The experience of parents with children with myelomeningocele who underwent prenatal surgery

1.1 Prenatal diagnosis of myelomeningocele

Myelomeningocele (MMC), or open spina bifida, represents the first non-lethal prenatal diagnosis that can be managed with maternal-fetal (MF) surgery. MMC is characterized by protrusion of the spinal cord through the open vertebrae into the amniotic fluid, resulting in lifelong disability varying in severity depending on the level of the neurologic lesion and the extent of the intracranial abnormalities [1]. The malformation is typically associated with neurogenic bowel and bladder, hindbrain herniation (Chiari II malformation), hydrocephalus, orthopedic abnormalities, as well as motor, sensory, and cognitive deficits. In addition, children with MMC are more likely to have psychosocial adjustment difficulties and report lower physical health-related quality of life than their peers [2].

Prenatal diagnosis of MMC leads to challenging decisions. In the past, and still in many developed countries, the only options were postnatal closure or termination of pregnancy. Now, surgeons can perform MF surgery and close the lesion prenatally. Open MF surgery aims to mitigate damage from chronic chemical and mechanical insults in utero [3]. The early experience of prenatal repair of MMC, first performed in 1997, showed promising results including the reversal of hindbrain herniation and a decreased need for ventriculoperitoneal shunting [4, 5, 6, 7, 8]. However, prenatal repair is associated with significant risk of maternal morbidity, fetal or neonatal death, and prematurity [5, 6, 7, 8]. In 2003, the NIH sponsored a multi-centered randomized controlled trial to test the efficacy of this treatment [9]. The Management of Myelomeningocele Study (MOMS) showed statistically significant benefits from the procedure. Prenatal closure reduced the need for shunting and improved motor outcomes at 30 months of age.

1.2 Impact of maternal-fetal surgery

However, MF surgery poses risks to the pregnant woman and fetus alike, and requires a significant emotional, physical, and financial commitment from the pregnant woman and her entire family. Pregnant women who undergo prenatal surgery will need to take time away from work both before and after birth. Common complications include pre-term labor and chorioamniotic membrane separation, which might require tocolytic therapy and/or bedrest. Given that only one-third of fetal care centers currently perform open MF surgery [10], many women must travel a great distance and relocate for months. This can place significant burdens on the pregnant woman and her family including her spouse or partner, other children, and extended family. Despite these significant sacrifices, a select group of pregnant women and their partners chose to undergo MF surgery hoping to improve their child’s outcomes. Critics of MF surgery for MMC argue that women who are counseled about prenatal surgery are vulnerable and susceptible to therapeutic misconception. In addition, their decision making may be compromised by scientifically unsubstantiated pressures from physicians, family, or society [11, 12].

There is a growing emphasis on addressing the familial impacts of severe childhood illness and disability [13]. Previous studies have described the familial impact of caring for children with Trisomy 13 [14, 15, 16], brain tumors [17], and congenital diaphragmatic hernia [18]. In particular, caring for a child with MMC can lead to increased parental stress and psychological strain [19, 20]. The effect on families depends on the parental relationship, family dynamics, and support from social networks [20]. Many families with a child who has MMC show incredible resilience and adaptation [2].

This study sought to investigate the perspective of parents who chose MF surgery, specifically regarding the information they received, their choices, and the impacts of these decisions on their family.

2.1 Participants

In the spring of 2016, parents who were members of a MF surgery social media group on a common online platform were sent a group message inviting them to participate in an online survey of their experiences. Parents who were over 18 years of age, could read and write in English, had already undergone MF surgery, and were not participants in the MOMS trial were eligible for inclusion in this study. Parents who accepted the invitation were sent a link to a 22-item self-completion questionnaire. They were also able to request that a paper version be mailed to them if preferred. The first page of the questionnaire informed respondents about the nature of the study and indicated that completing the survey implied consent to participate. This study was approved by the Children’s Hospital of Philadelphia Institutional Review Board.

