TOPIC :

DEFINITION

ntial amino acid, into tyrosine ( aprecursor of

nto thyroxine . Without it , body pigment fades and the child becomes fair skinn

alanine ) spills into the urine to give the disorder its name . It causes urine

average growth standards because of the lack of throxine production .

panying seizure disorder . The skin is prone to eczema ( Atopic dermatitis )

 PATHOPHYSIOLOGY
RISK FACTOR: autosomal recessive genetic disorder

Failure to produce phenylalanine hydroxylase

(PAH)

Decrease neurotransmitter
(Dopamine)

Increase blood phenylalanine

level

Inhibtion of
Passes in blood brain tyrosinase Neuropsychiatric symptoms:
barrier
üAnxiety
üDepression
Increase phenylalanine into the brain Decrease melanin üPhobias
synthesis üAttentional disorders
üDefects in executive
functioning
üDecline in IQ
üAbnormal hypopigmentatio
n
myelination
üSmall brain size
üSeizures
üDiminished IQ
 DIAGNOSTIC EVALUATION
Phenylketonuria Test
( PKU Test , Guthrie Test , Phenylalanine )

TEST DESCRIPTION

borns for PKU is required in all states. Testing is done either on the serum (Guth
PROCEDURE AND NURSING RESPONSIBILITiES

Pretest
• Explain the purpose of the test to the mother and the need for a blood sample to be obtained.
• No fasting is required prior to the test.

Procedure
For Guthrie ( serum ) test
A.
•Warm sites with soft cloth, moistened with warm water up to 41°C, for three to five minutes.
Rationale: To easily obtain blood sample.

•. Cleanse site with alcohol prep. Wipe DRY with sterile gauze pad.
Rationale: To have a sterile area for puncture.

• Blood is collected by pricking the baby's heel.

Rationale: Puncture in heel, it is where blood samples are taken from infants.
Wipe out first drop of blood since it is not clean.

B. This is then used to cover four circles on an absorbent card, which is then
forwarded to the public health laboratory.

Posttest
• Apply pressure on the newborn’s heel for 5 to 10 minutes and then leave the site open to
the air for healing.
 SIGNIFICANT FINDINGS

Normal Values
Blood: Negative

Contributing Factors to Abnormal Values :

Possible Meanings of Abnormal Values
• Testing for PKU too early may lead to false-negative res
Increased be collected from infants older than 24 hours and younger
Delayed enzyme system
• Drugs development
which may alter test results: antibiotics, aspirin,
Galactosemia
Hepatic disease
Hyperphenylalaninemia
Low birth weight
Phenylketonuria
 THERAPEUTIC MANAGEMENT

MEDICAL MANAGEMENT

milk may be added to the infant ’ s diet so the child does recei
enefits can be obtained), while adjusting the diet appropriate
van is First FDA-Approved Drug for PKU
junction with a p henylalani ne - restricted diet , the drug can help reduce blood phenylalan

ropeptide
ey ( GMPover
) that is left ) for PKUcheese
after DietisTreatment
made . GMP protein is naturally low in phe
 NURSING CARE PLANS

Defining EXPECTED OUTCOME

CRITERIA INTERVENTION AND RATIONALE BEHAVIORAL OUTCOME
Characteristics
Diagnosis: Deficient Knowledge, diet Short Term : Independent: Bi bli ogr aph y :
•Ackley, A.( 2004), Nursing Diagnosis Handbook
restrictions related to unfamiliarity with After 8 hours of I>Observe the client's ability and readiness to learn 7th ed.
information resources nursing care, R>Astute assessment is needed to determine the person's learning ability
Subjective cues: I don’t really know why S>Forrest, 2004
•
Bohny B(1997) A time for self-care: role of the
I have this kind of disorder; I’m not client will be I>Assess barriers to learning like perceived change in lifestyle, financial home healthcare nurse, Home Healthc Nurse
familiar with this. able to verbalize concerns, cultural patterns, lack of acceptance by peers or co-workers 15(4):281,
Objective cues: understanding on R> The client brings to the learning situation a unique personality, •Edwards C (2002): A proposal that patients be

