Living in limbo--patients with cystic fibrosis waiting for transplant

Lung transplant for patients with end-stage cystic fibrosis (CF) in the UK is recognized as the only successful treatment for CF patients with advanced lung disease. This study uses an exploratory approach to examine how patients with CF and their carers cope with the rigours of chronic illness and life on a transplant waiting list. Eight patients with CF, four awaiting transplant and four who had been transplanted within the previous 3 years, along with five of their carers, were asked to recount their experiences using a semi-structured interview technique. Four themes emerged from the interview data; displacement, disorder, life in limbo and readjustment to wellness. Support appears to be particularly important to patients and families after false alarms occur, and upon return home after transplant. The small sample size precludes generalization of the results to all patients with CF but gives an in-depth insight into the lived experience of waiting for transplant.     

Family physician: problems and realities

The experience gained in the Soviet Union and abroad with family physician service is reviewed. The scope of his duties is outlined which are to be adjusted for specific conditions of rural and city health care. The family physician is to be neither a particular specialist nor an expert in all fields of medicine, rather a broad-minded and skilled internist informed about social and psychological matters and able to attend both adults and adolescents. The chief goal of his practice should be prophylaxis medicine, regular check-ups. Extensive experimental research is needed to adopt the system of family physician in this country as it necessitates revision of conventional principles of industrial, school, outpatient services, training of medical staff, etc.     

Living with an implantable cardioverter defibrillator: impact on the patient, family, and society

By improving survival, the advent of implantable cardioverter defibrillators (ICDs) creates a new patient population that is distinct from the general cardiac population. In these patients, focus is shifting from evaluating the physical response of the treatment to evaluating the emotional, spiritual, and psychological responses. Until now, the effect of this treatment on families and society at large has been underestimated. Cardiovascular nurses are continually being challenged in meeting the unique needs of ICD patients, assisting patients and their families to develop coping strategies, and empowering these people to live full and active lives in society.     

Intensive care management of the patient with cystic fibrosis

Cystic fibrosis was previously thought to be a disease of childhood. With a better understanding of this condition along with improvements in therapy, patients with cystic fibrosis are now living well into adulthood. The aim of this article is to familiarize the intensive care unit physician with cystic fibrosis care, to discuss complications associated with cystic fibrosis specifically related to the intensive care unit, and to detail the current recommendations for the clinical management of the patient with cystic fibrosis. With advancing disease, the most severely affected organs are the lungs. Obstruction, infection, and inflammation contribute to the decline of pulmonary function, ultimately leading to death. Some patients may be eligible for lung transplantation, but choosing wisely will affect posttransplant survival. Because other organs are affected by the genetic defect and associated treatments, serious complications related to the liver, pancreas, intestines, and kidneys must be considered by the intensivist faced with a patient with cystic fibrosis. As practitioners, the fact that not all patients will survive and help our patients and families gracefully through the end-of-life process should be accepted.     

Paradoxical embolization in an adult patient with cystic fibrosis

To our knowledge, we describe the first reported case of paradoxical embolization via a patent foramen ovale (PFO) in an adult with moderately severe cystic fibrosis (CF) and advanced lung disease. Fluctuating neurologic symptoms and signs suggestive of cerebrovascular disease in an adult patient with advanced CF may be due to paradoxical embolization via a PFO. The possibility of a PFO should be considered before placement of a totally implantable venous access device to avert unnecessary risk of stroke in CF patients. Further study is needed to determine whether the use of a totally implantable venous access device increases the risk of paradoxical embolization in adult CF patients with a PFO.     

Care of the adult patient with cystic fibrosis.

Recently published consensus statements and conference proceedings from the Cystic Fibrosis Foundation provide new direction with regard to the etiology, diagnosis, and treatment of the adult patient with cystic fibrosis (CF). Survival has improved through early diagnosis and improved treatment, and aggressive management of complications. The current state of knowledge, the most recent evidence-based treatment options, and essentials of nursing care for the adult patient with CF are outlined.     

