Reflux and Feeding Tubes!!!

So, the girls first year was filled with all of the regular baby check-ups and then Amelia also had check ups with her neurosurgeon, orthopedic surgeon, and urologist every 3 months. We also started seeing her ENT every 6 months to follow her hearing loss. We had to take her to the audiologist to be fit and followed with her hearing aid, and that was about every 2-3 months. She received services through early intervention, which started when she was about 3 months old. She had a teacher that came, physical therapy, speech therapy, and occupational therapy. Most of her therapy was home based, which made our schedule much more manageable. The girls also slept a lot when they were babies (so much that I often wondered if they were ok), so trying to fit in appointments and therapy in between 3 naps a day presented its own challenges. But, it all worked out and we just kept on pushing through. Other than her shunt surgeries at 3 months old, Amelia only had one other surgery during her first year. It was bladder surgery at 9 months to place a vesicostomy to allow her bladder to continuously drain into her diaper, which would keep her bladder from overfilling in hopes of preventing kidney damage. With the vesicostomy we did not have to place her on a catheterization program as a baby. Things were going well, and we were doing an ok job of adjusting.

During their first year the girls were definitely BIG spitters. Both of them would spit up with almost every feeding. Needless to say, we were happy that we had paid for fabric protection on our sofa. Around 9 months old Hannah's spitting really improved, but Amelia's continued and got worse. Her pediatrician was also concerned that she was gaining weight a little on the slow side. After the girls turned 1, Amelia was diagnosed with reflux and we tried several different medications but none of them really helped. She continued to vomit a lot, including her solids. We got to a point that we had to take her off of all solid foods and got her transitioned to Pediasure. During this time she stopped growing. She did not gain any weight for 4 months and she did not grow in length either. When she was 17 months old she was diagnosed as failure to thrive. Her reflux had become so severe that our best option for improvement was surgery. So, in Oct. 2004 she had a Nissen fundoplication (they made the top of her stomach tighter to prevent reflux). She also had a feeding tube placed at that time so that we could supplement her nutrition while she slept at night. By the time she had the surgery and then during her recovery she developed a total aversion to taking food or drink by mouth. She was tube fed only for several months. It has been 4 years since she had the surgery, and getting her to eat is still a huge struggle. We still use her feeding tube to supplement nutrition and fluids, but at least she is not on the continuous pump at night anymore. She is still not able to take in enough by mouth to meet her calorie of fluid needs, but she has made improvements. She has had 4 years of feeding therapy, but we still struggle with her on a daily basis. Out of all of her issues, feeding has been the one that has been the most frustrating for me to work with. She has so many underlying medical issues that could be affecting her feeding, but it is the one thing that we struggle with at least 3 times a day (and then top it off with the tube feeds) and have seen so little progress. I am almost at the point now where I am thinking that: 1- she probably won't go on her first date still eating baby food and goldfish and 2- if she does then he is probably not going to care. (The picture above is Amelia a few days after her surgery for reflux).

Amelia always does things in her time frame and no one else's. I work with her and she has lots of therapy, but I have taken away MY time frame. This has helped me tremendously. As long as I see progress being made I am happy. Sometimes it is painfully slow, but other times she just takes off.

More Shocking News!!!

After being home with both girls for almost a month and getting settled into a routine with them, it was time for Amelia's second surgery. When she was 5 weeks old she had surgery to place her shunt for hydrocephalus. That surgery went well and she only had to stay in the hospital one night. It was so hard for me to have to leave Hannah for the night, but it was something we knew that we would face over and over again. We also now knew that we would be facing a lifetime of "shunt issues". While shunts are life saving and a blessing, they can also be very tricky. Some people have the same shunt their entire life, others have multiple failures and infections. Amelia has had 7 shunt surgeries plus one failure without surgery, some due to failure and some due to infection. Her last failure was in December 2007, but she did not require surgery because her current shunt drains into the large blood vessels into her heart. This failure was resolved with some "clot busting" drugs. So needless to say, whenever Amelia is not acting quite like herself I usually worry and start thinking that her shunt is failing.

After Amelia's first shunt surgery we went ahead and scheduled to have her hearing retested. She was 8 weeks old when I took her in. The night before her hearing test I remember sitting with her in my lap at the kitchen table. I would talk to her and she would "talk" back. She and Hannah both made the same type of baby sounds, so why should I worry. I was so convinced that everything was fine, that I took her to be tested by myself. Steven went to work that day and his mom came over to stay with Hannah.

Amelia slept in my arms through most of her hearing test, which made it a little easier to get through. Finally, the first test was finished. I was completely shocked when the audiologist told me that Amelia had a profound hearing loss. I remember saying "but when I talk to her she talks back". What did a profound loss mean? Well, it meant that she might be able to hear a chainsaw or jet plane at close range. It meant that she was deaf. I remember crying in that dark, little room and feeling guilty for being sad that she could not hear. I told myself that I should just suck it up and be grateful that she was alive. I now wish that I would have given myself permission to fully grieve about her hearing loss at that time. The audiologist did a second test to find out what type of hearing loss she had, and we found out it was a sensorineural hearing loss.

