Surgery for Adria

"Mom, you shouldn't have surgery. It's really boring," Adria said tonight while lying on the couch.

Sit as little as possible and lie down at home for almost a week. No swimming for 2 weeks. No sports for 4 weeks. That's the doctor's orders after Adria's surgery on Friday. It's not ideal for our little seven year old, who's disappointed to be missing a week of fun at the end of the school year, but we are so thankful she's doing great!

At home Adria has not been in pain and seems to be recovering well, but she needs to limit her activity and time sitting for her body to heal properly. Especially considering, the doctors encountered a little surprise during her surgery.

She has been pretty fortunate when compared to many children with spina bifida, as this is the first surgery she's had since she was only six months old. But now she is old enough to be aware and apprehensive of what's involved with "surgery."

Johanna and Adria painting picture frames. Trying to find fun things to do while lying down can be tricky,
but we are trying to keep Adria still this week to allow her body time to heal after surgery.

Why did she need surgery?

You are probably asking this question (especially if you missed the recent post Steps Forward...and Back).

I'll try to summarize without getting too detailed. Adria lacks sensation on some areas of her bottom and feet, and early this year she developed a sore that became an open wound in the high pressure area where she sits. We've been treating it daily and seeing doctors for months, but it simply would not heal.

Finally, the plastic surgeon said it was time to stitch it closed. She had wanted to give it time to heal naturally, because with stitches, there's still a risk it could open again and possibly be worse.
Thankfully, the wound had never gotten infected. Yet, after almost four months, it was time to take action.

For the average person, this wound would be very painful. But this type of wound does not usually develop because we feel pain when the pressure gets too much, and we adjust our sitting position accordingly. Adria lacks the nerve endings to give her these cues, thus leading to this problem on her bottom.

Telling Adria

We did not tell Adria about the surgery until a few nights before Friday. She likes to know what is happening, but we knew she would be anxious about it, so we waited.

Those two nights before, she was pretty weepy and nervous. I think it was more the fear of the unknown and being in a room without us. The hospital had a helpful video for children to watch in preparation for surgery, but Adria was still afraid. Thankfully, she was still able to rest well and be distracted at school.

Surgery Day Surprise

We did it. We woke up at 4:30 a.m. on Friday and made it out the door around 5:30 a.m. That's kind of amazing for us.

Adrian and I headed north to A.I. duPont Hospital for Children with Adria. Johanna and Judah woke up early with the commotion, but stayed home under the watchful care of Adrian's mom.

The children's hospital really does a great job of putting children and parents at ease. Adria got her arm bands, and they even gave one to her special bear friend Foo-Foo! She loved on that bear and her turtle friend Celia, who both went back to surgery with her, while mommy and daddy waited outside.

Adria smiles just a few minutes before going back with the anesthesia team.
Her "babies" Foo-Foo and Celia were great comfort to her.

Adria and her babies.

The pre-op area was a buzz of activity with dozens in blue scrubs milling about. There were many questions and vitals taken before Adria went back for the anesthesia. They gave her some calming medicine before they took her back and next thing I knew, her eyes were droopy and distant. I think that's the last thing she remembered until we saw her again about 2 hours later.

Waiting is tough. You know your child is being operated on and you just wait. They have a screen with colors and codes for you to follow your child's general progress, but you'll go crazy if you stare at it too long. We had a nice interruption during our wait, as a friend just happened to be up there for an appointment with her son at the same time.

Surgery started around 9 a.m. A lady gave us an update halfway through, then sometime after 10:30 a.m. the plastic surgeon came out to talk.

It was finished! Adria was doing good but still needed time to come out of anesthesia.

The surgeon explained that while operating, they found that Adria's tailbone was just under the skin where the wound had occurred. That helped explain why the wound struggled to heal, because of the extreme pressure from the unusual angle of her tailbone. So they removed it!

She said it was about an inch of bone that was non-essential for Adria and would not be missed. It should also help prevent this issue from happening again.

Adria no longer has a tailbone!

That was our surprise, but it seems like it's no big deal. Her tailbone was at a much different angle than normal, so it caused more harm than good.

Recovery

We went back to the PACU (Pediatric Anesthesia Care Unit) to be with Adria while she continued to come out of anesthesia. Poor thing was so dazed and sleepy for a while. It upset her. Even the blue raspberry ICEE slushy they gave her did not help much. She was very surprised to realize that the surgery had already happened and almost disappointed that she did not remember it. Silly girl.

