Fetal Myelomeningocele Repair - AORN Journal

CONTINUING EDUCATION Fetal Myelomeningocele Repair: A New Standard of Care 4.2 SUSAN M. SCULLY, BSN, RN, CNOR; MAUREEN MALLON, MBA, BSN, RN, CNOR; JOY C. KERR, BSN, CNOR; ALLISON LUDZIA-DeANGELIS, BSN, RN

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Purpose/Goal To educate perioperative nurses about fetal myelomeningocele repair and developing a fetal surgery program.

Objectives 1. Identify conditions for which fetal surgery may be performed. 2. Describe the types of spina bifida. 3. Discuss outcomes of spina bifida. 4. Describe perioperative care of a fetus undergoing fetal myelomeningocele repair. 5. Discuss how a simulation program can be used for competency development in a fetal surgery program.

Ms Scully, Ms Mallon, Ms Kerr, and Ms Ludzia-DeAngelis have no declared affiliations that could be perceived as posing potential conflicts of interest in the publication of this article. The behavioral objectives for this program were created by Rebecca Holm, MSN, RN, CNOR, clinical editor, with consultation from Susan Bakewell, MS, RN-BC, director, Perioperative Education. Ms Holm and Ms Bakewell have no declared affiliations that could be perceived as posing potential conflicts of interest in the publication of this article.

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http://dx.doi.org/10.1016/j.aorn.2012.05.009

Ó AORN, Inc, 2012

August 2012

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Fetal Myelomeningocele Repair: A New Standard of Care 4.2 SUSAN M. SCULLY, BSN, RN, CNOR; MAUREEN MALLON, MBA, BSN, RN, CNOR; JOY C. KERR, BSN, CNOR; ALLISON LUDZIA-DeANGELIS, BSN, RN

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ABSTRACT Myelomeningocele, also known as spina bifida, is one of the most common congenital anomalies of the central nervous system and the most common open, prenatally repaired birth defect. The Management of Myelomeningocele Study (MOMS), which compared the results of prenatal and postnatal myelomeningocele repair, found prenatal surgery to be much more effective than postnatal surgery. During surgery, the surgeon makes a hysterotomy and repairs the fetal myelomeningocele without removing the fetus from the uterus. After the repair is completed, the surgeon closes the uterus. The obstetric team monitors the mother closely with a goal of performing a cesarean delivery at 37 weeks’ gestation. The fetal surgery nursing team used innovative simulation teaching methods to establish and maintain fetal surgery team member competence, resulting in a successful program with good outcomes. AORN J 96 (August 2012) 176-192. Ó AORN, Inc, 2012. http://dx.doi. org/10.1016/j.aorn.2012.05.009 Key words: fetal surgery, spina bifida, meningocele, myelomeningocele, rachischisis, prenatal open intrauterine repair, fetal surgery program.

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here has long been interest and intrigue related to pregnancy and fetal development. For example, Hippocrates (BC 440 to BC 377) concluded that amniotic fluid primarily consisted of urine as a result of the fetus urinating in utero.1 In 1877 and 1885, respectively, two researchers studied fetal guinea pigs. They found that although these intact fetal guinea pigs could survive suspended in warm saline, after they were removed from the saline and allowed to breathe, they could not be returned to the uterus and survive.1,2 In 1918, a researcher performed the first successful surgery on fetal guinea pigs.1,2 Seven years later, another researcher demonstrated that a normal delivery of an animal fetus was possible after in utero manipulation.1,2

During the following half century, many more discoveries were made as researchers were able to simulate fetal anomalies in animals, subsequently treat the fetuses, and close the uteruses with pursestring sutures to prevent the loss of amniotic fluid.1 In 1981, researchers at the University of California, San Francisco (UCSF), were able to surgically correct the following fetal defects in animals: n

congenital hydronephrosis, diaphragmatic hernia, and n obstructive hydrocephalus resulting in normal fetal development after surgical correction.1 n

In 1961, the first successful human fetal surgery was performed in New Zealand. The surgeon performed an intrauterine transfusion for hydrops http://dx.doi.org/10.1016/j.aorn.2012.05.009

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FETAL MYELOMENINGOCELE REPAIR fetalis (ie, edema in two or more body areas caused by Rh incompatibility).1 During the 1970s, many developments in the biomedical and medical fields, including the development of ultrasound imaging and glucocorticoid therapy for fetal lung maturity, increased the hopes that other successful human fetal surgeries could be accomplished.1,2 These developments decreased the mortality rates of premature neonates from respiratory distress syndrome and made fetal defects easier to identify, which subsequently made fetal surgery less risky.1,2 In 1982, the first fetal intervention was successfully performed in the United States by Michael Harrison, MD, and his team at UCSF. The surgical team included a perinatologist, sonographer, and surgeon who repaired a fetal urinary tract obstruction by placing a pigtail stent from the fetus’ kidney to the bladder. In 1983, the first open fetal surgery for an obstructed bladder (ie, vesicostomy) was performed at UCSF by this same team on a fetus at 18 weeks’ gestation. It was around this same time that the first symposium of fetal experts from around the world was held in Santa Ynez, California, and from that symposium, the International Fetal Medicine and Surgery Society was established.1 Today, pediatric surgeons perform fetoscopic and open fetal procedures for a small number of fetal conditions. The most common open, prenatally repaired birth defect is myelomeningocele, a type of spina bifida. Other fetal conditions that can be treated include n n n n n n

congenital cystic adenomatoid malformation, congenital diaphragmatic hernia, sacrococcygeal teratoma, twin-reversed arterial perfusion syndrome (ie, TRAP syndrome), twin-to-twin transfusion syndrome, and vesicostomy or stent placement for obstructive hydronephrosis.3

SPINA BIFIDA During fetal development, the neural tube differentiates into the brain, spinal cord, and central nervous system at approximately 14 weeks’ gestation.

