Contraception for individuals with sickle cell disease: a systematic review of the literature

BackgroundWomen with sickle cell disease have an increased risk of pregnancy-related complications and need safe, effective contraceptive methods to prevent unintended pregnancy.Study DesignWe conducted a systematic review to examine the safety of hormonal and intrauterine contraceptive use among women with sickle cell disease.ResultsEight articles met the inclusion criteria. The evidence was of fair to poor quality and suggested that progestin-only and combined hormonal contraception had no effect on frequency of sickle crises or other adverse events and no effect on hematologic parameters associated with sickle crises. No studies examined the risk of thromboembolism in combined hormonal contraceptive users with sickle cell disease. There was insufficient evidence to comment on the safety of intrauterine contraception.ConclusionWhile data are limited, there is no evidence to suggest that hormonal contraceptive use among women with sickle cell disease is associated with an increased risk of clinical complications.     

Cephalosporin-resistant pneumococci and sickle cell disease

Sickle cell anemia patients have 600 times the risk for invasive pneumococcal disease than their healthy peers. High-level cephalosporin resistance was described in the 1990s in healthy children from Tennessee, but its prevalence in sickle cell disease patients is unknown. Pneumococcal isolates from sickle cell disease patients from Tennessee were subjected to multilocus sequence typing to characterize antimicrobial drug-resistant strains. Twenty-one percent of strains were resistant to cefotaxime and penicillin. Of the 14 cephalosporin-resistant strains, 9 were sequence types previously described as highly cephalosporin resistant, while resistance was found for the first time in 3 clones: Maryland6B, ST660, and a novel clone, ST1753. High-level cephalosporin resistance exists in more settings than initially recognized, and its high prevalence in sickle cell disease patients may decrease the efficacy of third-generation cephalosporins in invasive pneumococcal disease.     

Human genome project and sickle cell disease

Sickle cell disease is one of the most common genetic blood disorders in the United States that affects 1 in every 375 African Americans. Sickle cell disease is an inherited condition caused by abnormal hemoglobin in the red blood cells. The Human Genome Project has provided valuable insight and extensive research advances in the understanding of the human genome and sickle cell disease. Significant progress in genetic knowledge has led to an increase in the ability for researchers to map and sequence genes for diagnosis, treatment, and prevention of sickle cell disease and other chronic illnesses. This article explores some of the recent knowledge and advances about sickle cell disease and the Human Genome Project.     

Homocysteine elevation in sickle cell disease

OBJECTIVE: Ischemic complications are common in patients with sickle cell disease. Hyperhomocysteinemia is a risk factor for arteriosclerosis and venous thrombosis, and given the propensity of patients with sickle cell disease to develop ischemic complications, we hypothesized that they might have elevated plasma homocysteine concentrations. METHODS: Plasma concentrations of homocysteine, vitamin B12 and folate were measured in 49 adults with sickle cell disease and 16 normotensive Black controls. All subjects with sickle cell disease had been prescribed folic acid 1 mg by mouth daily. RESULTS: The median plasma concentration of homocysteine of subjects with sickle cell disease was approximately 1.5-fold higher than that of controls (p=0.0008). This difference persisted, even when subjects with renal insufficiency were excluded. Plasma folate levels were 1.5-fold higher in subjects with sickle cell disease than in controls (p=0.0498). There was no significant difference in plasma vitamin B12 concentrations between the two groups. There was no difference in plasma homocysteine concentrations between transfused and non-transfused sickle cell subjects. CONCLUSIONS: Patients with sickle cell disease have elevated plasma concentrations of homocysteine in spite of elevated plasma folate levels and vitamin B12 concentrations similar to those observed in controls. Based on these data, we hypothesize that the concentration of folate required to normalize plasma homocysteine levels in patients with sickle cell disease may be higher than that of normal controls and that patients with sickle cell disease have a higher nutritional requirement for folic acid than the general population.     

Osteoarticular manifestations of sickle cell disease

Sickle cell disease is a genetic chronic anemia caused by existence of abnormal haemoglobin. Osteo articular manifestations are often observed in the evolution of the disease, and in some cases, they inaugur the disease. Authors discuss physio-pathological mechanisms and describe main osteoarticular signs of sickle cell disease.     

Acute chest syndrome in sickle cell disease

Acute chest syndrome (ACS) is a leading complication of sickle cell disease (SCD) with significant morbidity and mortality. ACS is the most common cause of death and the second most common cause of hospitalization in patients with SCD. Delineating the specific cause of ACS is often difficult, and multiple risk factors that precipitate ACS frequently coexist. The prominent risk factors include infection, hypoxia, bronchial hyperresponsiveness, the SCD genotype, and opioid use. The key to the successful treatment of ACS is early recognition and initiation of treatment without delay. The main goal is to prevent and treat acute respiratory failure and, thus, minimize irreversible lung damage. This review focuses on the risk factors, pathogenesis, clinical presentation, and management of ACS.     

Organized ambivalence: when sickle cell disease and stem cell research converge

OBJECTIVE: This article analyzes sickle cell patient families' responses to stem cell transplant recruitment efforts. It identifies key dynamics that explain why sickle cell patient families are not undergoing stem cell transplants at the rate of other patient populations. It challenges the conventional focus on 'African-American distrust' as a set of attitudes grounded in collective memories of past abuses and projected on to current initiatives, by examining the sociality of distrust produced daily in the clinic and reinforced in broader politics of health investment. DESIGN: It draws upon a two-year multi-sited ethnography of a US-based stem cell research and cures initiative. Fieldwork included participant observation in a state stem cell agency, a publicly-funded stem cell transplant program, a sickle cell clinic, and semi-structured, open-ended interviews with caregivers and stem cell research stakeholders, all of which were subject to qualitative analysis. FINDINGS AND IMPLICATIONS: This paper finds ambivalence-in-action structured by three contextual strands: therapeutic uncertainties of the clinic, institutionalized conflation of healthcare and medical research, and political contests over scientific and medical investments. The paper posits that organized ambivalence is an analytic alternative to individualized notions of distrust and as a framework for implementing more participatory research initiatives that better account for the multiple uncertainties characteristic of regenerative medicine.     

Observations on the epidemiology of sickle cell disease

The four common genotypes of sickle cell disease in Jamaica are homozygous sickle cell (SS) disease, sickle cell-haemoglobin C (SC) disease, sickle cell-β⁺ thalassaemia, and sickle cell-β⁰ thalassaemia with respective incidence at birth of 3·2, 2· 0,0· 34, and 0· 16 per 1000 live births. Haematological indices, clinical features, and over-all prognosis vary between these genotypes and also between patients within individual genotypes. Although symptomatic selection has tended to emphasize more severely affected patients, this wide variation of clinical and haematological severity is especially apparent in SS disease. Factors contributing to this variability in SS disease include the persistence of foetal haemoglobin, the association with alpha thalassaemia, and the interaction with environmental factors of which socioeconomic status is the most obvious. Further elucidation of factors determining the severity of SS disease will increase understanding of the pathogenetic mechanisms in the disease and may also identify new possibilities for therapeutic intervention.     

Iron status of children with sickle cell disease

BACKGROUND: Dietary iron requirements are unclear in children with SS-type sickle cell disease. METHODS: Iron status was assessed in 104 nontransfused African American children (aged 0.5 to 17.6 years) with sickle cell disease who receive no iron supplement. Dietary iron intake was not measured at the time of this study. RESULTS: Serum ferritin was normal or high in all children. Other hematologic and biochemical indicators of iron deficiency were in the normal range in most children. CONCLUSIONS: Unlike previous studies, this sample of children and adolescents did not show signs of iron deficiency.