Responsiveness of Patient-Reported Outcome Measurement Information System (PROMIS) pain domains and disease-specific patient-reported outcome measures in children and adults with sickle cell disease.

Case 1: A 33-year-old man with hemoglobin SS (homozygous hemoglobin S) disease presents for his regular clinic visit. He had 6 hospital admissions for pain over the past year. He also has avascular necrosis of the right hip. He takes daily hydroxyurea with hematologic changes indicative of compliance. He also takes morphine sustained release twice daily and morphine immediate release every 6 hours as needed for pain. He feels that more optimal pain control at home would help him reduce his number of hospital admissions in the upcoming year and improve his daily functioning at home. His hematologist decides to use Patient-Reported Outcome Measurement Information System (PROMIS) and Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-ME) to follow changes in the patient's pain. Case 2: An 11-year-old girl with hemoglobin SS disease presents with her mother for her regular clinic visit. She had 2 admissions for pain over the past year. Her mother is concerned because she has been participating less in activities she previously enjoyed and missing classes to go to the school nurse because of pain. She is currently taking hydroxyurea and uses ibuprofen for pain. Her doctor prescribes morphine for home use but wants a way to measure if it is effective in improving her pain. Thus, her physician decides to use PROMIS and the Pediatric Quality of Life Inventory SCD (PedsQL SCD) module to determine the effectiveness of her pain control.