Professional Documents
Culture Documents
❖ Allergic reactions range from urticaria, wheezing, watery eyes, and rashes to anaphylactic shock (worst
one)
➢ BE SURE TO HAVE EPI PENS or Epinephrine if suspected allergic reaction
❖ Foods and Latex Allergy (the book only mentions these p. 1956)
➢ milk, kiwi, bananas, or chestnuts
Daily maintenance fluids (distribute x into 10 x + 10 x + remainder x.Ex. 30 kg= 10 kg + 10 kg +10 kg)
100 ml/kg for the first 10 kg of body weight
50 ml/kg for the second 10 kg of body weight
20 ml/kg for the remaining kilograms of body weight
Characteristics
➢ most severe and the most common MD of childhood. It is inherited as an X-linked recessive
trait, and the single-gene defect is located on the short arm of the X chromosome
➢ Early-onset, usually between 3 and 7 years old
■ Most children with DMD reach the appropriate developmental milestones early in life,
although they may have mild, subtle delays
➢ Progressive muscular weakness, wasting, and contractures
■ Difficulties in running, riding a bicycle, and climbing stairs are usually the first symptoms
noted
➢ Calf muscle pseudohypertrophy in most patients
➢ Loss of independent ambulation by 9 to 12 years old
➢ Slowly progressive, generalized weakness during teenage year
● Relentless progression of muscle weakness; possible death from respiratory or
cardiac failure
● Waddling gait
● Lordosis
● Frequent falls
● Gower sign (child turns onto side or abdomen; flexes knees to assume a
kneeling position; and then with knees extended, gradually pushes torso to
an upright position by “walking” the hands up the legs)
● Enlarged (hypertrophied) muscles (especially calves, thighs, and upper arms);
feel unusually firmer woody on palpation
● Later stages—profound muscular atrophy
● Mental deficiency (common)
◆ • Mild (≈20 IQ points below normal)
◆ • Mental deficit present in 25% to 30% of patients
● Complications:
◆ • Contracture deformities of hips, knees, and ankles
◆ • Disuse atrophy
◆ • Cardiomyopathy
◆ • Obesity and at times undernutrition
◆ • Respiratory compromise and cardiac failure
Nursing Care
■ Therapeutic management: Primary goal is to maintain function in unaffected muscles as
long as possible
● Keep the child as active as possible
● Range of motion, bracing, the performance of activities of daily living, surgical
release of contractures as needed
■ Help child and family cope with chronic, progressive, debilitating disease
■ Help design a program to foster independence and activity as long as possible
■ Teach child self-help skills
■ Arrange for appropriate health care assistance as the child's needs intensify (home
health, skilled nursing facility, respite care for family, etc.)
■ Medications: corticosteroids
Clinical Manifestations
➢ Cardinal Signs
■ Emotional lability
■ Physical restlessness,
characteristically at rest
■ Decelerated school performance
■ Voracious appetite with weight loss in 50% of cases
■ Fatigue
➢ Physical Signs
■ Tachycardia
■ Widened pulse pressure
■ Dyspnea on exertion
■ Exophthalmos (protruding eyeballs)
■ Wide-eyed, staring expression with eyelid lag
■ Tremor
■ Goiter (hypertrophy and hyperplasia)
■ Warm, moist skin
■ Accelerated linear growth
■ Heat intolerance (may be severe)
■ Hair fine and unable to hold a curl
■ Systolic murmurs
➢ Thyroid Storm
■ Acute onset:
● • Severe irritability and restlessness
● • Vomiting
● • Diarrhea
● • Hyperthermia
● • Hypertension
● • Severe tachycardia
● • Prostration
■ May progress rapidly to:
● • Delirium
● • Coma
● • Death
❖ Nursing Care
➢ Nursing care focuses on treating physical symptoms before a response to drug therapy is
achieved.
■ Children with hyperthyroidism need a quiet, unstimulating environment that is
conducive to rest.
■ Increased metabolic rate may cause heat intolerance and increased food intake in these
patients.
■ Mood swings and irritability can disrupt relationships, creating difficulties within and
outside the home.
● Nurses can help parents understand the medical reason for behavior changes
and offer ways to minimize them.
