Optimizing Hydroxyurea use in children with sickle cell disease: low dose regimen is effective.

BACKGROUND AND OBJECTIVES: Hydroxyurea (HU) is the standard treatment for severely affected children with sickle cell disease (SCD). Starting dose is 15-20 mg/kg/day that can be escalated up to 35 mg/kg/day. Ethnic neutropenia is common in this area of the world that requires judicious usage of myelosuppressive drugs. Aim was to assess the efficacy of a lower initial dose of HU and cautious dose escalation regimen in patients with SCD. METHODS: We assessed 161 patients with SCD on HU, at Sultan Qaboos University Hospital (SQUH), Muscat, Oman, retrospectively from 1998 to 2008 and prospectively from 2009 to 2011. Starting dose of HU was 10-12 mg/kg/day, adjusted based on response or side effects. Patients were divided into two groups according to the dose of HU (10-15.9 mg/kg/day and 16-26 mg/kg/day). RESULTS: Nineteen patients were excluded for various reasons. Forty-four children were in the low-dose group and 98 were in the high-dose group. There was significant reduction in the annual number of admissions due to vaso-occlusive crisis in both groups (P < 0.001). However, the difference between the two groups was statistically insignificant (P > 0.05). In addition, there was an observed clinical improvement regarding the acute chest syndrome (ACS). Both groups had comparable significant improvements in their laboratory markers [e.g., hemoglobin (Hb), Mean Corpuscular Volume (MCV), and absolute neutrophil count (ANC)]. All 142 patients tolerated the treatment well. Reversible toxicities occurred in both low- and high-dose groups. CONCLUSION: In SCD patients, low-dose regimen of HU is a feasible option that ensured safety and yet did not affect efficacy.

Metered-dose inhaler technique among healthcare providers practising in Oman.

OBJECTIVE: To evaluate the correctness of metered-dose inhaler (MDI) technique in a sample of healthcare providers practising in Oman, considering that poor inhaler technique is a common problem both in asthma patients and healthcare providers, which contributes to poor asthma control. METHOD: A total of 150 healthcare providers (107 physicians, 33 nurses and 10 pharmacists) who were participants in symposia on asthma management conducted in five regions of Oman, volunteered for the study. After the participants answered a questionnaire aimed at identifying their involvement in MDI prescribing and counselling, a trained observer assessed their MDI technique using a checklist of nine steps. RESULTS: Of the 150 participants, 148 (99%) were involved in teaching inhaler techniques to patients, and 103 of 107 physicians (96%) had prescribed inhaled medications. However only 22 participants (15%) performed all steps correctly. Physicians performed significantly better than non-physicians (20% vs. 2%, p <0.05) Among the physicians, internists performed better (26%) than general practitioners (5%) and accident and emergency doctors (9%). CONCLUSION: The majority of health-care providers responsible for instructing patients on the correct MDI technique were unable to perform this technique correctly indicating the need for regular formal training programmes on inhaler techniques.