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Leading to..

Leading up to...

leads to...

Can be..

Facilitates...

In Addition

Leading up to

Cystic Fibrosis Concept Map

Ashton Tomlinson

Samantha Stewart

...The Pathophysiology

Management of Pulmonary Problems

Management of GI problems

  • Pancreatic enzyme replacement
  • High calorie, High protein diet
  • Necessary to meet growth needs
  • Breastfeeding parents need to give enzyme supplement
  • Formula-fed infants should be given cows milk based formula
  • Vitamins A, D, E and K should be given with enzymes
  • Uptake is decreased
  • Nighttime NG tube feedings
  • Given to patients with anorexia
  • Rectal prolapse treatment
  • Guiding the rectum back in place
  • Management focuses on preventing infection, improving ventilation, removing secretions and use of antimicrobials
  • Airway clearance therapies (focus on percussion, and high-frequency chest compression, PEP)
  • Huff coughing
  • Forced expiration to remove secretions from smaller airways so it can eventually be coughed up
  • Exercise is always a good thing!
  • Helps move mucus, increases blood flow to the lungs, and improves the self-esteem of the child

Characterized by:

  • Increased viscosity of secretions from the mucus glands
  • Elevation in the sweat electrolytes
  • Increase in enzymatic and organic constituents of saliva
  • Abnormalities of the central nervous system

The DEFECT in CF seems to be in abnormal chloride movement.

Management of Endocrine problems

  • Cystic Fibrosis related Diabetes is the main concern
  • Must be monitored closely
  • Need nutrition counseling for a proper diet
  • A1c measurements are done quarterly
  • Children should do their own blood glucose monitoring
  • CF exacerbation's can cause hyperglycemia
  • Poor bone growth with steroid use and pancreatic insufficiency

Where it all begins..

  • RESPIRATORY DISORDER
  • Inherited autosomal recessive trait
  • Mutated gene lies on chromosome 7
  • Mutated gene codes a protein "Cystic fibrosis transmembrane regulator (CFTR)"
  • CFTR responsible for transporting and ultimately has a role in mucus production

Pharmacologic Treatment

Broken into 3 sections :

The actual clinical manifestations we see..

1) Management of Pulmonary Problems (prevention and treatment of pulmonary infection)

  • GI manifestations...
  • Pulmonary manifestations
  • Initial: Wheezing, non-productive cough
  • Eventually: dyspnea, obstructive emphysema
  • Progressive: Barrel-chest, cyanosis, clubbing of fingers/toes, bronchitis

2) Management of GI problems (pancreatic insufficiency)

Looking further at the "clinical features"

3) Management of Endocrine problems (insulin resistance and insulin deficiency)

Nursing Interventions

  • Children with cystic fibrosis have an increase in both sodium and chloride in the saliva and sweat
  • Mechanical obstruction of organs from mucus glands secreting thick mucus
  • Usually see meconium ileus, pancreatic fibrosis, steatorrhea
  • Chance for diabetes is greater in patients with CF due to changes in the pancreas
  • Prolapse of the rectum
  • Obstruction of the bowel
  • Pulmonary manifestations seen in almost ALL children
  • Tissue destruction from bacteria formation
  • Atelectasis and hyperinflation
  • Reproductive system issues
  • Growth issues from poor nutrient absorption

Resources

-Assess pulmonary (focus on lung sounds, cough and fatigue)

**watch for any evidence of growth failure**

(weight loss, muscle wasting pallor, anorexia, decreased activity)

-Monitor pt tolerance and effectiveness of treatment

**stress treatment compliance

  • Hockenberry. W, Wilson. D, 2013, Wongs Essentials Of Pediatric Nursing

Holistic Interventions

Pulmonary Medications

GI Medications

Diagnosed by..

Endocrine Medications

IV antibiotics

-Tobramycin, ticarcillin and gentamicin

**2-3 antibiotics and 1 antifngal medication to treat coexisting pulmonary infections

-PICC may be used for home administration

Aerosol Therapy

~ Bronchodilator (opens bronchi for easier expectoration. admin before percussion and postural drainage when pt exhibits evidence of reactive airway disease or wheezing)

~ Human Deoxyribonuclease (decreases the viscosity of mucus)

~ Nebulized hypertonic saline (improves airway hydration and increases mucus clearance)

-Family support

~Recommend support groups

~Need to be very involved in care...educate about medications, equipment (PICC, ACT), diet, signs and symptoms of infections, and the importance of physical activity

-"Play Periods"

include PT

-Assess for signs of depression, anxiety and disturbed self image

-Flexible care plan so that child can have as normal a life as possible

-Provide O2 in acute situations

  • Newborn screening
  • Allows for early identification
  • Improved growth
  • Quantitative sweat chloride test
  • Chloride of greater than 60mEq/L is diagnostic of CF
  • Chest radiography
  • Shows atelectasis and emphysema
  • Stool analysis
  • Look for fat in the stool

-Administer pancreatic enzymes as prescribed 30 min within eating

(to ensure that digestive enzymes are mixed with food in the duodenum)

-Vitamin A, D, E, K

(due to inadequate uptake of fat-soluble vitamins. growth failure despite adequate nutritional support may indicate deterioration of pulmonary status. )

-Polyethylene-glycol electrolyte solution (GoLYTELY) via NG tube for constipation as prescribed

-Histamine-receptor antagonist and motility medications for GERD

- TPN

USE CAUTIOUSLY -- CF pts have chronic CO2 retention

- administer insulin PRN

pt present with a combination of insulin resistance and insulin deficiency with unstable glucose homeostasis in the presence of acute lung infection and treatment.

**may be at increased risk for glucose management problems as a result of decreased nutrient absorption, anorexia, and severity of pulmonary illness.

***with repeated infection and inflammation, bronchial cysts and emphysema may develop. If the cysts rupture --> pneumothorax

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