Pediatric Asthma and Cystic Fibrosis -Quizzes

non-invasive test used in assessment of CF, where there is placement of electrodes on the forearms and thighs of the patient, which will generate sweat. It is the primary test used to rule out CF. AND value ranges

Gibson Cook Qualification test - ANYTHING 60meq/L or HIGHER indicates CF- If the range is 45-50, repeat the test in ONE WEEK.

Peak Flow Meter Green Zone Yellow Zone Red Zone

Green >80% -Daily prevention meds -breathing good, no cough/wheeze, sleep through the night, can work and play. Yellow 50-79% -Add quick-relief med First signs of a cold, exposure to known trigger, cough, chest tightness, mild wheeze, coughing at night. Red <50% -Danger zone, get help from Dr. Medicine not helping, breathing hard and fast, nose wide open, ribs show, can't talk well. **Call Dr immediately or go to ER, follow up with HCP within 2 days of ER visit

PRIMARY nursing diagnosis for CF patients

Guilt, anxiety, fear, anticipatory grieving

CF Diet

High fat (4000-5000 calories a day Total)

Goals/Outcomes for CF Pts

- Minimize respiratory complications - Provide adequate nutrients for growth - No cure, just management (Lung transplant a possible option) - Educate the family - Chest Pt Jacket, Postural Drainage & Nebulizer - Exercise, Diet & Pancreatic Enzymes - Protection from infections

Progressing Respiratory signs of CF

- Moist Crackles, Wheezing- Distended BARREL Chest/ Atelectasis (Alveoli are over inflated) <--Worst case scenario when children get older- Decreased O2 Sat- Hypoxia- Hypercapnea (Too Much CO2)- Acidosis

Respiratory signs of CF

-Dry, nonproductive cough -Frequent resp infections -Culture of secretions that have Pseudomonas

Asthma med classifications

-ICS, oral steroid -Short acting Beta 2 agonist (albuterol) -Anti IgE

A nurse is caring for a 17 year old female client with cystic fibrosis who has been admitted to the hospital to receive I.V. antibiotics and respiratory treatment for exacerbation of a lung infection. The client questions her future and the consequences of the disease. Which statements about the course of cystic fibrosis are true? (Select all that apply) 1 - Breast development is commonly delayed 2 - The client is at risk for developing diabetes 3 - Pregnancy and child bearing aren't affected 4 - Normal sexual relationships can be expected 5 - only males carry the gene for the disease 6 - By age 20, the client can probably decrease the frequency of respiratory treatment.

1 - Breast development is commonly delayed 2 - The client is at risk for developing diabetes 4 - Normal sexual relationships can be expected

Breathing exercises for CF patients

NOT incentive spirometry. Blowing bubbles, blow into a bag, homemade devices

These are SECONDARY nursing diagnosis related to CF

Airway: impaired gas exchange, ineffective airway clearance, ineffective breathing patterns

Common lab abnormalities with CF patients

Hyponatremia Hypochloremia Hypoalbuminea

This is the most consistent pathology in Cystic Fibrosis patients. It is considered a Gold Standard for definitive diagnosis of CF.

Abnormal Sweat Chloride Test A test is performed, which analyzes sodium and chloride levels in sweat. A chloride concentration of 60meq/L or greater is a diagnosis of CF.

This organ affected by CF is the most serious threat to life.

Lung problems. Thick, sticky secretions pool here and can cause atelectasis (collapse) and serve as a median for bacterial growth. - Thick mucus can lead to obstruction of the AIRWAY and cause INFECTION

These are prescribed for respiratory infections

Antibiotics

Contraindicated treatment in patients with CF

Antihistamines -Have a drying effect and make mucus more thick and harder to expel

Results from failure to break down nitrogen compounds

Azotorrhea (excessive nitrogen in feces or urine). Commonly caused by high protein diets

Supplement that is high cal and helps digest nutrients.

MCT Oil It is drawn up in a syringe and added to formula. It is very thick and will most likely clog the syringe.

