NUR 308 EXAM #2

AGA measurements in the neonate

• head circumference: 33-35 cm

• chest circumference: 30.5-33 cm

• birth weight: 2700-4000 gm

cephalocaudal

head to toe

• measures growth in length

proximodistal

proximodistal to torso outwards

• measures chest/abdomen

Nutrition for the first 6 months

• breast milk

• Infant formula should be iron-fortified

Adding additional fluids (water or juice) in the first 6 months may result in...

failure to thrive due to really low weight and dehydration

nutrition in the second 6 months

• Breastmilk/formula is still the primary source of nutrition

• If exclusively breastfed still after 6 months → give Vitamin D

Calorie requirements for infants

• about 100 cal/kg/day

• Commercial formula is about 20 cal/oz

calories to oz conversions

baby weight (kg) x 100 = how many calories they need

• ex. 4 kg baby x 100 cal = 400 cals

  • Then divide 400 cals by 20 cals = 20 Ounces

congenital abnormalities

• serious health problems that occur in 2-4% of all live births

Congenital amnomalies usually occur in the ______ trimester

first trimester

What are the 2 classifications of congenital anomalies?

1. Syndrome → a recognized pattern of malformations due to a single cause

2. Association → a non-random pattern with no specific cause

What are some etiologies of birth defects

• heredity/genetics (20%)

• maternal environment → mother taking OTC drugs/street drugs, illness

• Combination of both

What is neural tube defects (NTDs)

Defects/failure of neural tube closure

• (normally a neural tube closes around 30 days after conception)

neural tube defect etiology (cause)

• Maternal nutritional deficiency in folic acid (Mothers should take prior to pregnancy)

• Multifactorial → could be heredity

Characteristics of neural tube defects

• The neural tube is embryonic beginning for the brain & spinal column

• The brain & spinal cord become encased in a protective sheath of bone and meninges

• May involve entire length of neural or small portion

Neural tube defects are more common in what gender and race?

• Found more in girls than boys

• 3 times more in whites than African Americans

Treatment/Prevention of neural tube defects

• folic acid supplementation of 0.4 mg/day

• If history of NTD: 4 mg/day

Foods that contain folic acid

• Dark green leafy vegetables → spinach, kale, cabbage, peas

• Beans

• Peanuts

• Sunflower seeds

• Fresh fruit

• Whole grains → fortified cereals

antenatal diagnosis of NTD

• elevated a-fetoprotein in amniotic fluid at 16 to 18 weeks gestation

  • amniocentesis is how we identify if there is an NTD

• Uterine ultrasound

  • Can check out spine

Why do we want to know if a fetus has a NTD?

So that parents are prepared to know the extent of the lesion (major or not)

to schedule a C-section to manage sac without labor/stress

What are the two most common types of neural tube defects (NTD)?

1. Anencephaly

2. Spina bifida/myelomeningocele

Anencephaly

• Absence of cerebral hemispheres

• Brainstem function may be intact

• Incompatible with life:

  • survive only a few hours/days and die due to respiratory failure

spina bifida/myelomeningocele

failure of the osseous spine to close

What are the two types of spina bifida/myelomeningocele?

1. spina bifida occulta: not visible externally

2. spina bifida cystica: visible defect & saclike protrusion coming from spine

spina bifida occulta

• lumbosacral L5-S1 (lower portion of spine)

• skin indicators:

  • sacral dimple

  • sacral tufts of hair

  • sacral lipoma (fatty tumor below skin)

• abnormal adhesion to bone or fixed structure

• Traction on the cord causes:

  • altered gait

  • bowel/bladder problems

  • foot deformities

spina bifida cystica

• visible defect with external saclike protrusion

• two types:

  • meningocele

  • myelomeningocele

meningocele (within spinal bifida cystica)

• sac contains meninges and spinal fluid but no neural elements

• no neurological deficits (just need surgery to fix)

myelomeningocele (within spinal bifida cystica)

• Maybe anywhere along the spinal column

• Sac contains:

  • meninges

  • spinal fluid

  • and nerve

• Varying and serious degrees of neurologic deficit (depends on where on the spine it’s located)

myelomeningocele sac

• may be fine membrane → prone to leakage of CSF; easily ruptured

• may be covered with dura, meninges, or skin

Nursing care for Myelomeningocele Sac

• Keep moist and intact with a sterile solution

• Prone position

myelomeningocele degree

• Location and magnitude of defect determine the nature and extent of impairment:

