a. Formation of abnormal hemoglobin chains containing
hemoglobin S: When red blood cells (RBCs) are exposed to
low oxygen saturation states, hemoglobin S causes the beta
cells to contract and clump together inside the cell, thus
distorting its shape.
b. Hemolysis occurs as the sickled cells clump together and
obstruct blood flow, rendering the individual vulnerable to
repeated painful crises, which can progressively destroy
i. Vaso-occlusive and thrombocytic crisis
ii. Hypoplastic and aplastic crisis
iii. Hyperhemolytic crisis
iv. Splenic sequestration crisis
c. Plasma clotting factors likely participate in the microthrombi
in the prearterioles.
d. After recurrent episodes of sickling, membrane damage
occurs, and the cells are no longer capable of resuming their
normal shape upon reoxygenation.
a. Sickle hemoglobinopathies compose a group of genetic
diseases, with the most common forms being homozygous
hemoglobin SS disease (HbSS) or sickle cell anemia, hemoglobin
SC disease, and sickle cell beta-thalassemia or Thal.
b. Primarily affects black populations of African descent as
well as people of South and Central American, Caribbean,
Mediterranean, Arabian, and East Indian descent
(Distenfeld & Woermann, 2007, Taher & Kazzi, 2007)
c. Vaso-occlusive crisis is often triggered by infection,
dehydration, fever, or local trauma.
a. Morbidity: 1 in 500 African Americans has inherited sickle
cell hemoglobin from both parents and therefore has the
disease; more than 70,000 people in the United States have
sickle cell disease (National Heart, Lung and Blood
Institute [NHLBI], 2008), accounting for over 83,000
hospital admissions in 2004 (Steiner & Miller, 2006).
b. Mortality: In 2004, there were 699 adult deaths
(Steiner & Miller, 2006).
c. Cost: In 2004, approximately $488 million was spent for
hospital costs (Steiner & Miller, 2006).
Sickle cell disease is generally managed at the community
level, with many of the interventions included here being
appropriate for this focus; however, this plan of care
addresses sickle cell crisis, which usually requires hospitalization
during the acute phase to address oxygenation
and severe pain.
Cerebrovascular accident (CVA)/stroke,
Cholecystitis with cholelithiasis,
Chronic obstructive pulmonary disease (COPD) and asthma,
Cirrhosis of the liver,
Heart failure: chronic,
Psychosocial aspects of care,
1. Promote adequate cellular oxygenation and perfusion.
2. Alleviate pain.
3. Prevent complications.
4. Provide information about disease process, prognosis,
and treatment needs.
1. Oxygenation and perfusion are adequate to meet cellular
2. Pain relieved or controlled.
3. Complications prevented or minimized.
4. Disease process, future expectations, potential complications,
and therapeutic regimen understood.
5. Plan in place to meet needs after discharge.