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Cystic fibrosis ppt Nursing

3. Cystic Fibrosis - Overview of Disease • Cystic fibrosis (CF) - inherited autosomal recessive disorder in children • Most common cause of chronic lung disease in children - 30,000 children & adults in US • Incidence - Annually 1,000 children diagnosed - Most common lethal, hereditary disorder among Caucasians - 1 in 1,000 live. INTRODUCTION• Cystic fibrosis also known as CF or mucoviscidosis is a common recessive genetic disease which affects the entire body,causing progressive disability and often early death.•. Approximately 30,000 Americans have CF, making it one of the most common life-shortening inherited diseases in United States. 3

Cystic fibrosis 1. PRESENTED BY: A.PRIYADHARSHINI M.Sc(N), LECTURER,JAI INSTITUTE OF NURSING AND RESEARCH, GWALIOR. 2. It is an autosomal recessive geneticdisorder that affects most criticallythe lungs, and also the pancreas, liver,and intestine Cystic fibrosis presentation 1. CysticfibrosisParul Shrestha 2. Background• Cystic fibrosis, also known as CF is a commondisease that are inherited and mostly found inyoung population.• It is an autosomal recessive disorder whichmeans that a person must receive two altered CFgenes in order to get this condition. Cystic fibrosis Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is the most common type of chronic lung disease in children and young adults, and may result in early death. 4 C YSTIC F IBROSIS. . The most common lethal inherited disease affecting Caucasians (not uncommon. Cystic fibrosis 1. Cystic fibrosis is an autosomal recessive disorder that affects epithelial cells of the respiratory , gastrointestinal and reproductive tracts and leads to abnormal exocrine gland secretions. An individual must inherit a defective copy of the CF gene (one from each parent) to have CF. Although it can affect many organ system, CF is particularly damaging to the lungs, leading.

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Cystic Fibrosis Gina Brandl, RN BSN Nursing Instructor, Pediatrics Cystic Fibrosis Overview of Disease Cystic fibrosis (CF) - inherited autosomal recessive - A free PowerPoint PPT presentation (displayed as a Flash slide show) on PowerShow.com - id: 3b7835-Yzdi Cystic Fibrosis and nursing Kristine Colpaert CF Nurse, UZ Leuven Belgium. CF AND NURSING Teamwork Education and knowledge Tasks Long term care for children and adults with CF. Disease progression Severe psychosocial impact Multidisciplinary team TEAMWORK Cystic Fibrosis ( CF) = Complex disease. CF patient Doctor Nurse Physio-therapist Psycho.

Cystic fibrosis - SlideShar

Cystic fibrosis (CF) is a disease of exocrine gland function that involves multiple organ systems but chiefly results in chronic respiratory infections, pancreatic enzyme insufficiency, and associated complications in untreated patients. Cystic fibrosis is an autosomal recessive disorder, and most carriers of the gene are asymptomatic Cystic fibrosis is an autosomal recessive genetic disorder that is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR) present in the mucus-secreting cells of the body, primarily affecting the respiratory, reproductive, and gastrointestinal tracts.. The hallmark manifestation of CF is chronic, progressive lung disease, resulting from. Case 12. Cystic Fibrosis Cystic Fibrosis Posted 11-29-04 Key Points Cystic fibrosis (CF) is caused by mutations in the CFTR gene and is inherited in autosomal recessive manner. Parents need help adjusting to the diagnosis of CF and accessing the best medical treatment available for their child Cystic Fibrosis Nursing Care Plan & Management. Prev Article Next Article . Notes. Description. Is an autosomal recessive disorder affecting the exocrine glands, in which their secretions become abnormally viscous and liable to obstruct glandular ducts. It primarily affects pulmonary and GI function Overview Autosomal recessive trait Mutation of cystic fibrosis transmembrane conductance regulator (CFTR) gene leading to buildup of mucus that obstructs pathways in the body. Life expectancy is 37 years old (According to the NIH, 2016) Most common cause of death is respiratory failure Nursing Points General Primary clinical features Mechanical obstruction caused by very thick [

