The documentation template has enhanced the quality and safety of practice at the authors' institution and provides a framework for other nursing units when initiating immunotherapy care. .
This review deals with the treatment of inherited classical galactosemia by a lactose-free diet. Although, with dietary treatment, there is a remarkable improvement in the acute phase of the disease, the long-term outcome has been disappointing for most patients, especially regarding the central nervous system and ovarian dysfunction in females. There is a need for new approaches to treatment, in combination with diet therapy, that could improve the outcome of patients with galactosemia.
In response to this trend, a team was developed to plan and implement a Quality Improvement (QI) project addressing the process for administration and drug monitoring for patients receiving these drugs by implementing the Institute for Healthcare Improvement (IHI) model for QI. The IHI model utilizes the Plan-Do-Study-Act process in which a series of small tests of change are implanted to achieve desired results. The team established the standard nursing practice as administration of tacrolimus or cyclosporine through the white lumen of the tunneled CVC and lab draws for drug monitoring through the red lumen of the tunneled CVC. BMT nurses were informed of the new standard practice during twice daily safety huddles. Nurses were instructed to remember the standard as "red for blood" meaning to draw from the red lumen. Since implantation of this standard practice there have been zero reported line contaminations.
Iron (Fe) status and Fe supplementation were assessed in 20 children with phenylketonuria (PKU) through dietary intake and through measurements of ferritin, hematocrit, hemoglobin, mean cellular hemoglobin, mean cellular volume, serum Fe, total iron binding capacity, unbounded iron binding capacity, transferrin saturation and transferrin. Findings were compared to reference values and to data from age-matched controls. The prescribed phenylalanine-restricted diet supplied all the recommended nutrients. Dietary Fe was present in the diets, but its bioavailability is questionable as several laboratory results were not within accepted reference values. A ferrous sulphate supplement (5 mg elemental Fe/kg daily) was given for 120 days to a group of PKU children with lower Fe parameters, thus changing some of the parameters studied. Serum ferritin (p < 0.1), transferrin saturation and serum Fe (p < 0.05) increased after the treatment. The need for improved diagnosis of Fe status and determination of whether PKU children can benefit from therapeutic Fe is discussed.
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