Used in infants to help liquify secretions

Mist Tent

This will check out the outline of the lower intestine, looking for the presence of meconium ileus & stool obstructions.

Barium Enema

Most important intervention for management of respiratory complications with CF

Chest Physiotherapy. Performed as frequently as possible

This diagnostic test will show patches of lung disease and early signs of obstruction and atelectasis.

Chest X-Ray. Pulmonary Function Test also important

This is a multi-system disorder of the exocrine glands, leading to an increased production of thick mucus in the bronchioles, small intestines, and pancreatic and bile ducts. It effects the lungs, sweat glands, and can also be found in reproductive tissue.

Cystic Fibrosis Increased viscosity of secretions obstructs small passage ways of these organs and interferes with normal pulmonary and digestive function. - This can cause a delay in puberty for females and sterility in males

Recommended formula for infants with CF due to high calorie and protein content

Enfamil

Side effects of bronchodilators

Excitatory side effects. Jittery, tachycardia, restless, dry mouth, palpitations, headache

Vital vitamins to be replaced in CF

Fat soluble vitamins (lost in the GI tract) ADEK

Organ that exocrine glands affected by CF

Liver Enzyme studies important diagnostic tool.

Thick secretions clog these and cause absorption issues

Pancreatic ducts become clogged with secretions and prevents pancreatic enzymes from reaching duodenum, impairing digestion and absorption of fats and proteins.

Intervention for malabsorption issues

Pancreatic enzymes on food (young children) or capsules PO at mealtimes. If child is NPO do NOT give the enzymes

CF Pt experiencing tachypnea, spitting up blood, cyanosis, pallor, tachycardia.

Pneumothorax... can lead to resp failure

S&S of acute infection in Pts with CF (pneumothorax vs pneumonia)

Pneumothorax: SOB, dyspnea, coughing up blood, tachypnea, tachycardia, cyanosis, pallor.

Which test result is a key finding in a child with CF? 1. Chest X Ray 2. Neck X ray showing areas of upper airway narrowing 3. Lateral neck X ray revealing enlarged epiglottis 4. Positive pilocarpine iontophoresis sweat test

Positive pilocarpine iontophoresis sweat test

Primary respiratory med used in management of CF

Pulmozyme and Dornase Bronchodilators. Nebulizer that decrease viscosity of mucus, may cause voice alterations

Consequence of heavy-like stools related to GI problems in CF patients

Rectal prolapse

Enzymes must be given at X temperature

Room temp or they will be denatured.

S&S of asthma

SOB, chest tightness, wheezing on exhalation, coughing. Worsened by a trigger. Infants may appear failure to thrive due to unmanaged asthma and difficulty feeding

This will appear in patients with CF, often first noticed when the mother kisses the infant on the forehead.

Salty skin Mothers notice when they kiss baby's forehead

This area of the intestines, in the absence of pancreatic enzymes, is unable to absorb proteins and fats, stunting growth and puberty. The malabsorption results in excess fat and protein in the stool.

Small intestines

Do NOT encourage this in terms of diet and nutrition of CF patients.

Soy Milk

Signs asthma therapy is working

SpO2>90%

The 4 F's

Stool of CF patients Foul smelling, Fatty, Frothy, Floating.

CF patients at risk for developing what chronic disease?

Type 2 Diabetes Increase insulin

Dietary recommendations for CF Pts

Varies based on Pt. High Calorie, High Protein, normal to high fat.

GI S&S with CF

abdominal distention, dehydration, weight loss. Results from nutrients being lost in GI tract due to malabsorption. Tissue wasting, appear to have failure to thrive. Nasal polyps (common in CF) impair ability to smell and eat effectively.

Important CF intervention

chest physiotherapy/percussion

CF Management in warm, summer months

increase Na and H2O

Physical appearance of CF patients

malnourished, decreased weight

Earliest sign of CF in newborns

meconium ileus. Meconium doe not pass and impacts/blocks the large intestines.