The Perinatal Hospice - Ploughing the Field of Natal Sorrow
By Byron C. Calhoun, M.D., and Nathan Hoeldtke, M.D.
Case Presentation
Ms. Johnson came to Madigan Army Medical Center (MAMC) as a referral patient for a possible fetus with short limbs. She arrived from her base as a 19-year-old dependent wife at 26 weeks gestation and subsequently underwent diagnostic ultrasound in our unit.
The measurements of the fetal long bones all demonstrated a baby with values well below 5 standard deviations from the mean and diagnostic of congenital dwarfism. Further, the child's head size was above 3 standard deviations with a small chest. In fact, the child's chest was curved inward compared to the abdomen and was gravely concerning for a presumptive diagnosis of thanatorphic dwarfism with probable maldevelopment of the lungs. This diagnosis is uniformly fatal with lack of lung function due to compression of the chest. Ms. Johnson was given her various options to include pregnancy termination and perinatal hospice. Ms. Johnson chose to continue her care at MAMC. Her pregnancy lasted until 36 weeks when she presented in active labor. The fetus had continued to show limb growth lag and significant chest wall compression. The baby delivered vaginally without difficulty and lived for a few minutes until he expired from respiratory failure. Ms. Johnson and her husband were able to spend precious time with their newborn and have closure with him. Subsequently Ms. Johnson wrote a moving letter to our unit thanking us for our care and "allowing her to choose life."
Introduction
Congenital anomalies now account for the majority of the causes of death in the first year of life. [Centers for Disease Control. 1994. Infant Mortality - United States, 1992. MMWR 905-909.] Many lethal anomalies may now be diagnosed antenatally including anencephaly, bilateral renal agenesis or acrania. Prenatal diagnostic capabilities continue to accelerate while thinking regarding hospice care for terminal perinates has lagged. We propose renewed thinking about how to approach a fetus that will die in utero or live a short time ex utero. We describe our method of approaching the perinatal hospice at MAMC under the perinatal hospice concept since July 1995.
Hospice Care for Adults and Newborns
MAMC provides tertiary and referral obstetrical/gynecologic care in the Department of Defense for TRICARE Region IV. There are 24 OB/GYN residents, three maternal-fetal medicine fellows and 15 staff. Military medicine provides a unique environment for obstetrics/gynecology and neonatology - federal law restricts the provision of abortion services except where the life of the mother is at risk. Thus, infants with lethal congenital anomalies are frequently delivered at our facility. We have developed the concept of the perinatal hospice from our experience with these patients and their families.
Adult hospice care began in the '60s in Great Britain by Cicely Saunders when she realized that end-of-life issues for terminally ill patients were not being addressed in a coherent, thoughtful and supportive environment. [Saunders, C. March 1965. The last states of life. American Journal of Nursing, 65:70-75, and Saunders, C. December 1966. Terminal patient care. Geriatrics, 21:70-74.] The hospice idea was particularly developed to provide nursing and medical care in an environment which addressed the common fear of pain and abandonment. [Ferrell, B., Grant, M., Padilla, G., Vermuri, S., Rhiner, M. 1991. The experience of pain and perceptions of quality of life: Validation of a conceptual mode. Hospice Journal, 7(3):9-24. Ferrell, B.R., Ferrell, B.A., Rhiner, M., Grant, M. 1991. Family factors influencing cancer pain management. Postgrad Medical Journal, 67 Supplement 2:S64-S69. Muzzin, L.J., Anderson, N.J., Figuerdo, A.T., Gudelis, S.O. 1994. The experience of cancer. Social Science and Medicine, 38(9):1201-1208. Vincent, V.A., Edwards, J.G., Young, S.R., Nachtigal, M. 1991. Pregnancy termination because of chromosomal abnormalities: a study of 26,950 amniocenteses in the southeast. Southern Medical Journal, 84(10):1210-1213.]
