Asthma and Cystic Fibrosis
Chronic Illnesses in Children
ASTHMA
 Why study Asthma?
– The most common chronic disease of childhood
– The primary cause of school absences
– Is responsible for a major proportion of pediatric
admissions to emergency departments and hospitals
– Healthcare alone for asthma cost the nation $6.2
billion in 1990, with hospitalization accounting for
about $1.6 billion of that figure. Prevalence has
increased by 29% in last decade.
– CDC Statistics re: current amounts
– Nearly 5,000 Americans die each year from asthma
Definition
 A diffuse, pulmonary disease characterized by
1.)Airway inflammation
2.)Airway hyperreactivity
manifested by difficulty breathing resulting
from generalized narrowing of the airways.
Triggers tending to precipitate &/or
aggravate asthmatic exacerbations.
 Allergens--
Outdoors: trees, shrubs, weeds,





grasses, molds, pollens, air
pollution, spores
Indoors: dust &/or mites,mold,
cockroach antigen
Irritants– tobacco smoke, wood
smoke, odors, sprays
Exposure to occupational chemicals
Exercise
Cold air
Changes in weather or temperature
 Colds & infections
 Environmental change: moving
to new home, starting new
school, etc.
 Animals: cats, dogs, rodents,
horses
 Medications: ASA, NSAID’s,
Antibiotics, beta blockers
 Strong emotions: fear, anger,
laughing, crying
 Conditions: GE reflux, TEF
 Food additives: sulfite
preservatives
 Foods: nuts, milk/dairy products
 Endocrine factors: menses,
pregnancy, thyroid disease
Asthma Severity Classification in Children
0-11 yrs (Nat’l Asthma Education & Prevention Program)
Severe Persistent
Moderate Persistent
Mild Persistent
Intermittent
(see Box 32-16, p. 1264 in Hockenberry, 9th
ed, 2011)
Steps in progression of Asthma-See Fig. 32-9 and 32-10 pp.1266-7; 9th ed. Hockenberry.
 First, stimuli activate the release of
inflammatory mediators from mast cells,
macrophages, eosinophils, & other
inflammatory cells within the airways.
 Next, inflammatory mediators signal other
inflammatory cells to migrate into the airways
and to become activated.
 This leads to epithelial injury, increased smooth
muscle contraction & mucus secretion,
swelling, & changes in the parasympathetic
control of airway function.
Steps in progression of Asthma(cont’d)
 Airways become more narrowed & obstructed.
 Inflammatory processes lead to airway
hyperresponsiveness.
 Airway obstruction or narrowing causes the sx
of coughing, wheezing, chest tightness,
shortness of breath, & decreased endurance.
Can be abrupt in onset or gradual.
Major Symptoms & Associated Features
 Major Symptoms
*Wheezing- especially
on expiration
*Tachypnea
*Cough- harsh, often
non-productive
*Retractions-sub or
intracostal, suprasternal,
or supraclavicular
*Use of accessory muscles
*Gas-trapping dyspnea
*Respiratory Acidosis r/t
retention of CO2 & over
inflation of lungs
 Associated Features
*Rhinitis
*Hx of Bronchitis
* Eczema
* Increased AP diameter
* Elevated shoulders
*Cyanosis
* Assuming “tripod”
position to maximize
oxygenation
*See “Interpreting Peak
Expiratory Flow Rates “ p1336
Diagnostic Testing
 Hx & physical findings
 Pulmonary Function Tests (PFT): lots of different ones.





*PEFR: greatest flow velocity during a one second forced
expiration.
KNOW significance of Red, Yellow, & Green Zones
Skin testing: scratch skin to detect allergenicity
Provocative testing: inhalation of allergen to detect
allergenicity
RAST(Radioallergosorbant) Test: blood test that detects
seasonal or environmental allergies
Blood tests: CBC may help determine etiology of Asthma
exacerbation by detecting infectious process, or
Eosinophelia
Chest x-ray
Treatment:
Goal is to PREVENT ASTHMA ATTACKS
 Allergen control in the home & environment
 Chest physiotherapy: breathing exercises, physical
training, & inhalation therapy to make breathing
more efficient
 Hyposensitization: “Allergy Shots” to create an
acquired immunity by desensitization.
 Exercise: important to maximize lung function. May
need to be medicated pre-exercise to facilitate
success. Swimming or musical instruments that
require breathing are all good.
