Pediatric Chronic Respiratory Disorders PDF

Chronic Respiratory Disorders Asthma Cystic Fibrosis Respiratory Distress Syndrome (RSV) Bronchopulmonary Dysplasia (BPD) Sudden Infant Death Syndrome Apnea  Asthma  The most common pediatric chronic illness. Also called Hyp...

Chronic Respiratory Disorders Asthma Cystic Fibrosis Respiratory Distress Syndrome (RSV) Bronchopulmonary Dysplasia (BPD) Sudden Infant Death Syndrome Apnea  Asthma  The most common pediatric chronic illness. Also called Hyperreactive Airway Disease  Highest mortality rates are among inner city African American and Hispanic youth  Characterized by:  Chronic inflammation  Bronchoconstriction  Bronchial Hyperresponsiveness  Diagnosis  Lab and Diagnosis findings:  Client History and Physical  Chest X-Rays to rule out other diseases like pneumonia or lower respiratory tract infection  Pulmonary function tests (PFTs)- measure amount of air upon inspiration and expiration   forced expiratory volume (cannot get much out)   peak expiratory flow rate   diminished forced vital capacity   diminished inspiratory capacity  Treatment  Frequent Respiratory Assessments q 2 hours or q 1 hours if in severe distress  Working to breathe and compromised airway due to bronchoconstriction: decreased diameter, increased respiratory effort and airway resistance  Signs of respiratory distress: anxiety, cough, SOB, crackles, cyanosis  Cough: hacking, non-productive, paroxysmal, esp. at night  Expiratory wheeze bilaterally and one of the first things you hear. If more severe, have both inspiratory and expiratory wheeze  Humidified Oxygen if necessary  Cold mist vaporizers or tent contraindicated; can cause bronchospasm  Pulse Oximetry: 95% O2 normal. Anything less shows compromised oxygen. Mild hypoxia is 93%. < 90% is hypoxic and going toward hypoxemia  Position in High Fowler’s  Medications  SABAs: Albuterol, Proventil, Ventolin. For an acute exacerbation, works fast and relaxes smooth muscle  Inhaled Corticosteroids: Beclomethasone, Flonase, Budesonide. Decrease swelling and inflammation  Systemic Corticosteroids: Prednisone, Prednisolone  Inhaled NSAIDS: Cromolyn Sodium (Intal) prophylaxis. Not for acute exacerbation, used for maintenance  Antileukotrienes: Zafirlukast (Accolate) Montelukast (Singular)  Anticholinergics (LAMA): ipratropium bromide (Atrovent)  Systemic beta-2 agonists: Epinephrine, Theophylline, Breathine  LABA: Advair. Used in conjunction for maintenance  Combo asthma therapy: ICS + LABA SABA is PRN for asthmatic attack Cromolyn is ONLY used daily for prophylactic Step 1: SABA prn Step 2: low dose ICS Step 3/4: LABA + medium dose ICS Step 5: ICS higher dose + LABA Step 6: High dose ICS + LABA + PO corticosteroids  Side Effects of Theophylline  Nausea  Headache  Hyperirritability  Insomnia  Early Signs of Toxicity:  Nausea/vomiting  Tachycardia  Seizures  Dysrhythmias Monitor Theophylline Levels!! Normal: 5-10, > 20 indicates toxicity  Patient Education  GOAL: Prevent acute asthma attacks  Teach family to ID and avoid potential triggers  Teach S&S of asthma attacks (cough, wheezing, waking up at night)  Use of peak flow meters: Important with children. Blow out into peak flow meter (personal best 3x). Identifies if inhaler needed  Use of MDI’s with spacers to get whole dose down deep within lungs. Wait one minute between each puff of inhaler so they get 100% of second dose  Teach breathing exercises that  expiratory time and expiratory pressure: Blowing ping pong ball across table, windmills  Discuss physical exercise in relation to asthma: swimming is one of best exercises for asthma Three colors on Peak Flow Meter: green (80-100), yellow (50-79), red (< 50). When measuring how much they can blow out, the personal best will give a value. Green- good Yellow- caution Red- need inhaler or aerosol with the medication Severe red- ED AVOID cold liquids (cause bronchospasm) Meter Dose Inhaler connected to spacer, which will provide means of getting full amount of medication, wait one minute between puffs Triggers in children: Cold weather, allergies, second hand smoke, perfumes, dander, dust  Cystic Fibrosis p. 528  Most common Inherited Autosomal Recessive Disorder (both mom and dad carry trait)  Affects the exocrine (mucus producing) glands  Alteration in sweat electrolytes (especially chloride) and