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

NURSING INTERVENTIONS

For Nursing Students and Nurses caring for the patient with Cystic Fibrosis...


Diagnoses:

1. Ineffective airway clearance, related to accumulation of mucus.
2. Imbalanced nutrition, less than body requirements, related to malabsorption from ineffective production of pancreatic enzymes
3. Deficient knowledge, related to the diagnosis and condition of child.
4. Risk of infection, related to bacterial invasion of respiratory system.
5. Ineffective coping related to new diagnosis and management of disease.


More specific diagnosis with what you can do as a nurse to help!

Diagnosis:  
                    Ineffective airway clearance, related to accumulation of mucus.

PLAN/ OUTCOME

NURSING INTERVENTION

-absence of dyspnea and tachypnea (breathing improved to normal range based on age) -can effectively remove mucus from lungs through coughing -improved heart rate for age -maintain oxygen saturation above 93%

ASSESSMENTS: -lung sounds -breathing rate and rhythm -heart rate -oxygen saturation THERAPY/TREATMENT: -assist with chest physiotherapy ·      Chest percussion and drainage ·      Apply external percussion vest ·      Use of airway oscillating device -administer medications per orders ·      Bronchodilators ·      Nebulizers ·      Antibiotics ·      Anti-inflammatories -provide oxygen as needed based on saturations EDUCATION: -breathing exercises -medications MONITOR AND REASSESS: -effectiveness of treatment

References:

Price, D., & Gwin, J. (2008). Respiratory Disorders. In Nurse's Pocket Guide: Diagnoses, Prioritized Interventions, and Rationales (11th ed.). F A Davis Company.

http://www.elsevieradvantage.com/samplechapters/9781437717099/9781437717099.pdf

How do they stay healthy anyway?

PREVENTATIVE THERAPIES:

   -vaccinations: influenza and pneumococcal: reduce viral infections
   -unfortunately... set limitations for proximity to other CF patients reduces exchange of infection



TREATMENT RECOMMENDATIONS:

1. CHEST PHYSIOTHERAPY:

COUGH IT OUT!

there are different methods of shaking up the chest walls to break free that THICK, STICKY MUCUS from the chest walls

-Chest percussion and drainage: physical slapping on with hands on different parts of the chest

Details from Cystic Fibrosis Foundation: You can Percuss to!

-external percussion vest (does the slapping for you and makes you an incredible singer)

-airway oscillating devices

if > 6 years old...

Other therapies:

NEWEST and COOLEST fighting at the CORE of the problem! 

Ivafactor:  (a CFTR modulator) 150 mg every 12 hours by mouth
              -restores function of the mutant CF protein at the base!
              -improves chloride movement
              -reduces pulmonary exacerbations
              -improves cough clearance!  (helps COUGH IT OUT!)

  **only works on specific mutations so genotyping is needed


Nebulizers:  inhaled medications

      mucolytics: Mucus busters
      hypertonic saline: draws water into airways to thin mucus and COUGH IT OUT!
      bronchodilators: open up airways before chest physiotherapy


Antibiotic Therapies:  dependent on the type of bacterial infection, give after Chest Physiotherapy

                    *popular antibiotics for popular infections

AZITHROMYCIN: an anti-inflammatory and antibiotic 
                            -research shows chronic usage improved chronic cough and lung function!

for P. aeruginosa bacteria
      -Tobramycin or gentamycin (aminoglycides) 
      -"Cillins": penicillin, amoxicillin, Cephalosporin, Carbopenams (beta-lactams) 

Intravenous antibiotics for treating exacerbations of cystic fibrosis 

A TABLE OF ANTIBIOTICS

ANTI-INFLAMMATORY:

chronic high does ibuprofen: new research proven helpful if CF lung function > 60%

      
Other choices for more severe CF:

-supplemental oxygen
-BiPAP: to help with difficulty expanding chest walls (positive pressure ventilation)
-Lung Transplant: contraindicated for patients < 12 y.o.
                            -none for infection with Burkholderia gladioli


MULTISYSTEM TREATMENTS:

may also need to treat for CF-related:
    -nutritional deficiencies: provide enzymes, tube feeding
    -diabetes: insulin management
    -osteoporosis: exercise or calcium supplements


References:

Cystic Fibrosis Foundation - Home. (n.d.). Retrieved February 14, 2015, from http://www.cff.org

Simon, R. (2014, December 18). Cystic fibrosis: Overview of the treatment of lung disease. Retrieved February 14, 2015, from http://www.uptodate.com/contents/cystic-fibrosis-overview-of-the-treatment-of-lung-disease?source=search_result&search=cystic fibrosis treatment&selectedTitle=1~150

Seattle Children's Hospital Policies and Procedures: Cystic Fibrosis Pathway

What to look for.. Signs and Symptoms

Signs vs. Symptoms

What do these mean?

Signs: what your doctor will find via examination or lab test results
Symtoms: what the patient feels


Presentation of CF in infancy:

*20% of CF infants or children have meconium ileum: baby's first poop blocks bowel

*45% of CF infants or children have

• respiratory symptoms: cough, wheezing difficulty breathing         
• respiratory signs: hyperinflation of lung fields,  obstructive airway disease determined from pulmonary function tests (PFTs)

*28% of CF infects or children have Failure to Thrive diagnosis: failure to meet growth milestones             despite adequate nutrition

Presentation of CF in adults (only 7% of patients with CF are diagnosed > 18)

• GI symptoms: failure to properly absorb nutrients due to abnormal enzyme production from pancreas
• diabetes mellitus: pancreatic insufficiency of insulin production (needed for glucose tolerance)
• infertility: defects in sperm transport or reduced fertility in women due to malnutrition or abnormal production of cervical mucus

Presentation if Pictures and Graphs:

BODY PARTS AFFECTED BY CF:

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SYSTEMS THROUGH THE AGES:

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OVERVIEW FROM A NURSING PERSPECTIVE: what to look out for as a healthcare professional