Patient Centered Comprehensive Medication Adherence Management System in Patients With Sickle Cell Disease

Sickle cell disease (SCD) is an inherited chronic multi-organ system disorder that affects
approximately 100,000 individuals in the United States, mostly belonging to minority,
under-served populations. SCD is associated with substantial morbidity, premature mortality,
individual suffering, health care costs and loss of productivity. Hydroxyurea (HU) the only
disease modifying therapy for SCD is efficacious in reducing complications such as pain
crisis and acute chest syndrome and improving survival. It is however, vastly underutilized
and poorly adhered to because of barriers at the health care system, provider, treatment,
socioeconomic, and patient levels. The investigator's overarching hypothesis is that barriers
to acceptance and adherence to HU are multi-factorial and that a structured set of
interventions can lead to improved adherence to medication and patient centered outcomes.

Inclusion Criteria:

- Be >2 years of age up to 65 years of age, inclusive

- Have a diagnosis of SCD, with either βS/βS, βS/βC, βS/βD , βS/β0 , βS/βO-Arab, or
βS/β+ genotype

- Prescribed Hydroxyurea for at least the 6 months prior to study entry

- Have daily access to a smart phone, tablet, personal computer or other device capable
of producing and transmitting videos over the internet

- Be willing and able to record and transmit videos

Exclusion Criteria:

- Patient or caregiver refuses Hydroxyurea.

- Diagnosis of significant psychiatric disorder of the subject that could seriously
impede the ability to participate in the study.

- An assessment by the investigator that the subject will not comply with the study
procedures outlined in the study protocol.

- Patient is on liquid form of Hydroxyurea since MPR - calculations are difficult.

- Patients receiving automatic home delivery of medications since medication possession
ratio is reflective of the patient initiation the refill when they have exhausted the
home supply of HU.