University of Perpetual HelpDr. Jose G. Tamayo Medical University

College of Nursing

A Group Report of:G6PD

Presented by:BSN3 – L Group 42

Presented to:Mr. Gian Kua R.N.

Introduction and History

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.

Anatomy and Physiology: Blood

Function of Bloodo Blood transport gases, nutrients, waste products, processed molecules and regulatory

moleculeso Blood regulates pH, fluid, and ion balance\o Blood is involved with temperature regulation and protects against foreign substances.o Blood clotting prevents fluid and cell loss and is part of tissue repair

Composition of Bloodo 55% plasma and 45% formed elementso Plasma contains protein (7%), water (91%), and other solutes (2%)o Formed elements contains platelets, white blood cells and red blood cells.

Red Blood Cells (Erythrocytes)o Red blood cells are disk-shape cells containing hemoglobin, giving its red color.

Hemoglobin transports oxygen and carbon dioxideo Worn out RBC are phagocytized by macrophages in the spleen or liver.

White blood Cells (Leukocytes)o WBC protects the body against microorganism and remove dead cells and debriso 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.

o 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)o Platelet are cell fragments involved with preventing blood loss

 

 

Pathophysiology

Defected X chromosome

Low G6PD

Decreased protection to RBC against oxidants

Infection or medication or diet

oxidants

Hemolysis of RBC

RBC was taken to circulation and destroyed to spleen

Hemoglobin metabolized to bilirubin

jaundice

Hemolytic Anemia

Build up of glucose Diabetes

Medical Management

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);

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.

Nursing Management

As nurse, it is very important that the patient is oriented and educated about the disorder.

Educate the client about foods and medications that may cause hemolysis.

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.

If infant, instruct the mother that best milk for the baby is breast milk.

In some agency, the patient should be instructed to wear MedicAlert bracelets that identify that they have G6PD.

Provide a sterile environment to avoid infection induced hemolysis.

Vaccination and Immunization is needed to prevent other communicable disease. This is also to avoid hemolytic attacks.