Lori Alley
Erika Lemerande
Nicole Carpenter
Maureen Joseph
June Reyes
Clinical Description and Definition of the Disease.
“Cystic Fibrosis is a life threatening, genetic disease that causes persistent lung infections, and progressively limits the ability to breathe” (“Cystic Fibrosis,” n.d.).
Pathophysiology and
History of the Disease
• Cystic Fibrosis is caused by defects in the cystic fibrosis gene.
• Cystic Fibrosis was recognized over 400 years ago in Germany.
• There is no cure for Cystic Fibrosis.
Impact on Adults to the Health
Care System and Nursing Practice
• Increased heath care costs
• Over crowed clinics
• Individuals received less personal attention • Limited number of hospital beds
• Nursing education
Impact
of the Disease on Childhood
• Altered body image
• Impaired relationship with peers
• Impact of living with a chronic disease
Impact of the Disease on Adults
• Burden of care increases
• Taking more medicines and receiving more treatments.
• Lung damage is greater in adulthood than earlier in life.
• Fertility issues (Heintz, 2010, para. 6)
Reasons People with CF are living into Adulthood
• GENE THERAPY
• ANTIMICROBIAL THERAPY
• THE MODERN CF CARE
CENTER
• MUCOLYTICS
• MUCOCILIARY CLEARANCE
Impacts this Disease will have on the Adult Client.
• Mentally and emotionally
• Physically
• Sexually
• Economically, specifically as it relates to the ability to access health insurance and health care
• Susceptibility to engage in substance abuse
• Prenatal care and childbearing (see additional slide)
• Occupational considerations and hazards (see additional slide)
• Ability to cope with stress (see additional slide)
Pregnancy
Pregnancy
is now possible with cystic fibrosis Pregnancy
• Importance of Prenatal Care
• Pregnancy Statistics
• Focus on Family Planning
Infertility causes
• The majority of men with cystic fibrosis have associated congenital bilateral absence of the vas deferens ( mutations,
1995).
• Female infertility is less clear. There is a theory that the vaginal mucosa is to thick for sperm passage. Other reasons are thought to be low BMI, poor nutrition, and chronic infection
(Hodges, 2008).
Choosing a career
• Cystic Fibrosis should not prevent one from pursuing the career they seek
• Some careers pose greater health risks for Cystic Fibrosis patients (Careers, 2013)
Learning to cope
The key is to limit stress by:
– Knowledge: ask, watch, study and read
– Support: support groups, get in touch with other families that know the ropes.
– Planning: getting through daily life will be overwhelming, write a daily plan.
– Strength: do not listen to well meaning people with negative advise listen to your healthcare providers
(About, 2014).
Outcomes for the Adult Patient
• Adequate airway clearance
• Reduction of risk factors associated with respiratory infections
• Ability to perform activities of daily living (ADLs)
• Active participant in planning and implementing a therapeutic regiment
Health Screening / Health
Promotion / Health Interventions /
Education For Adults with CF
• Screening / Promotion:
•Genetic testing
• Health Intervention /
Education:
•Airway Clearance Techniques
•Inhaled Medications
•Nutrition
•Fitness
•CFTR Modulators
•Lung Transplantation
Ensuring the best possible future
• Establish a routine • Be diligent
• Learn to juggle
Relevant Resources
(Community & National) for
Adults with CF
• Great Strides – Cystic Fibrosis Foundation
(Central Oregon 2015) http://greatstrides.cff.org • Cystic Fibrosis Living http://cfliving.com
• Support * Education * Hope http://cysticfibrosis.com References
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About cystic fibrosis. (n.d.). Retrieved from https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/
Alma, L. (2014, June 10). How to Cope When Living With Cystic Fibrosis. Retrieved August 5,
2015.
American Lung Association. (n.d.). Retrieved from www.Lung.org
Boas, S. R., & McColley, S. A. (2010). Advances in Cystic Fibrosis (CF). Retrieved from https://www.answers4families.org/family/special-needs/medical-disabilities/advances-cystic-fibro sis-cf
CareersPortal.ie - Cystic Fibrosis. (2013). Retrieved August 5, 2015
Conway, S., Stableforth, D., & Webb, A. (1998). The failing health care system for adult patients with cystic fibrosis. Thorax, 53, 3-4. http://dx.doi.org/10.1136/thx.53.1.3
Cystic Fibrosis (CF), (2010). Retrieved from http://www.lung.org/assets/documents/publications/solddc-chapters/cf.pdf, on August 5, 2015.
Cystic Fibrosis Foundation (CFF) (n.d.). Retrived from https://www.cff.org, on August 3rd, 2015
Darmouth-Hitchcock website. (2015). Retrieved from http://www.dartmouthhitchcock.org/cystic_fibrosis/your_emotional_health.html Heintz, J. S. (2010). Adults with Cystic Fibrosis. Retrieved from http://www.netwellness.org/healthtopics/cysticfibrosis/adultscf.cfm History of cystic fibrosis. (2012). Retrieved from http://www.genemedresearch.ox.ac.uk/cysticfibrosis/history.html Hodges, C., Palmert, M., & Drumm, M. (2008, March 6). Infertility in Females with Cystic Fibrosis
Is Multifactorial: Evidence from Mouse Models. Retrieved August 5, 2015.
References
• Mayoclinic website. (n.d.). Retrieved from: http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/com plications/con-20013731
• Mutations in the Cystic Fibrosis Gene in Patients with Congenital Absence of the Vas Deferens — NEJM. (1995, June 1). Retrieved August 5, 2015.
• Pregnancy and cystic fibrosis. (2008, April 1). Retrieved August 5
• Psychological impact. (n.d.). Retrieved from http://www.cysticfibrosis.org.uk/about-cf/living-with-cystic-fibrosis/psycho logical-impact
• Sharma, G. (2015). Cystic fibrosis. Retrieved from http://emedicine.medscape.com/article/1001602-overview#a3 • Van Gool, K., Norman., R., Delatycki, M.B., Massie, J., Understanding the costs of care for cystic fibrosis: An analysis by age and health state. The journal of Value Health. 16, 3345-55
• Vandenbranden, S. (2015). A nurse’s perspective on advances in care for cystic fibrosis. Medscape Education Pulmonary Medicine. Retrieved from
http://www.medscape.org/viewarticle/746276