She lie in the hospital bed with an IV in her arm cuddling her Foo-Foo. For a minute, my imagination took over and fast-forwarded 20 years and thought of seeing her in a bed cuddling a newborn baby. That was weird. And kind of emotional.

Around noon, Adria was improving enough to be discharged and put in her wheelchair. She was able to eat some lunch before we headed home.

We THANK everyone who prayed for her and showed support through text messages and Facebook love!!! Adria appreciated reading the messages with me before and after her surgery.

She even got a surprise package in the mail from her school nurse this weekend! It held some activity books to help keep her occupied during this "boring" recovery period.

We are so thankful she is recovering well!

Treasured Times

Judah is 3 weeks old today! If I'm totally honest, I cannot believe how "easy" these first few weeks with Baby Judah have been. He's already been to the Zoo, the Beach, and a Strawberry Festival (since Mama has recovered well.)

Now before you get jealous or annoyed at our good fortune, hear me out.

I look at him now, sleeping peacefully in the small cradle by our bed with an adorable head full of dark hair, precious closed eyes, perfect little lips, and a pudgy nose that clearly comes from my side of the family. I look at him and have a heart full of gratitude.

Daddy and Judah relax with some skin to skin time.

We have a son. A healthy, calm-tempered, beautiful baby boy.

He sleeps well and often. He nurses like a champ and has since the day he was born. He rarely spits up or has massive diaper blow-outs. His skin is dark, clean and clear. His eyes are wide and alert when he's awake. He's pretty patient with his adoring older sisters. What more could a mother ask for?

Precious baby Judah

As the mother of three children, I know that it's not always this good. And I know that this precious newborn phase lasts ever so briefly. Some parents are relieved when it passes because it can be so difficult. But this time around, I want so desperately to treasure this sacred time. Especially since this will likely be the last time I ever experience this phase.

My expectation was that these first few weeks would be so difficult. That's how I remember them with my daughters. They were precious times too but they came with so much more drama. And when I hold Judah, I cannot help but compare his first couple of weeks with his older sisters...

Johanna was born in December in Indianapolis, In. We were 600 miles from our families, though they did come out to visit and help as they could. The support network was so much smaller out there than what we've experienced here, and I felt sad that so few people had the chance to meet Johanna the first month of her life.

While Johanna was an adorable baby, she struggled more with nursing at first and spit up A LOT. I was constantly changing her...and myself. She was very restless at night and kept us awake many nights.

But the most difficult memories about her first weeks and months of life was the fact that it was the worst "season" of Adrian's seizures. During the two months I was home on maternity leave, my husband had three grand mal seizures that left him impaired physically and mentally. Because of that, he has almost no memory of holding her as a baby :( And in truth, he did not hold her much.

And I struggled with the Baby Blues. Maybe it was because of the seizures, or the challenges with Jo-Jo, or the dreary winter weather, or the on-slaught of medical bill issues that arose in the midst of it all. Whatever the case, I battled post-partum depression for the majority of the first year of Johanna's life.

Welcoming Baby Johanna in December 2011

Adria holding baby sister Johanna for the first time.

Adria was born in September in northern Delaware. The first two weeks of her life, she and I were both recovering from surgery, my C-section and her spina bifida back closure. We spent every day sitting in the NICU at A.I. duPont Hospital watching her and as she improved, we gently held her and tried to avoid getting tangled in the wires connected to our tiny girl.

We spent our nights sleeping across the street at the Ronald McDonald House. I don't think we drove the 1.5 hours home at all in the first 12 days of her life. We didn't want to leave our baby that long.

Daddy with baby Adria soon after her birth.

We were inundated with information about her spina bifida: the need to monitor the fluid on her brain, how to catheterize her if needed, watching for leg movement, and when she would be able to lie on her back once her wound healed.

It was overwhelming at times, yet still we felt thankful as we heard stories of what other parents were experiencing with their babies in the NICU. Some had been there for more than four months already.

Treasured Times

Now, we have Judah who has been such a healthy boy. Our love for him is certainly no more than his sisters, but we sure do appreciate how good he's been to us! Plus, we have been so blessed with meals and gifts from our friends, family and neighbors. What a treat! Tonight I cooked dinner for the first time since he's been born.

As I hold another little baby in my arms, I try desperately to impress on my memory these treasured times. And I enjoy the ease of holding Judah without worrying about wires or back wounds or getting regularly covered in spit-up.

