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Peds Unit #2

Endocrine, Respiratory, Hematogolgical, Child Abuse

Difference in endocrine system at birth Less developed
Hormonal control of many body functions is lacking until what age? 12-18 months
Endocrine system imbalances include; Electrolytes, amino acids, glucose, fluid concentration, trace substances
Diagnostic Work Up & Tests for Endocrine Stressors Thyroid Fuction tests TSH & T4; Blood glucose, Growth hormones, Tanners sexual maturatio, DDST (Denver screening)
Galactosemia Can't digest milk or sugar. Lactose restricted diet (lifelong)
Tay Sachs Disease post 6 months; progressive neurologic deterioration & development delays
Maple syrup urine disease Affects metabolism of certain amino acids within 48-72 hrs post birth
Congenital Hypothyroidism cause defective embryonic development, decreased ocncentrations of T3 and T4
Signs & Symptoms of Congenital Hypothyroidism Large fontanels, poor linear growth, sleeps thru night, lethargic, difficulty feeding (thick tongue) prolonged jaundice, constipation, poor muscle tone, hoarse cry, anemia, cool skin, umbilical hernia, protruding belly
Treatment for congenital hypothyroidism Thyroid hormone replacement- levothyroxine (synthroid)10-15 mcg/kg/day - should see normalization of TSH at one month
Nursing Diagnosis for Cong. Hypothyroidism Ineffective thrmoregulation r/t decreased basal metabolic rate can influence the dosing of synthroid. Delayed G&D r/t disase process.
Congenital Hypothyroidism if untreated can result in Mental retardation
Acquired Hypothyroidism (hashimoto's disease) clinical manifestation depends on the extent of dysfunction and the age of the child at the onset.
Most common cause of hashimotos disease aka hypothyroidism thyroiditis associated with goiter. MR or neurological sequelae are not problem
S&S of hashimotos (hypothryoidism) Decelelerated growth, dry skin, puffiness around eyes, sparse hair, constipation, sleepiness & mental decline
Nursing Diagnosis for hypothyroidism Disturbed body image r/t wt. gain & obesity
Hashimotos Disease Treatment Thyroid hormone levothyroxine (synthroid). Administer increasing amts over 4-8 wks to avoid symptoms of hyperthyroidism that can occur w/treatment of chronic hypothyroidism. Monitor G&D
Hyperthyroidism (Graves Disease)facts; 4X more common in girls, occurs at 12-14yrs. Manifests gradually over 6-12mo. Follows a viral illness or period of stress.
Diagnosis of Hyperthyroidism (graves) Elevated thyroid function studies. TSH concentration suppressed or no measurable levels
Management of Hyperthyroidism (graves) Propythioracil (PTU); Methiamazole (MTZ, Tapazole)Subtotal thyroidectomy, ablation with radioiodine
Propythiroacil (PTU) Side effects skin rashes, urticaria, lympadenopathy, sore throat
Cardinal Signs of Graves Disease (hyperthyroidism) Emotional liability, physical restlessness, decreased performance at school, excessive appetite without wieght gain and fatigue.
Physical signs Tachycardia, Widened pulse pressure, dyspnea on exertion, exophthalmos, wide eyed expression with lid lag, tremor, goiter, warm, moist skin, accelerated linear growth, heat intolerence, fine hair, systolic murmors
Thyroid Storm Acute onset S&S; Severe irritability & restlessness, vomiting, diarrhea, hyperthermia, hypertension, severe tachycardia, prostration, progress to death
Nursing Care for Graves Disease Maintain a quiet, unstimulating environment- rest; maintain a regular routine to minimize stress of coping. Restrict physical activity. Increase need for calories, good hygience (sweating) monitor for neutropenia & hepatotoxicity
What to do if surgery is planned for Graves disease Administer iodine a few weeks before. mixed in a strong tasting fruit juice given thru a straw. Thyroid hormone for life after surgery.
Juvenile Diabetes (Type 1) Absolute insulin deficiency. Usually precipitated by a viral event.
S&S Juvenile Diabetes Polyphagia, polyuria,polydipsia, weight loss, enuresis or nocturia, irritability, shortened attention span, fatigue, dry skin, blurred vision, headache, poor wound healing, frequent infections, hyperglycemia, keosis, ketoacidosis, vaginal infections.
Acanthoisis nigricans dark pigmentation on neck line in pts with juvenile diabetes.
Serum Glucose levels...diagnosis Fasting Glucose >126 or 2 hr GTT >200
HgA1c normal levels vs. DM 1.8-4.0 normal, >6.0 = DM
Diet therapy for Diabetes No special food or supplements. 55% CHO, 15% protein and 30% fat; consume food at the same time each day.
Diet Therapy notes; Consistent intake & timing of food to time & action of insulin prescribed. CHO determined to fit the actiity pattern of each child and Fiber decreases the rise in blood glucose level after meals.
Exercise therapy for DM Encouraged, never restricted, lowers blood glucose levels, aids in the bodys use of food and decreases insulin requirements.
Insulin therapy Amt is based on capillary blood glucose levels. Admin q BID. Typical; short acting (regular) and int. acting (NPH) before breakfast & evening meal.
Insulin pumps Fixed amts of short acting insulin continuously. Worm on belt. Chg tubing & catheter q48hr Do not remove more than 1-2 hrs. Skin infections common, subject o malfunction. Must be self motivated.
Diabetes Insipidus defined A metabolic disorder caused by injury head trauma or surgery
Characteristics of Diabetes Insipidus Copious excretion of urine & excessive thirst, sudden onset.