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Dura mater, pia mater, and the arachnoid membranes make up the meninges, which protect the central nervous system. Spina bifida (ie, division of the spine) is a generic term for neural tube defects (ie, developmental defects of the spinal cord). These include spina bifida occulta, meningocele, myelomeningocele, and rachischesis.3 Spina bifida occulta is the least serious type of neural tube defect and is usually asymptomatic. It is characterized by a “dimple” or depression in the lumbosacral area caused by the defective closure of one or more vertebrae.3 A meningocele occurs when the meninges herniate through an opening in the vertebrae during fetal development. A sac is formed that contains cerebral spinal fluid, but no neural tissue.3 Spina bifida occulta and meningocele are not external issues that can or should be repaired prenatally. A myelomeningocele occurs when meninges herniate into a sac filled with cerebral spinal fluid that includes the spinal cord or neural tissue.3 The most serious form of spina bifida, rachischisis, occurs when there is fusion of one or more of the vertebrae.3 In complete rachischisis, the entire neural tube is open from head to pelvis, which is not repairable. It often is associated with cleft lip or palate and hydrocephalus. Complete rachischisis is not usually compatible with life and is often fatal.3 Myelomeningocele is one of the most common congenital anomalies of the central nervous system.3 When this neural tube defect occurs, other congenital abnormalities also may occur, including hydrocephalus, abnormalities of the hips or feet, and a dysfunctional bowel or bladder.3,4 The incidence of myelomeningocele in the United States is 3.4 per 10,000 live births.4 Early ultrasound studies performed throughout the pregnancies of women with fetuses identified as having myelomeningocele suggest that spinal cord injury and its symptoms worsen the longer the myelomeningocele is exposed in utero.4 This can result in decreased movement of the fetus’ lower limbs and worsening herniation of hindbrain and hydrocephalus as the pregnancy progresses.4 AORN Journal j 177

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THE MANAGEMENT OF MYELOMENINGOCELE STUDY (MOMS) The first intrauterine repair of myelomeningocele on a late-gestation fetus was performed in 1997 at Vanderbilt University in Nashville, Tennessee, by Noel Tulipan, MD, and his team. In 1998, N. Scott Adzick, MD, and his team at Children’s Hospital of Philadelphia, Pennsylvania, performed the first intrauterine repair of myelomeningocele on an early-gestation fetus. By 2003, more than 200 early-gestation fetuses had undergone in utero surgical repair of a myelomeningocele.4 Early data suggested substantial improvement to hindbrain herniation and possible increased postbirth mobility in children who had undergone fetal surgery. There were many complications of this surgery, however, including increased maternal risk of preterm labor and uterine dehiscence as well as increased risk of fetal death and preterm birth.4 For these reasons, the National Institute of Child Health and Human Development in Bethesda, Maryland, instituted the MOMS to compare the safety and efficacy of fetal versus postnatal repair of myelomeningocele. Three hospitals, Children’s Hospital of Philadelphia, Vanderbilt University, and UCSF, were chosen to participate in the study. George Washington University, Washington, DC, was enlisted as an independent data coordinating center. All other hospitals in the United States where this fetal surgery was performed agreed not to perform this surgery for the length of the trial. Not having the surgery performed at other facilities helped in two ways. An adequate sample size for both the study and control groups would be attained more quickly, and the results would not be skewed by parents leaving the study to go elsewhere to have the surgery completed. It was estimated that to obtain an adequate sample size in both groups, the trial would take five years.4 Proposed study enrollment was 200 patients (ie, 100 patients whose fetuses would undergo fetal surgery and 100 whose children would undergo postnatal surgery). Any woman interested in the 178 j AORN Journal

study could contact the coordinating center and, if eligible, would be referred to one of the participating hospitals according to her geographic location. Women included in the study were US residents 18 years of age or older with singleton pregnancies whose fetuses had a gestational age of 19.0 to 25.9 weeks and were diagnosed with n

myelomeningocele with an upper boundary between the first thoracic spinal level and the first sacral spinal level, n positive hindbrain herniation, and n a normal karyotype (ie, a complete set of chromosomes). Exclusion criteria were n n n n n n

a fetal anomaly unrelated to myelomeningocele, severe kyphosis (ie, curving of the spine), a mother’s increased risk of preterm labor (eg, short cervix, previous preterm delivery), placental abruption, a maternal body mass index of 35 or more, or contraindication to surgery (eg, previous hysterotomy in the active uterine section).4

The researchers randomly assigned the patients 1:1 (ie, fetal versus postnatal surgery) using a secure web site maintained by the coordinating center.4 Women who were assigned to the fetal surgery group relocated to the area near their assigned hospital, which provided them with a support person from the time of surgery to their spontaneous delivery or cesarean birth at 37 weeks’ gestation. Women assigned to the postnatal surgery group stayed at home until 37 weeks’ gestation and then returned to their assigned hospital for cesarean birth and postnatal surgery.4 The researchers evaluated physical, neurological, and developmental testing conducted on all children born to mothers participating in the trial at 12 and 30 months of age. The tests were performed by independent physicians who were unaware of study group assignments and who reported their findings directly to the coordinating center.4 The researchers stopped the study after outcomes were known on the 183 participants who had

