Transcript Gastrointestinal Alterations - NURSING FDTC Batch Spring 2011

Gastrointestinal Alterations
NUR 264
Pediatrics
Angela J. Jackson, RN, MSN
Developmental Differences
• GI system of the newborn very ineffective
•
•
•
because of immaturity
Sucking and swallowing automatic reflexes until
nerves and muscles develop by 6 weeks of age
Newborn stomach capacity 10 -20ml, expanding
to 200ml by one month of age and to adult
capacity of 2000-3000ml by late adolescence
Peristalsis is greater in the infant, emptying time
of the stomach 2-3hrs in the newborn increases
to 3-6hrs by one to two months of age
Developmental Differences
• Infant’s metabolic rate is faster than an adults. Infant’s
•
•
•
•
require 100calories per kg, adults require 30-40 calories
per kg
Regurgitation common in infants (relaxed cardiac
sphincter tone)
Length of small intestine is proportionally greater in an
infant, six times the body length
Infants secretes more fluids and electrolytes into the
intestine than the adult
Large intestines of the infant are proportionately shorter
than an adult’s, resulting in less epithelial lining available
for absorption of water
Developmental Differences
• Liver function is immature at birth. Toxic
•
•
substances are inefficiently detoxified and
medications are inefficiently processed.
Infant’s are deficient in several digestive
enzymes, including amylase (carbohydrate
digestion), lipase (fat absorption), trypsin
(protein digestion)
Infant’s intestines are more permeable to
proteins, allowing passage of protein into the
bloodstream, increasing susceptibility to food
protein allergens
Cleft Lip and Cleft Palate
Cleft Lip and Cleft Palate
• Most common craniofacial malformation
• Cleft lip: Simple notch in vermillion border
of lip to complete opening extending to
floor of the nose. May be unilateral or
bilateral
• Cleft palate: Fissure or small opening to
complete opening between mouth and
nasal cavity. May be isolated or occur
with cleft lip
Cleft Lip and Cleft Palate: Etiology
• Mulitfactorial: Genetic –
•
increased incidence in
families
Environmental factors:
Drugs (anticonvulsants),
alcohol, smoking,
maternal illness,
infection, folic acid
deficiency, parental age
Cleft Lip and Cleft Palate: Etiology
• Cleft lip with or
•
without palate – more
common in males
Cleft palate more
common in females
Cleft Lip: Complications
• Cleft Lip and Cleft Palate:
– Facial disfigurement may cause shock, guilt and grief
for the parents and may block parental bonding with
the child
• Cleft Lip:
– Abnormal development of external nose, nasal
cartilages, nasal septum, usually nose is flattened
– Dental anomalies: missing, malpositioned teeth on
side of abnormality
Cleft Palate: Complications
• Cleft Palate:
– Infants have feeding problems because they
are unable to develop suction due to opening
between mouth and nasal cavity.
