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Family Health Exam 1-4


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What is PKU?
Phenylketonuria (PKU) is a genetic disorder that is characterized by an inability of the body to utilize the essential amino acid, phenylalanine.

Some phenylalanine is needed for normal growth. This requires a diet that has some phenylalanine but in much lower amounts than normal. High protein foods, such as: meat, fish, poultry, eggs, cheese, milk, dried beans, and peas are avoided. Instead, measured amounts of cereals, starches, fruits, and vegetables, along with a milk substitute are usually recommended.
What is the treatment for scarlet fever?
A child with severe strep throat may find that eating is painful, so providing soft foods or a liquid diet may be necessary. Include soothing teas and warm nutritious soups, or cool soft drinks, milkshakes, and ice cream. Make sure that the child drinks plenty of fluids.

Use a cool-mist humidifier to add moisture to the air, since this will help soothe the sore throat. A moist warm towel may help to soothe swollen glands around your child's neck.

If the rash itches, make sure that your child's fingernails are trimmed short so that he or she doesn't damage the skin through scratching.
What are some presumptive signs of pregnancy?
- Breast changes
- Amenorrhea
- Nausea, vomiting
- Urinary frequency
- Fatigue
- Quickening
What are some probable signs of pregnancy?
- Goodell's Sign
- Chadwick's sign
- Hegar's sign
- Positive pregnancy test
- Braxton Hicks contractions
- Ballottement
What are some positive signs of pregnancy?
- ultrasound, x-ray
- Fetal movements palpated
- Visible
When is the Hep B vaccine given?
At birth - 2 months, 1 - 4 months, and 6 - 18 months.
How are respiratory infections described?
According to their anatomic area of involvement.
What does the upper respiratory tract consist of?
Oronasopharynx (nose), pharynx, larynx, and upper part of the trachea.
What does the lower respiratory tract consist of?
The lower trachea, bronchi, bronchioles, and alveoli.
What is croup?
Croup is a general term applied to a symptom complex characterized by horseness, a resonant cough described as barking or brassy, varying degrees of inspiratory stridor, and varying degrees of respiratoy distress resulting from swelling or obstruction in the region of the larynx.
What are some infectious agents that the repiratory tract is subject to?
- Strep
- Staphylococci
- influenza
- pneumococci
What is RSV?
Respiratory syncytial virus (RSV) is the most common cause of bronchiolitis and pneumonia among infants and children under 1 year of age. Illness begins most frequently with fever, runny nose, cough, and sometimes wheezing. During their first RSV infection, between 25% and 40% of infants and young children have signs or symptoms of bronchiolitis or pneumonia, and 0.5% to 2% require hospitalization. Most children recover from illness in 8 to 15 days. The majority of children hospitalized for RSV infection are under 6 months of age. RSV also causes repeated infections throughout life, usually associated with moderate-to-severe cold-like symptoms; however, severe lower respiratory tract disease may occur at any age, especially among the elderly or among those with compromised cardiac, pulmonary, or immune systems.
Infants and infection rate
Infants younger than 3 months have a lower infection rate presumably because of the protective function of the maternal antibodies.
Infants 3-6 months and infection rate
The infection rate increases due to the time between the disappearance of maternal antibodies and the infant's own antibody production.
Toddler and preschool infection rate
High infection rate.
Infection rate by 5 years old
Viral respiratory infections are less frequent, but pneumonia and strep increases.
How do the anatomical differences in children influence the response to respiratory tract infections?
The diameter of airways are smaller, and distances between structures are shorter, and organisms may move rapidly down the resp. tract, causing more extensive involvement, they can also access the middle ear more easily due to short eustachian tubes.
How do young children normally breathe?
Through their nose.
What conditions decrease resistance to infection?
Deficiencies in the immune system, malnutrition, anemia, fatigue, chilling of the body.
What conditions weaken the defenses of the respiratory tract and predispose children to infection?
Allergies, asthma, cardiac anomalies that cause pulmonary congestion, and cystic fibrosis.
What increases the likelihood of infection in children?
Day care and if the caregivers smoke.
What infections occur during the winter and spring months?
Common respiratory pathogens, "RSV Season"
What infections occur during autumn and early winter?
Mycoplasmal infections.
What are Mycoplasmal infections?
M pneumoniae is perhaps best known as the cause of walking or atypical pneumonia, but the most frequent clinical syndrome caused by this organism actually is tracheobronchitis or bronchiolitis, often accompanied by upper respiratory tract manifestations. Pneumonia develops in only 5-10% of persons who are infected. Acute pharyngitis and myringitis are less common.
What infections occur during cold weathers?
Infection-related asthma
Where are retractions sites and how severe are they?
- supraclavicular: severe
- suprasternal: severe
- intercostal: mild
- substernal: ↑severity
- subcostal: ↑severity
What is the most common type of retractions?
How do infants and small children react to respiratory tract infections (clinical manifestations)?
Those children between 6 months and 3 years react more severely than older children.
What are some generalized symptoms for young children?
Fever, anorexia, vomiting, diarrhea, abdominal pain, cough, sore throat, nasal blockage or discharge, and respiratory sounds.
Nursing interventions for respiratory tract infections
- Ease respiratory effort
- Fever management
- Promote rest and comfort
- Infection control
- Promote hydration and nutrition
- Family support and teaching
How can you ease respiratory effort in children?
Nebulizer, give O2, chest PT, humidifiers, raise the head of the bed.
What are some nursing diagnoses for resp infections?
- ineffective breathing pattern r/t inflammatory process
- Ineffective airway clearance r/t inflammation, ↑secretions & obstruction
- Risk for spread of infection r/t...
- Activity intolerance r/t inflammatory process, O2 imbalance
- Fear/anxiety r/t diff. breathing
What are some kinds of upper respiratory tract infections?
- Nasopharyngitis
- Pharyngitis
- Tonsillitis
- Influenza
- Otitis Media
- Croup Syndromes
What is nasopharyngitis?
The "common cold". It's caused by rhinovirus, RSV, adenovirus, influenza, and parainfluenza virus.
What is/causes Pharyngitis?
Pharyngitis is an inflammation of the pharynx that frequently results in a sore throat. It may be caused by a variety of microorganisms. Pharyngitis is caused by a variety of microorganisms. Most cases are caused by a virus, including the virus causing the common cold, flu (influenza virus), adenovirus, mononucleosis, HIV, and various others. Bacterial causes include Group A streptococcus, which causes strep throat.
What are the signs of Pharyngitis?
- Sore throat
- Strep throat may be accompanied by fever, headache, and swollen lymph nodes in the neck
- Viral pharyngitis may be associated with runny nose and postnasal drip
- Severe cases may be accompanied by difficulty swallowing and, rarely, difficulty breathing
- Additional symptoms are dependent on the underlying microorganisms
What is the treatment for pharyngitis?
Analgesics, antipyretics, penicillin, and discarding the old toothbrush.
Tonsillitis is an inflammation of the tonsils, the fleshy clusters of tissue on both sides of the back of the throat that fight off germs that enter the body through the mouth. The tonsils become enlarged and red, and can be coated with a yellow, gray, or white substance. An affected child typically has a sore throat, fever, swollen glands in the neck, and trouble swallowing.
How is Tonsillitis treated?
How doctors treat tonsillitis depends on whether it was caused by a virus or by group A streptococci bacteria. Doctors often can tell the difference just by looking at the tonsils, and can detect strep bacteria with a rapid strep test or a throat culture.

