Pediatric Nursing Flashcards
1
Q
Tetralogy of Fallot manifestation
A
cyanosis at birth
murmur
blue spells or tet spells during crying or feeding
complications include: emboli, seizures, loss of consciousness, death from anoxia
2
Q
tetralogy of Fallot diagnostic studies
A
echocardiography
chest radiography
3
Q
Tetralogy of Fallot management/nursing intervention
A
place infant on side during a tet spell; knees to chest position
older kids can squat which can help with circulation
remain calm and apply 100% oxygen via facemask
administer morphine and IV fluids
monitor adequate fluid status through weight, strict intake and output
give parenteral nutrition
4
Q
Tetralogy of Fallot caregiver teaching
A
promptly treat diarrhea, vomiting and fever
good handwashing technique
antibiotics
regular check ups
5
Q
what is tetralogy of fallot
A
congenital heart disorder that has 4 types of defects:
VSD, overriding aorta, pulmonic valve stenosis, right ventricular hypertrophy
6
Q
what is kawasaki disease
A
happens from an unknown factors but is a acute systemic vasculitis
small and medium vessels become inflamed
then it tries to go back down but then there is scarring, thickening and calcified
most common cause is coronary artery disease
7
Q
kawasaki disease manifestations
A
acute: high fever that lasts at least 5 days and is unresponsive to treatment strawberry tongue reddened conjunctivitis rash on main body and genital region reddened palms/sores irritability and cervical lymphadenopathy reddened cracked lips
subacute: acute symptoms go thrombocytosis coronary artery dilated skin peeling of the fingers and toes arthritis affecting the large weight bearing joints
convalescent phase: most of the symptoms go away but abnormal lab values still occur: anemia increased WBCs elevated ALT thrombocytosis albumin <30 urine WBC >10
8
Q
kawasaki disease diagnostic studies
A
no specific test is diagnosed
CBC, liver enzymes, urinalysis
echocardiogram
9
Q
kawasaki nursing managment
A
pharmacological intervention:
IVIG with salicylate (with this the child needs to be on continuous cardiac monitoring)
- may be premedicated with diphenhydramine and acetaminophen
the nurse should:
monitor cardiovascular status by daily checking weights, intake and output
offer cool cloths, mouth care, lubrication of dry lips and unscented lotion
soft and cool foods
give aspirin for inflammation and acetaminophen for fevers
perform gentle range of motion exercises if child develops arthritis
encourage rest in a quiet environment
10
Q
kawasaki teaching
A
teach side effects of aspirin and toxicity
follow up visit with cardiologists within the week of discharge and 4-6 weeks later
avoid MMR and varicella immunizations for about 11 weeks after IVIG therapy
IVIG therapy needs to be started within 10 days of symptoms
11
Q
what is acute epiglottitis
A
life threatening bacterial or viral upper airway inflammation that can lead to airway obstructions
often caused by H influenzae type B
12
Q
manifestations of acute epiglottitis
A
abrupt onset of sore throat symptoms of upper respiratory infection difficulty swallowing pain drooling, sitting in a tripod position tongue protrusions, agitation, muffled voice
sxs of respiratory distress: retractions, cyanosis and shallow breathing or stridor
13
Q
acute epiglottitis diagnostic studies
A
lateral neck radiograph
throat culture
14
Q
acute epiglottitis management
A
children with severe respiratory distress will need emergency intubation
iv antibiotics followed by oral antibiotics 7-10 days
the nurse should
remain calm and decrease child’s anxiety
allow child to remain upright and prepare for emergency intubation
monitor for icnreasing respiratory distress
initiate and maintain droplet isolations for 24 horus after antibiotics are started
15
Q
what is cystic fibrosis
A
impacts that glands that produce mucous
defect on the chromosome number 7
-cells experience can increase in mucus secretion and obstruction
16
Q
what are common sites of obstructions in cystic fibrosis
A
bronchi
small intestines
pancreatic ducts and bile ducts
if it gets in the reproductive tract that could lead to infertility
17
Q
manifestations of cystic fibrosis
A
wheezing nonproductive cough chronic cough bronchial obstruction respiratory infections dyspnea atelectasis barrel chest appearance pneumothorax chronic hypoxia (clubbing, cyanosis)