 Medications
Status Asthmaticus
 Considered a medical emergency that can result in
respiratory failure and death if untreated.
 Humidified O2 is administered via NC, hood, or mask
to keep SaO2 > 90%
 If no PEFR obtainable, give epinephrine subq
0.01ml/kg/dose of 1:1000 epinephrine with a
maximum dose of 0.3ml
 If PEFR obtainable, give 3 tx of beta-2 agonists(
Albuterol 0.15mg/kg/dose)neb tx spaced 20-30 min.
apart
 Systemic corticosteroids are also administered IV–
SoluMedrol 1mg/kg/dose q 6hr is often recommended
initially.
Status Asthmaticus (Cont’d)
 Theophylline/Aminophylline is avoided in ER
 IV fluids are given to rehydrate at maintenance rates to
avoid pulmonary edema.
 Antibiotics are administered for bacterial infections or to
prevent 2ndary infections with corticosteroid use.
 Therapy is directed toward correction of
dehydration, & respiratory acidosis, electrolyte
disturbance, improvement of ventilation, & tx
of any concurrent infection.
Anti-inflammatory Meds
 Corticosteroids (See Handout "B")
– Methylprednisolone (SoluMedrol) IV
– Pediapred/Orapred/Prednisone.) PO
– Budesonide Inhaler (Pulmicort turbulaler)
•
•
•
•
•

Action
Dose
Side effects
Principles of use
Inhaled Corticosteroids (ICS) are the tx of choice for longterm management~ less systemic side effects
Cromolyn Sodium (Intal) / Nedocromil —no longer recommended.
Bronchodilators
 Sympathomimetic
– Epinephrine
• Dose—0.01ml/kg of 1:1000 solution with a
maximum dose of 0.3ml
• Use—only in emergencies
• Side effects—tachycardia, palpitations
Bronchodilators (cont’d)
 Beta-adrenergic agonists
–
–
–
–
Albuterol (Ventolin, Proventil)
Levalbuterol (Xopenex)
Metaproterenol (Alupent, Metaprel)
Terbutaline (Brethine)
•
•
•
•
Routes of administration
Action
Side effects
Used for Exercise-induced bronchospasm (EIB)
MDI—metered-dose-inhaler with spacer
•Children will often be encouraged to use a spacer to
increase the effectiveness of the inhaler.
•Patient video on MDI
Methylxanthines
Primarily, Theophylline
– Now used as a 3rd line of defense
– Weaker bronchodilator
• Can be given IV, IM, PO, PR
– Potential for serious side effects if outside of
therapeutic serum levels: 5-15mcg/ml.
– Theophylline toxicity: >20mcg/ml
• Nausea, tachycardia, irritability, distractibility,
hypotension
Anticholinergics
Oldest form of bronchodilator
–
–
–
–
Action
Used for relief of acute bronchospasm
Atropine and ipratropium (most common)
Website with info on Atrovent inhaler
Leukotriene Modifiers
 Block the potent inflammatory mediators
called leukotrienes
– Montelukast (Singulair)~ ok for children 6yrs and >
– Zafirlukast (Accolate)~ only one safe in pregnancy
– Zileuton (Zyflo)
 All these are given PO once/day, may be used as an
alternative to low-dose ICS for mild persistent asthma
or an addition to ICS for moderate persistent asthma
 NOT for treatment of acute exacerbation of asthma.
Heliox
 Used rarely
 When children don’t respond to other treatment and
there is difficulty with ventilation, a combination of
Helium and Oxygen may be used
 CO2 diffuses more readily in Helium
 Administered through a non-rebreathing face mask
See Nursing Care Plan
Hockenberry, 9th ed.( pp.1274-1276) (case study)
Good review of primary Nursing
Diagnoses, Pt. goals, and interventions
Website with patient-friendly info on
asthma, also available in Spanish.
National Heart, Lung, and Blood
Institute of the NIH website
Asthma Action Plan from the American
Lung Association is a teaching tool for
patients and providers.
Cystic Fibrosis
Definition
A disease transmitted by an autosomal
recessive trait whose affected gene leads to
EXOCRINE GLAND DYSFUNCTION.
Exocrine glands are mucus-secreting
glands, so this disease affects any organ
that has any exocrine glands
75% of all mutations are the F508
alteration, but there are >1000 different
mutations that make the symptoms and
severity unique for each individual
Etiology
 ~25,000 affected individuals in US
 Median life expectancy is 33 years (range= 0-70)
 Autosomal recessive trait therefore, both parents
must carry the gene, but may not be affected by
the disease.