If your child's tonsillitis is caused by a virus, the body will fight off the infection on its own. If it's caused by strep bacteria, the doctor probably will prescribe an antibiotic. If so, make sure that your child completes the full course of treatment to prevent the development of any complications.

If your child gets frequent bouts of tonsillitis (more than 5 to 7 times during a 12-month period) or repeat infections over several years, the doctor may consider a tonsillectomy to remove the tonsils.
Nursing considerations for tonsillitis
A child with tonsillitis needs plenty of nourishment and rest. If your child finds swallowing so painful that eating is difficult, try serving liquids and soft foods, like nutritious soups, milkshakes, smoothies, popsicles, or ice cream.

Make sure that your child drinks lots of fluids and gets plenty of rest, and take his or her temperature regularly. Use a nonprescription pain reliever, such as acetaminophen or ibuprofen, for throat pain.

All forms of tonsillitis are contagious. Tonsillitis usually spreads from person to person by contact with the throat or nasal fluids of someone who is already infected. Be sure to keep your sick child's drinking glasses and eating utensils separate, and wash them in hot, soapy water. All family members should wash their hands frequently. After a bout of tonsillitis that's caused by the strep bacteria, throw out your child's toothbrush and replace it with a new one.
Symptoms of Influenza
Fever of 100 °F(37.8 °C) to 104 °F(40 °C), which can reach 106 °F(41 °C) when symptoms first develop. Fever is usually continuous, but it may come and go. Fever may be lower in older adults than in children and younger adults.
Shaking chills.
Body aches and muscle pain (often severe), commonly in the back, arms, or legs.
Pain when you move your eyes.
Fatigue, a general feeling of sickness (malaise), and loss of appetite.
A dry cough, runny nose, and a dry or sore throat. You may not notice these during the first few days of the illness when other symptoms are more severe. As your fever goes away, these symptoms may become more evident.
How influenza managed?
In most healthy people, influenza will go away in 7 to 10 days. The worst symptoms usually last 3 to 4 days. Home treatment to ease symptoms and prevent complications is usually all that is needed.

Antiviral medications can be taken to:
Reduce the severity and duration of symptoms caused by infection with influenza A or B virus.
Shorten the length of the illness.
Control outbreaks of the flu in nursing homes.
Reduce the spread of the virus to people at high risk for severe complications of the flu (high-risk groups).
Reduce complications from the flu.
What are children usually treated with for the flu?
Acetaminophen or ibuprophen for fever and fluids to maintain hydration.
What should not be given to children with the flu? Why?
The should not be given aspirin b/c of its possible link with Reye syndrome.
What is the greatest danger to children with the flu?
Developing a secondary infection
What is a prolonged fever or appearance of fever during early convalescence a sign of?
It's a sign of secondary bacterial infection and should be reported to the practitioner for antibiotic therapy.
What is one of the most prevalent diseases of early childhood?
Otitis media
When is Otitis media most prevalent?
the winter
Who does Otitis media affect?
<24 months: affects the most
5-6yrs: slight increase due to school
<7 years: occurs infrequently