mucus production  Damages the Pulmonary (respiratory infection due to trapped mucus), GI (prevents nutrient absorption), Intestinal, Pancreas (blocks lipase and amylase and insulin, will need replacement of these and become diabetic), Liver (impaired function), Reproductive function (mucus in males blocks vas deferens and around cervix in females, cause infertility in both genders)  Increases susceptibility to pulmonary colonization of bacteria  Chromosome 7 responsible gene for CF  Affects Caucasian children; rare in Asians or African Americans  Life Expectancy significantly improved to 40-70 years  Clinical Manifestations  Meconium ileus at birth (mucus now in intestines, hard to pass first stool)  Chronic respiratory infection  Cough, Hemoptysis  Sputum production- thick, tenacious secretions  Hyperinflation of the alveoli  Bronchiectasis from so much mucus  Clubbing of Nails from chronic hypoxia  Protuberant abdomen and thin extremities from failure to absorb nutrients (smaller size child, weight loss)  Insulin Deficiency- DM may result  Malabsorption of nutrients- vitamins A,D,E,K  **Steatorrhea- large, loose, bulky stools, high fat content that float (because cannot absorb anything from excess mucus)  Rectal prolapse/Hemorrhoids  Hypochloremia alkalosis from salt wasting (sodium and chloride losing, salty tasting sweat)  Infertility in males/females Too much mucus is being produced, causing an obstruction to bronchi (common bronchia pneumonia and obs. emphysema). Mucus in small intestine (intestinal obstruction). Mucus in pancreatic ducts (malabsorption syndrome). Mucus in bile ducts (biliary cirrhosis which leads to pulmonary hypertension)  Diagnosis  Sweat Chloride Test greater than 60 Meq/L is diagnostic of CF reveals high sodium and chloride levels in child’s sweat  Small pads put on child’s chest and arms, and give pilocarpine (make them sweat), then collect sweat and send to lab, which is analyzed for sodium and chloride content. If greater than 60, it is diagnostic of CF  72-hour fecal fat study for steatorrhea  CXR reveals patchy atelectasis; generalized obstructive emphysema  Prenatal DNA analysis shows intestinal alkaline phosphatase is   Treatment  Goals  Maximize lung functioning and nutritional intake  Prevent and Treat Respiratory Infections  Aggressive management to remove secretions from lungs (on daily basis)  Medications  Recombinant human deoxyribonuclease (DNase)***- given in aerosol to expectorate mucus to prevent URI and have better quality of life  Antibiotics- in conjunction for infections  Mucolytics- remove mucus (bronchodilators) to treat SOB  Bronchodilators- treat SOB  Pancreatic Enzymes- given with every meal and every snack (Altrase) to absorb missing enzymes (amylase and lipase)  Fat Soluble vitamins- A, D, E, and K are replaced daily  Salt supplements in warm weather- losing so much salt, need supplemented  Laxatives- can't pass stool that is so fatty and bulky Diet: High in Protein and Carbohydrates Infant: Predigested formulas (Pregestimil or Nutramigen) Possible Lung Transplantation / Gene Therapy  Patient Education  Multisystem Assessment  Teach Importance of Pulmonary hygiene  Aerosols with bronchodilators  Postural drainage 3-4/day  Breathing exercises  Assist child with use of Flutter Mucus Clearance Devices  Home care needed  Explain S&S of respiratory infections  Explain how exercise & PE will loosen secretions and promote lung expansion  Discuss genetic counseling  Provide information about support groups (groups and camps)  Aerosol Treatments  Purpose of Aerosol Treatment: Prevent or treat respiratory tract disorders, deliver an decrease inflammation and promote expectoration  Medications used in aerosol treatments:  Mucolytics: Mucomyst (Acetylcysteine)  Decongestants for children > 6  Bronchodilators  Antimicrobials  Used in the treatment plan for: Cystic Fibrosis, Pneumonia with atelectasis, Asthma, Croup  Aerosols given BEFORE postural drainage/percussion to facilitate removal of mucus  Aerosols given BEFORE meals or 1 ½ hrs after or at bedtime to avoid inducing emesis  Access if child can tolerate length of aerosol, ie. cardiac failure, hypoxic, extreme fatigue Manual postural drainage: (cupping) on every lobe of the lung. Do anterior, lateral, posterior, use stoppers to percuss each lobe to remove