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.
Outcome of Sickle Cell Anemia: A 4-Decade Observational Study of 1056 Patients
The need for preventive therapy to ameliorate the progression of the sickle vasculopathy is underscored, and the influence of calendar era, age, sex, and prior medical conditions on the subsequent development of irreversible organ damage and survival is investigated.
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 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.
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
Survival analysis in natural history studies of disease.
The application of methods for truncated survival data are described, and these are compared with the standard product limit estimator and proportional hazards models in the measurement of survival from entry.
The unseen sample in cohort studies: estimation of its size and effect. Multicenter AIDS Cohort Study.
For data with known truncation times, non-parametric maximum likelihood estimates of the size, exposure/infection dates and distribution of incubation time in the unseen sample are developed and consistency properties are given and variability is assessed using bootstrap methods.