FETAL MYELOMENINGOCELE REPAIR

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her to the assigned room for preoperative insertion enrolled from February 2003 to December 2010 4 of a large-bore IV and placement of an epidural because the fetal surgery was so effective. One of anesthesia catheter. During this time, the OR staff the primary outcomes of fetal surgery was a demembers are busy setting up the equipment and creased need for a ventriculoperitoneal shunt for preparing the room. When everything is completed hydrocephalus by age 12 months.4 Only 40% of the and all team members are ready, the circulating children from the fetal surgery group required shunt nurse assesses the patient in the SDU, after which placement by age 12 months compared with 82% of the nurse develops individualized care plans for the the children from the postnatal surgery group.4 This mother and fetus (Tables 1 and 2). The nurse then study also demonstrated that at 12 months of age, transports the mother to the OR. Surgical team 64% of the babies who had undergone fetal surgery members help her onto the OR bed and place elecexperienced the complications of hindbrain herniatrocardiogram and oxygen saturation monitors. The tion, whereas 96% of the babies who underwent circulating nurse places sequential compression surgery after delivery experienced hindbrain device stockings on both her legs and applies a safety herniation by 12 months. Furthermore, only 25% belt. The surgical team then performs a preinduction of the children from the fetal surgery group who time out with the paexperienced hindbrain tient. General anesherniation had modThe day before a scheduled myelomeningocele thesia is used instead erate to severe hindrepair, representatives from all of the involved of regional anesthesia brain herniation compared with 67% of teams meet to discuss the patient and specifics for two reasons. First, of the procedure. it is necessary to adthe children from the minister high-dose postnatal surgery volatile anesthetic group. By age three agents to the mother to quickly and reliably manipyears, 42% of the children who had undergone ulate uterine tone. The uterus must remain totally fetal surgery were able to walk without orthotics relaxed during the procedure. Second, the mother’s or assistive devices compared with only 21% of anesthesia also affects the fetus, thus minimizing the the children from the postnatal surgery group.4 amount of intramuscular anesthesia that needs to be administered to the fetus. The additional shot of FETAL SURGERY PROGRAM intramuscular anesthetic is given to the fetus to help The day before a scheduled myelomeningocele eliminate movement when the fetus is optimally repair at Children’s Hospital of Philadelphia, reppositioned for myelomeningocele repair. resentatives from all of the involved teams meet to After induction, the anesthesia professional discuss the patient and specifics of the procedure inserts an endotracheal tube, an additional IV line, before bringing the parents into the room. The team an arterial line, and a nasogastric tube. The circuincludes members of the surgery, obstetrics, labor lating nurse inserts an indwelling urinary catheter and delivery, nursing (eg, special delivery unit and places gel positioning rolls under both of the [SDU], OR, advanced practice nurses), anesthesia, patient’s knees and gel pads under her ankles. The and social work departments. After the parents nurse also places a wedge under the patient’s right arrive, team members review preoperative and hip to relieve pressure on the major abdominal postoperative instructions with them (eg, what to vessels from the uterus. Before the patient has been expect, potential complications, future follow-up prepped and draped, the ultrasound technologist plans) and answer any questions they may have. performs an ultrasound to determine the fetus’ After arrival of the mother in the SDU on the and the placenta’s position. morning of surgery, a labor and delivery nurse takes AORN Journal j 179

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TABLE 1. Nursing Care Plan for a Maternal Patient Undergoing Hysterotomy for Fetal Myelomeningocele Repair

Diagnosis Anxiety, ineffective coping, compromised family coping, decisional conflict

n n n n n n n n

n n n n

Risk for perioperative positioning injury

n n n n

n n n n n n n

Risk for imbalanced body temperature

Interim outcome statement

Nursing interventions

n n n n n n

Identifies psychosocial status. Assesses coping mechanisms. Identifies barriers to communication. Identifies patient’s and designated support person’s educational needs. Identifies expectations of home care. Implements measures to provide psychological support. Includes patient or designate support person in perioperative teaching. Seeks necessary referrals to assist mother and one support person in relocating to the area between surgery and expected delivery time. Explains expected sequence of events. Provides status reports to designated support person. Evaluates psychosocial response to plan of care. Evaluates response to instructions.

n

Assesses baseline skin condition. Identifies baseline tissue perfusion. Identifies baseline musculoskeletal status. Identifies physical alterations that require additional precautions for procedure-specific positioning. Verifies presence of prosthetics or corrective devices. Positions the patient. Implements protective measures to prevent skin/ tissue injury from mechanical sources. Applies safety devices. Evaluates tissue perfusion. Evaluates musculoskeletal status. Evaluates for signs and symptoms of physical injury to skin and tissue.

n

Assesses risk for inadvertent hypothermia. Identifies physiological status. Implements thermoregulation measures. Monitors body temperature. Monitors physiological parameters. Evaluates response to thermoregulation measures.

n

After the patient has been prepped and draped but before the initial incision, all surgical team members participate in a surgical time out. The 180 j AORN Journal

n

n

Outcome statement

The patient verbalizes the sequence of events to expect before and immediately after surgery. The patient states realistic expectations regarding recovery from the procedure.

n

The patient or designated support person demonstrates knowledge of the expected psychosocial responses to the procedure.

The patient has full return of movement of the extremities at the time of discharge from the OR or procedure room. The patient is free from pain or numbness associated with surgical positioning.

n

The patient is free from signs and symptoms of injury related to positioning.

The patient’s temperature is greater than 36 C (96.8 F) at time of discharge from the OR or procedure room.

n

Patient is at or returning to normothermia at the conclusion of the immediate postoperative period.

surgeon then makes a horizontal laparotomy incision halfway between the patient’s umbilicus and pubic bone. After careful dissection, the surgeon


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TABLE 2. Nursing Care Plan for a Fetal Patient Undergoing Surgery in Utero to Repair a Myelomeningocele

Diagnosis Risk for deficient fluid volume

n

n n n n n n n n n

Risk for infection

n n n n n n n n n n n n n n

Risk for imbalanced body temperature

Interim outcome statement

Nursing interventions

n n n n n n n

Outcome statement

Identifies factors associated with an increased risk for hemorrhage or fluid and electrolyte imbalance. Identifies physiological status. Reports deviation in diagnostic study results. Implements hemostasis techniques. Monitors physiological parameters. Establishes vascular access. Administers prescribed solutions. Collaborates in fluid and electrolyte management. Administers electrolyte therapy as prescribed. Evaluates response to administration of fluids and electrolytes.

n

The patient’s vital signs are within the expected range at discharge from the OR, procedure room, or postanesthesia care unit.

n

The patient’s fluid, electrolyte, and acid-base balances are maintained at or improved from baseline levels.

Assesses susceptibility for infection. Classifies surgical wound. Implements aseptic technique. Protects from cross-contamination. Initiates traffic control. Administers prescribed prophylactic treatments. Administers prescribed medications. Administers prescribed antibiotic therapy as ordered. Performs skin preparations. Monitors for signs and symptoms of infection. Minimizes the length of invasive procedure by planning care. Maintains continuous surveillance. Administers care to wound sites. Evaluates for signs and symptoms of infection through 30 days following the perioperative procedure.

n

The patient has a clean, primarily closed surgical wound at discharge from the OR.

n

The patient is free from signs and symptoms of infection.

Assesses risk for normothermia regulation. Assesses risk for inadvertent hypothermia. Identifies physiological status. Implements thermoregulation measures. Monitors body temperature. Monitors physiological parameters. Evaluates response to thermoregulation measures.

n

The patient’s temperature is greater than 36 C (96.8 F) at time of discharge from the operating or procedure room.

n

The patient is at or returning to normothermia at the conclusion of the immediate postoperative period.

positions the uterus for a hysterotomy. If the placenta is lying in a posterior position, the surgeon performs an anterior hysterotomy and uses a ring

retractor to provide clear visual access. If the placenta is in an anterior position, the surgeon props the uterus on the sterile field with cloth AORN Journal j 181

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Figure 1. Specially designed stapler to quickly open the uterus.

towels and performs a posterior hysterotomy. Before the surgeon makes the initial uterine incision, the ultrasound technologist performs another ultrasound to map the edges of the placenta and confirm the fetus’ position. The surgeon places holding stitches on the uterus and makes a small opening in the uterine wall using electrosurgery. A specially designed uterine stapler (Figure 1) with absorbable staples is used to make the hysterotomy incision. This stapler is important not only to maintain hemostasis but also allows for easier closure of the uterus by providing single-layer closure as it tacks the amniotic sac to the uterine wall along the staple line. Before the surgeon places the staples, the ultrasound technologist performs another ultrasound to ensure that the fetus’ umbilical cord or other important structures are not in the way. At this time, the surgeon places a catheter that delivers warmed physiologic solution in the uterus to keep the fetus warm and buoyant during the procedure. The fetus is positioned so its back is 182 j AORN Journal

visible (Figure 2) and the surgeon administers intramuscular vecuronium and fentanyl anesthesia to the fetus. The ultrasound technologist performs an intraoperative echocardiogram throughout the myelomeningocele repair to monitor the fetus. The neurosurgeon removes the meningeal sac and frees up surrounding tissue to allow closure of the defect. To accomplish this, the neurosurgeon must suture multiple layers of tissue to ensure a water-tight seal. If the skin edges cannot be approximated to cover the defect, the surgeon sews a reconstituted regenerative tissue matrix (Figure 3), cut to size, into place to repair the defect. After the neurosurgeon closes the myelomeningocele, the surgeon returns the fetus to the uterus (Figure 4) and begins a rapid uterine closure. The surgeon removes from the uterus the catheter that delivers heated fluid at the last possible moment so that lost amniotic fluid can be replaced with warmed physiologic solution and antibiotics can be instilled before removing the catheter. Replenishing the uterus to the same volume as before


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Figure 2. The exposed fetal myelomeningocele.

Figure 3. Fetal myelomeningocele with reconstituted regenerative tissue matrix patch.

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Figure 4. Closing the myelomeningocele in preparation to place the fetus back in the uterus.

surgery helps keep the baby and cord free floating to minimize entanglements as well as warming the fetus after the fetus has been exposed to ambient air. The surgeon then uses double-armed, interrupted stitches followed by a running stitch to complete the uterine closure. The surgeon then tacks the omentum to the uterus to bring additional blood supply to the uterine closure to help the incision heal and ensure another water-tight seal before beginning a multiple-layer closure of the abdominal wall. The surgeon will remove the omentum from the uterus during the cesarean delivery to inspect the fetal surgery hysterotomy incision for thinning or dehiscence. A clear plastic dressing is applied over the patient’s incision to accommodate the need for frequent ultrasounds of the fetus during the first few postoperative days. At the end of the procedure, the ultrasound technologist performs another ultrasound to evaluate how the fetus tolerated the procedure. If the fetal heart rate is stable, the 184 j AORN Journal

anesthesia professional begins to allow the mother to emerge from anesthesia, and when she is awake, the team moves her to her bed and wheels her to the postanesthesia care unit for recovery. CREATING A FETAL SURGERY SERVICE Fetal surgery is now the standard of care for myelomeningocele repair (N. Scott Adzick, surgeon-in-chief, Children’s Hospital of Philadelphia, Pennsylvania; oral communication, March 2012). As a result, many facilities will be working to develop a competent team able to replicate the MOMS results. Developing a nursing team to support this endeavor may not be an easy task. In 1995, Children’s Hospital of Philadelphia hired N. Scott Adzick, MD, as the chief of surgery. Dr Adzick planned to create a fetal surgery program at Children’s Hospital similar to the one he had run on the West Coast. The complexity and uniqueness of this program presented members of the perioperative department with challenges. We

FETAL MYELOMENINGOCELE REPAIR knew how to perform surgery on neonates weighing 1 kg (2.2 lb) or less; however, trying to perform a surgical procedure on a tiny fetus still cocooned in its mother’s womb would be a new experience for us all. It would be challenging to acquire and learn about all the equipment needed and become proficient in a surgical procedure we had yet to participate in. Dr Adzick had the nurses from his previous practice setting send copies of preference cards for every procedure he performed before his arrival at Children’s Hospital. They also sent a list of his instrumentation needs so that we could have the instruments ready when he arrived. We were quickly able to learn everything that we would need for his day-to-day surgical procedures and make a few adjustments to our current sets. The fetal surgery instrumentation list, however, included some unfamiliar instruments and some that had been custom made, such as the unique retractor blades for the Turner-Warwick ring or the instrument known as a “uterine spear.” We needed to order new disposable items, such as a uterine stapler and sterile ultrasound gel and probe covers. After reviewing Dr Adzick’s preference cards, we compiled a list of questions: n

Do pulse oximetry probes come prepackaged as a sterile product or will we need to package them ourselves and then resterilize them? n How do we maintain adequate amniotic fluid levels during and after the procedure? n If the pulse oximeter becomes wet, can it create an electrical shock? We had to create many unique solutions, such as packaging and sterilizing our own aluminum foil pieces to assist in keeping pulse oximetry probes dry and to reflect any light that might interfere with their performance. Because most of the perioperative staff members had only pediatric surgical experience, the idea of performing a hysterotomy on an adult woman was new to us. To prepare for this, we obtained temporary privileges at the University of Pennsylvania

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Medical Center, Philadelphia, and spent time in the delivery room circulating and scrubbing on cesarean births. This also provided staff members the opportunity to build a rapport with physicians in the Maternal Fetal Medicine Group with whom we would be working on this new endeavor. Although this was a useful experience, we found out later that the hysterotomy we perform only somewhat resembles that performed during a cesarean birth. The First Procedure Dr Adzick booked the first fetal procedure at Children’s Hospital in January 1996. The pregnant mother was from Florida and her fetus had congenital cystic adenomatoid malformation (ie, an enlarged benign lung mass that compromises cardiac function). We felt confident that we had the instrumentation and supplies needed for the proposed procedure. We cautiously anticipated this first procedure, and after the team meeting with the family, we were again reminded how precious this baby’s life was to this family. We checked and double checked to make sure that we would not have any unexpected problems with our supplies and preparations. The evening before, team members stayed beyond their shifts to set up the OR. On the morning of surgery, the team arrived early to set up, scrub, and prepare for the mother’s arrival in the OR. Two circulating nurses, a primary scrub person, and a backup scrub person were scheduled to assist with the procedure. After the surgeon exposed the mother’s uterus, the ultrasound technologist mapped the placenta to facilitate a safe hysterotomy incision. After opening the uterus, Dr Adzick exposed the fetus’ tiny arm and chest wall, which made everyone in the room pause for a moment in awe. The team quickly performed the thoracotomy to expose the cystic mass. At that moment, the fetus’ heart began to quiver because it could not compensate for the significant changes that were occurring; mass delivery and the abrupt removal of AORN Journal j 185


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are imperative to provide safe patient care and optimal outcomes. In addition, ensuring that all team members are given the opportunity to participate in these procedures regularly is necessary to maintain the skills of the team members and maintain a cohesive team. Although the concept of specialty teams is not new, subspecialization can be challenging. Often a nurse can work outside his or her specialty area if partnered with a strong colleague; however, the demands of the fetal surgery nursing team are such that it is not possible for these procedures. Creating a strong nursing team is integral to developing the program. However, doing so can result in a small, segregated team, which ultimately affects both the unit and the team itself. Challenges and Solutions Increased call responsibilities for this small subThe challenges of fetal surgery are unique. Although specialty group of nurses decreases the number of all the basics of perioperative nursing apply (eg, nurses available to take call in the general OR. sterile technique, knowledge of instrumentation This increases the pressure on both teamsdthe specific to the type of surgery, collaboration with the subspecialty fetal surgical and anesthesia surgery team as well teams), the differences as the general OR are many. First, in this The challenges of fetal surgery are unique team, whose call situation, there are two because the two patientsdthe adult mother and her fetusdhave different needs. responsibilities inpatientsdan adult crease with fewer mother and her fetus. nurses rotating to take These patients, while call. In addition, with high-risk, low-volume physically in the same OR, have different needs. surgery, nontraditional and innovative plans to The adult patient has a mature and accessible train the team must be developed by those who airway, viable lungs, and generally good vascular have the most experience in the specialty. access. The fetus does not. The healthy adult has The services of multiple specialties are necesa generous supply of blood and is able to recover sary. For example, performing the myelomefrom minor blood loss. The small blood volume ningocele procedure involves nursing, anesthesia, of a fetus leaves little room for error. The adult general surgery, obstetrics/maternal fetal medipatient has the ability to respond to infection and, cine, fetal cardiology, ultrasonography, and neuif necessary, can be treated aggressively; postrosurgery services. Multidisciplinary surgeries are operative infection in a fetus would likely be not new to most ORs; however, the procedures fatal. are usually separate from one another and distinct As unique as these challenges are, the chalto the service performing them. Fetal surgery lenges of establishing and maintaining a fetal demands the seamless flow of one service into surgery nursing team are unique as well. As with another, with multiple hand offs occurring at any high-risk, low-volume surgery, developing, various points in time. For example, the general maintaining, and refining perioperative nursing surgeon opens and closes the mother’s abdomen clinical skills to meet the needs of the fetal team cardiac compression resulted in fetal hemodynamic collapse and reactive bradycardia. One surgeon administered two-finger cardiac compressions while another surgeon quickly inserted a sterile butterfly needle and administered a blood transfusion. Soon the fetus’ heart resumed a normal rhythm, after which the surgeons were able to ligate the vessels attached to the mass and remove it from the fetus’ chest. The surgeons closed the fetus’ chest wall, returned the fetus to the womb, and began uterine closure. After the procedure, the mother and fetus were monitored closely and continued to do well. Weeks later, the mother delivered a healthy baby boy.

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PATIENT EDUCATION Fetal Repair of a Myelomeningocele (MMC) Overview An MMC (spina bifida) occurs when a baby’s spinal cord remains exposed. This may result in lifelong paralysis, water on the brain, bowel and bladder incontinence, and developmental delays. Untreated, it may cause death. How is MMC diagnosed? Blood tests early in pregnancy can diagnose MMC. Your health care provider may test the amniotic fluid that surrounds the baby during pregnancy or perform an ultrasound or an ultrafast fetal magnetic resonance imaging. What are the treatment options? An MMC may require surgical correction to repair the defect. Surgery may be performed during pregnancy, when there is still potential for recovery, or may be performed after delivery to prevent infection and reduce further physical disability. What does prenatal preoperative care include? n You may eat a light meal (tea and toast) up to 8 hours before surgery and drink clear liquids (water, clear fruit juice without pulp, carbonated beverages, clear tea, or black coffee without milk) up to 2 hours before surgery. n Ask your doctor about taking any prescribed medications the morning of surgery. n On admission, a nurse will check your vital signs and ask questions about your health. Tell the nurse about any allergies, previous surgeries, current medications, and any injuries (cuts, skin abrasions), especially on your abdomen. n An anesthesia care provider will explain what kind of anesthesia (medicine that keeps you asleep during surgery) you will receive. Your baby will be given pain medicine during the procedure. What happens during surgery? After the surgeon opens your abdomen, he or she will use uterine ultrasound to see where the baby and the placenta are. After opening your uterus, the surgeon delivers enough of the baby to see and close the defect. During surgery, your baby’s heart is monitored to make sure the baby is doing well. The surgeon returns the baby to your uterus and closes your uterus and abdomen. After surgery, you will have a clear plastic dressing on the incision so that ultrasounds can be performed to check the baby.

n care for the incision.

