Transcript Week 5 Classroom focus - University of Windsor

Week 5 Classroom focus
Toddlers &
•
•
•
•
•
Lead Poisoning, Pica
Drowning
Anaphylaxis
Asthma
Viral Respiratory
Conditions
• Bacterial respiratory
Conditions
• Poisonings- vapours,
oil, petroleum, ASA
& meds, etc.
• Genetic AnomaliesCF
Resources used
•
•
•
•
Sources from Hockenberry et al 2007
Wong Essentials of Pediatric Nursing, 2001
Mosby’s Electronic Image File Collection
Ppte presentation by K. Fryer and P.
Durocher 2007 & 2008, 63-274
• Pptes from Partners in the Collaborative
B.Sc.N. Programme, University of Windsor,
63-277
• Notes of Dr. S. McMahon 63-373 and 277
• Assorted websites as indicated on slides
Respiratory Variations for
Infants and Toddlers
• Neck lengthening
• Trachea longer
• Glottis co-ordinated &
voluntary control
• Cough and swallow
reflexes present
• Bifurcation of bronchi
approximates clavicle
• Sternum shorter and
softer
•
contd.
Stomach and heart take up space in chest
Ribs more pliable – can create retractions
Diaphragm anchored higher in posterior
Abdominal muscles assist in laboured breathing
Location of Lobes of Lungs:
note that in infants and young children,
these are placed higher in the thorax
• CautionDon’t listen
Too low- you may
hear stomach or
bowel Sounds;
Too high???
Is of little use eitherPractice &
use landmarks
Gentle percussion can yield data.
Visualize what’s beneath
Infants and toddlers may breathe with
visible effort and movement.
• The older the child,
the less evidence of
assistance from other
muscle groups to
breathe.
Need to inspect A P &
lateral .
Expiration is usually
passive
Types of Retractions
• Suprasternal
• Clavicular
• Substernal
• Intercostal
• Subcostal
Look p. 1250 & Link to lung sounds
Paediatric Respiratory
chemistry influenced by
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
Haemoglobin- mature or foetal types, anemia
Body core temperature
Hydration, dehydration, clotting
Toxins
Air Particulates, Pollutants and heavy metals
Acid-base balance
Medications
Fear and stress
O2, Pco2 ratio & tissue uptake( hypoxia)
Carbonic acid, Na bicarb levels
Calcium, magnesium, copper
Lighting, radiant warmers
Obstructive lung conditions, hypoventilation
Diabetes. Insulin, glucose
Altitude and atmospheric pressure
Some toddlers are considered medically fragile as
they must rely on oxygenation via respirator, with
humidification, and protection from infections.
Nurses support families as they provide total care at
home while creating a developmentally rich
environment for their child.
The respiratory system
must include:
• Mouth, ears, nose, & throat &
Developmental stage and
Environment
• Consider: Latex and other allergies- anaphylaxis
• Variations in young children before and after 3
years of age- Dentition, eustachian tube angles,
Swallowing, cough reflex, Sinuses, lymph glands
/ adenoids
• Safety : mobility, curiosity, Use of soother,
fingers, tooth brushing, hand washing
• Normative hand to mouth & taste- smell-mouthtesting behaviours .Colour & Smell of things
attract children
• Learn YUCKY tastes and smells- link to symbols
Positioning of the eustachian tube
requires external adjustment for
visualization.
The Nose knows!
•
•
•
•
•
•
•
•
•
•
•
Able to detect
mother’s scent
from birth.
Can tell when
feeding
prepared.
Have preferences.
Alai Nasi flare
when in distress
Different EENT in
genetic variations
Looking at the neck is difficult in infants.
Toddlers have distinct structures that are palpable ,
but still not visible.
Cross section of larynx and
placement of epiglottis
Normal
Inflamed &
Obstructed
Tonsils are lymphoid tissue.
Functions relate to filtering and immunity.
Enlargement and hypertrophy occur when active.
Surgical removal when health growth and development are
negatively impacted- hearing loss, sleeplessness, dental
malocclusion, anorexia, fevers, on repeated courses of
antibiotics and anti virals.
Close examination of the mouth must be
done with extreme care to prevent
laryngospasm, airway occlusion, vomiting.
Look up nurse alerts
No tongue blades used in practice.
