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Care of the Neonate with Erythroblastosis Fetalis
Care of the Neonate with Erythroblastosis Fetalis PATRICIA A . DUNN, RN, MSN, VINOD BHUTANI, MD, STUART WEINER, MD, AND A VI L UDOMIRSKI, MD Erythroblastosisfetalis, hemolytic disease of the newborn, occurs when an isoimmunized mother produces antibodies that cross the placenta and cause hemolysis of fetal red blood cells. This hemolysis can be accompanied by severe anemia, ascites, pleural and pericardial effusions, congestive heart failure, and neurological damage with resultant perinatal mortality. Rh isoimmunizationin pregnancy still occurs in spite of the advent of Rh immune globulin. This article describes the complex management and nursing implications associated with caring for the neonate with erythroblastosisfetalis.
Erythroblastosis fetalis, a disease of the fetus and newborn, is characterized by hemolytic anemia due to incompatibility between the blood group of the mother and that of the fetus. The incidence of Rh isoimmunization has decreased markedly with the advent of Rhocam@' in 1968. The incidence of infant mortality from Rh disease has declined from 2.7 deaths per 10,000 live births in 1968 to 0.9 deaths per 10,000 live births in 1975.' However, isoimmunization in pregnancy, with resultant erythroblastosis fetalis may still occur in the current pregnancy due to fetalmaternal bleeding or as a result of previous failure to administer RhoGam, administration of an insufficient dose of Rhocam, or maternal sensitization to other irregular antibodies such as Kell, Kidd, Duffy and others.',' Also, a mother may have been immunized by an antigen in the fetal red blood cells from a previous pregnancy, abortion, or blood transfusion. During pregnancy, the maternal antibodies cause destruction of the fetal red blood cells, which then may result in severe hemolytic anemia in the fetus. Accepted: March 1988
382
To compensate for the excessive hemolysis, active proliferation of erythropoietic activity occurs in the liver, spleen, and bone marrow. The manifestations of erythroblastosis fetalis reflects an interplay between the degree of red blood cell destruction and red blood cell production. Intrauterine red blood cell destruction leads to anemia associated with generalized edema, ascites, pleural effusions, petechiae, and tachycardia-the classic picture of hydrops fetalis. Jaundice is generally absent at birth because of placental excretion of bilirubin. Kernicterus, a lifethreatening condition, results from unconjugated bilirubin entering the brain tissue and causes neurological damage and aeficits. Postnatal red blood cell destruction contributes to progressive, kernicterusthreatening jaundice. While anemia and jaundice are two obvious features af erythroblastosis, management of the fetus or neonate needs to focus on both these obvious hemodynamic and metabolic alterations as well as on prevention of kernicterus.' Some severely affected fetuses can be transfused in utero, which can decrease the risk of fetal morbidity and In utero transfusion may alter the neonatal course of treatment. The purpose of this article is to familiarize the perinatal nurse with the
November/December 1988 JOCNN
neonatal management and nursing implications related to care of the newborn with severe hemolytic disease, and potential hemodynamic compromise, and hyperbilirubinemia. MANAGEMENT
Infants with severe hemolytic disease sometimes sustain significant hypoxia in utero, and women who deliver such infants should be managed in perinatal centers capable of the full range of neonatal intensive care.5Abruptio placenta, prolonged bleeding, or placental-fetal insufficiency can further compromise the fetus with hemolytic disease. These conditions, along with anemia and hydrops, are some factors that may lead to perinatal asphyxia. Therefore, delivery should be anticipated and timed so that the resuscitation team can be present.
Respiratory Problems The neonate with severe erythroblastosis fetalis has the potential for respiratory compromise secondary to hydrops. Besides standard resuscitation equipment and medications, butterfly needles and syringes for performing immediate paracentesis, thoracentesis, and pericardiocentesis should be available in the delivery room. Infants born with erythroblastosis fetalis are likely to have intense pulmonary vasoconstriction secondary to hypoxemia, acidosis, or hypotension. A pattern of persistent fetal circulation (PFC), with rightto-left shunting, may need to be reversed by standard management for persistent fetal circulation.6 This management involves oxygenation, hyperventilation, and support of intravascular pressure^.^ Erythroblastoic infants also develop hyperplasia of beta cells in the pancreatic islets of Langerhans, which causes secondary hyperinsulinism.6*8This same mechanism may account for delayed synthesis of mature surfactant and the need for ventilatory support for respiratory distress syndrome. Prompt intubation and mechanical ventilation may need to be established immediately after delivery. If ascites or pleural effusions are present, causing some degree of pulmonary hypoplasia, ventilation is best accomplished with relatively high pressures.6 Thus, the respiratory compromise seen in erythroblastoic neonates can be secondary to factors such as accumulated pleural effusions, ascites, and pulmonary immaturity, each requiring unique modalities of treatment.
