Academic literature on the topic 'Cystic fibrosis, child, nursing care, case study'

Background and objective: Integration of patient simulations into the nursing student curricula have been shown to be effective and innovative teaching enhancements leading to enhanced knowledge, clinical reasoning and judgment for students, whilst promoting optimal patient care. This pilot study aimed to explore how the use of a simulation, with a genetic component of a Cystic Fibrosis (CF) case scenario, improved the self-perceived knowledge comprehension of pre-licensure baccalaureate nursing students of a large diverse urban School of Nursing.Methods: Three assessment surveys were utilized to glean data: nine multiple choice questions explored factual content of CF pre/post simulation; five question survey explored self-perception of knowledge and one open-ended simplified critical incident report provided qualitative data.Results: Twenty-four pre-licensure third year nursing students participated (three groups of eight students). All participants agreed that their understanding of the genetic component of CF improved post simulation. Four major themes emerged from the qualitative data: genomics and nursing; patient education; teamwork exercise and patient-nurse relationship. Conclusions: Integrating a genetically-based condition into a simulation, whereby students are expected to research the condition, engage in patient education, facilitate effective and appropriate nursing care enriches their critical thinking, confidence, skills and knowledge acquisition.

Through a case study of a child with cystic fibrosis, the interactions among various domains of health have been discussed—namely, biomedical, physical, psychological/behavioural, and social. In pediatrics, development is another key domain relevant to the management of a chronic disease. An individualised management plan for this case has been outlined, and consideration of this framework may be worthwhile when managing other paediatric patients with chronic disease. Patient empowerment and parental education, as well as good co-ordination of health service delivery, are imperative to holistic patient care.

Abstract Context: Cystic fibrosis (CF) is a genetic disease that affects multiple organ systems, and symptoms include chronic cough, gastrointestinal (GI) malabsorption, exercise intolerance, and chronic pain. Examples of standard treatments are nebulizers, supplementary enzymes, chest percussive therapy, respiratory therapy, and lifestyle modifications. Objective: The purpose of the current review was to determine whether manual therapies, such as osteopathic manipulative treatment (OMT), in conjunction with standard treatments, provide symptom relief for patients with CF. Methods: PubMed was searched to identify studies investigating the role of manual medicine in the care of CF patients. Search terms included chiropractic, physical therapy, physiotherapy, osteopathic, manipulation, massage, and manual medicine. All terms were searched in combination with cystic fibrosis. Studies investigating only exercise or chest percussive therapy as adjunctive treatments were excluded. Results: Eight studies were found that investigated manual therapies alone or in conjunction with exercise for treatment of CF symptoms. All studies reported improvement after manual therapy in 1 or more symptoms, such as posture, GI symptoms, peak airflow, anxiety, back or chest pain, or breathing. Study types included a case study, prospective observational studies, and randomized controlled trials. Most studies were small and statistically underpowered. In an inpatient/outpatient observational study of adults with CF, patients reported a significant reduction in pain after a single physiotherapy treatment, and inpatients also reported significantly improved breathing after treatment. In a randomized controlled trial involving adult CF outpatients, there was a significant improvement in chest and back pain scores between the OMT and control groups. Conclusions: Results of the current review suggested patients with CF can experience symptom relief after OMT and similar manual therapies. Patients subjectively reported improvement with manual therapies, and studies found statistically significant decreases in pain after a single treatment. However, larger studies with sufficient statistical power are needed to further define the role of manual therapies as adjunctive treatment for symptom relief in CF patients.

Cystic fibrosis (CF) is a genetic, chronic disease that results in thickened secretions in the respiratory, gastrointestinal, and reproductive tracts. Over 95% of males with CF have congenital bilateral absence of the vas deferens causing infertility. This is a case study of a 29-year-old male who underwent a lung transplant after 8 months of oxygen dependency secondary to poor lung function. Approximately 1 year posttransplant, he and his wife decided that they wanted to start a family and consulted a fertility specialist who advised them to utilize donor sperm due to the teratogenic effects of posttransplant medications. Taken by surprise with this news, they expressed regret about the missed opportunity for pretransplant sperm aspiration and cryopreservation to conceive a biological child. He reported, “If we would have known, we would have made sure I [banked] my own [sperm].” This case study highlights a critical gap in CF comprehensive clinical care.

ABSTRACT Objective: Dornase alfa (rhDNase) reduces the viscosity of purulent sputum in the lungs. The use in patients with cystic fibrosis (CF) is proven. However, the evidence of its applicability to other conditions is limited. This study aims to present the authors’ experience with the use of rhDNase in non-CF patients admitted to the Pediatric Intensive Care Unit (PICU). At the study center, rhDNase was used during flexible bronchoscopies in 24 cases, of which 20 (83%) had atelectasis and seven (29%) were admitted to PICU. Four patients (57%) were on invasive mechanical ventilation (MV). Case description: Two cases of daily rhDNase administration at PICU are presented: patient A was an 8-year-old boy admitted with septic shock and acute respiratory distress syndrome (ARDS). The patient required mechanical ventilation with aggressive settings and experienced several clinical complications. On D50, he started rhDNase treatment with an improvement in FiO2, PaCO2 and PaO2/FiO2 ratio according to radiologic findings. He was extubated on D23 of treatment. Patient B was a 17-month-old girl admitted with a convulsive status epilepticus who experienced respiratory complications (infectious and barotrauma) with ARDS, requiring aggressive ventilation. She initiated rhDNase treatment on D60. During the treatment an improvement in FiO2, PaO2/FiO2 ratio and a tendency of PaCO2 decrease were found. She had radiological improvement. No complications were described. Comments: RhDNase may be a helpful and safe tool to use in PICU prolonged intubated patients with ventilator-induced lung injury. Further studies are needed to assess and propose valid indications.

Vitamin K deficiency may cause unexpected bleeding (0.25% to 1.7% incidence) during the first week of life in previously healthy-appearing neonates (classic hemorrhagic disease of the newborn [HDN]). The efficacy of neonatal vitamin K prophylaxis (either oral or parenteral) in the prevention of classic HDN is firmly established. It has been the standard of care since the recommendation by the Committee on Nutrition was adopted as policy by the American Academy of Pediatrics in 19611 Late HDN, a syndrome defined as unexpected bleeding due to severe vitamin K deficiency in infants aged 2 to 12 weeks, occurs primarily in exclusively breast-fed infants who have received no or inadequate neonatal vitamin K prophylaxis. In addition, infants who have intestinal malabsorption defects (cholestatic jaundice, cystic fibrosis, etc) may also present with late HDN. The rate of late HDN (often manifested by sudden central nervous system hemorrhage) ranges from 4.4 to 7.2 per 100 000 births based on reports from Europe and Asia. When a single dose of oral vitamin K has been used as neonatal prophylaxis, the rate has decreased to 1.4 to 6.4 per 100 000 births. Parenteral neonatal vitamin K prophylaxis prevents the development of late HDN, with the rare exception of infants with severe malabsorption syndromes. Oral regimens that have a similar efficacy as parenteral vitamin K in prevention of late HDN include the repeated administration of oral vitamin K1 (Germany) or K2 (Japan) at birth, 1 week, and 2 to 4 weeks. In 1990 Golding et al2 reported a study of a 1970 birth cohort in Britain in which they noted an unexpected association between childhood cancer and pethidine given in labor and the neonatal administration of vitamin K. Subsequently, Golding and others3 conducted a case-control study designed to examine the risk of cancer associated with intramuscular vitamin K administration among infants born in two hospitals in Avon between 1965 and 1987 and diagnosed with cancer between 1971 and 1989.