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HEMOLYTIC DISEASES OF THE

NEWBORN

INTRODUCTION
Although the diagnosis of hemolytic disease of the fetus or newborn includes
a number of conditions, it usually is reserved for infants having an increased
rate of red blood cell destruction caused by maternal isoimmunization during
the first 4 weeks of life.

TYPES OF HEMOLYTIC DISEASES


 The Rh incompatibility
 ABO incompatibility

ISOIMMUNIZATION

Isoimmunization is the development of antibodies against an antigen derived


from a genetically dissimilar individual. When there is a maternal-fetal blood
incompatibility, isoimmunization occurs in the neonate. Routine ABO and Rh
typing should be done on all expectant mothers early in pregnancy to
determine those who are type O,Rh-negative or both.

RH INCOMPATIBILITY

Three blood combinations are always compatible-that is,the Rh factors of the


blood of each parent are such that an infant-mother sensitization cannot
arise. These occur

1. When both parents are Rh positive(DD or Dd)


2. When both the Rh-negative (dd)
3. When the mother is Rh positive(DD or Dd) and the father Rh-
negative(dd)

Erythroblastosis fetalis occurs when the mother is Rh-negative and the


father is Rh positive.Erythroblastisisfetalis does not always occur in the
infant born of an Rh-incompatible mating, because of the maternal
sensitization, paternal heterozygosity and small families.

PATHOPHYSIOLOGY
During pregnancy, the circulatory system of the mother and fetus are
thought to be separate. There may be fetal-maternal exchange of blood
when some villi of the placenta and a small amount of fetal blood enter
the maternal circulation during pregnancy, or when a relatively large
transplacental hemorrhage occurs at delivery. Fetal red blood cells leak
into the maternal circulation and maternal antibodies are formed. The first
child is less likely to be affected than are later children. The reason for this
is that the mother who produces antibodies easily begin to do so in
response to the stimulus of the first fetus. Most of the production of anti-
Rh(antiD) antibodies occurs in the first 72 hours after delivery.

During the second and subsequent pregnancies with an Rh-positive fetus,


these anti-Rh agglutinins are carried from the mother’s blood across the
placenta into the blood stream of the fetus, where they destroy the fetal
erythrocytes by their specific reaction with the Rh-positive cells. In
response to this destruction or hemolysis of red blood cells, the rate of
erythropoiesis in the fetus is increased. This results in the production of
immature red blood cells, which are found in the fetal circulation. It is
because of the presence of increased erythroblast that this condition is
termed erythroblastosis fetalis.

ASSESSMENT
Great variability exists in the severity and manifestations of
erythroblastosis fetalis.The colour of the amniotic fluid seen when the
membranes break gives some indication of the severity of the condition. If
the fluid is straw coloured,the infant is not affected,or only mildly so. If it
is deep yellow, the newborn is severely affected, and if it is brown or
green, the fetus is probably extremely ill or stillborn from hydrops
fetalis.Hydrops fetalis indicates a severe edema due to cardiac failure in
utero.

After birth, antibodies attached to the circulating erythrocytes of affected


infants (direct coomb’s test)can conform the diagnosis. Because of
hemolysis of erythrocytes occurs in utero,live infants at birth have an
anemia often have evidence of asphyxia,acidosis,and elevated venous
pressure .Heart failure may occur rapidly because of the inability of the
blood to carry oxygen or because of mobilization of fluid from extra
vascular spaces during transfusions to correct the anemia.

The fetus in utero does not become jaundiced, because the bile pigment
is excreted through the placenta into the maternal circulation. After birth,
the serum bilirubin level rises in relation to the degree of red blood cell
hemolysis.Progressively increased jaundice results. This jaundice can be
detected through laboratory blood tests or with the use of a recently
developed reflectometer that measures light reflected from the infant’s
skin and indicates the amount of bilirubin in the blood.

