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cystic_fibrosis_powerpoint_final.pptxCystic Fibrosis
Created By: Tammy Wagner, Karen Byfield, Sherry Santineau, Jack Lavoie, Shawna Maggard
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, health interventions, health promotion and education in patients with Cystic Fibrosis
Identify national and community resources for patients with Cystic Fibrosis
Describe effects of CF on prenatal care and child bearing
Identify occupational consideration and job hazards related to CF
Identify and describe susceptibility to substance abuse and ability to cope with stress
Description:
Cystic Fibrosis is a genetic disease of the secretory glands that affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.
Definition:
Cystic Fibrosis is characterized by an abnormally thick and sticky production of mucus in the body.
Mucus is normal in the body, it lubricates and protects passages such as the respiratory airways and digestive tract (Genetics Home Reference. 2017). Cystic Fibrosis is a mutation in the the Cystic Fibrosis Transmembrane Regulator (CFTR) gene that causes the over production of thick, sticky mucus that can block these passages and create breeding grounds for infection (National Human Genome Research Institute 2013). This infection can lead to scaring, permanent lung damage and even death. This mucus in the digestive tract can prevent the absorption of nutrients leading to weight loss, malnutrition, failure to thrive, as well as numerous long term problems caused from poor nutrition even though the person may be eating a solid health diet (Genetics Home Reference. 2017). The affects this mucus has on the pancreas can alter insulin production leading to diabetes. Reproductive health is also affected from the mucus produced from cystic fibrosis often leading to infertility (Genetics Home Reference. 2017).
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History
1938- American Pathologist Dr. Dorothy Andersen named the disease cystic fibrosis of the pancreas
Early 1900’s- Physicians name the disease mucoviscidosis
1595- Texts reference the link between children with salty skin and early death rates
1948- Dr. Paul di Sant’Agnese noted the correlation of increased salt in sweat of CF patients
1989- The gene (CFTR) was identified and its genetic code was sequenced
1938, American Pathologist Dr. Dorothy Andersen described a disorder in medical literature based on autopsy findings of children that died from malnutrition; She named the disease cystic fibrosis of the pancreas (Nick 2012). The disease has also been called mucoviscidosis by other physician during the same time period because of the thick mucus present (Nick 2012). Children in the middle ages were thought to be heed or bewitched if they had salty skin, a symptom of CF (Nick 2012). Dr. Paul di Sant’Agnese noted that cystic fibrosis patients secreted more salt in their sweat during a heat wave in New York in 1948. This lead to sweat testing for cystic fibrosis (Nick 2012). In 1989 the Cystic Fibrosis Transmembrane Regulator (CFTR) gene was identified and sequenced allowing for further treatments to be developed (Nick 2012). What was once a fatal childhood disease has now become a disease that is being treated more successfully with a longer life expectancy. At the beginning of the century children with CF were not expected to life longer than a few years; with breakthroughs in treatment the median predicted survival age today is around the age of 40 (Cystic Fibrosis Foundation 2016).
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Pathology
Cystic Fibrosis is caused from a mutation of the Cystic Fibrosis Transmembrane Regulator (CFTR) gene
This gene is responsible for the transport of chloride, and in turn water across cell membranes in the body.
The mutation of the CFTR gene prevents the chloride channels from regulating the flow of chloride and water, this causes the cells to become thick and sticky.
These thick and sticky cells, mucus, interfere with airways and passages throughout the body and influence overall health.
The mutated CFTR gene must be passed from both parents to be present in a child. This gene is responsible for sodium chloride channel permeability across cell membranes. The mutation of this gene prevents sodium chloride from crossing cell membranes and therefore dehydrates cells causing a build up of thick, sticky mucus. This mucus build up affects the lungs, pancreas, digestive system, liver and reproductive organs (National Human Genome Research Institute 2013).
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Impacts Adults
Increasing number of Cystic Fibrosis patients are surviving into adulthood.
These people function well until the disease becomes severe.
