[Translated article] Good humanisation practices in the healthcare of patients with rare diseases in Pharmacy Services.

OBJECTIVE: To analyse the presence of Good Humanisation Practices in the care of patients with rare diseases in Hospital Pharmacy Services and to identify the strengths and prevalent areas for improvement in the humanisation of healthcare. METHODS: An online questionnaire structured in 2 parts was developed using Google Form®. The first one was designed to collect identifying data and the second one included questions related to compliance with the 61 standards of the Manual of Good Humanisation Practices in the healthcare of patients with rare diseases in Hospital Pharmacy Services. Access to the questionnaire was sent by email to the Heads of the Hospital Pharmacy Service of 18 hospitals. The study period was from October 2021 to October 2022. The analysed variables were the number of criteria that were considered met, total compliance (percentage of criteria met), by strategic line and by type or level of standard, globally and grouped by regions of Spain. RESULTS: 18 Hospital Pharmacy Services were included. The overall mean of standards met was 31.1 (95% CI: 24.8-37.6) and mean total compliance was 52.1% (95% CI: 44.4%-59.7%). The mean compliance by strategic line was: Line 1, Humanisation culture: 46.5% (95% CI: 35.3%-57.7%), Line 2, Patient empowerment: 47.4% (95% CI: 37.1%-57.8%), Line 3, Professional care: 49.7% (95% CI: 39.8%-59.1%), Line 4, Physical spaces and comfort: 55.6% (95% CI: 46.3%-64.8%), and Line 5, Organisation of healthcare: 63.8% (95% CI: 55.8%-71.9%). CONCLUSION: The average compliance with the standards is between 40% and 60%, which indicates that humanisation is present in the Hospital Pharmacy Services, but there is a wide margin for improvement. The main strength in the humanisation of Hospital Pharmacy Services is a patient-centred care organisation, and the area with the greatest room for improvement is the culture of humanisation.

The association of salivary caffeine levels with serum concentrations in premature infants with apnea of prematurity.

The purpose of this paper is to verify whether the concentrations of caffeine in saliva are comparable to serum concentrations in preterm infants who are treated with caffeine for apnea of prematurity. This is a prospective observational study. Eligible participants were newborn infants < 37 weeks of gestational age treated with oral or intravenous caffeine for apnea of prematurity. Two paired samples of saliva and blood were collected per patient. Tube solid-phase microextraction coupled online to capillary liquid chromatography with diode array detection was used for analysis. A total of 47 infants with a median gestational age of 28 [26-30] weeks and a mean of 1.11 ± 0.4 kg of birth weight. Median postmenstrual age, when samples were collected, was 31 [29-33] weeks. Serum caffeine median levels of 19.30 µg/mL [1.9-53.90] and salivary caffeine median levels of 16.36 µg/mL [2.20-56.90] were obtained. There was a strong positive Pearson's correlation between the two variables r = 0.83 (p < 0.001). CONCLUSION: The measurement of salivary caffeine concentrations after intravenous or oral administration offers an alternative to serum caffeine monitoring in apnea of prematurity. Measurement of salivary concentration minimizes blood draws, improves blood conservation, and subsequently minimizes painful procedures in premature infants. WHAT IS KNOWN: • Salivary sampling may be useful when is applied to extremely low birth weight infant, in whom blood sampling must be severely restricted. WHAT IS NEW: • The measurement of caffeine salivary concentrations after intravenous or oral administration offers an alternative to serum caffeine monitoring in apnoea of prematurity. • Salivary sampling may be a valid non-invasive alternative that could be used to individualize and optimize caffeine dose.

Use and handling safety of Mini-Spike 2® chemo and puresite for safe chemotherapy compounding in a hospital pharmacy.

We wanted to evaluate the impact of Mini-Spike 2® Chemo + Puresite (MSCP) use on contamination surface levels, professionals' satisfaction and compounding time at pharmacy compared with Phaseal™. Presence of cyclophosphamide (CYP) and 5-fluorouracil (5FU) was evaluated at three sampling times: baseline; after a decontamination procedure and six months after MSCP use for CYP and 5FU compounding. Testing was carried out using an independent laboratory and wipe testing kit. To test compounding time, four different nurses followed the same compounding protocol with each device. We also developed a questionnaire to obtain feedback from the nurses. We did not find statistically significant differences in the median contamination surface levels between basal and final sampling time, CYP (0.140; 95% CI -1.135, 1.601), 5FU (-0.506; 95% CI -1.756, 0.287). We observed a difference of 10 s on compounding times between the two devices tested (p < 0.001) favoring MSCP. Finally, eight nurses answered the survey, with the best valued aspect as the aspiration/injection flow and resistance, and the worst value the comfort using Puresite and valve connection. MSCP maintains low surface contamination levels in our setting assuring compounding time standards. Satisfaction survey let us know which were the major advantages and disadvantages of the device.

Lithium citrate as treatment of Canavan disease.

Canavan disease is a rare autosomal recessive leukodystrophy characterized by abnormal accumulation of N-acetyl aspartate (NAA) in brain white matter. Currently, there is no cure for this disease, and management of patients consists mainly of treating symptoms. We describe a 3-month-old girl who was hospitalized for poor head control and decreased muscle tone. A battery of laboratory and genetic (homozygous mutation p.C218X) analysis revealed the presence of Canavan disease. Lithium citrate was initiated at a dosage of 45 mg/kg per day after diagnosis. Periodic controls of thyroid and liver function, and lithium levels in blood showed that this drug was sure and well tolerated. After 1 year of treatment, NAA levels decreased by approximately 20% in the brain region, urinary NAA levels showed a reduction of 80%, and patient improved alertness and visual tracking but continued with no heat support, axial hypotonia, and spastic diplegia. In our patient, the results obtained after drug administration are important with respect to the decrease in NAA and more discreet in clinical improvement. However, given the absence of adverse effects and limited treatment options, lithium citrate may be a good alternative to stop the progression of the disease and improve the quality of life of patients.