Sickle Cell Anemia

Posted on
December 6, 2024
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Sickle Cell Anemia

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Tanabe, P., Spratling, R., Smith, D., Grissom, P., & Hulihan, M. (2019). CE: Understanding the Complications of Sickle Cell Disease. The American journal of nursing, 119(6), 26–35. https://doi.org/10.1097/01.NAJ.0000559779.40570.2

Sickle cell anemia is a blood disorder that causes red blood cells to become rigid and sickle-shaped, which can lead to anemia, pain, and organ damage. Inherited genetic mutations cause the body to produce an abnormal form of hemoglobin, which leads to the abnormal red blood cell shape. The most common symptom is fatigue, but other symptoms include shortness of breath, painful tissue damage, infections, jaundice (a yellowing of the skin, eyes, and mouth).

Sickle cells can get stuck in small blood vessels, which can deprive organs and tissues of oxygen-rich blood. This can lead to organ damage, and in some cases, pulmonary hypertension, which can lead to heart failure. Treatments include medicines, blood transfusions, stem cell transplants, and gene therapies. In the United States, sickle cell anemia is the most common inherited blood disorder. It occurs in about 1 in 625 people at birth, but the actual prevalence is lower due to early mortality. The sickle gene is present in about 8% of Black Americans.

Question:
Per the ACNS guidelines hyperventilation should be used routinely unless medical or other justifiable reasons contraindicate it. Sickle cell disease/trait and all of the following are listed as a contraindication except?
A
Recent intracranial hemorrhage
B
Patient age
C
Significant cardiopulmonary disease
D
Patient inability or unwillingness to cooperate
E
Correct Answer: 
B

Absolute contraindications to HV include recent (i.e. within the last 12 months) stroke (intracranial or subarachnoid hemorrhage) or myocardial infarction (MI), significant cardiac disease (i.e. poorly controlled or unstable angina), pulmonary disease (i.e. COPD causing the patient to be breathless at rest), sickle cell disease or trait, and known moyamoya disease. In situations when adequate HV may be not possible, e.g. the patient is unable to co-operate, has restrictive lung disease or is physically unable (e.g. pregnancy, frail elderly), recording physiologists should use intuition and document their decision.

References: Journal of Clinical Neurophysiology

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