scholarly journals Diagnosis patterns of sickle cell disease in Ghana: a secondary analysis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Alexandra M. Sims ◽  
Kwaku Osei Bonsu ◽  
Rebekah Urbonya ◽  
Fatimah Farooq ◽  
Fitz Tavernier ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Screening Program ◽  
Medical Center ◽  
Academic Medical Center ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Mortality And Morbidity ◽  
Pain Crisis ◽  
First Pain

Abstract Background Despite having the highest prevalence of sickle cell disease (SCD) in the world, no country in Sub-Saharan Africa has a universal screening program for the disease. We sought to capture the diagnosis patterns of SCD (age at SCD diagnosis, method of SCD diagnosis, and age of first pain crisis) in Accra, Ghana. Methods We administered an in-person, voluntary survey to parents of offspring with SCD between 2009 and 2013 in Accra as a part of a larger study and conducted a secondary data analysis to determine diagnosis patterns. This was conducted at a single site: a large academic medical center in the region. Univariate analyses were performed on diagnosis patterns; bivariate analyses were conducted to determine whether patterns differed by participant’s age (children: those < 18 years old whose parents completed a survey about them, compared to adults: those > = 18 years old whose parents completed a survey about them), or their disease severity based on SCD genotype. Pearson’s chi-squared were calculated. Results Data was collected on 354 unique participants from parents. Few were diagnosed via SCD testing in the newborn period. Only 44% were diagnosed with SCD by age four; 46% had experienced a pain crisis by the same age. Most (66%) were diagnosed during pain crisis, either in acute (49%) or primary care (17%) settings. Children were diagnosed with SCD at an earlier age (74% by four years old); among the adults, parents reflected that 30% were diagnosed by four years old (p < 0.001). Half with severe forms of SCD were diagnosed by age four, compared to 31% with mild forms of the disease (p = 0.009). Conclusions The lack of a robust newborn screening program for SCD in Accra, Ghana, leaves children at risk for disease complications and death. People in our sample were diagnosed with SCD in the acute care setting, and in their toddler or school-age years or thereafter, meaning they are likely being excluded from important preventive care. Understanding current SCD diagnosis patterns in the region can inform efforts to improve the timeliness of SCD diagnosis, and improve the mortality and morbidity caused by the disease in this high prevalence population.

scholarly journals Diagnosis patterns of sickle cell disease in Ghana: a secondary analysis using cohort data

2021 ◽  
Author(s):  
Alexandra M. Sims ◽  
Kwaku Osei Bonsu ◽  
Rebekah Urbonya ◽  
Fatima Farooq ◽  
Fitz Tavernier ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Screening Program ◽  
Severe Disease ◽  
Secondary Analysis ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Screening Programs ◽  
Pain Crisis ◽  
First Pain

Abstract Background: Despite having the highest prevalence of sickle cell disease (SCD), Sub-Saharan Africa lacks a robust screening program. We sought to capture the diagnosis patterns of SCD, particularly age and mechanism at SCD and age of first pain crisis, in Accra, Ghana. Methods We administered a survey to parents of offspring with SCD between 2009–2013 in Accra as a part of a larger cohort study and analyzed a subset of the data to determine diagnosis patterns. Univariate analyses were performed on diagnostic patterns; bivariate analyses were conducted to determine if patterns differ by offspring’s age, or their disease severity. Pearson’s chi-squared were calculated. Results Data was collected on 354 unique participants from parents. 44% were diagnosed with SCD by age four; 46% had experienced a pain crisis by the same age. 66% were diagnosed during pain crisis, either in acute (49%) or primary care (17%) settings. Younger (< 18 years) offspring were diagnosed with SCD at an earlier age (74% by four years old); in the adult ( > = 18 years) group, 30% were diagnosed by four years (p < 0.001). Few were diagnosed via newborn screen. Half with severe disease were diagnosed by age four, compared to 31% with mild disease (p = 0.009). Conclusions These findings reflect a reliance on diagnosis in acute settings, and a relative underutilization of systematic screening programs. By understanding current patterns, opportunities remain to more effectively detect and treat SCD in this high prevalence population.