Treatment of DI replace fluids, DDAVP (vasopressin), diuretics (vasopressin enhances absorption of water in kidneys)
Signs & symptoms of hypoglycemia Rapid onset, irritable, nervous, difficulty concentrating, shaky feeling, hunger, headache, dizziness, diplopilia, pallor, sweating, tachycardia, tremors, glucose <60mg/dl, normal blood & urine values.
Signs & symptoms of hyperglycemia Gradual onset, lethargic, dulled sensorium, confused, thirst, weakness, n/v, abdominal pain, signs of dehydration, deep, rapid respirations, fruity breath, diminished reflexes, glucose 250mg/dl, lg ketones in blood & urine, polyuria, blurred vision.
Hypoglycemia commonly occurs: Before meals or when the insulin effect is peaking, burst of physical activity without additional food, delayed, omitted, or incompletely consumed meals or snacks.
Treatment of hypoglycemia Simple concentrated sugar s/a; glucose tabs, hard candy, sugar cubes, low-fat milk, orange juice. Follow with complex carb & protein (slice of breat or cracker with peanut butter) Glucagon IM for severe hypoglycemia (may cause vomiting, prevent aspirate)
What is the honeymoon period? A newly diagnosed diabetic may show an initial regulation of blood glucose and may not need any insulin from one month to one year. Some md's continue the smallest amt of insulin so not to confuse parent & child.
Growth Hormone deficiency Decrease activity of pituitary gland. Caused by poor growth, short stature. Normal at birth by age 1 they drop below 3% on growth chart.
Growth Hormone deficiency is treated with; Replacement therapy of synthetic GH, sq injections 6-7 X week until growth plates close or child reaches accept. height or predicited final height.
What does a hand x-ray show? Gives information re; growth plates.
Otis Media peak incidence and definition One of the most common illnesses in infancy and childhood. Peaking at age 6mo to 6yrs. Infection or blockage of the middle ear. Acute, Chronic or Serious OM
Causes of fluid backing up the eustachian tubes of infants and children Short eustachian tube that is horizontal and not yet vertical; allergies, cold formula and milk (giving a bottle to bed)
Risks of development of AOM Second hand smoke, allergies, bottle fed infants
Acute Otis Media (AOM) S&S Sudden temp incr., sharp pain, earache (otalgia); pulling onear/face; bulging, opague red typanic membrane, irritability, sleep disturbance, crying, fever, vomiting, diarrhea, anorexia, sudden relief and discharge =rupture of membrane
Treatment for AOM Can be viral or bacterial; acetaminiphen (pain, fever) ABX (Amoxicillan) if bacterial an alternative = wait 72 hrs to see if it resolves then treat.
Serious Otis Media or Otisis Media w/effusion; Result of chronic OM (3 in 6 mo OR 4 in 1yr). Epithelial cells of middle ear begin producing secretions instead of absorbing them (the fluid stays there)
Patient teaching for Post Op (tubes) Monitor for ear drainage; report fever or increase in pain; avoid blowing nose for 7-10 days; swimming, showers allowed only w/earplugs; diving and swimming in deep water prohibited.
Pharyngitis (tonsillitis) defined peak age; Inflammation & infection of the palatine tonsils. Can be Viral vs Bacterial; peak age 4-7 yrs.
Viral Pharyngitis S&S Gradual sore throat; erythema, inflammation of pharynx and tonsils; vesicles or ulcers on tonsils, low grade fever; hoarseness, cough, rhinitis, conjunctivitis, malaise, anorexia; cervical lymph nodes may be enlarged, tender. Runs course 3-4 days
Myringotomy surgical incision of the typanic membrane (mucoid material removed from middle ear)
Tympanostomy tubes; placed to equalize pressure on both sides of the typanic membrane, keeps ear aerated. Allows middle ear mucosa to return to normal and growth of the Eustachian tube to continue.
Bacterial Pharyngitis Abrupt onset, severe sore throat, erythema, inflammation of pharynx and tonsils; high fever (103-104) may be moderate, abdominal pain, headache, vomiting, cervical lymph nodes may be enlarged, tender, requires antibiotics.
Management of Pharyngitis Pain relief; rest; bland, soft diet, PCN if bacterial.
Tonsillectomy Nursing Care pre-op Assess current infection and bleeding hx; check for loose teeth, teach child and parent what to expect post op; may see dried blood in mouth and teeth, will still be able to talk, pain mgmt for optimal recovery.
Tonsillectomy Post Op nursing care Assess for bleeding number one priotity!!; Elevated P, Decreased BP, restlessness, frequent swallowing, vomiting, bright red blood, fresh blood in throat; Clear cool liquids (no red) full liq, soft foods 2nd day if no bleeding; Pain Releif 2nd Priority
Nursing care post op continued Encourage child to chew and swallow, no straws, forks or sharp pointed toys, discourage; couging, clearing the throat and blowing the nose
Manifestations of croup Begins at night; may be preceded by upper resp tract inf. Sudden onset of harsh, barky cough, sore throat, inspiratory stridor; hoarseness. Accy muscles to breathe, frightened, agitated, cyanosis; mostly viral resolves spont humidification & cold air
Epiglottis A bacterial form of croup (H influenza) with unique symptoms and treatment. Inf invades tissues surrounding the epiglottis wich becomes edamatous, cherry red & may obstruct airway. Unable to swallow; drooling.