Most bilirubin in the blood binds itself to albumin, a protein. Only the free
bilirubin that is left over can enter the brain.Unconjugated bilirubin that is
left over can enter the brain.Uncongugated bilirubin can pass from the
placenta to the brain through the blood brain barrier when there is a high
concentration of this substance in the blood. The result in damage to the
basal ganglia of the brain stem, producing the syndrome of
kernicterus.Kernicterus is manifested by central nervous system
depression or excitation. The indications of kernicterus include
leathargy,poor sucking reflex, muscular rigidity or
flaccidity,opisthotonos(a type of spasm in which the head and the heels
are bent backward and the body bowed foreword),and the high pitched
cry. In the final phase of kernicterus, athetoid cerebral palsy, loss of
vertical gaze, and high frequency nerve deafness occur as a result of
brain damage. To determine whether a jaundiced neonate is at risk for
brain damage, a difficult laboratory test can be done on the blood.

ABO INCOMPATIBIITY
The mother with blood group O has got naturally occurring anti-A and
anti-B agglutinins. These antibodies are mainly ig M and do not cross
the placenta. If the fetus happens to be blood group A or B
corresponding to that of father, the immune antibodies are formed in
response to the entry of A or B antigen bearing fetal red cells, into the
maternal circulation. As these are mainly IgG,they can cross the
placenta into the fetal circulation and cause a variable amount of
haemolysis due to antigen antibody reaction.
Hemolytic diseases caused by ABO incompatibility is more
common but causes fewer problems before or after birth than does Rh
incompatibility. Generally ,in ABO incompatibility, the mother is type O
and the fetus type A or, less often, type B.Since the presence or
absence of antigens and antibodies determines whether agglutination
can occur, the AB blood group, which contains no antibodies, cannot
produce an incompatibility.
PATHOPHYSIOLOGY
The fact that ABO factors in the fetus and neonate have low
antigencity may explain why there is a low incidence of severe ABO
hemolytic disease in comparison with the incidence of incompatibility
between mother and infant. Although anti-A and anti-B antibodies
occur naturally without prior immunization in the maternal IgM fraction
of gamma globulin, these do not cross the
placenta.Univarient,incomplete antibodies to A antigen may be present
in the IgG fraction, however and can cross the placenta. This is the
reason that A-0 Isoimmune hemolytic disease may occur in the firs-
born babies.
ASSESSMENT
Usually, neonates with ABO incompatibility are affected only midly at
birth. They may become jaundiced during the first 24 hours after
delivery, but severe anemia with pallor or hydrops fetalis is extremely
rare. The liver and spleen are not usually enlarged. Increasing jaundice
with signs and symptoms of kernicterus occurs rarely.
The diagnosis is made on the basis of ABO incompatibility and a weakly
to moderately positive directive Coombs test on red blood cells. The
indirect Coomb’s test may be strongly positive if the neonate’s serum
is mixed with adult A or B cells. The infant Hb is usually normal, but
hyperbilirubinemia is evident to a varying degree.
NURSING MANAGEMENT IN HEMOLYTIC DISEASE
 INTRAUTERINE TRANSFUSION
During pregnancy, if the maternal antibody titer rises, and if on
amniocentesis it is found that the concentration of bilirubinoid
pigments in the amniotic fluid is increased, an intrauterine
transfusion can be given to the erythroblastotic fetus.
The procedure is done if the fetus is too immature to survive
delivery if it is so ill that its life is in danger. Using the same
procedure utilized for amniocentesis, a radio opaque dye is
injected into the amniotic fluid. As the fetus swallows the dye,
the gastrointestinal tract is outlined. Type O Rh negative blood in
the form of packed red cells is then injected into the peritoneum
of the fetus. When these red blood cells are absorbed, the fetal
anemia is reduced. The transfusion may have to be repeated
until the fetus is mature enough to be delivered. During each
transfusion, the fetal heart sounds must be carefully monitored.
The mother must also observe for leakage of amniotic fluid or
bleeding following its procedure.
 IMMEDIATE CARE AT DELIVERY
At the time of delivery, the goals of nursing management of
erythroblastotic infants are to establish adequate ventilation and
to provide oxygen as necessary. Neonates who have hydrops
fetalis may require assisted ventilation at birth, often a high
inspiratory pressures. If these infants have ascites, paracentesis
may be necessary to remove excessive fluid from the abdominal
cavity. The infants must be dried thoroughly after birth and kept
warm in order to minimize cold stress.
At birth, cord blood samples are obtained for determination of
Hb,HCT,total and direct bilirubin concentrations, and albumin
concentration as well as for coomb’s test. Some of these tests
may need to be repeated after admission to the intensive care
nursery. If an intrauterine transfusion was given, the cord blood
may prove to be negative on the direct Coomb’s test and the
infant may type as O-negative.
If the neonate is severely affected, as evidenced by anemia,
inadequate oxygenation, or metabolic acidosis, packed type O
blood cells compatible with the mother’s blood may be given
after delivery. The nurse keeps the umbilical cord moist with
sterile saline dressings in case such a transfusion is not given for
kernidterus, because this condition is not a threat to the neonate
during the 1st hour of life.