■ School consultation is important to provide education and suggest ways to assist a child
after diagnosis.
■ Nurses should know the side effects of antithyroid drug therapy:
● urticarial rash, fever, arthralgias, vasculitis, liver dysfunction, and
agranulocytosis. Lymphadenopathy, edema, and diminished taste can also
occur.
❖ Hypoparathyroidism/ Hyperparathyroidism and Vitamin D tx: Clinical manifestation and nursing
care
HYPOparathyroidism
➢ Clinical Manifestations
■ Pseudohypoparathyroidism
● Short stature
● Round face
● Short, thick neck
● Short, stubby fingers and toes
● Dimpling of skin over knuckles
● Subcutaneous soft tissue calcifications
● Intellectual disability is a prominent feature
■ Idiopathic Hypoparathyroidism
● None of the above physical characteristics observed
● May include papilledema
● May have intellectual disability
■ Both
● Dry, scaly, coarse skin with eruptions
● Hair often brittle
● Nails thin and brittle with characteristic transverse grooves
● Dental and enamel hypoplasia
● Muscle contractions:
◆ • Tetany
◆ • Carpopedal spasm
◆ • Laryngospasm (laryngeal stridor)
◆ • Muscle cramps and twitching
◆ • Positive Chvostek sign or Trousseau sign
◆ • Paresthesias, tingling
● Neurologic:
◆ • Headache
◆ • Seizures (generalized, absence, or focal)
◆ • Swings of emotion
◆ • Loss of memory
◆ • Depression
◆ • Confusion possible
● Gastrointestinal:
◆ • Muscle cramps
◆ • Diarrhea
◆ • Vomiting
◆ • Retarded skeletal growth
➢ Nursing Care
■ Nursing care includes institution of seizure and safety precautions
● reduction of environmental stimuli
● observation for signs of laryngospasm, such as stridor, hoarseness, and a feeling
of tightness in the throat.
◆ A tracheostomy set and injectable calcium gluconate should be
available for emergency use.
◆ The administration of calcium gluconate requires precautions against
extravasation of the drug and tissue destruction
HYPERparathyroidism
➢ Clinical Manifestations
■ Gastrointestinal
● Nausea
● Vomiting
● Abdominal discomfort
● Constipation
■ CNS
● Delusions
● Confusion
● Hallucinations
● Impaired memory
● Lack of interest and initiative
● Depression
● Varying levels of consciousness
■ Neuromuscular
● Weakness
● Easy fatigability
● Muscle atrophy (especially proximal muscles of lower limbs)
● Tongue twitching
● Paresthesias in extremities
■ Skeletal
● Vague bone pain
● Subperiosteal resorption of phalanges
● Spontaneous fractures
● Absence of lamina dura around teeth
■ Renal
● Polyuria
● Polydipsia
● Renal colic
● Hypertension
➢ Nursing Care
■ The initial nursing objective is RECOGNITION of the disorder.
● Because secondary hyperparathyroidism is a consequence of chronic renal
failure, the nurse is always alert to signs that suggest this complication,
especially bone pain and fractures.
● Because urinary symptoms are the earliest indication, assessment of other
body systems for evidence of high calcium levels is indicated when polyuria and
polydipsia coexist.
◆ Clues to the possibility of hyperparathyroidism include change in
behavior, especially inactivity; unexplained gastrointestinal
symptoms; and cardiac irregularities
Vitamin D:
❖ The nurse educates the family about continuous daily calcium and vitamin D.
❖ Because vitamin D toxicity can be a serious consequence of therapy, parents are advised to watch for
signs that include weakness, fatigue, lassitude, headache, nausea, vomiting, and diarrhea. Polyuria,
polydipsia, and nocturia are signs of early renal impairment.
❖ Insulin injection sites
Keypoints:
● ALWAYS rotate injection sites
● Abdomen is most common and fastest absorption site
● when injecting, use a 45°
○ Pinch the skin and let go once the needle is in
● DO NOT USE DORSOGLUTEAL ON INFANTS AND TODDLERS, CONSIDER FOR OLDER CHILDREN
⚠️
Remember for insulin drawing:
⚠️
**WHEN DRAWING UP TWO TYPES OF INSULIN, CLEAR FIRST (fast-acting) THEN CLOUDY (NPH)**
⚠️
If necessary, roll the vial, then wipe the surface before using an insulin based syringe and inject air
USE ONLY INSULIN SYRINGES. MAKE SURE THEY HAVE THE U-BASED MEASUREMENT.