Hemodynamic Problems The neonate with erythroblastosis fetalis also has the potential for hemodynamic compromise includ-
November/December 1988 JOCNN
ing anemia and altered intravascular pressures. Since anemia can be anticipated, a unit of fresh, irradiated, whole blood should be available in the delivery room for exchange or booster transfusion. The blood should be group 0, Rh-negative, and cross-matched against the mother’s serum to avoid further hemolysis. For subsequent transfusions, the blood type of the donor should match that of the infant.
I
Erythroblastosis fetalis is a hemolytic disease of the newborn resulting from an isoimmunized pregnancy. Care of the neonate with erythroblastosis fetalis involves obtaining a history, performing a physical assessment, monitoring signs and symptoms, and providing emotional support Ito parents. Initial umbilical cord blood specimens should be sent for blood gas analysis, complete blood count with differential, reticulocyte count, and platelet count. Blood group, type, antiglobulin (Coombs’ test), serum albumin, globulin, and direct and total bilirubin should also be assessed.6 The nurse should consider that blood type and Coombs’ test results may be altered as a result of possible intrauterine transfusions. An umbilical artery and the umbilical vein should be catheterized as quickly as possible and aortic (central blood pressure) and central venous pressures should be monitored. Hematocrit changes occur rapidly after birth in hydropic infants due to rapid fluid shifts between the intravascular and extravascular compartments; therefore, hematocrit and blood gas analyses should be repeated as soon as the umbilical artery catheter is inserted.6 Depending on the rate of hemolysis, infants that were transfused in utero may, or may not, be severely anemic (hematocrit less than 35%). Anemia should be promptly corrected. To manage effectively the severely anemic neonate, continuous intravascular pressure measurements must be closely monitored. If central venous pressure is normal or low and the central blood pressure is low, then the intravascular volume is deficient. This decrease in intravascular volume can be seen in association with blood loss (Lea, placenta abruptia, intrapartum asphyxia with resultant peripheral vasoconstriction, and impending shock). These infants should receive a series of small transfusions of packed red blood cells, via an easily accessible umbilical vein catheter (10 ml/kg should be transfused over 3 to 5 minutes). Intravascular pressures and hematocrits should be checked
383
between transfusions. Two to three transfusions are usually enough to raise the hematocrit Over 35%, which is adequate correction of the oxygen-carrying capacity. If the infant with a contracted vascular compartment was severely asphyxiated, correction of hypoxemia, hypercarbia, acidosis, and anemia can produce a vasodilation followed by a fall in systemic pressures to a subnormal level. This infant would then need further volume Because albumin increases the plasma volume and may precipitate congestive heart failure, albumin administration is contraindicated in the severely anemic or hydropic infant. If the intravascular pressures are normal, the intravascular volume may be normal. However, the intravascular volume also may be subnormal, but the associated low pressure readings are offset by the systemic vasoconstriction associated with asphyxia. This difference may not be discriminated initially; however, blood volume should not be increased with a push transfusion while the possibility of contraction of the vascular compartment exists. Instead, exchange transfusion with packed cells should be started. The blood for the exchange should be infused through the umbilical vein catheter, and blood should be withdrawn through the umbilical artery catheter. Withdrawal of blood through the umbilical artery catheter should be done slowly to avoid hypoperfusion of the organs supplied by the descending aorta.'j Both central blood and central venous pressures may be elevated. This usually occurs with normal or slightly subnormal blood volume, with intense systemic and pulmonary vasoconstriction secondary to asphyxia.6 Acidosis, hypoxemia, and anemia should be corrected and intravascular pressures reassessed. Phibbs recommends that exchange transfusion with packed cells be carried out to raise the hematocrit above 35% (Table l).'j These infants are also more susceptible to pulmonary, intracranial, and gastrointestinal hemorrhage. Thrombocytopenia secondary to exchange transfusion may aggravate bleeding diathesis.' Furthermore, bleeding in neonates with erythroblastosis fetalis tends to occur at platelet counts higher than would be expected in other infants. Therefore, platelet counts in these neonates should be kept above 50,000 cu mm by transfusion of platelets if necessary. Hyperbilirubinemia
Once the infant is stable, a double-volume exchange transfusion should be considered. This exchange transfusion will control hyperbilirubinemia
384
Table 1. Pressure Management Guidelines for Neonates with Erythroblastosis Fetalid Central Blood Pressure
Central Venous Pressure
Low
Normal or low
Normal Low High
Normal Low High
Management Series of small transfusions of packed red blood cells Exchange transfusion Volume expansion Exchange transfusion of packed red blood cells
Exact levels are not reported because normal levels vary with gestational age and weight of the infant.
