Background: Sickle cell disease (SCD) and its variants are genetic disorders of mutant hemoglobins (Hb). The most common form found in North America is homozygous Hb S disease, first described by Herrick in 1910. Morbidity, frequency of crisis, degree of anemia, and the organ systems involved vary considerably from individual to individual.

Wheeless Textbook Discussion: - affects 1% of blacks - is more severe but less common than sickle cell trait (8% prevalence); - two main genotypes include Hb S and Hb C forms of disease; - crises usually begin at age 2-3 years and may lead to bone infarctions; (more)

Radiology of SCD

Showing AVN of humeral heads and vertebral changes

Sickle cell disease is a haemoglobinopathy with autosomal recessive inheritance. Beta chain of HbA has valine substituted for glutamine at position 6 10% of black people have this condition in the UK In the homozygote, deoxygenated HbS becomes insoluble, leading to red cells becoming rigid and sickle shaped The onset of sickle cell disease is more likely in hypoxia, acidosis, low temperature or cellular dehydration Sickling is initially reversible but when potassium and water is lost, it becomes irreversible

Sickle Cell Disease and Related Hemoglobinopathies POSNA Core Curriculum

General recommendations Sickle cell disease (SCD) is a chronic debilitating disease associated with significant perioperative morbidity and mortality. Meticulous perioperative care is required. Families are generally well informed about the condition and appreciate being involved in decisions about care. Great Ormond Street Hospital for Children, UK

Selected abstracts

Nursing oriented list of references on caring for patients with Sickle Cell Disease Haemoglobinopathy Association of Counsellors

Tropical Medicine Central Resource chapter 31

Covers the whole condition in detail. Chapter 21 has a good account of the musculoskeletal consequences of SCD and an excellent list of references

sickle cell anemia: bone manifestations

Radiology case 38--651