1. RespirationRespiration Elisa A. Mancuso RNC, MS, FNS Professor of Nursing

2. Respiratory Alterations ↑ Risk < 3 years  Smaller upper and lower airways  Underdeveloped supporting cartilage  ineffective clearing of organisms  Immature immune systems Compensatory Mechanisms Lungs- ↑ or ↓ RR Kidneys- retain or filter H+ affects pH Blood buffer system: H+, HgB, Na Interact to maintain pH

3. Interpreting ABG’s Know your normal values! PH 7.35-7.45 7.45 = Alkalosis PaCo2 35-45 45 = Acidosis HCO3 22-26 26 = Alkalosis PaO2 90-100% < 90 = Hypoxia

4. Respiratory or Metabolic? ROME Respiratory opposite (pH & CO2) Metabolic even (pH & HCO3) Respiratory reflects PaCO2 ↓ CO2 = alkalosis ↑ CO2 = acidosis Metabolic reflects HCO3 and BE ↓ HCO3 = acidosis ↑ HCO3 = alkalosis

5. Respiratory Alterations Respiratory Acidosis ↓ PH and ↑ PaCO2 Causes – ↓ RR – Neuromuscular problems: BPD, RDS, CF Respiratory depression and ↑ CO2 Respiratory Alkalosis ↑ PH and ↓ PaCO2 Causes – ↑ RR ↑ Fever Stress

6. Metabolic Alterations Metabolic Acidosis ↓ PH and ↓ HCO3 Causes –Renal failure, diarrhea, ketoacidosis Metabolic Alkalosis ↑ High PH and ↑ HCO3 Causes –Vomiting, Meds for ulcers, NaHCO3, – NGT = HCL loss & ↑ HCO3 – Diuresis

7. Case Study Mariska, 4 years old presents with following: RR = 54 C/O Chest tightness Bilateral expiratory & inspiratory wheezing Frightened appearance. ABG pH of 7.27, PaO2 88, PaCO2 48 and HCO3 24. What is her acid – base status? Identify each component. Find the cause Answer????

8. Upper Respiratory Infections URI Acute pharyngitis and nasopharyngitis Children get 7-10 colds/year! Majority is viral = Rhinovirus Signs and symptoms low grade fever sore throat spontaneous recovery –Self limiting 7-10 days

9. URIs Bacterial Group A beta-hemolytic strep (GABHS) Signs and symptoms Abrupt onset Fever >102, chills Fatigue, HA Nasal congestion Abdominal pain & Anorexia Vomiting, diarrhea Halitosis Fire red throat & petechiae Exudative

10. Treatment of Strep Pharyngitis Throat culture IN and OUT. –Rapid antigen detection test 60-95% sensitive. Antibiotics Prevents serious complication = Rheumatic fever PEN-VK BID-TID drug of choice x 10 days Amoxicillin 40-45mg/kg/day ÷ BID –↑ tasting and ↓ dosing needed –↑ better compliance! Zithromax 10mg/kg/day 1 – 5mg/kg day 2-5 Cefdinir (Omnicef) 14 mg/kg/day Cefixine (Suprax) 8mg/kg/day

11. Treatment (cont) Bed rest Tylenol 10-15mg/kg every 4 hours –√ Infant vs. Child concentration! Saline gtts and cool mist humidifier Hydration Decongestants > 6 months. Contagious: Separate from others! –Need meds x 24 hours –Then return to school – Feel better in 24-48 hours! – Must Complete all meds!

12. Tonsils Lymphoid tissue in pharyngeal cavity Filter and protect respiratory and GI tract ↑ Antibody formation –until 3 years & immune system mature ↑ ↑ size in children until puberty Inflamed with infections If chronically enlarged 3+ → 4+ – Obstructive Sleep Apnea (OSA) – Difficulty breathing and eating

13. Tonsillitis Persistent cough Dry mucous membranes White patchy exudate Secondary OM from blocked Eustachian tubes Viral- Self- limiting Palliative measures Pain & Hydration

14. Tonsillectomy Most common indication today is OSA 4 strep infections/season Peri-tonsilar Abscess Post-op care ↑ HOB with pt prone or on side Encourage fluids PO -No straws! Medicate for pain (no ASA) and N/V Ice pack to anterior neck √ Hemorrhage (5-20%) Go to OR! (1 st 48 hours and then 5-7 days) ↑ ↑ swallowing/vomiting bright red blood ↑↑ RR ↑↑ HR ↑↑ Restlessness Normal Eschar forms