– Increases the risk of aspiration: the increased
open space in the mouth causes some
formula to exit through the nose
– Increases the risk of upper respiratory
infection and otitis media
Cleft Lip and Cleft Palate: Diagnosis
• Prenatal by ultrasound (may be seen by
13 to 16 weeks gestation)
• Apparent on assessment at birth
• Unable to generate negative pressure in
oral cavity to suck and may impair ability
to swallow resulting in feeding difficulties
Cleft Lip and Cleft Palate:
Therapeutic Management
• Multidisciplinary care: plastic surgeon,
•
•
neurosurgeon, orthodontist, otolaryngologist,
speech pathologist, pediatrician
Surgical closure of Cleft Lip: 3 months or 12
pounds
Palate prosthesis may be used prior to surgery
for a cleft palate. Cleft palate surgery is done
before child develops faulty speech patterns
(approximately 12 - 18 months)
Cleft Lip and Cleft Palate:
Therapeutic Management
• Prevention of complications: infection,
speech difficulties, malocclusion
problems, hearing problems
• Facilitation of parent-child bonding,
normal growth and development,
speech and language development
Cleft Lip and Cleft Palate: Nursing
Management
• Newborn assessment: assess ability to suck, swallow
•
•
•
•
•
and feed
Assess parents reaction to infant
Teach feeding pre and post op: Feed in an upright
position, use soft, elongated nipple, frequent burping,
prevent aspiration, adequate nutrition, breastfeeding
Monitor weight gain
Postop care: Logan bow, restraints, pain medication,
cleansing wound site and mouth, positioning (side or
supine for cleft lip, side or prone for cleft palate)
Discharge teaching and Home care planning: feeding,
suture care, restraints, pain management and follow-up
Esophageal Atresia (EA) and
Tracheoesophageal Fistula (TEF)
EA & TEF
• Failure of esophagus to develop as a continuous
passage to the stomach and failure of the
esophagus and trachea to separate into distinct
structures
EA & TEF: Clinical Manifestations
• Frothy saliva in mouth
• Drooling, coughing and choking
• Sudden coughing, gagging at feedings.
Feeding comes out of the nose and
mouth, becomes cyanotic and stops
breathing as feeding is aspirated
• When the trachea joins the stomach, the
stomach becomes distended with air
EA & TEF: Diagnosis
• Polyhydramnios should suggest the possibility of
•
•
•
a high gastrointestinal obstruction
Inability to identify fetal stomach bubble on a
prenatal ultrasound
Inability to pass a nasogastric tube into the
stomach
Radiographic studies: curling of NG tube in
upper esophagus, air in the stomach
EA & TEF: Therapeutic
Management
•
•
•
•
•
Maintain a patent airway
Prevent pneumonia
Gastric or blind pouch decompression
Surgical repair as soon as possible
Preferably, surgical repair is done in one stage,
but may have to be done in two stages, based
on infants condition:
– 1st stage: Close TEF and insert gastrostomy tube
– 2nd stage: Connect ends of esophagus
EA & TEF: Nursing Considerations
• Preoperative care:
– Maintain patent airway: have suction available
– Thermoregulation
– Insert NG tube for gastric decompression
– Keep NPO
– Establish IV access to maintain hydration
EA & TEF: Nursing Considerations
• Postoperative care:
– Maintain patent airway
– Prevent trauma to the anastomosis
– Elevate gastrostomy tube and keep open to allow gas
to escape and gastric secretions to flow into the small
intestine
– Provide emotional support to family, encourage
bonding
– Teach parents gastrostomy care, signs of infection,
signs of esophageal stricture
Gastroschisis and Omphalocele
Gastroschisis
• Extrusion of the large
and small intestine
through a defect in
the abdominal wall to
the right of the
umbilical cord. The
bowel has no
covering membrane,
and is exposed to the
amniotic fluid
Omphalocele
• Herniation of
abdominal contents
through the umbilical
cord. The bowel is
covered by a
protective sac, and
the size of the defect
is variable, ranging
from 1cm to all of the
abdominal contents
Gastroschisis and Omphalocele:
Diagnosis
• Maternal serum
•
alpha-fetoprotein
(elevated)
Prenatal Ultrasound
Gastroschisis and Omphalocele:
Therapeutic Management
• Cover exposed contents loosely with sterile
•
•
•
saline-soaked pads and plastic drape to prevent
water loss, drying and temperature instability.
