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Hyperbilirubinemia

The life span of the red blood cell (RBC) is shorter in newborns than in older
children and adults {70 to 90 days versus 120 days}. This increased RBC
destruction and the fact that the newborn's liver is immature can lead to
physiologic jaundice in the newborn (Cohen, 2006). Hyperbtlirublnemla, an
abnormally elevated serum bilirubin level, requires timely assessment and
appropriate interwntion to prewnt central nervous system injury (Smitherman,
stark , & Bhutani, 2006).

Etiology and Pathophysiology

Physiologic jaundice is described as jaundice in the newborn without any


other signs of illness (Cohen, 2006). Jaundice occurs in 60% of newborns who are
otherwise healthy during the first week of life (Brethauer & Carey, 2010;
Smitherman et aI., 2006) and SO% of preterm infants {Cohen, ZOO6}. Jaundice
is generally noticed when the bilirubin reaches 5-6 mgldL (Brethauer & Carey,
2010). Symptoms generally resolve by 7- 10 days of age without complication
(Smitherman et aI., 2006).

Clinical Manifestations

Jaundice in the infant is first evident on the face, and then progresses to the
trunk and fmally to the extremities. Jaundice may be difficult to see in babies with
dark skin color. In addition to jaundice, symptoms include lethargy or irritability,
and poor feeding {Cohen, 2006}.
COllABORATIVE CARE

Diagnostic Tests

A blood test, performed by heelstick or venipuncture, measures total serum


bilirubin (TSB) in the newborn. A transcutaneous bilirubin (TcB) measurement
device is a noninvasive method for estimating serum bilirubin in infants. This
method for measuring bilirubin is generally within 2- 3 mgldL of the TSB and can
be performed instead of the TSB in many cases. especially for those infants in
which the TSB is less than 15 mgldL (Amerkan Academy of Pediatrics [AAP],
2004).

Clinical Therapy Phototherapy most effecliwly reduces serum bilirubin in


newborns with physiologic jaundice. The point at which phototherapy is
implemented depends on whether the infant is full or preterm and the age of the
infant in hours at the time the bilirubin rises. The goal of phototherapy is to keep
the TSB below the exchange transfusion level. The American Academy of
Pediatrics (2004) has provided specific guidelines that clinicians can follow in
determining the appropriate treatment.

Phototherapy is thought to reduce the amount of indirect, or unconjugated,


bilirubin in the baby's bloodstream by promoting excretion via the intestines and
kidneys. Phototherapy exposes the infant's skin to blue light, which changes
bilirubin into water-soluble forms that can be excreted. The infant may be placed
on fiber-optic pads as the sole means of providing phototherapy when mild
jaundice exists or in conjunction with overhead phototherapy when bilirubin lewis
are higher (Cohen,2006).

Many newborns with hyperbilirubinemia are also mildly dehydrated. When


the newborn is breastfed, supplemental breast milk or formula may be given to
improw hydration. Supplementation with water or dextrose walter is not
recommended. If the infant is unable to take adequate fluids and is dehydrated,
intravenous fluid should be administered (Moerschel, Ciandaruso, & Tracy,
ZOOS).

NURSING MANAGEMENT

The nurse in the newborn nursery and in the outpatient setting plays a critical
role in identifying the newborn at risk and providing parent education related to
hyperbilirubinemia. Although the nurse in the newborn nursery may be providing
care to an infant for a few days after birth, many infants are discharged home
within 24 hours. The infant maybe evaluated for jaundice in the outpatient setting
and then admitted to the pediatric unit for treatment. Nurses working in acute care
settings must familiarize themselves with the care of very young infants who
require treatment for hyperbilirubinemia.

Nursing Assessment and Diagnosis

The newborn should be assessed for jaundice at least every 8-12 hours
{Moerschel et al., 200S}. If the nurse suspects the preSt'nce of jaundice, the
infant's primary care provider should be notified and TcB {transcutaneous
bilirubin} measurement or TSB (total serum bilirubin) lewl should be obtained
{Cohen, 2006}.