Generally decreases with age and affects more boys than girls.
What 2 viruses precipitate OM?
RSV and influenza.
What causes OM?
Etiology is unknown but may be caused by blocked eustachian tubes and breastfed infants have a lower incidence.
What are symptoms of OM?
An older child may complain of an earache, but a younger child may tug at the ear or simply act irritable and cry more than usual, a child may eat less than the normal amount or have trouble sleeping.
What is done for OM?
Antibiotics are not initially given, but given to children with persistent effusion for more than 3 months. Child usually has to wait 72 hours before antibiotics are given. Infants less than 6 months are usually treated immediately with antibiotics.
What is a myringotomy and why is it done?
It's a surgical incision of the eardrum which alleviate pressure and reduce pain.
What other surgical procedures are done to treat OM?
Tympanostomy tube placement and adenoidectomy are surgical procedures that may be done to treat recurrent OM.
What are tympanostomy tubes?
They are pressure-equalizer tubes that facilitate continued drainage of fluid and allow ventilation of the middle ear.
What are some nursing objectives for AOM?
relieving pain, drainiage, preventing complications or recurrence, education, and family support.
How is OM prevented?
Upright bottle feeding/breast-feeding and eliminating tobacco smoke and known allergens from the environment.
What do croup syndromes affect?
They can affect the larynx, trachea, and bronchi. They are described according to the primary anatomic area affected.
Acute Epiglottitis
A serious obstructive inflammatory process that occurs predominantly in children 2-5 years of age but can occur at any age.
Do LTB and epiglottitis occur together?
Clinical manifestations of epiglottitis
Child usually goes to sleep without symptoms and awaked with sore throat and pain when swallowing. The child has a fever, tripod positioning, drooling, sternal retractions, inspiratory stridor, mild hypoxia, and distress.
When should throat inspection for epiglottitis be done?
When intubation equipment is present as well as staff.
What is hypercapnia?
A condition where there is too much carbon dioxide (CO2) in the blood.
What does the progressive obstruction of epiglottitis lead to?
Hypoxia, hypercapnia, and acidosis followed by decreased muscle tone, reduced level of consciousness, and eventually death.
What is the treatment for epiglottitis?
Children are given antibiotics via IV.
How is epiglottitis prevented?
The Hib vaccine.
Who gets acute laryngitis more often?
It's more common in older children and adolescents.
What causes laryngitis and what is the chief complaint?
It's caused by a virus and the chief complaint is usually hoarseness.
How is laryngitis treated?
Treatment is symptomatic with fluids and humidified air.
What is acute LTB?
It's acute laryngotracheobronchitis.
What is the most common croup syndrome?
Who is primarily affected by acute LTB?
It primarily affects children less than 5 years of age.
What organisms are responsible for Acute LTB?
RSV, parainfluenza virus, Mycoplasma pneumoniae, influenza A and B
What are the mainfestations of LTB?
- Inspiratory stridor
- Suprasternal retractions
- Barking or “seal-like” cough
- Increasing respiratory distress and hypoxia
- Can progress to respiratory acidosis, respiratory failure, and death
Therapeutic management of LTB
- Airway management
- Maintain hydration—PO or IV
- High humidity with cool mist
- Nebulizer treatments: epinephrine and steroids
Acute Spasmodic Laryngitis
AKA spasmodic croup or midnight croup. Paroxysmal attacks of laryngeal obstruction. Occur chiefly at night. Inflammation: mild or absent. Most often affects children ages 1-3
A severe attack or a sudden increase in intensity of a disease, usually recurring periodically.
Therapeutic management of acute spasmodic laryngitis
Cool mist, warm mist from shower, sudden exposure to cold, sleep in humidified air.
Bacterial Tracheitis
An infection of the mucosa of the upper trachea, is a distinct entity with features of both croup and epiglottitis. Occurs in children 1 month to 6 years of age. Could be a complication of LTB.
Clinical manifestations of bacterial tracheitis
Similar to LTB but unresponsive to LTB therapy. Thick, purulent secretions result in respiratory distress
Therapeutic Management of bacterial tracheitis
Humidified oxygen, antipyretics, antibiotics, suctioning, may require intubation
Infections of the Lower Airways
- Considered the “reactive” portion of the lower respiratory tract
- Includes bronchi and bronchioles
- Cartilaginous support not fully developed until adolescence
- Constriction of airways
How many lobes does the left lung have?
How many lobes does the right lung have?
(AKA tracheobronchitis) Inflammation of the large airways (trachea and bronchi), frequently associated with a URI.
Causes of Bronchitis
Viral agents are the primary cause of the disease, although M. pneumoniae is a common cause in children older than 6 years of age.
Clinical manifestations of Bronchitis
A dry hacking, nonproductive cough that worsens at night and becomes productive in 2 to 3 days.
Treatment for Bronchitis
It's a mild, self-limiting disease that requires only symptomatic treatment, including analgesics, antipyretics, and humidity. Recovery is usually with 5 to 10 days.
Bronchiolitis and RSV
Bronchiolitis is an acute viral infection with maximum effect at the bronchiolar level. Infection is rare in children older than 2 years of age.