gastrointestinal: meconium ileus fatty/smelly stools (steatorrhea) poor weight gain failure to thrive distended abdomen prolapsed rectum poor vitamin absorptions (A, D, E, K) pancreatic duct blockage - inability to absorb proteins/facts, hyperglycemia
reproductive:
delayed puberty for girls
increased risk for miscarriage and premature delivery
integumentary:
salty skin because salt loss through skin
18
Q
cystic fibrosis diagnostic studies
A
sweat chloride test
- positive if over 60
genetic testins for mutation of CFTR gene
chest radiography
pulmonary function tests
contrast edema
stool anaylsis
19
Q
cystic fibrosis management
A
pharmacotherapy will include:
bronchodilators,
nebulized hypertonic saline,
dornase alpha - clears airway secretion
collaborate with respiratory therapists
airway clearance therapy will help limit mucus and respiratory infection
percussion and postural drainage through expiratory therapy,
breathin techniques,
high frequency chest compression therapy
monitor for signs of sepsis and pneumothorax
20
Q
cystic fibrosis nursing goals
A
prevent respiratory infection
improve nutrition
encourage physical activity
promote overall good quality of life
21
Q
cystic fibrosis nutrition
A
assess food intake, weight gain, BMI
assess stools, bowel sounds and abdominal distention
reduce rectal prolapse through manual manipulations
pancreatic enzyme replacements
22
Q
what is reye syndrome
A
metabolic encephalopathy
fatty changes to the liver and cerebral edema
23
Q
reye syndrome manifestation
A
fever
vomiting
lethargy
delirium
seizures
coma
increasing intracranial pressure
cerebral edema
hepatic dysfunction
24
Q
reye syndrome diagnostic studies
A
liver biopsy
elevated ammonia levels
25
reye syndrome nursing management
the nurse should:
- place the child in a quiet environment
- monitor for increasing intracranial pressure
- monitor intake and output
avoid aspirin or aspiring-containing meds
26
what are neural tube defects
neural tube that contains the spinal cord fails to close in utero
occurs more often in girls and the Hispanic population
27
neural tube defects risk factors
exposure to drugs
malnutritions
radiation
chemicals or genetic mutations
28
neural tube defects manifestations
spina bifida:
no sac but you may have a dimple or a tuft of hair
bladder and bowel dysfunction
port wine angiomatous veti
meningocele:
saclike protrusion that contain the meninges and cerebrospinal fluid
```
myelomeningocele:
saclike protrusion that contains the meninges, nerves and cerebrospinal fluid
may result in lower limb paralysis
bladder and bowel dysfunction
rectal prolapse
joint deformities
```
29
neural tube defects diagnostic studies
ultrasonography
CT
MRI
history and physical assessment
30
neural tube defects management
surgery in order to close the sac
prior to surgery, use padding instead of diaper
- change often to decrease infection
a protective covering on the sac
checks vitals, intake and output, pain and incision
urinary catheterization may be necessary
moist covering on sac
- changed every 2-4 hours
the infant should be lying in a prone position in an incubator or a temperature radiant
you can place the infant on a pressure reducing mattress
they may have an increasing risk of getting a latex allergy so monitor for reactions
perform gentle range of motion to prevent contractures
caregiver teaching:
bond with infant through touching, caressing and stroking
best position is prone or side lying
monitor incision site for pain and infection
perform prescribed exercises and monitor for complications
31
what is duchenne muscular dystrophy
most common and severe form of muscular dystrophy
X-linked recessive trait found in biopsy
symptoms typically begin to appear at the ages 3-5 years old
small muscles are destroyed and joint deformities happen
in the end stage, the muscles get to the respiratory and cardiovascular --> so it gets to the heart and lungs
32
duchenne muscular dystrophy manifestations
initial symptoms are:
lordosis= inward curvature of the spine especially the lower back
waddling gait
frequent falls
toe walking
difficulty climbing stairs or getting up from a chair
progressive symptoms are:
muscle wasting
contractures
