays a rle in the synthesis f antibdies by the immune system, which are needed t fight many diseases. It helps maintain nrmal nerve functin and als acts in the frmatin f red bld cells. Vitamin B6 is als required fr the chemical reactins needed t digest prteins. The higher the prtein intake, the mre the need fr vitamin B6. Vitamin B12 - Vitamin B12 is a water-sluble vitamin that is part f the vitamin B cmplex. Vitamin B12, like the ther B vitamins, is imprtant fr metablism. It helps in the frmatin f red bld cells and in the maintenance f the central nervus system. Vitamin C - Vitamin C is a water-sluble vitamin that is necessary fr nrmal grwth and develpment. Vitamin C prmtes healthy teeth and gums, helps in the absrptin f irn, aids in the maintenance f nrmal cnnective tissue, and prmtes wund healing. It als helps the bdy's immune system. Vitamin E - Vitamin E is a fat-sluble vitamin that acts as an antixidant. Vitamin E is an antixidant that prtects bdy tissue frm damage caused by unstable substances called free radicals. Free radicals can harm cells, tissues, and rgans, and they are believed t be ne f the causes f the degenerative prcesses seen in aging.vitamin E is als imprtant in the frmatin f red bld cells and it helps the bdy t use vitamin K. Vitamin K - a fat-sluble vitamin that plays an imprtant rle in bld cltting.

5 White Bld Cells - White crpuscles in the bld. They are spherical, clrless, and nucleated masses invlved with hst defenses. Elevated cunts can be seen in cases f inflammatin and infectin. Zinc - Zinc is an imprtant trace mineral. This element is secnd nly t irn in its cncentratin in the bdy. Zinc plays an imprtant rle in the prper functining f the immune system in the bdy. It is required fr the enzyme activities necessary fr cell divisin, cell grwth, and wund healing. It plays a rle in the acuity f the senses f smell and taste. Zinc is als invlved in the metablism f carbhydrates.