>> Hi, I'm Dr. Natalie Rintoul, a Neonatologist at the Children's Hospital of Philadelphia.
If you are watching this video, you or someone you love is expecting a baby that has the
diagnosis of spina bifida.
Your baby will have postnatal surgery, which means that spina bifida will be closed after
the baby is delivered.
After the delivery, he or she will be transferred to a neonatal intensive care unit or a NICU.
While your baby's in the NICU, he or she will meet a number of people from a variety of
specialties — urology, neurosurgery, orthopaedics, and physical therapy.
The neonatologist will be the leader of the care team.
That person will coordinate the multidisciplinary care that every baby with spina bifida needs.
Babies with spina bifida typically are delivered by cesarean section.
Sometimes the NICU is in the same hospital where the mother delivers, and sometimes it is not.
The mother typically is in the hospital for several days.
During that time, there will be a NICU nurse taking care of your baby, and she or he will
be an incredible advocate, and really help to coordinate all the visits that will be
occurring from all the specialists.
The time while your baby is in the NICU can be a very scary time, and it really helps
to know what to expect.
In this video, we will review the things that typically happen while your baby is in the NICU.
In the first 24 to 72 hours, your baby will go to the operating room to have the opening
in the back closed.
>> When a baby is born with spina bifida, oftentimes at the site in the back there'll
be a little bubble of tissue.
Within that bubble, you can see the placode, which is the spinal cord that's connected
to the skin.
Some babies are born without this, and that bubble we call a myelomeningocele.
If they don't have that little bubble, but there's still an opening in the back without
that tissue surrounding the spinal cord, we call that myeloschisis.
Both of those conditions are treated exactly the same way by closing the tissue and the
skin over the back, usually the day after the baby is born.
>> At the time of delivery, a baby with spina bifida is essentially pre-op.
So, they are not able to eat.
So what we usually do is place an IV, and we give them IV fluid, which is essentially sugar
water to keep them safe before the surgery.
They're usually placed lying on their belly, and typically will have some warm saline-soaked
gauze placed over the cystic part of their back so that that doesn't dry out before the
surgery.
>> What typically happens with your child when they go to the operating room is they're
brought down to the operating room by the pediatric anesthesiologist or his team, and
then I get to do my job, which is closing the spina bifida lesion.
The first thing we do when the baby is brought into the operating room is we release the
spinal cord tissue from the skin.
Then, what we do is there's layers that are usually over the top of the spinal cord;
we cut those layers and close them midline.
A baby that has their lesion closed right after they're born is typically then brought
back down into the neonatal ICU and observed.
There's a very close interplay between the ICU team, the neonatal ICU team, and the neurosurgery
team to care for the baby.
The management of pain after a baby has surgery can be difficult, not because we don't have
medicines that can treat pain but, you have to have a nursing team and a doctor team that
recognizes pain in a baby that can't tell you they're in pain.
So, oftentimes, this requires a pretty specialized care, but the neonatal nurses are very good
at this.
So, they recognize by some of the factors, some of the vital signs of the baby, or if
they are used to looking at if the baby's in distress.
And pretty aggressive about making sure the baby's not in any pain.
A baby in pain doesn't do well, they don't heal well, they're agitated; this doesn't
allow their back to heal well.
So, the nurses and the doctors in the ICU are very aggressive about treating pain and
discomfort in these little kids.
>> The CHOP team here definitely prepared us for what could be, and they also prepared
us for what might be so that we might know what to expect, you know, in the days ahead.
>> After the delivery, they took McKenzie, our daughter, to the room that's off the side
of the operating room where Crystal delivered, and they got her cleaned up and checked out.
The neonatal care unit team was there, they checked her all out and prepared her for the
move down to the NICU.
The first 24 hours 'till she had the surgery to close her back lesion — we weren't able
to hold her, she was basically laying on her belly.
She was awake, I mean for the best she could be, and it was all so fast.
By the next morning, they were taking her into surgery to get her back closed up, and
it was a fairly quick operation.
She was back in the NICU within a couple hours recovering.
>> It really is a team effort of following these babies.
At most institutions, the neurosurgeon will follow the baby every single day until that
baby leaves the hospital.
We're monitoring for the babies to develop hydrocephalus.
Hydrocephalus is the buildup of spinal fluid, either from a blockage or from trouble absorbing
that spinal fluid, in a baby's head.
And that buildup of spinal fluid leads to a buildup of pressure.
The children will develop clinical signs of hydrocephalus about three to 10 days after
that back is closed.