  • If the defect is below second lumbar vertebra (L2):

    • Flaccid paralysis of lower extremities → can’t walk, feel legs, or have bowel functions

myelomeningocele initial management

• pre op → lay on stomach (prone)

• prevent infection → hand hygeine

• assess neurological anomalies

• Early closure in 12-72 hours after birth

  • prevent stretching of other nerve roots and further damage

Myelomeningocele is always associated with/linked to...

latex allergy

cerebrospinal fluid

• secreted by choroid plexus

• circulates throughout the ventricular system

• absorbed within the subarachnoid spaces

hydrocephalus

• Extra CSF in the ventricles in the brain ("water on the brain")

• Commonly associated with myelomeningocele

• 80-85% of spinal bifida cases will develop hydrocephalus

• May not be apparent after birth

• May appear after primary closure of defect (need to do daily head circumferences)

hydrocephalus monitoring

• Measure head circumference

• fontanel tension (bulging outward on top of head)

• serial ultrasounds (shows ventricle size)

hydrocephalus initial management

• Treatment of excessive CSF by shunt

  • surgical intervention: goes into the brain down the neck into the stomach

• treatment of complications

• Manage problems related to psychomotor development

signs and symptoms of shunt malfunction

• SHUNT MLAFUNCTION = EMERGENCY

• increased ICP

• worsening neurologic status

• altered LOC

signs and symptoms of shunt infection

• shunt malfunction

• fever and inflammation of tract

• abdominal pain

• Redness or drainage at site

microcephaly

• abnormally small head

• primary exposure: intrauterine (mother) exposure to toxins

  • CMV, Rubella (measles), toxoplasmosis, radiation

• secondary exposure:

  • third-trimester, infection, early closure of cranial sutures, metabolic disorders, anoxia

microcephaly effects

• mild hyperkinesis (muscle spasms/stiff)

• mild motor impairment

• complete unresponsiveness: autistic behavior

• Impaired brain development

craniosynostosis

• premature closure of one or more cranial sutures

• some increased ICP

• progressive papilledema (swelling in face), optic atrophy, and blindness

• generally sagittal suture closes prematurely & results in elongation of skull

craniofacial abnormalities

• usually not life-threatening

• surgical corrections if possible

  • ex. Pierre robin syndrome

    • small chin & feeding problems

congenital clubfoot

• AKA talipes equinovarus

• Incidence is 1-2 per 1000 live births

• more common in males

• bilateral clubfeet in 50% of cases

• familial tendency (history)

clubfoot categories

• positional (mild) - believed due to intrauterine crowding

• syndromic (tetralogic) - associated with other congenital abnormalities

• congenital (idiopathic) - unknown reason, wide range of severity and prognosis

therapeutic management of clubfoot

• correction of the deformity

• maintenance of the correction until normal muscle balance is regained

• follow-up observation for recurrence

management & treatment of clubfoot

• serial casting shortly after birth (progression of frequent casting)

• prognosis available

• nursing considerations → circulation, education

chromosomal alterations

• occur in 1/150 live births

• deviation in either structure or number of chromosomes

• leading cause of mental delay and spontaneous abortions

• can be related to maternal age/prenatal care

trisomy 21

• down's syndrome

• most common chromosomal abnormality

• occurs in 1/600-800 live births

• occurs more often in whites

trisomy 21 etiology

• extra copy of chromosome 21

• cause is largely unknown

• statistically greater risk for women over 35

  • women over 40 have 1/110 chance

• only 5% of time, the extra chromosome is from the father

trisomy 21 clinical manifestations

• usually diagnosed clinically, but chromosome analysis is always done to confirm outward

• outward, upper slant of eyes

• small ears

• large, protruding tongue, mouth kept open

• short, broad neck

• short, broad hands, transverse palmar crease

trisomy 21 outstanding features

• Intelligence:

  • varies from severely delayed to low-average intelligence

• Social development:

  • strength in sociability 40-45% have congenital heart defects

• Congenital abnormalities

  • 40-45% have congenital heart disease

trisomy 21: other problems

• Sensory problems:

  • cataracts

  • hearing loss

• altered immune systems:

  • leukemia

  • prone to respiratory infections

• Growth:

