A guide for health professionals working with
immigrant and refugee children and youth

Hereditary Anemias: An Overview

Key points

  • All children and youth new to Canada should be screened for anemia upon arrival.
  • Upon arrival, all children and youth new to Canada – particularly those of high-risk ethnic populations – should be considered for screening for hemoglobinopathies using high-performance liquid chromatography (HPLC), or hemoglobin analysis (e.g., Hb electrophoresis), especially if microcytic anemia is present.
  • Early recognition of sickle cell disease and major thalassemias, as well as management by a multidisciplinary team, potentially decrease hospitalizations and significant complications. 
  • All children and youth of Asian, African and Mediterranean descent should be considered for screening for glucose-6-phosphate dehydrogenase (G6PD) deficiency upon arrival, particularly if they are male, if newborn jaundice was prolonged or severe, or if treatment for malaria is required.

An approach to anemia

All children and youth new to Canada should be screened for anemia upon arrival.1 Those who have experienced substantial poverty, such as refugees, are at particularly high risk of iron deficiency anemia. Iron deficiency anemia is often due to the complex interaction of malnutrition and chronic or recurrent gastrointestinal parasitic infestations.

Iron deficiency anemia and thalassemia are the most common causes of microcytic anemia in children. Less common causes are inflammation of chronic disease, lead toxicity and, rarely, sideroblastic anemia.2

Children and youth with microcytic anemia should be screened or treated for iron deficiency empirically. Further testing for hemoglobinopathies (using HPLC or Hb electrophoresis), including sickle cell disease and thalassemia (using hematological and genotype testing), is recommended when microcytic or normocytic anemia exists in the presence of normal iron studies, and:

  • there is a clinical history consistent with these diseases
  • the patient is at risk because of ethnicity or family history.

Iron deficiency and iron deficiency anemia are further discussed in the Malnutrition section of this website. 

Children and youth of certain ethnicities are at increased risk of hereditary anemias, including:

  • sickle cell disease
  • thalassemias
  • glucose-6-phosphate dehydrogenase (G6PD) deficiency

The thalassemias and hemoglobinopathies (e.g., sickle cell disorders) affect the quantity and quality, respectively, of hemoglobin molecules within red blood cells. Poor diet and weight loss due to parasites or chronic illness can compound the adverse effects of hereditary anemias.

Health care providers should be aware that hemoglobinopathies are more common among newly arrived women from high-risk regions, and these conditions may lead to serious maternal and neonatal complications.3 Carrier screening for hemoglobinopathies is not standard practice in most parts of the world. The Society of Obstetricians and Gynaecologists of Canada recommends that women in at-risk ethnic populations be screened pre-conceptionally or as early as possible in pregnancy.4

More information is available in this resource about:

Selected resources

References

  1. Pottie K, Chambers A, Brockest B, et al; Canadian Collaboration for Immigrant and Refugee Health. Appendix 15: Iron-deficiency anemia: Evidence review for newly arriving immigrants and refugees. In: Pottie K, Greenaway C, Feightner J, et al. Evidence-based clinical guidelines for immigrants and refugees. CMAJ 2011;183(12):E824-925.
  2. Richardson M. Microcytic anemia. Pediatr Rev 2007;28(1):5-14.
  3. Pottie K, Greenaway C, Feightner J, et al. Evidence-based clinical guidelines for immigrants and refugees. CMAJ 2011;183(12):E824-925.
  4. Langlois S, Ford JC, Chitayat D, et al. Carrier screening for thalassemia and hemoglobinopathies in Canada. J Obstet Gynaecol Can 2008;30(10):950-71.

Other works consulted

Editor(s)

  • Andrea Hunter, MD
  • Anna Banerji, MD

Last updated: May, 2013

Also available at: http://www.kidsnewtocanada.ca/conditions/anemias
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Caring for Kids New to Canada is a resource for health professionals. The information here is not a substitute for medical advice, nor does it indicate an exclusive course of treatment or procedure to be followed. Variations, taking into account individual circumstances, may be appropriate.

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