❖ Ketoacidosis: Hypoglycemia and Hyperglycemia and DKA : Clinical manifestation and nursing care
➢ Ketoacidosis - When insulin is absent or insulin sensitivity is altered, glucose is unavailable for
cellular metabolism, and the body chooses alternate sources of energy, principally fat.
■ Consequently, fats break down into fatty acids, and glycerol in the fat cells is converted
by the liver to ketone bodies (β-hydroxybutyric acid, acetoacetic acid, acetone)
■ The ketone bodies in the blood (ketonemia) are strong acids that lower serum pH,
producing ketoacidosis
➢ Clinical Manifestations
■ Hyperglycemia >330
■ Acidosis pH 7.3 and Bicarb 15
■ This results in the body breaking down fat for energy and ketones accumulating in the
blood, urine and lungs
■ Kussmaul respirations & acetone breath
➢ Nursing Care
■ Nursing: Admitted to PICU.
■ Rapid isotonic fluid replacement. Add glucose to IVF when sugar reaches 250mg
■ Monitor glucose levels hourly and prn
■ Administer regular insulin continuously
❖ Hypoglycemia and Hyperglycemia
❖ Botulism: Clinical manifestation and nursing care
➢ Botulism is a rare poisoning caused by toxins produced by Clostridium botulinum
bacteria.
➢ PEDIATRIC EMERGENCY
General Signs
■ Weakness
■ Dizziness
■ Headache
■ Difficulty talking and speaking
■ Diplopia
■ Vomiting
■ Progressive, life-threatening respiratory paralysis
➢ Infant Botulism*
● Caused by ingestion of honey on children < 1 year od age
■ Constipation (a common symptom)
■ Generalized weakness
■ Decrease in spontaneous movements
■ Diminished or absent deep tendon reflexes
■ Loss of head control
■ Poor feeding
■ Weak cry
■ Reduced gag reflex
■ Progressive respiratory paralysis
❖ Nursing Care
➢ IV botulism immune globulin (BIG-IV [BabyBIG]) to treat infants
➢ Trivalent equine botulinum antitoxin and bivalent antitoxin to treat older children
➢ Supportive measures—respiratory support
➢ Continue therapy until paralysis abates
➢ Supportive measures
■ Respiratory support
■ Nutritional support
■ Prevention of complications
■ Emotional support
■ Family support and education
❖ Myelomeningocele: Clinical manifestation and nursing care and feeding pre/post operative
➔ Myelomeningocele is the MOST serious form of spina bifida and most
common
● Part of the group of spina bifida cystica
Clinical Manifestations
◆ Cranioschisis: A congenital skull defect through which various tissues
protrude
◆ Exencephaly: Brain totally exposed or extruded through an associated
skull defect; fetus usually aborted
◆ Anencephaly: If fetus with anencephaly survives, degeneration of the
brain to a spongiform mass with no bony covering; incompatible with
life usually beyond a few days to weeks
◆ Encephalocele: Herniation of brain and meninges through a defect in
the skull, producing a fluid filled sac; can be frontal or posterior.
○ SB
○ Meningocele
○ Myelomeningocele
Nursing care:
● Immediate Nursing management is the protection of the site from trauma (prone position)
● keep the sac from drying out with saline-soaked sterile gauze
● Risk of latex allergy due to multiple exposures from catherizations and surgeries
● Abdominal assessment revealing bladder distention, even with a wet diaper, may indicate urinary overflow
in a retentive bladder.
● Head circumference measurement
!!! Feeding a baby with myelomeningocele involves putting the head on the side while remaining prone , hence
early repair is done.
Pre-op:
● the myelomeningocele is prevented from drying by the application of a sterile, moist, nonadherent
dressing.
○ The moistening solution is usually sterile normal saline.
■ Dressings are changed frequently ( q 2-4 hrs), and the sac is closely inspected for leaks,
abrasions, irritation, and any signs of infection.
