Article Text

Download PDFPDF

P14 Are we treating patients on hydroxycarbamide for sickle cell appropriately against national British society for haematology guidance
Free
  1. Letisha Brown,
  2. Ghazala Javid
  1. University Hospitals Leicester NHS Trust

Abstract

Aim Sickle cell disease (SCD) is an inherited condition which causes the haemoglobin-S subunit to become mis-shaped, this results in abnormal RBC function and blockages of small blood vessels which commonly leads to vaso-occlusive crisis.1 2 Historically, sickle cell disease had a substantial mortality rate, however, over recent years, life expectancy has increased to 67 years old in 2016 where the local population was 82.2 3 This improvement in life expectancy is due to better diagnosis and medical care, one of which being hydroxycarbamide (HU).

HU has multiple modes of action, with the most clinically relevant being its ability to increase HbF%. HU is currently the only medication licensed in the UK for SCD. The 2011 Baby HUG study published in the Lancet HU is a safe and effective treatment and should be considered for all young children with SCD to reduce number of attacks, as well as long-term organ damage.4 Despite the reduction in morbidly and mortality with use of HU, initiation of HU has to be done with care due risk of myelosuppression which could be fatal, due to this baseline and routine monitoring are a mandatory requirement of treatment.

As of November 2019, University Hospitals guidelines were updated to recommend that all patients who met initiation requirement be started on HU at on, following base line tests, at a dose of 20 mg/kg/day with this up titrated by 5 mg/kg/day every 8–12 weeks following routine monitoring to the maximum tolerated dose, or maximal dose of 35 mg/kg/day.

This audit will review compliance to the British society for haematology (BSH) guidance detailed in the University Hospitals Leicester policy, including compliance rates for HU initiation in eligible patients, monitoring and dose escalation.

Standard 1: 100% of all appropriate patients have a baseline pregnancy test

Standard 2: 100% of patients are started on the correct dose mg/kg.

Standard 3: 100% of patients with FBC after 8–12 weeks.

Standard 4: 100% of patients with dose increase have FBC after 2 weeks.

Standard 5: 100% of patients have dose increased by 5 mg/kg

Method We completed a retrospective analysis of all patients who received hydroxycarbamide on ward at University Hospitals from 01/09/2020 till 01/09/2022 resulting in 36 patients. Raw data including sex, weight, and dose and blood results were documented and anonymised, data were then assessed if the audit criteria were met.

Results The use of hydroxycarbamide is in perfect adherence with BSH guidance. None of the patients in this group were post-pubescent females thus none required a pregnancy test, this criteria was not monitored. 94% of patients started on 20 mg/kg/day dosing. 6% (2 patients) were started 15 mg/kg/day alongside guidance due to clinical co-morbidities. 71% of patients from the total cohort had their doses optimised, with all patients whom had a dose increase done by the appropriate 5 mg/kg/day. All patients had blood tests 2 weeks after a dose change, and 8–12 weeks routinely.

Conclusion The audit shows an excellent adherence to BSH guidance, 100% compliance.

References

  1. Sickle cell disease – Hydroxycarbamide hospital Haematology Guidelines C2/2019 https://secure.library.leicestershospitals.nhs.uk/PAGL/Shared%20Documents/Sickle%20Cell%20Disease%20%E2%80%93%20Hydroxycarbamide%20UHL%20Haematology%20Guideline.pdf (Accessed 1 October 2020).

  2. Qureshi A, Kaya B, Pancham S, et al. Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease, A British Society for Haematology Guideline. British Journal of Haematology 2018;181:460–475 https://doi.org/10.1111/bjh.15235.

  3. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994; 330:1639–44. https://pubmed.ncbi.nlm.nih.gov/7993409/

  4. Wang WC, Ware RE, Miller ST, et al. Hydroxycarbamide in very young children with sickle-cell anaemia: A multicentre, randomised, controlled trial (Baby hug). Lancet 2011;377(9778):1663–1672. https://doi.org/10.1016/s0140-6736(11)60355-3

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.