Cystic Fibrosis • Inheritance • CF is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. • Heterozygous carriers (those who have inherited only one copy of the altered gene) are asymptomatic. • Two altered genes must be present for CF to appear. This means that if both parents are CF carriers, their offspring. Cystic fibrosis NCLEX nursing review: Cystic fibrosis is a genetic disorder that causes the exocrine glands to work incorrectly. This can lead to major compl.. Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis (2009) Consensus Recommendation Health Maintenance 4. The CF Foundation recommends that children with CF, ages 2 through 5 y, receive the fi rst dose of the pneumococcal polysaccharide vaccine (PPSV23), given at least 8 wk after las OBJECTIVES: To define Cystic Fibrosis To explain and discuss history and causes of Cystic Fibrosis To describe impact of Cystic Fibrosis in healthcare and nursing. Identify and recognize that living with a chronic disease like cystic fibrosis is possible through maintaining a balanced lifestyle. Identify expected outcomes in patients with Cystic Fibrosis Collect and explain health screenings.

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Cystic fibrosis (CF) clinical care guidelines exist for the care of infants up to age 2 years and for individuals ≥6 years of age. An important gap exists for preschool children between the ages of 2 and 5 years. This period marks a time of growth and development that is critical to achieve optimal nutritional status and maintain lung health Nursing Care Plan for Cystic Fibrosis Assessment Anamnesis Data collected during the assessment is used as a basis for nursing care plans. Nursing assessment process must be done very individualized (corresponding problems and current needs). In reviewing the status of breathing, the nurse did an interview and a physical examination to maximize. Northern New England Cystic Fibrosis Consortium .Case-mix adjustment for evaluation of mortality in cystic fibrosis. Pediatric Pulmonology, ;33(2):99-105. • O'Connor GT, Marshall, B, Quinton H, et al.( 2006). Public Reporting of Cystic Fibrosis Outcomes: Methods for Case-Mix Adjustment [abstract]. Pediatric Pulmonology - Supplement.;29S:119.

Box 1. Cystic fibrosis (CF), the most common life-shortening disease among whites in the United States, affects more than 30,000 people in the United States and 80,000 people worldwide. 1 CF occurs in about 1 out of 3,500 births per year in whites and northern Europeans. Although CF is a multiorgan system disease, its effects on the pulmonary system are the leading cause of patient morbidity. The autosomal recessive disease cystic fibrosis (CF) was once untreatable and deadly in childhood, but now most patients survive to adulthood. Many countries have instituted CF newborn screening because early diagnosis improves outcome. CF research has greatly intensified following the discovery of

cystic fibrosis - SlideShar

5 Cystic Fibrosis Etiology and Pathophysiology Enzymes trypsinogen, lipase, and amylase do not reach the intestine to digest ingested nutrients. Endocrine function may also be affected by advanced pancreati This report is the result of the Allied Health and Nursing Professions Working Group meeting which took place in Verona, Italy, November 2009, which was organised by the European Cystic Fibrosis Society, and involved 32 experts Want create site? With Free visual composer you can do it easy.1. Identify expected outcomes for an adult client living with cystic fibrosis. 2. Develop Health screening, health promotion, health interventions and education for adults with this condition. 3. Identify a comprehensive set of relevant resources, both community (Nevada) and national, for adults with this [ Cystic fibrosis is an inherited disorder that causes repeated lung infections, digestive problems and other complications. It is progressive, meaning it worsens over time. Children with cystic fibrosis have an abnormal gene that results in a defective protein. The protein (cystic fibrosis transmembrane conductance regulator, or CFTR) disrupts. As the nurse, it is important to know how to care for a patient with cystic fibrosis. In addition, the nurse needs to know the complications, signs and symptoms, testing procedures, medications used to treat, and nursing interventions for cystic fibrosis. Don't forget to watch the lecture on cystic fibrosis before taking the quiz

The following Australasian Nursing Standards of Cystic Fibrosis Care document has been written by nurses with specialist knowledge of, and experience in, the physiological, psychological and functional impact of cystic fibrosis on affected individuals and families. The clinical practice standards are evidence Cystic fibrosis causes lung damages from thickened secretions. A risk-related nursing diagnosis for cystic fibrosis would describe interventions that address complications from the disease. For example, recurrent lung infections are a symptom of cystic fibrosis, so Risk for infection related to chronic pulmonary disease would be a typical.

