New technology may facilitate early detection of sickle cell disease for infants in developing countries

University Hospitals Case Medical CenterDec 8 2015

Researchers from Seidman Cancer Center at University Hospitals Case Medical Center and Case Western Reserve University School of Medicine presented new research findings this weekend at the 57th Annual Meeting of the American Society of Hematology (ASH) in Orlando.

In a poster presentation (Abstract #3379), Yunus Alapan, Umut Gurkan PhD and Jane Little, MD presented promising findings related to new technology aimed at facilitating early detection of sickle cell disease for infants in developing countries. Current standardized screening methods are too costly and take too much time to enable equitable and timely diagnosis to save lives in economically challenged nations. However, an innovative mobile biochip device, the HemeChip, has the unique ability to rapidly screen for sickle cell disease with just a few drops of blood.

"While sickle cell newborn screening is standard in the U.S., very few infants are screened in Africa because of the high cost and level of skill needed to run traditional tests," says Dr. Little, Director of the Adult Sickle Cell Anemia Center, UH Seidman Cancer Center and Associate Professor at the School of Medicine. "This new mobile technology provides an easy to use, cost-effective tool that takes us closer to standardizing newborn screenings on mobile devices, thus simplifying diagnosis. It could make a huge difference in developing nations worldwide, enabling early treatment for this disease."

Over half of babies born with sickle cell disease (SCD) in countries with limited resources die before age five. Over 6 million people in West and Central Africa suffer from the disease, which causes pain crises, widespread organ damage and early mortality.

Today, newborn screening tests can only be performed in central laboratories in third-world countries. Results can take several weeks and it may be impossible to reach the parents after they have left the health center. This may delay the onset of important interventions, including immunizations, antibiotics and vitamins. Therefore, there is a need for simple, rapid and mobile analyses of hemoglobin types in newborn blood with which to diagnose hemoglobinopathies while the baby is still on-site.

With a miniscule blood sample, the HemeChip, a micro-electrophoretic device, examines and identifies hemoglobins, including hemoglobinopathies sickle cell anemia (HbSS), sickle trait (HbAS) and SC disease (HbSC). Using this new hemechip platform, the research team is planning to travel to Ghana to implement validation of screening in pediatric patients.

The HemeChip was developed by Dr. Gurkan, assistant professor of mechanical and aerospace engineering at Case Western Reserve University, working in collaboration with a team of researchers, including Dr. Little who is also a member of the Case Comprehensive Cancer Center at Case Western Reserve.

Additional UH Seidman Cancer Center/ School of Medicine Presentations

Leukocyte XII Regulates Venous Thrombosis Risk
Author: Evi Stavrou, MD, Hematologist and Oncologist at UH Case Medical Center and Assistant Professor at Case Western Reserve University School of Medicine
Oral Presentation, Abstract #238: Sunday, December 6, 2014, 12:45 PM in the Vascular Biology Session

This investigation examined the contribution of Factor XII (XII) in the inflammatory response and venous thrombosis with the aim to develop a target for drug development to prevent and treat blood clots without effect on bleeding.

"We have shown that Factor XII is an excellent target to prevent blood clots and are hopeful this study will lay the foundation for important work in developing new treatments," says Dr. Stavrou, lead author of study, Director of Anticoagulation Clinic at Louis Stokes Cleveland VA Medical Center and Associate Professor at the School of Medicine.

"Using these findings, we will develop the next generation of anti-blood clotting agents that does not carry the bleeding risk," says Alvin H. Schmaier, MD, co-author, the Director of the Benign Hematology Program at the UH Seidman Cancer Center and Robert W. Kellermeyer Professor at the School of Medicine. "Developing such agents would be a tremendous benefit for our cancer patients and other patients at risk for developing blood clots who also are at higher risk to bleed from their disease or therapy."

Acute Care in the Emergency Department Differs before and after Transition for Adolescents and Young Adults with Sickle Cell Disease
Authors: Nate Stehouwer, MD, Chief Medical Resident, Connie Piccone, MD, Director of Pediatric Sickle Cell Care at UH Rainbow Babies & Children's Hospital, and Jane Little, MD, Director of the Adult Sickle Cell Anemia Center, UH Case Medical Center and Associate Professor at the School of Medicine
Poster Presentation, Abstract #3267: Sunday, December 6, 2015, 6 PM - 8 PM

Painful vaso-occlusive crises (VOC) are the most common cause of emergency department (ED) visits in patients with sickle cell disease (SCD) and are a major point of contact between patients and the health care system. This study examined whether management of VOC in adolescents and young adults differed between the pediatric and the adult ED.

Researchers found that opioid medications were administered in 96% of all visits. However, time to medication administration in adult ED was nearly twice as long as in the pediatric ED (131 minutes vs. 72 minutes), and the first opioid administered was parenteral hydromorphone in 4 percent of pediatric visits and 72 percent of adult visits. Preliminary analysis suggests that transition-age patients also received higher medication dosages during ensuing admissions to the adult hospital when compared with the pediatric hospital.

These significant differences between pediatric and adult acute pain management suggest that optimal transition management for adolescents with SCD must include strategies for transition in acute care management, focusing on limiting wait times and consistent dosing and titration of pain medications, in the ED or other acute care setting.