Here we are again...

 Sorry… I know it has been a minute since we have last updated Gabrielle’s blog. In a way it is something to be very thankful. No news, is good news, right? Gabby has been doing very well since I last updated you all. She has been doing great in 5^th grade. Lately, she has competed in a few cooking contests for 4H in which she has placed in each event she has entered. We are so very proud of her!

Gabby this past Christmas

Over the last 12 months Gabrielle has experience quite the growth spurt. While this is wonderful news being, she is so petite, it does also have the potential to cause problems in a child with Gabby’s condition. If you are just reading our blog for the first time, I will take a moment to quickly update you on Gabrielle’s birth defect and what it pertains to. Gabby was born with a birth anomaly called OEIS. This affects 4 major areas of her body. O- omphalocele- the abdominal wall does not complete the closure correctly to protect the internal abdominal organs. She had 2 surgeries (first- 2010 and second-2012) to repair this abdominal wall opening. E- exstrophy of the bladder- the bladder is typically on the outside of the body, split in half with the inside of the bladder facing outside of the body. She has had 3 operations on her bladder to get her to the point she is today (first- 2010, third- 2013, and her most recent 2018). I- imperforate anus- unfortunately currently for Gabrielle’s case this is not able to be corrected, so since she was 3 days old, she has had a colostomy bag. S- spina bifida- this what we are here to update you on today.

Gabrielle has a very mild form of spina bifida called a Lipomyelomeningocele. The following is a description of what that entails found on the Columbia.edu website from New York City, NY one of the hospitals that Gabrielle has seen physicians in her 10 years. 

Lipo = fat
Myelo = the nerves of the spinal cord
Meningo = the meninges, or membranes (coverings) around the spinal cord
Cele = a swelling

Lipomyelomeningocele is a condition in which an abnormal growth of fat attaches to the spinal cord and its membranes.

Lipomyelomeningocele arises from an event very early in an embryo’s development. About the third week after conception, a sheet of cells called the neural plate folds to form a tube called the neural tube. The top of the neural tube becomes the brain, and the rest of the tube becomes the spinal cord. Lipomyelomeningocele occurs when an error in the closure of the neural tube allows a type of cell called mesenchyme to contact the inside of the neural tube. These cells prevent the tube from closing properly, disrupting the formation of meninges (membranes, or coverings) and bones around the spinal cord.

Wherever the mesenchymal cells touch the outside of the neural tube, they develop into spinal meninges as usual. Everywhere else, they develop into fat cells. The end result is a fatty growth called a lipoma that begins in or near the spinal cord, connects with the meninges, and extends past the bones of the spinal canal to form a pad of fat beneath the skin.

Normally, the spinal cord is able to move somewhat freely within the spinal canal. But in lipomyelomeningocele, the cord’s movement is restricted by the lipoma’s connection with the meninges and the area outside the spinal canal. This condition is called a tethered spinal cord, and it may have severe consequences.

Lipomyelomeningocele is one type of spinal dysraphism. It may be associated with other forms of dysraphism, dermoid or epidermoid cysts or Chiari malformation.

This past November we started really pursuing seeing a neurosurgeon after continuing to see a decline in her balance and her steadily getting worse. She had a sedated MRI at Childrens New Orleans and they discovered that her syrinx (a fluid pocket on her spine) had grown since her last MRI. Over the last year due to her growth spurt she has developed a fluid pocket along her spine in the lumbar section. This fluid is putting pressure not only directly on her spine in that spot but also adding additional pressure to her tethered cord. The neurosurgeon we have met with in New Orleans think that this is causing problems with her balance and with her feet. Gabrielle’s feet have always been slightly misshapen which is not uncommon, sometimes children born with OEIS will also have other issues such as club feet. Thankfully in Gabby’s case her feet are not clubbed however they are missing muscle tone and the flexibility that you and I have in our feet. The neurosurgeon believes that the excessive pulling her on her spine is sending the wrong message to her brain, which is turn sending the wrong message to her feet. Due to the miscommunication her feet are starting to turn and draw up causing her balance to be much worse and making getting around harder. She must exert much more effort than the average person to get herself from one place to the next. After talking to the neurosurgeon and consulting with an orthopedic surgeon we have decided that her next step is spinal surgery. This is the one surgery that Bart and I have tried to avoid her having for the last 10 years. The very first neurosurgeon from New Orleans was extremely pushy about having Gabby detethered by the time she was 18 months old. By the time Gabby was 2 we had made the move to Cincinnati Children’s and their philosophy on detethering children like Gabby was much more relaxed and not as rushed. Dr. Volk, her new neurosurgeon, explained to me that American medicine tends to want to do things very fast and early whereas Eastern medicine tends to take things slower and stage things out as a matter of importance. Basically, do not treat until a problem presents itself. He has stressed to me that is perfectly fine that we have chosen to wait for Gabby to have this surgery. As of right now Gabrielle is scheduled to have surgery on 2/11/2021 in New Orleans. We are hoping to be home in 4-7 days and then she will stay home from school and hopefully be able to attend virtually for 2 to 3 weeks and then return to her regular life. 

Gabby waiting to have her MRI done @ Childrens New Orleans

 

Please continue to pray for Gabby and our family. This has not been an easy decision for Bart and me to make. We have made sure to keep Gabby herself informed every step of the process because we feel that this is very important as she is starting to get older. Avery is struggling with accepting her sister have yet another surgery. I’m not sure that I even know what to qualify as a surgery really to give you a count on how many she has had. In my mind a surgery is qualified by being put under anesthesia and having a procedure done. IF that is the case then her surgeries are as follows.

 

First- 3 days old @ New Orleans Childrens (colostomy placement, omphalocele closure)

Second- 2 years old @ Cincinnati Childrens (omphalocele repair and colostomy revision)

Third- 3 years old @ Cincinnati Childrens (bladder revision & double hip osteotomy)

Fourth- 3 years old @ Cincinnati Childrens (removal of external fixature/drainage tubes)

Fifth- 8 years old @ MUSC Charleston, SC (mitrofanoff surgery)

Sixth- 8 years old @ MUSC Charleston, SC (exploratory & drainage removal)

 

This spinal surgery will be her 7^th major surgery in just shy of 11 years. It does sound like a lot but really the realm of where we live our life comparatively against other OEIS families that is very minimal. I can tell you something; she has gone through more than any child should. She is special, unique and I would not change a single thing that I do not medically need to for her to strive to become the young lady she is turning into. She is the bravest person I know, and I am so beyond blessed to her mama. 

This was our most recent trip to Farmerville to visit family for Christmas.