Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study

  title={Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study},
  author={Klaas J Wierenga and Ian Hambleton and Norma A Lewis and Sickle Cell Unit},
  journal={The Lancet},

Causes of death and early life determinants of survival in homozygous sickle cell disease: The Jamaican cohort study from birth

Early life biomarkers predictive of decreased survival in SS disease identify a patient group likely to benefit from close clinical supervision and potentially high risk therapies.

The changing face of homozygous sickle cell disease: 102 patients over 60 years

Elderly survivors of homozygous sickle cell disease present some features of intrinsic mildness but also manifest age‐related amelioration of painful crises and falling haemoglobin levels from progressive renal damage.

Identifying homozygous sickle cell disease when neonatal screening is not available: a clinic-based observational study

Almost 65% of children affected by homozygous sickle cell disease not identified at birth will not benefit from important early-life clinical intervention, and half will not enrol for specialised care by their 18th birthday.

Survival and mortality among users and non-users of hydroxyurea with sickle cell disease

The results indicate the importance of the nurse incorporating therapeutic advances of hydroxyurea in her care actions, and the survival curve is greater among the users of hydroXYurea.

East Mediterranean region sickle cell disease mortality trial: retrospective multicenter cohort analysis of 735 patients

The results show that despite recent progress in the treatment of SCD, disease-related factors continue to result in mortality in young adult patients, and highlight the importance of evaluating curative treatment options for patients who have an appropriate stem cell donor in addition to improving patient care and patient education.

Morbidity-mortality in sickle cell disease

The rates of hospitalization in HC-U and of visits to HR and the low mean age at death confirm the high morbidity and mortality related to SCD, however, a large number of children do not present with complications or require hospitalization which reflects the efficacy of preventive measures provided by the early diagnosis implemented over the last 10 years.

Frequent and prolonged hospitalizations: A risk factor for early mortality in sickle cell disease patients

Frequent and prolonged hospitalizations are a risk factor for early mortality in patients with SCD and patients who died had a higher mean number of hospitalization days in the year before study entry and were also more depressed than those who survived.

Mortality in Sickle Cell Anemia in Africa: A Prospective Cohort Study in Tanzania

Mortality in SCA in Africa is high, with the most vulnerable period being under 5-years old, and prompt and effective treatment for anemia inSCA is recommended as it is likely to improve survival.

Case Series and Review of Hematological and Non-Hematological Malignancies in Aging Patients with Sickle Cell Disease in the Hydroxyurea Era

Assessment of the prevalence of malignancies in aging patients with sickle cell disease at one institution with or without HU therapy found that the number of patients on HU was too small to make any comment on relationship to malignancy or mortality.

Population estimates of sickle cell disease in the U.S.

  • K. Hassell
  • Medicine
    American journal of preventive medicine
  • 2010



Improved survival in homozygous sickle cell disease: lessons from a cohort study

Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.

Mortality in sickle cell disease. Life expectancy and risk factors for early death.

Modeling revealed that in patients with sickle cell anemia, the acute chest syndrome, renal failure, seizures, a base-line white-cell count above 15,000 cells per cubic millimeter, and a low level of fetal hemoglobin were associated with an increased risk of early death.

Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease.

Comparison of this study's overall mortality with previous reports indicates improvement of survival in US patients less than 20 years of age with sickle hemoglobinopathies, and this improvement is most likely due to parental education and counseling about the illness and the early institution of antibiotics in suspected infections.

Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease.

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of

Diagnosis at birth improves survival of children with sickle cell anemia.

Studies reported from New York City, Los Angeles, New Haven, and Jamaica clearly demonstrate the calendar era change in survival that has occurred during the 1970s and 1980s, and the greatest improvement is found among those children who have sickle cell anemia.

Newborn screening for sickle cell disease: effect on mortality.

Ten years of experience in newborn screening for sickle cell disease have been summarized and hemoglobinopathies are the most prevalent congenital disease.

The Development of Haematological Changes in Homozygous Sickle Cell Disease: a Cohort Study from Birth to 6 Years

Children with SS disease were partially protected from iron deficiency in early childhood, perhaps by increased intestinal absorption of iron, and the associated increase in intracellular haemoglobin concentration might be disadvantageous during this high risk period.

Early deaths in Jamaican children with sickle cell disease.

In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87%, compared with 95% in children with sickle cell-haemoglobin C (CS) disease, and 99% in

The haematology of homozygous sickle cell disease after the age of 40 years

Age-related decreases in HbF, total haemoglobin and platelet counts and a falling reticulocyte count are consistent with a progressive bone marrow failure which is not explained by the commonly occurring renal impairment in older SS patients.