Successful meropenem desensitization in a patient with cystic fibrosis

OBJECTIVE: To report a case of successful meropenem desensitization in a patient with cystic fibrosis (CF) with documented hypersensitivity to multiple antibiotics including carbapenems. CASE SUMMARY: A 20-year-old white man with CF was admitted to the hospital for treatment of an acute pulmonary exacerbation caused by multidrug-resistant Burkholderia cepacia complex and methicillin-resistant Staphylococcus aureus (MRSA). Past treatments of his CF exacerbations were complicated by urticarial eruptions following administration of beta-lactams, including meropenem, and ototoxicity from aminoglycosides. Skin testing revealed hypersensitivity reactions to beta-lactam antibiotics including penicillin, piperacillin/tazobactam, ceftazidime, and imipenem. A literature review (MEDLINE, January 3, 2002) and communication with the manufacturer of meropenem revealed no specific information on desensitizing patients to this agent. Because of meropenem's activity against B. cepacia complex alone and in combination with other antimicrobials, a desensitization protocol was adapted and applied to meropenem in an effort to provide the most beneficial treatment available. A 12-dose escalation protocol was successfully employed without incident. DISCUSSION: Antimicrobial therapy is limited in CF patients by susceptibility profiles of common infecting organisms (e.g., Pseudomonas spp., B. cepacia complex, MRSA). Unfortunately, host responses may further reduce the utility of many effective antibiotic classes due to hypersensitivity and/or adverse reactions. Desensitization is a useful alternative that allows the administration of beneficial medications to patients with documented allergy histories. CONCLUSIONS: Meropenem is an important treatment option in the CF population, particularly due to its activity against B. cepacia complex. Successful desensitization using a dose-escalation protocol in patients with a documented carbapenem allergy will allow the most beneficial therapy to continue.     

Potential for alterations in family process: when a family has a child with cystic fibrosis

Cystic fibrosis (CF) is a disease that has profound consequences on a family unit. As a genetically transmitted, chronic illness, it influences every aspect of family life, including time constraints, finances, and relationships among family members. The purpose of this article is to demonstrate the cumulative effect of the nursing diagnoses related to the pathophysiology of CF on the nursing diagnosis, Potential for Alterations in Family Process, when a family has a child with cystic fibrosis. This nursing diagnosis was first recognized by the North American Nursing Diagnosis Association (NANDA) in 1982. It is vital that nurses are able to differentiate between a family having difficulty coping with a child with CF in the home, and one that is coping well on its own. The children of families that are not coping well tend to be hospitalized more than those of families that are coping well. The families use the hospitalizations as rest periods for themselves. By recognizing the cues identified by NANDA of a family having problems, early intervention can be initiated before the family is in a crisis. The family must live with CF on a daily basis for the remainder of the child's life; therefore it must be able to function at its optimum level. It is nursing's responsibility to be able to recognize and assist the family in need. A knowledge of the nursing diagnosis, Alterations in Family Process, will assist nurses in fulfilling this obligation to the family.     

Potential for alterations in family process: when a family has a child with cystic fibrosis

Cystic Fibrosis (CF) is a disease that has profound consequences on a family unit. As a genetically transmitted, chronic illness, it influences every aspect of family life, including time constraints, finances, and relationships among family members. The purpose of this article is to demonstrate the cumulative effect of the nursing diagnoses related to the pathophysiology of CF on the nursing diagnosis, Potential for Alterations in Family Process, when a family has a child with cystic fibrosis. This nursing diagnosis was first recognized by the North American Nursing Diagnosis Association (NANDA) in 1982. It is vital that nurses be able to differentiate between a family having difficulty coping with a child with CF in the home, and one that is coping well on its own. The children of families that are not coping well tend to be hospitalized more than those of families that are coping well. The families use the hospitalizations as rest periods for themselves. By recognizing the cues identified by NANDA of a family having problems, early intervention can be initiated before the family is in a crisis. The family must live with CF on a daily basis for the remainder of the child's life; therefore it must be able to function at its optimum level. It is nursing's responsibility to be able to recognize and assist the family in need. A knowledge of the nursing diagnosis, Alterations in Family Process, will assist nurses in fulfilling this obligation to the family.     

Living with terminal illness: patient and family experiences of hospital end-of-life care

AIM: The study explored terminally ill patients' and their families' experiences of hospital end-of-life care. DESIGN: A qualitative approach--interpretive phenomenology--was applied prospectively. Sample and setting. Ten patients and their closest family members were recruited in one Swiss university hospital. METHODS: Data collection included repeated observations, conversations with patients, and interviews with family members. FINDINGS: People led unique lives; when a terminal illness intruded and during hospital stays, they continued to live their particular life. The hospital was experienced differently, as prison, heaven, or necessary sojourn. A caring stance of the professionals was crucial to patients' and families' sense of being well cared for. When care providers lacked skills or did not recognize patients and family members as persons, they suffered. In contrast, adequate care interventions improved their quality of life. CONCLUSIONS: The experience of being in the hospital cannot be separated from patients' and families' life stories.     

Acquired segmental megacolon in an adult patient with cystic fibrosis

Cystic fibrosis (CF) is characterized by symptoms related to pulmonary dysfunction and pancreatic insufficiency. Constipation, though a frequent complaint of patients with CF, receives less attention. We report a case of acquired segmental megacolon and constipation necessitating surgical colonic resection in an adult patient with CF. The differential diagnosis and possible causes of megacolon in this setting are discussed.