NURSING CARE for the PATIENT with CF

What NURSES can do for PATIENTS!

Overall Assessments and Possible Findings:

System	Assessment	Clinical Manifestations
Respiratory	Respiratory rate, rhythm, and effort Use of accessory muscles Pulse oximetry Presence of cough Presence of sputum – color, consistency, and amount Breath sounds	Fatigue Chronic cough Thick, yellow-grey mucous Positive sputum culture (e.g., Pseudomonas aeruginosa, Haemophilus influenzae) Fever Shortness of breath, dyspnea, and wheezing Cyanosis Difficulty exhaling air, resulting in hyperinflation of the lungs Barrel-shaped chest Clubbing of the fingers and toes
Gastrointestinal	I&O Weight Stool Bowel sounds Palpation for tenderness	Large, loose, fatty, sticky, foul- smelling stools Impaired digestion Failure to gain weight Delayed growth patterns Distended abdomen Thin arms and legs Atrophy of buttocks and thighs
Integumentary	• Color • Nails • Peripheral pulses	Positive sweat chloride test Sweat, tears, and saliva abnormally salty Report of salty taste when kissing the child
Growth and Reproduction	Height and weight Development of secondary sex characteristics	Delayed puberty Viscous cervical mucous Decreased or absent sperm count

Taken from NCLEX review website: 

http://www.atitesting.com/ati_next_gen/FocusedReview/data/datacontext/RM%20NCC%20PN%207.1%20Chp%2019.pdf

Interventions again:

Nursing Care of Children

• ∆  Respiratory Interventions
  
  • Promptly treat respiratory infections with antibiotic therapy.
    
    
  • Provide pulmonary hygiene with chest physiotherapy (CPT)
    
    

    -breathing exercises to strengthen thoracic muscles

    
    

    - minimum of twice a day (in the morning and at bedtime

    
    
    
    
  • Have the child use the Flutter mucus clearance device to assist with mucus removal.
    
    
  • Administer bronchodilators through metered dose inhalers (MDIs) or hand-held nebulizer to promote expectoration of excretions.
    
    
  • Administer dornase alfa (Pulmozyme) through a nebulizer to decrease viscosity of mucus.
    
    VERY IMPORTANT:
    *Promote physical activity that the child enjoys to improve mental well-being, self-esteem, and mucus secretion
    
    
• ∆  Gastrointestinal Interventions
  
  
•            give pancreatic enzymes with meals and snacks
•            capsules can be:
• swallowed whole  
• opened to sprinkle the contents on a small amount of food.

MEAL TIME

• ◊  Encourage the child to select meals and snacks if appropriate.
  
• ◊  Facilitate high-caloric, high-protein intake through meals and snacks.
  
• ◊  Multiple vitamins and water-soluble forms of vitamins A, D, E, and K are often prescribed.
  
  
  Hospitalization

• Perform CPT 1 hr before meals or 2 hr after meals if possible.
  
• Use oxygen with caution to prevent oxygen narcosis.
  
• Promote adequate nutritional intake, and provide pancreatic enzymes at meals and with snacks.
  
• Encourage adequate fluid and salt intake.
  
• Provide meticulous skin care and oral hygiene.
  
• Provide encouragement and support to the child/family by using family-centered nursing care.
  
  Care in the Home
  

• Ensure parents/caregivers have information regarding access to medical equipment.
  
• Provide teaching about equipment prior to discharge.
  
• Instruct parents/caregivers in ways to provide CPT and breathing exercises.
  
  For example, a child can “stand on her head” by using a large, cushioned chair placed against a wall.
  
  
• Administer antibiotics through a venous access port.
  Parents/caregivers need instruction in administration techniques, side effects to observe for, and how to manage difficulties with the venous access port.
  
  
• Promote regular primary care provider visits.
  
• Ensure up-to-date immunizations with the addition of initial influenza vaccine at 6 months of age and then a yearly booster.
  
• Encourage regular physical activity.
  
• Encourage participation in a support group(s) and involvement in community resources.

Primary Reference:
Hockenberry, M., Wilson, D., Winkelstein, M. (2005). Wong’s essentials of pediatric nursing care. (7th ed.). St. Louis, MO: Mosby. 


AND HAVE FUN!

OTHER NURSING CARE RESOURCES:  ie: for student nurses

• http://www.istudentnurse.com/peds/cf/
• http://www.elsevier.com/__data/promis_misc/2005.pdf
• http://www.nursingtimes.net/the-role-of-the-cystic-fibrosis-nurse-specialist/206445.article
• http://depts.washington.edu/pedspulm/cfcenter.htm