15. Epiglottitis Medical Emergency –↑ @ 3-6 years Haemophilus influenza type B (HIB) (50% pre-vaccination) –Dramatic ↓↓ since HIB vaccine Strep pneumoniae, staph aureous. Rapid & severe inflammation – of epiglottis and surrounding areas – Complete airway obstruction

16. Signs and symptoms Abrupt onset of sore throat Fever 102-104 - toxic appearing 4 D’s –Dysphonia (muffled voice) –Dysphagia ( ↓ swallowing) –Drooling –Distress/Dyspnea Inspiratory stridor Retractions ↑ RR ↑HR Pallor Tripod position Thumb sign on soft tissue x-ray

17. Treatment MEDICAL EMERGENCY: ANESTHISIA STAT!! DO NOT INSPECT THROAT! LIMIT UPSETING PROCEDURES! Establish Airway Respiratory Isolation! Humidified O2 Hydration Antibiotics (Meningitic doses) –Ampicillin 200-400 mg/kg/day ÷ q6H –Chloramphenicol 75-100mg/kg/day ÷ q6H Steroids – Methylprednisolone 2mg/kg/day ÷ q6H

18. Croup Laryngotracheobronchitis Acute spasmodic laryngitis –Upper airway ↑ 3 months to 5 years – peak @ 2 years Paroxysmal laryngeal edema –Attacks @ night Parainfluenza virus or allergic reaction ↑ in fall and winter months Precipitated with nasopharyngitis

19. Clinical signs Awakes suddenly with barking cough Inspiratory stridor Hoarseness Restlessness Anxious Retractions, ↓↓O 2 Stridor @ rest = severe croup ↑ Temp 101-102 Duration few hours, Repeat x 2 nights Symptoms improve with change in temp

20. Treatment Maintain airway Position upright Cool mist humidified O2 Steam shower or expose to cold night air Decadron 0.6mg/kg IM/PO x 1 dose Racemic epinephrine 2.25% nebulizer –for inspiratory stridor at rest Induce vomiting = stops laryngospasm Hospitalize only when: – ↑ Stridor ↓ O2 ↓ LOC

21. Otitis Media (OM) Acute inflammation & effusion of middle ear Common pathogens Strep pneumonia (50%) –↓ incidence with Prevnar vaccine Haemophilus influenza (30%)-not type B! Moraxella catarrhalis (20%) –↑↑ incidence with resistance Viruses Food Allergies

22. Pathophysiology Eustachian tube dysfunction < 5 years = shorter, wider and straighter Acute –bacteria/purulent exudates Signs and symptoms ↑ ↑ Pain, ↑ ↑ irritability Tugging on ears Fever >102 Rhinorrhea, cough and congestion Anorexia, vomiting and diarrhea Tympanic membrane Red & bulging Tympanogram No movement of TM Hearing loss

23. To treat or not treat? AAP guidelines to ↓ resistant organisms < 6 months: –with S/S of illness → Treat! 6 mos -2 years: –certain diagnosis → Treat! –Uncertain & no s/s of severe illness = Observe > 2 years: – certain diagnosis & no S/S of severe illness – Observation & Pain Relief AMERICAN ACADEMY OF PEDIATRICS, Guidelines for Acute Otitis Media, 2004

24. Treatments Amoxicillin 40-45mg/kg/day ÷ BID –Now recommending high dose: –80mg/kg- 90mg/kg/day ÷ BID Augmentin 40-45mg/kg/day BID for resistance to amoxicillin Ceclor 40 mg/kg/day Bactrim/Septra 8mg/kg/day Rocephin for resistant OM’s Myringotomy Tubes Frequent infections Prolonged fluid

25. Bronchiolitis (RSV) Disease of lower airways Respiratory syncytial virus (RSV) = common cause Can be fatal in <2 months/premature 90% of infants <1 year get RSV ↑ incidence winter/spring ↑ Contagious via direct contact & inhalation –Use alcohol based hand rubs. Pathophysiology RSV affects epithelia cells of lungs Bronchioles become edematous Lumen filled with mucous - green thick exudate Bronchioles infiltrated with inflammatory cells – Air trapping Severe cases mucous plugging & apnea= death

26. RSV Clinical signs Nasal Aspirate Culture = –(+) ELISA enzyme-linked immunosuppressive assay –(+) RSV Ag or rapid fluorescent antibody Peak @ 72 hours after onset Rhinorrhea with thick, tenacious, green secretions  RR, retractions & cyanosis Coughing, wheezing CXR –Hyperinflation (obstructive emphysema) –Atelectasis = ↓ Breath sounds (PN) Hypoxia → apnea and even death