But most of all, I love watching his Daddy and sisters hold him and love on him. Seeing them so happy with this little guy is beautiful. 

Sisters loving on the Baby Brother they had hoped for!

I hope Judah keeps his relaxed and chill demeanor throughout his life. Of course, I know quite a few toddler and pre-school boys and realize there is a lot of adventure to come!

The Incredible Shunt Alternative for Hydrocephalus

I still remember sitting in the hallway of A.I. duPont Hospital for Children and hearing that CRY. It was one of the most beautiful sounds I'd ever heard. It came from around the corner and before I could even see her, I knew it came from our six month old baby girl. It was the sound that reassured me Adria would be okay after having just undergone head and brain surgery.

Six month old baby Adria that Monday morning before her surgery.

That morning was exactly six years ago today, but I still get emotional thinking about my husband and I sitting in the parent waiting room for a couple hours while our precious little Adria become only the eighth child in the U.S. to have a unique and revolutionary procedure done to treat her Hydrocephalus.  This build up of fluid in the ventricles of the brain is very common in children/babies with spina bifida (Adria has L4-5 myelomeningocele), but the typical treatment is a VP Shunt, which is a tube inserted into the brain to allow the excess spinal fluid to drain down into the abdomen. While this helps the issue, most children (and adults) with a shunt will have complications and malfunctions over time that can require multiple surgeries to correct.

This image shows what a Shunt looks like in babies. Many with spina bifida need this,
but Adria never did because of the ground-breaking procedure her pediatric neurologist performed on her.

Instead of having a shunt to treat her hydrocephalus, Adria's neurologist recommended a procedure called Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization (ETV/CPC). Yeah...try to remember that name, let alone explain to someone what it means... But the fact is, this procedure successfully treated Adria's hydrocephalus and six years later, she has experienced NO COMPLICATIONS from it or every required another surgery!!! This is truly incredible. The only evidence that she even had an operation is a small scar buried in the hair atop her head.

Adria's Recovery

Soon after we heard the familiar cry in the hallway six years ago, we saw our sweet baby and held her in the Recovery Room. It was a large room full of young patients just coming out of surgery with just curtains separating us. Unfortunately, Adria's cries would not stop because she was so hungry. In preparation for the surgery, she had not eaten since the night before and the IV nourishment just didn't cut it for her.

The nurses were concerned about her throwing up if I fed her a bottle. But I started to be in extreme pain too...any mother who has nursed her baby and heard the cries of a hungry baby knows what I mean. Before too long, a nurse heard me complain about it and asked, "Oh, do you still breast feed her?" I told her Yes, and the nurse said, "Well then she can eat! She should be fine to nurse, breast milk is okay for her tummy." Praise the Lord! At that moment, I was more thankful than ever that I could nurse Adria. Within minutes, we were all Much Happier!

As Adria recovered from surgery, she did experience a lot of swelling on her head and looked so different. But Daddy held her in his arms for hours at a time and she recovered well and was transferred from the PICU (pediatric intensive care unit) to a regular patient room late that night.

Adria in the PICU after surgery with swelling on her head and paddles on her hands to hold the IVs in place.
Good thing she had her Soothie pacifier to provide extra comfort!

Daddy giving his baby girl some healing love.

Adria recovering in her patient bed that looked like a giant cage.

Within a few days, we were headed back home with a happy little girl who just had a little bald spot of her head where the surgeon had performed the operation.

The Man Behind the Surgery

Occasionally in life we find ourselves blessed and touched by an individual simply because we were IN THE RIGHT PLACE AT THE RIGHT TIME.

Now I don't necessarily believe this is coincidence; it's more like divine appointment. And for our family, this happened at a most critical time in our lives.

We met Dr. Benjamin Warf when I was five months pregnant with Adria. In the weeks following the ultrasound that first revealed our baby girl had spina bifida, we found ourselves sitting in front of many different doctors and specialists...desperate to learn and understand more about this unique life I carried.

Dr. Warf sat there with us in a tiny room at A.I. duPont Hospital for Children in Wilmington, Del. His kind and humble demeanor immediately put us at ease, even as our minds buzzed with the information he provided. We did not realize it then, but I know this to be true now, he is one of the BEST PEDIATRIC NEUROSURGEONS in the World! This is no exaggeration.

He is the father of six children and actually pioneered the ETV/CPC procedure to treat hydrocephalus while serving as a missionary doctor in Africa for six years. He realized the need to treat this condition without a shunt, particularly in a developing country where a shunt malfunction can quickly become life-threatening. He had fairly recently returned to share this procedure in the U.S. when we first met him.

