Sickle-cell anemia and pulmonary hypertension

Abstract

Purpose: To provide nurse practitioners (NPs) information about sickle-cell disease (SCD) and pulmonary hypertension (PHTN) as a complication. A case study is presented to illustrate the diagnosis of PHTN in a patient with SCD. Clinical manifestations, diagnosis, and interventions for both SCD and PHTN are also discussed.

Data source: A literature search to support this case review was conducted in PubMed and SCOPUS using key search words. Specific information for the case study was obtained from a patient in a primary care setting.

Conclusion: Pulmonary hypertension is frequently diagnosed secondary to hemoglobinopathies such as SCD. Patients with sickle-cell anemia, a lifelong hereditary hemoglobinopathy, are now surviving into adulthood because of the advances made in treatment. About one third of these patients develop pulmonary hypertension, a complication that carries a 40% mortality rate. Delayed diagnosis can affect the patient's quality of life. Timely diagnosis and referral is imperative, but only if the advanced practitioner is able to recognize this common complication.

Implications for practice: Many patients with SCD are seen in primary care practices. Such is the situation for the patient in this case study. Familiarity with pulmonary hypertension secondary to SCD can increase the awareness of NPs of this potential consequence and initiate early diagnostic evaluation.