From: Consensus guidelines for diagnosis and management of anemia in epidermolysis bullosa
Desirable consequences probably outweigh undesirable consequences in most settings, for this reason we suggest offering these options: | Consensus agreement in percentage (%) | References |
---|---|---|
We recommend screening for Anemia in EB | Â | Â |
 1. For clinical suspected severe or generalized forms of EB, anemia should be ruled out at the age of diagnosis | 95 | 5, 6, 7, 17, 20, 21 |
 2. For moderate types of EB, anemia screening should start at 1 year of age | 84 |  |
 3. For EB simplex anemia, screening should be done only if symptomatic | 81 |  |
We recommend for the diagnosis of anemia in EB patients | Â | Â |
 1. That diagnosis and severity of anemia should be based on the WHO recommendations | 91 | 22 |
 2. An evaluation/assessment of anemia in EB requires a careful history and physical exam looking at potential causes including: | 97 |  |
  (i) Diet, (poor oral intake, lack of protein in the diet) |  |  |
  (ii) Gastrointestinal (GI) symptoms (mouth blistering and erosions, difficulty swallowing due to esophageal stenosis, stomach pain, diarrhea, constipation) |  |  |
  (iii) History of pica or pagophagia (i.e., compulsive consumption of ice) |  |  |
  (iv) Signs of blood loss (e.g., wound bleeding, epistaxis, menorrhagia, melena, hematuria, hematemesis) |  |  |
  (v) Surgical history (e.g., esophageal dilatation, hand surgery) |  |  |
We recommend treatment of anemia in EB | Â | Â |
 1. In patients with moderate to severe forms of EB, the minimum desirable level of Hb is 100 g/L (10 mg/L) | 80 | 15, 23 |
 2. Iron infusion should be administered in moderate to severe forms of EB, if Hb levels of 80–100 g/L (8-10 g/dL) and symptomatic | 86 |  |
 3. For patients with moderate to severe forms of EB who failed iron infusion, transfusion should be considered | 90 |  |
 4. For patients with severe forms of EB, transfusion should be administered if Hb < 80 g/L (8 g/dL) in adults and < 60 g/L (6 g/dL) in children** | 78 |  |
We recommend monitoring biochemistry for anemia in EB, using | Â | Â |
 1. The gold standard for diagnosis of anemia is Hb level | 90 | 20, 21, 23, 26*, 27* |
 2. Ferritin level can support diagnosis of iron deficiency. (Ferritin can be unreliable since it is also an acute phase reactant and will be high in states of inflammation, masking iron deficiency) | 83 |  |
 3. If total iron-binding capacity (TIBC) is available, it can be useful to assess iron deficiency (if high it indicates iron deficiency) | 71 |  |
 4. Patients with severe forms of EB require regular screening at fixed intervals every 6 months or at any time when symptomatic | 93 |  |
 5. Laboratory testing that may be pertinent in the initial evaluation of anemia in EB patient should, in addition to the above, include | 93 |  |
  (i) Complete blood count (CBC): hematocrit, mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH) and mean corpuscular hemoglobin concentration (MCHC) |  |  |
  (ii) Reticulocyte count |  |  |
  (iii) Iron profile (Includes serum iron, ferritin, total iron-binding content (TIBC) and soluble transferrin receptor if locally available) |  |  |
  (iv) CRP |  |  |
We recommend other treatment options to manage anemia in EB patients, include | Â | Â |
 1. Dietary measures should be offered as part of the management of anemia in all EB patients | 95 | 2,4,7 8, 13,14, 21, 22*, 25*, 31, 30, 32, 34*, 36*, 39*, 40, 41*, 43* |
 2. Optimization of iron-rich food according to geographic location should be offered as part of the management of anemia in all EB patients | 93 |  |
 3. Oral iron preparation that is readily available in each geographic area and that is tolerated by the patient should be the iron of choice | 93 |  |
 4. Oral iron supplements should be administered every other day to maximize the absorption and minimize the side effects for all patient with mild to moderate anemia | 70 |  |
 5. Oral iron should be administered for at least 4 weeks before assessing clinical benefit | 86 |  |
We recommend the outcome(s) of treating anemia in EB patients should include | Â | Â |
 1. Improvement of symptoms (more energy, less fatigue, adequate wound healing) | 98 |  |
 2. Improvement of laboratory parameters | 89 |  |