Introduction
Spina bifida is referred to as one of the most severe congenital malformations compatible with life. The incidence of spina bifida is estimated at 3.5 per 10,000 live births per year in the United States. Myelomeningocele, or open spina bifida, is the most common and severe form characterized by protrusion of the spinal cord through the open vertebrae into the amniotic fluid. The severity of disability varies in accordance to the level of the neurologic lesion and the extent of the intracranial abnormalities. The malformation is associated with significant lifelong disability including motor and sensory deficits, neurogenic bowel and bladder, hindbrain herniation (the Chiari II malformation) and associated hydrocephalus, orthopedic abnormalities, and cognitive deficits. In addition, children with spina bifida are more likely to have psychosocial adjustment difficulties and report lower physical health-related quality of life than peers. Self-reported satisfaction with family functioning has been shown to be an important factor related to improved quality of life.
Prenatal spina bifida repair was developed with the hope that earlier repair would lessen these disabilities by ameliorating the secondary damage from the chronic mechanical and chemical in utero insults. The early experience of prenatal repair of myelomeningocele, first performed in 1997, showed promising results including the reversal of hindbrain herniation and a decreased need for ventriculoperitoneal shunting. However, prenatal repair was associated with significant maternal morbidity and the risk of fetal or neonatal death and prematurity. From 2003 through 2011, we conducted the Management of Myelomeningocele Study (MOMS), a multicenter randomized controlled trial to compare the efficacy and safety of prenatal repair of myelomeningocele with standard postnatal repair. Trial recruitment was halted in December 2010 when interim analysis demonstrated efficacy of prenatal repair. The prenatal group showed significant decrease in the rate of shunt placement (40% vs 82%) and improved motor outcomes at 30 months of age, including the ability to walk without orthotics (42% vs 21%). Not all fetuses benefited, however, and maternal and fetal risks included preterm rupture of membranes and prematurity.
The call to address the impact of illness and disability on family functioning and parental stress has become stronger and broader. Previous studies have described the familial impact of caring for children with trisomy 13, brain tumors, and congenital diaphragmatic hernia. In general, the presence of spina bifida in families is predictive of increased parental stress and psychological strain. The effect on families depends on the parental relationship, family climate, and support from social networks. Yet, many families also show increased resilience and adaptation. To further understand the impacts of prenatal surgery, beyond the health consequences to the pregnant woman/fetal dyad, women were asked to complete questionnaires to identify impact on families and psychological stress.
In this article we describe the impact on families caring for a child with spina bifida and compare the difference in impact between those who were randomized to prenatal vs postnatal repair. In addition, we explore other factors associated with the impact on families and parental stress. We hypothesize that families will experience less negative impacts and less parental stress at 12 and 30 months after prenatal myelomeningocele repair compared to postnatal repair because of the decreased shunt rate and improved neuromotor function.
Materials and Methods
MOMS was conducted at 3 maternal-fetal surgery centers: Children’s Hospital of Philadelphia, Vanderbilt University, and the University of California–San Francisco in collaboration with an independent data-coordinating center at George Washington University and with the Eunice Kennedy Shriver National Institute of Child Health and Human Development. The trial was approved by the institutional review board at each center. Details of the trial design and proceedings were previously described. Briefly, pregnant women between 19-25 weeks’ gestation with a fetus diagnosed with myelomeningocele who met eligibility criteria were randomly assigned to either prenatal or postnatal surgery. All women randomized were planned to deliver at 37 weeks of gestation at the MOMS center where randomized, if not already delivered. (Those randomized to prenatal surgery remained nearby while those randomized to postnatal surgery went home and returned.) Families returned to the maternal–fetal surgery centers for follow-up of the children at 12 and 30 months of age at which time the objective measures were completed.
