Managing Sickle Cell Patients During the Blood Crisis with Edward Ivy, MD, MPH

In January, The American Red Cross reported that it was facing “its worst blood shortage in over a decade” amid surging cases of the Omicron COVID-19 virus.

For the first time since its inception, the Red Cross declared a “Blood Crisis”, with blood and platelet donations reaching critically low levels.

Among the patients in dire need of blood transfusions such as accident victims and cancer patients are patients with sickle cell disease. Approximately 100,000 cases of sickle cell disease have been recorded in United States, and the current blood shortage could prove dangerous to those affected.

Edward Ivy, MD, MPH, Vice Chief Medical Officer of the Sickle Cell Association of America (SCDAA), spoke of the implications of the blood shortage for patients with sickle cell disease in a wide-ranging interview with HCPLive.

Depending on their disease severity, patients with sickle cell may require frequent blood transfusions to manage their condition. Certain demographics such as in children with sickle cell that are at high risk of stroke and those on chronic transfusion therapy, require blood transfusions frequently.

“Unfortunately, (the shortage) will impact sickle cell patients because it will reduce their ability, to get access to the blood they need for their therapies,” Ivy said. “With the coronavirus, unfortunately, we've seen across the board shortage due to limits on personal contact and individual's isolating and staying at home, so that has had a great impact on the blood supply. And of course, because individuals with sickle cell disease have that need for chronic transfusion therapies, in some cases, then you will see it have an impact on those individuals.”

Despite this, Ivy remained optimistic about new therapies and treatments for sickle cell disease that have been in development in recent years.

“We are fortunate that there are several new treatments in the pipeline for sickle cell disease. Prior to 2019, the only therapy that was out there for sickle cell disease really was hydroxyurea, and that was a drug that was approved or being experimented on and approved in the late nineties,” Ivy said.

Since 2019, pharmaceutical companies such as Novartis and Global Blood Therapeutics have brought several treatments to the market.

Novartis introduced crizanlizumab, a new therapy intended to reduce pain frequency associated with sickle cell disease, and Global Blood Therapeutics debuted voxelator, which reduces the signaling that occurs in individuals with sickle cell disease.

Ivy also spoke of the promise of gene therapy, a practice that could eclipse bone marrow transplants as a primary treatment of sickle cell disease.

“Bone marrow transplant has been a therapy that has been used since the 1990s, but it has those limitations around finding matching donors,” Ivy said. “With gene therapy, the idea is to use the individual's own bone marrow, make some modifications to the bone marrow, and then give that back to the patient so that they can be cured of sickle cell disease. So that is starting to move along, and there are several different strategies within gene therapy that's being looked at.”

In addition to examining clinical trials and utilizing new treatment options, Ivy called for greater access to care and an expansion on the knowledge of sickle cell disease through increased training and other instructional practices.

“It's not about us trying to develop some type of research registry that puts patients’ information at risk,” Ivy said. “But it's trying to find those mechanisms that can allow individuals with sickle cell disease to receive effective care no matter where they are or no matter where they travel to.”