Feeding Assessment

The mother who is breast feeding should nurse her infant at least 8-12 times
per day for the first sewral days (Moerschel et al., 2006). The nurse should be alert
to mothers and infants who are having difficulty and require lactation support
during the hospital stay and following discharge.

The infant who is breastfed should have 4-6 very wet diapers and 3-4 stools
per day by the fourth day of life. Meconium stool should have transitioned to
mushy, mustard-colored yellow stools by day 3 to 4. If these parameters are not
met, the infant maybeat risk for dehydration due to inadequate intake, thus
increasing the risk of hyperbilirubinemia (AAP, 2004). Because many mothers
and term newborns are discharged within 24 hours after birth, this is important
information to teach parents prior to discharge.

Nursing assesment

Nursing diagnoses that may apply to the newborn with hyperbilirubinemia


may include:

• Deficient Fluid Volume related to decreased oral intake and ineffective


breast-feeding

• Risk for Impaired Parent/Newborn Attachment related to disruption of


parental/newborn interaction due to hospitalization and treatment

• Risk for Imbalanced Body Temperature related to phototherapy

• Risk for Injury related to phototherapy

• Risk for Neurological Impairment related to hyperbilirubinemia.

Planning and Implementation

The role of the nurse is to identify the newborn at risk for hyperbilirubinemia,
educate parents about newborn jaundice, and cue for the newborn and family
undergoing treatment for this condition. For the infant undergoing phototherapy,
the nurse should monitor the infant frequently, ensuring that the infant is receiving
the phototherapy properly. Vital signs should be assessed every 4-8 hours,
especially the infant's temperature, which might indicate signs of infection or
signs of hypothermia in an infant whose clothing is removed for phototherapy. An
accurate measurement of intake and output is essential to make sure the infant is
not dehydrated. Assist the family in breastfeeding or bottle-feeding as
appropriate.n other professionals such as staff from a medical supply company (to
service equipment), a lactation specialist, and a pediatrician (to coordinate
services).

Nursing Alert

In cases of severe and untreated hyperbilirubinemia, bilirubin encephalopathy can


cause serious neurological sequelae. The term acute bilirubin encephalopathy is
used to describe the acute effect, of bilirubin toxicity in the first weeks of life. The
term kernicterus is used when referring to chronic and permanent brain damage
related to bilirubin toxicity (Moerschel et al., 2008).

Discharge Planning and Harne Care Teaching

Problems with breastfeeding in the first week of life can contribute to low caloric
intake, dehydration, and subsequent risk of neonatal hyperbilirubinemia (AAP,
2004). The nurse assesses adequacy of breast feeding prior to hospital discharge
and coordinates with the newborn's care provider in making appropriate referrals to
lactation specialists and support groups in the community when necessary.

For term infants who develop uncomplicated hyperbilirubinemia, home


phototherapy may be provided with the use of the fiber-optic pad, also known as a
biliblanket (Brethauer & Carey, 2010). Serum bilirubin levels must be monitored
regularly at the physician's office or neighborhood laboratory, or by the home health
care worker. A visiting or home health care nurse often visits the family to establish
the phototherapy and inform parents about the care needed. The nurse partners with
other professionals such as staff from a medical supply company (to service
equipment), a lactation specialist, and a pediatrician (to coordinate services).

Evaluation

Expected outcomes of nursing interventions include:

• The term or near-term newborn at risk for hyperbilirubinemia is identified prior


to discharge and receives appropriate follow-up.

• The infant's parents understand who and when to call if they suspect development
of hyperbilirubinemia.

• The infant receives appropriate intervention if hyperbilirubinemia occurs.

• The infant's nutritional and fluid intake are adequate to meet growth and
development requirements.

• The infant does not develop neurological sequelae as a result of


hyperbilirubinemia.