Gastroschisis: be careful not to wrap around
bowel – can cause pressure necrosis as exposed
bowel expands
IV fluids
Antibiotics
Surgical closure: primary or staged repair
Gastroschisis and Omphalocele:
Surgical Closure
• Primary closure: All of the exposed bowel is placed
•
inside the abdominal cavity and the abdomen is closed
during a single procedure
Staged repair: When the abdominal cavity is not large
enough to contain all of the exposed bowel, the
abdominal wall is stretched and a silo is created. The
edges of the abdominal wall are sutured to plastic
sheeting that is then suspended with mild tension to the
top of the incubator. The silo and the suture lines are
covered with providone-iodine to prevent infection. The
silo is gently compressed on a daily basis until all of the
exposed bowel is enclosed in the abdominal cavity. This
process takes several days (7-10) to complete. Once all
of the exposed bowel is contained within the abdominal
cavity, the silo is removed and the abdominal wall is
closed
Gastroschisis and Omphalocele:
Potential Complications
• Postoperative complications: respiratory
distress due to increased abdominal
pressure, infection, intestinal obstruction,
vena cava compression resulting in
decreased blood flow to extremities,
evisceration, intestinal volvulus
Gastroschisis and Omphalocele:
Nursing Considerations
• Preoperatively: careful handling and positioning,
•
•
prevent infection, thermoregulation, assess
respiratory status, fluid replacement
Postoperatively: mechanical ventilation, IV
fluids, TPN, antibiotics, monitor for signs of
complications, assess bowel function
Family support, discharge teaching – parenteral
nutrition, oral stimulation, signs of bowel
obstruction, follow-up care
Biliary Atresia
• Absence of bile duct or
obstruction of bile duct
prevents flow of bile from the
liver to the gallbladder and
small intestine
• Bile accumulates in the liver
and bile plugs form
• Inflammation, edema and
irreversible liver injury occur,
resulting in fibrosis of the
liver
• Causes excretion of bilirubin
and bile salts in the urine
Biliary Atresia: Clinical
Manifestations
• Jaundice, developing between 2 weeks and 2 months of
•
•
•
•
•
•
•
age
Tea colored urine due to bilirubin and bile salt excretion
Light colored stools due to absence of bile pigments
Hepatomegaly
Abdominal distention
Bruising due to prolonged bleeding time
Intense itching
Failure-to-thrive, malnutrition as fat and fat-soluble
vitamins can not be absorbed
Biliary Atresia: Diagnosis and
Treatment
• Diagnosed by ultrasound, liver biopsy and
increased bilirubin levels
• Fatal without treatment:
– Surgery: hepatic portoenterostomy (Kasai
procedure, formation of a substitute duct) –to
correct obstruction and allow for drainage of
bile from liver into intestines
– Surgery is not a cure; end stage liver disease
may result in need for liver transplant
Biliary Atresia: Nursing
Considerations
• Monitor nutrition – fat soluble vitamin
supplements, TPN, Intralipids, daily
weights
• Monitor I&O, stool patterns
• Good skin care
• Discharge teaching (nutrition, skin care,
support groups, signs of rejection)
Diaphragmatic Hernia
• Herniation of the
•
abdominal contents
through a defect in the
diaphragm into the chest
cavity and usually
develops on the left side
Intestines and abdominal
structures enter the
thoracic cavity,
compressing the lung
Diaphragmatic Hernia: Clinical
Manifestations
• Severe respiratory distress
• Cyanosis, tachypnea, retractions
• Decreased or absent breath sounds on the side
•
•
•
•
of the defect
Barrel-shaped chest
Heart sounds shifted to the right
Bowel sounds heard over the chest
Scaphoid abdomen
Diaphragmatic Hernia: Diagnosis
• Prenatal ultrasound
• Chest x-ray
Diaphragmatic Hernia: Treatment
• Respiratory support – O2, intubation, No
Bag/Mask ventilation, pulse oximeter
• Blood gases to monitor acid-base status
• Jet ventilator or High frequency ventilation
for severe respiratory distress
• ECMO (extra corporeal membrane
oxygenation)
Diaphragmatic Hernia: Treatment
• Position head and thorax higher than abdomen,
•
•
•
place on affected side
IV fluids
NG tube to decompress stomach/intestines
Medications
– Opoids (Fentanyl) or paralyzing agents (pancuronium)
if agitated
– Isotropic (Dopamine) for blood pressure support and