There are increasingly more adults living with cystic fibrosis due to the advances in medicine over the last 10 years. When quality of life was examined, it was found that those living with Cystic Fibrosis had a worse quality of life due to impaired physical function. They were less likely to be in employment than others. As they get older and their disease progresses, they suffer in self-esteem. Anxiety and depression become prevalent (Pfeffer & Pfeffer, 2003, p. 64).
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People With Cystic Fibrosis
Suffer with interpersonal relationships
Have sexual difficulties; body image, intimacy, and reduced fertility
Have problems with independence
Suffer psychological problems of embarrassment due to cough and sputum
People with Cystic Fibrosis have trouble fitting into society. Interpersonal problems arise due to isolation and social maladjustment. Fear of moving away from home is ever present. More people with Cystic Fibrosis live in the parental home into adulthood, especially men. Bilateral absence of the vasa deferentia occurs in most men with CF causing infertility. Mothers have been found to be over protective to the child/adult of CF causing independence problems (Pfeffer & Pfeffer, 2003, p. 66).
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Impact of Cystic Fibrosis
Healthcare System and Nursing Practice:
CF patients have trouble obtaining health insurance
Having to learn to navigate the healthcare system as adults
Daily treatments are complex and time consuming
Development of clinical care structure
Transfer to specialty center as adults
CF patients are now getting pregnant and delivering their children
Some CF patients have difficulty obtaining health insurance after they age out of their parents’ plans (Peach, 2011)
Mastering these tasks will help the patients stay healthy as an adult. These tasks include finding doctors, scheduling appointments, picking up prescriptions, etc. (Sawicki, 2009)
The treatment advances require more time and effort put into the treatment and challenges are placed onto the patient for better self-management strategies (Sawicki, 2009)
The structure supports the patients with complex treatment and preventions (Sawicki, 2009)
When children reach adulthood, they should transfer to a cystic fibrosis specialty center (Medline Plus, 2017)
The number of women who have become pregnant have doubled in the last 20 years. Because of improved health outcomes, women with CF face reproductive health decisions. This includes whether to have a child or not (Ladores, Kazmerski, and Rowe, 2016)
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Why live into adulthood with cystic fibrosis
Early diagnosis
Treatment plan
Good health until adulthood
Live to 37 years old
Improved management and knowledge of CF
People are not detected until age 18 or older
Milder form of disease
Most children with CF are diagnosed by age 2. Early diagnosis of CF and treatment plan can improve survival (Medline Plus, 2017)
They are able to take part in normal school activities and attend school (Medline Plus, 2017)
The life span for people with CF who live to adulthood is about 37 years old. Death is usually caused by lung complications (Medline Plus, 2017)
Adults are more aware of proper management and knowledge of CF.
For a small number, the disease is not detected until age 18 or older (Medline Plus, 2017)
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Validation of Cystic Fibrosis
Statistics
Life expectancy:
1950 6 years of age
2007 37.4 years of age
Number of people with CF in U.S. :
1 in 2,500 – 3,500 Caucasian-Americans.
1 in 4,000 – 10,000 Hispanic-Americans.
1 in 15,000 – 20,000 African-Americans.
1 in 100,000 Asian-Americans
Cystic Fibrosis (CF) is hereditary and begins at conception. A person does not “come down with CF”. As diagnostics improve however, people with chronic problems that are lesser in severity are being diagnosed later in life. Life expectancy has greatly increased since 1950 because of improved treatment techniques and proper diagnoses. People with CF are now living close to 40 years of age. Quality of life has also improved due to better understanding of the disease, improvement in treatment, and proper nutritional awareness (American Lung Association [ALA], 2017).
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Validation of Cystic Fibrosis
Demographics
According to Cystic Fibrosis Foundation Patient Registry, in U.S.:
Approximately 30,000 Americans are living with cystic fibrosis.
Nearly 1,000 new cases of CF are diagnosed each year.
More than 75 percent of people with CF are diagnosed by age 2.