by washing out bilirubin and antibody-coated cells and will increase serum bilirubin binding capacity by raising the plasma protein concentration. An increase in plasma oncotic pressure will follow exchange transfusion, and thereby, decrease edema through initiation of diuresis. Salt-poor albumin should be avoided as a volume expander, as albumin leaks rapidly from the circulation and may actually prolong edema in tissues and in the lungs. When vascular compartments are stable, furosemide (1 mg/kg) may be used with caution to evoke a diuresis not initiated by volume expanders.'jS8 The course of hyperbilirubinemia tends to be highly erratic in infants who were transfused in utero via an intravascular technique such as percutaneous umbilical blood sampling. According to the authors' experiences, some initial bilirubin levels at the time of birth have been over 6 mg/dl at birth. However, not all of these infants have required exchange transfusions, which may be attributed to the immediate and aggressive use of phototherapy at the authors' institution. Phototherapy alters the structure of the bilirubin molecule in light-exposed tissues so that this bilirubin can be excreted by the kidneys or liver.g At the authors' institution two or three phototherapy units, (blue spectrum) are used to increase radiant density. Use of multiple phototherapy units is done on an empirical basis to achieve optimal photo-illumination over maximal surface area. According to the authors' clinical observations, infants who were transfused in utero by an intravascular technique require less exchange and booster transfusions. However, anemia at 6 to 8 weeks of age is more common because of the prolonged persistence of maternal antibodies as exchange transfusion can assist in the clearance of these antibodies."*" Infants with erythroblastosis fetalis also need to be observed frequently by chemistry dip strip for hypoglycemia. Hypoglycemia is a further complication of infusion of glucose or exchange transfusion. Other
November/Decernber 1988 JOCNN
metabolic problems could occur and blood electrolytes, calcium, and phosphorus levels should be assessed periodically. NURSING IMPLICATIONS
Caring for the infant with erythroblastosis fetalis presents a tremendous challenge to the neonatal nurse. The goal of nursing management is to identify the infants at risk and intervene before further central nervous system sequelae develop. At the authors' institution, all infants with known hemolytic disease are admitted to the intensive care nursery. Nursing of these infants includes the following interventions: 0
0
0
0
0
0
Obtain a history. Information can be obtained from multiple sources including the perinatal and delivery room records, delivery room nurses and physicians, and the parents. Pay special attention to the details of the obstetric history and outcomes of previous pregnancies. If the fetus was transfused in utero, the data base should include the date of the last transfusion, interval between transfusions, and fetal blood studies including hematocrit and blood type. Perform a physical assessment. Auscultate the lungs noting any abnormal breath sounds. Observe for any edema and palpate the liver for size. Of particular importance in neonates who received in utero transfusions is to note the condition of the cord for any obvious thrombus o r hematoma. Collaboratively monitor vascular pressures, laboratory tests, and chemistry strip glucose levels. Any deviations from normal pressure readings should be reported to the physician. Bilirubin and glucose levels in neonates with erythroblastosis fetalis should be tested approximately every four to six hours until stable. Initiate phototherapy as ordered. As much of the skin surface as possible should be exposed to the photo therapy lights, and t h e neonate's eyes should be covered with opaque patches. All infants under blue phototherapy lights should be monitored as lights of the blue spectrum may mimic cyanosis.'2 Maintain nothing-by-mouth (NPO) status as ordered. Neonates are initially NPO in anticipation of exchange transfusion as regurgitation and aspiration are possible during the procedure. Breastfeeding is permitted when the infant is stable. In the interim. the nurse can Drovide information to the mother about maintaining milk supply by explaining use of a breast pump." Provide emotional support as indicated. Parental anxiety and an alteration in parenting process are expected reactions. Intense emotional responses
November/December 1988 JOCNN
can be expected from the parents of a newborn who was treated in utero even when the infant may not be critically ill. Some of the shock can be cushioned by providing a tour of the intensive care nursery before delivery. All parents are encouraged to see and touch their newborns in the delivery room if possible. Parents have unlimited visiting privileges in the intensive care nursery, and frequent contact is encouraged. Stressing the neonate's progress and positive features can help facilitate the bonding process between the parents and their newborn. SUMMARY
Perinatal management of the neonate with erythroblastosis fetalis is based upon the prenatal detection and treatment of fetal anemia. Postnatal management focuses on treatment of anemia and jaundice, and their sequelae. Prenatal treatment consists of in utero intravascular transfusions. Postnatal treatment consists of respiratory support, management of hemodynamic alterations, and the aggressive treatment of hyperbilirubinemia to prevent kernicterus. Nursing interventions include careful observation and assessment of the overall condition of the neonate, lung sounds, intravascular pressure measurements, and laboratory data as well as the anticipation of needs such as blood for transfusion, medications, and parental support. A cohesive, interdisciplinary approach is needed when caring for the neonate with erythroblastosis fetalis. REFERENCES 1. Queenan, J. 1987. Erythroblastosis fetalis. In Neonatalperinatal medicine:Diseases of the fetus and infant,ed. A. Fanaroff and R. Martin, 53-62. St Louis: C.V. Mosby. 2. Caine, M., and E. Mueller-Heubach. 1986. Kell sensitization in pregnancy. Am J Obstet Gynecol. 85-90. 3. Ludomirski, A., and S. Weiner. Percutaneous fetal umbilical blood sampling. Clin Obstet Gynecol. 4. Warsof, S., K. Nicolaides, and C. Rodeck. 1986. Immune and non-immune hydrops. Clin Obstet Gynecol. 29:53342. 5. Maisels, M.J. 1985. Hyperbilirubemia. In Current therapy in neonatal-perinatal medicine 1985-1986, ed. N. Nelson, 213-17. Ontario: B.C. Decker. 6. Phibbs, R. 1985. Hydrops. In Current therapy in neonatal-perinatal medicine 1985-1986, ed. N. Nelson, 201-06. Ontario: B.C. Decker. 7. Duara, s., and W.W. Fox. 1986. Persistent pulmonary hypertension. In Neonatal pulmonary care, ed. D.W. Thiebeault and G.A. Gregory, 461-82. New York Appleton-Century-Crofts. 8. Oski, F.A., and J.L. Naiman. 1982. Erythroblastosis fetalis. In Major problems in clinical pediatrics: Hemato-
385
9.