– Risk of transmission: 1 in 4 of passing on disease,
50% will carry trait, 25% unaffected.
The defective gene was discovered only in 1989 on
the long arm of the chromosome 7, along with its
protein product, cystic fibrosis transmembrane
regulator (CFTR)
Pathology
CFTR (the abnormal protein in CF) found
in the exocrine glands, causes increased
viscosity of mucous gland secretions,
causing mechanical obstruction in the
–
–
–
–
–
Lungs
Pancreas
Intestines
Bile ducts
Genital tracts
Pathology (cont’d)
Loss of Sodium and Chloride in excess
amounts through perspiration (sweat
glands), puts person at risk for electrolyte
imbalance especially in the summer with
increased perspiration.
Symptoms R/T pathophysiology
Pancreatic duct obstruction
– Blockage impedes enzymes:
• Lipase, trypsin, amylase
– Enzymes don’t reach duodenum, thus impairing
digestion and absorption of fats, proteins, and fatsoluble vitamins.
– Symptoms include:
• Steatorrhea
• Azotorrhea
• Bulky, foul-smelling, frothy stools, 2-3 times the
normal amount
• Excessive flatus
*Respiratory Obstruction
Upper respiratory tract
– Nasal polyps, chronic sinusitis
Lower respiratory tract
– Thick, sticky mucus with reduced mucus
clearance→
• Early airway inflammation→
• Recurrent bacterial infections and colonization
with bacterial pathogens→
• Airway obstruction with air trapping and reduced
elasticity→ barrel-chest, clubbing of fingers &
toes
• Bronchiectasis with lung destruction
Complications in the respiratory tract
 Pansinusitis
 Resistant pulmonary infections~ e.g.
Pseudomonas aeruginosa~common cause of
pneumonia ( website )
 Allergic Bronchopulmonary Aspergillosis
(ABPA)
 Hemoptysis
 Pneumothorax
 Pulmonary HTN leading to cor pulmonale
Bile duct obstruction
May cause biliary cirrhosis of the liver
Portal hypertension
Focal biliary fibrosis
Hypoprothrombinemia
Prevalence increases with age
Intestinal obstruction
Meconium ileus—seen on newborns if no
stool passed for 24-48 hrs after birth
Prolonged neonatal jaundice
Poor absorption →failure to thrive, slowed
weight gain
Distal intestinal obstructive syndrome
Rectal prolapse
Complications of the GI tract
 Intussusception
 Cholecystitis/cholelithiasis
 Pancreatitis
 Glucose intolerance & CF related diabetes (30%)
– Non-ketotic, hypoinsulinemic (not like type 1 or 2)
– Insidious onset…slow decline in insulin secretion
– Prevalence increases with age.
Genital tract obstruction
 Azoospermia in 98% of mailes
– Congenital bilateral absence of vas deferens
– Abnormal development of vas deferens
 Higher incidence of hydrocele and inguinal
hernia
 Increase in female infertility
–
–
–
–
Thickened cervical & vaginal secretions
Irregular ovulation from chronic illness/poor nutrition
Delayed puberty in both genders
Probably due to chronic lung disease and decreased
nutritional status (lower body fat composition)
Other clinical manifestations
Abnormality in sweat composition
– Hyponatremia
– Easily provoked dehydration
– Heat stroke
CF arthralgias and arthritis
Diagnosis
70% are diagnosed by age 1 year, however
there are still a fair number diagnosed later
in childhood or adulthood.
“Gold Standard” for diagnosis:
– Sweat Test —also known as pilocarpine test. It
measures for abnormal chloride content in
sweat secretions.
– Normal level= <40mEq/L
CF level->60mEq/L
Diagnosis (cont’d)
Other methods
– Genotyping of individual (blood or buccal smear)
– Prenatal genotyping (offered to all pregnant
couples by law) to detect gene F508
Absence of pancreatic enzymes—amylase,
trypsin, and lipase
– Stool analysis reveals high fat stools
Newborn screening
– Tests for elevated trypsinogen in immediate
postnatal period. Not diagnostic—must be
confirmed by one of the other methods
Current Management
Health maintenance
– Quarterly visits to care center
– Interdisciplinary team care
• MD—directs medical management & guides team
• Respiratory care—PFT’s, airway clearance, chest
physiotherapy
• Nutrition—goal of normal growth across lifespan
• Social work—family coping, insurance, disability
• Nursing—daily management, interface with school
– Surveillance for complications: i.e.annual
CBC, glucose chemistry, liver function, &
quarterly sputum cultures
Current Management
Health maintenance: changes in past 10 yrs
– Increased focus on nutrition—increasing
evidence for association between nutritional
status and lung health.