Warf remained at A.I. duPont Hospital only a short time, but he was there to operate on our daughter's spina bifida closure surgery at birth and this surgery six months later. That following summer he moved on to Boston to further develop and teach this new procedure to medical students at Harvard and work at Boston Children's Hospital. Before he left Delaware, he did teach the ETV/CPC to another neurologists who is still at A.I. duPont Hospital performing this life changing surgery on children.

Even today, six years later, this procedure is still NOT widely known or used in the medical and Spina Bifida communities. I am a HUGE supporter of it and highly recommend any parent research whether this is an option for their child!

Since it's very difficult to explain what this surgery exactly is, I'll let Dr. Warf do the talking.

I just found this great video where Dr. Warf explains Hydrocephalus, a shunt and the benefits of the ETV/CPC procedure! Take a look:

New Years Letter - 5 Years Ago

Tonight we removed the decorations from our Christmas tree as the holiday season ends. As we look back to 2013 and the surprises it brought with our sudden return to Delaware, we are most thankful that Adrian got his Christmas wish and had no seizure this holiday. We look forward to 2014 and hope that it is "the year of recovery" as Adrian said today.

As we hope and pray the best for each of you this coming year, I'd like to share a letter that I emailed out to friends and family on New Years Day 2009, five years ago.

New Years Letter 2009

I often try to send out a letter with my Christmas cards, but even though the most amazing thing possible happened this year (having our precious little bundle), I somehow never made time for a letter. So on this first day of 2009, I’d like to take a few minutes to fill you in on the latest at our household and wish you a Happy New Year!

My siblings and I as my youngest brother Aaron graduated from high school in 2008.

This photo was taken just weeks after we learned the baby girl I was carrying had spina bifida.

Here I am 7 months pregnant with dear friends, Summer of 2008

For the first time in my adult life, I did not stay up until midnight last night to welcome in the new year. With baby girl tucked away in her crib, we did not feel compelled to stay up on our couch and watch the giant ball drop in New York – not really that exciting. However, soon after midnight a cry from the nursery had me up welcoming the new year mommy style. Adria has actually been a champ about sleeping through the night for the last couple months (often from 9 p.m. to 7 a.m.), but something’s been waking her the past few nights, maybe teething or a little cold.

Our Family Photo Christmas 2008

 

She is really an amazing little gal. Smiling and laughing at us often now, and she’s learning to use her vocal cords to make a wide range of very audible vowel sounds. We love conversing with her. In early December we had another huge answer to prayer. We took her to AI Dupont Hospital for her monthly head ultrasound and visit with the neurosurgeon. There was a good chance she would need surgery if the fluid on her brain increased again, as it gradually had been. We were very nervous, but praise God, there was no increase, so now she most likely will not need surgery! At that visit, we also learned that she was almost 16 pounds. Yes, our little sweetheart has more “natural cushioning” than most babies her age, and we love every pound of her. (Adria was just four months old when I wrote this.)

 

As for Adrian and I, we celebrated 5 years of marriage on Nov. 22, and even managed a weekend getaway with baby to a charming log cabin in the Poconos. Adrian is an exceptional stay-at-home Dad, providing excellent care for our daughter, cleaning the house, washing laundry and occasionally making dinner as well as doing some side jobs and many projects around the house (impressive, I know). This has helped my transition back to work in November go fairly smoothly. However, as a new home sales rep for Ryan Homes, it’s not exactly an easy time to sell. I actually had a great year with them and have been thankful but am hopeful that 2009 can bring better economic times. (It was actually much worse in 2009 and 2010...as we now know.)

This is the townhome community in Salisbury, MD where I was sent to sell homes after Adria's birth.

 

For now, our little family will continue with our current lifestyle and be amazed as we watch the growth and development of our Precious Angel (who, by the way, is in my lap chatting to me right now).

Blessings to each of you this year!

Julleanna on behalf of the Seely Family – Adrian, Adria Mychelle, and Max 

 

To view more photos from 2008, click on this link: http://www.new.facebook.com/photo.php?pid=5268691&l=6406c&id=749275133

 

12 Days in the NICU, so long yet so short

Baby Adria spent the first 12 days of her life in the NICU (Neo-natal Intensive Care Unit) at A.I. DuPont Hospital for Children. Really, we were fortunate it wasn't more because many babies have to stay much longer. For instance, my friend Amanda's preemie little boy was recently in the NICU for 35 days and some of the mom's we met during our time in the NICU had been there for months with their babies.