improve cardiac output
– Surfactant to improve oxygenation
– Sodium bicarbonate to prevent acidosis
Diaphragmatic Hernia: Treatment
• Surgical repair
– Fetal surgery
– Post-birth surgery: return abdominal contents
to abdomen and close defect in diaphragm
Diaphragmatic Hernia: Nursing
Considerations
• Preoperative care: stabilization
• Postoperative care:
– Position on affected side to facilitate expansion of
unaffected lung
– Maintain respiratory support
– IV fluids, fluid and electrolyte balance
– Stress reduction
– Prevent infection
– Pain management
– Chest tube care
– Family support, promote bonding
– Discharge teaching (wound care, feedings, prevention
of infection)
Anorectal Malformations
• Congenital anomalies of the anus and
rectum caused by abnormal development
• Imperforate anus: any abnormality
without an obvious opening – may have a
fistula from rectum to perineum or GI
tract
Anorectal Malformation: Clinical
Manifestations
• No anal opening
• Failure to pass
•
meconium within 24
hours after birth
Passing meconium
through the urethra
or vagina
Anorectal Malformations: Diagnosis
• External physical exam
• X-rays with contrast
• Abdominal ultrasound
Anorectal Malformations:
Treatment
• Anal stenosis – manual
•
•
•
dilations
Imperforate anal
membrane – excise
membrane and manual
dilations
Perineal fistula –
Anoplasty and dilations
High or intermediate
malformations –
Colostomy, repair defects
between 6-12 months
Anorectal malformations: Nursing
Considerations
• Complete assessment at birth
– Rectal temperature
– Asses for passage of meconium stool
• Preoperative care: assist with diagnostic
evaluation, IV fluids, gastrointestinal
decompression
Anorectal Malformations : Nursing
Considerations
• Postoperative care:
– perineal care, protective ointments and dressings to
protect skin
– Positioning: side-lying with hips elevated or supine with
legs elevated to prevent pressure on perineal sutures
– Colostomy care
– NG tube for abdominal decompression
– IV fluids ( feedings begin when peristalsis returns)
– Family support
– Discharge teaching: perineal and wound care, prevention
of constipation, colostomy care, delayed toilet training,
manual dilations, observe for complications, follow-up care
Pyloric Stenosis
• An increasing hyperplasia
•
and hypertrophy of the
circular muscle at the
pylorus, narrows the pyloric
canal. Most commonly seen
in male infants between
ages 1 and 6 months
Hypertrophy eventually
obstructs the pylorus
opening. Obstruction
results in inability of food to
pass out of the stomach,
causing vomiting
Pyloric Stenosis: Clinical
Manifestations
• Vomiting beginning at 3-6 weeks of age. Infant begins
•
•
•
•
•
•
to regurgitate small amounts of formula immediately
after feeding, which progresses to projectile vomiting
Vomiting can occur during feedings, after feedings or
several hours later
Infant will be hungry after vomiting and will accept a
second feeding
Failure to gain weight
Sign and symptoms of dehydration
Palpable olive-shaped mass in right upper quadrant
Visible peristaltic waves
Pyloric Stenosis: Diagnosis
• History and physical
• UGI
• Ultrasound
Pyloric Stenosis: Treatment
• Correct electrolyte
•
imbalances and
dehydration
Pyloromyotomy: split
the muscle to make
the opening larger
Pyloric Stenosis: Nursing
Considerations
• Record emesis – frequency,
characteristics, relationship to feedings
• I&O
• Daily weights
• Pre-op care: correct electrolytes,
hydration, insert NG tube, family teaching
Pyloric Stenosis: Nursing
Considerations
• Post-op care:
–
–
–
–
–
–
IV fluids
Begin feedings as ordered
Place on right side with head elevated after feeding
Monitor I&O
Monitor labs
Wound care: observe for drainage, redness, change
dressings as ordered
– Pain management
– Discharge teaching: Wound care, S/S of infection,
feeding
Intussusception
• Invagination or telescoping of one portion of
intestine into another portion of intestine, most
common site is the ileocecal valve
Intussusception: Clinical
Manifestations
• Acute, colicky abdominal pain: screaming and
•
•
•
•
•
drawing knees to chest
Non-bilious vomiting in early stage, becomes bile
stained
Blood and mucus in the stool (currant jelly
stools)
Sausage-shaped mass in right upper quadrant
Abdomen is tender, distended