– Improvement in pancreatic enzyme
replacement
– Early recognition and treatment of
exacerbations
– Participation in clinical trials using a new
drug, Kalydeco, an oral med with great
promise
Current Management
Diet: high protein, high calorie diet, lower fat
foods, salt supplements in hot weather,
water-soluble forms of fat-soluble vitamins
A,D, E, and K given daily
– Usually have 3 meals + 3 snacks/day
– Some children have g-button and get hooked up
to supplemental feedings from 1900-0700 to
increase caloric intake.
Current Management
Pancreatic enzyme replacement is essential
– Take with meals and snacks
– Capsules can be taken whole or sprinkled on
food, but cannot be chewed or crushed as
they are enteric-coated enzymes that must get
to the duodenum to be effective
– # of capsules depends on amount of food
intake.
Current Management--video
Respiratory Therapy
– Chest physiotherapy (CPT)—is done on a
daily basis BID by parents when child is
healthy, QID by RT when hospitalized
• Schedule in AM upon waking, mid-morning, midafternoon, and at hs. Try to avoid meal time
• Vest System: review by Aetna Ins
• Case study demonstrating Pulmozyme neb tx and
“airway clearance therapy” with vest in cystic
fibrosis.
Current Management
 Nebulizer treatments & other inhaled meds
– Recombinant human deoxyribonuclease known
generically as dornase alpha or Pulmozyme, actually
breaks down thickened mucus secretions. Used on a
regular basis, this med has significantly assisted in
decreasing the number of pulmonary infections and
exacerbations requiring hospitalizations
– Beta-adrenergic agonists are also integrated into
daily plan of care, especially during exacerbation.
– Inhaled antibiotics are another new preventative tx:
• TOBI-Tobramycin solution for inhalation especially for
pseudomonas aeroginosa lung exacerbations w/ CF.
Current Management
Exercise: stimulates mucus secretion,
enhances pulmonary function and can be as
effective as CPT if done on a regular basis.
Encourage children to participate in any
aerobic activity to enhance their selfesteem and sense of well-being.
Current Management
 Antibiotic therapy:
– Besides inhaled TOBI, children may be placed on low
dose antibiotics to help prevent infections.
• Azithromycin may be used in low doses for its anti-infective
effect but also because it has a strong anti-inflammatory
effect.
– IV antibiotics are the drugs of choice when a child has
a pneumonia exacerbation. After sputum culture,
sensitive antibiotics are ordered round the clock to
aggressively treat the infection
• Ticarcillin, tobramycin, or gentamycin, pipercillin
• All are the “big guns” that can cause phlebitis and need to be
monitored closely.
• Many CF kids have Mediports for easy access when IV
antibiotics need to be adminisitered on a frequent basis.
Current Management
Lung Transplant —may give a
child/young adult a new lease on life.
Usually reserved as a last resort
Nursing Care
 Infection Control
– Sputum culture when symptoms present
– Contact Isolation until cultures are confirmed and
then as deemed necessary– organisms are spread by
contact with secretions/sputum and are not airborne.
– Careful handwashing essential—a nosocomial viral
infection for a CF child can be devastating!
 Appropriate antibiotic therapy
 Respiratory Tx and CPT integrated into care
Nursing Care
Focus also on nutritional support
– Increase caloric needs with illness via TPN,
g-tube feedings overnight, frequent high
calorie snacks e.g. Ensure
– Daily weight
– Monitor energy expenditure
– Give enzyme capsules with meals and g-tube
feedings as ordered.
Nursing Care
 Emotional Support to child and family
 Involvement with Cystic Fibrosis Foundation as a
support group and source of financial assistance
as well.
 Remember that despite many significant
advances in diagnosis and treatment, CF still
takes the life of many young people despite the
best of care.
 Supporting the family is critical at the time of
diagnosis, during illnesses, and at end stage of
the disease.
Cystic Fibrosis
A website with good patient education
and research on this disease:
http://www.nlm.nih.gov/medlineplus/cy
sticfibrosis.html
Here’s another website to the Cystic
Fibrosis Foundation that offers lots of
links.
http://www.cff.org
 Life expectancy for CF
That’s all folks!