2.2 Instrument

The survey questions were formulated after reviewing the literature, consulting with content experts, and conducting physician interviews at five maternal-fetal centers. Parental opinions were also solicited; one of the collaborators in this study is a parent who underwent MF surgery for MMC. All respondents were asked 6 multiple-choice, 2 open-ended, and 10 demographic questions. Study data were collected and managed using REDCap (Research Electronic Data Capture, a secure, web-based research application) hosted at the Children’s Hospital of Philadelphia [21].

Parents were asked multiple choice questions about the following: 1) What choices they were offered after the prenatal diagnosis. 2) Whether they felt pressured towards one option (prenatal surgery, postnatal surgery, or termination). 3) How they learned about prenatal surgery. 4) “Knowing what you know now, if you were able to go back in time, would you still undergo prenatal surgery”? 5) “Knowing what you know now, what would you tell another couple considering prenatal surgery for MMC”? 6) The impacts of MF MMC surgery on their family (as a couple, other children, grandparents, etc.). Response categories included: “it has had a positive impact on my family”, “it has had a negative impact on my family”, and “it has had both a positive and a negative impact on my family”. Depending on their answer to this question, parents were then asked the following one or two open-ended questions: “Please tell us up to three positive (and/or negative) impacts of having a child with MMC.”

Demographic questions included: age, sex, race, marital status, education, employment status, and total household income. Parents were also asked about the year they or their partner underwent prenatal surgery, what additional surgeries their child has required (i.e., placement of a shunt, revision of a shunt, surgery for a tethered cord, urological surgery, or orthopedic surgery), and whether they had other children at home when their child was diagnosed with MMC.

This study utilized mixed methodology. Descriptive statistics were used to report quantitative data using Stata statistical software, version 13.1 (StataCorp, College Station, TX). The open-ended questions were analyzed using thematic analysis [22, 23, 24]. The themes and sub-themes were developed by three researchers (RA, AH, AJ). Specifically, using a thematic qualitative content analysis approach, themes and coding definitions were developed based on the analysis of 30 respondent answers, after which we had achieved coding and thematic saturation [24]. The coding was then simultaneously done by two coders (RA and AH) to carefully and thoroughly define themes and sub-themes using Microsoft Excel, 2015 (Redmond, WA). Coding was compared between the two coding researchers and discrepancies were corrected by consensus.

3.1 Prenatal counseling

Parents reported learning about the option of maternal-fetal surgery from multiple sources including: the internet (39%), other health care providers (33%), family (8%), friends (7%), magazine or newspaper articles (6%), and from television (4%). When their child was first diagnosed with MMC, the majority reported that their obstetrician spoke to them about surgery after birth (84%), maternal-fetal surgery (71%), and termination (80%). Of those who were not counseled about maternal-fetal surgery, 17 of 31 (55%) were diagnosed after the MOMS trial. Twenty-seven (25%) parents reported that when speaking to their physicians about their treatment choices, they felt pressure to take one option. Among this group, 7 felt pressure to have postnatal surgery, 1 felt pressure to have maternal-fetal surgery, and 19 felt pressure to terminate.

3.2 Surgical procedures after MF surgery

Parents reported that their children needed the additional following surgical procedures: shunt placement (34%), shunt revision (for 41% of those who required a shunt), surgery for a tethered cord (28%), orthopedic surgery (28%), and urologic surgery (14%).

3.3 Decision to undergo surgery

Parents were asked whether they would still undergo prenatal surgery if they could travel back in time with their present knowledge. Of the 109 participants, 99 (91%) report that they would definitely undergo surgery again, 5 (5%) report that they would probably still undergo surgery, 3 (3%) were unsure, and 2 (2%) reported that they would probably not. Twenty-five parents (23%) stated that if they were talking to another couple considering prenatal surgery for MMC they would encourage them to undergo maternal-fetal surgery, whereas 84 (77%) report that they would encourage them to consider maternal-fetal surgery but to do whatever was best for them. None of the parents would encourage a couple not to undergo maternal-fetal surgery.

3.4 Family Impact

Half of parents (49%) report that having a child with MMC has had a positive impact on them and their family, while 51% report that having a child with MMC has had both positive and negative impacts. No parent reported that having a child with MMC has had only a negative impact on them and their family.