  • reduced height and weight

  • congenital heart anomalies

• Sexual development

  • delayed or incomplete

trisomy 21: therapeutic management

• surgical repair of serious congenital anomalies

• evaluation for sight and hearing

• special growth charts

trisomy 21: nursing considerations

• Family support

• Education regarding developmental potential and how uncertain it is

turner syndrome

• occurs in 1/10,000 female births

• missing or incomplete 2nd X chromosome

• normal growth until 3 years old

• behavioral problems:

  • difficulty with social relationships/rules

• Usually infertile

• most lead productive lives, independent adults

klinefelter syndrome

• most common sex chromosome abnormality

• have multiple X chromosomes along with Y (MALE)

• decreased masculinization with

  • gynecomastia (enlarged breasts)

  • hypogonadism (small testicles/not fully distended)

  • sterility

• mental development is usually normal

newborn chromosomal defective gene screenings → important to do as soon as birth to treat early

• Screenings exist that are reliable and inexpensive

• there is effective treatment or intervention

• if untreated, the baby will die or be severely impaired

  • some babies appear normal at birth

endocrine disorders (in Illinois)

• congenital hypothyroidism

• congenital adrenal hyperplasia

metabolic disorders (in Illinois)

• phenylketonuria (PKU)

• galactosemia

• cystic fibrosis

hemoglobinopathies (in Illinois)

sickle cell disease

congenital hypothyroidism

• autosomal recessive disorder

• deficiency of thyroid hormone

• occurs 1/3600-5000 live births

• more common in females

• Cause: thought to be caused by defective embryonic development of thyroid gland

congenital hypothyroidism symptoms

• usually appear by 6 weeks

• poor feeding

• lethargy

• prolonged jaundice

• decreased metabolism → weight gain

• respiratory difficulty

• cyanosis

• hoarse cry

• bradycardia

if congenital hypothyroidism is not treated...

• irreversible mental delays

• Develop classic features such as:

  • depressed nasal bridge, short forehead, puffy eyelids, large tongue

congenital hypothyroidism treatment

• Lifelong thyroid hormone replacement

  • synthetic levothyroxine sodium → given in morning before feeding

• education to parents:

  • need for evaluation of growth,

  • routine monitoring of thyroxine levels (frequent blood draws)

  • strict adherence to treatment (needs to occur everyday for rest of life)

congenital adrenal hyperplasia

• occurs in 1/12000-15000 births

• causes overproduction of adrenal androgens

• results in male hormones in female fetus:

  • excess androgen

  • body hair, deep voice, muscle mass

  • “masculine features”

congenital adrenal hyperplasia: clinical manifestations

varying degrees of ambiguous genitalia

• girls:

  • enlarged clitoris that resembles penis

  • fused labia which resembles a scrotum

  • internal sexual organs are normal

• boys:

  • do not display genital abnormalities

  • fast genital development

congenital adrenal hyperplasia: therapeutic management

• with early dx: cortisone is used Iif started early, very effective)

• if not diagnosed in infancy, early sexual maturation occurs

  • and both sexes appear outwardly male

• girls can develop normally with medication and surgery

phenylketonuria (PKU)

• An inherited genetic disease caused by an absence of the liver enzyme needed to convert phenylalanine to tyrosine

  • (missing the liver enzyme to break down protein)

• Seen in 1/4000-12000 live births

• Mostly affects whites

phenylketonuria (PKU) clinical manifestations

• failure to thrive

• frequent vomiting

• irritability

• hyperactivity

• in older children-

  • bizarre behavior, screaming, head banging, seizures

phenylketonuria (PKU) screening test

Guthrie test:

• detects increased levels of serum phenylalanine

• ideal if the test is done 3-4 days after ingesting formula/breast milk

• goal of screening and treatment is to prevent mental delays

• WANT TO CATCH EARLY TO TREAT EARLY

phenylketonuria (PKU) treatment

• restriction of dietary protein

  • beginning ASAP after birth and maintained throughout life

• Avoid all meat and dairy products, including

  • breads, pasta, legumes, flour, nuts, and eggs

• frequent blood phenylalanine monitoring

• Special formula

• periodic monitoring of intellectual, neurological, and behavioral parameters

phenylketonuria prognosis

• With early detection and treatment, may achieve normal cognitive development

  • Important to screen after birth before leaving the hospital

    • But outcomes vary

• Even with an adequate diet, a high % exhibit some degree of intellectual impairment

galactosemia

• inborn error of carbohydrate metabolism (problems digesting carbohydrates)