I ached for them because even just 12 days can feel like forever when that is your whole world. 

Her first three days in the NICU, I was not with her. It was a heart-wrenching experience to deliver my newborn baby and have her taken away from me for days after only getting to see her a few minutes. As I've mentioned before, she had to have spina bifida closure surgery at the children's hospital about 20 minutes away, while I had to remain at Christiana Hospital for a few days to recover from my own surgery.

It confused my body. Other than the piercing pain in my gut from the C-section, my body had no proof that my baby had come out. I had never gone into labor or had a single contraction that I felt, and there were no cries or coos from a newborn baby around me.

While it was painful during those days to be apart, I was comforted knowing I would see her soon. I couldn't help but think of mothers who do not have that comfort... Plus, Daddy was there with our little girl, while my mom offered support to me.

Daddy in the NICU while the nurse tended to Adria

I wanted desperately to be able to give our baby mommy's milk and the early colostrum that they call "liquid gold" for newborns. But, my body did not think it was time for milk yet. I tried to pump every three hours, but during those first few days only a few drops came out. The nurse would still put it in a tiny syringe, and have it taken up to A.I. DuPont Hospital where Ade could help feed it to her.

However, Adria was not allowed to eat anything her first day of life before her surgery. She was not happy about that! She had an IV that nourished her body and was quickly introduced to the Soothie pacifier, which Ade said she sucked on feverishly. That was the start of a special three year relationship between her and Soothie :)

Adria was blessed to have one of the best pediatric neurosurgeons in the world to operate on her back and put the exposed spinal cord back in place. Dr. Benjamin Warf was only at A.I. DuPont Hospital a couple years, but he was there when my baby needed his especially gifted hands and brain. I will share more about this incredible doctor another time.

Baby Adria still sleeping from the anesthesia on the day of her spina bifida closure surgery.

Her surgery did go as well as we could have hoped, and she was able to take formula from a bottle within a day. We were very fortunate that she did not need a feeding tube or oxygen like many NICU babies. There was a 3 or 4 inch area on her lower back wear she had stitches from the surgery, so she had to lie on her tummy only for several days.

She was born on Monday, had surgery on Tuesday, and on Thursday I was released to go to my baby girl. Praise the Lord!

From the time I got to the hospital that day until she was released over a week later, we did not go home. We spent every day in the private room she had in the NICU. We ate meals in the hospital cafeteria, where I was given a free pass for food since I was a "pumping" mother. The only time we left the hospital during those 12 days was at night to sleep in our room at the nearby Ronald McDonald House. Thank you, if you've ever supported that charity! We were able to stay there for less than $20 a night, and it was a blessing even if we could not sleep that well.

Our only break out into the "real world" was a couple times when friends came to visit and treated us out to dinner. We suddenly remembered there was more to the world than our little baby and her hospital room. It was a blessing to have many friends and family come welcome our little girl. They'd have to take turns coming in as only two visitors (including me or Ade) were allowed in her room at any time.

Aunt Leona, who would become a huge help over the years with Adria!

Mommy holds baby for first time without pillow

Ahh, Daddy loves his little girl

Granny Seely falling in love

Uncle Cameron, who is the best gift-giver and ultimate fun

Uncle Aaron, who can always make Adria laugh

Mommy's cousin Clairissa

Gramma Outten loving on her newest grand-daughter

Grandpa Outten gives unconditional love

Mommy doesn't mind the cries

Lovin' her Soothie

Mommy's friend Amanda

Mommy's marathon friend Lori

Mommy's friend Rita

Super sweet Aunt Bonnie

We did not have to stay at the hospital all the time, but we wanted too. Just to sit there and hear her breathing and look at her precious features kept us interested. Plus, doctors regularly came by without notice and had important updates and valuable information about her progress that we didn't want to miss.

She underwent many tests during those 12 days, but the most important were the ultrasound and then MRI of her brain. The ventricles of her brain were slightly enlarged with fluid, so they were monitoring her mild hydrocephalus to see if she would need a second surgery on that before going home. Thankfully, she did not and we were able to bring her home once Dr. Warf determined that her back was healed enough to ride in a car seat.

They also showed us how to catheterize Adria to empty her bladder if necessary, but the tests on her bladder and kidneys did not indicate that we would definitely need to do that.

