Iron overload in adults with sickle cell disease who have received intermittent red blood cell transfusions.

OBJECTIVE: To assess the prevalence of iron overload in adults with sickle cell disease (SCD) not on a chronic transfusion protocol. DESIGN: Retrospective chart review. DATA SOURCE: University of South Alabama Comprehensive Sickle Cell Center adult outpatient clinic. RESULTS: There was no significant difference in units transfused across the four genotypes (HbSS, HbSC, HbSß(0)-thalassemia, and HbSß(+)-thalassemia). Only individuals with HbSS (n = 63) met criteria for iron overload with ferritins of ≥1500 ng/mL. Forty-eight had ferritins <1500 ng/mL, eight (13%) had ferritins ≥3000 ng/mL, and seven (11%) had ferritins ≥1500 and <3000 ng/mL. The overall prevalence of iron overload was 9.74% in SCD cohort and 23.8% in the HbSS genotype. CONCLUSIONS: Our data support that patients with HbSS are at a particularly high risk for inadvertent iron overload as compared to HbSC, HbSß(0)-thalassemia, and HbSß(+)-thalassemia. IMPLICATIONS FOR PRACTICE: This study supports the need for healthcare providers to closely monitor the number of red blood cell (RBC) transfusions, RBC units transfused, and serial baseline, steady-state ferritin levels. With closer monitoring, the clinical significance of iron overload in SCD can be established and guide the healthcare provider's management in the prevention of iron overload.

The dangers of iron overload: bring in the iron police.

PURPOSE: To increase awareness of nurse practitioners (NPs) about the dangers of iron overload and to review common hematologic conditions where transfusions are essential, iron mechanism and transport within the body, effects of iron overload on the body, and treatment options available. Finally, the process for development of a protocol for monitoring such patients will be introduced. DATA SOURCES: Review of existing literature, myelodysplastic syndrome guidelines, sickle cell guidelines, thalassemia guidelines. CONCLUSIONS: Transfusions of packed red blood cells save lives. There are many hematologic conditions for which packed red blood cell transfusions are required as a result of bone marrow suppression. However, extended exposure to red blood cell transfusions places the patient at an additional risk of iron overload. Iron overload is a real concern for patients who become transfusion dependent, with increased risks of liver cirrhosis and cardiac arrhythmias. IMPLICATIONS FOR PRACTICE: NPs in all areas of practice can increase awareness of the dangers of transfusional iron overload, and become familiar with the treatment options available. Additionally, NPs can institute a monitoring program based on the protocol suggested here within their own practices to prevent poor outcomes for patients with transfusion-related iron overload.

Evaluation of at-home phlebotomy for iron overload: feasibility and satisfaction of patients and healthcare workers.

OBJECTIVE: This study aimed to evaluate at-home phlebotomy and the satisfaction of iron-overload patients and healthcare workers with the procedure. METHODS: Forty-two patients underwent at-home phlebotomy between 2003 and 2006. The phlebotomy was performed by the patient's nurse, who was trained by the private healthcare firm that also took charge of the disposal of the blood products. Data concerning these phlebotomies were collected via telephone interviews with all 42 patients, as well as 35 nurses and 40 primary-care physicians. The Limousin Regional Health Observatory processed the data collection. RESULTS: Ninety percent (38/42) of the patients, 80% (28/35) of the nurses and 67% (27/40) of the primary-care physicians responded. For 80% of the patients, phlebotomy volume and frequency were as prescribed. Patients chose home phlebotomy for personal reasons, or because of the limited availability of French Blood Establishment facilities (68%), or in response to being offered it by their hospital physician (32%). For 81.6% of the patients, at-home phlebotomy was more satisfactory than phlebotomy in hospital or at the French Blood Establishment and, for 84%, the constraints required were fully acceptable. The nurses considered that these homecare procedures were within their area of responsibility (100%), but felt that the remuneration was insufficient (65%). Ninety-six percent of the primary-care physicians said they were correctly informed, but only 40% felt that they were truly committed to the procedure. CONCLUSION: At-home phlebotomy is feasible, less costly than institutional phlebotomy and improves patient comfort.

Sobrecarga de ferro nas hemoglobinopatias: como o enfermeiro pode atuar? / Iron overload in hemoglobinopathies: how can the nurse act?

As hemoglobinopatias mais freqüentes em nosso meio são as doenças, falciforme e talassemia, cujos portadores geralmente são dependentes de transfusão de sangue e quelação do ferro. A desferroxamine administrada sub-cutâneamente é a terapia padrão ouro, cuja administração deve ser lenta e contínua. A baixa adesão constitui a ameaça mais importante para a ocorrência de complicações, e é o maior desafio para a atuação do enfermeiro. Este trabalho tem como objetivo identificar as principais barreiras à adesão e demonstrar como o enfermeiro pode intervir, por meio de um estudo de revisão da literatura. Neste estudo foi possível observar que a maioria dos fatores que afetam a adesão à terapia quelante é de ordem prática ou psicológica. Neste contexto, considero que a maioria destas barreiras pode ser resolvida ou minimizada pela intervenção do enfermeiro capacitado.