The primary outcome of interest was the impact on families caring for a child with spina bifida. The mother or the primary caregiver of the affected child completed the Impact on Family Scale (IFS) at 12 and 30 months after birth. The original IFS was developed by Drs Stein and Riessman to measure the impacts of chronic childhood illnesses on families. The measure consists of 24-items that assess impact in 4 domains: financial burden, social interactions within and outside the home, personal strain/distress felt by the parent, and sense of a lack of mastery resulting from stress. Response categories for each item include: strongly agree, agree, disagree, and strongly disagree. Items are scored 1-4 to compute 4 domain scores. Higher scores are reflective of a more negative impact in the particular domain. Since the development of the original scale, additional validation studies support the use of 15 of the original questions to assess the overall social and familial impacts of childhood illness. Therefore, we also computed a revised 15-item IFS (RIFS) score describing the overall impact. Scores for the RIFS ranged from 15-60, with higher scores indicating more negative impact.
The mother or primary caregiver also completed the Parenting Stress Index (PSI)-Short Form (SF). The PSI-SF is a 36-item measure taken directly from the PSI full-length test developed by Dr Abidin in the early 1980s. The SF has 3 subscales: parental distress, parent-child dysfunctional interaction, and difficult child behavioral characteristics. In addition, a total stress score is computed to indicate the overall level of parenting stress the participant is experiencing. Response categories for each item include: strongly agree, agree, not sure, disagree, and strongly disagree. Items are scored 1-5 with composite scores ranging from 36-180. Higher scores reflect greater stress. In addition to the 3 subscales and the total stress score, a defensive responding scale can be calculated. This scale assesses the extent to which the parent is simply trying to answer the items in a socially desirable way. A low score indicates responding in a defensive manner.
In addition to the IFS and PSI-SF, participants also completed a Family Support Scale (FSS) and a Family Resource Scale (FRS). The FSS is an 18-item measure that assesses perceived helpfulness of different social resources. The FSS assesses family support in the following areas: informal kinship, social organizations, formal kinship, nuclear family, specialized professional services, and generic professional services. Response categories to measure the degree of helpfulness include: not at all helpful, sometimes helpful, generally helpful, very helpful, and extremely helpful. The FRS is a 30-item measure that assesses perceived family resources in 4 domains: basic needs, money, time for self, and time for family. Responses are rated on a 5-point Likert scale: not at all adequate, seldom adequate, sometimes adequate, usually adequate, and almost always adequate.
Demographic and socioeconomic covariates used in this analysis include: maternal age at screening, years of schooling completed, number of live births prior to MOMS pregnancy, and household income at randomization. Fetal/neonatal covariates used include: sex, spina bifida lesion level on ultrasound, whether the child required a shunt by 12 months of age, and walking status at 30 months.
Baseline and other covariates were compared by univariable analysis; continuous variables were compared with the Wilcoxon test and categorical variables were compared with the χ 2 or Fisher exact test as appropriate. The outcomes (IFS and PSI-SF) obtained at 12 and 30 months were analyzed together, as repeated measures, using generalized estimating equations to account for the correlation between time points. For the RIFS and total PSI, generalized linear models were used in a multivariable analysis adjusting for treatment group, maternal age at screening, years of schooling completed, number of live births prior to MOMS pregnancy, and household income at randomization, FSS, and FRS. For all tests a nominal P value <.05 was considered to indicate statistical significance. No adjustment was made for multiple comparisons.
Materials and Methods
MOMS was conducted at 3 maternal-fetal surgery centers: Children’s Hospital of Philadelphia, Vanderbilt University, and the University of California–San Francisco in collaboration with an independent data-coordinating center at George Washington University and with the Eunice Kennedy Shriver National Institute of Child Health and Human Development. The trial was approved by the institutional review board at each center. Details of the trial design and proceedings were previously described. Briefly, pregnant women between 19-25 weeks’ gestation with a fetus diagnosed with myelomeningocele who met eligibility criteria were randomly assigned to either prenatal or postnatal surgery. All women randomized were planned to deliver at 37 weeks of gestation at the MOMS center where randomized, if not already delivered. (Those randomized to prenatal surgery remained nearby while those randomized to postnatal surgery went home and returned.) Families returned to the maternal–fetal surgery centers for follow-up of the children at 12 and 30 months of age at which time the objective measures were completed.