Age of first pain crisis and associated complications in the CASiRe international sickle cell disease cohort

2021 ◽  
Vol 88 ◽  
pp. 102531
Author(s):  
Immacolata Tartaglione ◽  
Crawford Strunk ◽  
Charles Antwi-Boasiako ◽  
Biree Andemariam ◽  
Raffaella Colombatti ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Pain Crisis ◽  
First Pain

scholarly journals Responsivity of Utilization Rates to the Intensity of Case Management over Time Among High-Utilizing Adults with Sickle Cell Disease

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5803-5803
Author(s):  
Wally R Smith ◽  
Daniel Sop ◽  
Shirley Johnson ◽  
Thokozeni Lipato ◽  
Sarah Hartigan
Keyword(s):  
Sickle Cell Disease ◽  
Case Management ◽  
Sickle Cell ◽  
Medical Center ◽  
Academic Medical Center ◽  
Fiscal Year ◽  
Cell Disease ◽  
Outpatient Visits ◽  
Academic Medical ◽  
Over Time

Background: Case management (CM) and community health workers (CHWs) are two evidence-based health management strategies that can help reduce health risks, reduce readmission rates, and improve patient-provider relationships, all of which may be suboptimal in adults with sickle cell disease (SCD). In Fiscal Year (FY) 2016, the actual average vs. expected average length of stay (ALOS) among SCD adults at Virginia Commonwealth University Medical Center (VCU), an urban academic medical center, was 6.7 days vs 4.2 days. The 30-day readmission rate was 33.7%. We have previously presented results of a year-long pilot CM and CHW intervention for five of VCU's highest adult SCD utilizers, measured by calendar year (CY) 2015 VCU charges (Sop, et al., Blood 2017). Comparing CYs 2015 and 2016, there were numeric improvements in inpatient discharges, 30-day readmissions, 3-Day Emergency Department (ED) returns, ED discharges, outpatient visits, inpatient days, and total VCU charges. Herein, we show results of an extension of the pilot to evaluate responsivity to change over time: whether these improvements were lost among these same patients in the subsequent year (CY 2017) with diminished CM due to loss of funding, and whether they recurred in CY 2018, when CM was more robust as a result of new, more stable funding. Methods: Using the VCU charge and utilization database, we compared the above utilization variables for the five highest CY 2015 utilizers in CYs 2018, 2017, 2016 and 2015. The five original patients included 2 males and 3 females aged between 25-31 years old. One of the females passed away in 2016. Results: There was a trend toward numerically lower utilization during the two CM and CHW years (2016 and 2018), compared to the two diminished CM years (2015 and 2017). Inpatient discharges, inpatient days, and total charges see-sawed according to the intensity of intervention, going down during the year of the initial intervention, rising during the year after intervention, and going down again with resumption of intervention. However, there were notable exceptions: 30-day re-admissions were the lowest in 2017 a diminished intervention year; 3-day ED returns were flat throughout; ED discharges declined throughout, and; outpatient visits rose throughout. Conclusions: These pilot results must be interpreted with caution, since the sample was very small, and interventions were not standardized or randomized, so that secondary trends could be excluded. We found that, after diminution in 2017 of an initial CM and CHW intervention in 2016, several of the initial 2015-to-2016 improvements in academic medical center utilization for five high-utilizing adult SCD patients deteriorated. Subsequently, these improvements recurred when CM and CHWs were reapplied in 2018. We also found that other utilization improvements were sustained despite diminution or resumption of the intervention. This pilot result lays the groundwork for larger, more rigorous CM and CHW Table Disclosures Smith: Novartis: Consultancy, Honoraria. Lipato:Novartis: Honoraria.