Biliary Atresia

Biliary atresia results when the extrahepatic bile duct fail to develop or are closed.
The disorder leads 10 cholestasis, cirrhosis, end-stage liver disease, and death by 2
years of age, if left untreated (Flanigan, 2007; Hartley, Davenport, & Kelly, 2009).
Biliary atresia occurs in approximately I in 14,000 births in the United States
(Wadhwani, Thrmelle, Nagy, et aI., 2(08). It is the most common cause of
pathologic jaWldice in infant~ and is the leading indication for pediatric liver
transplantation (HartII.")' et aI., 2009; Khalil, Thamara, Perera, et aI., 2009}. The
Gluse of biliary atresia is unknown. Absence or bloclGJge of the extrahepatic bile
ducts results in bloded bile flow from the livtr to the duod~num. Thi. altered bil~
flow .oon cau ..... inflammation and fibrotic changes in the liver. In addition to
blockage, the disease GIn also be caused by hepatocellular dysfunction. Lack of
bile acids also interferes with digestion of fat and absorption of fat-soluble vitamins
A, D, E, and K, resulting in steatorrhea and nutritional deficiencies. Without
treatment the disease is fatal. Initially the newborn is asymptomatic. Jaundice may
not be detected Wltil2 to 3 weeks after birth. At that point bilirubin levels increase,
accompanied by abdominal distention and hepatomegaly (see Appendix D = for
bilirubin levels and other liver function tests). As the disease progresses,
.lplenomegaly occurs. The infant experiences easy bruising, prolonged bleeding
time, and intense itching. Stools ha'·e puttyli:ke consistency and are white or day
colored because of the absence of bile pigments. Excretion of bilirubin and bile salts
results in teacolored urine. Failure to thrive and malnutrition occur as the
destructive changes of the disease progress. Diagnosis is based on the history,
physical examination, and laboratory evaluation. Laboratory fUldings reveal
elevated biliruhin le"",l<,el""~t....t ""nlm ~minotr~mfern..,.~nd .tkotine phn . • _
phatase values, prolonged prothrombin time, and increased allUltonia len·ls.
Percutaneous Ii'·er biopsy suggests bill1ry atresia, and cholangiography and an
exploratory laparotomy confirm the diagnosis (Roach & Bruny, 2008 ).

794 Chap,.,. H • Alter.ltioM in G3!troin~stinal Function

1're"lm .. nl inWll ..... . ."rB"'"Y '0 ""emp' mrrenion of 'he nh_ strudion
(hep~toportoenterostomy) and supportive care. In the hepatoportoenterostomy
(Kasai procedure), a segment of the intestine is anastomosed to the porta ht'patis.
The primary purpose of this procedure is to promote bile !low from the liver.
Intravenous antibiotics are administered in the postoperative period to prevent
cholangitis. Prophylaxis with oral antibiotics is continued for 1- 2 years after
surgery (Flanigan, 2007). Additional treatment includes administration of
intramuscular vitamin K prior to invasive procedures and surgery to decrease the
risk of bleeding afterward; ursodeoxycholic acid (Actigall) to promote bile flow;
and vitamins A. D, E, and K to provide supplementation since absorption of these
vitamins is impaired. The infant is breast fed or is given Pregestimil or Nutramigen,
formulas that contain medium chain triglycerides. As the liwr disease worsens, the
child may need cholestyramine and antihistamines to help de<:rease itching. Enteral
feedings and TPN may be needed as well (Hanigan, 2007). 'Vbile bile flow is
achieved with the Kasai procedure in many children with biliary atresia,
approximately 70-80% of children having this mrgery will eventually need a liver
transplant (Rooch & Bruny, 2(08). Advances in transplantation surgery now maIT
it pOlSible to perform partiallh-·er transplants from living donor resections. This
enables transplantation to be performed before the child develops end-stage liver
disease (Flanigan, 2(07). One-year survival rates of 85% and 86% haw been
reported in recent studies (Farmer, Venkk, McDiarmid, et a1, 2007; D'Alessandro,
Knechtle, Chin, et aI., 2(07).