Respiratory syncytial virus (RSV) is responsible for 80% or more of the cases during epidemic periods.
What is considered the single most important respiratory pathogen in infancy and early childhood? When does it occur? How is it spread?
RSV. Begins in late fall and reaches a peak during winter, and decreases in spring. It's easily spread from hand to eye, nose, or other mucous membranes.
Pathophysiology of RSV
The bronchiole mucosa swell, and lumina are filled with mucus and exudate. Varying degress of obstruction produced in the small air passages lead to hyperinflation, obstructive emphysema resulting from partial obstruction, and patchy areas of atelactasis. Air can get in but not out, causing progressive overinflation.
Clinical manifestations of bronchiolitis and RSV
Begins as a URI with runny nose, low-grade fever, OM and conjunctivitis may also be present. In time a cough develops and it can develop into a respiratory tract infection. Can lead to respiratory acidosis and hypoxemia.
How is RSV diagnosed?
By either ELISA, IFA from aspiration.
How are RSV and bronchiolitis treated?
It's treated with humidity, fluid intake, and rest. NO MILK!
How is RSV prevented?
- RSV immune globulin
- palivizumab
RSV immune globulin
An IV preparation of immunoglobulin G that provides neutralizing antibodies against RSV. Given in a monthly IV infusion beginning just before onset of the RSV season.
A monoclonal antibody is given monthly in an IM injection.
Who should receive RSV immune globulin and palivizumab?
Those children younger than 24 months that have chronic lung disease (CLD) or infants born earlier than 32 (35 weeks is the upper limit due to insurance) weeks.
Nursing care management for RSV
Isolate with other RSV-infected children. Use contact precautions.
How are pneumonias classified?
By location and organism.
Types of pneumonias
- Lobar pneumonia
- Bronchopneumonia
- Interstitial pneumonia
- Pneumonitis
Etiology of pneumonias
They are either viral or bacterial or aspiration.
What is an atypical pneumonia?
PCP or in HIV patients
How are pneumonias treated?
Steroids, antibiotics, fluids, fever management, and anti-inflammatories.
Pertussis (Whooping Cough)
Those not immunized are at risk as well as those underimmunized.
What is Pertussis caused by? And when?
Bordetella pertussis, most often in children who have not been immunized. Highest in spring and summer.
What is the treatment for pertussis?
Penicillin (antibiotic)
What is Respiratory Tuberculosis (TB) caused by?
Caused by Mycobacterium tuberculosis human and bovine varieties
How is TB transmitted?
It's airborne (coughing, breathing)
Clinical manifestations of TB
Fever, night sweats, weight loss (anorexia)
What is the recommended TB test?
Mantoux test. Uses purified protein derivative (PPD)
Therapeutic Management of TB
- Rifampin
- 6-month regimen
- Multidrug resistant-streptomycin IM
- Prophylaxis for high-risk patient: INH 9-12 months
What is a positive mantoux test reaction?
6mm or greater indicates a positive reaction.
Who is most at risk for foreign body aspiration?
Children 1-3 yrs old.
The severity of the aspiration is determined by what?
The foreign object and the location.
What is done for diagnostic evaluation of the aspiration?
endoscopy or x-ray
What are the signs for a child in distress due to foreign body aspiration?
1. Cannot speak, 2. becomes cyanotic, 3. collapses.
Aspiration Pneumonia
Occurrs when a child aspirates food or fluids or a foreign body. Usually occurs with kids that have a feeding problem, poor motor skills, an NG tube, poor feeders
What can be done to prevent aspiration?
Elevate the head of the bed and monitor them when they are being fed.
Children that are tube fed need some oral stimulation, why?
In order to prevent oral aversion.
Acute Respiratory Distress Syndrome (ARDS)
- Also referred to as adult RDS.
- Characterized as respiratory distress and hypoxia within 72 hours after serious injury or surgery in person with previously normal lungs
What's the treatment for ARDS?
O2, antibiotics (if there's an infectious agent)
What does the prognosis for ARDS depend upon?
When the distress is found, how quickly treatments were put into play and then given O2
Nursing care for ARDS
O2, maintain an open airway, antibiotics.
What does the severity of an inhalation injury depend upon?
Severity depends on nature of substance, environment, and duration of contact. Also whether the injury was local or systemic.
Therapeutic management of inhalation injury
ABGs, O2, cool mist
Nursing considerations for inhalation injury
airway, antibiotics, fluids
What makes up ABGs?
pH, PCO2, PO2, SaO2, HCO3
What's the range for pH?
7.35 - 7.45
What's the range for pCO2?
35-45 mmHg

Partial pressure of CO2 in blood. Outside of range indicates respiratory.
What's the range for PO2?
80-100 mmHg

Partial pressure O2 delivered by lungs to blood.
What's the range for SaO2?

Extent to which hemoglobin saturated with O2. Affected by pH, pCO2, and temp.
What's the range for HCO3?
22-28 meq/d

Regulated by kidneys. Indicates metabolic acidosis or alkalosis.
- Chronic inflammatory disorder of airways
- Bronchial hyper-responsiveness
- Episodic
- Limited airflow or obstruction that reverses spontaneously or with treatment
What can cause an asthma episode?
Allergies, irritants, smoking, cold air, bacteria, mold spurs, activity can cause an episode.
What is asthma?
Asthma is a chronic inflammatory disorder of the airways in which many cells play a role.
What are asthma episodes associated with?
Airflow limitation or obstruction that is reversible either spontaneously or with treatment.
What are the Asthma Severity Classifications in Children 5 Years and Older?
- Step I: mild, intermittent asthma
- Step II: mild, persistent asthma
- Step III: moderate, persistent asthma
- Step IV: severe, persistent asthma
What mechanisms are responsible for the obstructive symptoms in asthma?
1. Inflammation and edema of the mucous membranes.
2. Accumulation of tenacious secretions from mucous glands.
3. Spasm of the smooth muscle of the bronchi and bronchioles, which decreases the caliber of the bronchioles.
What is the drug therapy for asthma?
- LT control meds
- Quick relief meds (rescue drugs)
- MDI (Metered dose inhaler, should always have a spacer)
- Corticosteroids
- Cromolyn sodium
- Albuterol, metaproterenol, terbutaline
- LT bronchodilators (Serevent)
- Theophylline—monitor serum levels (seldom used)
- Leukotriene modifiers
- Others
A short-acting β2-adrenergic receptor agonist used for the relief of bronchospasm in conditions such as asthma and COPD. It is is usually given by the inhaled route for direct effect on bronchial smooth muscle. This is usually achieved through a metered dose inhaler (MDI), nebuliser or other proprietary delivery devices.