Lethargy, fever, weak thready pulse, shallow
respirations
Intussusception: Diagnosis
• Barium or air contrast
•
enema x-rays
Abdominal ultrasound
Intussusception: Treatment
• Non-surgical hydrostatic reduction with
barium, water-soluble contrast or air
enema
• Surgical intervention if there is evidence of
intestinal perforation, peritonitis or shock,
or if enema is unsuccessful
Intussusception: Nursing
Considerations
• Obtain history of symptoms from caregivers
• Explain procedures to caregivers
• Pre-intervention care: correct electrolyte
•
•
•
imbalance & hemorrhage with fluid replacement,
administer antibiotics, NG tube, monitor I&O
Post-intervention care: observe for passage of
contrast material
Support family and encourage family to stay
with pt
Discharge teaching: S&S of recurrence
Hirschsprung’s Disease
• Motility disorder of
the bowel caused by
the absence of
parasympathetic
ganglion cells in the
large intestine
Hirschsprung’s Disease: Clinical
Manifestations
• Infant: failure to pass meconium within 24-48
•
hours of birth, abdominal distention, bile stained
vomiting, refusal to eat, intestinal obstruction
Older infant and child: chronic constipation,
abdominal distention, explosive passage of stool,
inadequate weight gain, ribbon-like or pellet
shaped stool, foul-smelling stool, vomiting,
palpable fecal mass
Hirschsprung's Disease: Diagnosis
• Rectal exam –
•
•
absence of stool in
rectum with tight anal
sphincter
Barium enema
Rectal biopsy for
absence of ganglion
cells
Hirschsprung’s Disease: Treatment
• First stage surgery – colostomy to allow for
•
•
•
means of defecation and to rest bowel
Second stage surgery – aganglionic section is
removed and normal bowel is anastomosed to
rectum and the colostomy is closed. Performed
when child is between 6-15 months of age and
weighs 18-20 pounds
Newer surgery is to do second stage surgery
without first stage surgery
Laparoscopic Surgery – pull affected bowel
through anal opening to remove
Hirschsprung’s Disease: Nursing
Considerations
• Pre-op management: IV’s for fluid and
•
•
electrolyte balance, saline enemas to cleanse
bowel, NPO, NG tube, antibiotics and antibiotic
enemas
Post-op management: NG tube management
until peristalsis returns, monitor abdominal
distention and incisions, colostomy care
Family teaching: colostomy care, incision care,
complications such as enterocolitis, leaks or
strictures at anastomosis site (abdominal
distention, irritability, diarrhea, vomiting,
constipation)
Inflammatory Bowel Disease (IBD):
Crohn’s Disease and Ulcerative Colitis
• Chronic disorders that cause inflammation or
•
•
ulceration in the small and large intestine
Crohn’s disease may involve the entire
gastrointestinal tract and all layers of the bowel
wall, with an asymmetric distribution of lesions
(disease-free skip areas)
Ulcerative colitis involves inflammation of the
mucosa and submucosa of the colon and
rectum, with a symmetric, continuous
distribution of lesions
IBD: Clinical Manifestations
•
•
•
•
•
•
•
Diarrhea
Abdominal pain
Rectal bleeding
Weight loss
Growth retardation
Perianal disease (painful defecation, bright red
rectal bleeding, skin tags, hemorrhoids, fistulas,
and abscesses)
Fistulas
IBD: Diagnosis
• History and physical exam
• Endoscopy
• Colon x-rays: barium enema, upper GI
series, CT scan for bowel thickening
• Labs: CBC (anemia), elevated WBC & sed
rate, hypoproteinemia, fluid and
electrolyte imbalances, stool for blood,
leukocytes, and culture
• Mucosal biopsy
IBD: Treatment
• Control inflammatory process:
– Corticosteroids,
– Sulfasalazine (acts directly on the bowel
mucosa to reduce inflammation)
– Antibiotics, Flagyl (helpful for the perianal
conditions associated with CD)
– Immunosuppressive medications
IBD: Treatment
• Promote growth and development:
•
– High-protein, high calorie foods
– Vitamin, iron, folic acid supplements
– Enteral feedings
– TPN
Surgical treatment
– Total colectomy
– Subtotal colectomy with ileostomy
– Intestinal resections for obstructions
IBD: Complications
•
•
•
•
•
Malnutrition
Growth failure
Intestinal strictures
GI bleeding
Toxic megacolon (acute dilation of colon
secondary to severe inflammation of bowel
mucosa. S/S: spiking fever, acute abdominal
pain, distention – colon can thin out and shred
leading to hemorrhage, peritonitis, and death.
