Vanderbilt Meeting

   Our two week wait went by much faster than we thought it would. We both had a restless night, but got up bright and early and went to our appointment that started at 7:30. The drive to Vandy was awful due to rush hour, but we made it, nerves and all. We knew going in that our day was going to be packed with meeting after meeting. We were excited to get answers, but also scared. What if something else was found? What if our amnio results came back abnormal? So many what ifs...

   Our morning started with a 1 1/2 hour ultrasound. The tech took pictures of everything and explained when she could. When we asked a question that she wasn't able to answer, we got the "They will explain a little later" answer. It was amazing to be able to see Jackson for as long as we did. It was also amazing to see all of his organs. The best part was watching his heart work and see the four chambers.

   She looked at Jackson's spine last. Our little man is quite the stinker and was determined to make it difficult to see all of his lesion. Eventually though, she got a great picture of the lesion for the doctors to look at. We got several ultrasound pictures to take with us and even got some 3-D ones and were able to see his handsome face for the first time. Next stop, the conference room to get our official results and discuss prenatal surgery.

   The people that we were going to be meeting throughout the day are as follows:

• Dr. Carroll and Dr. Bennett are part of the OBGYN team and handle the obstetrics and maternal fetal medicine side of the surgery team. 
• Martha Dudek the genetics/amnio consult 
• Dr. Wellons the neurosurgeon 
• NICU

   Dr. Carroll and Ms. Mary (fetal program coordinator) were the first ones that we met with.  Dr. Carroll brought up Jackson's ultrasound pictures on the computer and started discussing the findings with Matt and I.  She explained that all of his organs were normal and functioning properly - she didn't see any issues which was a good thing.  She moved on to his head.  She discussed the finding of lemon and banana sign and confirmed Chiari malformation.  She then talked about Jackson's ventricles.  The ventricles in the brain are like pools that are filled with cerebrospinal fluid (CSF) which bathes and cushions the brain and spinal cord within their bony confines.  The ultrasound showed that while Jackson's ventricles were enlarged, they were within the normal limits which was great news.  The last thing that we looked at was Jackson's spine.  The news that we were waiting for - where is Jackson's lesion and what does it mean?  Dr. Carroll gave us an illustration showing the different levels of the spine and what each levels function is.  

   Dr. Carroll explained that Jackson's lesion level is L3-S5, much higher than we were hoping and a lot larger than we thought. With this lesion level, everything from the knee down could be affected, as well as, bladder and bowel function.  However, spina bifida is known as a "snowball condition."  This means that you could line up several kiddos with the same lesion level diagnosis and not one of them would have the same complications.  Once again, we were faced with the unknown. Yes, we now know his lesion level and what could be affected, but we don't really know how Jackson truly is affected and we won't know until he makes his arrival and shows us what he can do.  

   After explaining everything having to do with Jackson and his lesion, she went on to explain that we do qualify for fetal surgery if we were interested.  The first time I spoke with Ms. Mary on the phone, she explained that they would come across as not recommending the fetal surgery, but it was simply because they want to make sure that we understand the risks that come with the surgery for baby and mom.  Sure enough, I felt that we were being talked out of the surgery, but I kept reminding myself of what Ms. Mary said on the phone.

   So, let's talk risks.  

Outcome	Finding	Implication
Premature delivery	Premature births (with respiratory problems) were more common in the prenatally treated group (13% were delivered before 30 weeks of gestation). The average age at delivery was 34.1 weeks for babies who had prenatal surgery compared with 37.3 weeks for those who had surgery after birth.	Premature delivery is associated with serious problems such as difficulty breathing and even cerebral palsy.
Pregnancy complications	One-third of mothers who had prenatal surgery had thinning of the uterus or an abnormal opening (dehiscence) where surgery was performed. Other complications included decreased fluid in the uterus, and problems with the membranes surrounding the baby.	These complications may affect the mothers' ability to have subsequent pregnancies.
Tethered spinal cord	Infants in the prenatal-surgery group underwent more procedures for delayed spinal cord tethering.	Tethering of the spinal cord (tying down from scars) can cause additional loss of function; this could negate the advantages in mobility that were associated with prenatal treatment.

