N380 Clinical Case Study Presentation: Cystic Fibrosis By

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N380 Clinical Case Study Presentation Cystic
Fibrosis

• By Alex, Brittany Annette

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Our patient

• 13 year old
• Male
• Caucasian
• Irish Russian descent

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Nursing Diagnoses

• Ineffective Airway clearance related to cystic
  fibrosis as evidenced by SOB, wheezing, and
  continuous cough
• Impaired gas exchange RT lung infection
• Ineffective family/patient coping related to
  stress of chronic illness as evidenced by marital
  problems (family) and depression (patient).

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Organs Affected by Cystic Fibrosis
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Significant History

• Cystic Fibrosis Autosomal recessive disease with
  abnormally thick, abundant mucous gland
  secretion.
• - Chronic obstructive pulmonary disorder
• - Chronic lung infection damages lung tissue

- Becomes restrictive lung disease from fibrosis,
lung destruction and thoracic wall changes.
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Significant History (Cont.)

• Cystic Fibrosis
• Exocrine pancreatic insufficiency
• Mucus plugs pancreatic duct
• Causes fibrosis of acinar glands
• Lipase, amylase, proteases dont reach intestines
  to digest fat, protein and Vitamins ADEK
• Islets of Langerhans also become fibrotic
• Type I Diabetes

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Significant History (Cont.)

• Depression
• Asthma, Allergic Rhinitis, Chronic Sinusitis
• Hiatal Hernia, GERD, Lap Nissen x2
• Sensorineural BL Hearing Loss
• Short Stature
• Port Placement

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Significant Lab Values and Diagnostic Tests

• Blood based DNA testing for CF disease and
  carrier status
• Prenatal Test Chorionic vilus sampling.
• Sweat Chloride Test
• Meconium Ileus
• Glucose testing for DM
• LFT, Chem 7, CBC, UA

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Communication

• Hearing Loss
• Poor family communication

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Socio-economicHealth Care Barriers

• Transportation and visiting costs
• Moving for employment
• Child Protective Services

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Spiritual Factors

• Catholic
• Chaplain
• Observes Religious Traditions
• Prayer

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Medications

• Q-10 Co-enzyme, Vitamin D
• Megace
• Pulmozyme neb
• Ultrase MT-20 Pancrelipase
• Cipro Tobramycin
• Meropenem
• Aztreonem
• Zithromax
• Prozac
• Somatropin

• HGH
• Nexium
• Albuterol
• Zyrtec
• Singulair
• Novolog
• Lantus
• Prednisone
• Benadryl
• Motrin

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Treatments
Physiotherapy
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Ethical Considerations

• Median survival 35 years
• 5 of 7 siblings also have CF

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Developmental Level

• Language Cognitive Doing well in school despite
  frequent absences. Very mature.
• Physical Short stature, small muscle mass.
• Fine Motor Video games, coordinated.
• Gross Motor Tires easily
• Personal Social Isolated from peers, relocation,
  wants to live with mother.

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Play and Activities

• Needs more physical exercise
• Video games
• TV
• Lack of time with peers due to continuous
  grounding

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Plan of Care

• Lung health
• Breathing treatments/Respiratory Stats
• Exercise
• Glucose monitoring
• Nutrition and Hydration
• Therapeutic conversation

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Patient Family Teaching

• Facilitate Interfamily communication
• Nutrition for CF IDDM
• Exercise
• Support Groups

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Journal References

• Title The Flutter Device versus the PEP Mask in
  the Treatment of Adults with Cystic Fibrosis
• Purpose Compare effectiveness of flutter device
  to the Pep Mask in treating CF
• Sample 42 adults with CF
• Method
• Adults were randomly assigned one of the
  treatments
• Pulmonary function test, Quality of Wellbeing
  Scale, and Chronic Respiratory Disease
  Questionnaire were administered every 3 months,
  to measure effectiveness of treatment on adult
• Results There was no significant difference in
  pulmonary function or health-related QoL

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References

• Newbold, E., Tullis, E., Corey, M., Ross, B.,
  Brooks, D. (2005).The Flutter Device versus the
  PEP Mask in the Treatment of Adults with Cystic
  Fibrosis. Physiotherapy Canada. 57, 199-206.
• Swisher, A., Erickson, M. (2008, December).
  Perceptions of physical activity in a group of
  adolescents with cystic fibrosis. Cardiopulmonary
  Physical Therapy Journal, 19(4), 107-113.
  Retrieved April 23, 2009, from CINAHL with Full
  Text database.
• Wilkinson, J. M. (2005). Nursing Diagnosis
  Handbook (Eighth ed.). Upper Saddle River, New
  Jersey Pearson Prentice Hall.