By the '70s and '80s, hospice care became increasingly available as an alternative. Saunders' work laid the groundwork for awareness of end-of-life issues, and hospice care became the focus of research and improved methods of care delivery. The hospice concept developed further in the treatment of the terminally ill child. [Saunders, C. June 1969. The management of fatal illness in childhood. Pro Royal Soc Medicine, 62:550-553.] Due to this work, Whitfield, et al, further refined Saunders' work with the implementation of the neonatal hospice concept at the Children's Hospital in Denver to support families of dying infants. [Whitfield, J.M., Siegel, R.E., Glicken, A.D., Harmnon, R.J., Powers, L.K., Goldson, E.J. 1982. The application of hospice concepts to neonatal care. American Journal of Disabled Children, 136: 421-424.]
A New Concept - The Perinatal Hospice
The neonatal hospice construct of care is no longer sufficient for our families' needs. Because of the increasingly common scenarios of the prenatal diagnosis of a lethal congenital anomaly, the process of providing care for a grieving family no longer begins at birth, but at the time of diagnosis. We, therefore, utilize a concept of perinatal hospice that allows for a continuum of supportive care from the time of diagnosis until the infant's death. The availability of perinatal hospice becomes important during prenatal counseling of families.
Upon prenatal diagnosis of a lethal anomaly, options for the parents include termination of the pregnancy versus the delivery of the fetus. Historically, the discussion often focused on what the delivery would be like, whether or not to resuscitate the infant, and, if so, what should be the level of care provided to the infant. Thought of their decision may leave parents with the perceived dilemma of watching/allowing their infant to die versus an effort to futilely prolonging life which may lead to perceived increased suffering for their child, which may lead parents to view this "presentation" of options as tacit reasoning for terminating the pregnancy. Their decision may also be colored by the common fear of abandonment of themselves and their unborn child and the pain and suffering both may endure. [Kuleshov, N. P. Chromosome anomalies of infants dying during the perinatal period and premature newborn. Human Genetics, 31:151:60, 1979.] It is in this situation that an emphasis on perinatal hospice support throughout the pregnancy and the infant's life allows the parents full autonomy in coming to the best decision for their family.
Studies demonstrate that at least 20 percent of patients choose to deliver their children with known severe chromosomal or anatomic anomalies. [Vincent, V.A., Edwards, J.G., Young, S.R., Nachtigal, M. 1991. Pregnancy termination because of chromosomal abnormalities: a study of 26,950 amniocenteses in the southeast. Southern Medical Journal, 84(10):1210-1213. Verps, M.S., Bombard, A.T., Simpson, J.L., et al. 1988. Parental decision following prenatal diagnosis of fetal chromosome abnormality. American Journal of Medical Genetics, 29:613-622. Drugan, A., Greb, A., Johnson, M.P., Krivchenia, E.L., Uhlman, W.R., Moghissi, K.S., Evans, M.I. August 1990. Determinants of parental decisions to abort for chromosome abnormalities. Prenatal Diagnostic, 10(8): 483-490.] If we consider that roughly 0.5 percent to 1 percent (30,000 to 50,000 a year) of all live births have defects severe enough to cause fetal death, approximately 6,000 to 10,000 patients a year are possible candidates for perinatal hospice. [Kuleshov, N.P. Chromosome anomalies of infants dying during the perinatal period and premature newborn. Human Genetics, 31:151:60, 1979.] Patients who choose to deliver their infants rather than terminate the pregnancy had a perceived less severe anomaly on ultrasound or genetic amniocentesis. [Vincent, V.A., Edwards, J.G., Young, S.R., Nachtigal, M. 1991. Pregnancy termination because of chromosomal abnormalities: a study of 26,950 amniocenteses in the southeast. Southern Medical Journal, 84(10):1210-1213. Drugan, A., Greb, A., Johnson, M.P., Krivchenia, E.L., Uhlman, W.R., Moghissi, K.S., Evans, M.I. August 1990. Determinants of parental decisions to abort for chromosome abnormalities. Prenatal Diagnostic, 10(8): 483-490.] Perinatal hospice care provides these patients and their children a compassionate, supportive and desired alternative.
Antepartum Care
The burden of effort in perinatal hospice resides in the antepartum counseling and preparation. Patients need to see the baby on ultrasound and be allowed to grieve. Most birth defects are not as gruesome in appearance at birth as patients imagine.
