Well, we are back from Baltimore. If there is one thing I've learned this summer about traveling with a toddler, it's that you simply cannot leave home without a bag of lolly pops and a large package of baby wipes. Those 2 things can make or break your sanity. :)
Anyway, our consults went extremely well. We first met Dr. Gearhart, who was incredibly personable and smart. I immediately felt at ease about our child's condition when we talked with him. You just can't beat getting treated by someone who lives for babies with cloacal exstrophy!
We then had an u/s done by Dr. Crino. It was the best u/s yet b/c it didn't reveal any new problems. (That's a first for us.) Here is what we are dealing with:
1. A beautiful little one who loves to squirm and get into the worst positions possible while being observed on u/s. She's undoubtedly shy.
2. They cannot see an open spina bifida, although it was too hard to see in the u/s. They do see some scoliosis, hemivertebrae, and overall some deformity of the spine in the lumbar and sacral regions.
3. The pelvic bones are definitely spread out pretty widely.
4. The left foot is mildly clubbed, but the leg is measuring the correct length.
5. The right foot was too hard to see, but they are pretty sure her right fibula was missing. The leg looks a good bit smaller, as well.
6. She has a moderately-sized omphalocele (O) and exstrophy of the bladder, hence confirming the diagnosis of CE.
7. They still could not determine the sex.
A couple of great things that we learned during our visit with Dr. Crino were:
1. Her cerebellum looks good, so the chances of open spina bifida are lower.
2. She has 2 healthy-looking kidneys. Oftentimes with CE, the baby is only born with 1.
3. She was moving her left foot while we were observing it, and that indicates that she's probably not paralyzed - yippee!
4. Her heart was beating at a healthy rate - another yippee! Gosh, I can't tell you how wonderful it feels to see her beautiful 4-chambered heart beating.
He believes that since I was able to deliver Jack vaginally, that I can deliver vaginally this time, as well. They only like to perform C-sections for obstetric reasons, like if labor isn't progressing well or whatever. The O doesn't look too big to fit through the birth canal. Also, since Jack hung out in my womb until past my due date (I was induced 3 days after, and I wasn't even dilated at that point), the chances of me going into pre-term labor is small, so, he doesn't feel that there is a need to move to B'more before 37 weeks. Then, I will just wait it out and hopefully go into labor naturally. At ~39 wks, they will induce if necessary.
This news was huge for me. If the above paragraph was all I heard on Monday, it would've made all the inconveniences of traveling to B'more this week worth the trouble. Our plan is actually to arrive at 36 wks, since it will be a long drive, and things like sitting in a tiny Honda Civic is getting more and more uncomfortable everyday. The Children's House at Johns Hopkins, which is a place for families to stay while their children are receiving treatment (at a much lower rate than a hotel), will not allow us to stay there until after we give birth (and that's only if they have space), so I'm in the process of looking for extended-stay hotels outside the city. My guess is that John Mark will help us get settled, then fly back to Huntsville and work, so he doesn't use up all of his vacation time before the real fun begins.
OK, back to our consults...our last visit was with a neonatologist, Dr. Woods, who was a tremendous help in our understanding of our little one's stay in the NICU. We also toured the NICU, and although there were no babies with O's or anything, we were able to see what the feeding tubes, IVs, and ventilators looked like. She said that obviously, we don't know for sure everything that will be done and in what order, but we should expect our child to have her initial surgeries within the first couple days of life. When she's born, they will take her away, cover up her O and bladder with a bag to prevent the area from drying out and from getting infected. They will make sure she's breathing and all that jazz (minor details, right?), then get her ready for surgery. The initial surgeries will probably involve 1. repairing the open spina bifida, if there is one, or other neurological issues, 2. fusing the bladder, 3. placing all her organs into her abdominal cavity if possible, 4. fusing the pelvic bones and placing her on traction. She will be highly sedated and will have to be intubated during the surgery and recovery period. After about a week or two, she will be transferred to the infant floor, where she will have a private room...this will help us b/c the NICU doesn't have much privacy (at all), and this way, one of us can spend the night with her. She will probably be in traction for weeks, which I already know will be one of the hardest things for me to deal with. I can't believe how much I took for granted all the time I spent holding and cuddling with Jack when he was a newborn. I guess we'll have to replace all that with lots of kisses (eew, nevermind, it'll be flu season, lol) and hand-holding.