   The next person we met with was Martha Dudek, the genetics counselor.  The first thing that Martha explained was that Jackson having spina bifida was in no way my fault.  There was nothing that I did or didn't do that could have caused him to have this birth defect.  

***Those reading this that are feeling like it's your fault that your child has spina bifida, please hear me, it's NOT your fault... It's NOT your fault... It's NOT your fault.  I know that I can say that until I'm blue in the face and you would still have the feeling that you could have done something more - I get it, I was there.  However, please try and stop yourself from thinking this.  We've got to make sure that we aren't beating ourselves up for something that we had NO control over.  Our children already had spina bifida before we even knew that we were pregnant.  Remember, this diagnosis doesn't define your child. :)  OK, back on topic - oops!*** Martha also discussed our amnio results with us.  She assured us that the amnio was perfectly normal and there weren't any issues.  After hearing this, a huge weight was lifted off of my shoulders.  I had been anxiously waiting for my amnio results and couldn't get anyone on the phone at the doctor's office that took the test.  My anxiety level was extremely high due to not getting my results back in a week like I was suppose to.  It ended up taking two weeks and I had to get them from Vandy who didn't even draw the amnio.  However, that's an entire different issue that will be discussed with the doctor that was involved. :)  

   We quickly met with the NICU doctor and learned what would be involved when Jackson is born.  With Jackson being born at 37 weeks and with his spina bifida, he will need to stay in the NICU for a little while.  We talked about the tests that will be done once he's born and what to expect during his stay for Jackson and for us.          

    Dr. Wellons, the pediatric neurosurgeon was the last person that we met with.  Dr. Wellons went into a little bit more detail concerning Jackson's lesion level and size.  He explained that with Jackson's lesion level, he has seen children walk with knee high braces. However, with the prenatal surgery, he hopes that the ability level will be lowered to an L5 instead of L3.  We found out that Jackson's lesion is the largest that they have seen measuring 2cm by 4 cm.  Hearing that was so scary and heartbreaking.  He told us that he would probably end up having to cut "release incisions" on his sides to release the tension so that he could close Jackson's back since the opening was so large.  The release incisions would be covered with a type of mesh that would hopefully heal while Jackson is still in utero.  However, if they don't heal, Jackson would need another minor surgery once born to close those incisions to prevent infection.  Jackson's back would be closed with donor skin and told us that those heal very well while in utero and shouldn't be an issue.  He then explained that the main goal for the surgery is to try and prevent the need for a shunt. With Jackson's ventricles the size that they are in utero he wouldn't need a shunt.  However, if we chose to not have the surgery, that would most likely change once he was born - another unknown.  When we asked if he believed if Jackson would benefit from the prenatal surgery, he strongly said yes.  He told us that closing Jackson's back as quickly as possible will help prevent anymore damage from being done.  He also explained that by closing his back, the spinal fluid will have time to start circulating like it's suppose to and start conditioning his nerves.  When we heard him say that he highly recommended the surgery, our minds were made up.  

   Ms. Mary explained to us to take the weekend to think about everything that was discussed and to call on Monday with our decision.  We already knew what we were going to do, but we appreciated being given the time to think.  If having the surgery is the best option for Jackson, then that's what is going to happen.  Our son deserves every chance and every possibility out there.  Surgery has been scheduled for April 7th!  I am terrified, but I also know that this is the best possible thing for Jackson.       

Crystal 

Maternal Fetal Medicine Appointment

   Sleep?  Sleep is overrated!  Last night was extremely rough.  I spent most of the time researching spina bifida.  If you have recently been diagnosed, please do not Google!  Doing so will only make you more scared.  However, check out BabyCenter: Spina Bifida Kids.  This message board has been a wonderful resource for not only me, but a lot of parents.

   Today was our appointment with Dr. K - our second opinion.  At Dr. K's we had a more in depth ultrasound.  After researching I knew what lemon and banana sign looked like and up on the screen was my sweet boy's head with a very obvious lemon shape.  My heart shattered.  All of my thoughts of thinking this was all just a mistake became a reality.  

   The doctor came in midway through the ultrasound and after a quick intro, began talking with the ultrasound tech.  After about 10 minutes of looking at the baby, she sat on the edge of the bed and very softly explained that our son did in fact have spina bifida.  There it was - the truth that we didn't want to hear.  The truth that we fought so hard last night to tell ourselves was a mistake.  She showed us his head and pointed out the lemon and banana sign and explained what was causing it to happen.  We learned that due to the spina bifida, Jackson's cerebellum was being pulled down the base of his neck.  Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.  Dr. K also confirmed that both feet are clubbed.  She explained how the feet being clubbed could show nerve damage that has already occurred due to his open lesion.  However, his clubbed feet could also have nothing to do with nerve damage.  This was one of the many unknowns.  While Jackson's lesion could be seen, Dr. K couldn't  be certain of it's location, nor how large it was.  Another unknown to add to our list. 

   Dr. K spent a lot of time with us trying to explain everything to the best of her ability, but again, we were offered termination due to not knowing what his quality of life would be.  Even after that, I felt that she was very compassionate towards us and I thank her for that.  I thank her for spending so much time with Matt, my mother in law, and me.  I thank her for her hugs and kind words.  

   We were informed of a fetal operation that would take place no later than 25 weeks if we qualified and were interested in having the operation.  She set up an appointment at Vanderbilt Children's Hospital to meet with a team of specialists.  She explained that even if we decided to not have fetal surgery, meeting with the team of specialists would provide us with a lot of valuable information specific to our son.  Our appointment was scheduled for March 27th.  Two weeks - two weeks of wondering, worrying, praying, etc.  Two weeks of the unknown.

   While our meeting with Dr. K was heartbreaking with the confirmation of Jackson's spina bifida, we both left feeling like a small weight was lifted off of our shoulders.  There's no denying it, our son has spina bifida.  That's still hard to say/type.  I imagine that it's going to be hard to say/type for some time.  It's going to be a tough two weeks.  I hate the unknown. However, what I do know is that my husband and I love our son so much already.  I also know that he is going to do something big in his life.  Jackson has a purpose and a mission, we just don't know what it is yet.  

Crystal 

Diagnosis Day

   Matt and I were so excited to find out if baby Coleman was a boy or girl.  My family has all girls, but I was starting to think that the baby was a boy.  Matt even made a bet with my cousins that it was going to be a boy.  He felt so confident that we finally broke the girl trend.  :)  

   The ultrasound tech talked with us throughout the entire ultrasound while she was taking measurements.  Even though the baby was being stubborn, we found out that the baby was a... BOY! Wait, what?  My family doesn't have boys!  What about my plan of hair bows and dresses... Are you sure it's a boy?  There was no denying that baby Coleman was a he!  Matt and I couldn't be anymore happier.  We're going to have son.

   After the ultrasound we met with Dr. T to discuss the ultrasound and get an update on how everything was progressing.  I could tell that something wasn't right as soon as Dr. T entered the room.  Dr. T is a very bubbly person and always found a way to make me laugh - that didn't happen this time.  We went from being the happiest people on Earth to being scared and nervous about what was wrong.  I never knew it was possible to go through such a range of emotion in such a short amount of time.        

   Dr. T explained to us that our sweet boy was measuring appropriately and all his organs looked great, but he appeared to have an opening in his spine.  He went on further to tell us that he believed this opening meant that Jackson had a neural defect called spina bifida.  Through the ultrasound, Dr. T also saw that Jackson showed lemon and banana sign in his head and his feet were turned inward (clubbed feet).      

