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