Cystic fibrosis (CF) is a monogenic disorder that presents as a multisystem disease. was first described as a unique disease entity in 1938. Most common lethal genetic disease in Causasians. lethal autosomal recessive disease. incidence: 1 in 2000-3000; predominantly Caucausian populations (carrier frequency 1 in 22-28) Start studying ppt 3. cystic fibrosis. Learn vocabulary, terms, and more with flashcards, games, and other study tools

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Cystic Fibrosis makes the lungs more susceptible to chronic infection There is a spectrum of organisms that affect patients with CF Some of the organisms are resistant to certain antibiotics Acquisition of certain organisms can limit treatment options. How are infections spread? Patient to patient transmission is one way that patients develop. This nursing care plan for Cystic Fibrosis includes a diagnosis and care plan for nurses with nursing interventions and outcomes for the following conditions: Ineffective Airway Clearance & Activity Intolerance. Patients who have cystic fibrosis are at risk for lung infections due to the body's overproduction of secretions Cystic Fibrosis Nursing Care Plan & Management. Prev Article Next Article . Notes. Description. Is an autosomal recessive disorder affecting the exocrine glands, in which their secretions become abnormally viscous and liable to obstruct glandular ducts. It primarily affects pulmonary and GI function Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis Drucy Borowitz, MD, Karen A. Robinson, PhD, Margaret Rosenfeld, MD, MPH, Stephanie D. Davis, MD

Cystic Fibrosis What Is Cystic Fibrosis Cystic fibrosis (CF) is a chronic, progressive, and frequently fatal genetic (inherited) dis­ ease of the body's mucus glands. CF pri­ marily affects the respiratory and digestive systems in children and young adults. The sweat glands and the reproductive system are also usually involved. On the average Cystic Fibrosis. The purpose of this paper is to define what cystic fibrosis is and the aetiology and/ or predisposing factors involved with CF. I will then describe the diagnostic process involved with CF. Furthermore, I will discuss two clinical manifestations arising from CF as well as the pathophysiology behind these clinical manifestations

Cystic Fibrosis Nursing Diagnosis Care Plan NCLEX Review. Nursing Study Guide: Cystic Fibrosis. Cystic fibrosis (CF) is a genetic disorder that primary affects the lungs and the digestive system. CF involves a defective gene that triggers the body to secrete mucus via exocrine glands. This causes digestive juices and sweat in a thick and sticky. Cystic Fibrosis. Clinical Features Cystic fibrosis is a heterogeneous recessive genetic disorder with features that reflect mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Most common lethal inherited disease in white persons, Typically presents in childhood 7% of CF patients diagnosed as adults Classic cystic fibrosis is characterized by chronic bacterial.

Cystic Fibrosis Disease Presentation Premium Google Slides theme and PowerPoint template. Cystic fibrosis is a chronic and hereditary disease, which affects the lungs and digestive system, mainly, but also other areas of the body. Today we bring you this Slidesgo template that is intended for a medical presentation about this pathology Cystic Fibrosis. Gina Brandl, RN BSN Nursing Instructor, Pediatrics Cystic Fibrosis Overview of Disease Cystic fibrosis (CF) - inherited autosomal recessive disorder in children Most common cause of chronic lung disease in children 30,000 children & adults in US Incidence Annually 1,000 children diagnosed 70% of patients are diagnosed by age 2 40% of CF population is age 18 and olde Cystic fibrosis is a genetic empiricism that reduces the spirit trust of the populace that are abnormal. This is beggarly in Caucasians and affects 1 in 3,200 Caucasians. The contrariety in the gene varies according to the geographical elucidation. Cystic fibrosis occurs in undivided quenched of total 15,000 African-American births

Cystic fibrosis presentation - SlideShar

Cystic Fibrosis is an autosomal recessive disease Cystic Fibrosis is an autosomal recessive disease Respond to your colleagues and respectfully agree or disagree with your colleague's assessment and explain your reasoning. In your explanation, include why their explanations make physiological sense or why they do not At least 2 references in each peer responses! Cystic Fibrosis [ Cystic fibrosis is a lifelong disease that affects the respiratory, endocrine,reproductive, and digestive systems. About 30,000 children and adults in the United States (70,000 worldwide) have CF. This disease is caused by a defective gene that makes the body produce very thick, sticky mucus Cystic fibrosis (CF) is an autosomal recessive genetic disorder that causes a lifetime of debilitating and life-threatening complications affecting the lungs and other organ systems. Over 1,700 gene mutations that cause this rare disorder have been identified. This article describes the current treatment landscape for adults with CF, including.