If colon perforates, emergency surgery with
total colectomy and ileostomy)
IBD: Nursing Considerations
• Nutrition
– Several small meals/day
– Supplements (Ensure)
– Decrease bran and fiber foods
– Decrease dairy products
– Bland foods, avoid hot, spicy foods
• Medications
– Continue medications while in remission
– Monitor side effects
• Teach ostomy care and coping skills
– Give support and referrals
– Interventions to raise self-esteem
Necrotizing Enterocolitis (NEC)
• Necrosis of the mucosa of the small and large
•
•
intestine, usually distal ileum and proximal colon
Life-threatening condition of preterm infants
Risk factors: intestinal ischemia, bacterial
colonization of bowel, presence of hypertrophic
solutions (formula) in intestines, perinatal
asphyxia, respiratory distress syndrome,
exchange transfusions, umbilical artery catheters
NEC: Clinical Manifestations
• Abdominal tenderness
•
•
•
•
•
and distention
Redness of abdominal
wall
Bloody stools
Decreased bowel sounds
Increased gastric
residuals
Bilious vomiting
NEC: Clinical Deterioration
•
•
•
•
•
•
Apnea and bradycardia
Lethargy
Temperature instability
Decreased urine output
Increased abdominal distention
Shock (cool, mottled skin, pallor, decreased intensity of
peripheral pulses)
• Hypotension
• Acidosis
• Sepsis
NEC: Diagnosis
• Clinical findings
• Abdominal x-rays: dilated bowel loops,
pneumatosis intestinalis
NEC: Treatment
• NPO
• NG tube for continuous gastric drainage and
•
•
•
•
•
decompression
O2 and ventilation if indicated
IV fluids for parenteral nutrition, electrolytes,
antibiotics
Monitor labs: CBC, blood gases, electrolytes
Surgery if no improvement: resection of necrotic
bowel
Postop complications: intestinal obstruction due
to strictures, short bowel syndrome
NEC: Nursing Considerations
•
•
•
•
•
•
•
•
•
Early identification of clinical manifestations
Measure abdominal girth
Gastric residual prior to feedings
Presence of bowel sounds
Hemoccult all stools
Accurate I&O
Frequent VS
Observe response when feedings begin again
Family teaching: answer question and explain
procedures, care of ostomy, feedings, observation of
complications
Short Bowel Syndrome
• Disorder characterized by inadequate
surface are of the small intestine and
usually occurs after surgical resection of
the intestine in cases of necrotizing
enterocolitis, volvus, or Crohn’s disease.
