Processing Prevention over Plans

Another surgery has come and gone. Gabby is in a room and doing pretty good. She of course is in pain but is on Tylenol, Morphine, Toradol and Valium to help curb the pain. This surgery was called a lumbar laminectomy fancy words for detethering the spine. Bart and I had so many reservations going into this surgery. One going back to hospital that we left 10 years ago. Secondly, Gabby having a surgery in which Dr. Alam wasn’t performing or had a hand in personally selecting the other surgeons. Third and probably biggest, the possible formidable outcomes. The surgery was predicted to be 2-4 hours but turned into about 5 to 5 1/2 hours. While it makes for a long day I like to think that means that the surgical team took extra caution due to the difficulty of her diagnosis. Once we met with Dr. Volk we soon found out that indeed was the case. Apparently, tethered cords present many difficulties due to the way the spine forms and in Gabby’s case closed. The layers that envelop the spine are typically in a certain order but in Gab’s case they were kind of folded up. Dr. Volk said it took about 1 hour just to “open” her up and then 1 hour to try to do his best job and closing up a layer that in his description was like trying to stitch tissue paper closed tightly. This will be our special request prayers... 1) that she heals properly and quickly 2) that she is able to have patience as she is required to lay completely flat with no pillows or bed elevation for at least 48 hours 3) that she does not develop a spinal fluid leak. Number 3 may be the very most important. If she develops a leak they will have perform another procedure and put in a type lumbar drain. Dr. Volk says that she will not tolerate that type of drain well because of her age and having to stay in the bed even longer. Now moving forward to the title of this blog... Processing Prevention over Plans. Bart and I had a lot to process once talking to Dr. Volk after the surgery. As far as the presented “planned” results and the actual expected results of the surgery. We went into this surgery thinking this will help her feet relax back to more comfortable position. We are now being told that this may not be the case. Instead we should concentrate on the hope that this surgery will prevent her feet from getting worse than they already are. This was a huge disappointment, especially for myself. I was the one that went to all of the appointments prior to surgery and then relaying information to Bart. I feel like I did not listen good enough, ask the correct questions and then failed to communicate the information to Bart correctly. This is tough to swallow for me. If you know me well then you know I’m absolutely my own worst critic and have been beating myself up over whether I have made the correct decision in the best interest of Gabby and her future.Bart and I have come to the conclusion that the detethering surgery was unavoidable. It most definitely will help prevent the almost inevitable occurrence of scoliosis in the future. Also, we will pray that she will proves us all wrong as that her feet will correct even if it’s the tiniest bit. She is the most determined and hard headed little girl I know and she wakes up everyday and faces most any challenge head on. Bart and I want to thank you all for praying for all of us today and in the days to come. We can never repay our family and friends for the support that we receive not only in our major times of need but also daily.

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.

Dreams do come true...

Recently, our family was connected to a wonderful organization called Dreams Come True of Louisiana. Bart and I have had a small link to the organization for a few years now because we cook in the jambalaya competition at the South Louisiana Crawfish Festival. However, never did we think that our relationship with the organization would expand further than that. You just never realize when you meet people what those relationships may hold. Years ago Bart and I became friends with Ms. Shelby and her granddaughter Cassidy. We have remained friends with them over the years and they have followed Gabby’s journey as we have followed Cassidy’s all these years. Shelby and Cassidy have been affiliated with DCT for many years and this year they introduced our family to Mrs. Freddye and the DCT organization. We can not begin to express our gratefulness for this introduction. Dreams Come True gifted Gabrielle and our family with a dream vacation. Gabrielle’s dream vacation was to stay at a Great Wolf Lodge and she wanted to see real snow. The ladies at DCT got right to work and planned us a vacation to Great Wolf Lodge in Colorado Springs, Co. We took our trip last week from Wednesday to Sunday.         This is our first true family vacation EVER! If you have followed Gabby’s story over the years then you know we take trips out of town for her to see a urological specialist. These trips have taken our family from Cincinnati to New York City to Charleston. We always try to make the best of these trips with visiting local attractions, zoos, baseball games, and family. But regardless of what you do while you are in that city, there is always a surgery, a MRI or a follow up appointment scheduled and lots and lots of time is waiting rooms to dampen the spirit of the trip. This Dreams Come True trip was so far from our past trips. We had almost a full 5 days of nothing but snow, swimming and fun!

 

Our family want to say THANK YOU from the bottom of our hearts to the Dreams Come True organization for a wonderful trip. Gabby and Avery loved every minute of it. When I asked last night what their favorite part was, they listed off almost every thing we did… the water park, mini golf, the arcade, the SNOW; the list goes on and on! If you are ever looking to support and donate to a local organization please consider Dreams Come True. They have gifted our little girls with something that Bart and myself have been trying to accomplish for years… a few days of joy that didn’t revolve around Gabby’s birth condition. We look forward to many more fun times with this organization.

 

We also want to thank Ms. Shelby and Cassidy again for thinking of our family and helping make this connection.  

 

A person’s a person, no matter how small

When Gabby was born there were so many worries. In my mind one of the biggest was would she ever walk. It took her longer than the average baby to start walking but eventually in the tiny kitchen of the tiny trailer we lived in she took her first steps. I remember staying so quiet not wanting to startle her, she was around 19 months old. This story comes to mind today because it’s World Spina Bifida day. October 25th has been designated to bring awareness to this birth defect. Some people look at me like I’m crazy when I tell them Gabby has spina bifida. Most people assume that all people born when SB are wheelchair bound. I read earlier today that SB is sometimes called the snowflake disease because it comes in so many different varieties. Gabby was born with a closed spina bifida defect that they call a tethered cord. Her tethered cord can cause numerous problems from headaches to abnormal gait to urinary incontinence. Gabrielle suffers from all of those symptoms. Dr. Alam has given us permission to bring Gabby to a local neurologist. She hasn’t seen neurologist since before she started walking. It’s strange my biggest worry when pregnant with her turned into my least worry as the years have passed. Currently, I have her scheduled for an appointment in January. Gabby has been having a few headaches and her feet are starting to turn and give her more trouble when walking. I’m anxious for this appointment and to learn more about this part of her birth defect. Gabby has so much determination when she decides to put her mind to it she can accomplish it. Her confidence needs some building, that we are working on. 

Gabby has continued to heal beautifully since her surgery this summer. She is handling the cathetering well. She has jumped right back into school making honor roll her first 9 weeks of 4th grade and joining both 4H and Beta club. Girl Scouts is back in full swing and our social calendar stays full all the time. We are so blessed to have Gabby in our life. She teaches our family so much on a daily basis. Tolerance, strength, acceptance, sensitivity and list grows by the day. The picture below is Gabby and two of her best friends after their honor roll assembly today. I’m so thankful her group of friends that she has made over the last year. It definitely has helped with her self confidence having them on her side.            

5016 miles later.. and worth every mile!

Hey everyone! I'm sorry that I've just gotten around to writing this blog post. It has been a crazy busy week of work and school. Why not throw a trip to South Carolina in the mix? Because apparently we are crazy! We left around 3 am on Tuesday morning and got home around midnight on Wednesday night, then went to work and school the next day. I don't recommend you do that. But hey, here we are and we survived! Total this summer we have traveled 5016 miles back and forth from St. Amant to Charleston. Every mile has been worth it!

Peace out MUSC (at least for the year!)