 PHOTOTHERAPY
Increasing jaundice presents an other concern in the care of
these newborns. Phototherapy may be used to prevent the need
for blood replacement in mildly infants.However, it may not be
too effective in lowering the level of bilirubin.Although
Phenobarbital therapy of the mother may enhance the effect of
the glucuronyl transferase enzyme in the fetus, decreasing the
early rise in bilirubin in the infant, exchange transfusion is the
only acceptable therapy when the bilirubin reaches hazardous
levels.
 EXCHANGE TRANSFUSION
The purpose of an exchange transfusion are to prevent the
bilirubin from rising to a level that could lead to kernicterus and
to correct the anemia caused by the hemolytic process. More
specifically, an exchange transfusion circulating antibodies and
replaces sensitized erythrocytes and lowers the bilirubin level in
the blood.
Fresh donor blood that is compatible with the blood of both
mother and infant should be used for exchange transfusion. If
the blood is prepared before delivery, packed O Rh negative cells
mixed with AB plasma may be used.
The nurse assist the physician in starting the exchange
transfusion and checks the blood type and group against the
neonate’s blood before administering it to prevent a hemolytic
reaction caused by mismatched blood. The blood is kept the
refrigerated prior to its use. It is gradually warmed to a
temperature between 22 and 37°c, which is maintained
throughout the transfusion. The blood should be kept well mixed
by squeezing or agitating the bag in order to avoid the
sedimentation. The neonate’s temperature is maintained under a
radiant warmer and blankets should be used if the infant become
chilled.
Additional responsibilities of the nurse during the exchange
transfusion include evaluating the electronic monitoring of vital
signs, recording the amount of blood with the time it is
withdrawn and infused and the total volume exchanged, and
observing the infant’s general condition. The venous pressure
should be measured intermittently in order to detect congestive
heart failure. The nurse continually assess the condition of the
infant for indications of a transfusion reaction, metabolic
academia as evidenced by deep rapid respirations and decreased
level of consciousness as well as acid urine, and cardiac failure
caused by too rapid infusion of blood and possible excessive
replacement.
Throughout the procedure the nurse and the physician maintain
a sterile, closed system in order to avoid air embolism and
sepsis. After the procedure the infant must be observed for
bleeding from the transfusion site. The Cather may be left in
place, after being flushed with sterile normal saline to prevent
clot formation, in case it needed for another exchange
transfusion. An intravenous solution is infused or the area is
protected with sterile dressings and checked frequently for
indications of infection or bleeding.
Hypoglycemia may occur after an exchange transfusion because
the infant’s stomach was emptied before the procedure and the
infant was kept in a fasting state for a few hours before and
during the exchange to prevent aspiration. The infant’s blood
glucose level should be checked hourly, using Dextrostix,for 2 to
4 hours or more after the transfusion is completed.
 EMOTIONAL SUPPORT
The parents of newborns having hemolytic anemia may feel
guilty about their infant’s illness. This is especially true in the
case of an Rh incompatibility when the mother did not receive
RhoGAM after her first pregnancy. The nurse can help the
patients to verbalize their anxiety and guilt and can reassure
them about their neonate’s condition. The positive aspects as
well as the risks of any treatments given to their infant should be
thoroughly explained. The parents can be helped to recognize
that their continued parental interest is an important factor in
their infant’s progress.
OUTCOME
Most newborns recover well from hemolytic disease unless
indications of kernicterus are observed. Parents of infants with
this condition should share this condition should share this
information with the private physician or the health personnel in
the clinic where the infant will receive continuing care. The infant
should be assessed periodically for evidence of cerebral damage,
hearing loss, or developmental lags. Therapy should be planned
as appropriate for the individual infant.
PREVENTION
Erythroblastosis fetalis caused by Rh incompatibility can be
prevented by administering 300 mg immunoglobulin
intramuscularly to an Rh-negative mother within 72 hours of a
spontaneous or therapeutic abortion of an Rh-positive fetus or
the delivery of an Rh-positive infant. It can also be given in the
28th week of pregnancy for patients at risk.Rh D immune globulin
should also be given to the Rh-negative mother following
amniocentesis at any time during pregnancy.
NURSING DIAGNOSIS
Nursing diagnosis may include physiologic and psycho social problems
experienced by the individual mother or father, or problems occurring within
the couple or family because of the loss and subsequent grief. Examples of
nursing diagnoses include the following

