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

@article{Wierenga2001SurvivalEF,
  title={Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study},
  author={K. Wierenga and I. Hambleton and N. Lewis and Sickle Cell Unit},
  journal={The Lancet},
  year={2001},
  volume={357},
  pages={680-683}
}
BACKGROUND Information about life expectancy of patients with homozygous sickle-cell disease is needed for research and patient counselling. Our aim was to study two Jamaican populations, one clinic-based and one birth cohort and, by careful consideration of data quality and statistical analysis, to identify ways to increase the chances of obtaining valid and generalisable results. METHODS We investigated the survival experience of 3301 patients with homozygous sickle-cell disease attending… Expand
Causes of death and early life determinants of survival in homozygous sickle cell disease: The Jamaican cohort study from birth
TLDR
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. Expand
The changing face of homozygous sickle cell disease: 102 patients over 60 years
TLDR
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. Expand
Identifying homozygous sickle cell disease when neonatal screening is not available: a clinic-based observational study
TLDR
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. Expand
Survival and mortality among users and non-users of hydroxyurea with sickle cell disease
TLDR
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. Expand
East Mediterranean region sickle cell disease mortality trial: retrospective multicenter cohort analysis of 735 patients
TLDR
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. Expand
Morbidity-mortality in sickle cell disease
The high morbidity-mortality rates in sickle cell disease (SCD) led us to study the epidemiological profile and respective clinical complications of patients seen at a Regional Blood Center (HR) andExpand
Frequent and prolonged hospitalizations: A risk factor for early mortality in sickle cell disease patients
TLDR
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. Expand
Mortality in Sickle Cell Anemia in Africa: A Prospective Cohort Study in Tanzania
TLDR
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. Expand
Case Series and Review of Hematological and Non-Hematological Malignancies in Aging Patients with Sickle Cell Disease in the Hydroxyurea Era
TLDR
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. Expand
Outcome of Sickle Cell Anemia: A 4-Decade Observational Study of 1056 Patients
TLDR
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. Expand
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 31 REFERENCES
Improved survival in homozygous sickle cell disease: lessons from a cohort study
TLDR
Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease. Expand
Mortality in sickle cell disease. Life expectancy and risk factors for early death.
TLDR
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. Expand
Management of sickle cell disease; lessons from the Jamaican Cohort Study.
TLDR
The causes of early mortality in sickle cell disease in Jamaica are described and the major complications such as acute splenic sequestration, pneumococcal septicaemia, aplastic crisis, hypersplenism, and acute chest syndrome have been addressed with varying success. Expand
Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease.
TLDR
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. Expand
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 ofExpand
Newborn screening for sickle cell disease: effect on mortality.
TLDR
Ten years of experience in newborn screening for sickle cell disease have been summarized and hemoglobinopathies are the most prevalent congenital disease. Expand
The Development of Haematological Changes in Homozygous Sickle Cell Disease: a Cohort Study from Birth to 6 Years
TLDR
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. Expand
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% inExpand
The haematology of homozygous sickle cell disease after the age of 40 years
TLDR
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. Expand
Acute splenic sequestration in homozygous sickle cell disease: natural history and management.
TLDR
Of a cohort of 308 children with homozygous sickle cell disease diagnosed at birth, 89 experienced 132 clinically significant attacks of acute splenic sequestration (ASS) over a 10-year period, and the improvement in outcome is likely to have resulted from improvement in medical management and earlier detection of ASS. Expand
...
1
2
3
4
...