Treats acute exacerbations. Helps to prevent exercise induced bronchospasms, given 3 or 4 times a day.
Asthma Interventions
- Exercise
- Chest physiotherapy (CPT)
- Hyposensitization (trying to figure out what triggers a child's asthma)
Status Asthmaticus
Worst kind of asthma, has suprasternal retractions:
- Respiratory distress continues despite vigorous therapeutic measures
- Emergency treatment: epinephrine 0.01 ml/kg subQ (max dose 0.3 ml)
- Concurrent infection in some cases
It triggers vasodilation, it also dries up secretions, & opens airways
Goals of Asthma Management
- Avoid exacerbation
- Avoid allergens
- Relieve asthmatic episodes promptly
- Relieve bronchospasm
- Monitor function with peak flow meter**
- Self-management of inhalers, devices, and activity regulation
What are peak flow meters?
A peak flow meter is a device that measures how well air moves out of your lungs. During an asthma episode the airways of the lungs begin to narrow slowly. The peak flow meter can be used to find out if there is narrowing in the airways hours, even days, before you have any symptoms of asthma. By taking your medicine early (before symptoms) you may be able to stop the episode quickly and avoid a serious episode of asthma. Peak flow meters are used to check your asthma the way that blood pressure cuffs are used to check high blood pressure.
Cystic Fibrosis (CF)
- Exocrine gland dysfunction that produces multisystem involvement (affects respiratory and GI)
- Most common lethal genetic illness among white children
- Approximately 3% of U.S. white population are symptom-free carriers
Etiology of CF
- Autosomal recessive trait
- Inherits defective gene from both parents with an overall incidence of 1:4
What do the exocrine glands do?
They secrete mucous in lungs and GI system
Pathophysiology of CF
Characterized by several clinical features: increased viscosity of mucous gland secretions (and obstructions), a striking elevation of sweat electrolytes, an increase in several organic and enzymatic constituents of saliva, and abnormalities in autonomic nervous system function.
Increased Viscosity of Mucous Gland Secretion (CF)
- Results in mechanical obstruction
- Thick inspissated mucoprotein accumulates, dilates, precipitates, coagulates to form concentrations in glands and ducts
- Respiratory tract and pancreas are predominantly affected
What are some interventions for CF?
O2, chest PT, suctioning, nebulizers, antibiotics, & need pancreatic enzymes
What is portal hypertension?
Normally, blood is carried to the liver by a major blood vessel called the portal vein. If blood can’t flow easily through the liver because of cirrhosis, the blood gets slowed down in this vein and the pressure inside the vein increases. This higher blood pressure in the portal vein is called portal hypertension.

If blood can’t flow normally through the portal vein, it must return to the heart using other blood vessels. These vessels become swollen because of the increased amount of blood flowing through them. They are called varices. Varices have thin walls and can easily break open because they aren’t meant to handle such high-pressure blood flow. Bleeding from a broken blood vessel is serious and can even be fatal.
CF: Increased Sweat Electrolytes
- Basis of the most reliable diagnostic procedure—sweat chloride test
- Sodium and chloride will be 2 to 5 times greater than the controls
CF: Other Factors
- Increased organic/enzymatic constituents of saliva
- Abnormalities of the autonomic nervous system (decreases developmental milestones)
CF: Respiratory Manifestations
- Present in almost all CF patients but onset/extent is variable
- Stagnation of mucus and bacterial colonization result in destruction of lung tissue
- Tenacious secretions are difficult to expectorate—obstruct bronchi/bronchioles
- Decreased O2/CO2 exchange (increased CO2)
- Results in hypoxia, hypercapnea, acidosis
- Compression of pulmonary blood vessels and progressive lung dysfunction lead to pulmonary hypertension, cor pulmonale, respiratory failure, and death
How much O2 do you give a CF patient?
Not more than 2 liters, it could decrease their drive to breathe.
CF: Respiratory Progression
- Gradual progression follows chronic infection
- Bronchial epithelium is destroyed
- Infection spreads to peribronchial tissues, weakening bronchial walls
Peribronchial fibrosis
- Decreased O2/CO2 exchange
CF: Further Respiratory Progression
- Chronic hypoxemia causes contraction/hypertrophy of muscle fibers in pulmonary arteries/arterioles
- Pulmonary hypertension
- Cor pulmonale
- Pneumothorax
- Hemoptysis
What is Hemoptysis?
The coughing up blood from the respiratory tract. The blood can come from the nose, mouth, throat, the airway passages leading to the lungs, or the lungs. The word "hemoptysis" comes from the Greek "haima," meaning "blood," and "ptysis," which means "a spitting".
CF: GI Tract
- Thick secretions block ducts—cystic dilation—degeneration—diffuse fibrosis
- Prevents pancreatic enzymes from reaching duodenum
- Impaired digestion/absorption of fat—steatorrhea
- Impaired digestion/absorption of protein—azotorrhea
- Endocrine function of pancreas initially stays unchanged
- Eventually pancreatic fibrosis occurs (near last stages of CF); may result in diabetes mellitus
- Focal biliary obstruction results in multilobular biliary cirrhosis
- Impaired salivation
What is WOB?
work of breathing
What is Apnea?
absence of breathing
What is Respiratory insufficiency?
The condition in which the lungs cannot take in sufficient oxygen or expell sufficient carbon dioxide to meet the needs of the cells of the body. Also called pulmonary insufficiency.
What is Respiratory arrest?
cessation of respiration
CF: Clinical Manifestations of GI Tract
- Pancreatic enzyme deficiency
- Progressive COPD associated with infection
- Sweat gland dysfunction
- Failure to thrive
- Increased weight loss despite increased appetite (always hungry)
- Gradual respiratory deterioration
CF: Presentation
- Wheezing respiration, dry nonproductive 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
- Wasting of tissues
- Prolapse of the rectum
- Delayed puberty in females
- Sterility in males
- Parents report children taste “salty”
- Dehydration
- Hyponatremic/hypochloremic alkalosis
- Hypoalbuminemia
CF: Diagnostic Evaluation
- Quantitative sweat chloride test
- Chest x-ray
- PFT (pulmonary fx test)
- Stool fat and/or enzyme analysis
- Barium enema
CF: Goals
- Prevent/minimize pulmonary complications
- Adequate nutrition for growth
- Assist in adapting to chronic illness
CF: Respiratory Management
- Bronchodilator medication
- Forced expiration
- Aggressive treatment of pulmonary infections
- Home IV antibiotic therapy
- Aerosolized antibiotics

