SAN DIEGO -- The American Society of Hematology (ASH) previewed clinical guidelines on sickle cell disease (SCD) here, with the intention of releasing the full set of guidelines in 2019.
The chairs from each of the five SCD guideline panels -- sickle cell disease-related transfusion support; sickle cell disease-related cardiopulmonary and kidney disease; sickle cell disease-related cerebrovascular disease; sickle cell disease-related pain; and stem cell transplantation for sickle cell disease -- discussed a key recommendation from each of their panels at the ASH annual meeting.
These five SCD panels, which included more than 75 experts across the field, have formulated more than 50 recommendations on SCD. Some draft recommendations veer away from standard practice, or even address issues that haven't been addressed in previous SCD guidelines.
For example, John F. Tisdale, MD, of the National Heart, Lung, and Blood Institute in Bethesda, Maryland, pointed out that ASH is putting out the first set of guidelines addressing transplantation in SCD, and noted the stem cell transplantation panel's recommendation of "transplantation at a younger age over transplantation at an older age."
The Panel on Sickle Cell Disease-Related Pain has suggested that treatment for adults and children who require hospital care for acute pain episodes should veer "in favor of hospital-based entities (i.e., day hospitals and infusion centers, all with appropriate expertise to treat pain) over typical emergency department [ED]-based care."
Amanda Brandow, MD, of the Medical College of Wisconsin in Milwaukee, noted that SCD may face barriers in EDs that may impact care. "So, looking at these alternative care models for acute pain management, such as day hospitals and infusion panels, was a priority to our panel," she said.
Michael DeBaun, MD, of Vanderbilt University Children's Hospital in Nashville, said the Panel on Sickle Cell Disease-Related Cerebrovascular Disease was committed "to maintaining cognitive function as the highest priority -- and that priority was greater than being alive with minimal cognitive function."
"All members of our panel believe very strongly that identifying silent cerebral infarcts in school-age children, and in adults with hemoglobin SS and Hemoglobin β-Thalassemia, is an important priority," said DeBaun.
Consequently, that panel has recommended that early school-age children and adults with hemoglobin SS and hemoglobin β-thalassemia who can undergo an MRI study without sedation receive a one-time unsedated MRI to screen for silent cerebral infarcts.
On the other hand, ASH has recommended against performing routine screening echocardiograms to identify pulmonary hypertension in asymptomatic children and adults with SCD.
That recommendation is based on low quality evidence, said Sophie Lanzkron, MD, of Johns Hopkins Medicine in Baltimore. She added that it is important to make sure that people are clear on how the question of symptomatic vs asymptomatic is defined.
ASH has also recommended that new guidelines regarding blood pressure (BP) should apply to SCD as well. In 2017, the American College of Cardiology/American Heart Association updated their , which reduced blood pressure thresholds for antihypertensive therapy in high-risk adults to >130/80 mm Hg, and BP treatment goals to <130/80 mm Hg for most individuals.
"Evidence from the general population shows that intensive blood targets -- initiation of therapy at blood pressure of 130/80 -- resulted in decreases in cardiovascular mortality, stroke, major cardiovascular events, and heart failure," said Lanzkron. "And the panel has no reason to believe that the SCD population would have different outcomes from the general population regarding blood pressure management."
The Panel on Sickle Cell Disease-Related Transfusion Support looked at the question of whether patients with an acute need for transfusion, but a high risk of hemolytic transfusion reaction (HTR), should or should not undergo immunosuppressive therapy.
It suggested that immunosuppressive therapy (IVIg, steroids, and or rituximab [Rituxan]) be used "to minimize risk of a HTR in patients with SCD with an acute need for transfusion at high risk of a HTR, or with a history of multiple or life-threatening delayed HTRs."
Robert Liem, MD, of Ann and Robert Lurie Children's Hospital of Chicago, and chair of the ASH Sickle Cell Disease Guideline Coordination Panel, said one of the issues that complicated the formulation of many of these guidelines was the lack of quality data.
"I think the reality is we have a low level of evidence for a lot of these recommendations," said Liem. "It doesn't mean that we shouldn't have tried to address these questions, because I think the way we look at these sickle cell guidelines from ASH is that it is really to look at the hard questions and challenging clinical scenarios that may or may not have been addressed by the
He explained that the panels were instructed to address questions of practice that primary care providers, hematologists, and SCD providers may have trouble with, adding that "so even in the setting of low-quality evidence, we heard loud and clear that people needed some guidance."
Primary Source
American Society of Hematology
American Society of Hematology "Special Interest Session on ASH Clinical Guidelines on Sickle Cell Disease" ASH 2018.