Tracheotomy tray must be at bedside.
Foreign bodies can be found anywhere.
Anything smaller than the diameter of a
toilet roll can become aspirated.
Food, candies, toys, pills, buttons, batteries etc.
Children with upper airway obstruction need
a “trach”. May be permanent or temporary.
Nose drops must be given correctly or they drain
into the oral cavity and are swallowed. Need to
have head-low position to drain into the sinuses
and inner ear.
• Look up and review your skill .
DROWNING
WATER SAFETY
Drownproofing
Water Safety ? Drowning?
•
•
•
•
•
•
•
Not in my backyard!
We have gates.
My child can bath himself.
I’m right close by.
My child knows how to swim.
What good are those floaties?
We don’t swim in our
culture.
There is only a MINUTE between a lifetime
and a lifetime of regret !
Tubs, pools, ditches, boats,
reservoirs, ponds, lakes, rivers
•
•
•
•
•
•
•
Drowning
Age inquisitive, explore &
unawareness of danger
Accidental death ranks 2nd for
boys & 3rd for girls ages 1-4 years
little ∆ despite prevention
education
bathtubs, pools, ditches, hot tubs,
ponds, lakes etc
Near-drowning one of leading
causes of vegetative state in
young children
Can drown in as little as 6” of
water
http://www.childrendrowningprev
ention.com/index.html
Pathophysiology of
drowning
• Most organ systems affected
• Extent of damage affected by factors
such as anoxia and asphyxia
• Cardiopulmonary arrest after 4-6
min
• Problems: hypoxia, aspiration,
hypothermia , infection
• All children should be hospitalized for
12-48 hrs even in near-drowning
Path cont’d
• Laryngospasm & aspiration
• Hypoxia causes cellular damage—
brain after 4-6 minutes; heart and
lungs after 30 minutes
• Pulmonary edema, atelectasis,
airway spasm, pneumonitis
• Look at KLASSEN case :- Car
plunging over bridge in Montreal
( the Windsor Star, 2/7/08, p. B1)
Near Drowning
• Prognosis is best if the water is cool
and the victim is immersed for less
than 5 minutes
• 90% occur in swimming pools
• Boys 5:1
• 50% occur under age 4 years
Management
• ICU-Respiratory assessments, V/S,
mechanical ventilation and/or
tracheostomy, blood gases, chest
therapy, IV therapy
• Emotional support for family
• If no purposeful movement within 24
hours = severe neurological damage
or death
POISON CONTROL
Poison Prevention
• Ingestion of toxic agent **can be
usual household items
• Curiosity as a toddler
• Underdeveloped taste
• Most common:
• Over 90% occur at home
• See case study p. 669
Facts on Poisoning in Ontario
from Health Canada 2004
• Most poisonings occur in children
under 6 years of age
• There is NO SUCH THING as a child
proof container
• Strong smells or bad tastes WILL
NOT stop a child from swallowing
something
• Most poisonings occur when a
product is in use – never leave a
child alone with a substance even for
a second
Over the Counter Items
• Can be as little as ¼ of a teaspoon or ½ a tablet
to cause serious complications
– Camphor
– Visine, Afrin, Otrivin, Clear Eyes
– Benzocaine
– Lomotil
*Most common: cosmetics, personal care products
• Babies & toddlers often eat diaper creams, baby
powder, mineral oil, vaseline – keep these out of
sight
• Children will climb cupboards above fridge &
stove
• Poisonings often occur when routines are
disrupted ie during times of stress
Common Poisons
•
•
•
•
•
•
•
•
•
Acetominophen – Tempra or Tylenol
ASA
Ibuprofen – Motrin or Advil
Household cleaners
Prescription & non prescription medications – ie
vitamins, birth control pills
Cosmetics & personal care products ie lotions,
perfumes, nail polish, remover, toothpaste,
mouthwash, deodorants
House plants
Alcohol
Gasoline, car oil, Antifreeze, windshield washer
fluid
DO NOT INDUCE VOMITING
IF
• Client is less than 1 year old
• Client is semicomatose, inebriated, in
shock, seizing or unable to swallow (no gag
reflex)
• Client has ingested
• Corrosive substances such as cleaning
compounds such as ammonia, detergents
• Thick oily substances such as mineral oil,
gasoline, kerosene, lighter fluids
• Convulsants such as strychnine or iodine
***In any of these cases vomiting
could cause more damage,
aspiration or asphyxiation
• Save all emesis – it may need to be
sent to lab for contents analysis
• May also order urine & blood tests
• Closely monitor vital signs of client
• Observe for any other symptoms
such as confusion, tremors,
convulsions, visual disturbances, loss
of consciousness, respiratory distress
or cardiac arrhythmias
• Poisoning by Inhalation
• Requires fresh air, oxygen maybe CPR
• If the person is not breathing begin CPR
• Poisoning of Skin or Eyes
• Requires removing any clothing touched by
the poison
• Flushing with continuous stream of water for
15 mins
Client Teaching
• Teach hazard symbols
Client Teaching
Heavy Metal Poisonings
• Mercury
• Sources: thermometers, food sources
• Neuro, digestive, kidney damage
• Chelation therapy
• Lead
• Learning disabilities, behavioral problems,
seizures, neurological damage, anemia,
coma
• Chelation therapy
Lead House
Look up Lead poisoning
Guidelines and care plan on
the CD that comes with the
text Can print off or save to
desktop.