Though the days felt long sitting in that hospital room, we couldn't imagine driving home an hour and a half away and leaving our precious baby. We so appreciated the great care that she received at the hospital, and it gave me the opportunity to rest and heal from my surgery as well. But, when they gave us the okay to be released, we were more than happy to be packing up and heading home with our newborn!

What exactly is Spina Bifida?

When it comes up in conversation that my daughter has Spina Bifida, people usually find polite ways to say they are not too familiar with what that is. I am never surprised by this, as I would be in the same place had it not become so personal to me.

The hard part is, coming up with a quick and simple answer to the question - What is spina bifida?

As is the case with many medical conditions, particularly those that occur before birth, an easily explained definition is hard to come by. Even for we parents, it is an on-going education as our child grows.

That said, here is my best attempt at a mother's quick answer to this question: She was born with spina bifida. It happens early in the pregnancy when the spinal cord doesn't form properly, which leads to nerve damage that can affect many areas, specifically her ability to walk and run.

Ok, that was easy enough. That answer satisfies most inquisitive minds. However, for the close friends who want to sit down and chat over an after-dinner coffee, the answer is much more complex and detailed. Since I won't have the opportunity to sit with most of you, go ahead and pour yourself a cup of coffee and read on.

Here is an education on spina bifida from my non-scholastic perspective.

The term spina bifida literally means "split spine" in Latin where the spinal column or neural tube does not close all the way while the baby is growing inside mommy. This occurs just about 28 days after conception, usually before mom even knows she's expecting. The cause is unknown and there are many different types and levels of severity when it comes to spina bifida, but the facts are that it is the most common permanently disabling birth defect.

Spina bifida is estimated to occur in 1 out of every 1,000 births worldwide, with a slightly lower incidence rate in the U.S. The Spina Bifida Association claims that every day about eight babies born in the U.S. have spina bifida or a similar birth defect of the brain and spine.

The image below comes from the Mayo Clinic website, and shows the progression of a life beginning to form and how spina bifida occurs.

Those nerves in the spinal cord that never formed properly to begin with, cannot magically grow back into place and start working. Children can learn to function well and adapt to lack of sensation in their limbs and limited bowel/bladder function, but whatever the initial damage is remains. Modern medicine has progressed significantly over the past few decades to help prevent further damage to the spinal cord. Just 100 years ago, the life expectancy and quality of life for someone with spina bifida was significantly worse.

The types of spina bifida have such a broad range that those with the more mild forms may not even know they have it, while others with the most severe are completely wheelchair bound and paralyzed.

I will refer to an information sheet from the Spina Bifida Association of America, to explain the different types:

Occult Spinal Dysraphism (OSD)
Infants with this have a dimple in their lower back. Because
most babies with dimples do not have OSD, a doctor has
to check using special tools and tests to be sure. Other
signs are red marks, hyperpigmented patches on the back,
tufts of hair or small lumps. In OSD, the spinal cord may
not grow the right way and can cause serious problems
as a child grows up.

Spina Bifida Occulta
It is often called “hidden Spina Bifida” because about
15 percent of healthy people have it and do not know
it. Spina Bifida Occulta usually does not cause harm,
and has no visible signs. People find out they have it after
having an X-ray of their back, normally done for other reasons.
However, in a small group of people with SBO, pain and
neurological symptoms may occur.

Meningocele
A meningocele causes part of the spinal cord to come
through the spine like a sac that is pushed out. Nerve
fluid is in the sac, and there is usually no nerve damage.
Individuals with this condition may have minor disabilities.

Myelomeningocele (Meningomyelocele), also called Spina Bifida Cystica
This is the most severe form of Spina Bifida. It happens
when parts of the spinal cord and nerves come through
the open part of the spine. It causes nerve damage and
other disabilities. Seventy to ninety percent of children with
this condition also have too much fluid on their brains.
This happens because fluid that protects the brain and
spinal cord is unable to drain like it should. The fluid builds
up, causing pressure and swelling. Without treatment, a
person’s head grows too big, and may have brain damage.
Children who do not have Spina Bifida can also have this
problem, so parents need to check with a doctor.

Unfortunately, the most severe type is also the most common type of spina bifida and that is what our little girl has - myelomeningocele.

As the image above demonstrates, with this type of spina bifida, the baby's spinal cord literally protrudes from her back in sac, or a bubble as we try to explain it to our 4 year old.

