Advances in treating and detecting chronic and rare diseases are improving the life expectancies of individuals with sickle cell disease (SCD) in many ways. For up to 90,000 individuals that are born with the sickle cell disease in the US, infant screening, improved therapies, and better management of the rare blood disorder have all improved the lives of such people in unprecedented ways. Presently, more than 95% of the people born with this condition grow into adulthood on account of the increase in the use of preventive medicine and other therapy related advances.
With the improved management of the disease, questions now arise on the experience and impact of the disease across the lifespan of an individual. Their well-being and optimal functioning are affected by the condition on account of the pain, multi-organ failure, and the neurocognitive deficits encountered as they age. The economic and social challenges encountered have also not been properly investigated as well as the barriers such individuals experience in accessing quality health care. There was, therefore, the need to have a better understanding of the long-term requirements of adults with this condition for health care providers to be in a position to offer skilled adult-oriented heath care.
The National Heart, Lung and Blood Institute (NHLBI) organized workshops and conferences in 2002 for stakeholders to suggest new ways of improving the treatment given to adults with SCD (Treadwell, 2014). The stakeholders suggested the need for a valid, reliable, and systematic method for recording the adult patient-reported outcomes. The NHLBI contracted the Children’s Hospital & Research Center Oakland to come up with a sickle cell disease quality of life quantification system that would address the gaps in understanding the long-term needs and experiences of patients. This collaboration led to the development of the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me), it was designed to address the information gap related to the management of this disease in this population. ASCQ-Me pronounced as “Ask Me” is a Patient-Reported Outcome system that targets adults with SCD and help document their perspective.