More than half of the CF population is age 18 or older.
According to the Cystic Fibrosis Foundation, there are about 30,000 Americans, 20,000 Europeans and 3,000 Canadians with CF. This disease occurs mostly in Caucasians of Northern European decent. In addition, about 1 in every 20 Americans is an unaffected carrier of an abnormal “CF gene.” Most of these people are unaware they are carriers There is only one risk factor for getting CF and that is having two parents who carry the abnormal CF gene and pass the abnormal gene to their child. (2016).
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Care Plan
Nursing Diagnosis: Ineffective Airway Clearance related to excessive secretions and ineffective cough
Nursing Intervention Classification: Airway management
Nursing Outcome Classification: Respiratory Status: Airway Patency. Patient will maintain clear open airways as evidenced by ability to cough up secretions, normal breath sounds, and normal rate/depth of respirations
Ongoing Assessment/Interventions:
Assess airway for patency. Maintaining airway is the first priority
Auscultate lungs for decreased, absent, or adventitious breath sounds that may indicate obstruction
Use pulse oximetry to monitor oxygen saturation
Assess respirations, noting quality, rate, depth, and presence of dyspnea on exertion
Position patient in upright position to promote lung expansion and improved air exchange
Lung disease and airway issues account for the majority of morbidity and cause of death for adult patients with cystic fibrosis (Gulanick, 2014). Due to the over secretion of thick mucus and inability to clear the lungs efficiently results in frequent chronic infection.
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Care Plan
Screen for Cystic Fibrosis in adults:
Swab the inner cheek for tissue sample
Sweat Test (high chloride level in sweat indicates CF)
Screen for Cystic Fibrosis Related Diabetes (CFRD)
Symptoms: weight loss, protein catabolism, lung function decline
Oral Glucose Tolerance Test
Screen for Osteoporosis
DEXA Test: measure bone density, done every 1-5 years
Maintain good health
Avoid being around ill/sick people, avoid kissing, hugging, or sharing personal items.
Get influenza vaccine, practice good hand hygiene, good nutrition (high calorie, high fat)
It is a necessity to constantly monitor nutrition and pulmonary function of CF patients as well as screen for complications associated with the disease. Though most cases of Cystic Fibrosis are diagnosed in early childhood, it still possible to find adults living with the disease and they might not be aware of it. The diagnosis is due to a wide range of CF manifestations that a patient may be presenting with. Adults that present with chronic bronchitis, sinusitis, male infertility or pancreatitis should be screened for CF (George & Hazle). One way of screening for CF is to swab the inner cheek for a tissue sample and another is the sweat test. High amounts of chloride in a person’s sweat indicates CF. Once the diagnosis of Cystic Fibrosis has been determined then immediate care can be implemented to treat the disease. Cystic Fibrosis Related Diabetes does not exhibit any symptoms in CF patients. Signs and symptoms of CFRD are weight loss, protein catabolism, a decline in lung function, and increased mortality” (Moran, 2010). A1C screening for CFRD is not recommended because it does not correlate with glucose tolerance in CF patients. The preferred testing method for CFRD is the oral glucose tolerance test. It works well because the decline in lung function has no effect on the test. Osteoporosis is another complication that may develop and it should be screened for in patients suffering from CF. The test of choice is the is the DEXA which measures bone density. The DEXA scan is an x-ray that can detect weak bones. The scan should be done every 1 to 5 years. Infection prevention is the key to maintaining good health. To prevent from getting ill, CF patients should avoid being around people who are ill, get their annual immunizations such as the influenza shot, and practice good hand hygiene. Good nutrition is another important factor in promoting health. Having a high calorie, high fat diet is important because malabsorption of nutrients occurs in CF patients. Some interventions that CF patients should implement are to avoid shaking hands or hugging others, kissing, do not share utensils of cups, and do not participate in exercise classes at a gym.
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Resources
Community
CF2Chat
a forum for people to chat about CF dedicated to therapy and health tips.
