Reducing stroke risk in kids with sickle cell disease

Medical University of South Carolina stroke neurologist Robert J. Adams, M.D., knows firsthand how innovation can bring about dramatic and positive change. He was one of the STOP, Stroke Prevention Trial in Sickle Cell Anemia, investigators who found that transcranial doppler ultrasonography (TCD) could be used to assess stroke risk in children with sickle cell disease (SCD) and stroke risk could be reduced by giving the highest-risk children regular blood transfusions.

“If you put them on regular blood transfusion every three to four weeks, you eliminate 90% of the strokes in these patients,” said Adams.

This therapy is now standard of care in developed countries and has saved many lives. However, it is less common in low-resource countries, and stroke rates among children with SCD there remain high. Unfortunately, the experts who could train local physicians in the use of TCD often live far away.  

“It’s a fundamental problem that comes up every day,” said Adams. “A problem exists in location A, and the experts or the resources to solve or address that problem are in locations B, C or D.”

“It’s a fundamental problem that comes up every day. A problem exists in location A, and the experts or the resources to solve or address that problem are in locations B, C or D.”

Dr. Robert Adams, co-founder and president of Zeriscope

Adams is banking that innovation – this time in telehealth technology – can help to ensure that children with SCD around the globe benefit from stroke prevention efforts. He is co-founder and president of the Charleston-based startup Zeriscope, which is commercializing a telepresence technology capable of livestreaming both audiovisual content and quantitative data derived from sensors or medical devices. Adams thinks of the technology as a Swiss army knife, which offers tools that can be used for a wide variety of telehealth and other purposes. 

In this case, Adams will use its Teletrainer functionality to train and then certify 10 Nigerian physicians in the use of TCD to detect stroke risk in children with SCD. The original plan had been for Adams and other experts to fly to Nigeria to provide the training and certification in person, but COVID-19 made that impossible. Teletrainer saved the day by enabling high-quality remote training and certification.

 

Once trained, the physicians will take part in the Nigerian site of a multisite clinical trial that will test whether voxelotor  (trade name Oxbryta, Global Blood Therapeutics, San Francisco, California) can help to prevent progression to stroke in children with SCD who are at risk but do not meet the STOP criteria for regular blood transfusions, i.e., blood flow velocity as measured by TCD of 200 cm/s or higher. 

The Nigerian physicians will wear headsets as they interact with the patients and acquire TCD imaging. Adams and two other remote specialists, one in London and one in Ohio, will be able to see the audiovisual feed in one of the four quadrants of the Zeriscope screen. The other three quadrants will present a side view of the physician performing TCD, the TCD imaging data and a chat window.

“The Zeriscope technology is a very cost-effective way of training people to use technology and assessing their competence as it is possible to see exactly what they are doing remotely,” said Fenella Kirkham, M.D., professor of Paediatric Neurology at University College London and one of the other trainers. 

This granular detail about the environment of the room, the physicians’ interactions with patients and the acquired TCD data enables the remote expert to assess and, when appropriate, certify physicians in use of TCD for this purpose.

Adams is also helping to design the TCD training for a University of Miami study that is seeking to bring greater stroke prevention care to Haitian children with SCD. As many as 1 in 125 babies born in Haiti has SCD, and many of the prevention protocols that are common practice in developed countries are lacking there. Led by Ofelia A. Alvarez, M.D., the National Institutes of Health-funded study will compare the incidence of SCD in Haitian children ages 6 and under living in Haiti and Miami and their health outcomes. Adams will again use Teletrainer to certify Haitian physicians in the use of TCD to detect stroke risk and to review their work periodically to ensure continued quality.

“I think we're going to be living with this need for remote training, remote access. It's not a flash in the pan kind of opportunity. I think it's our new reality.”

Bill Harley, co-founder and CEO of Zeriscope

Clearly, Teletrainer has the potential to improve stroke prevention efforts in SCD in countries across the globe by providing them virtual access to experts to train and certify care providers. However, the value of Teletrainer is not limited to SCD alone. Trainers could use it to provide high-quality remote education and certification in any number of medical techniques and procedures anywhere in the world.

COVID-19 has certainly revealed the need for such remote tools and their value, according to  Bill Harley, co-founder and CEO of Zeriscope. The use of telehealth has surged, and health care executives and physicians have learned what it can do. Harley thinks this new reliance on remote technology is here to stay. 

“I think we're going to be living with this need for remote training, remote access,” said Harley. “It's not a flash in the pan kind of opportunity. I think it's our new reality.”

To learn more about Zeriscope, visit http://zeriscope.com. Read more about how Zeriscope is being used to enhance the treatment of post-traumatic stress disorder.