About 100,000 people in the United States have sickle cell disease. Most are of African descent. The disease causes red blood cells to harden and form a C-shape (like a sickle). When hardened, the cells can get caught in blood vessels and cause serious complications for patients. These complications can include severe pain, respiratory conditions, organ failure, and even stroke.
There is no widely used cure for sickle cell disease. However, the Red Cross supports one of the most critical sickle cell treatments of all – blood transfusions. For many patients, a close blood type match is essential and is found in donors of the same race or similar ethnicity.
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More than one-third of adults with sickle cell disease (SCD) report using cannabis-based products. Many states list SCD or pain as qualifying conditions for medical marijuana, but there are few data to guide practitioners whether or whom should be certified. We postulated that certifying SCD patients may lead to a reduction in opioid use and/or health care utilization. Furthermore, we sought to identify clinical characteristics of patients who would request this intervention. Retrospective data obtained over the study period included rates of health care and opioid utilization for 6 months before certification and after certification. Patients who were certified but failed to obtain medical marijuana were compared with those who obtained it. Patients who were certified were invited to participate in a survey regarding their reasons for and thoughts on certification.
Patients who were certified for medical marijuana were compared with 25 random patients who did not request certification. Fifty adults with SCD were certified for medical marijuana and 29 obtained it. Patients who obtained medical marijuana experienced a decrease in admission rates compared with those who did not and increased use of edible cannabis products. Neither group had changes in opioid use. Patients who were certified for medical marijuana had higher rates of baseline opioid use and illicit cannabis use compared with those who did not request certification. Most patients with SCD who requested medical marijuana were already using cannabis illicitly. Obtaining medical marijuana decreased inpatient hospitalizations.
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Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. It is a medically and socially complex, multisystem illness that affects individuals throughout the lifespan. Given improvements in care, most children with SCD survive into adulthood. However, access to adult sickle cell care is poor in many parts of the United States, resulting in increased acute care utilization, disjointed care delivery, and early mortality for patients. A dearth of nonmalignant hematology providers, the lack of a national SCD registry, and the absence of a centralized infrastructure to facilitate comparative quality assessment compounds these issues. As part of a workshop designed to train health care professionals in the skills necessary to establish clinical centers focused on the management of adults living with SCD, we defined an SCD center, elucidated required elements of a comprehensive adult SCD center, and discussed different models of care.
There are also important economic impacts of these centers at an institutional and health system level. As more clinicians are trained in providing adult-focused SCD care, center designation will enhance the ability to undertake quality improvement and compare outcomes between SCD centers. Activities will include an assessment of the clinical effectiveness of expanded access to care, the implementation of SCD guidelines, and the efficacy of newly approved targeted medications. Details of this effort are provided.
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Sickle cell disease (SCD) is an inherited blood disorder that causes affected individuals to produce an abnormal hemoglobin that results in frequent pain events, organ damage, and early mortality. Disease- specific treatments include hydroxyurea, L-glutamine, voxelator, crizanlizumab, and blood transfusions. Hematopoietic stem cell transplant is curative but limited due to a requirement for a matched-related donor in most cases. Adolescence can be a difficult time for females affected by SCD. Pain events may increase in frequency, due to decline in protective fetal hemoglobin levels, medication noncompliance, hormone effects, or progression of the chronic illness. Some patients may develop chronic, daily pain in adolescence, from repeated pain crises, or opioid-induced hyperalgesia.
Transitioning from pediatric to adult providers may be a source of anxiety, and developing independence from parents is a challenge. Gynecological issues include a need to avoid estrogen containing birth control products due to the hypercoagulable state of SCD, difficulties in diagnosing dysfunctional uterine bleeding, and the high-risk nature of pregnancy in an individual with SCD. Individuals with SCD have high rates of psychological complications, including depression, anxiety, and body image dysfunction.
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