So, as you might have guessed, we pretty much decided to go with Hopkins. When we arrive in October, we will meet the neurosurgeon and orthopedic surgeon, as well as see Dr. Crino for continual monitoring. My prayer is that she feels comfy enough in my womb to make it to 37 wks, so that we don't have to worry about transferring her from Huntsville or B'ham.
One of the most rewarding parts of our trip (even though I wasn't present, since I was in the middle of the u/s) was when Dr. Gearhart brought a 6 yr old boy and his mother up to meet John Mark and Jack. The boy has CE and fibular hemimelia, has gone through 26 surgeries and counting, but plays and acts like any other boy. He was in B'more to get a bladder placed in his tummy (he was born without one, I believe). He also got his belly button! (Babies with O's don't have them unless a surgeon artificially creates one.) He was born with a very small foot on the side that had the missing fibula, so they simply fit a prosthetic over the area - no amputation was necessary. Although I wasn't able to meet him, it brought infinite comfort to know that our child will get through this and will eventually be able to be a kid. She won't remember a thing about having her legs stuck up in the air for 8 weeks, and she will look at her colostomy bag and catheter and think nothing of it. It certainly beats having to find a bathroom while hiking in the mountains, right? :)
Wednesday, August 22, 2007
Friday, August 10, 2007
Birmingham Surgery Consults
Well, earlier this week, I was blessed to see 3 surgeons at Children's in Birmingham. My sister-in-law took notes for John Mark (who had to work) while her family entertained Jack. It was one of the most overwhelming days of my life, and by the end, I was simply numb from all of the information that was thrown at me. We are discerning at this point whether to deliver and get treated in B'ham or travel up to Johns Hopkins in Baltimore to get treated by Dr. John Gearhart, a pediatric urologist who specializes in cloacal exstrophy . He sees about 5 to 6 new CE patients each year, while most urologists only see 1 every couple of years or more. I feel very confident that if we go there, we will receive the best treatment, but the logistics of it all is intimidating. Very often, CE babies arrive prematurely, so even if we schedule a C-section for 37 weeks, the chances of me delivering unexpectantly here are high. We are flying to B'more next week to have another u/s done with a perinatologist, Dr. Crino, who is very experienced at diagnosing CE. We are also meeting with Dr. Gearhart and a neonatologist who would be treating our child while in the NICU.
So, the gist of my meetings with the B'ham surgeons are described below:
Dr. Scott Doyle, the Chief of Pediatric Orthopedic Surgery, explained his roll in the initial treatment, which usually involves adjusting and realigning the pelvic bones and placing the child in traction. Later down the line, we will need to address her clubfeet and right leg. Her leg, which is missing the fibula, is measuring 11 weeks behind, so our options will probably include amputating part of her leg and fitting her with a prosthetic. They can also lengthen her leg. Considering the dozens of other surgeries that she will have to go through, I imagine that amputation is probably the best option for her. She would probably have it done within the first year or two of her life, when she starts learning how to walk (if she is in fact ambulatory and not paralyzed). The leg lengthening is much more drawn out and sounds really painful, so in my heart, I feel that amputation would be best for her. Obviously, it's too soon to tell. Her left clubfoot doesn't appear too severe, but since there is a high chance that these deformities are a result of nerve under-development, they may have difficulties treating it conventionally (via the Ponseti Method) w/o resorting to surgery.
Dr. Joseph, the pediatric urologist, was very awesome, and did a wonderful job explaining what was involved in CE surgeries. It is an extremely complicated birth defect b/c it involves so many organs, and there is much that we won't know until she is born. He discussed the possibility of kidney dysfunction (our child's kidneys look ok, thank God), and the surgery involving the bladder fusion and genitalia. There is a big chance that she will remain incontinent, but that's not something we will know until later down the line. There's also the issue of gender. Since we haven't gotten an amniocentesis done, we aren't sure whether we have a little daughter or son. It will definitely be more complicated if it is a boy, but again, we're just going to worry about that when the time comes. In regards to her bowels, it is almost always the case that these kids have inperforate anuses. Therefore, she will most likely have a colostomy for the rest of her life. Oh yeah, and I almost forgot about her omphalocele...it was 3.8cm last week, but all we can do is pray that it doesn't grow much bigger.