Look for:
- Pneumothorax
- Hemoptysis
- Nasal polyps
- Steroid use/nonsteroidal anti-inflammatory
- Transplantation
CF: GI Management
- Replacement of pancreatic enzymes
- High-protein, high-calorie diet as much as 150% RDA
- Intestinal obstruction
- Reduction of rectal prolapse
- Salt supplementation
Prognosis of CF
- Estimated life expectancy for child born with CF in 2003 is 40 to 50 years
- Maximize health potential:
Nutrition, Prevention/early aggressive treatment of infection, Pulmonary hygiene
- New research—hope for the future: Gene therapy, lateral lung transplants, Improved pharmacologic agents
CF: Family Support
- Coping with emotional needs of child and family
- Child requires treatments multiple times a day
- Frequent hospitalization
- Implications of genetic transmission of disease
GI: Nutritional Disturbances
- Vitamin deficiencies
- Mineral Disturbances
- Vegetarian diets
- Protein and Energy Malnutrition
- Food Sensitivity
- Obesity
- Anorexia/bulimia
Clinical Manifestations of GI Dysfunction
- Failure to thrive
- Spitting up/regurgitation
- Nausea, vomiting, diarrhea, constipation
- Abdominal pain, distention, GI bleeding
- Jaundice
- Dysphagia
- Hypoactive, hyperactive, or absent bowel sounds
Types of dehydration
- Isotonic
- Hypotonic
- Hypertonic
Definition of dehydration
Output exceeds input
Isotonic dehydration
This is the primary form of dehydration in children, occurs in conditions in which electrolyte and water deficits are present in approximately balanced proportions.
Hypotonic dehydration
When the electrolyte deficit exceeds the water deficit. (Decreased sodium)
Hypertonic dehydration
Results from water loss in excess of electrolyte loss and is usually caused by a proportionately larger loss of water or a larger intake of electrolytes. This is the most dangerous. (Having water loss but a high potassium level)
Therapeutic management and Nursing consideration
Monitor intake and output (all)
- Acute diarrhea is leading cause of illness in children <5 years
- 20% of all deaths in developing countries are related to diarrhea and dehydration
- Acute infectious diarrhea: variety of causative organisms
Types of diarrhea
- acute diarrhea
- chronic diarrhea
- intractable diarrhea of infancy
- chronic nonspecific diarrhea (CNSD
How is diarrhea classified?
As either acute or chronic
How is chronic diarrhea defined?
As an increase in stool frequency and increased water content with a duration of more than 14 days.
What do diarrhea disturbances involve and what are they called?
- the stomach and intestines (gastroenteritis)
- the small intestine (enteritis)
- the colon (colitis)
- the colon and intestines entercolitis)
What organism is the most common cause of diarrhea in children under 5 years old? How is it spread? What other organism causes a lot of diarrhea?
The rotavirus. It's spread via the fecal-oral route.

C. Difficile.
What is the etiology of diarrhea?
It's always related to some bacteria or virus.
What are the major goals in the management of acute diarrhea?
- assessment of fluid and electrolyte imbalance
- rehydration
- maintenance fluid therapy
- reintroduction of an adequate diet
How should children with diarrhea and dehydration first be treated?
With oral rehydration therapy (ORT). Oral rehydration solutions (ORS) enhance and promote the reabsorption of sodium and water, reduce vomiting, volume loss from diarrhea, and duration of the illness.
What is not encouraged when children have diarrhea?
Clear fluids by mouth such as fruit juices, carbonated soft drinks, and gelatin as these fluids have high carbohydrate content, a low electrolyte content, and a high osmolality. A BRAT diet is contraindicated.
Care of a child with diarrhea includes what?
- strict I&Os
- rehydration
- diet as tolerated
- handwashing
- monitor for dehydration, fluid and electrolytes
What is constipation?
An alteration in frequency, consistency, or ease of passage of stool. It can be idiopathic.
What is constipation usually due to?
Environmental or psychosocial problems. Medications can also cause diarrhea.
What also tends to bind children?
When should the first meconium be passed? If not, what should the infant be assessed for?
within the first 24 - 36 hours.

