Nursing104 exam 2
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Nursing104 exam 2
Child Cystic Fibrosis
Diagnosis, prevention, treatment
What are charecteristics of Cystic Fibrosis (CF)?
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
The gene causes the dysfunction of theexocrine glands.
Growth & Development from Moderate to severe CF?
: Increased O2 demands for pulmonary functioning
: Delayed bone growth
Failure to thrive
Increased wt loss despite an increased appetite
Gradual deterioration of respiratory system
Earliest postnatal manifestation of CF?
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?
oul smelling (compared to a cat's stool)
at containing “greasy”
Clinical Manifestations in CF?
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
: 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
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)
Marked tissue wasting
Failure to grow
Sallow skin, anemia, ADEK deficiency
Clinical Manifestations of Pulmonary Involvement in CF?
Stagnation of mucus in airways w/subsequent bacterial growth
: Wheezy respirations, dry nonproductive cough
: 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
w/Chest anteroposterior diameter-- enlarged
Repeated bronchitis, bronchiectasis and bronchopneumonia occur
Infectious Pathogens in CF?
Effects of Increased Viscosity of Mucus Glands Secretion in CF?
: Chronic bronchial pneumonia and Generalized obstructive emphysema
: Insytestinal Obstructive of Newborn
: Malabsorption Syndrome
: Portal Hypertension
Presentation of CF?
Wheezing respiration, dry non-productive cough
Generalized obstructive emphysema
Clubbing of fingers and toes
Repeated bronchitis and pneumonia
Distal intestinal obstruction syndrome
Excretion of undigested food in stool—increased bulk, frothy, and foul
Prolapse of the rectum
Delayed puberty in females
Sterility in males
Parents report children taste “salty”
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
Immunoreactive trypsinogen (IRT) analysis
erformed 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)
: <40 mEq/L with a mean of 18mEq/L
: >60 mEq/L: diagnostic of CF
Infants < 3 mos
: >40 mEq/L: highly suggestive CF
Stool fat analysis or enzyme analysis
Pulmonary testing for CF?
(show abnormal airway function)
: for diagnosing meconiumileus
What are the Goals for CF?
Adequate nutrition for growth
Assist in adapting to chronic illness
What are Therapeutic Managements for CF?
: 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
: aggressive CPT frequently; 2-4x per day
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
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
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
Growth & development-- expose to as many normal life experiences as possible
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
Implications of genetic transmission of disease
Prognosis of CF
Decreased life expectancy for child born withCF
Maximize health potential--
– 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
CF Pancrealipase (Cotazym)
class and indication?
: Enzyme replacement
: 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