NURSING MANAGEMENT .•

Nursing care in the initial.tages of biliary atresia is the same as that for any healthy
newborn. As symptoms develop, the focus of nursing care becomes long-term
management and support. Diagnosis of this potentially fatal disorder can be
devastating to parents. Provide emotional support and offer frequent explanations
of te!ts during the initial diagnostic eVAluation. As the dise .... pros~ 'he infant
becomes irritable because of intense itching and the accumulation of toxins. Thpid
baths may help to reliew itching and provide comfort Dry skin by patting rather
than rubbing to avoid further skin irritation. Promote rest by grouping r.ursing
activities while the infant is awake. Care following a h~toportoenterostomy is
similar to that for a child undergoing ~bdominal surgery. (See the earlier diS{ussion
of postsurgical nursing management for appendicitis and Nursing Care Plan: The
Child Undergoing Surgery in Chapter II = .) R>sttransplar.t care includes
immunosuppressant drugs and close monitoring for vascular complications.
Discharge planning focuses on teaching parents how to care for the child's skin,
providing for nutritional needs, administer· ing medications, and monitoring for
progressing symptoms of liver disease. When the child has received a transplant,
teach par· ents how to identify signs of rejection {nausea, vomiting, fewr, and
jaundice}, as well as the administration and side effects of immunosuppressant
medications. Refer parents to support groups, clergy, or social services if indicated.
Theywill need ongoing visits from a home health care nurse to help them manage

'he ~hil<l' . • enm!,l"" ",re. The main ""p"ctecl nmenm"" of nnr . • _ ing care ue the
parent's ability to cope with the child's health status and to provide the necessary
care. Palliatiw care may need to be discussed with the family if it becomes evident
the child will not survi,·e. See Chapter 13 oc,.

Viral Hepatitis Hepatitis is an inflammation of the liwr (amed by a viral infec· tion
(Figure 25--16 )0 ). It maybe acute or chronic. Acute hepatitis is rapid in onset and
if untreated may dewlop into chronic hepatitis. The most frequently diagnosed
causative organisms are hepatitis A virus (HAV). hepatitis B virus (HBV), and
ht'patitis C virus (HCV). A lesser known type is hepatitis D virus (HDV). This type
of ht'patitis only occurs in individuals who have HBV infection (Holloway &
D'Acunto, 2006). Hepatitis E virus (HEV) occurs primarily in developing countries
and is rarely seen in the United States (CDC, 2008b). In 2006, the incidence of
hepatitis A declined to its lowest rates with only 1.2 cases per 100,000 population
reported in the United States. The incidence of hep.1titis E has also decreased
remarkably owr the past se'~ral years to a low in 2006 of 1.6 cases per 100,000
population in the United States (Wasley, Grytdal, & Gallagher, 2(08). The decline
in both of these illnesses is related to routine vaccine administration esp,dally in
children.

Etiology and Pathophysiology Hepatitis A is highly contagious and traditionally


has been called infectious hep.1titis. infection occur. primarily through the fecal-
oral route. Transmission is by direct person-to-person spread or through ingestion
of contaminated water or food (particuLuly shellfish). Hepatitis A frequently occms
in children in childcare settings where hygiene practices are poor. Food handlers
can spread hepatitisAif not aware of their infection; it is a common cause of
foodborne illness. The virus can liw on surfaces for 1 month. Beamse the virus is
transmitted in the early stages of the disease when individuals are often
asymptomatic or only mildly ill, luge numbers of people may be exposed before
lh~ uiagHUIob;" LUHfin".,,J (l'dul., 25-4). Mu,( Lhilu[t"ll [<:<.u,." from hepatitis
A:, however, in rare instances end-stage liver disease can dewlop (Yazigi &
Balistreri, 2(07). Hepatitis B, known traditionally as serum hepatitis, is a serious
disease and is the mOlt common type of hepatitis in the United States (McNabb,
2007). Transmission is usually by the parenteral route through lh<' exchange of
blood or any Jxxly secretion or fluid. Other common transmission routes include
sexual activity and transmil.sion from mother to fetus in utero. Adolescents who
use intravenous drugs and have unprotected sexual intercourse are at risk for
contracting hepatitis B. Major sources for the spread of HBV are healthy chronic
carriers. All body fluids of infe<:ted individuals are potentially contaminated with
the virus. The hepatitis C virus is transmitted primarily through blo<Xl and blood
prOOucts,and blood banks now test for this virus. Infected children have commonly
had repeated transfusions (as in sickle cell disease or hemophilia). Intravenous drug
use, body piercing, and multiple sexual partners are also risk factors. Infected
mothers may infect their infants before birth or during

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