10.
11.
12.
logic problems in the newborn. Philadelphia: W.B. Saunders. McDonagh, A. 1983. Molecular mechanisms of phototherapy in neonatal jaundice. In Neonatal jaundice, ed. F. Rubaltelli and G. Jori, 173. New York Plenum Press. Ludomirski, A., V.K. Bhutani, S. Weiner, and R.J. Bologanese. 1987. Fetal intravascular transfusion in the management of Rh isoimmunization. Am J Perinatol. 4:379. Bhutani, V., A. Ludomirski, J. Gerdes, and S. Weiner. 1988. Effects of prenatal intravascular transfusion on Rh isoimmunized neonates. Pediatr Res. Abstract in press. Doenges, M., J. Kenty, and M. Moorhouse. 1988. Maternal/newborn care plans: Guidelines f o r client care, 514-22. Philadelphia: F.A. Davis.
Address for correspondence: Patricia A. Dunn, 2800 Hargrave Street, Philadelphia, PA 19136.
Patricia A. Dunn is a clinical nurse specialist at Thomas Jefferson University Hospital in Philadelphia, Pennsylvania. Ms. Dunn is a member of NAACOG, Sigma Theta Tau, and the Pennsylvania Perinatal Association. Vinod K. Bhutani is an assistant professor of pediatrics at the University of Pennsylvania and associate director of Newborn Pediatrics at Pennsylvania Hospital in Philadelphia, Pennsylvania. Dr. Bhutani is a member of the American Academy of Pediatrics and the American Physiology Society. Stuart Weiner is the director of the Antenatal Testing Unit at Pennsylvania Hospital and an associate professor of Obstetrics and Gynecology at the University of Pennsylvania School of Medicine in Philadelphia, Pennsylvania. Dr. Weiner is a member of ACOG and the Society of Perinatal Obstetricians. Avi Ludomirski is a perinatologist at Pennsylvania Hospital and an assistant professor of Obstetrics and Gynecology at the University of Pennsylvania School of Medicine in Philadelphia, Pennsylvania. Dr. Ludomirski is a member of the Society of Perinatal Obstetricians.
~~
NATIONAL OSTEOPOROSIS EDUCATION PROGRAM
The Osteoporosis Education Advisory Board in conjunction with NAACOG, the Organization for Obstetric, Gynecologic, and Neonatal Nurses, offers an osteoporosis education kit, an educational program for nurses and the community. As part of a national program, this kit is designed to provide health-care professionals with a complete educational package on osteoporosis increase awareness of osteoporosis within communities throughout the country encourage women to discuss osteoporosis and appropriate preventive measures with their health-care providers and increase public awareness of the NAACOG nurse as a key source of health-care education in the community. The kit can be used for the development or supplementationof an educational session or program for consumers or nurses. This complete education package includes two, 20-minute slide presentations with accompanying scripts, a publicity guide for implementing a consumer education program in the community, background information on osteoporosis and risk assessment, and program evaluation forms. The kit is available free of charge on a loan basis only. For more information about the program, contact the Department of Education and Research, NAACOG, P.O. Box 71437, Washington, DC 20024-1437; (202)638-0026. Erratum: The above telephone number was incorrect in the March/April issue of JOGNN. The above telephone number is correct.
