Peds exam 4... PKU

take with food dissolve in apple juice or water and drink within 15 minutes may take many tablets.

Sapropterin special teaching??

phenylalanine levels above 10,, usually occurs about 7-10 days of age

when should therapy start with PKU....

can be measured in the blood

where can phenoalanine be found?

2

which teaching would be important to discuss with the family of a newborn with PKU? 1. studies have shown that children with PKU outgrow the disease 2. consumption of decreased amounts of protein and dairy products is advised 3. high protein and high dairy products consumption must be maintained 4. exclusive breastfeeding is encouraged for maximal nutrition for the child

250mg/day

A baby is brought into the clinic for follow up review of PKU. The baby is 9 months old and weighs 22 pounds. If the reccommendation is to limit the phenylalanine in the diet to 25mg/kg/day this infant should have no more than ____ of phenylalanine/day.

digestive problems and vomiting( high levels of PKU) failure to thrive ( very thin) seizures( secondary to high levels of PKU) musty odor to urine hyperactivity cognitive impairment

clinical manifestations of PKU:

all natural food protein contains phenylalanine and will need to be limited. treatment will continue for life.

dietary teaching

meet nutritional needs for optimal growth maintain phenylalanine levels with in a safe range...

goals of therapy for PKU

dont layer the blood...DONT DOUBLE BLOT be sure they have eaten before tsking it best done if 24-48 hours after birth

guthrie test teaching

brain damage

if a mama has high levels of PKU while pregnant the baby in utero can be at high risk for___

3 mnths and 2 times a week

low levels of phenylalanine should be reached ___ months before conception. and testing should be done ___ times per week

true

solid foods such as cereal, fruits, vegtables, are introduced as usual except for meat..

pigment of the skin, forms epineprhine, and thyroxine in the body..

tyrosine does wha?

1.6 4mg/dl

what is a normal value of guthrie test is ____mg/dl this test will pick up values higher than______

25% having the disease 25% normal 50% carrier

what is the chance of a mother with PKU having a baby who has the same disorder?

blood test based on the fact that bacillus subtilis( present in the culture medium) grows if blood contains an excessive amount of phenylalanine in the blood.

what is the guthrie test?

1. dietary info

An 8 month old infant born outside the united states is brought to the endocrine clinic with a musty body odor, seizures, and an eczema like rash. The infnt is diagnosed with PKU, what shoudl the nurse consider when planning care for this infant. 1. need for dietary info about a low phenylalanine diet 2. need for admission to a long-term care setting for handicapped infants 3. preparing the family for the childs early demise 4. providing instructions on medication management for PKU

2-6

ages neonates-12 phenylalanine levels should be between

congential heart disease, microcephaly, delayed physical growth, and cognitive impairment.

being pregnant with high levels of phenylalanine can result in babies with ___,____,____,______.

only if the parent avoids phenylalanine in their diet, mom has to follow PKU diet in order to.

can parents breastfeed children with PKU?

eczema hypopigmentation of hair, skin bizarre behavior-( cranky,moody)

clinical manifestations of older children:

lumpy and distinct odor or taste use a belnder lofenalac or phenex 1

formula

TRUE

meat and dairy should be eliminated with PKU

all children are tested before being released from the hospital

what children are tested for PKU?

PKU

with this disorder there is a deficeincy of phenylalanine hydroxylase, the hepatic enzyme needed to convert phenylalanine to tyrosine

true

High levels of phenoalalnine levels in the blood can led to cognitive impairment and abnormal development of the CNS...t/f

2-10

12- adolsecnce phenlalanine levels between

artificial sweetners has protein in itFYI

altered nutrition , less than body requirements

aspartame( amino acid)

artificail sweetner should be avoided why?

melanin(pigment), dopamine , and tryptophan usually blonde hair, blue eyes, and fair skin then decreased catecholamines, serotonin, thyroxine ( dopamine and trypophan)

as the disease progresses their is decreased____

PKU( phenylketonuria)

autosomal reccesive disorder that results that results in CNS damage from toxic levels of phenoalanine in the blood.

50% carrier 25% disease 25% normal

autosomal recissive disorder transmission pattern

10-15mg/dl

brain damage usually occurs when phenylalanine levels are higher than

Guthrie test

Diagnostic test for PKU??

4- diet soda should be avoided

Which teaching is most important for the child with PKU? 1. the child is able to eat 1/4 lb burger and drank a milkshake daily 2. if the child wants soda, diet soda is preferred over milk or dairy products 3. the child may have ice cream in an unlimited quantity oce a week 4. diet soda or anything with the sweetner aspartame should be avoided

3. low phanylalanine diet

Which treatment would the nurse anticipate for a 2 week old boy diagnosed with PKU? 1. ther e is no treatment or special diet 2. a high phenylalanine diet 3. a low phenylalanine diet 4. the mother would be advided not to breastfeed to infant

Kuvan ( sapropterin)

a synthetic form of natrural salt that may be used along with a restrcited diet to control phenylalanine levels in some people. It is used to help the body activate the enzyme needed to control blood levels... take with food, dissolve the tablet in 4-8 oz of water or apple juice and drink within 15 minutes