Content from text CD
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
Nursing Diagnosis
•
Risk for Injury related to ingested or inhaled lead
Patient Goal 1
•
Child will exhibit signs of reduced lead in body.
Nursing Interventions and Rationales
•
Ask family if child is allergic to peanuts; if so, child should not be given chelating agents
such as dimercaprol (also called BAL [British antilewisite]) or D-penicillamine.*
•
Administer chelating agents as prescribed to reduce high blood lead levels.
•
Observe for and control seizures for which child is at risk.
•
Implement measures to reduce nausea, which is a side effect of some chelating agents.
•
Monitor intake, output, and serum electrolyte levels.
•
Maintain adequate hydration, especially when chelating agent succimer is given.
•
Monitor absolute neutrophil count, since neutropenia may occur if child is receiving
succimer.
•
Administer cleansing enemas or cathartic, if ordered, for acute lead ingestion.*
•
Avoid giving iron during chelation because of possible interactive effects. Rotate injection
sites if chelating agent is given intramuscularly.
•
If home oral chelation therapy is used, teach family proper administration of medication.
*Dependent nursing action.
Expected Outcomes
•
Child receives chelation therapy without complications.
•
Child exhibits signs of reduced lead in body (specify).
Pica
• Compulsive & excessive ingestion of
food & non food substances
• Common in children, pregnant
women, autism, anemia, chronic
renal failure, celiac disease
• Often unrecognized
• Consider with abdominal pain, GI
symptoms or anemia
Asthma
• Chronic, obstructive, often reversible
• Hyper responsiveness of trachea &
bronchi
• Inflammation & edema;  tenacious
mucus
• Trapped air
• Severe respiratory acidosis &
metabolic acidosis
• Causes-genetics, triggers, allergies
Prevalence
• 1997 - 12.2% of children under age 20
years : Today 2007- doubled +
• 6.7% of all hospitalizations for children
• Males more frequently than females
• Mortality rate is increasing yearly
• Increasing along highways with heavy
truck traffic and in drift areas due to
micro particulates from coal powergeneration and pollution from industries.
• Watch the animation videos on the
CD with the text. Asthma Care Plans
• Also look up guidelines and Nclex
questions
• Text pp.1355-1373
Medications
Treatment
– Bronchodilators-by inhalers,
compressors, aerochambers e.g.
Ventolin
– Leukotriene modifiers e.g. Singulair
– Corticosteroids e.g. bechlomethasone
inhalers
Allergy-proof the home, avoid triggers
Chest physio, education, “normalization”
NSG Care pg. 1402-1404
Refer to CD with text for
• Guidelines,
• Nursing care plans,
• Animated videos
• RNAO Best Practice Guidelines
• Peak Flow Meter use and indications
Complications
• Status Asthmaticus
• Chronic bronchitis, bronchiolitis
• Pneumonia,
• Emphysema,
• Cor Pulmonale,
• atelectasis, pneumothorax,
• Death
Allergic Rhinitis
• Common to 20-40% of pediatric
population- aggravated by tobacco
smoke , early intro of foods and milk
• Cyclic, Perennial, or Seasonal:
hayfever- trees, grasses, weeds,
flowers, pollens, decay
• Need familial predisposition and
exposure to allergen for sensitization
WHAT HAPPENS?