Cystic fibrosis.ppt - Google Slide

Cystic Fibrosis is a topic covered in the Diseases and Disorders. Nursing Central is an award-winning, complete mobile solution for nurses and students. Look up information on diseases, tests, and procedures; then consult the database with 5,000+ drugs or refer to 65,000+ dictionary terms. Explore these free sample topics Dorota Lelek Cystic fibrosis 18 Mucus glands that secretes increase quantity of thick,tenacious mucus Assess kung sounds and respiratory status,VS and oxygenationsaturation obtain sputum culture and sensitivity provide support to the child and familyprovide pulmonary managment ,gastrointestinal and endocrine managment administer prescribed. Oct 11, 2013 - Explore Connie McGee's board Nursing (Cystic Fibrosis), followed by 181 people on Pinterest. See more ideas about cystic fibrosis, cystic fibrosis awareness, pediatric nursing The Cystic Fibrosis Foundation recommends these infection prevention strategies when caring for patients with cystic fibrosis (CF). • Collaborate with the organization's infection prevention department to establish protocols and checklists for standards of practice when caring for more than one patient with CF. • Ensure disposable surgical masks are available for patients with CF to wear. Cystic fibrosis (CF) is a genetic disease that causes thick, sticky mucus to build up in organs, including the lungs and the pancreas. In a healthy person, mucus that lines organs and body cavities, such as the lungs and the nose, is slippery and watery. In people with CF, thick mucus clogs causes symptoms in the lungs and pancreas

PPT - Cystic Fibrosis PowerPoint presentation free to

  1. Cystic fibrosis primarily affects the glands that secrete. An abnormal protein is produced making secretions sticky. These thickened secretions are found in the body's sweat, the lungs' mucus, the pancreas' output, liver output, and reproductive organs. D. A 2 1/2 -year old boy with cystic fibrosis
  2. Cystic fibrosis (CF) is a genetic disorder that doctors usually diagnose in early childhood. Find out about the diagnostic procedure and the treatments that may help reduce CF symptoms
  3. g more important to consider how diet can keep you feeling healthy as you grow older
  4. Her parents have been in contact with the local Cystic Fibrosis Foundation chapter and have made an appointment to talk to a support counselor there. Darla's parents have demonstrated and verbalized the appropriate needs and care that she will need to continue to grow and thrive with this disease
  5. al childhood disease; however, because of numerous medical advances, individuals with CF have experienced a remarkable increase in life expectancy. The median predicated survival age is 43.6 years (Cystic Fibrosis Foundatio
  6. Treatment. There is no cure for cystic fibrosis, but treatment can ease symptoms, reduce complications and improve quality of life. Close monitoring and early, aggressive intervention is recommended to slow the progression of CF, which can lead to a longer life.. Managing cystic fibrosis is complex, so consider getting treatment at a center with a multispecialty team of doctors and medical.
  7. Kids with cystic fibrosis (CF) tend to get frequent respiratory infections, sometimes caused by bacteria or fungi. A sputum (mucus) CF respiratory screen or culture helps doctors detect and identify these bacteria or fungi so they can prescribe the most effective antibiotics to target a specific infection

Cystic Fibrosis Nursing Care Management: Study Guid

5 Cystic Fibrosis Nursing Care Plans - Nurseslab

  1. cystic fibrosis the disease developed when they were adults. SIGNS, SYMPTOMS AND COMPLICATIONS OF CYSTIC FIBROSIS Cystic fibrosis is primarily a disease that affects the lungs and the pulmonary problems of cystic fibrosis will be discussed in detail. However, cystic fibrosis can also have serious adverse effects on many other organ systems
  2. Refer people with suspected cystic fibrosis to a specialist cystic fibrosis centre if: • they have a positive or equivocal sweat test result • their assessment suggests they have cystic fibrosis but their test results are normal • gene testing reveals 1 or more cystic fibrosis mutations. 1.2 . Information and support . 1.2.
  3. Cystic Fibrosis case for first year medical students - Team Based Learning Format - Student Learning Objectives: 1. Learn to work in small groups effectively to solve a clinical problem. 2. Describe the how cystic fibrosis is diagnosed using a sweat test. 3. Describe the normal versus pathologic transport mechanisms of the CFTR channel
  4. Cystic fibrosis (CF) is a genetic disorder that causes problems with breathing and digestion. CF affects about 35,000 people in the United States. People with CF have mucus that is too thick and sticky, which. prevents proteins needed for digestion from reaching the intestines, which decreases the body's ability to absorb nutrients from food.