1. Ineffective family coping related to


 Inability to make decisions as a family
 Difficulties in communication within the family
 Conflicting coping patterns between mother and father
2. Powerlessness related to
 High risk pregnancy and birth
 Unexpected cesarean birth
 Inability to prevent infant’s death
3. Interrupted family processes related to
 Maternal depression leading to changes in role function
 Inadequate communication of feelings between the grieving
mother and father
 Lack of expected support from family
 Behavioral and emotional reactions of siblings
 Grief within the family system including grandparents and
other relatives
4. Fatigue and disturbed sleep pattern related to
 Inability to fall asleep because of grief
 Waking in the night and thinking about the loss
 Restrict number of visitors
5. Dysfunctional grieving related to
 Prolonged denial or avoidance of the loss
 Intense guilt related to the loss
 Continued anger about the loss
 Serious depressive symptoms and despair
 Loss of self-esteem
 Intense grieving patterns that continue for more than a year
 Social isolation due to grief
6. Spiritual distress related to
 Anger with God
 Confusion about why prayers were not answered
SUMMARY
In this seminar I had dealt with the hemolytic diseases of the
newborn(Rh incompatibility, ABO incompatibility),its
assessment,pathophysiology,clinical
manifestations,prevention,management and its nursing
diagnosis.

CONCLUSION
Hemolytic disease of the new born (HDN) is a blood disorder in
a fetus or new born infant. In some infants, it can be life –
threatening.
HDN may develop when a mother and her unborn baby have
different blood types (called “incompatibility”). The mother
produces substances called antibodies that attack the developing
baby’s red blood cells.
BIBLIOGRAPHY

 Dorothy R Marlow,Barbara A Redding,Textbook of Pediatric


Nursing 6th edition,2009,Page no:442-446
 D.C.Dutta’s,Textbook of obstetrics,6th edition,2004,Page
no:480-481.
 Nelson Text book of Paediatrics,7th edition,Sunders
Publishers,Page no:442-444
 Mehaban Singh,Textbook on Care of the newborn Page
No:242-245
 Annamma Jacob,A comprehensive Textbook on midwifery
and gynaecological nursing,1st edition,2005,Jaypee
publishers.Page No:322-345
 Lowdermilk,Perry,Cashion.Maternity Nursing,8th
edition,Page No:804.
JOURNALS
 The Indian journal of Paediatrics Dr.Varma I C,Volume
79/number 2/March 2012 Page Number 58-61.
 The Indian journal of Paediatrics Dr.Varma I C,Volume
79/Number 12/March 2010 Page No:97-99.

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