• Immune response to the allergenstimulates IgE production in B
Lymphocytes. These sensitized cells patrol
and trigger rapid release of histamine,
prostaglandins and leukotrienes from the
mast cells when allergen is recognized.
Rapid onset is followed by slower acting
cytokines.
• Histamines cause vasodilation,swelling,
mucosal edema, mucus.
• Cytokines produce inflammation,and cell
destruction- exudate, redness
Common Allergens: Pet dander, feathers,
cockroach dung, dust mites, pollens, mold,
grass, dust, micro particles, wool, foods,
medications, chemicals, insect stings/bites
Treatment of allergies is vital:
but first step is recognition
• Assessment : Allergic shiners, nasal
obstruction, snoring, mouth breathing,
hearing deficit and ear infections, allergic
salute, horizontal nasal crease , pale and
swollen nasal turbinates, red swollen eyes,
red-rimmed, pale colour generally, nasal
drip , tickle in throat, headache, wheezing,
eczema(atopic dermatitis), pruritis,
urticaria, exercise intolerance, delayed
speech, abdominal pain, cramps, digestive
upsets, swollen lips and tongue, burning
skin, clear nasal discharge, sneezing
ANAPHYLAXIS
• Sudden and severe systemic response to
allergens- foreign proteins. Body reacts in
dual mode, instantly and chronically.
• Life threatening- odours, oils, particles,
proteins- miniscule amounts create
dramatic response, can occlude airways,
cyanosis, mucosal membranes swell up,
cause spasms, create kidney shutdown,
cerebral edema, cell rupture, death.
• EMERGENCY – NO TIME AT ALL ! STAT
Management
• Avoid contact
• Eliminate allergens- triggers
• Create a zone of protection right on the person- carry an
Epipen and know how to use it. Everyone needs to know
and practice
• Medic Alert Bracelet; tell everyone
• Read labels, eliminate offending things
• Fragrance –free zones, peanut-free zones.
• No eating out
• Private Member’s Bill passed into Law- anaphylaxis plan in
place- on buses, in public places, Epipen training
• food labels, consumer products( cosmetics)
• nut free products
Allergy Testing should take place but no
sooner than 2 years
• Other diagnostics are:
• Observations associated with first
immunizations- beyond normal reactions
• Nasal Swab for eosinophils, Blood work for
immunogobulin assay( all
Immunoglobulins ) particularly IgE,
• Skin scratch tests for specific reagentsallergens [done in an allergists office or
hospital setting in case of anaphylaxis &
need for Epinephrine]
• Food challenge
Food allergies
Foreign Protein response
• Commonly to eggs, citrus fruits, strawberries,
tomatoes, grains, cow’s milk, peanut butter, nuts,
sesame seeds & oil, chocolate, seafood
• Red dyes, food additives, preservatives, artificial
flavourings
• After periods of avoidance and perhaps
desensitization treatments, some children will
want to be “medically challenged” [at about 7
years]- admitted to hospital[HSC] to test their
responses to “deadly” allergens . May not be
overly sensitized with maturity of physiologyoutgrown it
Allergy management
• Clean house and environment. Vacuum, remove black mould, pets
• Immunotherapy- desensitization “hayfever shots” start 2-6 weeks
before season.
• Antihistamines- nasal spray, pill, liquid, injection,( 1st generationBenadryl: 2nd Generation- Claritin, Zyrtec) Ok for kids 6 months
and older. Allegra for >6 years. Watch for sedation effects. School
performance,driving, riding bikes, sports co-ordination
• Leukotriene modifiers – montelukast approved for >2 yrs.
• Nasal decongestants and anti inflammatory sprays –
cortocosteroids
• care taken with ephedrines and pseudoephedrines- now off the
shelves,
• Prevent Drug misuse, rebound, habituation
• Cromolyn Sodium prevents histamine response
• Caution re decreased linear growth with ongoing use of
corticosteroids
Respiratory Infections
• Upper Respiratory tract
• Lower Respiratory tract
• Viral
• Bacterial
• Primary
• Secondary
• Episodic acute
• Chronic acute
Croup Syndromes
•
•
•
•
General term: croup
Boys>girls
Children between 2-6 years of age
Pathophysiology
– obstruction of airway (larynx)
– usually viral cause
• S/S: hoarseness, resonant cough
“barking”, “seal”, inspiratory stridor,
respiratory distress
– Spasmodic or laryngotracheobronchitis
(LTB)
– Differentiate between croup and potential
life-threatening epiglottitis
– Treatment: cool mist – take child outside
if cool enough, keep child calm, oral
steroids
• Racemic Epinephrine given
via nebulizer Why?
