Nursing104 exam 2

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  1. What are charecteristics of Cystic Fibrosis (CF)?
    • Characterized by multiple dysfunction that produces multisystem involvement
    • Mucus secretion of the body, esp. pancreas &lungs are very tenacious
    • Marked electrolyte change: Cl concentration of sweat is 2-5x above normal
  2. Statictis of CF?
    Most common lethal genetic illness: Caucasain children, adolescents & young adults
  3. Etiology of CF?
    • An inherited autosomal-recessive trait:
    • Inherits defective gene from both parents with an overall incidence of 1:4
    • Defective mutated gene in CF is locatedon the long arm of Chromosome 7
    • The gene causes the dysfunction of theexocrine glands.
  4. Growth & Development from Moderate to severe CF?
    • Physical growth: restricted
    • Respiratory: Increased O2 demands for pulmonary functioning
    • Musculoskeletal: Delayed bone growth
    • Failure to thrive
    • Increased wt loss despite an increased appetite
    • Gradual deterioration of respiratory system
  5. Earliest postnatal manifestation of CF?
  6. Later manifestation of CF?
    Loss of appetite
  7. Impaired digestion/absorption of fat terminology in CF?
  8. Impaired digestion/absorption of protein terminology?
  9. Pancreatic Involvement in CF?
    • Thick secretions mix with pancreatic enzymes (trypsin, amylase, lipase) and block the pancreatic duct (pancreatic fibrosis)
    • Acinar cells of pancreas: produce lipase,trypsin and amylase; enzymes become thickened that the ducts become plugged.
  10. Pancreatic Involvement of Stools
    What are Four F’s in CF?
    • 1. Frothy (large/bulky)
    • 2. Foul smelling (compared to a cat's stool) azotorrhea
    • 3. Fat containing “greasy” Steatorrhea
    • 4. Floaters
  11. Clinical Manifestations in CF?
    • Protuberant abdomen
    • Signs of malnutrition-only benefit from 50% of food taken in (Later in disease:loss of appetite)
    • Fat-soluble vitamins are not absorbed: A,D, E, K
    • Infants: MECONIUM may cause intestinal obstruction (Meconium ileus)
  12. Clinical Manifestations GI in CF
    • Earliest postnatal manifestation-- Thick, putty like, tenacious, mucilagious meconium blocks the lumen of small intestine ator near ileocecl valve
    • Abdominal distention
    • Vomiting
    • Failure to pass stools
    • Rapid development of dehydration w/e-lyte imbalance
    • Gum-like masses-- cecum obstruct the bowel leading to pain, n/v--Meconium ileus equivalent
    • Distal intestinal obstruction syndrome
    • Partial or complete intestinal obstruction that canoccur
  13. Clinical Manifestations GI Involvement in CF?
    • Large, bulky, frothy, foul-smelling stools
    • Voracious appetite (early in disease)
    • Loss of appetite (later)
    • Weight loss
    • Marked tissue wasting
    • Failure to grow
    • Distended abdomen
    • Thin extremities
    • Sallow skin, anemia, ADEK deficiency
  14. Clinical Manifestations of Pulmonary Involvement in CF?
    • Stagnation of mucus in airways w/subsequent bacterial growth
    • Initial signs: Wheezy respirations, dry nonproductive cough
    • Eventually: Increased dyspnea, paroxysmal cough; evidence of obstructive emphysema & patchy areas of atelectasis
  15. Respiratory Manifestations of CF?
    • Decreased O2/CO2 exchange: Results in hypoxia, hypercapnia, acidosis
    • Compression of the pulmonary blood vessels and progressive lung dysfunction lead to-- Pulmonary hypertension, cor pulmonale, respiratory failure, and death
  16. Clinical Manifestations of Pulmonary Involvement. and how does it Progressive in CF?
    • Over-inflated barrel-shape chest (secondary emphysema) w/Chest anteroposterior diameter-- enlarged