What are possible complications? You could get an infection in your incision, pneumonia, a blood clot in one of your legs, or bleed too much. There is a chance that the surgery will cause you to go into preterm labor, which could have many complications for your baby. Although not very likely, it is possible for your baby to die after surgery. What happens after I go home? n After surgery, you must stay within 30 minutes of the hospital. n Follow your surgeon’s instructions about eating and activity (ie, strict bed rest) and come back to the hospital twice a week for ultrasound tests to watch how your baby is doing. n Keep the incision dry for the first seven to 10 days and then wash it daily with mild, unscented soap and gently pat it dry; do not put any lotion or powder on the incisions until they are completely healed. n You will be given oral medications to prevent uterine contractions for the rest of your pregnancy. Call your doctor immediately if you experience any n shortness of breath, sudden dizziness, or weakness; n increased redness, swelling, or drainage at your incision; n fever greater than 101 F or chills; n nausea or vomiting unrelieved by medication; or n pain or labor that is not controlled with medicine. Resources Myelomeningocele fetal surgery. Cincinnati Children’s Fetal Care Center of Cincinnati. http://www.cincinnatichildrens .org/service/f/fetal-care/conditions/mmc/default. Accessed April 25, 2012. Spina bifida surgery: your experience depends on our experience. The Children’s Hospital of Philadelphia. Center for Fetal Diagnosis and Treatment. http://www .chop.edu/service/fetal-diagnosis-and-treatment/spina -bifida.html. Accessed April 25, 2012.

What does postoperative care include? After surgery, you will n be admitted to the recovery area and monitored closely. n stay in the hospital for approximately four days. n be asked to breathe deeply and use an incentive spirometer to prevent pneumonia. n possibly feel tired or uncomfortable. Any pain or nausea will be treated. A nurse will teach you how to n control pain and swelling with medicine, rest, and ice, and

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and uterus and the neurosurgeon performs the myelomeningocele repair, with continued monitoring and assistance from the obstetrician. Also involved are team members who perform the intraoperative ultrasonography, fetal echocardiography, and anesthesia monitoring of the mother and the fetus throughout the procedure. With all of this activity, the surgical field for a fetal myelomeningocele repair is an extremely crowded place. As with any new procedure, the anxiety levels of team members can run high because of the potential unknowns of the procedure, the instrumentation, and the risks to the fetus. Finding perioperative nurses who are willing to commit to this team initially was not an easy task. Fetal surgeries are high-risk procedures, and they require a great deal of flexibility and dedication to n

cover on-call time, n come in early or stay late for procedures, and n update preference cards after every procedure. Initially, many nurses voiced an interest in being part of the team but also quickly voiced their concerns. We began with two experienced nurses who were willing to scrub on these procedures as well as take call. Nursing staff members on the day, evening, and night shifts were educated about the role of the circulating nurse. Because we recognized the need to be prepared at a moment’s notice, we established a fetal surgery cart that contained all necessary items that could easily be brought to any room. With this new specialty, the fetal service and the hospital grew exponentially during the following decade. In 2003 Children’s Hospital of Philadelphia began participating in the MOMS, and in July 2008 the SDU was opened. The decision was made that all fetal surgeries would be performed in the surgical suite attached to the new unit, two floors away from the general OR. At the same time, the few perioperative nurses who knew how to scrub for fetal procedures moved on to pursue different avenues in their nursing careers so, once again, the fetal surgery program had no experienced nurses to staff these 188 j AORN Journal

procedures. As a result, a new set of challenges emerged. We wondered n

how an interested nurse would learn to scrub for these specialized surgeries when the team demanded excellence but the procedures were few and far between, n how we would provide seamless service to patients two floors away from the main OR while maintaining the safety and efficiency provided on the fourth floor OR, and n how we would continue to staff for the fetal myelomeningocele repairs scheduled through the ongoing MOMS. As the number of members on our fetal surgery nursing team gradually decreased, misconceptions about the surgery increased, so there was little interest in joining the team. Therefore, we established a core focus group to evaluate the situation and brainstorm ideas. One key factor associated with this problem was lack of knowledge and experience with a fetal surgery program. As a result, the director of fetal surgery prepared and delivered a lecture to approximately 20 perioperative team members to encourage them to consider joining the team. In addition, core team members decided to take advantage of simulation, a new and innovative learning opportunity at Children’s Hospital. The core team members further recognized that the fetal surgery nursing team should not be segregated from general surgery staff members but that fetal surgery should be incorporated into the department of general and thoracic surgery. As a subspecialty of general surgery, secluding the fetal surgery nursing team had led to misconceptions about the team and the procedures. We felt that this discouraged staff members from expressing interest in joining the team as well as alienating the fetal surgery nursing team from being integral members of the general perioperative nursing team. For these reasons, care must be taken to ensure that both the fetal surgery nursing team members and members of the perioperative unit are properly educated and supported. In addition, it is important to recognize