The primary outcome of interest was the impact on families caring for a child with spina bifida. The mother or the primary caregiver of the affected child completed the Impact on Family Scale (IFS) at 12 and 30 months after birth. The original IFS was developed by Drs Stein and Riessman to measure the impacts of chronic childhood illnesses on families. The measure consists of 24-items that assess impact in 4 domains: financial burden, social interactions within and outside the home, personal strain/distress felt by the parent, and sense of a lack of mastery resulting from stress. Response categories for each item include: strongly agree, agree, disagree, and strongly disagree. Items are scored 1-4 to compute 4 domain scores. Higher scores are reflective of a more negative impact in the particular domain. Since the development of the original scale, additional validation studies support the use of 15 of the original questions to assess the overall social and familial impacts of childhood illness. Therefore, we also computed a revised 15-item IFS (RIFS) score describing the overall impact. Scores for the RIFS ranged from 15-60, with higher scores indicating more negative impact.
The mother or primary caregiver also completed the Parenting Stress Index (PSI)-Short Form (SF). The PSI-SF is a 36-item measure taken directly from the PSI full-length test developed by Dr Abidin in the early 1980s. The SF has 3 subscales: parental distress, parent-child dysfunctional interaction, and difficult child behavioral characteristics. In addition, a total stress score is computed to indicate the overall level of parenting stress the participant is experiencing. Response categories for each item include: strongly agree, agree, not sure, disagree, and strongly disagree. Items are scored 1-5 with composite scores ranging from 36-180. Higher scores reflect greater stress. In addition to the 3 subscales and the total stress score, a defensive responding scale can be calculated. This scale assesses the extent to which the parent is simply trying to answer the items in a socially desirable way. A low score indicates responding in a defensive manner.
In addition to the IFS and PSI-SF, participants also completed a Family Support Scale (FSS) and a Family Resource Scale (FRS). The FSS is an 18-item measure that assesses perceived helpfulness of different social resources. The FSS assesses family support in the following areas: informal kinship, social organizations, formal kinship, nuclear family, specialized professional services, and generic professional services. Response categories to measure the degree of helpfulness include: not at all helpful, sometimes helpful, generally helpful, very helpful, and extremely helpful. The FRS is a 30-item measure that assesses perceived family resources in 4 domains: basic needs, money, time for self, and time for family. Responses are rated on a 5-point Likert scale: not at all adequate, seldom adequate, sometimes adequate, usually adequate, and almost always adequate.
Demographic and socioeconomic covariates used in this analysis include: maternal age at screening, years of schooling completed, number of live births prior to MOMS pregnancy, and household income at randomization. Fetal/neonatal covariates used include: sex, spina bifida lesion level on ultrasound, whether the child required a shunt by 12 months of age, and walking status at 30 months.
Baseline and other covariates were compared by univariable analysis; continuous variables were compared with the Wilcoxon test and categorical variables were compared with the χ 2 or Fisher exact test as appropriate. The outcomes (IFS and PSI-SF) obtained at 12 and 30 months were analyzed together, as repeated measures, using generalized estimating equations to account for the correlation between time points. For the RIFS and total PSI, generalized linear models were used in a multivariable analysis adjusting for treatment group, maternal age at screening, years of schooling completed, number of live births prior to MOMS pregnancy, and household income at randomization, FSS, and FRS. For all tests a nominal P value <.05 was considered to indicate statistical significance. No adjustment was made for multiple comparisons.
Results
A total of 183 patients were randomized, 91 in the prenatal surgery group and 92 in the postnatal surgery group ( Figure ). The baseline covariates of the cohort are presented in Table 1 . There were no differences between the surgery groups except for fetal sex (46% female in the prenatal surgery group vs 62% in the postnatal surgery group; P = .03). There were no differences in FSS or FRS scores at 12 months between the surgery groups ( Table 2 ).
