NUR331 Exam 3 Flashcards
phenylketonuria (PKU)
- inborn genetic error of metabolism where there is an absence of the enzyme phenylalanine hydroxylase (causes inability to metabolize the amino acid, phenylalanine)
- can build up and cause brain damage
- will ALWAYS be on a PA+ free formula
- limited fruits, vegetables, and grains, no meat
- must keep PA+ in a safe range of 2-6
CM of untreated PKU
- restricted height and weight
- musty odor to urine and sweat
- hypopigmentation
- vomiting
- irritability
- seizures
- hyperactivity
- mental delay
- adults have: short attention span, poor short term memory, visual perception problems, poor motor coordination, mood disorders, brain structural changes, etc
NC for PKU
- ensure proper testing as a newborn
- teaching
- referral for genetic counseling
- supportive
- flexible feeding schedule
- encourage early treatment
galactosemia
- inborn error of carb metabolism
- lack of enzyme needed to convert galactose to glucose
- CM - vomiting, weight loss, jaundice, lethargy, hypotonia, cataracts
- long term consequences - learning disabilities, lower IQ, short attention span, behavior problems
- lactose-free diet, soy formula
- calcium supplements, sensitive to medications
- don’t allow mom to breastfeed unless she is dairy free
juvenile hypothyroidism
- s/s - hypothermia, large protruding tongue, short and thick neck, delayed detention, hypotonia, poor feeding, prolonged jaundice, constipation, goiter
- englarged thyroid could obstruct airway
- levothyroxine for life, prompt treatment needed for brain growth in infants, dose changes with growth
- teach med administration - avoid heat exposure, do NOT mix with soy formula
hyperthyroidism
- mimics s/s of adults
- diagnosed based on high T3 and T4
- given anti thyroid drugs - most likely will receive remission in 1-2 years
- subtotal thyroidectomy or ablation with radioiodine
- high cal and nutritious diet, limit activity
agranulocytosis
toxic reaction of anti-thyroid drugs that causes sore throat and fever
- should isolate and call HCP
thyrotoxicosis
- thyroid storm
- can be life threatening
- can be precipitated by infection, surgery, or discontinuation of antithyroid therapy
- treated with antithyroid drugs and propanolol
hypopituitarism
- causes - tumors, encephalitis, radiation, head trauma, congenital, and idiopathic
- absence or regression of secondary sex characteristics
- normal height and weight at birth but patterns deviate
- diagnosed by ruling out short stature and Turner’s
- CM - short, normal to heavy weight, slow bone age, appear younger than chronological age, delayed growth of permanent teeth
- given growth hormone until reaching adult height, may need thyroid extract, cortisone, testosterone/estrogen (expensive and painful)
- injections are given at bedtime, requires frequent blood draws
precocious puberty
- sexual development before age 9 in boys or 8 in girls
- cause - disorder of gonads, adrenal glands, or HPA axis
- included are premature thelarche, premature pubarche, and premature menarche
- treat specific cause if known
- Lupron - monthly IM until puberty that slows growth
congenital adrenal hyperplasia
- decreased enzyme activity that is required for cortisol production in the adrenal cortex
- overproduction of adrenal androgens results in virilization of female fetus
- decreased cortisol and aldosterone
- CM - ambiguous genitalia, decreased stress response, hypoglycemia, increased inflammatory response, hypotension, compromised immune system, dehydration
- ultrasound to determine organs, chromosome typing for positive sex determination
- given cortisone to suppress abnormally high secretion of ACTH
- teach parents of s/s of salt-losing crisis and dehydration
diagnostic criteria of diabetes
- FPG >126
- PG >200
- casual >200 with 3 p’s
CM of DM1
- hyperglycemia and acidosis
- weight loss
- 3 p’s
- enuresis
- irritability
- unusual fatigue
- abdominal pain
CM DM2
- overweight
- fatigue
- frequent infections
- acanthosis nigrocans
diabetic medical management
- nutrition
- medication
- developmental issues
- glucose and urine monitoring
- hypoglycemia management
- hyperglycemia management
- sick day management
snack management with DM
- balance of carbs, fats, and proteins
- extra food during exercise
- consistent meal times
- avoid high sugar
- decrease insulin with exercise
- eat snacks when drinking alcohol
carb-free sources
meat, jello, cheese
onset, peak, duration of insulin lispro
- 15min
- 1 hr
- 3-4 hr
onset, peak, duration of insulin glargine
- 4-6 hours
- 8-20 hrs
- 24 hours
When should urine ketones be monitored?
- any time glucose is 240+
- during illness
- when on an insulin pump and sugar is 240+
What is bolus insulin matched with?
carb intake
correction is everything above the desired level
developmental issues with 3-6years DM
- reassure child who thinks it’s punishment
- encourage child to participate
- teach child to report lows
- teach child what to eat when they get low
developmental issues with 6-12 years DM
- educate school personel about DM
- encourage independence, but all activities must be supervised
- encourage extra curricular activities
adolescent DM developmental issues
- know which foods fit into meal plan and how to adjust
- they like to take risks
- need continued parental support
s/s and management of hypoglycemia
- s/s - low BS, hunger, headache, confusion, shakiness, dizziness, sweating
- treated when below 60
- treat with rule of 15
- follow with meal or snack
- use glucagon pen if severe (pen has expiration date)
s/s and management of hyperglycemia
- s/s - high BS, glucose in urine, frequent urination
- check fluids, increase fluids (no caffeine), do not increase activity
- treat DKA if applicable
- insulin
ketosis patho
cells don’t get glucose needed for energy so your body burns fat for energy
ketones are created from broken down fats
make blood more acidic
DKA s/s
kussmaul’s respirations
confusion, lethargy
abdominal pain
vomiting without diarrhea
dehydration symptoms
respiratory distress without lung pathology
What does a high anion gap indicate?
metabolic acidosis
usually happens in DM because bicarb is low
causes of mortality from DKA
failure to make diagnosis
cerebral edema*
hypo/hyperkalemia
hypoglycemia
hypovolemia
what do electrolytes look like in DKA?
high extracellular K
low Na (dilutional)
both are lost in urine
treatment of DKA
fluid replacement*
electrolyte replacement
insulin therapy
careful monitoring
goals of DKA treatment
correct dehydration
correct acidosis and reverse ketosis
restore normal blood glucose levels
avoid therapy complications
how are insulin drips adminsistered?
continuous IV infusion of regular insulin
DO NOT give as a bolus
should be at a rate of 0.1u/kg/hr until acidosis resolves
once glucose reaches 250-300, maintain insulin and begin dextrose infusion
check glucose hourly
when is bicarb administration done?
severe acidemia
life threatening hyperkalemia
hourly assessment of DKA patient includes…
- VS
- neurological status
- accurate fluid intake/output
- POC glucose level
- K level
what should be monitored every 2 hours in DKA?
- urine ketones
- serum bicarb
- labs q2-4
- continuous cardiac monitoring
when to transition off of IV insulin
when pH is above 7.3 and bicarb is >15-18
patient is able to eat
able to give SQ insulin
developmental musculoskeletal considerations
- skeletal maturity happens by 17 in boys and after 2 years of menarche in girls
- they have growth plates
- porous bone
- thicker periosteum (allows for faster healing in infants)
managing care of pt with a cast
- handle wet cast with palms of hands
- turn wet cast every 2 hours until dry
- avoid ibuprofen, but treat pain
- ice can help with itching
- elevate extremity
- watch skin for breakdown
- don’t put anything in the cast, can use benadryl for itching
- exercise non-affected side and do isometric exercises on affected
- prevent foot drop
- good diet and lots of fluid