Screening of the Mother During Early Pregnancy

PEDIATRICS ◽  
1989 ◽  
Vol 83 (5) ◽  
pp. 884-885
Author(s):  
Yolanda Rooks
Keyword(s):  
At Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Screening Program ◽  
Medical Center ◽  
Medical Community ◽  
Cell Disease ◽  
Sample Collection ◽  
The Family

Screening for the early diagnosis of sickle cell disease has been advocated as essential for reducing infant mortality and morbidity. The provision of comprehensive and preventive care optimizes the chance for a longer and more healthy life by minimizing the detrimental complications that often occur in early life. Accordingly, the goals of early identification and intervention include preventing complications through the anticipation of medical problems, educating the family so that they become knowledgeable about sickle cell disease and how it may affect their child, and assisting the family in identifying specialized medical centers where appropriate care and follow-up will occur. MATERIALS AND METHODS The target population in New Haven, CT, was defined as black and Puerto Rican gravida women giving birth at Yale Medical Center. Screening these at-risk pregnant women and the fathers permitted prior identification of the newborn at risk. This was perceived to be a method of decreasing the number of cord blood specimens to be screened as well as increasing the yield. Screening guidelines mandated education, informed consent, and appropriate follow-up for parents whose infants were identified to be heterozygous or homozygous for a sickle variant. The protocols for sample collection and sample testing were established. Hemoglobin electrophoresis was performed by the following two techniques: (1) cellulose acetate at pH 8.4 and (2) citrate agar gel at pH 6.2. The latter technique facilitates the identification of Hb S and Hb C in the presence of large amounts of Hb F3. The screening program was introduced to the medical community and support staff.

Newborn Screening for Sickle Cell Disease in Tanzania: The Past, Present and Future

2020 ◽  
Vol 31 (2) ◽  
pp. 79-82
Author(s):  
Fredrick Luoga ◽  
Agness Jonathan ◽  
Lulu Chirande ◽  
Emmanuel Balandya
Keyword(s):  
Sickle Cell Disease ◽  
Newborn Screening ◽  
Sickle Cell ◽  
Medical Center ◽  
National Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
High Prevalence ◽  
Sub Saharan ◽  
Haemoglobin Molecule

Sickle Cell Disease (SCD) is an inherited disorder of the Haemoglobin molecule of the red blood cells that is associated with serious complications and reduced life expectancy. Over 75% of people with SCD live in Sub-Saharan Africa (SSA), and this proportion are projected to increase to 85% by the year 2050. In Tanzania, approximately 11,000 babies are born with SCD each year, ranking 5th in the world. The high prevalence of SCD in SSA is compounded by the disproportionately higher mortality compared to that observed in the high-income countries. In Tanzania, SCD is a major contributor to under-five mortality and is estimated to account for 7% of all-cause mortality in this age group. Newborn screening (NBS) is the practice of testing babies right after delivery to ascertain whether they have diseases that are potentially lethal if not treated early. Where routinely practiced, NBS has significantly reduced morbidity and mortality associated with such diseases. The Sickle Cell Programme at Muhimbili University of Health and Allied Sciences (MUHAS) in Dar-es-salaam and Bugando Medical Center in Mwanza have both conducted pilot NBS for SCD, showing that the intervention is generally feasible and acceptable in Tanzania. The successful introduction and expansion of NBS in Tanzania will require careful planning and advocacy at community to national level.

Impact of Intravenous Opioid Shortage on Managing Pain Crisis in Sickle Cell Disease

2021 ◽  
pp. 106002802110245
Author(s):  
Jin Han ◽  
Santosh L. Saraf ◽  
Michel Gowhari ◽  
Faiz Ahmed Hussain ◽  
Shivi Jain ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Pain Crisis

Living with sickle cell disease: voices from sub-Saharan Africa

2021 ◽  
Author(s):  
Arafa Said Salim ◽  
Emmy Mwita ◽  
Joseph Sarfo Antwi ◽  
Olamide Agunkejoye ◽  
Paul Mdliva
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Sub Saharan

Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Hemoglobin Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Jet Velocity ◽  
Sub Saharan

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.

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