•Received patient on bed, asleep

the disease established social interaction patterns, cultural norms and values, and considered honorary members of the healthcare
team, J Clin Nurs 11(3):340
•With ongoing #2 PNSS 1 L @30 gtts/min
process, environmental influences
•Forrest S (2004): Learning and teaching: the
•States that she doesn’t understand the S>Bohny, 1997
recognize need reciprocal link, J Contin Educ Nurs 35(2):74-9,
disease process I>Involve client in writing specific outcomes for the teaching session, such
•Always asking about her diet as
for medication as identifying what is most important to learn from their viewpoint and
•Johnson Young (2004),Medical-Surgical

observed and understand lifestyle. Nursing 10th ed. Philadelphia: LWW

•High school level as reported her specific diet R > This study indicated that clients were willing to take responsibility for
•inaccurate follow-through of instruction and treatment. playing their part in trying to optimize the outcome of their surgery
noted Long Term : S>Edwards, 2002
•inaccurate performance of test noted I>Use easy to understand language when giving information to clients.
•Health behaviour and knowledge is
After 4 days of Encourage clients to ask the following questions: What is my main
limited nursing care, client problem? What do I need to do? Why is it important for me to do this?”
L a bor ato ry Res ult s : will be able to state Have clients' repeat back information.
There is no related laboratory test
T h eor eti cal Ba sis : an ability to deal Renhance
> the client understand easily if she knows the language and can
her learning well
Deficient Knowledge is the absence or with health S> Edwards, 2002
deficiency of cognitive information related situation and I>give additional information about the disease process and the diet she
to a specific topic
PKU problem is a rare disorder that
remain in control of must follow in order to maintain a good health
R>enhances patients’ knowledge about the disease process
affects a few people only. This disease life and S>Ackley,2004
needs an appropriate diet that should be demonstrate how I>Carefully evaluate information that is given to client regarding "disease
strictly followed in order to attain a to perform health state” focus on wellness
balance health. Adequate knowledge is R> to check if the patient understand the information clearly
necessary to be able to achieve an related procedures S>Ackley,2004
optimum level of understanding to satisfactorily. Co l la b or a ti v e :
accomplish what is needed I > Administer medication as prescribed by the physician
R > helps lower the levels of phenylalanine in the body
S > Ackley,2004
 NURSING CARE PLANS
Defining Characteristics EXPECTED OUTCOME INTERVENTION AND RATIONALE BEHAVIORAL OUTCOME

Nsg . Dx: Delayed Growth and Short term : I>determine existing condition(s) contributing to I>provide positive feedback for
Development r/t cognitive After 8 hours of growth/developmental deviation, such as limited intellectual efforts/ successes and adaptation
impairment secondary to PKU nursing capacity, physical disabilities, etc. while minimizing failures
S> no verbal cues intervention, R>to assess causative factors R>encourages continuation of efforts,
O > delay/difficulty in client’s SO will SI> Doenges, p. 365
>determine the use of drugs
thus improving outcome
S> Doenges, p. 365
performing skills typical to verbalize R>these could affect body growth I>recommend involve in regular
age group understanding of S> Doenges, p. 365 exercise/sports medicine program
> altered physical growth the condition of I>identify present growth age/stage R> to enhance muscle tone/strength and
> decreased responses child. R>provides baseline for identification of needs and appropriate body building
> listlessness Long term : After effectiveness of therapies S> Doenges, p. 365
> flat effect a week of nursing S> Doenges, p. 365 Dependent / Collaborative :
Laboratory Findings : intervention, I>review expectations for current height/weight percentile D> evaluate home/day
No significant lab findings client will R>compares measurements to “standard” ornormal range for care/hospital/institutional
Theoretical Basis : perform motor, children of same age and gender to determine degree of environment
deviation R>to determine adequacy of care
PKU (Phenylketonuria) is a social, and/or S> Doenges, p. 365 provided, including nourishment,
disorder defined as the expressive skills I>record height/weight over time healthy sleep/rest time, stimulation,
inability to metabolize the typical of age R>to determine trends diversional or play activities
essential amino acid group within S> Doenges, p. 365 S> Doenges, p. 365
phenylalanine. This can cause scope of present I>monitor growth and development factors periodically D>note findings of psychological
mental retardation, if capabilities R>aids in evaluating effectiveness of interventions over evaluation of client and family
untreated, although sufficient time and promotes early identification of need for R>to determine factors that may
treatment can occur additional actions impact development of client or
immediately after birth. The S> Doenges, p. 365 impair psychological health of family
main symptom consists of mild I > provide anticipatory guidance for parents /caregivers S> Doenges, p. 365
regarding expectations for client’s development D>consult appropriate professional
to moderate mental R>to clarify misconceptions and assist them in dealing with resources
retardation, but this is reality of situation R>to address specific individual
easily prevented through S> Doenges, p. 365 needs
treatment. Delayed growth and I>encourage recognition that deviation/behavior is S> Doenges, p. 365
development is also evident appropriate for a specific age level D>refer family/client for
in 3 years of age.However, R>promotes acceptance of client, as presented, and helps counseling/psychotherapy
other side effects include shape expectations reflecting actual situation R>to deal with issues of abuse/neglect
seizures, vomiting, a “mousy S> Doenges, p. 365 S> Doenges, p. 365
odor”, and behavioral self- I>involve client in opportunities to practice new behaviors
R>strengthens learning process
mutilation. In some cases, S> Doenges, p. 365
treatment can reduce or
reverse the mental
retartadtion.
 NURSING CARE PLANS
DEFINING EXPECTED OUTCOME
CRITERIA
CHARACTERISTICS
N s g . Dx: Deficient fluid volume r/t Short term :
excessive fluid loss from GI tract
 
