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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
Statictis of CF?
Most common lethal genetic illness: Caucasain children, adolescents & young adults
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.
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
Earliest postnatal manifestation of CF?
Later manifestation of CF?
Loss of appetite
Impaired digestion/absorption of fat terminology in CF?
Impaired digestion/absorption of protein terminology?
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.
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
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)
Clinical Manifestations GI in CF
and MECONIUM ILEUS
- Earliest postnatal manifestation-- Thick, putty like, tenacious, mucilagious meconium blocks the lumen of small intestine ator near ileocecl valve
- Abdominal distention
- 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
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
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
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
Clinical Manifestations of Pulmonary Involvement. and how does it Progressive in CF?
- Over-inflated barrel-shape chest (secondary emphysema) w/Chest anteroposterior diameter-- enlarged
- Clubbed fingers
- Respiratory acidosis
- Repeated bronchitis, bronchiectasis and bronchopneumonia occur
Infectious Pathogens in CF?
- Pseudomonas aeruginosa
- Burkholderia cepacia
- Staphylococcus aureus
- Haemophilus influenzae
- Escherichia coli
- Klebsiella pneumoniae
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
Presentation of CF?
- Wheezing respiration, dry non-productive cough
- Generalized obstructive emphysema
- Patchy atelectasis
- 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”
- Hyponatremic/hypochloremic alkalosis
Sweat Gland Involvement
- Electrolyte composition of perspiration is changed
- Level of Na to Cl is increased 2-5times above normal
- “SALTY TASTE TO SKIN”
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
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
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.
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
Pulmonary testing for CF?
- CXR– PFTs (show abnormal airway function)
- Barium enema: for diagnosing meconiumileus
- Serum albumin: decreased
What are the Goals for CF?
- Prevent/minimize pulmonarycomplications
- Adequate nutrition for growth
- Assist in adapting to chronic illness
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
Cf Respiratory Management?
- Chronic respiratory infections
- Nasal polyps
- Steroid use/nonsteroidal antiinflammatory
- Signs of pneumothorax:
- Nonspecific and include: Tachypnea, tachycardia, dyspnea, pallor, cyanosis
- Subtle drop in O2 saturation (by pulseox): early sign
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
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
Appropriate immunizations for CF children?
Trivalent inactivated FLU vaccine is appropriate
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
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
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
Prognosis of CF
- Decreased life expectancy for child born withCF
- Maximize health potential--
- – Nutrition
- – Prevention/early aggressive treatment of infection
- – Pulmonary hygiene
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
- 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
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
CF Pancrealipase (Cotazym) class and indication?
- Class: Enzyme replacement
- Indication: Aids digestion in children with CF
- AD--N/D, abdominal cramps, hypersensitivity
- – 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