Sickle-cell anemia can become severe and dangerous during pregnancy.
Sickle cell anemia affects one in every five hundred African American pregnancies. The stress of pregnancy can initiate painful sickle cell crises. These women are also at increased risk for infection, worsening anemia, pregnancy induced hypertension and other complications. Fetal risks include premature delivery, hypoxia, small size for gestational age and stillbirth. Only individuals who have inherited a gene from each parent will be affected. A less severe form of this disease, sickle cell trait, results when the gene is inherited from only one parent, therefore the pregnancy is not usually affected.
The author reminds readers that it is essential that the mother be closely supervised and receive nutritional and emotional support. A variety of management strategies to prevent complications during pregnancy are discussed. Counseling and education are stressed by the author. Persons with sickle cell disease have increased levels of anxiety, which can trigger a pain crisis. Topics for prenatal education listed by Montgomery include adequate hydration to prevent sickling, nutrition, folic acid supplementation, importance of rest and good hygiene practices as well as recognition of signs of infection. The author clearly outlines the pathophysiology of this disease and describes various interventions commonly used during pregnancy, such as oxygen therapy.
She states that "while sickle cell crises are extremely painful, they are not life threatening. Pain management includes relaxation techniques, imagery and narcotics." Morphine, a pregnancy Category B drug, is the drug of choice. Studies indicate that no evidence of major congenital defects have been linked to morphine when it is used therapeutically. Morphine can cause neonatal respiratory depression when used in close proximity to delivery. The risk of crises is increased toward the end of the second trimester, therefore women should be encouraged to rest frequently during the day and lie with hips elevated at least once daily to promote adequate blood flow from the pelvic area.
Labor and delivery can be especially frightening for a woman with sickle cell disease and this stress may initiate a sickle cell crisis. Montgomery describes the pain as unbearable. "The woman will have generalized pain throughout her body making it difficult to distinguish labor pain from the pain of the crisis. The woman may have difficulty focusing and relaxing during labor, leading to a cycle of increasing pain." Although sickle cell anemia can be dangerous and severe during pregnancy, the chances of delivering a healthy infant are good with recent advances in care.
Montgomery, K 1996. Caring for the pregnant woman with sickle cell disease. MCN 21:5, 224-228.
By Mary Anne Ernzen