Cardianal S&S of Epiglottis Mild URI few days prior; drooking; dysphasia, dysphonia, distressed resp efforts; tripod pos; supported by arms, chin thrust out, mouth open
ER Management of Epiglottis what is the main priority and what DONT you do?? Priority is a patent airway ASAP, monitor o2 status (pulseox, humidified o2) Calm patient; No not examine or culture throat or start IV/Blood samples (will upset child)
Further care of epiglottis after airway Antipyretics suppository, calm parent, intubation, throad & blood cultures after intubation (48hrs) antibiotics for 7-10 days; discharge
Bronchiolitis defined, who gets, when and pathogen Inflammation of the fine bronchioles and small bronchi; occurs in children <2 yo; peak age 6mo; highest in winter & spring; pathogen usually RSV (viral)
S&S of Broncholitis 1-2 days of URI then sudden worsening; nasal flaring, intercostal and subcostal retractions, wheezes, crackles or rhonchi, increased respiratory rate, low pulse oximetry, tachycardia and cyanosis
management of Severe Symptoms of broncholitis Hospitalization, monitor resp status, pulse ox, blood gases, broncohilator therapy no antibiotics its viral
Management of mild symptoms of broncholitis Antipyretics, hydration, humidification, watch for increased severity, acute phase usually last for 2-3 days; no antibiotics its viral.
nursing interventions for broncholitis; Position for comfort, semi fowlers, decrease anxiety, admin IV fluids, humidified 02 (40% then wean) use BB; Ribavarin therapy (see if child is candidate) ^antiviral therapy for severe RSV cases
Pneumonia (PN) defined, occurence Inflammation of the alveoli usually following a URI occurs late winter/early spring; Pneumococcal (bacterial) vs. Viral pneumonia (ABX vs no ABX)
S&S of Pneumonia Viral/Bacterial Viral may have mild cold symptoms; Bacterial is distinctly ill; high emp, diaphoretic, cough (productive or not); tachypnea, abnormal BS (crackles/rhonchi) Dull percussion, chest pain, incr resp effort; CXR chgs; incr WBC, irritable, restless, N/V/D
Interventions for ineffective breathing pattern Assess breath sounds, VS resp status q1-2 h and PRN; admin humidified 02 via face mask, obtain ABG's & Pulse ox; admin ABX, chest physiotherapy as ordered, engage child in play activities
Interventions for activity intolerance Balance activity w/rest period, cluster nursing care; provide small frequent meals, increase activity gradually
Risk for Deficient Fluid Volume interventions; Obtain baseline weight, monitor daily; admin IV fluids as ordered, offer fluids frequently (jello, ices etc) Administer antipyretics, monitor I&O, urine for spec gravity increases
Tuberculosis defined Bacterial infection that multiples in the lung tissue, alveoli and lymph nodes; initially asymptomatic; incubation 2-12 wks will test +PPD; immune system can ward off full infection & become dormant (Children rarely dev active TB but are carriers)
Risk factor for TB Contact w/inf adults; chronic illness, immunosuppression, HIV, malnutrition; young age; nonwhite racial, ethnic, immigrants, urban low income living cond; incarcerated adults, contact w/adults from high-risk groups
Active TB symptoms +PPD, Malaise, Fever, Night sweats, slight cough, weight loss, anorexia, lympaadenopathy, confirmed by CXR, sputum sample or gastric washing
Management of TB in asymptomatic children INH x 9 months; 12 month if HIV+, household contacts threat for 12 weeks
Management in Symptomatic children INH, rifampin and pyrazinamide x2 months followed by INH and rifampin X 4 months side effects: GI, orange tears, urine=noncompliance
Asthma defined A reversible obstructive airway disease characterized by; Hypersensitivity of many cells (mast, eosinophils, t-lymphocytes) Increased airway responsiveness to a variety of stimuli
Pathophysiology of Asthma Bronchospasm resulting from constriction of bronchial smooth muscle. Inflammation and edema of the ucous membranes that line the small airways and the subsequent accumulatin of thick secretions in the airways.
What causes wheezing?? decreased expiratory flow
Initial symptom of Asthma are; Cough w/o illness usually at night
Acute Asthma exacerbation symptoms; Chest tightness, wheezing, SOB, nonproductive cough (w/or w/o wheezing) later becomes productive, tachypnea, orthopnea, tripod position or straight
Asthma triggers; Cold air, smoke/fumes, viral infection, stress, exercise, odors, animal dander, dust, cockroaches, rodents, certain drugs (aspirin, nsaids) GI reflux, food allergens, outdoor allergens
Management of acute exacerbation of asthma Monitor resp rate and effort, color. Provide oxygen therapy; warmed & humidified at 30-40% not 100%. Keep 02 sat >95%; need C02 stimulation for inhalation
Medications administered for acute asthma exacerbation; Short acting beta 2 agonist bronchodilators (ventolin, proventil, albuterol) Corticosteroids (prednisone, prednisolone, solumedrol) Monitor effectiveness of meds
Satus asthmaticu occurs when child fails to respond to treatment; often caused by pulmonary infection. CALL MD
Asthma that is mild intermittent occurs; <2 X week
Asthma that is mild persisten occurs >2 x week but less than one x day
ASTHMA that is moderate occurs day symptoms 2x week, one or more night symptoms per week
Asthma that is severe occurs continual day symptoms, frequent night symptoms
Asthma maintenance medications for mild; Mild Asthma; PRM anti inflammatory, corticosteroids (flovent inhaler QD)
Asthma maint meds for moderate Anti-inflammatory corticosteroids QD and long acting bronchodilator (theophylline, serevent) HS
Asthma maint meds for severe cases Oral corticosteroid qd; inhaled corticosteroid qd, long acting bronchodilator HS, short acting beta 2 agoniist bronchodilator (albuterol) if attack begins also mast cell inhibitors (intal) Leukotriene blocker (singulair) prevents severe bronchospasm
Discharge teaching for asthma Teach self management, identify triggers, avoid allergens, may need skin testing and hyposensitization
Peak flow meter Measure max peak expiratory flow rate, need to first use when healthy to mark baseline; casn use to predict actue exacerbation in kids 5-6 yrs and older, take deep breath blow out hard and fast. If peak flow is 30-50% below baseline take child to ER
Cystic Fibrosis definted Mutated gene on chromosome 7 CFTR, inherited autosomal recessive trait, both parents carry gene (1/4 chance of conceiving affected child)
Cystic Fibrosis pathophysiology Chronic multisystem disorder affecting the exocrine glands; affect: bronchioles, sm intestines, pancreatic & bile ducts. Incurable; median life exp = 33; usually diagnosed vefore 1yr symptoms worsen as disease progresses
Cystic fibrosis in a nut shell Bocy produces too much mucous (lungs & GI) Too much sodium & choloride in sweat.
