Approximately 4.4 million1 people globally suffer from sickle cell disease, while an additional 300 million2 have the sickle cell trait.

Global Prevalence

Sickle cell disease (SCD) is an inherited blood disorder that affects red blood cells causing a debilitating systemic syndrome characterized by chronic anemia. SCD causes acute painful episodes and often severely impacts the patient’s health starting at a very young age, and in severe cases, reduces life span by 20-30 years. Sickle Cell Disease can cause multi-organ failures, and kidneys are one of the most affected organs. It also affects neurological functions as children with Sickle Cell Disease (SCD) face more developmental delays and cognitive challenges than unaffected peers.

About 80% of all Sickle Cell Disease cases are known to occur in Sub-Saharan Africa, with an estimated 240,000 children4 born with SCD annually in this region. It is also becoming more common in the United States, Cuba, Central America, Saudi Arabia, India, Turkey, Greece, and it has recently been found in the UK, France, and Italy. Most experts think a medical cure is at least a few decades away; therefore, earlier symptomatic treatment is key to reducing suffering and prolonging life. Available remedies are often scarce, and diagnostic blood tests using syringes are difficult to administer and dispose of properly.

ILSC’s Initiative

Develop a novel urinary test device for early detection of kidney disease in SCD patients. This easy-to-administer and inexpensive test could lead to earlier treatment and prolong life.

The Science

ILSC has access to a novel, patented screening technology for kidney disease. This technology, designed for detecting small molecular weight (20 to 90 kilodaltons), non-albumin, proteins, and peptides in human urine, has significant clinical advantages over the current clinical standard – measuring albumin in the urine. Applying the technology to a simple urine dipstick will also be effective in regions without sophisticated healthcare systems. If clinical trials are successful, these devices will be inexpensive to produce and distribute globally.

–

With guidance from Elijah Paintsil, MD of the Yale Medical School and a clinical trial contract with the University of Ghana College of Health Sciences, ILSC evaluated the clinical utility of our screening technology in a proof-of-concept study of 150 patients. In this 2022 completed study, we demonstrated clinical utility in detecting SCD-associated proteinuria, i.e., detecting urinary proteins/peptides that are smaller than albumin.

After the successful completion of this proof-of-concept study, more detailed clinical validation studies will follow in 2023, including adapting the urinary protein reagents to a dipstick format. Although this device is not a cure for SCD, early detection of proteinuria will enable SCD patients to obtain earlier treatments, thereby reducing symptoms, significantly improving quality of life, and ideally prolonging lives.

About Sickle Cell Disease

“Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels. When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There is currently no universal cure for sickle cell disease.”  Sicklecelldisease.org

Sickle Cell Disease has since long been identified as a significant factor in kidney function decline. Recent studies are now suggesting that individuals with the Sickle Cell Trait (SCT) are also at risk to experience a significant decline in their kidney function. Instead of 4.4 millions of patients with SCD, additionally 300 millions of individuals are at a significant risk for Chronic Kidney Disease (CKD) and ultimately End-Stage Renal Disease (ESRD). All of which making it even more important to invest in to finding earlier treatments and prolong life.

  1. “Global Burden of Disease Study 2013”. Lancet. 386 (9995): 743–800
  2. Ashorobi D, Ramsey A, Yarrarapu SNS, et al. Sickle Cell Trait. [Updated 2022 Jul 18]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537130/
  3. Piel, FB et al, Plos Med 2013; 10(7): e1001484.
  4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6858853/