weakness
scoliosis
atrophy
infection
obesity
death from respiratory failure and cardiac failure
loss of ambulation by age 9-12
positive gowers sign: when trying to sit up, you need to walk your hands up your legs to stand
33
duchenne muscular dystrophy diagnostic studies
genetic testing
muscle biopdy
serum enzyme measurements
serum CK levels
EMG
DNA anaylsis of blood and muscle tissue
34
Duchenne muscular dystrophy management
there is no curative treatment
corticosteroids
- to improve respiratory function
bracing, knee-ankle orthotics may be used to prevent contractures
the nurse should:
include stretching and exercises that strengthen the muscles
use incentive spirometry:
- BiPAP,
- MAC ventilator,
- chest physiotherapy
- tracheostomy
consider long term and palliative care
caregiver teaching:
teach how to record and use pulse oximetry to monitor when they are sleeping
keep them active as long as possible
only use a wheelchair as a last resort
fall prevention and safety
siblings who feel isolated may need psychological counseling
get genetic counseling
35
duchenne muscular dystrophy care team
```
pulmonologist
cardiologist
speech therapy
physical therapist
dietician
orthopedist
```
36
what is hirchsprung disease
it is due to the absence of ganglionic cells
there will be a delayed in peristalsis
wont be able to relax the colon sphincter in order to pass stool
portion of the colon may be affected or the entire colon and small intestine
37
hirchsprung disease manifestations
newborn symptoms;
no meconium stools after birth
bilious vomiting
abdominal distention
refusing feeding
infancy symptoms:
abdominal distention
fever
explosive watery, diarrhea or vomiting
constipation
childhood symptoms:
ribbon like stool
constipation
palpable fecal mass
enterocolitis is a common complication of hirchsprung disease
38
hirschsprung disease diagnostic studies
rectal biopsy
contrast enema
anorectal manometry
39
hirschsprung disease management
surgery is necessary in order to fix it. you will take out the aganglionic portion in order to restore function
preoperatively:
for older children, give saline enema before surgery
give oral or IV antibiotics
monitor vitals, bowel movements, measure abdominal distentions and review fluid and electrolytes
child with malnourishment should
- eat low in fiber,
- high calories and high protein
postoperatively:
monitor their bowel movements, vitals, and abdomen
child may need anal dilations daily, so teach caregivers how to perform those
if a child has a colostomy, teach how to manage those
make sure that the skin around the colostomy is intact
40
what is pyloric stenosis
occurs when the pyloric sphincter is obstructed
happens in the first few weeks of life--> mostly seen in white infant males
41
pyloric stenosis manifestation
projectile vomiting
chronic pain
hunger
palpable olive-shaped mass in the right upper quadrant
dehydration
metabolic alkalosis
**dehydration and electrolyte status is something you really need to consider in pyloric stenosis because it will be imbalanced
42
pyloric stenosis diagnostic studesi
ultrasonography
history and physical assessment
labs:
- decreased sodium and potassium,
- increased BUN and pH
43
pyloric stenosis management
pyloromyotomy is the standard surgical procedure for this condition
preoperatively:
- make sure that they are getting their fluids and electrolytes
- should be NPO before surgery
insert a NG tube if you need to decompress the stomach
postoepratively:
- monitor intake and output, vitals, incisions and pain after procedure
- feedings should occur 4-6 hours after surgery
- feedings should starts with fluids with added electrolytes and small, frequent feedings
once they are able to tolerate that then move them to breast milk or formula
have moms give breast milk in a bottle in order to measure accurate intake
44
what is intusussception
intestinal obstruction
the proximal section of the intestine telescopes into the distal part of the section
blockage of arterial blood flow --> tissue necrosis of the intestine
45
intusussception manifestations
sudden onset of abdominal pain
vomiting
red, current, jelly-like stools
tender, distended abdomen
palpable sausage-shaped mass in the right upper quadrant
signs of peritonitis
- fever,
- board-like abdomen
46
