Makinano, Jubelle T.

November 14, 2019

C4 – Ma’am Tobongbanua BSN II – NCM 34

Topic Outline: Hemoglobinopathies

I. Introduction to Hemoglobinopathies
i. What is Hemoglobinopathy?
ii. Causes
iii. Early signs and symptoms
iv. Treatment

II. Newborn Screening for Hemoglobinopathies

III. Common Hemoglobinopathies Disorders

i. Sickle Cell Disease (SCD)
 Etiology
 Clinical manifestations
 Therapeutic Management

ii. Thalassemia
 Etiology
 Clinical manifestations
 Therapeutic Management

IV. Nursing Considerations

a. Sickle Cell Disease (SCD)
i. Educate family and child. Begin with an explanation of the disease and its
consequences.
ii. Emphasize the importance of adequate hydration to prevent sickling and
to delay the stasis-thrombosis-ischemia cycle in a crisis. Give specific
instructions on how many daily glasses or bottles of fluids are required.
iii. Promote supportive therapies during crises.
iv. Recognize other complications. Nurses should also need to be aware of the
signs of acute chest syndrome and CVA, both potentially fatal
complications.
v. Support family in discussing their feelings regarding transmitting a
potentially fatal, chronic illness to their child.

b. Thalassemia
i. Assist the child in coping with the anxiety-provoking treatments and the
effects of the illness.
ii. Foster the child and family’s adjustment to a chronic illness.
iii. Observe for complications of multiple blood transfusions.
iv. Explain to parents the defect responsible for the disorder, its effect on
RBCs, and the potential effects of untreated iron overload.