• May not be a permanent disorder because
the intestine can grow and adapt
Short Bowel Syndrome: Clinical
Manifestations
•
•
•
•
•
•
•
Malabsorption
Malnutrition
Diarrhea
Electrolyte imbalances
Vitamin and mineral deficiencies
Skin irritation and breakdown on the buttocks
and perineum
Bacterial overgrowth in the remaining small
intestine
Short Bowel Syndrome: Treatment
• TPN via a central line
• Enteral feedings via an NG or gastrostomy
tube to stimulate growth of the small
intestine
Short Bowel Syndrome: Nursing
Considerations
•
•
•
•
•
Provide good skin care
Monitor I&O
Administer TPN and tube feedings
Monitor for signs and symptoms of infection
Monitor lab values: liver function tests, renal
function tests, liver enzymes, calcium,
magnesium and phosphorus levels
Inborn Errors of Metabolism:
Phenylketonuria (PKU)
• PKU: Disease of protein metabolism,
characterized by the absence of the
hepatic enzyme needed to metabolize the
amino acid phenylalanine
• Inherited as an autosomal recessive trait
• Detected in 1:10,000 – 25,000 births
• Highest incidence in Caucasian children
PKU: Clinical Manifestations
•
•
•
•
•
•
•
Failure to thrive
Frequent vomiting
Irritability
Hyperactivity
Unpredictable, erratic behavior
Bizarre behavior patterns in older children
(screaming episodes, head banging, arm biting,
disorientation, catatonia)
Seizures
PKU: Diagnosis
• Guthrie blood test performed on fresh heel
•
•
•
blood, not cord blood
Test is most reliable if blood sample is taken
after the infant has ingested a source of protein
Initial specimens that are taken within the first
24 hours of life should be repeated by the
second week of life
After 3 months of treatment, a natural protein
challenge test should be performed to confirm
the diagnosis of PKU
PKU: Treatment
• Low-phenylalanine diet
• Diet is calculated to maintain serum
•
•
•
phenylalanine levels between 2 and 8mg/dl
Significant brain damage occurs when levels are
greater than 10-15mg/dl
Growth retardation occurs at levels less than
2mg/dl
Daily amounts are individualized for each child
and require frequent changes based on appetite,
growth and development and blood levels
PKU: Treatment
• This target is an easy
way to visualize the
foods allowed on the
diet for PKU. PhenylFree* is the center of
the target diet. As the
foods get further away
from the bull's-eye, they
are higher in
phenylalanine. The
foods outside the target
are not included in the
low-phenylalanine meal
plan.
PKU: Nursing Considerations
• Assessment and treatment of symptoms
• Teaching family dietary requirements:
low-phenylalanine foods, eliminated or
restricted protein foods, no aspartame
• Assist school-age children and adolescents
to cope with peer pressure, and to
monitor daily allowance of phenylalanine
• Family support
Inborn Errors of Metabolism:
Galactosemia
• Error in carbohydrate metabolism
• Autosomal-recessive disorder
• Affects approximately 1:50,000 births
• Death occurs during the first month of life
if untreated
Galactosemia: Clinical
Manifestations
•
•
•
•
•
•
•
•
•
Jaundice
Vomiting
Weight loss
Hepatosplenomegaly
Diarrhea
Drowsiness
Lethargy
Hypotonia
Sepsis
Galactosemia: Diagnosis
• Guthrie blood test
• History
• Physical exam
• Galactosuria
• Increased levels of galactose in the blood
• Decreased levels of enzyme
Galactosemia: Treatment
• Eliminate all milk and lactose-containing
foods, including breast milk
Galactosemia: Nursing
Considerations
• Same as for PKU except for lactose dietary
restrictions
• Many drugs contain lactose as a filler and
must be avoided
• Diet is easier to maintain because many
more foods are allowed
Protein and Energy Malnutrition:
Kwashiorkor and Marasmus
• Kwashiorkor:
Deficiency of protein
with low or adequate
supply of calories
from carbohydrates
Kwashiorkor
• Growing cells are damaged due to deficient
•
protein for tissue growth and cell repair
Clinical manifestations include:
–
–
–
–
–
–
–
–