intusussception diagnostic studied
ultrasonography
history and physical assessment
rectal exam
47
intusussception management
first treatment is giving a radiologst guided gas enema or a ultrasound guided saline enema
- but if none of these works, then surgery
preoperatively:
- IV fluid
- nasogastric decompression
- antibiotics
- monitor all stools for color and consistency
postoperatively:
- vitals
- assess wounds
- monitor bowel sounds
- intake and output should be carefully monitored
teach caregivers the signs of intestinal obstruction
also if the clients stool become brown, that means that intussusception has resolved and let the HCP know
48
what are hypospadias and epispadias
both are abnormal congenital defects of the male urethra
happens in embryonic development
hypospadias: is more linked to genetic and environmental factors
epispadias: is linked to a more serious condition like bladder exstrophy which is when the bladder is on the outside of the abdominal cavity
additional defects:
- displaced anus,
- genitalia defects
- inguinal hernia
49
hypospadias and epispadia manifestation
hypospadias:
urinary meatus is on the underside of the penis rather than the tip
epispadias:
urinary meatus opening fails to close and may present with bladder exstrophy
50
hypospadias and epispadias diagnostic studied
no actual diagnositc studies done
| it is visualized on assessment
51
hypospadias and epispadias management
surgical interventions in order to improve voiding and physical appearance of the penis
postoperatively:
- urinary catheter may be placed
- teach caregivers how to empty and care for the catheter
- avoid tubs while catheter is in place
- children may receive anticholinergics
- analgesics for the pain and bladder spasms
52
what is bronchiolitis
often linked to RSV infections
commonly occuring in the fall to winter months
with RSV infections, the cells in the resp tract swell and fill with mucus
traps air into the lungs which can hyperinflate and lead to atelectasis
53
bronchiolitis manifestation
coughing
wheezing
fever
rhinorrhea
copious secretions
tachypnea
retractions
listelessness
periods of apnea
cyanosis
54
bronchiolitis diagnostic studies
nasopharyngeal secretions testing for RSV antigen/antibody
55
bronchiolitis management
pharmacological interventions:
- give palivizumab to decrease risk for RSV
- Rivabarin (inhaled antiviral)
mild cases:
- may be managed at home
- teach to provide adequate fluids
- suction upper airway as needed
- monitor worsening resp infection
severe cases:
- monitor and maintain oxygen >90%
- give humidified oxygen
- IV
- vitals, intake and output
- ABGs
- private room and initiate contact/droplet precautions
- give antipyretics and antibiotics
56
bronchiolitis caregiver teaching
cease smoking in home and around child
handwashing to prevent infection
resume breastfeeding once acute phase has passed
suction regularly esp before feeding
57
what is pertussis
whooping cough
caused by Bordetella pertussis
transmitted through direct contact and droplet spread
58
pertussis manifestations
2 stages catarrhal and paroxysmal
catarrhal:
- sneezing
- coughing
- low grade fever
- dry hacking cough will start and progressively get worse
- lasts for 1-2 weeks
paroxysmal:
- worsening cough that occurs more at night
- short rapid coughs, followed by a high pitched whoop
- flushed or cyanotic
- bulging eyes
- protruding tongue during paroxysm
- lasts 4-6 weeks
young infants will not have characteristic whooping cough instead:
- difficulty maintaining adequate oxygen
- frequent vomiting of mucus and formula or breast milk
59
pertussis diagnostic studies
nasopharyngeal swab or aspirate
polymerase chain reaction and serology tests
sputum culture
clinical symptoms
60
pertussis management
immunization
antibiotic therapy
adequate fluid intake
monitor for complications
oxygen therapy
ventilatory support and hospitalization if severe
61
what is hydrocephalus
buildup of fluid in cavities deep within the brain
caused by imabalance of cerebrospinal fluid absorption and production in ventricles
62
hydrocephalus manifestations
infancy:
- bulging fontanels
- head enlargement
- dilated scalp veins
- frontal protrusions
- persistent downward gaze "setting sun"
- irritable
- lethargic
- feed poorly
- changes in level of consciousness
