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 G6PD deficiency was discovered as an outgrowth of an investigation of hemolytic anemia
occurring in some individuals treated for malaria with 6-methoxy-8-aminoquinoline drugs.
Favism is a disorder characterized by hemolytic anemia in response to ingestion of fava beans.
Favism as a diagnosis has been known since antiquity. One theory for the Pythagoreans'
avoidance of beans is avoidance of favism, but more likely, this was a philosophical matter,
such as the belief that beans and humans were created from the same material.
It is worth noting that the clinical investigations needed to understand why 8-amino
quinoline antimalarials cause hemolysis could not have been carried out without the
participation of prisoner volunteers serving sentences in the Illinois State Penitentiary at Joliet.
Today such studies could not be performed. If the idea that it was improper to study prisoner
volunteers had been in vogue then as it is now, these men would have been deprived of
making a contribution to society, and the discoveries that were to be made would have been
held back for years. The discovery of G6PD deficiency is a clear example of how clinical
studies carried out on prisoner volunteers can benefit society and save lives without harm to
anyone.
Glucose-6-phosphate dehydrogenase deficiency is an X-linked recessive hereditary
disease characterised by abnormally low levels of glucose-6-phosphate dehydrogenase
(abbreviated G6PD or G6PDH), a metabolic enzyme involved in the pentose phosphate
pathway, especially important in red blood cell metabolism. Individuals with the disease may
exhibit nonimmune hemolytic anemia in response to a number of causes, most commonly
infection or exposure to certain medications or chemicals.
Function of Blood
ë Blood transport gases, nutrients, waste products, processed molecules and
regulatory molecules
ë Blood regulates pH, fluid, and ion balance\
ë Blood is involved with temperature regulation and protects against foreign
substances.
ë Blood clotting prevents fluid and cell loss and is part of tissue repair
Composition of Blood
ë 55% plasma and 45% formed elements
ë Plasma contains protein (7%), water (91%), and other solutes (2%)
ë Formed elements contains platelets, white blood cells and red blood cells.
Red Blood Cells (Erythrocytes)
ë Red blood cells are disk-shape cells containing hemoglobin, giving its red color.
Hemoglobin transports oxygen and carbon dioxide
ë Worn out RBC are phagocytized by macrophages in the spleen or liver.
White blood Cells (Leukocytes)
ë WBC protects the body against microorganism and remove dead cells and debris
ë Granulocytes are leukocytes that contain cytoplasmic granules. There are 3 types of
it, neutrophils are small phagocytic cells, basophil promote inflammation and
eosinophils reduces inflammation.
ë Agranulocytes are leukocytes that have very small granules and there are 2 types:
lymphocytes are involved in antibody production and other immune response and
the monocytes that became macrophages that ingest microorganism and cellular
debris.
Platelet (Thrombocytes)
ë Platelet are cell fragments involved with preventing blood loss
Defected X
chromosome

Build up of
Low G6PD glucose Diabetes

Hemolytic
Decreased
Anemia
protection to
RBC against RBC was taken to
oxidants Hemolysis of circulation and
RBC destroyed to spleen

oxidants Hemoglobin
metabolized to
bilirubin
Infection or
medication
or diet jaundice
s Diagnostic Test
Liver enzymes (to exclude other causes of jaundice);
Lactate dehydrogenase (elevated in hemolysis and a
marker of hemolytic severity)
Haptoglobin (decreased in hemolysis);
s Other tests that may be done include:
Bilirubin level
Complete blood count, including red blood cell count
Hemoglobin - blood
Hemoglobin - urine
Haptoglobin level
LDH test
Methemoglobin reduction test
Reticulocyte count
 The most important measure is prevention - avoidance
of the drugs and foods that cause hemolysis. Vaccination
against some common pathogens (e.g. hepatitis A and
hepatitis B) may prevent infection-induced attacks.
In the acute phase of hemolysis, blood transfusions
might be necessary, or even dialysis in acute renal failure.
Blood transfusion is an important symptomatic measure,
as the transfused red cells are generally not G6PD
deficient and will live a normal lifespan in the recipient's
circulation.
Some patients may benefit from removal of the spleen
(splenectomy), as this is an important site of red cell
destruction. Folic acid should be used in any disorder
featuring a high red cell turnover. Although vitamin E and
selenium have antioxidant properties, their use does not
decrease the severity of G6PD deficiency.
s As nurse, it is very important that the patient is oriented and
educated about the disorder.
s Educate the client about foods and medications that may
cause hemolysis.
s Explain to the patient the importance of antioxidant to their
diet. Antioxidants play an important role in preventing
hemolysis since red blood cells that are G6PD Deficient
cannot defend themselves from oxidative stress. Make them
a daily addition to your diet.
s If infant, instruct the mother that best milk for the baby is
breast milk.
s In some agency, the patient should be instructed to wear
MedicAlert bracelets that identify that they have G6PD.
s Provide a sterile environment to avoid infection induced
hemolysis.
s Vaccination and Immunization is needed to prevent other
communicable disease. This is also to avoid hemolytic
attacks.