S> no verbal cues
O> decreased urine output
> decreased urine concentration
> decreased venous filling
> sudden weight loss
> dry skin / mucous membranes
> change in mental state
 
L a bor ato ry Fin din gs :
 
 
Urine specific gravity: 1.035
Serum sodium 155 mEq/L
Serum potassium 3.2 mEq/L
Chest x-ray negative
 
 
 
T h eor eti cal Ba sis :
 
Nausea and vomiting are not diseases, client will
but rather are symptoms of many
different conditions, such as
infection ("stomach flu"), food
poisoning, motion sickness,
overeating, blocked intestine,
illness, concussion or brain injury,
appendicitis, and migraines. Nausea
and vomiting can sometimes be
symptoms of more serious diseases
such as heart attacks, kidney or
liver disorders, central nervous
system disorders, brain tumors, and
some forms of cancer
INTERVENTION AND RATIONALE BEHAVIORAL OUTCOME
After 8 hours of
I> Auscultate bowel sounds, noting absence or hyperactive
sounds. Dependent / Collab
nursing
R> Inflammation or irritation of the intestine may be
accompanied by intestinal hyperactivity, diminished water orative :
intervention,
absorption and diarrhea.
client’s SO will S> Doenges, p. 324  
verbalize I> Eliminate smells from the environment. D> Monitor BUN, protein, prealbumin or
R> Reduces gastric stimulation and vomiting response. albumin, glucose, nitrogen balance as
understanding of S> Doenges, p. 324 indicated.
causative I> Avoid foods that might cause or exacerbate abdominal R> Reflects organ function and
factors and cramping like caffeinated beverages, chocolate, orange nutritional status and needs.
purpose of juice. S> Doenges, p. 324
individual R> Might increase abdominal cramping. D> Advance diet as tolerated.
S> Doenges, p. 324 R> Careful progression of diet when
therapeutic I> Measure abdominal girth. intake is resumed reduces risk of
interventions R> Provides quantitative evidence of changes in gastric or gastric irritation.
and medications intestinal distention. S> Doenges, p. 324
Long term : S> Doenges, p. 324 D> Refer to physical therapy.
I> Observe skin or mucous membrane dryness, and turgor. Note S> Doenges, p. 324
After a week of peripheral edema and sacral edema.
nursing R> Hypovolemia, fluid shifts and nutritional deficits
intervention, contribute to poor skin urgor, edematous tissue.
S> Doenges, p. 324
maintain fluid Iappearance
> Assess abdomen frequently for return to softness,
of normal bowel sounds, and passage of flatus.
volume at a R> Indicates return of normal bowel function and ability to
functional level resume oral intake.
S> Doenges, p. 324
I> Weigh daily.
R> Initial losses or gains reflect changes in hydration.
S> Doenges, p. 324
ng Diagnosis Handbook 7th ed.
for self-care: role of the home healthcare nurse, Home Healthc Nurse 15(4):281,
proposal that patients be considered honorary members of the healthcare team, J Clin Nurs
ning and teaching: the reciprocal link, J Contin Educ Nurs 35(2):74-9,
),Medical-Surgical Nursing 10th ed. Philadelphia: LWW
 CONTRIBUTION OF THE MEMBERS

April Ann Alderite – Nursing Care Plan

Sedfrey Jay Arnado – Laboratory Results and Implications

Niña Ciara Baring – Laboratory Procedures and Rationale

Princess Dawn Boquia – Nursing Care Plan

Stephen Burgos – Laboratory Procedures and Rationale

Ellen Rose Funtanar – Nursing Care Plan

Loren Mharnne Gelbolingo – Laboratory Procedures and Rationale

Alma Moiselle Lastimosa – Concise Definition and Description

Mary Ann Modesto – Nursing Care Plan

Evan Jemmima Montes – Schematic Diagram on Pathophysiology

Francis Robles, Jr. – Medical Management

Mervic Marie Ypil – Laboratory Procedures and Rationale