- opisthonos
- high pitched cry
- seizures
childhood:
- depend on the location
- headache
- emesis
- papilledema
- strabismus
- ataxia
- irritable
- confused
- lethargic
- incoherent
- chlari malformation
which is muscle spasticity, weakness, atrophy
63
hydrocephalus diagnostic studies
fetal ultrasonography
fetal MRI
CT
MRI
head circumference measurements
64
hydrocephalus management
measure head circumference every day
report rapid increase in size
place child on unaffected side in flat position
antibiotics
monitor complications after VP shunt
how to prevent shunt malfunctions:
- avoid contact sports and wear protective equipment
VP shunt infection:
- fever, increased WBCs
- treated with IV or intrathecal antibiotics for 7-10 days
65
what is epilepsy
diagnosed when 2 seizures occur in 24 hours or when 1 seizures occurs with high probability
66
epilepsy manifestations
focal and generalized
focal:
- motor movement (clonus) without loss of consciousness
- eye twitching
- verbalized flashes of light
- nausea
- may begin with aura
- repeitive motion like lip smacking, chewing, drooling, jerking
- dazed expression
generalized:
- do not have aura
tonic clonic seizures
- stiffening of body following jerky motions
- may be incontinent or urine and stool
- sleepy
- no memory of event
absence seizures:
- loss of awareness
- blank stare
- brief loss of consciousness for 5-10 seconds
atonic seizures:
- sudden total loss of muscle tone
- may fall to ground and los consciousness
67
epilepsy diagnostic studies
detailed med and physical assessment
neuro assessment
CBC
electrolytes
BUN
calcium
amino acid
lactate
ammonia
urinalysis
toxicology testing
lumbar puncture
CT scan
MRI
EEG
68
epilepsy management
drug therapy:
- carbamezapine
- phenytoin
- valproic acid
- phenobarbital
- clonazepam
- felbamate
- gabapentin
- lamotrigine
- topiramate
- tiaglibine
rectal administration of med may be needed to stop seizure in home setting
avoid carbs and glucose
- ketogenic diet might be helpful
- consume high fats and proteins
once seizure has finished, suction mouth to remove secretions
implement seizure precautions
69
seizure precautions
time each seizure
raise bed side rails
pad hard objects
shower preferred over bath
swim with someone
wear med alrt bracelet
put nothing in mouth
move everything away
70
seizure precautions caregiver teaching
notify HCP if:
- child stops breathing
- eivdence of physical breathing
- seizure lasts longer than 5 minutes
- child cannot awake
- seizure occurs in water
- child vomits for 30 minutes after seizure
- pupils are unequal
71
bladder exstrophy and epispadias nursing goal
preserving renal and urinary function
reconstruction of bladder
preventing infection
maintaining sexual function
72
bladder exstrophy management
needs surgery
three separate surgeries
- close abdominal wall
- repair bladder
- address genitalia abnormalities
before surgery:
- cover exposed bladder with plastic wrap or transparent, nonadherent dressing to avoid bladder trauma
- address pain and immobilization management
after surgery:
- monitor intake and otput
- manage wound
- monitor drainage tubes
- teach caregiver to manage incision wound
- provide hygience and identify signs of complications
with hypospadias or epispadias, circumcision should be DELAYED until surgical correction is performed
73
dehydration diagnostic studies
history and physical assessment
plasma sodium levels
serum bicarbonate levels
acid base levels
potassium
74
dehydration management
administer prescribed antiemetics (ondansetron)
- decrease nausea and vomiting
start oral rehydration at 50ml/kg
with diarrhea,
- intiate additional fluids at 10ml/kg
- give small amounts of fluid every 2-3 minutes
if child cannot tolerate oral fluids, then give IV
during initial phase, isotonic fluids should be given at 20 ml/kg via a bolus over 5-20 min
monitor for hypovolemic shock
75
what is wilms tumor
most common kidney tumor
occurs before age 5
linked to genetic predisposition, congenital anomalies and chromosome alterations
76
5 stages of tumor growth
1. contained to kidney without lymph node involvement
2. extends beyond the kidney but is not in lymph node involvement
3. moves to abdomen with lymph node involvement
4. spreads to other organs
5. tumors are present in both kidneys