Gabby had a blood draw, renal ultrasound and urodynamic study done Wednesday morning. The urodynamic study was a new test for us. Gabby was very worried about it because she had never had one, turns out it wasn't very bad at all. They took us in a surgical room filled with an x-ray machine and table, lots of computer screens and some type of automated pump. The basically put a catheter in her mittrofanoff and also one in her colostomy stoma. The catheter in her mitt is hooked up to the automated pump that pumped what I assume is saline (forgive me I didn't ask) into her bladder. While the machine is pumping the nurse watches several computers reading how much volume is being pumped and at what rate it is going in. Dr. Alam had his own computer that he was watching. The x-ray was mobile and positioned above and below Gabby while she was being entertained by a sweet child life specialist. Every 5 minutes or so Dr. Alam would call out x-ray and a new image would pop up on another computer screen with an image of Gabby's bladder that Dr. Alam and Dr. Hyler (one of the urology fellows) would look at. This went on for around an 45 minutes or so. Eventually, her bladder reached an amount of 180 cc. The purpose for this test was for a few reasons. 1) to check how much her bladder can hold 2) how big her bladder is. 3) to make sure that her bladder didn't have any leaks. It was extremely laid back the whole time. We talked about Gabby and our home routine along with talking about how ridiculous and Top Gun sequel is at this point in Tom Cruise's life. :) Once the study was over he removed the supra pubic drain and said that the hole left should be gone in a day or two. After that she had her ultrasound and then blood drawn. Once we finished up there we headed upstairs to meet with Dr. Alam and then a nutritionist. During our meeting with him, he kept going on and on about how happy he was with the results of Gabby's surgery. He said pending in complications we won't have to go to Charleston until next summer for a follow up that will probably include another urodynamic study and possibly a cystoscopy of her bladder. We are to continue cathing her every 3 to 4 hours along with continuing her nightly Gentamicin antibiotic bladder flushes to help keep her bladder healthy and keep infections away.

While we were there I did take the opportunity to ask him about kidney disease. I have a private facebook group that I read almost weekly about different kids and their problems with kidney disease. After reading about it so frequently, I had to ask Dr. Alam why the subject has never come up with Gabby in the last 9 years. From his opinion in Gabby's case she has been very fortunate not to develop kidney disease as of yet. Kidney disease can develop due to many reasons but in most of these OEIS kids it tends to be because of reflux of urine back into the kidneys causing chronic infections. While Gabby does carry streptococcus bacteria in bladder all the time using the Gentamicin keeps it from overgrowing into her kidneys. We are also going to add a probiotic to help keep it at bay also. This doesn't mean that she can't develop kidney disease in the future but as long as we keep on the same path and stay vigilant it should definitely help prevent the development of kidney disease for a while at least.

Dr. Alam also had us meet with a nutritionist. Gabby's weight has been a concern for quite sometime now... well at least the last 6 years. When she was hospitalized for surgery in 2013 they tried doing NG feeds with Pediasure and Pedialyte but unfortunately her little belly just couldn't handle it. She would sit hooked up a machine for hours only to vomit it all up, we weren't getting anywhere. I did really enjoy talking to this nutritionist. She definitely understood that kids on the general don't have the best diets and gave us some tips that we can try at home. She also wants to add daily multivitamin, vitamin D, fish oil and multi mineral complex to Gabby's daily list of things to take. It sounds like a lot but if it will help her not have to do NG feeds I'm totally game.

We will have do to do ultrasounds through out the next year but those can be done locally and then the images will be sent to Dr. Alam to review. The biggest and most exciting news that Gabby received was that she could go swimming whenever she is ready!

Home sweet home

Home sweet home, Room 733

Good afternoon everyone! We are back in Charleston this week for a few things. 

1) cystoscope 

2) drain tube removal from mitrofanoff 

3) Suprapubic tube replacement 

4) mitrofanoff catheter training

After getting a good dose of meds to help her relax the morning progressed pretty quickly.  

