A case of sickle-cell crisis occurring near term pregnancy has been presented. This
case was further complicated by the development of severe preeclampsia which has been
shown in the literature to be more prone to develop in the patient with sickle-cell
The main problem presented is that of replacing hemoglobin to a blood volume greater
than would be found in the nonpregnant state. The fluid retention of toxemia added
to the difficulty of this problem. For these reasons the patient was treated rigorously
to accomplish diuresis while hemoglobin was being replaced very slowly with packed
erythrocytes. The object of treatment was to raise the hemoglobin level to a point
safe for delivery without overloading the cardiovascular system or precipitating further
difficulties with the sickle-cell disease.
The heart murmurs heard in this case are difficult to explain on other than a rheumatic
or congenital basis. It is a fact that the patient with sickle-cell disease in crisis
may have joint swelling, low-grade fever, and heart murmurs indistinguishable from
those of rheumatic fever; however, the persistence of these murmurs between episodes
of crisis would not be likely to occur. Burke and Rosenfeld3 reported a similar case in 1953 in which the diagnosis of rheumatic heart disease
had been made prior to the time sickle-cell disease was proved. In this case, however,
the murmurs did not persist between episodes of crisis, and it was justly assumed
that the original diagnosis was in error.
The over-all plan of therapy in this case follows that suggested in the literature.
This includes replacement of blood, use of antibiotics to prevent infection, reduction
of the amount of trauma associated with actual delivery, and rigorous treatment of
complicating factors such as toxemia. Caruso suggested that since the sickle cell
is a poor oxygen carrier, continuous oxygen therapy may be advisable during labor.
Postpartum care was directed toward replacement of blood lost during delivery, continuation
of antibiotic therapy, and general supportive care.
From this case presentation it can be seen that close supervision of the prenatal
course of all pregnant Negro patients with early efforts to diagnose the presence
of sickle-cell disease will facilitate early treatment and safe delivery.