Epiglottitis
•
Key differences from croup:
1. Absence of cough
2. Presence of dysphagia
3. High degree of toxicity
“Children with epiglottitis usually look worse
than they sound, in contrast to children
with croup who sound worse than they
look.”
Clinical Manifestations
Look for the 4 “D”’s
• Drooling
• Dysphonia (hot
potato voice)
• Distress
• Dysphagia
• Stridor
aggravated
when supine
• Rapid pulse & RR
• High fever
• Tripod position
“Tripod Position”
• Abrupt onset of symptoms
• Obstruction of airway IS A MEDICAL
EMERGENCY
• Constant monitoring is necessary
• Reduce stress – keep child calm
• NEVER examine the throat of a child
suspected of epiglottitis
Management
• PROTECT AIRWAY!!!!!
• IV antibiotics
• Steroids
• Allow child to remain in position that
is most comfortable
• Intubation may be necessary
• In severe cases: tracheostomy
Pneumonias
• Acute inflammation of lung
parenchyma
• Bacterial pneumonia
– High fever, nasal flaring, retractions
– Rx-antibiotics
– Consolidation,  air entry, rales &
rhonchi, dullness on percussion
• Viral Pneumonia
– Most common ; patchy or diffuse
infiltration
– Mild fever, malaise
– Rales & rhonchi
– Rx-no antibiotics
Chlamydial Pneumonia
 ages 1-3 months
severe, diffuse
 Persistent cough & dyspnea
 Rx - Erythromycin for 14-21 days
Viral
– most common type of pneumonia
-RSV, parainfluenza, influenza, adenovirus
-Onset acute or insidious
Rx – promote oxygenation & comfort
Primary Atypical
-mycoplasma most common age 5-12
Onset –fever, chills, myalgia, cough, crackles
Rx –symptomatic –recovery 7-10 days
Respiratory Therapy
• Oxygen–cannula, masks, tent 30-50%
– Oxygen toxicity, CO2 narcosis
• Aerosols
• Bronchodilators, steroids, antibiotics
• Nebulizers, metered dose inhaler, aerochamber
• Ventilators, Resuscibags, CPAP, IPPB,
BiPap( watch CD for text, Animation)
• Endotracheal airways, tacheostomy
• Suctioning
Chest physio,
Postural Drainage ( see p 1321)
Chest physio
– percussion, vibration,
squeezing, deep breathing,
coughing, usually after nebulizer
treatment
CYSTIC FIBROSIS
CYSTIC FIBROSIS
• Genetic anomaly defect on
Chromosome 7
• Discovered in Canada- HSC,Windsor
Family( Nadan) helped as 4
generations available for gene
testing
• Mostly a Caucasian defect
• Systemic effect defect in chloride
transport and use on cell membrane
Cystic Fibrosis (CF)
• Exocrine gland dysfunction that
produces multisystem involvement
• Most common lethal genetic illness
among white children
• Approximately 3% of U.S. white
population are symptom-free carriers
CF Incidence in U.S. Live
Births
• 1 in 3300 whites
(95% of cases)
• 1 in 16,000
African-Americans
• 1 in 32,000 Asians
• Grandparents are often
surprised and often
quietly share in burden
of inheritance
Etiology
• Autosomal recessive trait
• Inherits defective gene from both
parents with an overall incidence of
1:4
Pathophysiology
• Characterized by several unrelated
clinical features
Effects of Exocrine Gland
Dysfunction in CF
Increased Viscosity of
Mucous Gland Secretion
• Results in mechanical obstruction
• Thick inspissated mucoprotein
accumulates, dilates, precipitates,
coagulates to form concretions in
glands and ducts
• Respiratory tract and pancreas are
predominantly affected
Increased Sweat
Electrolytes
• Basis of the most reliable diagnostic
procedure—sweat chloride test
• Sodium and chloride will be two to
five times greater than the controls
Other Factors
• Increased organic/enzymatic
constituents of saliva
• Abnormalities of the autonomic
nervous system
Respiratory Manifestations
• Present in almost all CF patients, but
onset/extent is variable
• Stagnation of mucus and bacterial
colonization result in destruction of
lung tissue
• Tenacious secretions are difficult to
expectorate—obstruct
bronchi/bronchioles
Respiratory Manifestations
(cont.)