This sac can protrude anywhere from the lower spine, which is most typical, to one of the vertebrae at the top of the spine. The higher the lesion is on the spine, the worse the permanent damage. Amazingly, a child's function can vary significantly even if it's just one vertebrae higher on the spine. At the children's hospital, the doctor recently referred to Adria as "one of our L-4/L-5 kiddos." That is where her spina bifida occurred in the lumbar region of the spine and those that know about it, have a good sense of her abilities with just that statement.

The below diagram of the spine from spinabifidaassociation.org, details the different regions and expected functional abilities based on where the spina bifida occurred.

Now, all that may seem like a very lengthy, detailed description of this life-altering condition. Really this is just the beginning.

Because the spinal cord is the core of our central nervous system, damage from spina bifida can be far-reaching. This includes problems with walking and getting around, ability to go to the bathroom, hydrocephalus, gastrointestinal disorders, obesity, latex allergy, tendonitis, depression, sexual issues, and possible learning disabilities.

As the doctors say, every child is unique and no two individuals display all the same symptoms and challenges.

It is a daily education for us to learn our daughter's needs and how to best equip her for a full and fulfilling life. Through it all, there is one thing that remains the same, one thing that every parent can do to give their children the best life, disability or not. SHOW them LOVE.

Adjusting to our daughter's official diagnosis

Follow-up to July 30 post "The Worst Question I have ever been asked."

May 6, 2008 was a long and emotionally exhausting day. We had just spent the morning at the maternal fetal medicine specialist in Annapolis learning about spina bifida, getting asked that horrible question, and seeing first-hand 3D ultrasound images that showed our baby had an abnormal protrusion from her lower back.

After the ultrasound, the doctor shared the positive news that our baby's heart looked good, her arms and legs were very active, and her hands and feet were open and moving around, which is good because often spina bifida can cause clubfoot. He confirmed the diagnosis, that she has spina bifida (myelomeningocele) with an exposed opening in her back which usually does result in some level of paralysis, loss of bladder control, and excess water on the brain. However, the lesion is low on her back and is not likely to cause extreme paralysis.

With the official diagnosis on my heart and mind, I found myself curled up into an almost fetal position in the backseat of the car as we traveled home. I did not have the strength to talk about it just then, it needed time to sink in that my baby would likely be handicap to some extent.

I was so thankful that Doris, our pastor's wife, had come with us to offer comfort and prayer support as well as a clear head for driving home. My husband and I felt drained.

We stopped at a grocery store along the way to pick up a few things and get some lunch. But I had no appetite, I wandered in a haze and feeling incapable of making even a small decision, like what I wanted to drink.

Then I saw a sweet, young girl standing in a check-out line with her mother. She looked at me, and I could not help but wonder - will my daughter ever be able to stand or walk? I nearly lost it right then.

In just a day's time, our world entered an all new dimension to us and our priorities suddenly shifted. I was employed as a sales representative, and I had skipped our company's annual meeting and awards ceremony that day to meet with the specialist. Later, I learned that they had given me the new home sales Rookie of the Year award for our division that day. A special honor, but it did not carry the significance that it normally would have. I had gotten much bigger news that day.

I would continue to work and sell homes, but I knew that giving our baby the best life would require much of us, and that was okay.

That evening I penned these words in my journal:

"I am not sad or disappointed, I love the little one in me more than ever. I think we both just feel overwhelmed with the unexpectedness of it. But I just hold to the knowledge that God knit her together in my womb and has a purpose and plan for her life. I just pray that she will be able to have a happy and fulfilling life no matter what."

Our prayers are being answered every day. At 4 years old, she now walks around like a champ and has no obvious handicap! This is actually very rare for a child with her diagnosis.

Below are some photos of Adria's baby book pages that talk about that day and show 3D ultrasound images. By the way, I don't scrapbook often, but her precious story gave me motivation to do this while I was on maternity leave with her.

 

 

The WORST Question I've ever been asked. The answer is NO!

This post is a follow-up to my July 18 post titled "That day at McDonalds when I got the call...that changed us."

"Do you want to terminate the pregnancy?"

This horrible question was asked to us the day after our world was rocked with the call from my OB saying that it appeared our unborn baby had a abnormality of the spine. We found ourselves sitting in the office of a maternal fetal medicine specialist in Annapolis, MD. If you have never heard of that type of specialist (like me at that time), they are the doctors who help women with high-risk pregnancies. That includes moms who’ve had multiple miscarriages, those expecting twins or more multiples, women over 35, and those of us with babies who will require extra special care.