secretions Electronic percussor: Place child in different positions to promote expectoration  Respiratory Distress Syndrome  Related to developmental delay in lung maturation in preterm neonates  Also known as Hyaline Membrane Disease:  Immature pulmonary surfactant system: (what keeps alveoli open). So if born too soon, not enough surfactant produced to keep alveoli open  Stiffer Lungs that fail to inflate fully  Clinical Manifestations:  Prematurity  Tachypnea > 60 breaths/min  Nasal flaring, expiratory grunting, diminished breath sounds  Hypoxia, respiratory acidosis  Clinical Manifestations  Dyspnea  Expiratory Wheezing  Chronic Dry Cough  Recurrent Chest Tightness  Tachypnea (> 60)  Irritability and Restlessness  Use of Accessory Muscles  Nasal Flaring  Orthopnea  Diaphoresis  Growth and Development retardation  Treatment  Exogenous Surfactant (Exosurf) via endotracheal tube to replace surfactant  Mechanical ventilation with supplemental oxygen  Maintain neutral thermal environment (NTE): cannot control temp, so placed in isolette/radiant warmer to maintain a normal temp. Weighed daily.  Correct fluid and electrolyte imbalances: IVF, TPN  Provide chest physiotherapy: buildup of mucus that they cannot expectorate on their own  Complications of RDS  Bronchopulmonary Dysplasia (BPD): Child with significant RDS on vent too long, lungs get very stiff, and lose ability to keep alveoli open themselves  Pneumothorax: part of the lung collapses  Patent Ductus Arteriosus (PDA)  Infection  Intraventricular Hemorrhage (IVH): High risk. Anytime their O2 is low or have hypoxemia, a hemorrhage in the brain can develop  Bronchopulmonary Dysplasia (BPD)  Chronic Obstructive pulmonary disease in premature infants after prolonged oxygen therapy & ventilation  Want to get them off vent asap  High O2 and ventilation damages bronchial epithelium and alveoli  Causes scarring and fibrosis which leads to atelectasis, poor gas exchange, chronic low oxygenation  Results in  lung compliance and altered respiratory function Diagnosis: – CXR reveals lung changes air trapping (looks like emphysema) – Blood gases- hypercapnia & resp acidosis – Tachypnea, Tachycardia, Retractions, Wheezing – Barrel Chest (due to air trapping) Treatment: – Artificial Airway: Mechanical Ventilation to keep alveoli open, trach, etc. – Suctioning prn – Chest physiotherapy q2-4 hr prn – Medications: Bronchodilators, Corticosteroids, Diuretics, Antibiotics, – Patient Education: CPR, home monitor prn, infection control.  Sudden Infant Death Syndrome (SIDS)  Sudden, unexplained death of an infant < 1 yr in which a postmortem exam fails to confirm the cause of death  Peak age of occurrence is 3 mos. (90% before 6 mos.)  Higher incidence in winter months  Almost always occurs during sleep without noise or struggle  Risk Factors  Prematurity  Low birth weight  Male gender  Asphyxia  Multiple birth  SIDS in siblings  Risk Reduction Strategies  Place the infant to sleep on back!  Cribs comply to safety standards (not falling between mattress/cracks)  Avoid water mattresses or soft surfaces  Secondary smoke increases incidence of SIDS  Avoid bottle feeding in cribs (cause aspiration)  Avoid overheating the infant (can stop breathing if too comfortable)  Avoid bedsharing or co-sleeping  Autopsy findings:  Pulmonary edema  Intrathoracic petechiae  Chronic hypoxia  Apnea  Unintentional cessation in spontaneous breathing for more than 20 seconds  Occurs primarily in premature infants because their central respiratory centers are immature  Common medications used to manage apnea: caffeine, theophylline (stimulants)  Apparent Life-Threatening Event (ALTE)  Usually central, not obstructive apnea  Apnea greater than 20 seconds  Color change, usually pale then cyanotic  Marked muscle limpness  Choking or gagging  Treatment Diagnostic Tests to identify apnea  4 channel pneumogram: reads out every breath, looking for periods of apnea, the frequency. Done over 24 hours or longer Treatment: Initiate CPR/CPR classes Home Monitoring (commonly for 1 yr): alarms will wake parent up if child needs stimulated or CPR if stopped breathing Medications: theophylline, caffeine Support groups- when to call 911, how to stimulate, emotional support (can be very scary)  After age 1, apnea subsides  Apnea Monitoring Belt picks up every respiration.