SEMINAR
The 13th Annual Seminar for Nurse Practitionersin ambulatory gynecology, family planning, and women’s health care will be held February 15-1 7,1989, in Philadelphia, Pennsylvania. The program is sponsored by Planned Parenthood Federation of America, NAACOG, the University of Pennsylvania School of Nursing Center for Continuing Education, and the Family Planning Council of Southeastern Pennsylvania. For further information, contact the Ob/Gyn Nurse Practitioner Program, Suite 1900, 260 South Broad Street, Philadelphia, Pennsylvania 19102; (215) 985-2612, or Planned Parenthood Federation, 810 7th Avenue, New York, NY 10019, (212) 603-4696.
386
November/December 1988 JOCNN
Erythroblastosis fetalis, a disease of the fetus and newborn, is characterized by hemolytic anemia due to incompatibility between the blood group of the mother and that of the fetus. The incidence of Rh isoimmunization has decreased markedly with the advent of Rhocam@' in 1968. The incidence of infant mortality from Rh disease has declined from 2.7 deaths per 10,000 live births in 1968 to 0.9 deaths per 10,000 live births in 1975.' However, isoimmunization in pregnancy, with resultant erythroblastosis fetalis may still occur in the current pregnancy due to fetalmaternal bleeding or as a result of previous failure to administer RhoGam, administration of an insufficient dose of Rhocam, or maternal sensitization to other irregular antibodies such as Kell, Kidd, Duffy and others.',' Also, a mother may have been immunized by an antigen in the fetal red blood cells from a previous pregnancy, abortion, or blood transfusion. During pregnancy, the maternal antibodies cause destruction of the fetal red blood cells, which then may result in severe hemolytic anemia in the fetus. Accepted: March 1988
382
To compensate for the excessive hemolysis, active proliferation of erythropoietic activity occurs in the liver, spleen, and bone marrow. The manifestations of erythroblastosis fetalis reflects an interplay between the degree of red blood cell destruction and red blood cell production. Intrauterine red blood cell destruction leads to anemia associated with generalized edema, ascites, pleural effusions, petechiae, and tachycardia-the classic picture of hydrops fetalis. Jaundice is generally absent at birth because of placental excretion of bilirubin. Kernicterus, a lifethreatening condition, results from unconjugated bilirubin entering the brain tissue and causes neurological damage and aeficits. Postnatal red blood cell destruction contributes to progressive, kernicterusthreatening jaundice. While anemia and jaundice are two obvious features af erythroblastosis, management of the fetus or neonate needs to focus on both these obvious hemodynamic and metabolic alterations as well as on prevention of kernicterus.' Some severely affected fetuses can be transfused in utero, which can decrease the risk of fetal morbidity and In utero transfusion may alter the neonatal course of treatment. The purpose of this article is to familiarize the perinatal nurse with the
November/December 1988 JOCNN
neonatal management and nursing implications related to care of the newborn with severe hemolytic disease, and potential hemodynamic compromise, and hyperbilirubinemia. MANAGEMENT
Infants with severe hemolytic disease sometimes sustain significant hypoxia in utero, and women who deliver such infants should be managed in perinatal centers capable of the full range of neonatal intensive care.5Abruptio placenta, prolonged bleeding, or placental-fetal insufficiency can further compromise the fetus with hemolytic disease. These conditions, along with anemia and hydrops, are some factors that may lead to perinatal asphyxia. Therefore, delivery should be anticipated and timed so that the resuscitation team can be present.
Respiratory Problems The neonate with severe erythroblastosis fetalis has the potential for respiratory compromise secondary to hydrops. Besides standard resuscitation equipment and medications, butterfly needles and syringes for performing immediate paracentesis, thoracentesis, and pericardiocentesis should be available in the delivery room. Infants born with erythroblastosis fetalis are likely to have intense pulmonary vasoconstriction secondary to hypoxemia, acidosis, or hypotension. A pattern of persistent fetal circulation (PFC), with rightto-left shunting, may need to be reversed by standard management for persistent fetal circulation.6 This management involves oxygenation, hyperventilation, and support of intravascular pressure^.^ Erythroblastoic infants also develop hyperplasia of beta cells in the pancreatic islets of Langerhans, which causes secondary hyperinsulinism.6*8This same mechanism may account for delayed synthesis of mature surfactant and the need for ventilatory support for respiratory distress syndrome. Prompt intubation and mechanical ventilation may need to be established immediately after delivery. If ascites or pleural effusions are present, causing some degree of pulmonary hypoplasia, ventilation is best accomplished with relatively high pressures.6 Thus, the respiratory compromise seen in erythroblastoic neonates can be secondary to factors such as accumulated pleural effusions, ascites, and pulmonary immaturity, each requiring unique modalities of treatment.
Hemodynamic Problems The neonate with erythroblastosis fetalis also has the potential for hemodynamic compromise includ-
November/December 1988 JOCNN
ing anemia and altered intravascular pressures. Since anemia can be anticipated, a unit of fresh, irradiated, whole blood should be available in the delivery room for exchange or booster transfusion. The blood should be group 0, Rh-negative, and cross-matched against the mother’s serum to avoid further hemolysis. For subsequent transfusions, the blood type of the donor should match that of the infant.