We had asked our pastor’s wife and friend, Doris, to come with us and provide emotional and prayer support as we stepped into this unknown world.

Honestly, I felt pretty out of place as I sat in the waiting room. The other women in there all looked at least 10 years older than me and way more pregnant. At 26 years old, I was pregnant for the first time and my 5’7” frame mostly hid the fact that I was already five months along.

Here I am 5 months pregnant with my first child. This photo was taken May 17, 2008
about 2 weeks after I learned about Adria’s diagnosis.

Finally, it was our turn to talk with the genetic counselor. I had never heard of them before either, but a genetic counselor is a unique healthcare professional with training in both medical genetics and counseling who assist in educating and supporting families with high-risk pregnancies or those at-risk for complications. According to the National Association of Genetic Counselors, they discuss the medical, psychological and familial implications of genetic contributions to “diseases” by:

• Asking detailed questions about family and medical histories to consider what may have caused this issue and what’s the future likelihood
• Educating about inheritance, testing, management, prevention, and resources
• Counseling to promote "informed choices" and adjusting to the risk or condition

So there we sat, listing every member of our large families and any health challenges. Yes, there is a history of cancer and high blood pressure, but no known disabilities.  We were healthy with my only issue being a tendency to easily faint when in physical or emotional pain.

Bottom line, we had no apparent increased risk to bear a child with spina bifida, but in most cases of spina bifida there is no known family history. It can just happen.

So what is spina bifida? That came next. I’ll elaborate more another time, but in layman’s terms it’s when the baby’s spine does not form properly early on in the pregnancy and can cause the spinal cord to protrude from the baby’s back in a sac. Because critical nerves are never formed properly – spina bifida often impacts many functions of the body and can cause paralysis, lack of bladder and bowel control, fluid on the brain, educational delays, deformed limbs and more.

It affects each person differently and is actually the most common permanently disabling birth defect. There are different types, but the most common - and most severe type - is myloemeningocele. That is likely what our baby has, since we were sitting there that day.

This image shows what spina bifida (myloemeningocele) can look like on
a baby in the wound. Provided by Children's Hospital of Philadelphia website.

Deep breath. Too much information. Did not know what to say but still held out hope that they might be mistaken.

 

After all the questions and discussion, came the worst question I have ever been asked.

 

“Given this information, do you want to terminate the pregnancy?”

I liked our genetic counselor, but she may as well have asked, "Do you want to kill your baby because she's got some serious issues?"

NO! NO, NO and NO.

I thought, are you kidding me? Are you seriously asking me this horrible question just days before I will celebrate my first Mother’s Day anticipating the birth of our child. This information scares the crap out of me, Yes. But in no way does it change the level of affection I hold for the life growing inside me.

To put it into perspective, let me share an excerpt from my personal journal that I had written several weeks before that day after hearing my baby’s heartbeat for the first time.

I was in total awe and felt rather nostalgic and sentimental the rest of the day. It’s indescribable how incredible the miracle of life is. Wow, God. Quite Impressive…It feels as though the bond of carrying our child is drawing Adrian and I closer together than ever before. I know we’ll face our share of parenting issues, but there’s nothing like a life that’s formed as part of both of you out of love, to unify.

I had no idea what was to come when I penned those words in the privacy of my room. But as I read them again, they mean more to me than ever.

Genetic counselors are trained to ask that question under the guise of family planning, and from what I understand, a decent percentage of people say “Yes” when given the option to terminate after learning their “fetus” has issues. Granted, some issues are much more severe with limited life expectancy, but I still strongly believe in giving a baby the opportunity for Life.

I sit here and look at this beautiful, happy girl enjoying the Disney channel like thousands of other pre-schoolers and think how sad it is that someone would choose to deny her life and deny themselves the joy of being her parent.

After letting them know that we would continue with the pregnancy no matter what, I got to see my baby again. We went in for a level 2 ultrasound with the doctor. There on the monitor, in much higher definition was a precious, active little baby who they quickly confirmed was a girl.

Silent tears rolled down my cheek as I held my husband’s hand and looked at her.

And then I saw it clearly. Her bubble. The outline of a sac protruding from her lower back. Our baby girl had spina bifida.

Though I was afraid, I suddenly felt closer to this child inside me, realizing that life would be much different for her and us than I had ever anticipated. But I loved her even more with that knowledge.