    • Cyanosis
    • Clubbed fingers
    • Respiratory acidosis
    • Repeated bronchitis, bronchiectasis and bronchopneumonia occur
    • Hemoptysis
  17. Infectious Pathogens in CF?
    • Pseudomonas aeruginosa
    • Burkholderia cepacia
    • Staphylococcus aureus
    • Haemophilus influenzae
    • Escherichia coli
    • Klebsiella pneumoniae
  18. Effects of Increased Viscosity of Mucus Glands Secretion in CF?
    • Bronchi: Chronic bronchial pneumonia and Generalized obstructive emphysema
    • Small intestine: Insytestinal Obstructive of Newborn
    • Pancreatic ducts: Malabsorption Syndrome
    • Bile ducts: Portal Hypertension
  19. Presentation of CF?
    • Wheezing respiration, dry non-productive cough
    • Generalized obstructive emphysema
    • Patchy atelectasis
    • Cyanosis
    • Clubbing of fingers and toes
    • Repeated bronchitis and pneumonia
    • Meconium ileus
    • Distal intestinal obstruction syndrome
    • Excretion of undigested food in stool—increased bulk, frothy, and foul
    • Tissue wasting
    • Prolapse of the rectum
    • Delayed puberty in females
    • Sterility in males
    • Parents report children taste “salty”
    • Dehydration
    • Hyponatremic/hypochloremic alkalosis
    • Hypoalbuminemia
  20. Sweat Gland Involvement
    • Electrolyte composition of perspiration is changed
    • Level of Na to Cl is increased 2-5times above normal
  21. Diagnostic Evaluation in CF?
    • Documentation of chromosomal abnormality
    • History & combination of the abnormalconcentration of Chloride in sweat
    • Absence of pancreatic enzymes
    • Newborn- Presence of immunoreactivetrypsinogen in the blood
    • Pulmonary involvement- CXR
  22. Assessment in CF? What about the Children not dx at birth?
    • Feeding problems (1 month of age)
    • Colic or abdominal distention and vomiting
    • Appearance of typical CF stools incolicky children
    • Respiratory infections--occur 4-6 months of age
    • Wheezing & rhonchi-auscultation
    • By preschool age--Cough--prominent finding
    • Percussion-Hyperresonant (emphysema is present)
    • Rales & rhonchi also heard
    • Clubbing of fingers
  23. Screening CF in Newborn?
    • CDC recommends newborn screening for CF
    • Newborn screening--
    • Immunoreactive trypsinogen (IRT) analysis: Performed on dried spot of blood, followed by direct analysis of DNA for presence of F508 mutation orother mutations on same dried blood spot.
    • Benefits early screening & detection-- earlier nutritional intervention & preservation of lungfunction for identified infants.
  24. Diagnostic Tests for CF? Important slide
    • Quantitative Sweat Test (pilocarpine inontophoresis): most reliable
    • Normal Cl: <40 mEq/L with a mean of 18mEq/L
    • Abnormal: >60 mEq/L: diagnostic of CF
    • Infants < 3 mos: >40 mEq/L: highly suggestive CF
    • Duodenal analysis
    • Stool fat analysis or enzyme analysis
    • DNA testing
  25. Pulmonary testing for CF?
    • CXR– PFTs (show abnormal airway function)
    • Barium enema: for diagnosing meconiumileus
    • Serum albumin: decreased
  26. What are the Goals for CF?
    • Prevent/minimize pulmonarycomplications
    • Adequate nutrition for growth
    • Assist in adapting to chronic illness
  27. What are Therapeutic Managements for CF?
    • Aerosol therapy: Nebulizer tx 3-4 times to provide antibiotics and bronchodilators
    • Recombinant humandeoxyribonuclease (Dnase, known genetically as dornae alfa (Pulmozyme)
    • Mucolytic agent= Acetylcysteine (Mucomyst): help liquefy secretions
    • Do not give cough suppressants or codeine
    • Moistened O2 via mask, prongs, ventilators ornebulizers- to maintain bronchial secretions moistas possible to facilitate drainage