27. Therapy Respiratory Isolation Cool mist humidified O2 –√ O2 sats! >95% is nl ↑ Hydration Antibiotics for PN Bronchodilators Steroids Severe Cases –Racemic epinephrine –Mechanical ventilation Prophylactic Approach Respi Gam (RSV Immune Globulin) $600/vial Synagis (Monoclonal AB) 15 mg/kg IM – Binds with RSV to ↓ infection. – @ beginning of RSV season Oct - Nov – total of 5 monthly doses; Need ↑ titers to be effective

28. Asthma Inflammation & Hyperactivity Abrupt onset after URI or allergen RAD= Reactive Airway Disease –Reversible bronchospasm 8 million kids/year 1 st attack usually @ 3-8 years

29. Pathophysiology Inflammation –Histamine release to allergen/trigger (stimulus). –Edema→ Mucous Production → Bronchial Obstruction & Spasm Bronchoconstriction –Hyper-responsiveness of stimuli: Allergens: –Cigarette smokeDust mitesExercise –Cold air Stress Drugs (ASA/NSAID) Urban factors: – #1 Cockroach droppings – Diesel fumes

30. Early & Subtle Clinical signs Irritable Itchy Tired Dry mouth Dark circle under eyes Chronic cough worse @ night

31. Clinical Signs Older child SOB and Dyspnea Expiratory wheeze bilaterally Chest pain or tightness → ↑ HR Spasmodic or tight cough @ night Abdominal pain and nausea Mild Intermittent –<2 days/week Severe Persistent – Constant/daily

32. Warning signs Retractions ↑ RR and Hypoxia<92% (Admit to hospital) As symptoms progress → –Expiratory & Inspiratory wheeze ↑ HR Breathlessness Anxious & Restless Absent breath sounds – No air movement – Respiratory arrest!

33. Status Asthmaticus Limited or no response to therapy Respiratory distress → arrest ICU – IV Hydration & Intubation Medications: –Steroids –Magnesium Sulfate IV – Bronchodilators Nebulizer RX – Antibiotics

34. Diagnostic Tests Allergy testing- –4-8% have a food allergy Pulmonary Function Test (PFT) –Forced exhalation –√ before and after neb – Reliable when age > 5 years good effort

35. Peak Expiratory Flow Rate (PEFR) Assess asymptomatic lung changes and function. Based on child’s height Ex: 47”=PEFR=200 Peak flow zones –Visual = ↑ manage –Early interventions – Maintain control

36. Asthma Therapy The National Asthma Education and Prevention Program (NAEPP) 2002 4 components of asthma management: Measures of assessment and monitoring Control factors that contribute to severity Education for a partnership in asthma Pharmacologic therapy

37. Bronchodilators “Rescue meds” Inhaled Beta 2 Agonists Albuterol (Proventil,Ventolin) 0.15-5 mg/kg/dose Levalbuterol (Zopenex) > 6 years 0.31mg/kg/dose SE = Tremors ↑ HR Hyperactivity Bronchospasm = Overdose! Anticholinergic Ipratropium (Atrovent) MDI 1-2 puffs q6-8H SE = Dizzyness HA Cough ↓ BP Methylzanthines Theophylline (PO) Aminophylline (IV) √ serum levels (10-20) SE = ↑ HR Arrhythmias Systemic B2 agonists SC Epinephrine 1:1000=bronchodilation x 3doses Caution CARDIAC DOSE 1:10,000 SE = ↑ BP ↑ HR Tremors Terbutaline (Brethine) SQ/IV SE = Restlessness cardiac arrthymias Stops pre-term labor

38. Anti-Inflammatory meds Systemic Corticosteroids onset - 3 H Peaks in 6-12 H Loading dose 2mg/kg and taper slowly No need to taper if short term use Short-Acting (use 5-7 days ↓ SE) Hydrocortisone (Solu-Cortef) 0.25-2 mg/kg/day Methylprednisolone (Solu-Medrol) 1-2 mg/kg/dose Prednisone PO 1-2 mg/kg/dose Prednisolone (Orapred, Pediapred) PO 1-2 mg/kg Dexamethasone (Decadron) 0.6-1.5 mg/kg/day SE = Hyperglycemia GI distress ↓ Growth Cushing Syndrome = ↑ Wt. ↑ Infection Mood Lability

39. Controller Meds Inhaled corticosteroids- Not rescue drug Budesonide (Pulmicort) 2-4 puffs tid Fluticasone (Flovent) Triamcinolone (Azmacort, Kenalog) Advair discus –Synergistic effect with B2agonists –SE = Oral & pharyngeal irritation Non-steroidals- Cromolyn Na (Intal) –Stabilizes mast cells & prevents attack. Leukotriene Receptor Antagonists-(LRA) –Leukotrienes cause inflammation (capillary permeability) –Use at night when leukotrienes are highest. –Montelukast (Singulair) 5-10 mg PO/day – Zafirlukast (Accolate) 10-20 mg PO/day – Zileuton (Zyflo) 300-600 mg PO/day SE = HA Vasculitis Flu like symptoms