Scaly, dry skin
Loss of hair
Night blindness
Weight loss in conjunction with generalized edema
(hypoalbuminemia)
Hypokalemia
Hypernatremia
Muscle atrophy
Diarrhea
Marasmus
• General malnutrition
•
•
of both calories and
protein
Common in
underdeveloped
countries during times
of drought
Failure to thrive
Marasmus
• Gradual wasting and atrophy of body tissues,
•
especially subcutaneous fat
Clinical manifestations include:
–
–
–
–
–
–
–
–
Children appear to be very old, with flabby, wrinkled skin
Severely emaciated appearance
Small head with decreased brain growth
No skin sores
No edema
Infection-prone
Minimal body metabolism – thermoregulation problems
Apathetic, fretful withdrawn, lethargic
Kwashiorkor and Marasmus:
Treatment
• Diet with quality proteins, carbohydrates,
vitamins, minerals introduced slowly
• Rehydrate with fluids to replace
electrolytes
• Medications: antibiotics, anti-diarrheals
Kwashiorkor and Marasmus:
Nursing Considerations
• Provide essential physiological needs:
– Food
– Rest
– Protection from infection
– Hygiene
– Developmental stimulation
• Provide education about proper nutrition
Fluid and Electrolytes:
Developmental Differences
• Water constitutes approximately 80% of body
•
•
•
weight of infants, 65% of body weight in
children, and 50% of body weight in adults
Infants have more extracellular fluid than
intracellular fluid
Infants and children <2 years of age lose a
greater proportion of fluids per day
Infants have greater BSA to weight ratio and
higher metabolism, making them more prone to
fluid depletion and increased water lose through
the skin
Fluid and Electrolytes:
Developmental Differences
• Infants and children have greater GI surface area
• Infants and children take in and excrete a greater
•
•
volume of water daily
Infants and children <2 have immature kidney function
and are inefficient in concentrating or diluting urine,
conserving or excreting Na, and acidifying urine. They
are more likely to become dehydrated with concentrated
formula or overhydrated with dilute formula or excessive
water
Fluid disturbances in infants and children are usually
caused by dehydration, water intoxication and edema
Dehydration
• A critical condition that results from an
extracellular fluid loss
• Children are more susceptible to
dehydration than adults because a larger
portion of a child’s body fluid is located in
extracellular spaces
• Dehydration that is not corrected will lead
to hypovolemic shock and death
Dehydration
• Hypotonic: occurs when there is a sodium
loss that is greater than the water loss
• Isotonic dehydration: occurs when the
sodium and water loss are equal. Most
common type of dehydration in children
• Hypertonic: occurs when the loss of water
is greater than the loss of sodium
Dehydration: Clinical Manifestations
•
•
•
•
•
•
•
•
Weight loss
Rapid, thready pulse
Hypotension
Decreased peripheral
circulation
Decreased urinary output
Dry mucous membranes
Absence of tears
Sunken fontanel
• Dehydration is
classified as:
– Mild (<5% weight
loss)
– Moderate (5-10%
weight loss)
– Severe (>10% weight
loss)
Dehydration: Diagnosis
• Based on history, physical exam
• Measurement of acute weight loss
• Hypotonic and isotonic dehydration:
hemoglobin, hematocrit, glucose, BUN,
creatine and protein elevated. Urine is
highly concentrated, dark amber in color,
has strong odor
Dehydration: Treatment
• Oral rehydration is the preferred route,
using a solution that contains glucose,
sodium, potassium and bicarbonate.
• If child has persistent vomiting, severe
dehydration, or is unable to drink for other
reasons, rehydration is achieved through
administration of IV fluids, based on the
type of dehydration
Dehydration: Nursing
Considerations
• Physical assessment for signs and
symptoms of dehydration
• Daily weights
• Measure I&O
• Administer IV fluids
• Teach caregiver the signs of dehydration
• Teach parents about oral rehydration
fluids
Any Questions?