○ Early s/s include high temperature (axillary) , irritability, lethargy ,
increased ICP (which are also s/s of hydrocephalus)
○ NO RECTAL TEMPERATURES
○ Contraindications include putting diapers, cuddling the baby,
performing range of motion exercises that go beyond some extremities
■ Consulation with PT
Post-op:
● Vital signs
● I & O’s
● providing nourishment
● observing s/s of infection
● close observation of CSF leakage
● PRONE positioning
● Feedings can be resumed once there are no complication observed
● Educate parents and make them involved in care (CIC techniqued is taught often)
➢ Type I
■ Polyphagia
■ Polyuria
■ Polydipsia
■ Weight loss
■ Enuresis or nocturia
■ Irritability; “not himself” or “not herself”
■ Shortened attention span
■ Acetone breath (fruity smell)
■ Lowered frustration tolerance
■ Dry skin
■ Blurred vision
■ Poor wound healing
■ Fatigue
■ Flushed skin
■ Headache
■ Frequent infections
■ Hyperglycemia
● • Elevated blood glucose levels
● • Glucosuria
■ Diabetic ketosis
● • Ketones and glucose in urine
● • Dehydration in some cases
■ Diabetic ketoacidosis (DKA)
● • Dehydration
● • Electrolyte imbalance
● • Acidosis
● • Deep, rapid breathing (Kussmaul respirations)
➢ Type II
● Increased thirst
● Dry mouth
● Increased urination
● Sudden weight loss
● Headaches
● Fatigue
● Blurry vision
● Frequent infections (or slow healing sores)
● Pain/ tingling on hands and feet (pins and needles)
Nursing care:
● DKA is a medical emergency -> admit to PICU immediately
○ Once that is stabilized, I&O’s and insulin regimen begin
● Diabetes care:
○ The use of medical bracelets for diabetes is important
○ I&Os, daily weights
○ NUTRITION management is the biggest focus for DM
○ Glucose monitoring- goal is 80-120 mg/dL
■ Administration of insulin for every blood sugar check if needed
○ EDUCATION (new or onset) is the second biggest focus for patients with DM
■ It involves teaching parents and children about counting calories (like in a diary), glucose
monitoring, and self-injections of insulin.
Developmental issues in DM
● Infant: relies on the parent for care. Provide support for caregivers. Increased risk of adverse
consequences
● Toddlers: Inconsistent food intake, temper tantrums. Help parents establish routines, rituals, discipline.
● Preschoolers: Start to notice they are different, want to actively participate in care but do not have fine
motor skills. Use play therapy and encourage participation.
● School-age: Can participate in daily care, may feel different from peers. Encourage school attendance and
participation in activities. Practice injections on dolls.
● Adolescents: Undergoing rapid physical, emotional and cognitive growth. Slowly turn care over to
adolescents. Assess for risk-taking behaviors. Promote Identity/ Independence
❖ Growth Hormone deficiency: GH replacement and GH stimulation test
Hypopituitarism—Growth Hormone (GH) Deficiency
➢ Diagnostic evaluation
■ Family history
■ Growth patterns and health history
■ Definitive diagnosis based on radioimmunoassay of plasma GH levels
■ Hand x-rays to evaluate growth potential versus ossification
■ Endocrine studies to detect deficiencies
➢ Replacement -
■ replacement of GH, which is successful in 80% of affected children.
● Biosynthetic GH is administered subcutaneously on a daily basis
■ Other hormone replacements as needed
● Thyroid extract
● Cortisone
● Testosterone or estrogens and progesterone
➢ GH stimulation testing is usually required for diagnosis.
■ GH stimulation testing involves the use of pharmacologic agents such as levodopa,
clonidine, arginine, insulin, propranolol, or glucagon, followed by the measurement of
GH blood levels
● Children with poor linear growth, delayed bone age, and abnormal GH
● stimulation tests are considered GH deficient
➢ One vitamin supplement that is recommended for all women of childbearing age is a daily dose
of 0.4 mg of folic acid, the usual RDA.
■ Folic acid taken before conception and during early pregnancy can reduce the risk of
neural tube defects such as spina bifida by as much as 79%.
Mini map for hormones secreted by the anterior/posterior pituitary: