Abstract
BACKGROUND:
Prenatal surgery for myelomeningocele (MMC) has been demonstrated to have benefits over postnatal surgery. Nevertheless, prenatal surgery requires a significant emotional, physical, and financial commitment from the entire family.
METHODS:
Mixed methods study of parents’ perceptions regarding provider communication, treatment choices, and the family impact of having a child with MMC.
RESULTS:
Parents of children with MMC (
CONCLUSIONS:
A better understanding of the parental experiences and perspectives following prenatal surgery will play an important role in providing overall support for parents and family members.
Keywords
Background
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.
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.
Methods
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.
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.
Characteristics of the sample
Characteristics of the sample
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.
Results
Of those in the social media group, 109 parents completed the questionnaire (88
females, 6 males, and 15 unknown). The majority of parents (78%) reported that their child
underwent maternal-fetal surgery after the MOMS trial enrollment (2003–2010) and 22% prior
to the trial. The average reported time since surgery was 5.4 years (SD
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.
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%).
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.
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.
Positive family impact
Ninety-seven parents reported specific positive impacts. Five themes were expressed by parents to the request: “Please tell us up to three positive impacts of having a child with MMC”.
Theme 1: Outlook on life, transformation, and gratitude
The most common positive impact reported regarded the parent’s
attitude (
“She has taught us to look at life differently.”
“Has changed our perspective on what is important.”
“Opened my eyes and heart to a bigger, wider, more beautiful and meaningful world.”
“More accepting and appreciative of disabled people as a whole.”
Thirty-five of the 71 parents reported personal growth or transformation.
“It has made us more compassionate, empathetic, accepting.”
“I am a better person because of him, because of our journey. I am so many ‘things’ I never thought I’d be. An advocate, an educator, a nurse, a therapist, the list goes on.”
“We learned more about a disability that we didn’t know a lot about.”
Finally, 14 of the 71 parents reported increased gratitude after having a child with MMC.
“We appreciate milestones so much more. Some things people take for granted, we count as blessings.”
“Makes us grateful for the little things in life.”
“Helped us learn to be thankful for every gift, milestone, and moment with our kids.”
Theme 2: New horizons and new community
The second most commonly reported positive impact
(
“We have been all over the country for Spina Bifida Foundation Reunions and Spina Bifida Association Conferences which led to wonderful friendships.”
“My son has become quite adept at adaptive sports and we travel to places around the country we would never have gone otherwise.”
“It’s brought wonderful people into our lives who wouldn’t have been there had it not been for Spina Bifida.”
“We’re in contact with a lot of great doctors and medical staff we would never have had a chance to meet.”
Theme 3: Family functioning
Next, 32 parents reported that having a child with MMC has had a positive impact on their family functioning: strengthened marriage/relationship as well as benefits to siblings and extended family.
“It has brought my husband and I closer together.”
“We can teach our other children to be more compassionate to people with disabilities.”
“Everything we went through brought my family closer together.”
“Found how supportive extended family is.”
Theme 4: Child strengths
Parents also focused on their child’s strengths
(
“She has shown us what it truly means to be a fighter.”
“Being incredibly proud at how hard my daughter works to be able to do things most people take for granted.”
“I have an amazingly brave, persevering, beautiful, loving, kind, empathetic, kind- hearted teenager.”
“Using a wheel chair won’t make her any less important and driven than she already is.”
Theme 5: Spiritual enrichment
A minority of parents (
“It has taught me to rely on faith for everything.”
“It has helped deepen our faith and reliance on God.”
“Above all we have seen God in her and deeply believe that he has a path for us to follow no matter where it may lead.”
Negative family impact
Forty-four of the 109 parents reported specific negative impacts in addition to positive impacts. There were four major themes that emerged from the thematic analysis of parental responses to the question, “Please tell us up to three negative impacts of having a child with MMC.”
Theme 1: Functional impact and medical burden
The most common negative impact reported (
“There are things that are just difficult for a person with a disability, so it limits the family’s options. For instance, taking a wheelchair on a beach is tough, so we rarely go to the beach as a family. Bathroom needs for a person with a disability are different, so we have to plan day trips around catheterization schedules.”
“The bathroom routine is lengthy. Worries about school accidents.”
“It is difficult to watch your child lag behind in gross motor skills when others around them are far surpassing them.”
Other parents (
“Three surgeries after fetal surgery for our daughter.”
“Managing all the doc appointments and surgeries too.”
“Navigating equipment and therapy needs.”
Theme 2: Strain on the family
The second most common theme (
“Divorce - my child’s father couldn’t handle the life of SB.”
“It adds stress to our marriage regarding treatment and therapies.”
“There are always challenges on vacations with extended family that they don’t recognize or understand.”
Many parents also reported financial difficulties, occupational
strain, or loss of work (
“Medical bills and insurance paperwork are a constant challenge.”
“It’s expensive to have a child with a disability; medical copays and equipment becomes quite costly to take more time off work to attend the child’s medical needs as well.”
“Several doctor appointments and therapy appointments which can cause time away from work and loss of income.”
“I ended up resigning and becoming a stay at home mother until the appointments and therapies slow down.”
“Due to the fact my son has had so many neurological issues that have now lead up to some ventilator dependency, I am stuck in my home nearly all of the time.”
Theme 3: Parental emotions
The next reported negative impact of having a child with MMC was related to parental emotions. Parents reported stress, worry, exhaustion, or grief from having a child with MMC:
“Stress of the unknown, constantly wondering if a cold is just a cold not something spinal bifida.”
“Grieving for my child – sad for the struggles he’ll face.”
“You never really get over the emotions that are brought up during the process of delivery, birth, and watching them go.”
“The possibility of complications throughout childhood is ever present and you have to find a way to deal with that worry.”
“It can be emotionally draining, especially when multiple major surgeries are required.”
Theme 4: Disparaging views or remarks from others
A minority of parents (
“People do not understand that this can’t be cured. Comments about how my son is unable to do certain things that any other kid his age is able to do.”
“That some people don’t think these children’s lives are worth living.”
“They look at him like something is wrong with him. They don’t treat him the same as other family members and they always bring up the option of termination that I didn’t take.”
“Difficulty with handling other kids comments and maintaining our daughters love for life.”
Discussion
In this study, we report on the experiences and perspectives of a cohort of parents who elected to undergo MF surgery for fetuses diagnosed with MMC. Overall, these parents were well informed and reported gathering information prior to surgery from a wide range of sources. When they were given a fetal diagnosis of MMC, most were informed of all of their options by their obstetrician, although one-third were not informed of the option of prenatal surgery. About one-fourth of these parents said that they felt pressure to undergo one particular option. Half of the parents indicated that having a child with MMC has had a positive impact on them and their family, whereas the other half described both positive and negative impacts. The vast majority of parents said that knowing what they know now, they would definitely undergo prenatal surgery again.
This cohort gathered information about treatment options after their diagnosis from a variety of sources. Not surprisingly, over one-third gathered information on the internet. With a high proportion of homes with internet access, patients increasingly obtain health information online. Indeed, many maternal-fetal centers now have substantial websites that contain education about various diseases, treatment options, and patient testimonials. These and other internet-based sources of information can simultaneously empower and overwhelm parents; the abundance of available information creates challenges when attempting to promote an evidence-based approach to medical decision making [25]. Reliable internet sources that can be a decision support tool for parents should be developed by both professional organizations as well as by spina bifida advocacy groups. The MMC Maternal-Fetal Management Task Force has argued for standardized counseling including management options, the results of the MOMS trial, institutional experiences and outcomes, long-term obstetric and reproductive implications of MF surgery, and recommendations regarding preconception folic acid supplementation in subsequent pregnancies [26]. We believe that in addition to these important factors, counseling should include sharing the experiences of undergoing MF surgery and caring for a child with spina bifida.
Prenatal decision making for MF surgery poses ethical challenges, including weighing the risks and benefits to the pregnant woman and the fetus [27]. Different subspecialists involved in the care of women and fetuses diagnosed with MMC may vary in their interpretation of the MOMS trial results and in their self-reported subsequent recommendations [28]. Obstetricians encounter pregnant women earlier in the process of medical evaluation and decision-making than compared to neonatologists and pediatric surgeons. The profession strongly advocates discussing all possible management options, including termination, in a nondirective manner [25]. The majority of parents in our cohort report that their obstetrician informed them about surgery after birth, termination, and prenatal surgery. However, nearly one-third were not informed by their obstetrician about the option of prenatal surgery.