I
Erythroblastosis fetalis is a hemolytic disease of the newborn resulting from an isoimmunized pregnancy. Care of the neonate with erythroblastosis fetalis involves obtaining a history, performing a physical assessment, monitoring signs and symptoms, and providing emotional support Ito parents. Initial umbilical cord blood specimens should be sent for blood gas analysis, complete blood count with differential, reticulocyte count, and platelet count. Blood group, type, antiglobulin (Coombs’ test), serum albumin, globulin, and direct and total bilirubin should also be assessed.6 The nurse should consider that blood type and Coombs’ test results may be altered as a result of possible intrauterine transfusions. An umbilical artery and the umbilical vein should be catheterized as quickly as possible and aortic (central blood pressure) and central venous pressures should be monitored. Hematocrit changes occur rapidly after birth in hydropic infants due to rapid fluid shifts between the intravascular and extravascular compartments; therefore, hematocrit and blood gas analyses should be repeated as soon as the umbilical artery catheter is inserted.6 Depending on the rate of hemolysis, infants that were transfused in utero may, or may not, be severely anemic (hematocrit less than 35%). Anemia should be promptly corrected. To manage effectively the severely anemic neonate, continuous intravascular pressure measurements must be closely monitored. If central venous pressure is normal or low and the central blood pressure is low, then the intravascular volume is deficient. This decrease in intravascular volume can be seen in association with blood loss (Lea, placenta abruptia, intrapartum asphyxia with resultant peripheral vasoconstriction, and impending shock). These infants should receive a series of small transfusions of packed red blood cells, via an easily accessible umbilical vein catheter (10 ml/kg should be transfused over 3 to 5 minutes). Intravascular pressures and hematocrits should be checked
383
between transfusions. Two to three transfusions are usually enough to raise the hematocrit Over 35%, which is adequate correction of the oxygen-carrying capacity. If the infant with a contracted vascular compartment was severely asphyxiated, correction of hypoxemia, hypercarbia, acidosis, and anemia can produce a vasodilation followed by a fall in systemic pressures to a subnormal level. This infant would then need further volume Because albumin increases the plasma volume and may precipitate congestive heart failure, albumin administration is contraindicated in the severely anemic or hydropic infant. If the intravascular pressures are normal, the intravascular volume may be normal. However, the intravascular volume also may be subnormal, but the associated low pressure readings are offset by the systemic vasoconstriction associated with asphyxia. This difference may not be discriminated initially; however, blood volume should not be increased with a push transfusion while the possibility of contraction of the vascular compartment exists. Instead, exchange transfusion with packed cells should be started. The blood for the exchange should be infused through the umbilical vein catheter, and blood should be withdrawn through the umbilical artery catheter. Withdrawal of blood through the umbilical artery catheter should be done slowly to avoid hypoperfusion of the organs supplied by the descending aorta.'j Both central blood and central venous pressures may be elevated. This usually occurs with normal or slightly subnormal blood volume, with intense systemic and pulmonary vasoconstriction secondary to asphyxia.6 Acidosis, hypoxemia, and anemia should be corrected and intravascular pressures reassessed. Phibbs recommends that exchange transfusion with packed cells be carried out to raise the hematocrit above 35% (Table l).'j These infants are also more susceptible to pulmonary, intracranial, and gastrointestinal hemorrhage. Thrombocytopenia secondary to exchange transfusion may aggravate bleeding diathesis.' Furthermore, bleeding in neonates with erythroblastosis fetalis tends to occur at platelet counts higher than would be expected in other infants. Therefore, platelet counts in these neonates should be kept above 50,000 cu mm by transfusion of platelets if necessary. Hyperbilirubinemia
Once the infant is stable, a double-volume exchange transfusion should be considered. This exchange transfusion will control hyperbilirubinemia
384
Table 1. Pressure Management Guidelines for Neonates with Erythroblastosis Fetalid Central Blood Pressure
Central Venous Pressure
Low
Normal or low
Normal Low High
Normal Low High
Management Series of small transfusions of packed red blood cells Exchange transfusion Volume expansion Exchange transfusion of packed red blood cells
Exact levels are not reported because normal levels vary with gestational age and weight of the infant.
