6.6 Cystic Fibrosis and Asthma Flashcards
Cystic Fibrosis
- Genetic respiratory condition that is tested for in utero and in newborn screening
- Characterized by abnormally thick and sticky mucous (exocrine gland defect)
Affects
- Bronchial obstruction
- Small intestines
- Pancreatic ducts
- Bile ducts
Causes
- Pneumonia
- Obstructive Emphysema
- Thickened Meconium (in small intestines. First meconium may be delayed due to this)
- Intestinal obstructions
- Pancreatic degeneration
- Absence of gastric secretions
- Difficulty with absorption
- Bile ducts can cause portal hypertension and etc
Four F’s of Stools
- Sticky mucous in GI tract prevents them from absorbing fats and fat-soluble vitamins
This causes stools to look
- Frothy
- Foul smelling
- Contains fat
- FLOATS
Diagnosis of Cystic Fibrosis
Sweat Test
- Stimulate sweating on child’s forearm and test the sweat for levels of CHLORIDE.
- 60+ chloride is considered positive
- This is because kids with cystic fibrosis have altered chloride channels so they expel increased amounts of chloride on their skin. (skin may taste salty)
Symptoms of Cystic Fibrosis
- Chronic Cough
- Lung Infection
- Clubbing and Barrel Chest
- GI Issues (abdominal distension, fatty stools, meconium delays, struggle to absorb fat)
- These children require high fat, protein, calorie diet.
- These children have decreased digestive enzymes so artificial enzymes in capsules before each meal will help them breakdown fat in their food and absorb nutrients.
- We also need to supplement fat soluble vitamins (A,D,E,K) with artificial vitamins.
Cystic Fibrosis and Pneumonia
- Commonly admitted for broncho-pneumonia due to thick secretions
- Most common cause of hospitalization of cystic fibrosis
- They come to hospital for “tune up” for 14-21 days of antibiotic treatment
- We also need to make sure to supplement their diet such as artificial enzymes.
- We also need to supplement them with sufficient salt because they expel a lot of salt on their skin (hyponatremia).
Genetics of Cystic Fibrosis
- Autosomal Recessive
(If both mom and dad have the gene, there is a 25% chance of development)
(If one parent is not a carrier, child has 0% chance of getting it)
Asthma
- Chronic inflammatory disease of airway
- Characterized by airway obstruction, bronchial hyperresponsiveness, inflammation.
- Happens via triggers and hyperresponsive reactions (inflammation)
- Reversible
HIGH RISK
- Minority groups or Lower Socioeconomic Class due to increased risk of trigger exposure
- Asthma is called reactive airway disease in infant and toddlers. Asthma is not diagnosed until age 3-5
Pathology of Asthma
- Allergic hypersensitivity to some sort of allergen or irritant which causes hyperresponsive reaction from trachea and bronchi and develops inflammation
- Triggers can be airborne, exercise, stress, infection, tobacco smoke, viral infections (for infants).
Clinical Manifestations of Asthma
- WHEEZING
- Restlessness during active asthma attack due to lack of air
- Non-productive cough (dry-cough)
- Increased work of breathing
Goals for Asthma Patients
- Relief of symptoms and reduction in frequency and severity of asthma attacks.
- We want them to experience as normal of a life as possible by controlling triggers and balance of medication management
COMMON ALLERGENS
- Dust, roaches, smoke, cold/infections, weather changes, strong odors, exercise, pets, pollen, mold.
Asthma Management
Exercise
- Encourage exercise but use inhaler before exercise and have inhaler with them.
Medications
- Medications should be kept at home and school (no matter where they go have an inhaler. This should be communicated to parents and school nurse)
Postural drainage and breathing exercises
- To promote proper breathing
- Avoid triggers (signs and symptoms, medications, triggers)
Pulmonary Function Tests (PFTs)
- Helps determine severity of asthma
- Objective way to evaluate presence of lung disease and significance of asthma
- Spirometry
During an Asthma Attack
- If it is not relieved with medications they will need to be hospitalized
Interventions
- Oxygen (check pulse oximetry)
- Albuterol (every couple of hours)
- Systemic corticosteroids
- Comfortable breathing
- Promote normal breathing as much as possible
Categories of Asthma
Mild Intermittent
- Asthma symptoms less than 2 days a week and does not interfere with daily function. Medication less than 2 times a day
Mild Persistent
- More than twice a week but less than everyday. Night time symptoms a few times a month. Minorly limits daily activity. Medication more than 2 times a week but less than everyday
Moderate Persistent
- Daily symptoms and weekly nighttime symptoms and mildly limits daily activity. Medication everyday.
Severe Persistent
- Continual symptoms everyday, frequent nighttime symptoms, extremely limited activity. Medications are used multiple times a day.
Asthma Medications
Long-term medications
- Corticosteroids (first line)
- Suppress inflammation that trigger airways to narrow. Inhaled steroids are the most common (Flovent, Pulmicort)
- Important to have rescue medications on hand just incase (don’t want airway to be trigged by this medication especially the first few times taking the medication)
- These make airway less sensitive so you are less likely to be triggered
Quick Relief (rescue) medications
- Bronchodilators
- Help during acute episodes of wheezing.
- Beta-2 antagonist are the most common such as Albuterol.