CF: Respiratory System Wheezing, dry nonproductive cough, repeated URI's. Copius, thick sputum, crackles, wheezes, decreased breath sounds, incr. signs of resp distress=> emplysema & atelectasis, clubbing, barrel chest
CF: Digestive System Steatorrhea (frothy, foul smelling stools 2-3 times bulkier than normal; failure to thrive despite normal caloric intake; protuberant abdomen; fat soluble vit deficiencies K, A, D, E (inability to absorb fats) moconium ileus in the newborn 1st sign
CF: Exocrine Glands Abnormally high concentrations of sodium and chloride in the sweat. Sweat test; determines amt of sodium chloride in sweat >60 is diagnostic risk for electrolyte imb during hot weather
CF: Reproductive system; Avg of 2 yr delay in the dev of secondary sex characteristics. Females have thick cervical mucus (trouble conceiving) Some male pt are sterile due to lack of sperm
Management of CF Prevention and treatment of pulmonary infections. Maintain optimal nutritional status (high cal/high protein) Enzyme supplements. Home Management; Flutter device, CPT BID, Postural drainage, Exercise
Interventions for hospitalized CF child Facilitating airway clearance; prevent pooling of secretions; limit procedures, CPT q4h (1hr before or 2 hrs after meals, prior to bedtime; forced expiration ("huffing")
Interventions for CF client Admin bronchodilators and mucolytics; high humidity cool-mist tent to mobilize secretions; if 02 is req, low flow rate;
Long Term Support for CF client Cystic fibrosis fountation, American Lung Assoc., coordinationof care from home to school, increase self esteem, foster independence.
Dehydration and fluid loss in a child Lg portion of a childs fluids is located in extracellular fluid (incr BSA) Infants; 75-80% weight 2 yr old =60% of weight First two yrs kidneys are not functionally mature; inefficient at excreting waste products.
More on Dehydration/fluid loss Fluid & Electrolyte imbalances develop and progress very quickly. Sick children often have low PO intake and diarrhea and vomiting. Infants/children are highly susceptible to rapid and profound fluid and electrolyte imbalances.
Types of Fluid Loss Sensible and Insensible
Sensible fluid loss Measured & observed; urine output; drains and tubes, emesis, diarrhea
Insensible fluid loss Loss thru lungs 2/3 and skin 1/3; influenced by heat and humidity, body temp, RR (children have higher RR than adults) BMR incr 10% for each degree C above normal body temp.
NA Sodium; major electrolyte in ECF Needed to establish molarity
K Potassium; major electrolye in ICF; needed for excitability of neurons and muscles
Three types of dehydration; Isotontic, hypotonic, hypertonic
Isotonic dehydration No change in sodium. Sodium and water deficits are the same (salt & water lost in equal amts in ICF and ECF) Na is 13-150meq/l (normal) Most common in children from low PO intake, Can result in hypovolemic shock
Sodium normal level 130-150
Hypotonic Dehydration Low sodium, GI losses. Sodium deficit is greater than the water deficit. Water moves from ECF to ICF. NA <130 meq/L. Results from GI losses (vomiting, diarrhea). May result in shock
Hypertonic Dehydration Water oss exceeds sodium loss; body compensates w/fluid shifts from ICF to ECF; NA > 150meq/L. May be caused by severe vomiting, too much IV NA. Can result in seizures.
S&S of Mild Dehydration Normal VS, moist mucous membranes, alert, normal urine output, normal turgor, fontanelle, normal cap refill, thirsty
S&S of Moderate Dehydration Rapid pule, RR, normal BP, dry mucous membranes, irritable, dark urine and decreased output, poor turgor, sunken fontanelle, delayed cap refill, mod thirsty
S&S of Severe dehydration Changes in respirations depth and pattern, rapid weak pulse, low BP, mucous membranes parched, can be comatose, absent urine output, very poor turgor, sunken fontanelle, cool skin.
Monitor for dehydration; Urine output should be at least 1-2 ml/kg/hr. All children are on I+O pay attention to the balance. Monitor labs for; increased BUN; increased serum bicarb; hyponatremia, hyperkalemia, increase urine specific gravity
Prevent dehydration Monitor temp, prevent overheating; give frequent fluids, may need oral rehydration (pedialyte 50 ml/kg in 4 hrs when febrile and GI losses; give 1 tsp q few mins. Monitor IV fluid admin, ensure patent IV site.
