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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Improving routine outpatient monitoring for patients with sickle-cell disease on hydroxyurea
Joel Ward1, Natasha Lewis1, Dimitris A Tsitsikas1
1Haemoglobinopathy Service, Homerton University Hospital NHS Foundation Trust, London, UK.
Abstract:
Hydroxyurea is the gold standard treatment for prevention of vaso-occlusive crises in patients with sickle-cell anaemia. It has a narrow therapeutic index and dangerous side effects including cytopenias. There is high variation in dose-response across the population. Therefore, a robust outpatient monitoring programme is crucial to ensure efficacy and safety of treatment. However, there has historically been difficulty engaging the target population in regular laboratory test monitoring programmes. This project aimed to ensure that all patients on hydroxyurea had routine blood tests at least once every 2 months which were reviewed and acted upon within the 3-year project life cycle. A specialist haematology nurse prescriber clinic service was introduced, first informally, and then formally to take blood tests, alter medication dosing, prescribe it and then write a clinic letter. The mean number of tests per patient per year rose from 0.21 at baseline to 9.05 after 2 years of the formal nurse prescriber clinic. This led to an associated increase in dose changes from 0.23 to 1.45 per patient per year. This improved the number of patients on the optimum dose of hydroxyurea. Furthermore, due to increased confidence in the outpatient monitoring, the total number of people being prescribed hydroxyurea increased from 26 to 42. Restriction of prescriptions to only those enrolled in the service has prevented unmonitored patients being at risk of the potential toxicities associated with doses that are too high. The introduction of a formal nurse-led clinic has improved the safety, efficacy and compliance and increased the number of patients on the gold standard preventative treatment for vaso-occlusive crises in sickle-cell anaemia.
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