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Exocrine Gland Dysfunction Mucous secretions are thick and tenacious • Dysfunction of mucous producing glands leads to
multiple gastrointestinal absorption problems. • Blocked pancreatic ducts • No secretion of digestive enzymes
• Meconium ileus at birth • Failure to thrive • Steatorrhea stools / constipation • Voracious appetite with poor weight gain • Recurrent respiratory infections • Chronic cough • Malabsorption of intestines
• Positive sweat test • Genetic marker • Life long management
– Enzyme replacement with eating – Daily CPT postural drainage – Inhaled bronchodilators – Control of lung infections – Nutritional supplements as needed
• Enzymes to help digest food • Antibiotics to control infection • Bronchodilators to open airways • Vitamin C to improve absorption of other
meds • Vitamins E, A, D, K / fat soluble vitamins
Long Term Complications
• Nasal polyps • Sinusitis • Rectal polyps / prolapse • Hyperglycemia / diabetes • infertility
* Life span approximately 30 years of age
– Asthma is a chronic, inflammatory lung disease involving recurrent breathing problems.
– Caused by complex, multicellular reaction in the airway characterized by: Airway inflammation Airway hyper-responsiveness to a variety of triggers
* Asthma is the most common, chronic health problem among children.
• Wheezing • Cough • Tightness of chest • Prolonged expiratory phase • Hypoxemia • X-ray = hyper-expansion of lungs
• High fowlers position / bed rest • Pulse oximetry • Nebulized albuterol • CPT • Methylprednisone / Solu-medrol IV • IV fluids • Oxygen to keep oxygen sats > 95%
• Peak flow spirometer • Identify triggers • Maximize lung function • Optimal physical growth • Optimal psycho-social state • Maximum participation
Peak Flow Monitoring
Spirometry measures how much and how fast air is forcefully expelled from fully inflated lungs. Recommended standard of care for management of asthma.
• Rescue drugs: short acting albuterol beta 2 agonist – used as a quick-relief agent for acute bronchospasm and for prevention of exercise induced bronchospasm.
• Anti-inflammatory or preventative: low-dose inhaled corticosteroid: inhaled or oral prednisone
• Allergy: Singulair Docsity.com
• Bronchodilators rapidly relax the airway smooth muscle cells, thus reversing the bronchospasm until anti-inflammatory effect of steroids is attained. – Aerosols
• Via mouth piece 3 years and older • Via facial mask for less than 3 years
• Steroids reduce the inflammatory component of bronchial obstruction, decrease mucus production and mediator release, as well as the late phase (cellular) inflammatory process.
• Methyl prednisone IV in severe cases • May need Reglan if experiencing GI upset • PO prednisone – always give with food to decrease GI
• Oral prednisone (Pedia-pred, Prelone, Liquid pred) recommended for short course in moderate or severe exacerbation
• Inhaled: Pulmicort, AeroBid, Flovent – Infant: mask should fit firmly: cataracts – Older child: rinse and spit after treatment to
• Teach how to use medication • When to use and how often • No OTC drugs • Increase fluid intake • Signs and symptoms of respiratory distress
It occurs in newborns who are born prematurely and or have a variety of pulmonary disorders and who require ventilatory support with high pressure and oxygen in the first 2 weeks of life.
• Fibrosis of airways and marked hyperplasia of the bronchial epithelium
• Increased fluid in the lungs, as a result of disruption of the alveolar-capillary membrane
• Over distention due to damage to alveolar supporting structures resulting in air trapping
• Fibrosis, airway edema, and broncho-constriction
• Persistent respiratory distress • Dependent on supplemental oxygen • Failure to thrive • Gastro-esophageal reflux • Pulmonary hypertension
Long Term Management
• Supplemental oxygen • CPT • Bronchodilators • Diuretics (pulmonary hypertension) • Anti-inflammatory medication • Nutritional support: po formula + NG supplement • Gastrostomy tube (GER) • Bicarbonate in formula due to chronic state of acidosis
• Oxygen dependent • Visual problems • Feeding difficulties • Developmental delay • Learning difficulties