National
Youtube.com has a Cystic Fibrosis channel:
CysticFibrosisUSA
Mayo Clinic
Cystic Fibrosis Foundation
Cystic Fibrosis Living
Online Resource relevant to CF: CF Living http://www.cfliving.com/resources/resources-links.jsp
Cystic Fibrosis Foundation is a world leader in dedication to curing cystic fibrosis and improving the quality of life for those living with CF. https://www.cff.org/
Mayo Clinic’s offer resources including help for the family and spouse of those with CF.
There is so much information on the internet about CF (CF Living website, 2017).
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Resources For Cystic Fibrosis
Cystic Fibrosis Foundation (https://www.cff.org/)
Help One Love (http://helponeloveone.org)
CF Living (http://www.cfliving.com)
Cystic Fibrosis .com (http://cysticfibrosis.com)
A wealth of information and resources for people affected by cystic fibrosis. The CFF is one of the world’s largest organizations dedicated to finding a cure for cystic fibrosis.
Help One Love provides funding to adults with cystic fibrosis, primarily to aid with nutritional needs and other assistance when financially possible.
A place for community support and information for those living with cystic fibrosis. A place to find a management plan and to find care teams to help learn how to live with CF.
CysticFibrosis.com is one of the world's largest social media networks dedicated exclusively to the cystic fibrosis community. In addition to the forums, the site contains information on clinical trials, gene therapy, testing, associations, research and events.
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References:
American Lung Association. (2017). Learn about Cystic Fibrosis. Retrieved from: https://www.lung.org/lung-health-and-diseases/lung-lookup/cystic-fibrosis/learn-about-cystic-fibrosis.html
CF Living website. (2017). http://www.cfliving.com/resources/resources-links.jsp
Cystic Fibrosis Foundation. (2016). About Cystic Fibrosis. Retrieved from: http://www.cH.org/what-is-CF/Testing/Carrier-Testing-for-CF/
Genetics Home Reference. (2017). Cystic Fibrosis. US National Library of Medicine. Retrieved from https://ghr.nlm.nih.gov/condition/cystic-fibrosis
George, C., & Hazle, L. (n.d.). Cystic Fibrosis. Retrieved January 24, 2017, from http://nursing.advanceweb.com/Continuing-Education/CE-Articles/Cystic-Fibrosis.aspx
References:
Gulanick, M. & Myers, J. (2014). Nursing care plans : diagnoses, interventions, and outcomes. Philadelphia, PA: Elsevier/Mosby.
Ladores S., Kazmerski T., and Rowe S. (2016) A Case Report of Pregnancy During Use of Targeted Therapuetics for Cystic Fibrosis. Journal of Obstertric, Gynecologic, & Neonatal Nursing (Volume 46, Number 1) (pg. 72).
Medline Plus Medical Encyclopedia. (2017). Cystic Fibrosis. National Institutes of Health/ U.S. National Library of Medicine.
Moran, A., Brunzell, C., Cohen, R. C., Katz, M., Marshall, B. C., Onady, G., … the CFRD Guidelines Committee. (2010). Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes: A position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care, 33(12), 2697–2708. http://doi.org/10.2337/dc10-1768
References
National Human Genome Research Institute. (2013). Learning About Cystic Fibrosis. Retrieved from https://www.genome.gov/10001213/
Nick, J. (2012). Cystic Fibrosis: Hystory. National Jewish Health. Retrieved from https://www.nationaljewish.org/conditions/cystic-fibrosis/history
Pfeffer, P. E., & Pfeffer, J. M. (2003). The Psychosocial and Psychiatric Side of Cystic Fibrosis in Adolescents and Adults. Journal of Cystic Fibrosis, 2, 61-68. http://dx.doi.org/10.1016/S1569-1993(03)00020-1
Sawicki G., Sellers D., and Robinson W. (2009). High Treatment Burden in Adults With Cystic Fibrosis: Challenges to Disease Self-Management. Journal of Cystic Fibrosis.