If not the child should be assessed for Hirschsprung disease, hypothyroidism, meconium plug, and meconium ileus (CF).
Hirschsprung's Disease
a rare congenital (present at birth) abnormality that results in obstruction because the intestines do not work normally. It is most often found in males. It is commonly found in Down Syndrome children. It can be life-threatening or a chronic disorder. The inadequate motility is a result of an aganglionic (without nerve tissue) section of the intestines resulting in megacolon (dilated section of colon).
Hypothyroidism is a condition in which the body lacks sufficient thyroid hormone. Since the main purpose of thyroid hormone is to "run the body's metabolism", it is understandable that people with this condition will have symptoms associated with a slow metabolism.
Constipation in infancy
- Often related to diet
- Constipation in exclusively breastfed infant almost unknown: infrequent stool may occur because of minimal residue from digested breast milk
- Formula-fed infants may develop constipation
Constipation in Childhood
- Often due to environmental changes or control over body functions
- Encopresis: inappropriate passage of feces, often with soiling
- May result from stress
Encopresis is a complication of chronic constipation, and it is fecal soiling with the loss of semi-formed or usually liquid stools, which may be foul smelling, into a child's underwear. Once a child becomes constipated and has hard and painful stools, he will then begin to hold in his bowel movements to prevent it from hurting again. This creates a cycle that makes the constipation continue and become worse, eventually leading to a large fecal impaction and rectal distention (which can make the rectum less sensitive and unable to hold even small amounts of stool). Stool behind the impaction begins to leak around it, and eventually leaks out of the rectum, without the child noticing it or being able to hold it in.
Management of constipation in childhood
In childhood the diet should contain fiber and fluid.

During infancy, simply increasing the carbohydrate (sugar or corn syrup) in the infant's formula will often relieve the problem.
Nursing Considerations for constipation
- History of bowel patterns, medications, diet
- Educate parents and child
- Dietary modifications (age appropriate)
Clinical Manifestations of Hirschprung
- Aganglionic segment usually includes the rectum and proximal colon
- Accumulation of stool with distention
- Failure of internal anal sphincter to relax
- Enterocolitis may occur
Therapeutic management of Hirschsprung disease
Treatment is primarily surgical to remove the aganglionic portion of the bowel to relieve obstruction. Sometimes a temporary ostomy is created and then the surgery is performed.
Preoperative care for Hirschsprung disease
- empty bowel
- increase child's nutritional status
- children preschool age and above need to be told about the colostomy procedure in concrete terms, with the use of visual aids.
Postoperative care for Hirschsprung disease
stomal care is a major nursing task.
What is done for vomiting?
- hydration
- administer zofran (or another antiemetic)
- the key is to prevent dehydration
Gastroesophageal Reflux (GER)
- Defined as transfer of gastric contents into the esophagus
- Occurs in everyone
- Frequency and persistency may make it abnormal
- May occur without GERD
- GERD may occur without regurgitation
What is done to assess for GER?
an upper GI is used to look for anatomical anomalies.
Therapeutic management of GER
No therapy is needed for an infant who is thriving and has no resp complications. Some may need frequent feeding of thickened formula. Or if this doesn't work a child may go on to prilosec, pepsid, or zantac type med.

Head of the bed can be raised when sleeping, etc.
Acute Appendicitis
Inflammation of the vermiform appendix (blind end at the end of the cecum), is the most common cause of emergency abdominal surgery in childhood.

Average age of child is 10 yrs.
Etiology of Acute Appendicitis
Most often due to obstruction of the lumen of the appendix, usually by hardened fecal material.
Pathophysiology of acute appendicitis
the outflow of mucous secretions is blocked and pressure builds within the lumen, resulting in compression of the blood vessels. The resulting ischemia is followed by ulceration of the epithelial lining and bacterial invasion. Subsequent necrosis causes perforation or rupture.
Classic signs of Acute Appendicitis
The most intense site of pain may be at McBurney Point, located at a point midway between the anterior superior iliac crest and the umbilicus.

Other signs include fever, a change in behavior, anorexia, and vomiting. A white lood cell count greater than 10,000/mm is common (but not necessary).
Therapeutic management of acute appendicitis
Before perforation: rehydration, antibiotics, and surgical removal of the appendix.

Ruptured appendix: IV admin of fluids & electrolytes, systemic antibiotics, and nasogastric suction.
is defined as inflammation of the peritoneum (the serous membrane which lines part of the abdominal cavity and some of the viscera it contains).
What are the differences between ulcerative colitis and crohn's disease?

UC v. Crohn's
Inflammatory Bowel Disease (IBD)
Includes ulcerative colitis (UC) and Crohn disease (CD)
What characterizes Inflammatory Bowel Disease (IBD)?
Gi symptoms, extraintestinal and systemic inflammatory responses, and exacerbations and remissions without complete resolution characterize these diseases.
What are some of the differences between UC and CD?
CD is more disabling, has more serious complications, and has less effective medical and surgical treatment than UC. Because UC is confined to the colon, theoretically it may be cured with a colectomy.
Etiology of IBD
It is proposed that IBD is the result of one or more environmental influences such as infectious organisms, dietary habits, and environmental toxins that promote disease in genetically susceptible individuals.
What is the pathophysiology of Crohn's disease?
The chronic inflammatory process of CD involves any part of the GI tract from the mouth to the anus, but most often affects the terminal ileum. The disease involves all layers of the bowel wall in a discontinuous fashion. The most common symptoms are abdominal pain with cramps, diarrhea, and weight loss.
Therapeutic management of Crohn's disease
The goals of therapy are to 1. control the inflammatory process to reduce or eliminate the symptoms, 2. obtain long-term remission, 3. promote normal growth and development, and 4. allow as normal a lifestyle as possible.
Diagnostic evaluation of crohn's disease and UC
Derived from history, upper GIs, and lab work.
Medical treatment and Nursing considerations for UC and CD
Typically kids are on long-term corticosteroids, antibiotics, need to provide nutritional support.
Peptic Ulcer Disease (PUD)
A peptic ulcer is an ulcer of one of those areas of the gastrointestinal tract that are usually acidic. A more general term, peptic ulcer disease (PUD), is also in use. Most ulcers are associated with Helicobacter pylori, a spiral-shaped bacterium that lives in the acidic environment of the stomach. Ulcers can also be caused or worsened by drugs such as Aspirin and other NSAIDs. Contrary to general belief, more peptic ulcers arise in the duodenum (first part of the small intestine, just after the stomach) than in the stomach. About 4 % of stomach ulcers are caused by a malignant tumour, so multiple biopsies are needed to make sure. Duodenal ulcers are generally benign.
Etiology and pathophysiology of PUD
The exact cause is unknown, although infectious, genetic, and environmental factors are important. There is an increased familial incidence, and the disease is increased in persons with blood group O.

