Asthma and Cystic Fibrosis

Chronic Illnesses in Children

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.

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 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

That’s all folks!