by washing out bilirubin and antibody-coated cells and will increase serum bilirubin binding capacity by raising the plasma protein concentration. An increase in plasma oncotic pressure will follow exchange transfusion, and thereby, decrease edema through initiation of diuresis. Salt-poor albumin should be avoided as a volume expander, as albumin leaks rapidly from the circulation and may actually prolong edema in tissues and in the lungs. When vascular compartments are stable, furosemide (1 mg/kg) may be used with caution to evoke a diuresis not initiated by volume expanders.'jS8 The course of hyperbilirubinemia tends to be highly erratic in infants who were transfused in utero via an intravascular technique such as percutaneous umbilical blood sampling. According to the authors' experiences, some initial bilirubin levels at the time of birth have been over 6 mg/dl at birth. However, not all of these infants have required exchange transfusions, which may be attributed to the immediate and aggressive use of phototherapy at the authors' institution. Phototherapy alters the structure of the bilirubin molecule in light-exposed tissues so that this bilirubin can be excreted by the kidneys or liver.g At the authors' institution two or three phototherapy units, (blue spectrum) are used to increase radiant density. Use of multiple phototherapy units is done on an empirical basis to achieve optimal photo-illumination over maximal surface area. According to the authors' clinical observations, infants who were transfused in utero by an intravascular technique require less exchange and booster transfusions. However, anemia at 6 to 8 weeks of age is more common because of the prolonged persistence of maternal antibodies as exchange transfusion can assist in the clearance of these antibodies."*" Infants with erythroblastosis fetalis also need to be observed frequently by chemistry dip strip for hypoglycemia. Hypoglycemia is a further complication of infusion of glucose or exchange transfusion. Other
November/Decernber 1988 JOCNN
metabolic problems could occur and blood electrolytes, calcium, and phosphorus levels should be assessed periodically. NURSING IMPLICATIONS
Caring for the infant with erythroblastosis fetalis presents a tremendous challenge to the neonatal nurse. The goal of nursing management is to identify the infants at risk and intervene before further central nervous system sequelae develop. At the authors' institution, all infants with known hemolytic disease are admitted to the intensive care nursery. Nursing of these infants includes the following interventions: 0
0
0
0
0
0
Obtain a history. Information can be obtained from multiple sources including the perinatal and delivery room records, delivery room nurses and physicians, and the parents. Pay special attention to the details of the obstetric history and outcomes of previous pregnancies. If the fetus was transfused in utero, the data base should include the date of the last transfusion, interval between transfusions, and fetal blood studies including hematocrit and blood type. Perform a physical assessment. Auscultate the lungs noting any abnormal breath sounds. Observe for any edema and palpate the liver for size. Of particular importance in neonates who received in utero transfusions is to note the condition of the cord for any obvious thrombus o r hematoma. Collaboratively monitor vascular pressures, laboratory tests, and chemistry strip glucose levels. Any deviations from normal pressure readings should be reported to the physician. Bilirubin and glucose levels in neonates with erythroblastosis fetalis should be tested approximately every four to six hours until stable. Initiate phototherapy as ordered. As much of the skin surface as possible should be exposed to the photo therapy lights, and t h e neonate's eyes should be covered with opaque patches. All infants under blue phototherapy lights should be monitored as lights of the blue spectrum may mimic cyanosis.'2 Maintain nothing-by-mouth (NPO) status as ordered. Neonates are initially NPO in anticipation of exchange transfusion as regurgitation and aspiration are possible during the procedure. Breastfeeding is permitted when the infant is stable. In the interim. the nurse can Drovide information to the mother about maintaining milk supply by explaining use of a breast pump." Provide emotional support as indicated. Parental anxiety and an alteration in parenting process are expected reactions. Intense emotional responses
November/December 1988 JOCNN
can be expected from the parents of a newborn who was treated in utero even when the infant may not be critically ill. Some of the shock can be cushioned by providing a tour of the intensive care nursery before delivery. All parents are encouraged to see and touch their newborns in the delivery room if possible. Parents have unlimited visiting privileges in the intensive care nursery, and frequent contact is encouraged. Stressing the neonate's progress and positive features can help facilitate the bonding process between the parents and their newborn. SUMMARY
