Article type
Abstract
"Background: In sickle cell disease (SCD) sickled red blood cells lead to intravascular hemolysis, chronic hemolytic anemia, vaso-occlusion, acute pain, and damage to various organs. SCD is one of the most common genetic diseases in the world with a higher incidence in Afro-descendant populations. For this reason, the SCD was neglected and remained invisible for a long time due to a lack of interest resulting from structural and institutional racism. In Brazil, it is estimated that 4% of the population has the SC trait.
Objectives: Describe the main recommendations in the Unified Health System (SUS) clinical practice guideline (CPG) for the care of children and adults with suspected or diagnosed SCD, as well as the guideline update process.
Methods: The CPG update process followed the recommendations of the GRADE system. Researchers, hematologists, representatives of the Brazilian Ministry of Health (MoH), health professional society and patients' representatives participated in this process. After three planning meetings, the scope of the document was outlined, and technologies were prioritized regarding the potential for coverage in SUS.
Results: SCD diagnosis, treatment (pharmacological – prevention of crises and complications, treatment of infections, iron and folic acid supplementation, transfusional iron overload, and analgesia; and non-pharmacological treatment – transfusion, hematopoietic stem cell transplant, nutrition, physiotherapeutic, respiratory and ophthalmological support, oral health, priapism, lower limb ulcers, neurocognitive complications, and surgical procedures) and monitoring were recommended. Highlights included expansion of hydroxyurea indication to nine-months-old children (in addition to 24-years-old children), coverage of a 100 mg tablet of hydroxyurea (in addition to available 500 mg capsules), and indication of epoetin alpha to adults showing a decline in kidney function and worsening of hemoglobin levels. The document is under review by the MoH and will be considered by Conitec (National Committee for Health Technology Incorporation). The next steps comprehend Conitec evaluation and decision, public consultation, and CPG publication.
Conclusions: The guideline will consolidate recommendations for the care of people with suspected and diagnosed SCD, contributing to the quality of primary and secondary health care, and respecting the health system's principles of universality, comprehensiveness, and equity. "
Objectives: Describe the main recommendations in the Unified Health System (SUS) clinical practice guideline (CPG) for the care of children and adults with suspected or diagnosed SCD, as well as the guideline update process.
Methods: The CPG update process followed the recommendations of the GRADE system. Researchers, hematologists, representatives of the Brazilian Ministry of Health (MoH), health professional society and patients' representatives participated in this process. After three planning meetings, the scope of the document was outlined, and technologies were prioritized regarding the potential for coverage in SUS.
Results: SCD diagnosis, treatment (pharmacological – prevention of crises and complications, treatment of infections, iron and folic acid supplementation, transfusional iron overload, and analgesia; and non-pharmacological treatment – transfusion, hematopoietic stem cell transplant, nutrition, physiotherapeutic, respiratory and ophthalmological support, oral health, priapism, lower limb ulcers, neurocognitive complications, and surgical procedures) and monitoring were recommended. Highlights included expansion of hydroxyurea indication to nine-months-old children (in addition to 24-years-old children), coverage of a 100 mg tablet of hydroxyurea (in addition to available 500 mg capsules), and indication of epoetin alpha to adults showing a decline in kidney function and worsening of hemoglobin levels. The document is under review by the MoH and will be considered by Conitec (National Committee for Health Technology Incorporation). The next steps comprehend Conitec evaluation and decision, public consultation, and CPG publication.
Conclusions: The guideline will consolidate recommendations for the care of people with suspected and diagnosed SCD, contributing to the quality of primary and secondary health care, and respecting the health system's principles of universality, comprehensiveness, and equity. "