Within a month of the hearing loss diagnosis Amelia had a CT scan to look at her inner ear anatomy. We found out that her hearing loss was caused from an inner ear deformity called common cavity. Common cavity is pretty rare and amplification can be tricky. Amelia started wearing a hearing aid when she was 3 months old, but we were told that her best chance to hear sound would be with a cochlear implant. We quickly became overwhelmed with all of the information and choices for families with deaf children. Hearing aids and cochlear implants, to sign or not to sign (and if you do sign what type of sign), involvement in the deaf community/culture. With Amelia's hearing loss diagnosis, I knew that I would not be able to return to work full time. Her needs were going to be too great, but I knew that for my sanity that I would need to work some. I needed to keep something that resembled my "old" life. So, I work 1-2 days each week and it seems to be a nice balance for me.

When Amelia was 21 months old, she had surgery to place a cochlear implant. We did not know what type of benefit she would receive from it because of her inner ear deformity. The doctor told us that realistically she would probably only hear environmental sounds. We feel very fortunate that Amelia is able to detect speech with her implant at almost normal hearing levels. We attempted to use an oral approach with her after she was implanted, in hopes that she would develop speech. While she did develop some speech, it was at an incredibly slow pace and as she got older she was becoming more frustrated. So, in February of this year we made the decision to start signing with Amelia. It was very hard at first to make the decision. We had worked so hard for 3 years at trying the oral approach. But, seeing how well she is doing now is making it much easier. We are all learning sign, but she is learning faster than we are. She is so smart and funny, and now that she can communicate better she can really show it.

So, during all of this you might be wondering about what was going on with Hannah. She is completely typical and healthy. Before the girls were born I worried that she might not get enough attention, because of all of Amelia's needs. A few weeks after they were born, that worry was gone. Hannah was and still is "spirited". She always demanded her attention as a baby and makes sure that she gets enough even now. We love her for it and know that her "strong will" will be be a quality that will benefit her throughout her life.

May 13, 2003

I was 34 4/7 weeks pregnant with the girls. I was tired and huge (my waist was 44 inches around) and wondering how I was going to make it another week. My C-section was scheduled for May 20. The doctors wanted to deliver me by 36 weeks because of Amelia's spina bifida. My pregnancy went very smoothly- I was able to work until 33 weeks and never went on bed rest.

On the morning of May 13 I had been treated to a pedicure and then had a doctors appointment and ultrasound scheduled. At this point in my pregnancy I was seeing my doctor twice a week. The babies growth looked really good and Hannah was very active and doing everything that she was supposed to do. Amelia was just being a little less active that morning. So, the doctor wanted me to go get some lunch and come back for another ultrasound after to see if we could get her more active. I went to Wendy's for lunch and even drank my first real Coke in months. After that, Amelia was still not very actice. So, they put me on a monitor in the office and watched us for a little while. Around 4pm my doctor came back in and said that after consulting with the high risk OBs, they felt like it would be safest to go ahead and deliver the girls today- at 8pm. I managed to not completely freak out, but I was pretty scared. I wasn't even sure that I was completely ready yet, but the girls were going to come regardless of if I was ready or not.

After a very quick trip home to get our bags, Steven and I arrived at Vanderbilt at 6pm. After getting settled in and ready, I was taken back to the OR at about 8pm. I know that it was kind of busy in the OR before the girls came, but I don't remember all that much. But, I do remember the girls being born. Hannah came first at 8:21pm, and immediately started screaming and I knew that she was ok. It seemed like an eternity, but it was only 2 minutes later that Amelia was born. She did not cry immediately like Hannah did, but she did start up with a little cough and then she started to cry. We were so relieved that both girls were doing so well, they were not struggling with their breathing at all. Hannah was able to stay with Steven and I in the OR and then go to the well baby nursery, but Amelia had to go to the NICU. I only saw her for a few moments before she was taken to the NICU. 8 hours after she was born, I was able to get up and go see her in the NICU, but we were not able to hold her yet.

One of the biggest risks for Amelia for the first 18 hours (until her surgery) would be infection due to her open spine. They kept a sterile bandage over her back until surgery, and she had to stay on her tummy. So, when she was 18 hours old she had her first surgery. Steven and I were able to be with her before they took her in and then afterwards when they brought her into the recovery room. When we saw her in the recovery room, she was lying on her back and it was the first time I had gotten a really good look at her face- she was beautiful.

It is so hard to even put into words the range of emotions and thoughts that went through our minds. The reality of spina bifida was staring us in the face. We have this amazingly beautiful and perfect baby, but her legs did not move. Her knees would not completely straighten out. Her toes did not wiggle when I tickled her feet. She had a large spinal deformity on her back, that is essentially a bump. She had hydrocephalus and we did not know how long it would be before she had her second surgery to place her shunt. Not to mention keeping an eye on bladder and bowel function. All of this was stuff that is always in the back of your mind, but I could look in her eyes and see that sweet face and the stuff didn't matter. Of course I wanted her to be healthy, but that was not the case. We made the decision to try our best to give her every opportunity to be all that she can be regardless of how much work it takes from us.

