Is the group of older sickle cell disease patients from Trinidad and Tobago different?

Losada, Rafael; Bravo, Idania; Capildeo, Kavi; Charles, K. S.

doi:10.1002/ajh.20505

Cited by 3 publications

(1 citation statement)

References 5 publications

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“…We recognize that the rate of development of chronic disease complications is gradual and occurs at variable rates so that much of the evidence and recommendations we give will be applicable to all ages of adults. Observational studies from several centers confirm progressive renal disease and worsening anemia, [15][16][17][18][19] as well as incidental and overt neurological complications 20 with increasing age. Nonsickle-related comorbidities will also increase with age, 18 adding to the disease burden.…”

Section: Introductionmentioning

confidence: 93%

How I treat the older adult with sickle cell disease

Thein

Howard

2018

Blood

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With increasing survival, cumulative complications of sickle cell disease (SCD), which develop insidiously over time, are becoming more apparent and common in older patients, particularly those in their fifth decade and beyond. The older patient is also more likely to develop other age-related nonsickle conditions that interact and add to the disease morbidity. A common misconception is that any symptom in a SCD patient is attributable to their SCD and this may lead to delays in diagnosis and appropriate intervention. We recommend regular comprehensive reviews and monitoring for early signs of organ damage and a low threshold for the use of hydroxyurea and blood transfusions as preventative measures for end-organ disease. Treatable comorbidities and acute deterioration should be managed aggressively. Although the primary goal in management of the older adult with SCD is improving anemia and minimizing organ damage, the time has come for us to be more proactive in considering curative therapies previously offered to the younger patient. Curative or experimental interventions should be discussed early, before complications render the patients ineligible for these treatments.

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Section: Introductionmentioning

confidence: 93%

How I treat the older adult with sickle cell disease

Thein

Howard

2018

Blood

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Aging in Sickle Cell Disease: Co-morbidities and New Issues in Management

Sandhu

Cohen

2015

Hemoglobin

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Availability of hydroxyurea (HU) coupled with early therapeutic interventions has increased the life expectancy of patients with sickle cell disease. Hence, the sickle cell community needs to be aware of common diseases of aging that survivors are predisposed to. We chose to investigate the sickle cell disease-related complications as well as non sickle cell disease-related medical problems of aging in 45 sickle cell patients over the age of 40 years. The most frequent chronic complications of sickle cell disease were elevated tricuspid regurgitant jet velocity on echocardiogram, chronic renal disease, iron overload and leg ulcers. Medical co-morbidities in this patient group included hypertension, diabetes mellitus (DM), hypercholesterolemia and symptomatic coronary artery disease (CAD). In our cohort, only 38.0% had a primary care doctor. Only 11.0% over age 50 had a screening colonoscopy, and of the women, 42.0% had a screening mammography. Medical co-morbidities and lack of health maintenance in older sickle cell patients are likely to impact overall health and mortality. Aging patients with sickle cell disease may benefit from a primary medical home for age appropriate comprehensive health care.

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Association of Clinical and Hematological variables with the disease severity in Indian Sickle cell anemia patients

Bhaskar

Pattnaik

2021

RJPT

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Sickle cell anemia (SCA) is the most common genetic disorder that is caused due to mutation of the β globin gene. Although SCA is a monogenic disorder, the clinical presentation varies greatly among patients. The present study was designed to be a cross sectional study, aimed at analysing the SCA severity and its association with different clinical, biochemical and hematological variables in SCA patients of Indian origin. About 190 random homozygous SCA patients confirmed by hemoglobin electrophoresis were used in the study. Routine biochemical laboratory (liver function test and Renal function test) and hematologic tests (Total hemoglobin, fetal hemoglobin, hematocrit, MCV and MCH) were done. Values pertaining to complete blood count (CBC), Hb-HPLC and clinical investigations were collected from patient’s records. The mean age of patients with severe disease was significantly lesser than the moderate and mild disease patients. The body mass index (BMI) was also significantly lower in severe disease patients compared to the moderate and mild disease. The patients with severe disease had low levels of red blood cells, total hemoglobin (tHb) and fetal hemoglobin (HbF) compared to the other groups. There is no significant difference in the kidney and liver function among various degrees of disease severity. In summary, this study demonstrates that the tHb and HbF and total leucocyte count (TLC) are major prognostic factors for several clinical complications in SCA. Baseline measurement of these important variables is paramount in predicting important aspects of clinical course and improves the quality lives of these children.

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Is the group of older sickle cell disease patients from Trinidad and Tobago different?

Cited by 3 publications

References 5 publications

How I treat the older adult with sickle cell disease

How I treat the older adult with sickle cell disease

Aging in Sickle Cell Disease: Co-morbidities and New Issues in Management

Association of Clinical and Hematological variables with the disease severity in Indian Sickle cell anemia patients

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