| SCD causes multiorgan damage that accumulates with increasing age. |
| Emerging SCD-related morbidity includes PH, chronic hypoxia, ESRF, bony damage, chronic pain, and neurological impairment. |
| Regular outpatient comprehensive care allows early identification and treatment of these sickle-related morbidities and is likely to improve long-term outcome. |
| Clinicians should exclude nonsickle etiologies (eg, autoimmune hepatitis, lupus, rheumatoid arthritis) that add to disease burden and should be treated. |
| Regular outpatient comprehensive care also allows early detection and treatment of nonsickle aging-related comorbidities that add to the disease burden. |
| Current disease-modifying therapy is limited to HU, transfusion, and l-glutamine, and patients should be offered these therapies before they develop severe complications. However, the provider should also be alert to complications related to the therapy itself (eg, secondary iron overload). |
| Clinicians should be proactive in considering and discussing curative therapies, including HSCT, experimental drug therapy, or gene therapy, early in the disease course, prior to the development of disease complications. |
| Management of the adult with SCD includes holistic care with psychological and social support. |
| SCD causes multiorgan damage that accumulates with increasing age. |
| Emerging SCD-related morbidity includes PH, chronic hypoxia, ESRF, bony damage, chronic pain, and neurological impairment. |
| Regular outpatient comprehensive care allows early identification and treatment of these sickle-related morbidities and is likely to improve long-term outcome. |
| Clinicians should exclude nonsickle etiologies (eg, autoimmune hepatitis, lupus, rheumatoid arthritis) that add to disease burden and should be treated. |
| Regular outpatient comprehensive care also allows early detection and treatment of nonsickle aging-related comorbidities that add to the disease burden. |
| Current disease-modifying therapy is limited to HU, transfusion, and l-glutamine, and patients should be offered these therapies before they develop severe complications. However, the provider should also be alert to complications related to the therapy itself (eg, secondary iron overload). |
| Clinicians should be proactive in considering and discussing curative therapies, including HSCT, experimental drug therapy, or gene therapy, early in the disease course, prior to the development of disease complications. |
| Management of the adult with SCD includes holistic care with psychological and social support. |