Administering IV fluids; Always use infusion pump w/volume control device; prevents a sudden extracellular fluid volume overload; never use more than a 500 ml bag; Mechanical pumps can have faulty performance so check the inravenous line, bag and rate often
RBC Normal count for children 2-12 3.89-4.96
HgB Normal count for children 2-12 10.2 - 13.4 (usually 1/3 of Hct)
Hct Normal count for children 2-12 31.7 - 39.3%
Sed Normal for children 2-12 1-8
WBC Normal for children 2-12 5,400 - 11,000
Platelets for children 2-12 206,000 - 103,000
Fe for children 2-12 20-105
Ferritin for children 2-12 47 - 110
TIBC (Transferrin) Normal for children 2-12 240 - 508
PT normal for children 2-12 10-11 sec
PTT normal for children 2-12 52-54 sec
Bilirubin for children 2-12 < 11.7
Symptoms of Anemia in the child hypoxia, pulse ox, restless, not playing
Iron-deficiency anemia Most common hematologic disorder of infancy and childhood. Evaluated for at 9mo - 2yrs, adolescence. A nutrient deficiency of inadequate dietary iron.
Prevention of iron-deficient anemia Iron fortified products (formula and food)
Children at risk for iron deficient anemia low birthweight infants; infants born to mothers w/iron deficiency anemia; infants born w/GI defects and chronic blood loss in older children
Pathophysiology of Iron-Deficient Anemia Dietary Fe inbloodstream binds to transferrin (TIBC) and is delivered to RBC in bone marrow, combines with other cells to make HgB. Unused dietary Fe is stored in intestinal epithelial cells as ferritin. Look at Iron in blood (Fe)
Diagnosis of Iron Deficient Anemia CBC shows: Low RBC, HgB, Hct, Iron, Ferritin and High Transferrin (TIBC)
Symptoms of Iron Deficient Anemia Low Hgb = Low tissue perfusion; HgB 10.2 or less may be asymptomatic, pallor/pale mucous membranes, poor muscle tone, decr activity, fatigue, increased HR, RR; HgB >9= above plus irritability, lack of interest in play
The hx of pt. w/iron def anemia shows; Abnormall high milk intake >32oz day in toddler. Too full to eat food.
Management of Iron Def Anemia Iron fortified milk will fix hgB; limit cows milk to 24-32 oz/day for chldren >12mo. Incr age appropriate iron rich foods and Vit C. May need iron supplement (ferrous sulfate)
Iron rich foods include; Meats, fish, poultry, vegetables, dried fruits, legumes, enriched grain products, who grain cereal, iron fortified cereal
Vitamin C Rich foods; OJ, Citrus fruits, strawberries, tomatoes, broccoli, green leafy veggies & potatoes
Side effects of ferrous sulfate nausea, anorexia, constipation, abdominal distress, black stools; give on empty stomach if possible; moniter bowel movements & increase fluid and fiber
Nursing considerations for iron def anemia Monitor development, sleep and activity/fatigue patterns; monitor HgB to measure effectiveness of therapy; Teach families to keep ferrous sulfate locked away from children.
Sickle Cell Anemia defined A genetic disease. Autosomal recessive disorder; African Americans; abnormal hemoglobin HbS Cliiical manifestations caused by obstructions due to the sickled RBC's and destruction of sickled and normal RBC's.
How is sickle cell diagnosed? Amniocentesis, CVS, Newborn screening Moderatly lov Hgb and Hct, normal iron, TIBC, ferritan; elevated bilirubin (confirm w/genetic testing which takes time)
S&S of Sickle Cell Initially; fevder & anemia at 6 mo.; then pallor, fatigue, SOB, jaundice, irritability, breathlessness, back pain, arm & leg pain, hand/foot edema
What are the three sequalea of SCA? Vaso-occlusive crisis; Acute Chest Syndrome; Splenic Sequestration
Vaso-occlusive Crisis defined Severe sudden onset of sickling where many new sickled cells pool in a vessel and cause pain and tissue hypoxia (pale,swollen,cool exptremities=pain) Caused by: infection, dehydration, anxiety, cold. Most common from hypoxia s/t rapidy destroyed RBC
Early signs of vaso-occlusive crisis pallor, tachycardia, fever, inflammation
Late signs of vaso-occulusive crisis actute abdominal, back, extremity pain
First crisis in infants manifests as (vaso occlusive crisis) hand & food syndrome (dactylitis) which is swelling of hand and feet, jints may be warm & swollen
Management of vaso occlusive crisis Pain releif; hydration (reverses clog), oxygenation (reverses sickling)
Pain mgmt of vaso occlusive Assess pain q 1-2 hr or more; use pain scale appropriate for age; non-pharmacological pain methods; around the clock pain meds; tylenol for mild pain, narcotics for mod-severe pain
Prevention of occlusion in vaso-occlusive crisis Push PO fluids; IV hydration 1.5-2 x normal rate; risk for fluid overload (is done to prevent tissue infarct)
Altered tissue perfusion and prevent further sickling administer Oxygen to maintain saturation of 95% or higher (will pump up RBCs); pulse ox; semi-fowlers position; administer PRBC's (infusion of healthy RBC's)
Acute Chest Syndrome Sickled contents break off & get stuck in lung = PE; Bilateral pulmonary involvement; cases chest infection, embolism
Know the symptoms of Acute Chest Syndrome; Chest pain; fever; cough; wheeze; tachypnea
Treatment for Acute Chest Syndrome; Analgesics; oxygen; hydration; incentive spirometry, antibiotics, PRBC's
Splenic Sequestration Sickled cells block the spleen; pooled blood in spleen and/or liver and enlarges. Pooled blood leads to decrease in circulation volume and hypovolemic shock; CVA => Coma
Symptoms of Acute Chest Syndrome; Irritability, pale, tachycardia, pain to LUQ, enlarged spleen
Treatment for acute chest syndrome; Life threatening- get a child to ED ASAP; PRBC's, remove spleen
Risk for infection r/t chronic immunosuppression Administer PCN everyday; up to date vaccines; educate parents r/t s/s infection & resp distress, triggers, treat pain immed, adequate fluids.
