Transfusion therapy remains efficacious for SCD adults who have suffered

strokes or severe ACS, but is limited because of a lack of qualified providers comfortable with RBC exchange therapy. Moreover, the use of transfusion therapy in adults is complicated by iron overload and allo-immunization. Thus, many patients successfully treated with transfusion therapy in childhood are unable to continue that therapy as adults. On the other hand, acute care and inpatient providers may over-utilise transfusion for baseline Selleck AZD6244 anaemia or vaso-occlusive pain in adults because of a lack of SCD management experience [61]. Patients with SCD have a physiological adaptation to their anaemia; thus, it is crucial to know a patient’s baseline haemoglobin and transfuse only for life- or organ-threatening complications. Iron overload is a frequent complication in adult patients with SCD and requires chelation therapy and monitoring. Up to 10% of adult patients with SCD are noted to have complications of iron toxicity at the time of death [54]. HSCT is also curative in adults with SCD but is more difficult because

of the increased risk of treatment-related complications. Newer studies have demonstrated effective transplantation with reduced-intensity Ganetespib in vitro conditioning, which may increase the options for adult patients [58] and [59]. Additional complications for HSCT in adults include the lack of available donors and

lack of available adult transplantation centres with expertise in SCD. Regardless of treatment, pain is the most-common presenting symptom of SCD in adults. VOEs are often under-treated, Carnitine palmitoyltransferase II which may cause excessive hospital utilisation, including ED visits and inpatient hospitalisations, as well as lost work productivity [62]. Concerns regarding addiction, dependence, and tolerance to pain medication are often unfounded, but add an important layer of complexity to patient care. Pain contracts between patients and providers, as well as drug-monitoring, can be beneficial, but require outpatient follow-up. The manifestations of VOE in conjunction with a lack of preventative care and insufficient insurance coverage in this population can make it difficult to provide effective management in adults [63]. Primary and secondary prevention are also essential and are best addressed in a comprehensive setting. Some key points are presented in Table 2. Although many more children with SCD are living into adulthood, there has not been a corresponding increase in medical haematologists trained to treat older patients. Accessing adequate health and medical services for the young adult with SCD can be a challenge, and usually involves a change in the physician and location of care.