   What is spina bifida? Spina bifida (Latin: "split spine") is a developmental congenital disorder caused by the incomplete closing of the embryonic neural tube. The closing of the neural tube is complete by day 28 - before you even know that you're pregnant. Some vertebrae overlying the spinal cord are not fully formed and remain unfused and open which is called a lesion. If the opening is large enough, this allows a portion of the spinal cord to protrude through the opening in the bones. There may or may not be a fluid-filled sac surrounding the spinal cord. (http://en.wikipedia.org/wiki/Spina_bifida)

   What is lemon and banana sign?  The lemon sign, noted on antenatal imaging, is one of the many notable fruit inspired signs. It is a feature when there appears to be an indentation of the frontal bone (depicting that of a lemon). It is classically seen as a sign of a Chiari II malformation and also seen in the majority (90-98%) of fetuses with spina bifida.  The banana sign describes the way the cerebellum is wrapped tightly around the brain stem as a result of spinal cord tethering and downward migration of posterior fossa content. The shape of the cerebellum has appearance of a banana.  This is very frequent to hydrocephalus. (http://radiopaedia.org/articles/banana-sign)

   Ok, keep in mind that we weren't provided any of the information found in the previous two paragraphs - none!  However, I want to make sure that if you're reading this, you have an idea of some of the terminology that you have heard.  We weren't really offered much information about Jackson's diagnosis other than that he could have a poor quality of life and we still had time to terminate if we chose to do so.  This option really bothered me - how can someone make such a decision, but not know exactly what spina bifida means in general, let alone what it means for your child.  

   While we were left asking so many questions, wondering what the future may hold, and terrified, we were also recommended to go see a Maternal Fetal Medicine doctor to get a second opinion.  Thankfully our appointment was scheduled for the next day.  I'm not real sure how I would have handled having to wait to "try" and get some more answers.  Our families knew that we had our anatomy scan on this day.  How do you tell your family that your having a son and then crush the excitement by adding on the fact that he may have spina bifida?

Crystal       

Welcome

   Welcome!  I decided to create this blog for my son shortly after our diagnosis of him having spina bifida.  When Jackson was diagnosed, I was devastated, heartbroken, mad - basically every emotion that you can have, I went through in a matter of seconds and then hit repeat for the next several days.  I found comfort in reading other blogs by parents with children that have spina bifida.  Did it make me feel completely better? No, absolutely not.  Did it give me hope? Yes!  I was able to realize that my son having spina bifida doesn't define him.  He will be a normal little boy that will be very loved.     

   The worst thing that I did on diagnosis day was Google.  If your sweet little one has recently been diagnosed with spina bifida, please, please, please do not Google.  Google can be a great resource, but it can also be very scary when it comes to medical diagnoses.

  I know that you feel scared and alone.  Please know that you are not alone.  Being scared is a normal process, as is questioning what you should do.  If you're like the majority of other spina bifida parents, you were offered termination due to poor quality of life.  The only person that can tell you accurate information about your little one is a neurosurgeon (not neurologist--common confusion).  They are your go-to person for realistic, up-to-date information and treatment regarding spina bifida.  You'll also likely be pleasantly surprised at the positive and encouraging prognosis the neurosurgeon gives your child!  It is impossible to make an informed decision about which option to choose for your baby without first speaking to a pediatric neurosurgeon. 

   My goal for this blog is to document our process starting with diagnosis day and continue through Jackson's life.  In a way, this will be a journal that I hope one day he will read.  I hope that our experiences may help those that have just been diagnosed and even connect with more parents that have kiddos with spina bifida.  I welcome any comments and questions and will get back with you as quickly as possible.

   So again, welcome to our journey with my sweet Jackson that just so happens to have spina bifida. 
  
Crystal and Jackson