• lack of a liver enzyme that converts galactose into glucose

High levels of galactose results in…

• hepatomegaly, cirrhosis, jaundice

• enlarged spleen

• kidney failure

• cataracts

• lethargy

• hypotonia

• brain damage, death if untreated

galactosemia treatment

• Eliminate/avoid all milk and lactose-containing foods, including breast milk

  • lactose-free or soy protein formulas used

galactosemia nursing considerations

• Education on:

  • Diet → emphasize the need to read food labels and recognize early developmental delay

• Support:

  • Of mothers because they feel a great loss due to the fact they cannot feed their baby

sickle cell anemia

• most common genetic disease in the US autosomal recessive disorder

• Auto recessive gene (both parents have to have the gene)

Pathophysiology of sickle cell anemia

• obstruction caused by sickled red blood cells

  • cells stick together = get stuck/clump & can’t pass arteries

• increased hemolysis

sickle cell anemia clinical manifestations

• swollen hands/feet in newborn

• susceptibility to infection

• possible growth restriction

• chronic anemia

• jaundice

• abdominal tenderness

Vasoocclusive crisis - clinical manifestations

• painful swelling of:

  • hands & feet

  • joints

• severe abdominal pain

sickle cell therapeutic management

• Early treatment is significant

• Hydration → prevents clumping of RBCs

• Prompt treatment of sickle cell crisis

hydroxurea

The only drug approved by the FDA to reduce incidence of pain crises in children with sickle cell

sickle cell anemia nursing considerations

• adequate hydration

• no contact sports with enlarged spleen

• avoiding environments with low O2 concentration

• avoid sources of infection

• strict adherence to medication

• prompt report of fever or infection

  • even mild (get sick very quickly)

cleft lip/palate

• the most common craniofacial malformation

• more common in males

• results from incomplete fusion of the embryonic structures surrounding the oral cavity

cleft palate

• Occurs when the primary and secondary palates fail to fuse during embryonic development

• can involve only soft palate or extend into hard palate

cleft lip/palate diagnosis

• readily apparent at birth

• can be diagnosed in utero by sonogram at 14 weeks

• thorough examination of hard and soft palate with gloved finger at birth is essential

cleft lip surgery

usually involves no long term interventions after surgery

cleft palate surgery

Management after surgery usually involves a multidisciplinary team including plastic surgery, orthodontics, speech, etc.

Haberman feeders & Dr. Brown Valve/Disk

special nipple/bottle that can be squeezed in sequence with the babies feeding pattern

esophageal atresia

failure of the esophagus to develop as a continuous passage

• Esophagus doesn’t connect correctly to the stomach → food cannot reach the stomach

• Surgical intervention is needed right away

tracheoesophageal fistula

failure of esophagus and trachea to separate into distinct structures

• food will go into the trachea to the lungs

Clinical manifestations of esophageal atresia/tracheoesophageal fistula

• frothy saliva in mouth → choking and coughing

• with feeding → formula from nose and mouth

• cyanosis and cessation of breathing with aspiration of feeding (if choking)

Diagnostic evaluation of esophageal atresia/tracheoesophageal fistula

• Attempt to pass NG tube → inability warrants further investigation

• radiopaque catheter and x-rays

• bronchoscopy to visualize fistula

Therapeutic management of esophageal atresia/tracheoesophageal fistula

• maintenance of patent airway

• prevention of aspiration pneumonia

• surgical repair of abnormal structures

• crucial that they are NPO

Preoperative care for esophageal atresia/tracheoesophageal fistula

• Maintain NPO status

• IV fluids/parenteral nutrition

• careful suctioning of nose/mouth

• proper positioning

• Supportive care/Education to parents

Surgical repair: esophageal atresia/tracheoesophageal fistula

• usually one stage

• Done after adequate hydration, treatment of pneumonia proactively, and patient is well

• thoracotomy with division of TEF and end-to-end anastomosis of esophagus

Post-op care: esophageal atresia/tracheoesophageal fistula

• First feeding after surgery should be sterile water

  • If the suture isn’t correct only water will go into body

    • No formula/breast milk in case surgery is unsuccessful

pyloric stenosis

• Severe narrowing of the pyloric canal due to pylorus thickening

• Develops in the first few weeks of life

  • won’t gain weight & failure to thrive

pyloric stenosis clinical manifestations

• non-bilious vomiting in early stages → projectile vomit

• Infant is hungry, irritable

• prolonged vomiting leads to dehydration

• olive-shaped mass may be palpated (hypertrophy of pylorus)