We believe it essential to our management of these difficult pregnancies that we solicit input from the various services that will be involved in the terminal care of the infant. In concert with hospice ideals, this allows for open communications and prevents derailment of carefully crafted care plans by lack of information. In our hands, the perinatal hospice includes the combined efforts of the maternal-fetal medicine subspecialists, obstetricians, neonatologists, anesthesia services, chaplains/pastors/social work services, labor and delivery nurses and neonatal intensive care nurses. We participate in the ultrasound evaluation, amniocentesis if desired, birth planning and ongoing medical management in the antepartum, intrapartum and postpartum periods. Patients are given the fetal diagnosis and the expected prognosis during extensive time with the maternal-fetal medicine and neonatology staff. Patients are allowed to grieve, explore life issues and prepare for the precious time they may be allowed to spend with their special child.
Intrapartum Care
Extensive support is also provided in labor through encouragement by the nursing staff, and pain relief is administered by the anesthesia service. Labor management is conducted as other labors with the exception of fetal heart rate monitoring in lethal fetal conditions, including anencephaly, trisomy 13 or trisomy 18 where an abnormal fetal heart pattern is expected. [Garite, T.J., Linzey, E.M., Freeman, R.K., Dorchester, W. Fetal heart patterns and fetal distress in fetuses with congenital anomalies. OB/GYN, 53:716-720, 1979.] Patients and staff find ignoring an abnormal heart tracing difficult. Fetuses with conditions not expected to be lethal, such as Down or Turner's Syndrome, are managed with fetal heart rate monitoring in the same fashion as other labors.
When the time of delivery approaches, nursing staff who will be involved with the parents and the infant become increasingly involved in support and planning of delivery and postpartum care. The early and consistent involvement of the staff with the family prevents the "withdrawal" of care Elizabeth Kubler-Ross so elegantly described in her work On Death and Dying. [Kubler-Ross, E. 1969. On Death and Dying. New York: Macmillan Publishing Co.]
Postpartum Care
Method of delivery is based on obstetrical indications and the infant is handed immediately to the parents to share in the baby's life or death. Many of these infants are stillborn, but some live for minutes or days.
The parents are allowed to stay in the delivery suite with the child as long as they wish. We encourage dressing the baby, taking pictures of the baby and holding the baby by all family members, including children if appropriate. Non-anomalous features of the baby are emphasized to the parents. Descriptions of cute hands and/or soft skin give the parents a positive focus for their child's life and death.
Neonatologists and nursing personnel comfort the baby as needed. The infant is kept warm and cuddled. Some of these babies even feed. Those infants who survive for longer periods may be kept comfortable in the nursery during the postpartum period, if the patients are feeling overwhelmed. Comfort measures are emphasized to the family. Our chaplain service and social service provide key spiritual and emotional support as needed.
Conclusions
This supportive environment has been offered in our antenatal service for several years. Parents, when given loving support, freedom from abandonment and careful counsel as to clinical expectations, will choose the alternative of perinatal hospice, however brief that time may be. This decision is facilitated by our patients having access to comprehensive health care benefits in the Department of Defense. However, none of the delivery or prenatal care expenses would be obviated by termination. Delivery of the fetus is still necessitated. Other academic and private practice settings could arrange to ensure financial concerns are not prohibitive. Physicians and hospitals must be willing to waive or adjust fees, help set up payment plans and assist in the establishment of a perinatal hospice.
Parental responses have been overwhelmingly positive. These parents are allowed the bitter-sweetness of their child's birth and too-soon departure. Grief lessens as time passes and parents rest secure in the knowledge that they shared in their baby's life and treated the child with the same dignity as a terminally ill adult.
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Byron C. Calhoun, M.D., FACOG, FACS, is program director of the Maternal-Fetal Medicine Program, as well as the director of the Antenatal Diagnostic Center, at Madigan Army Medical Center in Tacoma, Wash.
Nathan Hoeldtke, M.D., FACOG, works in Maternal-Fetal Medicine at Tripler Army Medical Center in Honolulu, Hi .