Cystic Fibrosis Nursing Care Plan & Managemen

  1. Course Availability: 6/21/21 - 6/21/23 Audience: Members of pediatric cystic fibrosis care team: cystic fibrosis clinical care coordinators, dietitians, fellows, genetic counselors, newborn screen coordinators, nurses, nurse practitioners, pharmacists, pharmacy technicians, physical therapists, physicians, physician assistants, psychologists, respiratory therapists, social workers
  2. The National Consensus Standards for the Nursing Management of Cystic Fibrosis (Cystic Fibrosis Trust, 2001b) recognise that adolescence and young adulthood is a difficult time for those dealing with both the psychological and practical burden of CF, and that the roles of the CF nurse specialist at this time include the following: - Promoting.
  3. Introduction. Cystic fibrosis is a genetic disorder in which internal bodily secretions become thick and sticky, and hinder the function of certain organs, such as the lungs and digestive system. The condition is caused by a faulty gene that controls the movement of salt and water in and out of cells in the body
  4. Cystic fibrosis is a disease that causes mucus in the body to become thick, dry, and sticky. This glue-like mucus builds up and causes problems in many of the body's organs, especially the lungs and pancreas. Cystic fibrosis is also known as mucoviscidosis, pulmonary fibrosis, and pancreatic cystic fibrosis. Approximately 30,000 people in the United States have cystic fibrosis
  5. Cystic Fibrosis Nursing Interventions and Nursing ReviewIn this tutorial for nursing students we will cover Cystic Fibrosis for nurses.Full Article and more... Skip to main content Due to a planned power outage, our services will be reduced today (June 15) starting at 8:30am PDT until the work is complete
  6. >Cystic fibrosis (CF) is a life-long, genetic disease > Organs commonly affected: lungs, pancreas, and intestines > Cystic Fibrosis (CF) National Guidelines recommend quarterly multidisciplinary clinic appointments to best manage disease and individualize treatmen
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08.06 Cystic Fibrosis (CF) NURSING.co

Justin Ewing is a sev en-year-old boy with a history of cystic fibrosis (CF) w ho for the past two day s has had a fever o f 102.1 F (38.9 C) and h as felt m ore tired. Hi s cough has become m ore frequent, and he is bringing up thick yellow m ucus Nursing Diagnosis for Cystic Fibrosis. Nursing diagnoses describe problems that can be addressed by nursing measures. Because nurses can't diagnose a disease or prescribe medication, a nursing diagnosis doesn't describe a disease or prescribe medications or treatments beyond a nurse's scope of practice This document requires a coversheet. More Details... Allergic Bronchopulmonary Aspergillosis ABPA and Cystic Fibrosis (Cystic Fibrosis Foundation) This document requires a coversheet. More Details... An Introduction to CF for Patients and Their Familes, Sixth Edition (10 Videos) (Cystic Fibrosis Foundation) This document requires a coversheet Cystic fibrosis is the commonest inherited disease in white populations, with an incidence of 1 in 2500 newborns; over 7000 people in the United Kingdom currently have the disease . Until recently, the diagnosis has been largely clinical, although the widespread implementation of a screening programme for newborns is now complete in the UK. Cystic fibrosis (CF) is the most frequent cause of suppurative lung disease in the younger Caucasian population. A depleted volume of the airway surface liquid (ASL) layer in the respiratory system leads to abnormal mucociliary clearance.A chronic cycle of infection and inflammation results in progressive suppurative bronchiectasis and lung damage..

