ASTHMAWhy 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 inflammation2.)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, sporesIndoors: 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 PersistentModerate PersistentMild PersistentIntermittent
(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
CorticosteroidsCorticosteroids (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 long-term 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
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)
Bronchodilators (cont’d)
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.
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 USMedian 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 fat-soluble 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
PansinusitisResistant pulmonary infections~ e.g.
Pseudomonas aeruginosa~common cause of pneumonia ( website )
Allergic Bronchopulmonary Aspergillosis (ABPA)
HemoptysisPneumothoraxPulmonary HTN leading to cor pulmonale
Bile duct obstruction
May cause biliary cirrhosis of the liverPortal hypertensionFocal biliary fibrosisHypoprothrombinemiaPrevalence 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 gainDistal intestinal obstructive syndromeRectal prolapse
Complications of the GI tract
IntussusceptionCholecystitis/cholelithiasisPancreatitisGlucose 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/LCF 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 ManagementHealth 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, mid-afternoon, 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 ManagementNebulizer 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 self-esteem 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 therapyRespiratory 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 FibrosisA website with good patient
education and research on this disease: http://www.nlm.nih.gov/medlineplus/cysticfibrosis.html
Here’s another website to the Cystic Fibrosis Foundation that offers lots of links.http://www.cff.org
Life expectancy for CF