Perinatal management of the neonate with erythroblastosis fetalis is based upon the prenatal detection and treatment of fetal anemia. Postnatal management focuses on treatment of anemia and jaundice, and their sequelae. Prenatal treatment consists of in utero intravascular transfusions. Postnatal treatment consists of respiratory support, management of hemodynamic alterations, and the aggressive treatment of hyperbilirubinemia to prevent kernicterus. Nursing interventions include careful observation and assessment of the overall condition of the neonate, lung sounds, intravascular pressure measurements, and laboratory data as well as the anticipation of needs such as blood for transfusion, medications, and parental support. A cohesive, interdisciplinary approach is needed when caring for the neonate with erythroblastosis fetalis. REFERENCES 1. Queenan, J. 1987. Erythroblastosis fetalis. In Neonatalperinatal medicine:Diseases of the fetus and infant,ed. A. Fanaroff and R. Martin, 53-62. St Louis: C.V. Mosby. 2. Caine, M., and E. Mueller-Heubach. 1986. Kell sensitization in pregnancy. Am J Obstet Gynecol. 85-90. 3. Ludomirski, A., and S. Weiner. Percutaneous fetal umbilical blood sampling. Clin Obstet Gynecol. 4. Warsof, S., K. Nicolaides, and C. Rodeck. 1986. Immune and non-immune hydrops. Clin Obstet Gynecol. 29:53342. 5. Maisels, M.J. 1985. Hyperbilirubemia. In Current therapy in neonatal-perinatal medicine 1985-1986, ed. N. Nelson, 213-17. Ontario: B.C. Decker. 6. Phibbs, R. 1985. Hydrops. In Current therapy in neonatal-perinatal medicine 1985-1986, ed. N. Nelson, 201-06. Ontario: B.C. Decker. 7. Duara, s., and W.W. Fox. 1986. Persistent pulmonary hypertension. In Neonatal pulmonary care, ed. D.W. Thiebeault and G.A. Gregory, 461-82. New York Appleton-Century-Crofts. 8. Oski, F.A., and J.L. Naiman. 1982. Erythroblastosis fetalis. In Major problems in clinical pediatrics: Hemato-
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logic problems in the newborn. Philadelphia: W.B. Saunders. McDonagh, A. 1983. Molecular mechanisms of phototherapy in neonatal jaundice. In Neonatal jaundice, ed. F. Rubaltelli and G. Jori, 173. New York Plenum Press. Ludomirski, A., V.K. Bhutani, S. Weiner, and R.J. Bologanese. 1987. Fetal intravascular transfusion in the management of Rh isoimmunization. Am J Perinatol. 4:379. Bhutani, V., A. Ludomirski, J. Gerdes, and S. Weiner. 1988. Effects of prenatal intravascular transfusion on Rh isoimmunized neonates. Pediatr Res. Abstract in press. Doenges, M., J. Kenty, and M. Moorhouse. 1988. Maternal/newborn care plans: Guidelines f o r client care, 514-22. Philadelphia: F.A. Davis.
Address for correspondence: Patricia A. Dunn, 2800 Hargrave Street, Philadelphia, PA 19136.
Patricia A. Dunn is a clinical nurse specialist at Thomas Jefferson University Hospital in Philadelphia, Pennsylvania. Ms. Dunn is a member of NAACOG, Sigma Theta Tau, and the Pennsylvania Perinatal Association. Vinod K. Bhutani is an assistant professor of pediatrics at the University of Pennsylvania and associate director of Newborn Pediatrics at Pennsylvania Hospital in Philadelphia, Pennsylvania. Dr. Bhutani is a member of the American Academy of Pediatrics and the American Physiology Society. Stuart Weiner is the director of the Antenatal Testing Unit at Pennsylvania Hospital and an associate professor of Obstetrics and Gynecology at the University of Pennsylvania School of Medicine in Philadelphia, Pennsylvania. Dr. Weiner is a member of ACOG and the Society of Perinatal Obstetricians. Avi Ludomirski is a perinatologist at Pennsylvania Hospital and an assistant professor of Obstetrics and Gynecology at the University of Pennsylvania School of Medicine in Philadelphia, Pennsylvania. Dr. Ludomirski is a member of the Society of Perinatal Obstetricians.
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NATIONAL OSTEOPOROSIS EDUCATION PROGRAM
The Osteoporosis Education Advisory Board in conjunction with NAACOG, the Organization for Obstetric, Gynecologic, and Neonatal Nurses, offers an osteoporosis education kit, an educational program for nurses and the community. As part of a national program, this kit is designed to provide health-care professionals with a complete educational package on osteoporosis increase awareness of osteoporosis within communities throughout the country encourage women to discuss osteoporosis and appropriate preventive measures with their health-care providers and increase public awareness of the NAACOG nurse as a key source of health-care education in the community. The kit can be used for the development or supplementationof an educational session or program for consumers or nurses. This complete education package includes two, 20-minute slide presentations with accompanying scripts, a publicity guide for implementing a consumer education program in the community, background information on osteoporosis and risk assessment, and program evaluation forms. The kit is available free of charge on a loan basis only. For more information about the program, contact the Department of Education and Research, NAACOG, P.O. Box 71437, Washington, DC 20024-1437; (202)638-0026. Erratum: The above telephone number was incorrect in the March/April issue of JOGNN. The above telephone number is correct.
SEMINAR
The 13th Annual Seminar for Nurse Practitionersin ambulatory gynecology, family planning, and women’s health care will be held February 15-1 7,1989, in Philadelphia, Pennsylvania. The program is sponsored by Planned Parenthood Federation of America, NAACOG, the University of Pennsylvania School of Nursing Center for Continuing Education, and the Family Planning Council of Southeastern Pennsylvania. For further information, contact the Ob/Gyn Nurse Practitioner Program, Suite 1900, 260 South Broad Street, Philadelphia, Pennsylvania 19102; (215) 985-2612, or Planned Parenthood Federation, 810 7th Avenue, New York, NY 10019, (212) 603-4696.
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