- worst prognosis
77
wilms tumor manifestations
abdominal swelling
abdominal mass
abdominal pain
hematuria
fever
weight loss
anemia
hypertension
sxs of resp metastasis: resp distress, chest pain
78
wilms tumor diagnostic studies
abdominal x ray
ultrasound
CT scan
MRI
dopplet ultrasound
CBC
electrolytes
urinalysis
79
wilms tumor management
includes combination of radiation, surgery and chemotherapy
preoperative care:
- help prepare family for surgery
- monitor vitals
- report hypertension
- therapeutic play for child
postoperative care:
- monitor GI for obstruction
- assess bowel sounds, bowel movements, abodminal girth
- assess for signs of infections: fever, and increased WBCs
- support family
teach caregivers about side effects of chemotherapy and radiation
for child with wilms tumor,
- place a sign above bed to AVOID palpating the abdomen
80
growth hormone defiency
when the childs height is less than a -2 standard deviation
GH secreted from anterior pituitary gland
81
growth hormone defiency manifestations
short statue with proportional height and weight
delayed epiphyseal closure
retarded bone age, proportional to height
premature aging
increased insulin sensitivity
82
growth hormone deficiency diagnostic studying
fam history and review of growth
MRI
endocrine studies:
- GH deficiency levels,
- GH stimulation tests
- genetic testing
83
GH deficiency management
GH may be given prior to puberty if idiopathic
nurse should:
- teach about frequency of diagnostic screening tools
- frequency of blood testing during GH stimulation tests
- given every 30 min for 3 hours
provide emotional supports
encourage children to set realistic gaols and remain active
GH is most effective when administered at bedtime
84
what is precocious puberty
early sexual development in children
boys: before 9
girls: before 8
85
precocious puberty diagnostic study
no specific diagnostic test
identified by hitory and physical assessment
MRI
86
precocious puberty management
given leuprolide acetate IM every 4 weeks
- slow down sexual progression
GnRH analog histrelin subdermal implants:
- ALTERNATIVE TREATMENT
nurse should:
- dress child in clothing which matches their age
- children may be fertile, so caution should be taken if sexual activity is done
meds to slow down sexual progression is discontinued once child reaches appropriate age
87
scoliosis manifestations
clothing doesn't fit well
| asymmetry of shoulders, scapula, hips or pelvis
88
scoliosis diagnostic studies
radiography
girls:
screened twice at 10 and 12 years of age
boys: screened once between 13 and 14 years
scoliometer measures truncal rotation
MRI
89
scoliosis management
surgery for curvatures greater than 45 degrees
curvatures between 25 and 45, bracing should be used
daytime braces:
- TLSO (thoracolumbosacral)
- Boston
- Wilmington
nighttime braces:
- charleston brace
interdisciplinary team:
- nurses
- physical and occupational therapy
- orthopedists
- social workers
encourage to exercise to increase muscle strength and ROM
positive reinforcement and encouragement
90
scoliosis post op management
patient controlled analgesia
logrolling technique
assess vitals, neuro and circulatory system
fluid and electrolytes
urine output
GI system, pain
report epigastric pain, nausea and large amounts of vomiting
encourage early ambulation to decrease risk of blood clots
facilitate family involvement
91
what is hip dysplasia
abnormal hip development in utero and after birth or during childhood
92
factors that increase hip dysplasia
females
first pregnancy
breech positioning in utero
family history
high birth weight
joint laxity
93
hip dysplasia manifestations
asymmetry of gluteal and thigh folds
limited abduction of hip
shorter appearance of femur at knee level in flexion
waddling gait
positive trendelenburg sign
trendelenburg sign:
- child stands on one foot and then the other
- on the affected foot, the hip tilts downward
94
hip dysplasia diagnostic studies
physical assessment
barlow test:
- manually guiding the hips into mild ADDUCTION
and apply slight forward pressure with thumbs
- if hip is unstable, femoral head will slip
- a distinct "clunk" is felt as femoral head pops out of joint
ortolani test:
- hip is ABDUCTED and pressed slightly forward with the thigh from behind