40. Other Treatments ↑ Fluids –Dilute mucous & mobilize secretions May need allergy shots Zyrtec or Clarinex = ↓ allergy symptoms. Singulair now indicated for allergy use as well as asthma maintenance Nasal Lavage Treat cold symptoms>7-10 days –60-80% pt with allergic asthma have – sinusitis

41. Parent Teaching Remove allergens –Identify precipitating factors –↓ Rugs, heavy drapes, pets, foods (eggs, milk) –Mattress & pillow covers Dehumidifier - AC Review –Signs/symptoms of asthma –PEAK Flow daily –Meds SE & toxicity –Nebulizer use ↓ Antihistamines – May exacerbate wheezing Swimming = Best Exercise

42. Cystic Fibrosis Dysfunction of exocrine glands – ↑↑ Na++ ↑ Cl- in sweat & saliva –(2- 5x normal levels) – ↑↑ Viscosity of secretions –GI & Pulmonary systems Autosommial Recessive –1/25 whites carry gene. Chromosome # 7 CC = Healthy Cf = Carrier ff = Disease 25% risk = healthy/disease 50% risk = carrier Mom → Dad ↓ Cf CCCCf f ff

43. Pathophysiology Pulmonary ↑ Leukocyte DNA in sputum –Long, thick strands ↑↑ Thick mucous (yellow/grey) ↓↓ Diffusion of gases → ↓ O2 hypoxia ↑ CO2 ↑↑ Respiratory distress & Pseudomonas PN Obstruction = –Fibrotic and stiff lobes – ↓ compliancy & ↓ function

44. Pancreas Thick secretions block ducts Fibrosis = ↓↓ pancreatic enzymes Malabsorption Syndrome –Only 50% of food is absorbed –Inability to digest & absorb proteins & fats –“Steatorrhea” foul smelling bulky stools –↓↓ fat soluble vitamins A,D,E and K. Bile ducts –Occluded: biliary cirrhosis & portal ↑ BP

45. Hallmark – CF Signs Meconium Ileus (newborn) –No mec passed in 1 st 24 hours –Abdominal distention –10-15% & 1 st sign of CF Skin - “Infant tastes salty” –Sweat Test (Pilocarpine Ionophoresis) – > 1 month old – Cl> 60 mEq = (+) CF

46. Respiratory Signs Frequent sinus & respiratory infections. Bronchitis & PN Recurrent pneumothorax SOB, wheezing, hemoptysis Dyspnea, Hypoxemia Barrel shaped chest –AP>lateral Clubbed fingers

47. Gastrointestinal Signs Steatorrhea –Excretion of undigested fats and proteins –Bulky, frothy, foul smelling stool Abdominal Distension –3 rd spacing & edema RT ↓↓ protein & albumin Prolapsed rectum Voracious appetite RT starving –only 50% of food absorbed Failure to thrive –↓↓ drop on growth chart 10-25% = short stature

48. Diagnosis Genetic testing –DNA analysis: Chromosome # 7 –Prenatal screen ( ↑↑ mutations exist) –F508 mutation in 70% of pt with CF Sweat Test –Cl>60meq strongly suggests CF Stool specimen –5 day collection √ fat content Duodenal Enzymes –↓↓ trypsin and chymotrypsin – (absent in 80% of CF pt’s) –Immunoreactive Trypsin Test >140 = CF (+)

49. Therapy Goals – ↑ Life Expectancy > 30 – ↑ Quality of life – ↓ Sequella of CF Nutrition – ↑ protein ↑ calories and moderate fat –Need 150% of daily requirements to replace losses – ↑ Na intake in hot weather Medications Pancrelipase (Pancrease, Pancrease MT4) PO – (10,000u lipase/36,000u protease & amylase) – Enteric coated & must give before all meals! – ↑ digestion of fats, proteins and carbs. – SE: diarrhea and abdominal cramping

50. Therapy Supplements –Fat Soluble Vitamins A, D, E & K (2x dose) –H2O Soluble Vitamins C, B, B2, B6 (B-C complex) Niacin, B12, Folic Acid Pulmonary- –1 st Assess breath sounds and O2! –Nebulizer treatments then PD & C. – CPT x 15-20 minutes in trendelenburg. – Vibrate all lung fields =mobilize secretions