    • CPT/Postural drainage: aggressive CPT frequently; 2-4x per day
    • Activity-- need frequent position changes when in bed
    • Make sure they sit up part of each day to drain upper lobes– Exercise
    • Medication administration as ordered
    • Through Physical assessment & evaluation of affected system
    • Assist with diagnostic testing
  28. Cf Respiratory Management?
    • Chronic respiratory infections
    • Pneumothorax
    • Hemoptysis
    • Nasal polyps
    • Steroid use/nonsteroidal antiinflammatory
    • Transplantation
  29. CF Alerts?
    • Signs of pneumothorax:
    • Nonspecific and include: Tachypnea, tachycardia, dyspnea, pallor, cyanosis
    • Subtle drop in O2 saturation (by pulseox): early sign
  30. GI Management in CF?
    • Replacement of pancreatic enzymes
    • High-protein, high-calorie diet as much as 150% RDA
    • Prevention/early management of intestinal obstruction
    • Reduction of rectal prolapse
    • Salt supplementation
  31. Therapeutic Management of Pharmacologic management (p. 1272)
    • Pancreatic enzyme supplements before or within 30 min of eating or w/ meals & snacks-- Cotazym or Pancrease
    • Add capsule to small amount of food (1 tsp)
    • Do not add to infant’s bottle of formula
    • H2RA-- Pepcid, Zantac
  32. Appropriate immunizations for CF children?
    Trivalent inactivated FLU vaccine is appropriate
  33. Nursing Implication with Respiratory hygiene for CF children?
    • Frequent oral care,tooth-brushing and good tasting mouthwash
    • Adequate rest & comfort
    • Emotional support
    • Well-balance nutrition
    • Growth & development-- expose to as many normal life experiences as possible
    • Frequent observations
  34. Hospital Care for CF children?
    • Tx pulmonary infection, incontrolled DM orcoexisting medical problem
    • Aerosol therapy, CPT and postural drainage
    • Supplemental O2, IV therapy
    • Pulse oximetry monitoring
    • Meds--Pancreatic enzymes, vitamins, oral antifungals, antihistamines, antiinflammatory agents and PO/IV AB, inhaled bronchodilators, insulin
    • Blood glucose monitoring
  35. CF Family Supports ?
    • Coping with emotional needs of child and family
    • Child requires treatments multiple times per day
    • Frequent hospitalization
    • Implications of genetic transmission of disease
  36. Prognosis of CF
    • Decreased life expectancy for child born withCF
    • Maximize health potential--
    • – Nutrition
    • – Prevention/early aggressive treatment of infection
    • – Pulmonary hygiene
  37. Teaching Parents with CF children regarding Disease management?
    • Explain what CF is; s/s; complications
    • Importance of physical activity to improve overall fitness &lung function
    • Discuss the importance of playing games that require Debbie to do physical activity such as running, jumping, or skipping
    • Necessity for high, calorie, high protein diet
    • Use of pancreatic enzymes to aid digestion
    • Pancreatic enzymes are adjusted to stool formation
    • Discussion of CPT--postural drainage, percussion, vibration and coughing
    • Self-care
    • Types of aerosol therapy and care of equipment
    • Teach child and parents not to restrict salt to maintain appropriate e-lyte balance
    • During hot months, will need extra salt added tofood to replace what is lost via perspiration
    • Keep room temp. at 72 hours and have water available at all times
    • Teach parents to supervise kids playing outside toprevent overheating
    • Keep child well hydrated
    • Community resources referral-- CFF
  38. Transition to Adulthood in CF children?
    • Normal sexual relationships
    • Female pt--may be able to bear children; but inform of possible harmful effects on respiratory system from pregnancy burden
    • Need to know children will be carriers of CF gene- Genetic counseling is essential
    • Offer adolescent counseling regarding oral contraceptives
    • Encourage life as independent as possible
    • Anticipatory guidance
  39. CF Pancrealipase (Cotazym) class and indication?
    • Class: Enzyme replacement
    • Indication: Aids digestion in children with CF
    • AD--N/D, abdominal cramps, hypersensitivity
    • Nursing
    • – Administer within 30 min of eating or with meals and snacks.
    • – Instruct parent to do same.
    • – Caution child & parent to avoid inhaling powder or spilling on hands, it may irritate the skin or mucous membranes
    • – Instruct child and parents about possible adverse effects and encourage them to call healthcare provider should any become severe
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Nursing104 exam 2
Diagnosis, prevention, treatment
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