- " a distinct clunk will be noticed as head slides back into place
radiographs for children older than 6 months
95
hip dysplasia management
newborn to 6 months:
- adjusting straps if removing harness
- diapers go beneath straps
- massage under straps
- bond
6 - 24 months:
- change diapers frequently
- safe strollers and car seat
older children:
- monitor exercise
- allow child to participate in activities
96
what is clubfoot
bone deformite of the foot and ankle
genetic links are common
97
clubfoot manifestations
abnormal positioning of heels and toes, either unilaterally and bilaterally
98
clubfoot diagnostic studies
physical exam after birth
| radiography
99
clubfoot management
treatment should begin during newborn stage
casting should continue until maximum amount of correction has occurred
nighttime abduction brace may be applied
- used for 3-5 years to correct foot alignement after surgery
assess feet for casting complications and vascular circulation
educate about treatment
for best outcomes, serial casting for clubfoot, should begin one month after birth
100
what is hemophilia
bleeding disorder due to the lack of blood clotting factors
hemophilia A: lacks factors VIII
hemophilia B: lacks factors IX deficiency
mild hemophilia: bleeding occurs after sever trauma or surgery
- factor levels between 5-40%
moderate hemophilia: bleeding occurs with trauma
- factors level between 1-5%
severe hemophilia: spontaneous bleeding without trauma
- factors level below 1%
101
hemophilia manifesations
increased bleeding time
hemorrhage
hemarthrosis - joint bleeding
- stiffness
- tingling
- decreased range of motion
swelling
redness
warmth
pain at joints
hematuria
epistaxis
hematomas
blood in stool
102
hemophilia diagnostic studies
genetic testing
CBC
bleeding times
platelets
clotting factors
analysis of factors VIII and IX
103
hemophilia diagnostic studies
factor replacement
vasopressin
corticosteroids
children should AVOID NSAIDs and aspirin
gene therapy
genetic counseling
passive ROM
- should be avoided in those with hemarthrosis
non or minimal contact sports
- wera protective equipments: helmets, knee pads etc
soft toothbrush and water rinses
electric shavers instead of blades
104
what is sickle cell disease
autosomall recessive trait
RBCs clump together, decreasing circulation and leading to obstruction
105
sickle cell disease manifestations
growth delays
chronic anemia
pain
painful joints
pain in hands and feet
painful erections
abdominal pain
hepatomegaly
splenomegaly
circulatory collapse
hematuria
jaundice
long term complications:
- cardiomegaly
- pulmonary insufficiency
- renal failure
- increased risk of infection
- bone necrosis
- retinal detachment --> blindness
- skeletal deformities
- stroke, seizures
- paralysis, death
106
sickl cell disease diagnostic studies
stained blood smear
sickle turbidity test
hemoglobin electrophoresis
genetic screening
107
sickle cell disease management
rest
prevent crises
improve oxygenation
antibiotic therapy
blood transfusions
pain management
maintain hemoglobin between 9-10
splenectomy for those with splenic sequestration
108
sickle cell crisis management
asses:
vitals
neuro status
vision
resp
GI
renal
insulin
monitor signs of infection and splenic sequestration
report fever or 101.3 or higher
report increased spleen size and pallor
monitor intake and output
- take weight at administration
- observe for hyperkalemia
infection protocol
monitor reactions
assess pain thoroughly and frequently
give meds,
heat therapy,
passive ROM,
bed rest
MEperidine:
- opioid analgesics should be AVOIDED in sickle cell crises to avoid neuro symptoms
watch for acute chest syndrome and stroke
109
sickle cell crisis caregiver teaching
child should receive:
- pneumococcal
- H influenzae type B
- meningitis vaccines
teach how to palpate the spleen and seek medical care with enlargement
avoid high altitudes, infection sources, and contact sports
avoid overheating
meet daily fluid requirements
genetic counseling before future pregnancies
110
phenylketonuria management
monitor levels of phenylalanine
- normal 2-10mg
- 2-6 for less than age 12
put synthetic protein and special formula diet
eliminate high phenylalanine foods
- meat
- eggs
- milk
encourage foods low in phenylalanine
- fruits
- veggies