Dr. Oakes, the pediatric neurosurgeon, explained to us that oftentimes, the perinatologist cannot see an open spina bifida, so we should not rule that out. If she does have spina bifida, hydrocephalus is a very common result, which will require a shunt to be placed in her brain to drain the fluid. Normally, they will drain it into the abdomen, where there is a good bit of space to add extra tubing. This is necessary b/c as the baby grows and lengthens, there needs to be enough tubing to grow with it. But in our case, since her abdomen is kind of in shambles, they will need to drain it into the heart, which does not allow for extra tubing (smaller space). So, as she grows, they will need to cut her open and add extra as needed. This is a scary prospect, but I appreciate him preparing us, b/c I was not aware of how common hydrocephalus was for spina bifida patients.
Sorry...lots of information. There is a ton more, but I'm exhausted just thinking about it! Please keep our little one in your prayers. She has a long road ahead of her, as you can see. And please pray for us as we continue to discern where to get her treated. Hopefully after our trip to Hopkins, we will have a better idea of what to do.
Love,
Michele
So, the gist of my meetings with the B'ham surgeons are described below:
Dr. Scott Doyle, the Chief of Pediatric Orthopedic Surgery, explained his roll in the initial treatment, which usually involves adjusting and realigning the pelvic bones and placing the child in traction. Later down the line, we will need to address her clubfeet and right leg. Her leg, which is missing the fibula, is measuring 11 weeks behind, so our options will probably include amputating part of her leg and fitting her with a prosthetic. They can also lengthen her leg. Considering the dozens of other surgeries that she will have to go through, I imagine that amputation is probably the best option for her. She would probably have it done within the first year or two of her life, when she starts learning how to walk (if she is in fact ambulatory and not paralyzed). The leg lengthening is much more drawn out and sounds really painful, so in my heart, I feel that amputation would be best for her. Obviously, it's too soon to tell. Her left clubfoot doesn't appear too severe, but since there is a high chance that these deformities are a result of nerve under-development, they may have difficulties treating it conventionally (via the Ponseti Method) w/o resorting to surgery.
Dr. Joseph, the pediatric urologist, was very awesome, and did a wonderful job explaining what was involved in CE surgeries. It is an extremely complicated birth defect b/c it involves so many organs, and there is much that we won't know until she is born. He discussed the possibility of kidney dysfunction (our child's kidneys look ok, thank God), and the surgery involving the bladder fusion and genitalia. There is a big chance that she will remain incontinent, but that's not something we will know until later down the line. There's also the issue of gender. Since we haven't gotten an amniocentesis done, we aren't sure whether we have a little daughter or son. It will definitely be more complicated if it is a boy, but again, we're just going to worry about that when the time comes. In regards to her bowels, it is almost always the case that these kids have inperforate anuses. Therefore, she will most likely have a colostomy for the rest of her life. Oh yeah, and I almost forgot about her omphalocele...it was 3.8cm last week, but all we can do is pray that it doesn't grow much bigger.
Dr. Oakes, the pediatric neurosurgeon, explained to us that oftentimes, the perinatologist cannot see an open spina bifida, so we should not rule that out. If she does have spina bifida, hydrocephalus is a very common result, which will require a shunt to be placed in her brain to drain the fluid. Normally, they will drain it into the abdomen, where there is a good bit of space to add extra tubing. This is necessary b/c as the baby grows and lengthens, there needs to be enough tubing to grow with it. But in our case, since her abdomen is kind of in shambles, they will need to drain it into the heart, which does not allow for extra tubing (smaller space). So, as she grows, they will need to cut her open and add extra as needed. This is a scary prospect, but I appreciate him preparing us, b/c I was not aware of how common hydrocephalus was for spina bifida patients.
Sorry...lots of information. There is a ton more, but I'm exhausted just thinking about it! Please keep our little one in your prayers. She has a long road ahead of her, as you can see. And please pray for us as we continue to discern where to get her treated. Hopefully after our trip to Hopkins, we will have a better idea of what to do.
