Where to begin?!? We arrived at Hopkins at 5:30am to an operative waiting room absolutely full of families. Families who were quite obviously from all over the world. Pretty cool, not something one sees every day so dark and early. Once we were called back, the pre op nurse saw that Elise was an "exstrophy baby" and she became excited because "Exstrophy kids are always my favorites." We were already impressed since so many medical professionals we have come across don't really know what exstrophy fully entails. We met several people before Lisie went back for her EUA (examination under anesthesia) and we continued to be impressed by every one of them. Even the main surgeon we were there to see stopped in to chat with us before hand. If anyone has much experience with surgeons, especially a good one, they typically have pitiful social skills and bedside manner. We didn't run into this even once at Hopkins.
During her EUA, Elise was evaluated by a urologist, orthopedic surgeon and a general pediatric surgeon. She also had a clinic eval later on that day with a neuro surgeon. Here's what we learned from each doctor:
Neuro (detethering her spinal cord): He did suggest that her cord should be detethered before her major closure surgery. He also suggested waiting another 3-6 months for optimal timing for surgery and that recovery should be about a 5 day hospitalization. We are planning on doing this at JH sometime this summer. Btw, this neuro surgeon trained directly under Dr. Ben Carson (1st neurologist to ever successfully separate conjoined twins and hopeful presidential candidate for upcoming term. Pretty cool.)
The rest of the surgeons will be in coordination for her major closure surgery. The urologist is the "head" of this team although the orthopedic surgeon drives the timing of surgery based on growth and that her bones are not fully formed yet. He likes to wait until the kids are 18-20 months old so that would put her closure surgery at late 2015/early 2016.
General Pediatric Surgeon (deals with her omphalocele and intestines): Surgeon said not to wrap her omphalocele ("O") before surgery. He said it may make closure easier but it won't change the number of surgeries for closure and if it's making her throw up what's already hard to get in her then it's not worth it. (Yay!) He also is hopeful that he can close her "O" in one surgery with using synthetic and donor tissues. He also stimulated the area where her anus should be to see if she has any of the muscles needed for normal defecation. Another Yay, she does have those muscles! He thinks between the fact she has those muscles and her good growth should indicate a decent length of large bowel that a pull through could be in her future! This isn't 100% and may not be advised later on but it is encouraging!
Ortho (dealing with her pelvis): This is the hard part of this big surgery sequence. Typical people have a space of only 1cm between their pubic bones. Well, the ortho surgeon measured the distance between Elise's pubic bones and found that she has a separation of 9.3cm. This means when we go for the closure surgery she will need an initial surgery to just do the osteotomies (breaking her pelvic bones) and placing certain rods and cranks so that over the first 2-3 weeks they will slowly crank her pelvic bones together before the big closure surgery. Her separation is simply too wide to bring the pelvis together during one surgery. After the 2-3 weeks of slowly closing her pelvis, the major surgery of closing her bladder & omphalocele will occur. During this surgery, ortho will place different pins and rods that will stay in place while Elise is in traction for an additional 6-8 weeks. That means 8-12ish week hospitalization. Here we go again!
Urology (bladder): This surgeon is the "captain" of the team as the other surgeons call him. He is THE urologist who trains all the other top urologists across the country. He has patients who fly to him from all over the world for reconstruction. He sees more exstrophy cases than anyone else in the world. Exstrophy is rare in itself and Cloacal exstrophy is the rarest type of exstrophy. Even still he sees 6 new cloacal exstrophy cases per year. We're pretty excited to be working with him. He did have some disappointing news for us though. When Elise was born her bladder was separated into two sides with intestine in the middle. Those bladder halves were brought together during her first surgery though her bladder still has two distinct halves. The urologist told us that the right side of her bladder the tissue is "okay but not great" and the left side of her bladder the tissue is "simply junk". This means her current bladder tissue will probably not be useful to her for the long run. He is going to try to create a bladder out of this tissue during the closure surgery but it most likely will not last long or grow with her. She will continue to simply drain into a diaper like she does now (but out a urethra that will be created for her). She'll do this until she's older and starts asking to wear big girl panties and wants to be dry. At that point we'll do one of two things: She'll either have a pseudo bladder created for her out of part of her small bowel or have a urostomy placed until further notice. A urostomy is where the ureters bypass the bladder completely and are brought together into one tube and a stoma is created on her abdomen, much like her colostomy. She would then just drain into a bag and change the bag as needed. These are both kind of temporary fixes since the surgeon said he can guarantee that around the time Elise is in puberty, "they" will be growing bladders through research. What he didn't say but we understood is that "they" means Him. He has been doing research for some time and is already having success with growing human bladder tissue. So, even though she will have some years without a great means for urination, she already has a connection to the person that will change hers and the lives of so many others with this ground breaking research. Another thing that he mentioned is that they are not so focused on the here and now in terms of creating short term remedies for her struggles, but are trying to do what will be best for 40 year old Elise with two children and 60 year old Elise with grandchildren. (Talk about a tear jerker.) He even went into the logistics of what childbirth would look like for her! I just love thinking about Elise at that stage of her life and am so thankful that the Lord has brought us to the best surgeons available to reconstruct her little system in order to have a bright and "normal" future ahead.
The rest of our day was also informative in many ways. We were given a tour of all of the possible areas she would be in the hospital and were even able to meet a family who had a child currently in traction after bladder closure. They brought us into their son's hospital room with open arms, answered every question we had and even showed us every bit of what traction looks like up close and personal. We feel much more comfortable after seeing what her stay would look like. We learned from them that a big part of recovery is pain management and finding the right "cocktail" for that specific child. And then, of course, trying to wean them off of all that pain medication after coming off traction. We have already seen what it's like to wean Elise off heavy pain meds and it was terrible. Not looking forward to that. This is the first exstrophy family/child we have ever met in person and we felt an instant connection to them. I hugged that mama's neck tight before leaving and plan to keep in touch. Johns Hopkins actually hosts a picnic for all their exstrophy families every June! We look forward to getting involved in those and have Elise make some friends who understand what her day to day is like and maybe walk through life together with them, even if from a distance.
We were even given a tour of the temporary stay facility for families just down the street from the hospital. This facility seems to have answered all of the negatives about RMH (Ronald McDonald House) and are doing things much more catered to create a stress free place for parents. We would not be required to stay every night and family would be allowed to stay any time. They will not kick us out if we don't check in with them one day and there is no time limit. They view their part as a place for parents to come take a nap or shower or stay the night as needed and not have all the strict requirements that we experienced with our NICU time at RMH. Yet another stressor off our plate!
So in summation: everyone is wonderful at Johns Hopkins and we are excited to work with them for her reconstruction. The plan is hopefully to detether her cord in July-ish of this year and we're hoping for a January 2016 closure if the surgeons agree that would be good timing. We would like to have Thanksgiving/Christmas at home if it would not be hurting her to do so.
Thank you all for your continued prayers!!! You have no idea how much we feel them every day! Praise the Lord for our sweet angel baby, wonderful doctors and our amazing support system!
Yes, my dear. You are definitely Fearfully & Wonderfully made!
