Sickle Cell Disease/Sickle Cell Anemia

Purpose of Study

What is it?

Sickle Cell Disease encompasses the range of phenotypes (HbSS, HbSC, HbSB+, Thalassemia) that derive an inherited disorder of the RBC

SCA (HbSS) is the most common and most severe form of SCD. It is inherited when both parents have a defective hemoglobin subunit beta gene

SCN manifests as a range of renal dysfunctions seen in SCD

Why is it important?

most common genetic disorder

common in regions new the equator

Nephropathy and renal injury most common complications

Early detection of markers of renal injury is highly correlated with better prognosis

Common in Sub-Saharan Africa, those with out access to medical care have life expectancy less than a decade

Complications are growing in high income countries

Prevalence and outcomes of comorbidities with sickle cell nephropathy/ renal injury

does SCA presents higher risk of SCN that other phenotypes?

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Terms to Know

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Albuminuria: Elevated levels of albumin being lost in urine, microalbuminuria commonly used early indicator of renal dysfunction

Proteinuria: Repeated instances of excess protein being excreted in urine, high levels associated with poor renal prognosis

Hematuria: Blood in the urine (gross and microscopic), common indicator of renal injury

Glomerular Filtration Rate (GFR): Measure of how well the kidneys filter blood, overall index of kidney function (%)

What did we find out?

comorbidities associated with SCA observed changed in the kidney
reflected in hematuria, proteinuria, and albuminuria ---Collectively this was acute renal changed (glomeruli filtration) as well as renal inflammation that was chronic and acute

Study period 2006-2016 identified 4 eligible studies

countries of study: US, Sub-saharan Africa, Caribbean

SCA greatest burden on those with African decent

Less access to comprehensive care compared to other genetic disorders (hemophilia, cystic fibrosis)

Whats Next

high economic cost

poor health care delivery

global problem? yes

Focus on the US?

does the problem grow into adulthood

US has acute care model but needs chronic care model

prevent crisis

coordinated health care system for adults with SCD

75% of adults dont get treatment for pain

more than 30,000 per year

most promising drug not recommended (hydroxyurea)

required screening in al 50 states for children

major gap in care begins with adults, the cost of children is still high

main issues are insurance reimbursement/coverage for care

Lack of SCD knowledge for hemotalogists

more trianing

children risk of stroke, 1/3 are monitered

how patientes are treated, previours trauama prevents going to care

treated by generalits who know so little

racism is a big factor

drs nurses not equipped

predominately AA

1/4 of SCD patients get proper medication

20% family physicians comfortable treating SCD patietns

pediatric pain 0-5 is a 5 and not treated

children into adult hood, become dependent on emergency care
opposed to hemotologist