Beta Thalasemia Major (Cooley's anemia) Severe hereditary anemia due to abnormal synthesis of hgB, life long disorder, mediterranean descent, life threatening symptoms (Body is unable to make enough HgB)
Diagnosis of Beta Thalasemia Major (cooleys anemia) Lob RBC's; extremely low HgB <5 and increased serum iron * key because body cant use iron for synthesis. Pt. also has symptoms of hypoxia
Symptoms of Beta Thalasemia Major Protruding forehead, maxillary prominence wide set eyes w/flattened nose; Bronze skin color (too much iron) Growth & maturation retardation
Management of Beta Thalasemia Major RBC transfusions q2-4 wks to get HgB to 10-12; Iron Chelation therapy (because iron can't be seperated fr. PRBC's) Splenectromy. Cure= bone marrow stem cell transplant
Nursing Considerations for Beta Thalasemia Major Observe for complications of transfusion-iron overload; Supporting the child and family in dealing with a chronic life-threatening illness; monitor growth and development- refer the family for genetic counseling.
Three Bleeding Disorders; Hemophilia A; Von Willebrand's Disease; IPT (Immune Thrombocytopenic Pupura)
Clotting Host of factors; platelets aggregation at site of injury; tested by coaglation time (PT/PTT)
Hemophilia A A hereditary blood coagulation deficiency (factor 8); Ability to clot is slower; X-linked recessive (white, male)
Symptoms of Hemophilia Vary according to concentration of factor 8; Soft tissue bleeding and painful hemorrhage into joints; severe bleeding may occur in GI tract, peritoneum or CNS
Interviewing the child w/hemophilia Subjective data; Recent trauma & measures to stop bleed; length of time pressure was applied before bleed subsided; whether swelling incr after surface bleeding subsided; whether swelling and stiffness occurred w/o apparent trauma.
Diagnosis of Hemophilia Subjective info; suspect labs; platelets-Normal; PTT >60 (clotting problemo) confirmed by genetic testing for missing factor.
Management of bleeding for Acute Therapy Bleeding must be controlled by IV admin of factor 8; (after trauma surgery) Pressure to laceration
Management of Prophylactic therapy Hemophilia; Children age 1-2 receive PO factor 8 replacement on a regular schedule if freq symptomatic prior to surgery, dental work
Parental Education of Hemophilia Primary goal is injury prevention; promote oral hygiene, up to date immunizations, no aspirin, avoid activities that induce bleeding, provide activities for normal G&D, Admin of factor replacement PRN
Von Willebrand's Disease Most commonly inherited bleeding disorder, autosomal dominant (m&F). Lacks production of VWF; platelets are normal in #; inability of platelets to aggregate; varying degrees of disease VWF (clotting factor) is deficient to defective.
Diagnosis of Von Willebrands Disease Platelets are normal; Pt;PTT is normal; confirmed by genetic testing for VWF.
S&S Von Willebrand's Can be so mild that disease in undiagnosed; bleeding from gums; epitaxis, prolonged bleeding from cuts, excessive bleeding following surgery
Management of Von Willebrands Prophylacic therapy- Replace dysfunctional factor in blood; treatment of choice; DDAVP which stimulates body to release clotting factor (VWF)
ITP (Immune Thrombocytopenic Pupura) Autoimmune disorder (antiplatelet antibody) or cause is unknown (idiopathic) Occurs most commonly at 2-4yrs; reduction in and destruction of platelets. Typical 2wks after a viral illness
S&S of ITP Excessive bruising and petechiae, epitaxis, bleeding into joints, tourniquet test; shows many petechiae after inflation of BP cuff
Labs for ITP Platelets <150 (marked thrombocytopenia); PT and PTT; normal
Management of ITP Prednisone, IVIG (IV immunoglobulin) PLT transfusion (temp solution) Most cases are self-limiting; Avoid; iM injections, aspirin, aspirin containing prod, NSAIDS, rectal temps invasive procedures = procede w/caution
Differentiate between cancer in adults and cancer in children Cancer in adults is usually a result of exposure to a tetragon (toxins). Cancer in children arises from chromosomal abnormalities, genetic mutations and profliferation of embryonic cells.
Oncology treatments Chemotheraphy; antineoplastic agents attempt to destroy tumor cells by interfering w/cellular functions and reporduction. Cytotoxic drugs that are designed to cause cell death. Toxic sides effects, normal cells w/rapid growth are affected (hair)
Surgical intervention, Radiation and children Neither is typically a choice for children
Types of Cancer in children Sm. % carcinoma (vs lg % adults) Mostly Leukemia or Lymphoma, the lesser % is solid or soft tissue tumors.
Clinical manifestations of cancer in children Differ by type; Many are similar to common childhood illnesses and may be in a site other than the cancer = delay in diagnosis. Often diagnosis made when cancer is advanced.
Common S&S of Cancer Pain, anemia, anorexia, weight loss, infections, bruising, neurological symptoms, palpable mass
Psychosocial concerns related to cancer Parents disbelief; healthy child suddenly becomes ill; potentially life-threatening; treatment decisions can last months to years; travel for treatment, heavy financial responsibilities; effects of siblings.