Love,
Michele
Wednesday, August 1, 2007
26 weeks...another update
Well, we just got back from B'ham, where we had another u/s done. Good news first: her heart and brain still look great. And it doesn't look like the liver is inside the omphalocele. Bad news: her condition may be way more complicated than we had originally thought. The perinatologist thinks she has cloacal exstrophy, which is a very rare congenital birth defect. We aren't even sure if it's a girl (hopefully it is, b/c things sound way more complicated if male genitalia are involved). The reasons the doctor suspects it's cloacal exstrophy is b/c her pelvic area is wider, as if during early development, that area didn't fuse together properly. It was even apparent to us on the u/s when we looked at the spinal column. As it reaches the sacrum, the parallel vertebrae curve outward. Omphaloceles are also a result of this condition. She cannot see the bladder, which she suspects is because it is split in two. We saw no opening through the skin, so spina bifida is not probable.
In regards to her extremities, the left foot doesn't look severely clubbed. The right foot was too hard to see, but she is banking on it being severe and irreparable, mainly b/c her fibula isn't present. (Yes, a slightly minor detail they failed to notice at the last u/s.) Without a fibula, obviously her Achilles tendon and whatever else can't function properly. Her right calf is also smaller than her left, so she assumes there is less muscle mass, as well. I can't exactly imagine what not having a fibula is like, but the likelihood of her having use of that leg seems minimal.
So, our priorities are to safely deliver her and have immediate surgery on her omphalocele (although, this may not be possible...her abdominal area is measuring small, so there may not be room initially for whatever organs need to be squeezed back in). In addition, they need to fuse the two halves of the bladder together (this also needs to be done to the uterus, clitoris, penis, or whatever else is involved). Our other major concern is making sure there is proper urine and bowel output...often with this condition, the rectum is closed, and there are issues with the nerves controlling the lower urinary tract. The clubfeet will definitely take a back burner until she is stable.
But on a brighter note, she looks beautiful. We saw a wonderful profile of her face, and she looked just like me. ;-) I feel her moving around constantly, and we are so anxious to finally meet her. We will most likely deliver in B'ham b/c they have the only pediatric urologist in the state. It will be nice to have a pediatric neurosurgeon available, too. I asked the perinatologist if there are any physicians in the U.S. who are more experienced with this type of diagnosis, but she thinks it is too rare for any one doctor to be considered an "expert".
So, please keep praying. I'm sorry if my post doesn't make a lot of sense. Hopefully, we'll have more concrete answers soon, although I'm guessing we won't know what's really going on until she is born.
Thanks again and take care,
Michele
In regards to her extremities, the left foot doesn't look severely clubbed. The right foot was too hard to see, but she is banking on it being severe and irreparable, mainly b/c her fibula isn't present. (Yes, a slightly minor detail they failed to notice at the last u/s.) Without a fibula, obviously her Achilles tendon and whatever else can't function properly. Her right calf is also smaller than her left, so she assumes there is less muscle mass, as well. I can't exactly imagine what not having a fibula is like, but the likelihood of her having use of that leg seems minimal.
So, our priorities are to safely deliver her and have immediate surgery on her omphalocele (although, this may not be possible...her abdominal area is measuring small, so there may not be room initially for whatever organs need to be squeezed back in). In addition, they need to fuse the two halves of the bladder together (this also needs to be done to the uterus, clitoris, penis, or whatever else is involved). Our other major concern is making sure there is proper urine and bowel output...often with this condition, the rectum is closed, and there are issues with the nerves controlling the lower urinary tract. The clubfeet will definitely take a back burner until she is stable.
But on a brighter note, she looks beautiful. We saw a wonderful profile of her face, and she looked just like me. ;-) I feel her moving around constantly, and we are so anxious to finally meet her. We will most likely deliver in B'ham b/c they have the only pediatric urologist in the state. It will be nice to have a pediatric neurosurgeon available, too. I asked the perinatologist if there are any physicians in the U.S. who are more experienced with this type of diagnosis, but she thinks it is too rare for any one doctor to be considered an "expert".
So, please keep praying. I'm sorry if my post doesn't make a lot of sense. Hopefully, we'll have more concrete answers soon, although I'm guessing we won't know what's really going on until she is born.
Thanks again and take care,
Michele
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