Finally, Monday rolled around. With all of our hospitalizations, one of the things I have grown to hate is weekends in the hospital. In our experience, the weekend “doctors” usually consists of residents. Not that they are bad people or Dr.’s, but when you are dealing with a rare condition to begin with, residents do not have the knowledge to advise you. In some cases, they cannot even give you confidence that the hospital knows what it is doing. During our journey there has been many times where I received misguided or misinformed information from residents. There have even been episodes where I have thrown residents out of my daughter’s hospital room, more on that later.
Upon consultation with surgeons in the areas of urology, general surgery, genetics and orthopedics we found out several bits of information. First, our daughter was indeed a girl!!! Second, she had exstrophy (OK, we sort of knew that already), but they did not yet know how severe it was (ie. They didn’t know what her insides looked like). And the final bit of news was that she would be having surgery later that day.
In the last couple of days, we learned that the effects of exstrophy can be “repaired”. Not “cured,” as in make it go away completely, but “repaired” to improve the quality of life for the child. Surgical repair of exstrophy is referred to as “staged reconstruction.” This means that several different surgeries are required to accomplish the best possible results. The actual number of surgeries varies from child to child and is also dependent on the severity of the exstrophy.
The first goal of my daughter’s first surgery was to find out what her exact anatomy looked like. Tests that had been run, up to this point, were inconclusive as to how everything fit together and looked inside. The surgeons also hoped to close the bladder, urethra and abdominal wall and to create a colostomy.
The surgeons explained to us that after surgery our daughter was going to be placed in traction. Her legs would be placed together, wrapped and then positioned in a 90 degree angle to her body and held there with weights. They explained that this would bring the pelvic bones together and would be done for two reasons. One reason was to hold together the closure of the bladder and abdominal wall after surgery so that it could heal.
The second reason was to try and correct one of the effects of exstropy, which was wide set pelvic bones. The pelvic bones provide protection of the lower part of the bladder, urethra, and the muscles of the wall of the abdomen. The muscles of the pelvic floor, which help to stop urination and provide continence, also are located there. The surgeon’s explained that the opening in the pelvic bones would get bigger as my daughter grew and would not provide the protection and support needed for my daughter’s internal organs and muscles in that area. They explained that trying to close the pelvic bones, in this way, at this time was important because the pelvic bones were still soft. They hoped that this attempt would prevent my daughter from having a hip osteomy later.
At about 7pm that night my daughter went in for what was supposed to be a couple hour “exploratory” surgery. Five hours later, the two surgeons (urologist and general surgeon) emerged with good news. They reported that my daughter had all her internal organs (with the exception of one kidney) including all her female organs. They closed the bladder and internalized it, reconstructed a portion of the urethra and did some cosmetic work on the outside where only one tiny opening existed before. Her abdomen cavity was closed and a colostomy was created. They said they did a little more than expected because they found more to work with than they expected. They did say that the bladder was still attached to the intestines through a fistula and would need to be separated later in life, but because she was so small they wanted to allow her time to grow and gain strength before the next reconstruction and further separation.
All in all it was good news. I already knew that there would be more surgeries in the future so that was not a surprise. Everyone already knew that she had a form of bladder exstrophy and that the severity could not be determined until after this surgery. Her diagnosis was now cloacal exstrophy, but the surgeons felt confident that, with further reconstructive surgeries, her outcome was going to be good. The surgeons prepared us for the worse and it was finally a pleasant surprise to find out that something was better than expected.
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