Effects of cancer on infants unaware of diagnosis
Effects of cancer on toddlers aware they do not feel well
Effects of cancer on preschooler beginning understanding of illness, not cancer
Effect of cancer on school age child understand cancer, benefit from talking about it
Effect of cancer on adolescent mature understanding, benefits from other adolescents with cancer
General Nursing considerations for children with cancer; Provide optimal nutrition (high metabolic rate of cancer depletes stores). Ensure adequate hydration- ice pops,jello. Manage pain; promote growth and development. Prevent infection
Risk for infection r/t immunosuppressed state Monitor VS q4h; instruct parents how to measure temp at home; proper handwashing; inspect child's skin for breakdown; inspect childs mouth for ulcers; teach child and parents meticulous oral hygiene; no live virus administration
Leukemia A broad term describing a group of malignant diseases; normal bone marrow is replaced by abnormal immature cells. Etiology; variety of agents throught to incr. risk (virus, toxins, drugs) combined w/genetics.
Two forms of leukemia ALL - Acute Lymphocytic Leukemia AML - Acute Myelogenous Leukemia
Acute Lymphocytic Leukemia details; Most freq type of cancer in children <15 (peak 2-6) Distored and uncontrolled proliferation of immature WBC's (lymphoblasts) Causes decr. RBC's, platelets and mature WBCs production & invasionof body organs by rapidly incr. lymphoblasts
Signs & Symptoms of ALL Fever; bone or joint pain; bruising; decr RBC, Decr platelets; abnormal high WBC counts, lymphadenopathy, hepatosplenomegaly, CNS invasion. WBC usually very high (50K)
Diagnosis of ALL Based on S&S; CBC changes, Bone marrow aspiration (>25% of lymphoblast cells present)
Management of ALL Chemotherapy in three stages for 2-3 yrs. (Induction, Sanctuary, Maintenance)
Induction 1st month; aim is to induce remission (blast cells to <5%, normal physical findings); approx 95% of children achieve remission within 1 month
Sanctuary or Consolidation Begins after remission, 4 weeks; Goal; to maintain remission; prevent disease from invading sanctuary sites.
Maintenance Goal to maintain remission; eliminate residual leukemic cells; combination of drugs, outpatient basis; girls treated for 2 yrs boys for 3. CURE: Free of disease for 4-5yrs.
High doses of chemotherapy can lead to : Tumor lysis syndrome (metabolic emergency) Results from the lusis (dissolving or decomposing) of tumor cells and rapid release of their contents into the blood.
Tumor Lysis Syndrome is Rapid cell destruction that released high levels of ; uric acid, potassium and phosphates. Uric acid overloads the kidneys. Leads to cardiac arrhythmias and renal failure.
Nursing consideration for children receiving chemotheraphy; monitor for; hyperuricemia, hyperkalemia, hyperphosphatemia and Hypocalcemia.
Prevention for Tumor Lysis Syndrome Administer vigorous hydration (2-4X rate for maintenance fluid) and Administer allopurinol or urate oxidase (rasburicase) to reduce conversion of metabolic by-products to uric acid.
Soft tissue tumors (3) Hodgkins, non hodgkins and retinoblastoma
Lymphomas are; name two types A malignancy that arises from the lymphoid system. Two types: Hodgkins and non hodgkins (abnormal production of lymph)
Hodgkins Disease Neoplasm of cervical lymphatic tissue. Starts in a single or group of lymph nodes then spreads predictably to nonnodal sites s/a the spleen, liver, bone, marrow, lungs, mediastinum. Affects adolescents to late 20's Males > Females.
Signs & Symptoms of Hodgkins Painless enlarged cervical node; Unexplained weight loss, unexplained fevers, night sweats.
Diagnosis of Hodgkins Biopsy of enlarged lymph node; staged 1-4
Treatment for Hodgkins Chemotheraphy, Radiation-low doses, higher if physiologically mature (older teen who has achieved full height); Good prognosis- single origin
Non-Hodgkins lymphoma No single origin; males > females; cause unknown; aggressive proliferation of B or T lymphocytes in lumph nodes; rapid in onset (ages 5-15) Usually found with wide-spread involvement via bloodstream (multiple enlarged nodes) Responds quickly to therapy
Signs & symptoms of non-hodgkins lymphoma Acute abdominal & chest pain, constipation, cramping; anorexia, weight loss; painless enlarged lumph nodes in cervical or axillary region; ascites & obstruction w/vomiting are late signs; Adv signs; CNS symptoms, Headache, N&V, mediastinal mass, petichaie
Diagnosis of Non-Hodgkins lymphoma Biopsy from bone marrow or lymph node; staging 1-4
Treatment of Non-Hodgkins Lymphoma Aggressive multi-agent chemo for 6 mo to 2 yrs. Risk for tumor lysis syndrome; intrathecal chemo and crainal radiation.