Hannah and I spent 4 days in the hospital. Hannah did really well, other than having a little trouble eating. She was so sweet and cute, and we couldn't get enough of her. Amelia spent a total of 17 days in the NICU. Those were some of the hardest days. I was so torn between my two babies- Hannah was at home with us, while Amelia was at the hospital by herself. We visited her everyday, but couldn't wait to get her home. Also, the hospital would not allow us to bring Hannah into the NICU, so the girls were seperated for their first 17 days.

When Amelia was discharged from the hospital, we were given a list of referrals and follow-up appointments. We would need have her hydrocephalus monitored very closely, keep a close eye on her bladder and kidneys, make sure that she was eating enough to gain weight, make appointments with early intervention, and then follow up on her newborn hearing screen. Amelia failed her newborn hearing screen in the hospital, but I had decided to completely blow it off. A lot of babies failed, but in actuality they could hear. Plus, when I talked to her she would look at me. I do remember looking at her ears while she was still in NICU and noticing that she had a skin tag in front of her right ear and I even made a comment to Steven about it. An ear tag could indicate either hearing problems or kidney problems, but usually they meant nothing. I TOTALLY blew all of this off. We decided that we would wait until after she had her shunt surgery before we would take her to have her hearing tested again.

First Pictures

I decided that an interesting way to start our family's blog would be by sharing our story. It is kind of long, so it might take a more than one posting to get it all in. What better way to start than by sharing the very first picture of the girls.

Obviously, this is not your average first picture, but I think it is really neat. The girls were conceived through in vitro fertilization on Sept. 27, 2002. This picture was taken 5 days after conception, just a few hours before they were placed into my uterus. 9 days later, I found out that I was pregnant (there was speculation that it might be twins due to the high levels from my blood test). My first ultrasound was on Oct. 30, 2002. I was 6 weeks pregnant. There were two babies with two heartbeats. We were thrilled that we were having twins, but still cautious. A little over a week later I became very worried, and just felt like something was not right. I called the doctor, and they brought me in for another ultrasound. Everything looked perfect and I was released to my regular OB, who I would see in 2 weeks. I had another ultrasound at 10 weeks, and then again at 13 weeks. Everything continued to look perfect, and at 13 weeks they took a really early guess at the sexes- they thought it was 2 girls.

January 13, 2003
I was scheduled for another ultrasound to check on the babies' growth, and this was the day that we should definitely find out the sexes of the babies. I was 17 weeks. Steven decided to take the day off, and we were going to go look at nursery stuff after my appointment and we were going to celebrate our anniversary a few days early (our anniversary is Jan. 16). About a week earlier I started getting worried, thinking that something was wrong. In my mind I had gone through all sorts of things that could go wrong with a multiple pregnancy (this is where being an OB nurse is not a good thing). It had never crossed my mind that something could be wrong with one of the babies.

Baby A's ultrasound was perfect. It was a girl and she was growing well, all measurements were on target. Steven and I had decided long before we were pregnant that our first girl would be named Hannah.

During Baby B's ultrasound was when our life as we knew it would change forever. Steven and I had both noticed that the technician seemed to be taking so much longer with this baby and she was printing off a lot of pictures. We both noticed that baby B's head did not look as round as baby A's. In fact her head looked almost oval. Neither of us said a word, but we both were getting concerned. As the technician finished up she showed us the sex of the baby, and it was another girl. Then, she wanted to show us some things that she saw during the scan. She showed us her head and then her spine. During this time she told us that our baby had spina bifida and hydrocephalus. Absolutely nothing could have prepared us for that moment. It was like somone had come and completely knocked the breath out of you. I at least knew what spina bifida was, but Steven did not have a clue. The next few days were filled with appointments to find out more about her diagnosis. About 2 weeks later we decided on Baby B's name, Amelia Grace.

After our appointments, we found out so much more about Amelia's spina bifida. For those that are not familiar with spina bifida, it is when the neural tube fails to close during gestation. It occurs within the first 28 days after conception. The lesion can occur anywhere along the spine, and depending on where the lesion occurs determines how affected the child will be. We found out that her lesion was at T-10 and was open to her sacrum. She would not be able to walk and would not have control of her bladder and bowels. She also had hydrocephalus, which would require a shunt to drain the excess fluid off of her brain. Hydrocephalus affects about 80% of children with spina bifida. We also found out that she had Arnold Chiari II malformation, which is a structual abnormality involving the lower brainstem, upper spinal cord, and cerebellum. About 85-90% of children with spina bifida have this malformation. Complications from this malformation only affect a small percentage of children, but they can be severe. We also found out that she had a spinal deformity called kyphosis, which is an outward curvature of the spine. We were told that most children with spina bifida have average to above average intelligence, but often have learning disabilities.

All that we could do for the next 4 months was wait. We would not know her true level of functioning, when she would require a shunt, or if she would even survive. We were scared and overwhelmed. We prayed a lot, cried a lot, and had faith that everything was going to be ok. We tried to prepare ourselves for what it would be like after the girls were born, but it is hard enough to imagine the impact that newborn twins bring. Much less, adding all of the medical issues that we would be facing with Amelia. Despite all of this, we were still so excited and ready to have our babies.