Daddy got to put on his fancy moon suit so he could escort her to the OR while stayed I behind with little sister. After that we went to catch a quick breakfast downstairs and came back up to wait in the waiting room. As we exited the elevatorDr. Alam was standing there typing a text to me to find us, he was done? We couldn’t have been gone more than 30 minutes. He had pictures of her mitt stoma and bladder. He said he was ableto easily see that her bladder augmentation and mitrofanoff are healing really good. Dr. Alam came into the PACU after the procedure to teach mehow to catheter Gabby via her mitt stoma. I’ve been catching her for over a year now but this is definitely different. So far it doesn’t seem to cause her any actual pain or discomfort. I think she just doesn’t enjoy having a tube stuck in her every 2 hours. The time span will increase with time as her bladder stretches. The goal time period isgoing to be every 3-4 hours. Gabby and I will getto stay tonight at the hospital. We should check out in the morning. We will stay in Charleston another night and then have an office visit on Wednesday morning at 9am. Pending no issues we will head back to Louisiana after the appointment and get home late Wednesday night. Then it will be Granny D’s turn to train on cathing. 

We will follow up again in 8-12 weeks. That trip should be a pretty quick one. I think I remember him saying come in Monday go home Tuesday. We will have one more follow up before the end of the year for a urodynamic study. He said that weput a camera in both her colostomy stoma and mitt stoma. This will give the urology team the ability to watch how her bladder holds and releases urine over a period of time. He told us that she will be awake during that procedure. 

We also discussed her spinal defect and her footdrawing up further over the last couple of years. He gave us permission to see a neurologist and orthopedic surgeon at home. He said he is comfortable with other surgeons operating as long asthey aren’t touching her abdominal area and are in complete communication with him. We have come too far for someone else to stumble in and mess everything up. As far as her next surgery with him, well, that all depends on her, her growth and when she really hits puberty. From the sound of things we should be able to go a few years without a surgery on her abdominal area. Praise God!!! 

Again, Bart and I will never be able to express our thanks for all of the support and prayers. Without your support she would not be as far along as she is today. 

The final days

Good morning everyone! After my frustrated post the other day, I really did feel worried that this trip was about to be longer than hoped. Dr. Alam is always extremely cautious. Don’t get me wrong that is appreciated. I mean would you want to operate for 12 hours and then rush the recovery along just to have something go horribly wrong. No, probably not. Apparently, it was discovered during the surgery that Gabby has a large amount of scar tissue that had attached itself to her gallbladder. I believe Dr.Alam told me that a piece of bowel was were the scar tissue originated from. He spent a good amount of time during surgery dissecting the scar tissue to separate the bowel from the gallbladder. Once the surgery was completed one of the worries with giving her fluids or food orally was that the bowel would get overly heavy in one area and cause it to invert and twist. If that were to happen it could be disastrous With possibility of an emergency surgery. So, that was the reason for waiting the extra few days for the bowel to wake and strengthen. When you are in the moment though it is hard listening to your baby cry that their throat hurts and they are hungry. Since that day... she has gotten to drink and then about 12 hours after that food came. Her first pick... PIZZA! 

Since then she has been eating and drinking small amounts. The food is important but not as much as drinking. Her drinking does need to increase but hopefully it won’t hinder us from getting out. I was fully expecting to be in the hospital another 2-3 days but Dr. Alam says that we can be allowed to check out on Sunday... like tomorrow!! We will go back to the hotel and hangout a day or so. She is already scheduled for a follow up appointment at the clinic on Tuesday afternoon. From what we are understanding as long as everything goes well at the appointment then can head home. HOME!! By the time we arrive home Gabby and I will have been gone right at 20 days. She will be coming home with 2 large drains from her new mitrofanoff and her bladder. Bart and I have been trained on how to flush them 2 times daily plus clean the drain sites and the tubes. There will be limitations on what she will be allowed to do the remainder of the summer but we are going to try to make the best of it. We will come back for 3-5 days at the end of July to have the drains pulled and be trained on how to catheter her the new way. Thank you to everyone that has followed us along this journey and all of the others up till now. Keep the prayers coming for us to get out of here and headed home and for no complications while at home the rest of the summer.