FETAL MYELOMENINGOCELE REPAIR the role that general perioperative staff members play in the success of the fetal program by supporting members of the fetal surgery nursing team. As a result of the brainstorming session, the perioperative nursing leaders at Children’s Hospital asked the general and thoracic surgery specialty nurse to assume responsibility for the fetal service instead of maintaining it as a “stand-alone” specialty. The next challenge was to educate this specialty nurse about how to scrub for fetal myelomeningocele repair surgeries. To accomplish this, we asked for the help of two previous fetal surgery nurses who had moved into new positions within Children’s Hospital. In addition, maternal/fetal nurse practitioners and OR nursing leaders perform the circulating role during these procedures. Buy-in from all perioperative staff members is integral in setting up and managing a team such as this. With the SDU removed from the general OR by two floors, we had to ensure that everyone recognized that fetal surgery was now part of the general and thoracic surgery department because fetal surgery is as dependent on the support of the unit as that of any of the other services. Initially, fetal surgery depended on in utero diagnosis and referral and acceptance into the MOMS. Since the trial ended in 2010, the hospital has continued to rely on diagnosis and referrals. Due to their unpredictability, surgeries are often scheduled with only a few days’ notice. As a result, the OR schedule changes to accommodate the addition of a fetal surgery. We close a room in the main OR complex so the anesthesia and nursing teams can staff the fetal surgery room on the hospital’s sixth floor. This can affect staffing in the main OR because, as a result of the high-risk nature of these procedures, a third circulating nurse must be assigned to the procedure, in addition to the leadership support required. The solution is closing at least one room in the main OR to accommodate the staffing needs upstairs. If there is not an extra staff member available in the morning to act as the third circulator, one of the clinical nurse leaders acts as the third circulator in the room, and the

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clinical supervisors or charge nurse are available for any additional leadership support. SIMULATION We eventually formed a multidisciplinary team to develop a simulation program for fetal myelomeningocele surgeries. The team consisted of the clinical manager and one of the nurse practitioners from the fetal surgery service, two representatives from our simulation department, and the fetal surgery specialty nurse. The simulation scenario was designed using surgical dictation and procedural preference cards as a guide and encompassed the procedure from the time the room and equipment were set up to discharge of the patient from the OR. Participants from all disciplines (ie, nursing, surgery, anesthesia, cardiology, obstetrics) were invited to participate. To make this simulation as realistic as possible, we crafted a “fetus” with a myelomeningocele and a foam “uterus” that would fit inside a pregnancy simulation model (Figures 5, 6, and 7). After we developed the scenario and put plans in place to run the simulation, the next challenge was to ensure that all those who expressed an interest in fetal surgery were given the opportunity to attend the simulation sessions. Because this would take place during a regular surgical day, we had to accomplish the simulation training without it affecting OR staffing or, most importantly, the safety of our patients. We considered, rejected, and revised many plans to try to meet the needs of all involved. The plan we selected best met the needs of the unit and the learning objectives of the simulation. In lieu of having nurses work a break shift for the days of the simulation, the fetal team nurses were scheduled as the break shift nurses who came in at 7 AM rather than their typical 9 AM start time. This allowed fetal team nurses to participate in the simulation and the debriefing session that followed. After completing the simulation, all fetal team nurses returned to the OR to provide breaks and lunches. In this way, we were able to get all interested staff members through the simulation AORN Journal j 189


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Figure 5. Handcrafted uterus, with the doll from Figure 7 inside, placed inside a pregnancy simulation model.

Figure 6. The finished fetal myelomeningocele simulation model.

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Figure 7. Doll with myelomeningocele used for simulation.

program while providing the OR with the necessary support to run the unit. The combination of the lecture given by the director of fetal surgery and the simulation proved to be successful in identifying and recruiting interested nurses to become part of this subspecialty fetal team. Working closely together during the procedures and behind the scenes, the clinical manager and specialty nurse were able to ensure that members of the team were provided with the necessary learning experiences and proper support for safe and efficient nursing care. In addition, we provided regular rotation of the fetal team to give all members adequate experiences to maintain and refine their skill sets while we continually recruited and trained new team members. CONCLUSION All of this hard work has paid off. The fetal surgery nursing team is now nine members strong, with five members who can competently perform as the primary scrub person or circulating nurse and four

members who are able to circulate and who are currently in the training stages for the primary scrub role. As new nurses become interested in fetal surgery, the team members orient them individually. For scheduled procedures, we have three team members staff the room. One functions as the primary scrub person and one as the primary circulating nurse. The third team member acts as a second circulating nurse and helps with room setup and counts. After the procedure has begun, that team member scrubs in and performs as the secondary scrub person to help with the rapid pace of the repair and closure. The MOMS was successful in showing the advantages of fetal surgery for myelomeningocele, and we have been equally successful in creating, developing, and maintaining a fetal surgery nursing team. References 1. Jancelewicz T, Harrison MR. A history of fetal surgery. Clin Perinatol. 2009;36(2):227-236. 2. Han S, Hwang E. The development of fetal surgery. Yonsei Medi J. 2001;42(6):602-608.

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3. Crapanzano S, Pounders T. The unique role of folic acid: preventing serious and disabling birth defects. Pelican News. 2002;58(2):6-11. 4. Adzick N, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):993-1004.

Susan M. Scully, BSN, RN, CNOR, is a clinical level IV nurse and general, thoracic, and fetal surgery specialty nurse at the Children’s Hospital of Philadelphia, PA. Ms Scully has no declared affiliation that could be perceived as posing a potential conflict of interest in the publication of this article. Maureen Mallon, MBA, BSN, RN, CNOR, is a clinical coordinator for the general, urology, and otolaryngology surgical nursing specialties at the Children’s Hospital of Philadelphia, PA. Ms Mallon has no declared affiliation that could

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be perceived as posing a potential conflict of interest in the publication of this article. Joy C. Kerr, BSN, CNOR, is an RN first assistant for the division of urology and the division of orthopaedic surgery at the Children’s Hospital of Philadelphia, PA. Ms Kerr has no declared affiliation that could be perceived as posing a potential conflict of interest in the publication of this article. Allison Ludzia-DeAngelis, BSN, RN, is a clinical level III nurse and general, thoracic, and fetal surgery specialty nurse at the Children’s Hospital of Philadelphia, PA. Ms Ludzia-DeAngelis has no declared affiliation that could be perceived as posing a potential conflict of interest in the publication of this article.

EXAMINATION

4.2

CONTINUING EDUCATION PROGRAM

Fetal Myelomeningocele Repair: A New Standard of Care

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PURPOSE/GOAL To educate perioperative nurses about fetal myelomeningocele repair and developing a fetal surgery program.

OBJECTIVES 1. 2. 3. 4. 5.

