Poor Access to Care Is a Risk Factor for Iron Overload in Adults with Sickle Cell Disease in Southern California

The lack of access to quality comprehensive care is a major problem for adults with sickle cell disease (SCD) and contributes to increased morbidity and mortality. This problem is especially acute in Los Angeles where the average lifespan is 10 years shorter than the national average. Without access to disease modifying therapies, patients utilize Emergency Department services and are repeatedly hospitalized. During inpatient stays many may be inappropriately transfused. The result can be severe iron overload, a significant cause of morbidity and mortality. Inattention to iron chelation in patients who are not cared for by knowledgeable SCD providers perpetuates this issue. In 2016 we cared for a new patient at our clinic in South Los Angeles who previously had a liver transplant due iron overload. This patient received multiple transfusions during her lifetime and was never seen in a comprehensive SCD clinic. This experience prompted us to evaluate our population of clinic patients to assess how lack of access to care might be a risk factor for iron overload.

Methods: We performed a chart review of all patients with SCD who presented to either of two adult sickle cell clinics in Southern California: MLK Jr. Outpatient Center in Los Angeles and Center for Inherited Blood Disorders (CIBD) in Orange. We examined records from 2013-2018 from CIBD and from 2016-2018 from MLK. Patients were considered evaluable if they had a least one visit to clinic with labs that included iron studies and /or MRI. Iron saturations greater than 50% and /or a ferritin greater than 1000 was deemed indicative of iron overload when combined with a history of prior transfusions. Lack of access was defined as the absence of appropriate care for SCD for at least 12 months prior to the initial visit in our clinics. Patients were deemed in good SCD care if they had been transitioned from a pediatric SCD program or another adult SCD program where they received team based comprehensive services.

Results: 74 patients were able to be evaluated. (Table 1). 53 patients were Hgb SS. Of those, 27 (50.9%) were not receiving appropriate SCD care for the prior 12 months when they first presented, 20 (74.1%) of those patients were iron overloaded. There were 9 patients with Hgb S/beta thalassemia 0 or +. Five (55.6%) of those patients were not receiving appropriate SCD care for the prior 12 months; one (35.6%) was iron overloaded. 12 patients had Hgb SC. Although 5 (41.7%) were not receiving appropriate SCD care at the time of presentation, none had evidence of iron overload. Using these data, the odds ratio of having iron overload if a patient was out of appropriate SCD care for the 12 months prior to evaluation was 6.8 (95% confidence interval 2,23.13), indicating that being out of good care is a risk factor for iron overload.

Discussion: Although guidelines exist to prevent unnecessary transfusions in adult patients with SCD; lack of access to knowledgeable providers both in the inpatient and outpatient setting places them at a nearly 7 times higher risk for iron overload, a serious and preventable comorbidity. Our study indicates that many adults with SCD are not appropriately monitored or treated for transfusion related iron overload using guideline-based care, placing avoidable burdens on individual health and adding to healthcare costs. Further education of providers to address this issue is warranted.

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