Every baby is born with a soft spot, or a fontanelle.
When the pressure builds up, what happens is that fontanelle gets bulging outward.
Another sign is that the head in a baby literally gets larger.
The baby may have trouble with breathing, they may have a low heart rate, and they may
have drowsiness or vomiting.
And those are all signs that there could be something, a building pressure in the baby's brain.
>> As we started working with all the teams here at CHOP, they explained what we'd have
to do on a daily basis to keep McKenzie healthy.
We had to measure McKenzie's head just to be sure that it wasn't growing too fast, which
it was, and then we had the VP shunt placed.
>> The mainstay of treatment is trying to lower the pressure in the head, and we do
that with a device called a shunt.
What a shunt is, is a tube that's put into those fluid-filled spaces in the brain.
It's then connected to a pressure regulated valve which has an on/off.
The way I describe it to a lot of families is if you had a river that had a dam on it,
there are valves on that river that allow the fluid to go past when the water reaches
a certain level.
The same idea is happening with a shunt; it drains the fluid from an area where it's not
being absorbed to an area where it can be absorbed, down into the abdomen.
And the fluid's allowed to drain there and is absorbed naturally,
thus keeping the pressure low in the baby's brain.
The neurosurgery team will see the baby every day, looking for issues that are neurosurgical
and relying on the neonatal ICU team and the urology team and the spina bifida team, all
to take care of the baby in a comprehensive way.
>> The urology team will meet your baby in the NICU.
>> Amongst many problems that children with spina bifida face, one of them pertains to
how the urinary bladder works.
Your baby's bladder may not empty well, and the backup of urine can cause urinary tract
infection and serious damage to the kidneys.
That's exactly the situation that we want to avoid.
Two elements that are critical to preventing urinary tract infection, and first is to make sure
that the child's bladder is capable of emptying at low pressure, and thus preventing bladder overfilling.
And the second is that in many instances, we will recommend the use of low-dose prophylactic
antibiotic dosing.
What the NICU team wishes to do during your stay here is assure that your child's urologic
health is maintained in the short term during this stay, but also set you up to think about
what's necessary for the long term, and begin the process of educating you about how the
bladder works, and what the things are that you will need to do to maintain good bladder
health going into the future.
There are a couple of things that urology is expected to play a role in early on in
your baby's care in the NICU.
First and foremost is to have a look at what the bladder scans are showing to make
sure your child's bladder is emptying.
It's somewhere between 48 to 72 hours of life, your child will undergo an initial kidney
and bladder ultrasound.
And that's very important because what we want to get a sense of is what do the kidneys
look like at the start, and what does the bladder look like at the start of your child's
life, as this forms an important baseline.
Bladder scanning is done by the nursing staff, usually at two to three hour intervals.
And the purpose of that is just to make sure that your child's bladder is not becoming
overdistended, or overfilled.
A happy bladder is one that can store urine at low pressure and empty at regular intervals,
usually somewhere between two to three hours in the newborn period.
If we have evidence that the child is not emptying their bladder, that's a time where
we do need to step in to prevent bladder overdistention.
And we can prevent that by using intermittent catheterization.
A catheter is basically a medical straw passed through the urethra and into the bladder to
empty out the urine.
Many children with spina bifida are going to require intermittent catheterization in
order to empty their bladders and achieve urinary continence.
For a small group of these kids, that need for catheterization will extend on past your
discharge from the NICU.
The nursing staff are very, very good at teaching parents how to do intermittent catheterization.
They will make sure that your supplies are ordered, and that you really understand how
to do this.
Not just by showing you and telling you how to do it, but by having you practice yourself
with them there to guide you and coach you.
By preventing bladder overdistention through intermittent catheterization, I think a great
way to look at this is you're investing in your child's future bladder health.
What really will determine who gets put on a catheterization regimen early rather than
late is the video urodynamic study that we recommend be done somewhere when the child
is between 8 to 12 weeks of age.
And the purpose of that study is to make sure that the bladder storage pressures are low
and that the kidneys will be able to function safely.
During the first years of life, bladder function can change for the child with spina bifida.
So, we recommend that the study be done, and again when your child is about a year of
age, and then again at about 3 years of age, as we are approaching the years where potty
training begins to become a consideration.
What we are hopeful for is that by paying fastidious attention to detail early on in
your child's life, what we end up with is a child who has the biggest possible bladder
capacity, using the organ that nature has given them.
The less that we have to surgically do to a child's lower urinary tract in order to
achieve continence for your child, the better for all of us.