Identify conditions for which fetal surgery may be performed. Describe the types of spina bifida. Discuss outcomes of spina bifida. Describe perioperative care of a fetus undergoing fetal myelomeningocele repair. Discuss how a simulation program can be used for competency development in a fetal surgery program.

The Examination and Learner Evaluation are printed here for your convenience. To receive continuing education credit, you must complete the Examination and Learner Evaluation online at http://www.aorn.org/CE.

QUESTIONS 1.

2.

Fetoscopic and open fetal procedures can be performed for some fetal conditions, including 1. bowel resection for necrotizing enterocolitis. 2. congenital diaphragmatic hernia. 3. myelomeningocele. 4. sacrococcygeal teratoma. 5. twin-to-twin transfusion syndrome. 6. vesicostomy or stent placement for obstructive hydronephrosis. a. 1, 3, and 5 b. 2, 4, and 6 c. 2, 3, 4, 5, and 6 d. 1, 2, 3, 4, 5, and 6 Spina bifida that occurs when the meninges herniate through an opening in the vertebrae during fetal development and form a sac that contains cerebral spinal fluid but no neural tissue is called

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a. meningocele. c. rachischisis.

b. myelomeningocele. d. spina bifida occulta.

3.

Early ultrasound studies performed throughout the pregnancies of women with fetuses identified as having myelomeningocele suggest that spinal cord injury and its symptoms remain consistent through the fetus’ intrauterine time. a. true b. false

4.

Primary outcomes of the Managment of Myelomeningocele Study were that fetuses that underwent fetal surgery had a decreased need for ventriculoperitoneal shunt at 12 months of age. a. true b. false

5.

Nursing diagnoses appropriate for a fetus undergoing fetal myelomeningocele repair surgery would include

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1. 2. 3. 4.

6.

the small blood volume of a fetus leaves little room for error. 4. postoperative infection in a fetus would likely be fatal. a. 1 and 3 b. 2 and 4 c. 1, 2, and 4 d. 1, 2, 3, and 4

decisional conflict. ineffective coping risk for deficient fluid volume. risk for infection. a. 1 and 2 b. 3 and 4 c. 1, 2, and 4 d. 1, 2, 3, and 4

For fetal myelomeningocele surgery, the ultrasound technologist performs an ultrasound to 1. determine the fetus’ and the placenta’s position before the patient has been prepped and draped. 2. determine the baby’s gender. 3. map the edges of the placenta and confirm the fetus’ position before the surgeon makes the initial uterine incision. 4. ensure that the fetus’ umbilical cord or other important structures are not in the way before the surgeon places the staples. a. 1 and 3 b. 2 and 4 c. 1, 3, and 4 d. 1, 2, 3, and 4

7.

If the skin edges cannot be approximated to cover the defect, the surgeon sews _________ into place to repair the defect. a. a polyethylene terephthalate graft b. a reconstituted regenerative tissue matrix c. a polytetrafluoroethylene graft d. an autologous split-thickness skin graft

8.

The challenges of fetal surgery include that 1. there are two patients. 2. the fetus does not have a mature and accessible airway, viable lungs, or good vascular access.

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3.

9.

Performing the myelomeningocele procedure involves the services of 1. anesthesia. 2. fetal cardiology. 3. general surgery and neurosurgery. 4. nursing. 5. obstetrics/maternal fetal medicine. 6. ultrasonography. a. 1, 3, and 5 b. 2, 4, and 6 c. 2, 3, 5, and 6 d. 1, 2, 3, 4, 5, and 6

10.

In this article, the simulation program for fetal myelomeningocele surgeries described includes 1. a multidisciplinary team. 2. a scenario designed using surgical dictation and procedural preference cards. 3. a scenario encompassing the procedure from the time the room and equipment were set up to discharge of the patient from the OR. 4. participants from all disciplines to participate in the simulation. 5. a “fetus” with a myelomeningocele and a foam “uterus” that fits inside a pregnancy simulation model. a. 4 and 5 b. 1, 2, and 3 c. 1, 2, 3, and 4 d. 1, 2, 3, 4, and 5

LEARNER EVALUATION CONTINUING EDUCATION PROGRAM

Fetal Myelomeningocele Repair: A New Standard of Care

T

his evaluation is used to determine the extent to which this continuing education program met your learning needs. Rate the items as described below. The Learner Evaluation is printed here for your convenience. To receive continuing education credit, you must complete the Learner Evaluation online at http://www.aorn.org/CE. OBJECTIVES To what extent were the following objectives of this continuing education program achieved? 1. Identify conditions for which fetal surgery may be performed. Low 1. 2. 3. 4. 5. High 2. Describe the types of spina bifida. Low 1. 2. 3. 4. 5. High 3. Discuss outcomes of spina bifida. Low 1. 2. 3. 4. 5. High 4. Describe perioperative care of a fetus undergoing fetal myelomeningocele repair. Low 1. 2. 3. 4. 5. High 5. Discuss how a simulation program can be used for competency development in a fetal surgery program. Low 1. 2. 3. 4. 5. High CONTENT 6. To what extent did this article increase your knowledge of the subject matter? Low 1. 2. 3. 4. 5. High 7. To what extent were your individual objectives met? Low 1. 2. 3. 4. 5. High

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4.2 www.aorn.org/CE

8. Will you be able to use the information from this article in your work setting? 1. Yes 2. No 9. Will you change your practice as a result of reading this article? (If yes, answer question #9A. If no, answer question #9B.) 9A. How will you change your practice? (Select all that apply) 1. I will provide education to my team regarding why change is needed. 2. I will work with management to change/ implement a policy and procedure. 3. I will plan an informational meeting with physicians to seek their input and acceptance of the need for change. 4. I will implement change and evaluate the effect of the change at regular intervals until the change is incorporated as best practice. 5. Other: ________________________________ 9B. If you will not change your practice as a result of reading this article, why? (Select all that apply) 1. The content of the article is not relevant to my practice. 2. I do not have enough time to teach others about the purpose of the needed change. 3. I do not have management support to make a change. 4. Other: ________________________________ 10. Our accrediting body requires that we verify the time you needed to complete the 4.2 continuing education contact hour (252-minute) program: _________________________________

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