• Decreased O2/CO2 exchange
• Results in hypoxia, hypercapnea,
acidosis
• Compression of the pulmonary
blood vessels and progressive lung
dysfunction lead to pulmonary
hypertension, cor pulmonale,
respiratory failure, and death
Infectious Pathogens
• Pseudomonas aeruginosa
• Burkholderia cepacia
• Staphylococcus aureus
• Haemophilus influenzae
• Escherichia coli
• Klebsiella pneumoniae
Respiratory Progression
• Gradual progression follows chronic
infection
• Bronchial epithelium is destroyed
• Infection spreads to peribronchial
tissues, weakening bronchial walls
• Peribronchial fibrosis
• Decreased O2/CO2 exchange
Further Respiratory
Progression
• Chronic hypoxemia causes
contraction/hypertrophy of muscle
fibers in pulmonary
arteries/arterioles
• Pulmonary hypertension
• Cor pulmonale
• Pneumothorax
• Hemoptysis
GI Tract
• Thick secretions block ducts → cystic
dilation → degeneration → diffuse
fibrosis
• Prevents pancreatic enzymes from
reaching duodenum
• Impaired digestion/absorption of fat
→ steatorrhea
• Impaired digestion/absorption of
protein → azotorrhea
GI Tract (cont.)
• Endocrine function of pancreas
initially stays unchanged
• Eventually pancreatic fibrosis occurs;
may result in diabetes mellitus
• Focal biliary obstruction results in
multilobular biliary cirrhosis
• Impaired salivation
Clinical Manifestations
• Pancreatic enzyme deficiency
• Progressive COPD associated with
infection
• Sweat gland dysfunction
• Failure to thrive
• Increased weight loss despite
increased appetite
• Gradual respiratory deterioration
Presentation
• Wheezing respiration, dry
nonproductive cough
• Generalized obstructive emphysema
• Patchy atelectasis
• Cyanosis
• Clubbing of fingers and toes
• Repeated bronchitis and pneumonia
Presentation (cont.)
• Meconium ileus
• Distal intestinal obstruction
syndrome
• Excretion of undigested food in
stool—increased bulk, frothy, and
foul
• Tissue wasting
• Prolapse of the rectum
Diagnostic Evaluation
• Quantitative sweat chloride test
• Chest x-ray
• PFT
• Stool fat and/or enzyme analysis
• Barium enema
Presentation (cont.)
• Delayed puberty in females
• Sterility in males
• Parents report children taste “salty”
• Dehydration
• Hyponatremic/hypochloremic
alkalosis
• Hypoalbuminemia
Goals
• Prevent/minimize pulmonary
complications
• Adequate nutrition for growth
• Assist in adapting to chronic illness
Respiratory Management
• CPT
• Bronchodilator medication
• Forced expiration
• Aggressive treatment of pulmonary
infections
• Home IV antibiotic therapy
• Aerosolized antibiotics
Respiratory Management
(cont.)
• Pneumothorax
• Hemoptysis
• Nasal polyps
• Steroid use/non-steroidal antiinflammatory
• Transplantation
Flutter Mucus Clearance
Device
GI Management
• Replacement of pancreatic enzymes
• High-protein, high-calorie diet as
much as 150% RDA
• Intestinal obstruction
• Reduction of rectal prolapse
• Salt supplementation
Family Support
• Coping with emotional needs of child
and family
• Child requires treatments multiple
times per day
• Frequent hospitalization
• Implications of genetic transmission
of disease
Prognosis of CF
• Estimated life expectancy for child
born with CF in 2003 is 40 to 50
years
• Maximize health potential
– Nutrition
– Prevention/early aggressive treatment
of infection
– Pulmonary hygiene
Prognosis of CF (cont.)
• New research—hope for the future
– Gene therapy
– Bilateral lung transplants
– Improved pharmacologic agents