>> Once your baby's recovered from surgery, he or she will be assessed by orthopaedics
and physical therapy.
They'll look to see what type of movement your baby has and what type of tone they have
in their lower extremities.
Based on that, they will assign a functional level to your baby.
The baseline functional level is very important.
That establishes where that baby is, where they're starting out and the spina bifida
clinic will use that level and know your baby and know what they need.
>> Orthopaedics is the specialty really that deals with the musculoskeletal system.
And we work in conjunction as a team with physical therapists, brace makers and neurologists to
try to come up with a treatment strategy to get the child to meet his or her potential.
After you've given birth and your child's been taken to the NICU, a consultation for
orthopaedic surgery will be an examination primarily of the spine and the extremities,
looking at the bones, joints and muscles to try to get an assessment, an idea of whether
or not there are any other musculoskeletal conditions that go along with spina bifida.
These can include things like scoliosis, which is a curvature of the spine; hip problems,
such as dislocation or looseness of the hips; knee problems, like knee hyperextension or
knee flexion; and certainly the foot issues, such as foot positional problems, like clubfeet,
or where the feet are positioned where they are bent upwards.
Treatment for clubfoot typically starts with casting.
So, serial casting you stretch out the foot and ankle for a few minutes and then with
the foot held in the correct position, we apply a cast.
And what that does is that holds the foot in position, allowing the tissues to stretch out,
allowing more range of motion to be achieved.
And we take the cast off in about a week's time, reapply it, restretch it out, gain a
little more correction in each week.
Typically, serial casting is used for any sort of contracture where the joint is tight.
The most common, obviously, is for clubfeet, where we do serial casting, changing the cast
every, typically every week.
And most of the orthopaedic issues can safely be observed, and usually is not any emergency
to them.
The issue I'd like to address while in the NICU is if the knees happen to be hyperextended
so that the ... instead of being bent, they're actually extended.
So, sometimes the top of the foot can be touching the child's head or in sort of uncomfortable
or worrisome position.
I'll address that usually right away with casting.
Most other conditions I find or associated findings can safely wait until the child is
healthier and more definitive care can be established.
The families have lots and lots of questions when I examine the babies, as you'd expect,
as is normal and appropriate.
And generally, there's two kind of types of questions I find.
First, are sort of the short term, if you will, problem-focused issues, what will you
do about the child's clubfoot?
What's the plan?
What's the plan for further evaluating or treating my child's hip or spinal issues?
The second general category I find is the families have, again, appropriate questions
about longer-term issues.
Will my child walk?
Will they need braces?
What kind of further orthopaedic surgery will they need?
And that's, I find much more difficult to answer with any certainty, and so, I tend
to try not to make any firm predictions.
It's really is a question of constant re-evaluation and trying to see what the child's needs are
and how we can help, allow the child to fulfill their potential.
>> I think part of being in the NICU is parent education, and the neonatal nurses are really
very important for making that transition from the ICU to home.
>> Transition is very important for spina bifida patients for the mere fact that they
are handled under many surgical subspecialties.
My role as coordinator begins at the bedside.
We need to make sure that all subspecialties appointments are made so that there's no lapse
in care, as well as coordinating and communicating with your pediatrician, early intervention,
insurance companies.
So, it's a well-rounded approach.
We just don't focus on the medical issues that take place acutely in the NICU.
We're looking forward as to how we can pave the path to make it easier for the family.
So, when they go home, they just need to be parents.
>> In the NICU, we felt very supported, we felt very comfortable.
We almost felt like it was kind of an extended family.
The nurses were so caring; the doctors explained everything 100 percent.
If we had a question, they didn't leave until we had an answer that we were happy with.
>> Some of the best questions that you can ask as an expecting parent would be, what's
going to happen directly after birth?
Where is the baby going to be?
Am I able to see the baby?
>> Some families wonder how long they'll be in the NICU.
We typically say about two weeks.
Some kids are a little bit faster, some take a little bit longer.
During that time, we really try to have families meet the multidisciplinary care team that
they are going to have a lifelong relationship with.
It is just the very beginning.
Our role in the NICU in those first couple of weeks is just to try to make everything
as normal as possible, and support the family so that they can be ready and prepared to
take their baby home.
>> Just take one day at a time and just enjoy all of the milestones that come, just like
any other normal baby.
McKenzie does physical therapy once a week. And she's now standing, she's taking steps
with her walking toy.
She wears AFOs.
She attends day care just like all of her other little friends that go to day care, and she's
happy and she's healthy, and she's smart.
And she's just a joy to have.