Sickle Cell Anemia (Subscribe)
Categories
- Patient Information SCD (8)
- Information aimed at educating patients and parents of patients with Sickle Cell Disease
Links
Sickle Cell Anemia eMedicine
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.
Sickle Cell Anemia Musculoskeletal Aspects
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)
Sickle Cell Disease
Showing AVN of humeral heads and vertebral changes
Sickle Cell Disease AnaesthesiaUK
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 POSNA
Sickle Cell Disease and Related Hemoglobinopathies
POSNA Core Curriculum
Sickle Cell Disease suggested perioperative management
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
STAC Reading List
Nursing oriented list of references on caring for patients with Sickle Cell Disease
Haemoglobinopathy Association of Counsellors
The Hemoglobinopathies Sickle Cell Disease and Thalassemia
Tropical Medicine Central Resource chapter 31
The Management of Sickle Cell Disease
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
sickle cell anemia: bone manifestations
Editors
- Chris Oliver