51. Inhalation Therapy Dornase Alfa-Pulmozyme –Recombinant DNAse 2.5 mg –Breaks down DNA in sputum –↓↓ viscosity of sputum –SE- laryngitis –Administer via neb before PD&C Proventil Thoracic expansion exercises – Stretching & Breathing – Swimming ( ↑ mobility)

52. Family Support Educate –Disease process and S/S of illness –Meds and diet Pulmonary care ATC –Need ↑ support group to assist q 3-4 H –Breathing exercises –Antibiotics only for documented infections! Encourage verbalization of fears –Numerous Hospitalizations –Invasive Procedures (CT) lung transplants –Anticipatory Grieving -Fatal Illness –Support group CF Foundation www.cff.org www.cysticfibrosis.comwww.cff.orgwww.cysticfibrosis.com

53. Foreign Body Aspiration ↑ R isk @ 1-3 years of age –Developmental stage ↑ curious and –hand–to-mouth or nose –4 th cause of accidental death < 5 years –Acute and dramatic onset Common Objects Small toys Buttons Paper clips Batteries (Acid leaks = chemical PN) Food ↑ in size as absorbs H2O – ↑ Edema = ↑ Obstruction – Hotdogs Grapes Nuts Seeds

54. Clinical Signs Laryngeal Choking & Coughing Aphonia = No cry or speaking Rapid color change → blue Inspiratory stridor ↓ O2 → Change in LOC → Collapse/Unconscious Bronchial # 1 site = R main stem bronchus Wheezing Lung Persistent respiratory infections –Cough & congestion – Purulent secretions – Foul smelling breath Acute or chronic pulmonary lesions

55. Interventions Immediate Intervention (Death in 4 mins!) CPR – Obstructed Airway –Infants- alternate 5 back blows with 5 chest thrusts –Kids >1 year Heimlich CXR – Identify object & location Bronchoscopy –Removal of object ASAP! Post removal – Humidity – Steroids ↓ Edema & ↓ inflammation – Antibiotics

56. Pneumonia Classified according to agent or location: –Viral (RSV) most common –Bacterial (strep pneumoniae, pseudomonas) –Fungal (candida) –Chemical/Aspiration (Oil, lotion, cleaners) Pathophysiology Inflammation of lung parenchyma Consolidation - aveoli fill with exudate Bronchial Obstruction – RT ↑ restriction of lung – ↓ Impaired gas exchange ↓ O2 & ↑ CO2

57. Primary Atypical Pneumonia Mycoplasma pneumoniae Most common pathogen in older children 5-12 years of age ↑ incidence in Fall and Winter ↑ Highly populated areas Diagnosis: –CBC & Differential –BC or Tracheal aspirate – CXR – ELISA test

58. Clinical signs Sudden or gradual onset –could be a 7-10 day duration of symptoms Fever - low grade Chest pain Flushed cheeks with generalized pallor Hacking cough Pharyngitis Coarse Crackles or rhonchi ↓ Breath sounds with dullness (consolidation) Hypoxemia Anorexia Malaise

59. Therapy O2 √ Pulse oximeter ↑↑ Hydration PO/IV Humidity CPT –Blow Bubbles ↑↑ HOB & Rest Medications Azithromycin (Z-Pack) (10 mg/kg day 1 then 5 mg/kg day 2-5) Erythromycin 30-50 mg/kg/day PO/IV ÷ q 6-8 x 14 -21 days No IM causes tissue necrosis! Acetaminophen (Tylenol) – 10-15 mg/kg/dose √ (infant vs. children) – ↓ Pain & Fever Expectorants only No cough suppressant!

60. Bacterial Pneumonia ↑ R isk @ birth-5 years Strep pneumoniae (90%) Clinical signs/symptoms Abrupt onset after viral illness - URI –↓↓ immune system High fever 104-105 Retractions, tachypnea, hypoxia Rales/rhonchi Chest Pain with deep inhalation –Pleural effusion → Shallow respirations & ↑ CO2 Abdominal pain – Lower lobe infiltrate

61. Therapy Similar to Mycoplasma Maintain patent airway! Isolate with same pt if hospitalized Lying on affected side ↓ pleural rub/pain CT for thoracentesis Medications Antibiotics- appropriate drug for the bug! –High dose Amoxicillin or Augmentin (40mg/kg/day PO) –Ceftriaxone (Rocephin) (50-75 mg/kg/day) ↑ WBC or based on S/S – Cefotaxime (Claforan) 100-200 mg/kg/day – Ceftiazidine (Fortaz) 150 mg/kg/day Tylenol Expectorants