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Cystic fibrosis (CF) is a serious genetic condition that causes severe damage to the respiratory and digestive systems. This damage often results from a buildup of thick, sticky mucus in the organs Cystic fibrosis. Cystic fibrosis is a disease that can be passed down from one generation to the other. It affects secretary glands that produce mucus and sweat. The disease results after the fibrosis transmembrane conductance regulator (CFTR) gene that is found on chromosome 7 has undergone some sort of mutation Approved by the CSW Cystic Fibrosis team for May 4, 2016 go-live Cystic Fibrosis Pathway Team: Cystic Fibrosis, Owner: Ron Gibson, MD, PhD, Principal Investigator, Supv Thida Ong, MD Medical CNS: Coral Ringer, MN, RN, CPN Clinical Nurse Specialist Janine Cassidy, Clinical Dietitian III Susan Casey

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Cystic fibrosis affects the sweat glands in a different way. The sweat does not become thick, but instead contains high levels of salt, resulting in salty skin. While cystic fibrosis is a chronic, progressive disease, improved treatments have significantly extended life expectancy for children with the condition CFTR, the gene associated with cystic fibrosis, encodes the protein cystic fibrosis transmembrane conductance regulator (also abbreviated CFTR, but not italicized). The gene was identified in 1989 and is found at 7q31.2, the long arm (q) of chromosome 7 at position 31.2. Survival has increased for patients with cystic fibrosis from late teens to mid-30s because of the many advances in. The Cystic Fibrosis Foundation has Cons (virtual conventions) for patients and families. The scheduled virtual events in 2021 are: April 15 and 17: ResearchCon. ResearchCon is a virtual event dedicated to cystic fibrosis science and research for anyone with a personal or professional connection to CF. June 11 and 12: CF FamilyCon

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cystic_fibrosis_powerpoint_final - CYSTIC FIBROSIS Created

Discussion: Cystic Fibrosis Study. Module 2: Discussion Melissa, a 12-year-old girl with cystic fibrosis comes to the primary care office with complaints of increased cough and productive green sputum over the last week. She also complains of increasing shortness of breath. She denies sore throat or nasal congestion Cystic fibrosis (CF) is a multisystem disorder caused by pathogenic mutations of the CFTR gene (CF transmembrane conductance regulator). Pulmonary disease remains the leading cause of morbidity and mortality in patients with CF. (See Cystic fibrosis: Genetics and pathogenesis and Cystic fibrosis: Clinical manifestations and diagnosis . Cystic Fibrosis (CF) is a progressive genetic disease that affects the lungs and digestive system as well as many other organ systems. It causes thick, sticky mucus to build up in the lungs, which leads to life-threatening lung infections. There are approximately 30,000 people with CF in the United States and 70,000 worldwide Cystic fibrosis causes the sweat glands to produce sweat that has an abnormally high salt content. The high salt content in perspiration is the basis for the sweat test, which is the definitive diagnostic test for the presence of cystic fibrosis. Mutations associated with cystic fibrosis can be detected in screening tests. These tests are effective in the identification of adult carriers.

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cystic fibrosis is a topic covered in the Taber's Medical Dictionary. Nursing Central is an award-winning, complete mobile solution for nurses and students. Look up information on diseases, tests, and procedures; then consult the database with 5,000+ drugs or refer to 65,000+ dictionary terms. Explore these free sample topics Cystic fibrosis (CF) is a genetic disease causing sticky, thick mucus for building up in the digestive tract, lungs and other parts of the body, as a result of which there is serious damage to the digestive and respiratory systems. The disease is a condition of the exocrine gland function involving multiple organs When a nurse is taking care of a patient with cystic fibrosis, there are several nursing diagnoses that she should keep in mind. Below are 5 nursing diagnoses that can commonly be applied to a patient with CF: 1) Impaired gas exchange related to increased mucus production and thick mucus secretions. This nursing diagnosis is especially. MIRIAM - A Cystic Fibrosis Case Study. My niece, Miriam, was born in April 1996. At 6 pounds 12 ounces, she had difficulty putting weight on after she was born. In fact at one point it dropped to 6 pounds. After a spell in hospital in December 1996, when she was suffering from bronchitis, it was thought that she had asthma but my sister-in. Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the body's salt, water- and mucus-making cells. While it is a chronic, progressive disease, improved treatments have significantly extended life expectancy. Most people with CF live into their late 30s, and many even into their 50s or longer Cystic fibrosis, or CF, is an autosomal recessive disorder in which there's a mutated cystic fibrosis transmembrane conductance regulator or CFTR protein.. The mutated CFTR protein causes secretions to be abnormally thick and sticky - and they build up in the lungs, pancreas, and other organ systems