The pathology is due to an imbalance between the destructive factors and defensive factors in the GI tract.
Diagnostic evaluation of PUD
Barium swallow, chest x-ray. An upper endoscopy is the most reliable procedure to diagnose PUD. A biopsy can also be taken.
Therapeutic management of PUD
Relieve discomfort, promote healing, prevent complications, and prevent recurrence. Management is primarily medical and consists of administration of medications to treat infection and to reduce or neutralize gastric acid secretion.
Nursing considerations for PUD
The primary nursing goal is to promote healing of the ulcer through compliance with the medical regimen. Stress should also be considered.
Acute Hepatitis
Hepatitis is the Latin word for liver inflammation. It is characterised by the destruction of a number of liver cells and the presence of inflammatory cells in the liver tissue.
What can cause hepatitis?
A virus, a chemical or drug reaction, or other diseases.
Hepatitis A
Is the most common form of acute viral hepatitis in most parts of the world. The virus produces a contagious disease transmitted primarily in contaminated stool (and shellfish) spread via the fecal-oral route from person to person. The highest incidence occurs among pre-school or school-age children younger than 15 years.
Hepatitis B
Infection can occur as an acute or a chronic infection and may range from being asymptomatic and limited to causing fatal fulminant (rapid and severe) hepatitis. Transmission is usually via the parenteral route through the exchange of blood or any bodily secretion or fluid.

Most HBV in fection in children is acquired perinatally.
Hepatitis C
7% of HCV-infected mothers transmit the HCV to their newborns. The second most common route is through transfusion of blood or blood products, transplantation of organs, or sharing used needles.
Concept of non-A, non-B hepatitis
Previously, viral hepatitis that was not caused by the type A or type B virus was called "non-A, non-B hepatitis." Recently three more viruses have been identified that cause some of these non-A, non-B infections. These new viruses are hepatitis C, D, or delta, and E.
Therapeutic management of hepatitis
Primarily symptom control and education on preventing transmission.
Prognosis of hepatitis
Depends on what else is going on with the patient. Depends on the type of virus involved.
Cleft Lip and/or Cleft Palate
- Facial malformations that occur during embryonic development
- May appear separately or together

These are the most common congenital deformities of the head and neck. Cleft palate is the most common cranial deformity (4th most common in US).
How is Cleft Lip and/or Cleft Palate prevented?
With Folilc Acid
When are Cleft Lip and Cleft Palate repaired?
Cleft lip repaired after 10 weeks.

- Closure of lip defect precedes correction of the palate
- Z-plasty to minimize retraction of scar
- Protect suture line with Logan bow or other methods
- Typically 12 to 18 months of age to repair cleft palate.
What is the biggest issue with Cleft Lip and/or Cleft Palate?
Feeding and respiration. At risk for aspiration, held upright, sometimes the formula is thickened, sent home with suction.
Cleft lip/palate effect on speech
Children usually have intense speech therapy and occupational therapy.

Prognosis is very good.
Nursing considerations for Cleft lip/palate post-surgery
Infection control, logan bow, feeding, pain management, fever control, therapies. Have an inability to suck before surgery. High-risk for Otitis Media, some conductive hearing loss. Increased risk for dental carries and gingivitis.
Esophageal Atresia and Tracheoesophageal Fistula (TEF)
Esophageal atresia is a disorder of the digestive system in which the esophagus does not develop properly. The disorder is a congenital defect, which means it occurs before birth. There are several types.

The esophagus is the tube that normally carries food from the mouth to the stomach. In most cases of esophageal atresia, the upper esophagus ends blindly and does not connect with the lower esophagus and stomach. In these cases, the top end of the lower esophagus is connected with the trachea (windpipe). This connection between the lower esophagus and the trachea is called a tracheoesophageal fistula (often abbreviated TEF).

Over 30% of people affected by this condition will also have other abnormalities, such as heart disorders or other gastrointestinal tract disorders. Other types of esophageal atresia involve narrowing of the esophagus and may also be associated with other birth defects.

This condition is frequently complicated by the infant's breathing saliva and secretions into the lungs, causing pneumonia, choking, and possibly death. It is considered a surgical emergency.

The disorder is usually detected shortly after birth when feeding is attempted and the infant coughs, chokes, and turns blue (cyanosis). Immediate surgical repair of this disorder is required so that the lungs are not damaged and the baby can be fed.
S/S of Esophageal Atresia andTracheoesophageal Fistula (TEF)
Cyanosis, choking, coughing. The have increased salivation (foaming).

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