Prophylactic blood transfusion has come to be regarded as necessary in the treatment of patients with sickle cell disease during pregnancy. Because of the risks associated with blood products and reports of successful outcomes without the use of blood transfusion, we conducted a prospective randomized controlled study of this issue. Seventy-two pregnant patients with sickle cell anemia were randomly assigned to one of two treatment groups: 36 received prophylactic transfusions of frozen red cells, and 36 received red-cell transfusions only for medical or obstetric emergencies. Twenty-eight patients with sickle cell anemia who did not qualify for randomization (mainly because they had other medical disorders), 66 with sickle cell-hemoglobin C disease, and 23 with sickle cell-beta-thalassemia were also followed and received transfusions only for emergencies. There was no significant difference in perinatal outcome between the offspring of mothers with sickle cell disease who were assigned to treatment with prophylactic transfusions and those who were not (15 vs. 5 percent). The occurrence of a perinatal death in a previous pregnancy and the presence of twins in the present pregnancy were two major risk factors for an unfavorable outcome; when they were present, perinatal mortality was 50 percent. Perinatal mortality was somewhat higher in the two groups that were randomized than in the three groups that were not. Prophylactic transfusion significantly reduced the incidence of painful crises of sickle cell disease (P less than 0.01) and substantially reduced the cumulative incidence of other complications of this disorder (P = 0.07). Other medical and obstetric complications occurred with nearly equal frequency in the two randomized groups. Increases in costs, the number of hospitalizations, and the risk of alloimmunization were disadvantages of prophylactic transfusion. We conclude that the omission of prophylactic red-cell transfusion will not harm pregnant patients with sickle cell disease or their offspring.

https://www.nejm.org/doi/pdf/10.1056/NEJM198812013192204?listPDF=true

Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study.

Outcome of pregnancy in homozygous sickle cell disease.

Some epidemiologic features of the painful crisis in homozygous sickle cell disease were examined in a retrospective study of 995 painful crises. Previously reported associations with cold weather and pregnancy were confirmed. There was a striking increase in painful crises in male patients between the ages of 15 and 25 years, whereas female patients showed little age-related change. The frequency of painful crises correlated positively with hemoglobin levels and reticulocyte counts in both sexes and negatively with mean corpuscular volume in female patients. There was a striking increase in painful crises in male patients with hemoglobin levels above 8.5 g/dL (greater than 85 g/L). High hemoglobin levels appear to be an important risk factor for painful crises.

https://jamanetwork.com/HttpHandlers/ArticlePdfHandler.ashx?journal=intemed&articleId=608503&pdfFileName=archinte_147_7_007.pdf

The painful crisis of homozygous sickle cell disease. A study of the risk factors.

Sickle cell disease in pregnancy: twenty years of experience at Grady Memorial Hospital, Atlanta, Georgia.

Millions of people across the world have sickle cell disease (SCD). Although the true prevalence of SCD in Europe is not certain, London (UK) alone had an estimated 9000 people with the disorder in 1997. People affected by SCD are best managed by a multidisciplinary team of professionals who deliver comprehensive care: a model of healthcare based on interaction of medical and non-medical services with the affected persons. The components of comprehensive care include patient/parent information, genetic counselling, social services, prevention of infections, dietary advice and supplementation, psychotherapy, renal and other specialist medical care, maternal and child health, orthopaedic and general surgery, pain control, physiotherapy, dental and eye care, drug dependency services and specialist sickle cell nursing. The traditional role of haematologists remains to co-ordinate overall management and liase with other specialities as necessary. Co-operation from the affected persons is indispensable to the delivery of comprehensive care. Working in partnership with the hospital or community health service administration and voluntary agencies enhances the success of the multidisciplinary team. Holistic care improves the quality of life of people affected by SCD, and reduces the number as well as length of hospital admissions. Disease-related morbidity is reduced by early detection and treatment of chronic complications. Comprehensive care promotes awareness of SCD among affected persons who are encouraged to take greater control of their own lives, and achieves better patient management than the solo efforts of any single group of professionals. This cost-effective model of care is an option for taking haemoglobinopathy services forward in the new millennium.

The comprehensive care of sickle cell disease

Pregnancy in homozygous sickle-cell anaemia

Pregnancy in abnormal haemoglobins cc, s‐thalassaemia, sf, cf, double heterozygotes

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Pregnancy in homozygous sickle-cell anaemia

Pregnancy in abnormal haemoglobins cc, s‐thalassaemia, sf, cf, double heterozygotes