Retinoblastoma Malignant tumor of retina; inherited autosomal dominant; immature retinal cells become malignant; 6 weeks of age to preschool age; unilateral or bilateral; no outward signs
Signs & Symptoms of Retinoblastoma Absent red reflex, Whitish glow to pupil; strabismus develops; eye pain; metastases to optic nerve, subarachnoid space, brain, 2nd eye
Treatment for Retinoblastoma If small; cryosurgery, partial vision; If Mets; chemo & radiation; If large; enucleation, eye prosthesis 3 wks post op; survival rate 90%
Solid tumors (4 types) Neuroblastoma, Nephroblastoma, Osteosarcoma, Ewing's Sarcoma
Neuroblastoma Solid tumor of infants and pre-school children (peak 22mo) Cancer cells arise from sympathetic nervous system called crest cells (embryonic cells of adrenal glands)
S&S Neuroblastoma Depend on extent of disease & location of tumor. 65% present w/ protuberant, firm, irregular abdominal mass that crosses the midline. "THINK ASSESSMENT"
Other Manifestations of Neuroblastoma imparied ROM & mobility; pain & limping; lg abdominal mass; respiratory symptoms if chest tumor
Diagnosis of Neuroblastoma Chest X-Ray; CT scan of abdomen, pelvis, spine; bone marrow aspiration
Management of Neuroblastoma Depends of the presence and extent of metastasis
Wilm's Tumor (Nephroblastoma) Malignant tumor of the kidneys; peak age 3-4 yrs; girls > boys, cause is unknown; Other GU problems; Occurs in asymptomatic child; may have genetic predisposition also assoc w/congenital anomalies
S&S Of Nephroblastoma Parents usually notice a lg. mobile abdominal mass while bathing or the diaper doesn't fit anymore. Grows extremely quickly, in a matter of days. DO NOT PALPATE ABDOMEN!! Can rupture the tumor and cause the spreading of cancerous cells
Other S&S of nephroblastoma Microscopic gross hematuria; hypertension; abdominal pain; fatigue, anemia, fever
Diagnosis of Nephroblastoma Sustpected from a good hx; CT scan; definitive dx made at time of surgery; staged 1-5
Stage 1 (Nephroblastoma) Tumor confined to the kidney and completely removed surgically (good prognosis survival rates near 90%)
Stage 2 (Nephroblastoma) Tumor extending beyond the kidney but completely removed surgically
Stage 3 (Nephroblastoma) Regional spread of disease beyond the kidney w/residual abdominal disease postoperatively
Stage 4 (Nephroblastoma) Metastasis to lung (primary site) liver, bone distant lymph nodes
Stage 5 (nephroblastoma) Bilateral kidney disease
Treatment for stage 1-2 nephroblastoma; Nephrectomy & Chemotherapy
Treatment for stage 3-5 nephroblastoma; Nephrectomy, radiation, chemotherapy
Osteogenic Sarcoma (bone cancer) Most common bone malignancy in children (teens); Occurs in distal long bones; attributed to extrmity injury or growth spurt; originates fr bone producing cells; 40-50% occur at distal femur and knee
Signs & Symptoms of Osteogenic Sarcoma Progressive, insidious or intermittent pain at site of tumor. Palpable mass & swelling; limping, progressive limited range of motion, pathological fractures.
Diagnosis of Osteogenic Sarcoma X-Ray, CT, MRI and tumor biopsy; Look for chest metastases
Management of Osteogenic Sarcoma Remove tumor, prevent spread of disease; combination of surgery & chemo; amputation may be necesssary; limb salvage operation; cure rate= 60-65% w/o overt metastases
Nursing care of pt. w/osteogenic sarcoma Comfort, infection control, potential hemorrhage, phantom limb pain, prosthesis, chg in body image and functioning.
Ewing's Sarcoma Highly malignant in bone marrow of long bones. Can present in any bone; spreads longitudianlly thru bone; affects young adolescents & older children; metastases is usually present at time of dx (lungs, bone, CNS, lymph nodes"
S&S of Ewing's Sarcoma Intermittent pain attributed to injury; Swelling at tumor site; pain becomes constant; progresses into=> weight loss, fever, incr sed rate
Diagnosis of Ewings Sarcoma Bone Scan Bone marrow aspiration & biopsy; CT of lungs; definitive dx; biopsy of tumor site
Treatment for Ewings Sarcoma Surgery; multi agent chemo (at risk for tumor lysis syndrome) and Radiation
Nursing Diagnoses for the chronically ill child; Fear; death anxiety, anticipatory grieving, hopelessness
Goals for the care of the chronically ill child Achieve and maintain normalization; obtain the highest level of health and function possible;
Goals for the family of the chronically ill child Remain intact; achieve and maintain normalization; maximize function throughout the illness
Nursing care for children w/ chronic conditions and their families Attend to the needs of the family system; revise goals frequenctly to meet the child's changing developmental needs. Listen carefully to the childs perception of the condition
A child is being admitted to the unit with thalassemia major (cooleys anemia) in preparing client assigments, the charge nurse wants to assign a nurse the his child who can; Administer blood transfusions
A 14 yo boy w/ sickle cell is admitted w/ severe pain in his abdomen and legs. He asks why the dr ordered oxygen when he is not having any breathing problems. The nurse states that the therapeutic action of O2 is prevention of further sickling (to plump up RBS's)
A 10 yo in the ER has a CBC results that incl a Hgb of 8, a Hct of 24. The nurse determines that based on the lab results which nursing action has a high priority? Conserving energy
A 4-yo is diagnosed w/ ALL/ Following teaching about the staging and therapy, the nurse evaluates the familys understanding of the problem. The statement by the family that indicates appropriate knowledge is that Staging will Determine the extent of malignant process and stage the leukemia
A 17yo is being admitted for an amputation related to a bone tumor. The nurse is developing a nursing care plan and determines the most appropriate age related diagnosis is; Disturbed body image
The nurse is admitting a child for a swollen elbow. The hx indicated multiple bruising. Which of the following lab results heightens the nurses suspicion for hemophilia? PTT 73 seconds (>60)
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