Bone Pain Crisis Research Articles

Pregnancy in homozygous sickle-cell anaemia.

SummaryIn 12 years (1958–69) 38 patients with homozygous sickle‐cell anaemia (SS) were studied during the course of 61 pregnancies. Maternal prognosis was poor, with 7 deaths. The causes of mortality and morbidity were severe anaemia, acute sequestration, bacterial infections, bone pain crises, and bone marrow embolism. The proportion of these patients requiring operative deliveries was high chiefly because of fetopelvic disproportion from generally contracted pelvis. For obstetric operations various types of anaesthesia were used with excellent results, provided gross anaemia was corrected and hypoxia was avoided. When bone pain crises occurred during late pregnancy, labour and in the early puerperium, heparinization and blood transfusions were freely used.Fertility was reduced in these women, and both the spontaneous abortion rate and fetal wastage were at least twice as high as they were in the general population. The average birthweight of the babies was comparatively low, for no obvious reasons.

Pregnancy in abnormal haemoglobins CC, S-thalassaemia, SF, CF, double heterozygotes.

SummaryIn 12 years, 90 pregnancies were studied in 51 Nigerians with the following abnormal haemoglobin types: C homozygotes (39 patients), S‐β‐Thalassaemia (5 patients), high fetal haemoglobin combined with haemoglobin S (4 patients) double heterozygotes (2 patients) and high fetal haemoglobin combined with haemoglobin C (1 patient). There were no maternal deaths.Homozygous C haemoglobin is associated with anaemia and splenomegaly. High fetal haemoglobin combined with S, or C, and also double heterozygotes are apparently innocuous in pregnancy. Therefore, no specific treatment is required in these haemoglobin types, apart from the prevention of anaemia by folic acid supplements in all cases, with the addition of oral iron and antimalarial drugs in areas where iron deficiency anaemia is common and malaria is holoendemic.By contrast patients with haemoglobin S‐β‐Thalassaemia require close supervision throughout pregnancy since this haemoglobinopathy is associated with anaemia, bone pain crises and an increased risk of postpartum haemorrhage. However, it is less morbid in comparison with haemoglobin SS and SC.

The influence of hemoglobinopathies on reproduction

The effect of hemoglobinopathies on reproduction is discussed.Fertility appears to be reduced in sickle cell anemia but not in sickle cell hemoglobin C disease. Both these conditions cause profound morbidity and mortality in pregnancy. Severe anemia is preventable by the administration of folic acid, and in addition antimalarials in endemic areas. Bone pain crisis is increased in pregnancy particularly during the last trimester, in labor and in the puerperium. Bone pain crises in themselves are not dangerous, but marrow embolism following infarction in a long bone may occur and can be fatal. When the syndrome of “pseudotoxemia” occurs during a bone pain crises, death from marrow embolism is imminent and urgent measures have to be adopted to prevent death. The prevention and treatment of bone pain crisis is discussed, and it appears that heparin has eliminated death due to marrow embolism. Acute sequestration of red cells to the liver and spleen sometimes occurs during a bone pain crises or as a terminal event in severe megaloblastic anemia, and exchange blood transfusion with normal blood (hemoglobin A/A) is lifesaving. The formidable mortality of these diseases can be reduced by vigilant care during pregnancy, labor, and the puerperium.Sickle cell disease in pregnancy does increase the risk to the fetus but with close supervision and the prophylactic measures described, the prematurity and perinatal death rates can be considerably reduced.Sickle cell thalassemia, sickle cell high fetal hemoglobin disease and homozygous C disease do not cause any anxiety in pregnancy, provided folic acid is administered.There is no evidence that fertility is reduced in women with the sickle cell trait. The incidence of pyelonephritis in pregnancy and the puerperium is increased but earlier views that the abortion rate was also increased have not been confirmed. Similarly there is no evidence that pre-eclamptic toxemia and prematurity are increased in women with A/S in pregnancy. It is pointed out however, that A/S is not as innocuous as formerly believed.Homozygous hemoglobin E and hemoglobin E thalassemia cause anemia in pregnancy and megaloblastic erythropoiesis and premature labor are likely to occur.Homozygous α-thalassemia in pregnancy causes hydrops and erythroblastosis fetalis and intrauterine death in Southeast Asia. It is considered that in Asian countries abnormal hemoglobin production is a more important factor in the causation of this condition than Rh isoimmunization.