The majority of parents in our cohort report that when speaking to their physicians about their treatment choices they did not feel pressured to choose one option. Among parents who did report feeling choice pressure, only one parent felt pushed to have maternal-fetal surgery, while the rest felt compelled to have postnatal surgery or to terminate the pregnancy. Some have argued that providers may feel that counseling women about the option of prenatal surgery places undue pressure on them [29]. In a recent study of 332 parents who had a child with trisomy 13 or 18 (conditions, we note, that are not uniformly lethal), 61% of parents reported feeling compelled to terminate [28]. For other non-lethal conditions such as trisomy 21, 33% of a cohort of women in Holland who underwent a pregnancy termination reported that their obstetrician was influential in their decision to terminate [30]. Our results demonstrate that the vast majority of parents who undergo prenatal surgery did not feel pressure to do so by their physicians.
In our current study, half of parents report that having a child with MMC has had a positive impact on them and their family, while the other half 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 an exclusively negative family impact. These findings are consistent with results from the MOMS trial, which examined both family impact and parental stress levels [31]. While the overall parental stress was not different between the two arms of the MOMS trial, the overall negative family impact of caring for a child with MMC was significantly lower in the prenatal surgery group compared to the postnatal surgery group. Additionally, prenatal repair resulted in a lower impact on social interactions outside the home as well as parental and sibling relationships [31]. Interestingly, among the parents in our survey, the most prevalent positive impact theme dealt with parental attitude. Many reported personal growth, a change in their outlook on life, and increased gratitude. These parents have been greatly shaped by caring for a child with MMC. In addition, many parents have developed new friendships and support networks. Social media has enabled many parents of children with disabilities to connect with similar families. These positive family transformations are commonly referred to as “posttraumatic growth”, where one experiences positive changes following a personal adversity [32]. Recent studies have described parents’ resilience and growth after difficult hospitalizations for children with a serious illness [33] and critically ill newborns in the neonatal intensive care unit [34].
Of note, parents were open and forthright regarding the challenges of caring for a child with MMC after MF surgery. Not surprisingly, these challenges are very similar for the parents of children with spina bifida who did not undergo MF surgery [19, 20]. The two dominant themes for negative impacts were functional impact/medical burden and strain on the family. The functional challenges faced by some of these children, including motor deficits and bowel and bladder difficulties, remain considerable despite the benefits of prenatal surgery. These data may help prospective parents have realistic expectations about their child’s functioning after surgery. For some parents in our cohort, caring for a child with MMC was a significant stressor in their marriage or family relationships. Other parents reported growing closer to their partner through this experience. Previous studies have reported negligible effects of caring for a child with spina bifida on marital happiness and stability [19]. More work is needed to study the effects of MF surgery on parents’ relationships and to differentiate those effects from the general stressors of caring for a child with a disability. Many parents also reported significant financial stress associated with caring for a child with MMC. Despite these negative impacts, the vast majority of parents reported that if they were able to go back in time they would definitely still undergo fetal surgery. It is important that during prenatal counseling, parents receive information pertaining to both positive and negative impacts.
Limitations
Our findings need to be interpreted acknowledging important limitations of the study. We did not conduct a representative population-based study. Consequently, these parents are a self-selected sample of all parents of children with MMC who have undergone prenatal repair. In addition, we only included parents who were members of a particular social media group. Parents who are not part of this group may differ in their experience and perspective. Furthermore, we do not have any information on the participation rate of the parents who are members of the social media group, nor do we have information about those parents who chose not to respond. This study did not include the women and their partners who participated in the MOMS trial. Those individuals are still being studied under the NIH funded MOMS2 study as the children enter school. Finally, these data are self-reported questionnaires with some likely degree of recall bias. Nonetheless, these data reflect the experiences of a large cohort of parents, and while a more representative sample may have identified additional themes or altered the proportions we report, our core findings would likely remain embedded in the experience of many parents.
Conclusion
Parents of children with MMC who have undergone MF surgery have a broad range of experiences and perspectives. While many parents report negative impacts on their children and families, the majority of parents also report a range of positive impacts. The vast majority of parents, knowing what they know now, indicate that they would undergo prenatal surgery again. These data should further enhance providers’ understanding of the experience of our patients and help to further develop models of counseling that effectively address parental experience. This report may help inform parents currently considering prenatal surgery about the range of experiences reported by those who made the choice to undergo MF surgery.
Footnotes
Conflict of interest
The authors have no conflict of interest to report.