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
60% longer to get pain medication https://www-statnews-com.proxy.library.upenn.edu/2017/09/18/sickle-cell-pain-treatment/
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