The First Surgery

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. 

Day 2

I spent the night in the hospital, away from my baby.  The next morning, as waves of consciousness washed over me, I thought, for those few blissful moments, that everything was alright. I was still pregnant and everything was alright.  A few moments later I opened my eyes and it all hit me like a dump truck of bricks.

I knew my baby was in Children’s Hospital of Wisconsin and, from the phone call from the hospital the night before, she had settled in well.  There was nothing for me to do in a hospital 40 miles away.  I wanted to be with my baby.  I wanted to find out what was wrong and what I could do to make her life all it could be.  They had given me the number to the NICU and I called it right away.  They informed me she was doing well, but she was an ornery little girl.  They told me she had pulled out all of her tubes, iv’s, etc.  They had to shave a portion of her head (yes, she had thick beautiful brown hair – and it was long in the back!) to insert an iv into her head.  I remember half laughing at her my daughter’s antics and half crying about the still unknown situation we faced.

My obstetrician came in later in the morning and fully understood that I wanted to be released and wanted to be with my baby.   He informed me, before signing my discharge papers, that he reviewed every ultrasound I had during my pregnancy looking for something that would indicate my baby was going to be born with exstrophy.  He said the way the pictures were taken and the way she was positioned there was just no way to see it.  That may or may not be true, but at this point I figured what happened, happened and we will learn to deal with it.

A nurse came in and gave me my discharge papers, a prescription for pain meds, and the new baby packet.  It contained the card with my daughter’s footprints on it, coupons for baby items, a pacifier, a soft baby book, and the memento birth certificate.  I looked at the memento certificate and saw that they left the “sex” of the baby blank.  The nurse saw me looking at the certificate and again indicated, “We cannot mark the sex of the baby because we don’t know.”  I thought to myself, yeah, you and everyone else in this hospital have said that.  The packet was also supposed to come with a little baby bracelet, beaded in either blue or pink beads, reading either “boy” or “girl”.  They had omitted that altogether.

When we left there were no Congratulations or good wishes that I had anticipated for the last nine months.  It was, “I’m sorry” and “Good Luck” said solemnly and with pity. It was at that point that I said to myself I will not allow my daughter’s life to be filled with “I’m sorry” and pitiful “good luck” wishes and glances.  She was beautiful and she was feisty.  With all the problems I had during my pregnancy (almost miscarrying at 9 nine weeks among other things) and now with the complications she was born with, she was still fighting and giving the NICU at Children’s Hospital hell.  If she was going to fight so was I and I decided that she was going to be all that she could be.  At that point, I was not sure what that was, how much she could do, or quite frankly, how long she would be around to do it, but she was going to be all that she was set on this earth to be.

What I found at the Children’s Hospital of Wisconsin was miraculous.  Up until that point in my life, I had never had any reason to experience the inside workings of a major medical center, especially an NICU.  The monitors, machines, beeps, buzzes, wires, tubes, incubators, warming lights and many other forms of equipment met us.   The smell was medicinal and chemical.  I’ll never forget that smell. The incubators stood in rows and some of them were covered with baby blankets and quilts, but some were open and you could see the tiniest little beings grasping on to life.  I tried not to look, because really all I wanted to see was MY baby.

She was toward the back and when I came up to her incubator I was surprised.  Not shocked as the nurse told me some of what to expect but still surprised.  She had an ng (nasogastric) tube, and the iv in the side of her head.  She had little arm boards on as she was being ornery about pulling at her tubes.  She was supposed to be covered with a blanket or quilt, but as she was to become known for, she had kicked them off.  Her exposed bladder was covered with a dressing and she had a catheter inserted.  She had a diaper fastened loosely around her waist but I think it was more cosmetic because she had no openings down below (no anal or vaginal opening) except one tiny one sitting about an inch below her exposed bladder where they had the catheter inserted.  She was awake and alert when we got there and was taking in all of her surroundings.

We were allowed to visit with her awhile and then the information barrage started.  They told us what tests they had run and what they knew upon initial examination. I got to talk to the genetics Dr. (the Dr. not the residents on duty during the weekend) though and finally someone told me that my daughter’s condition was not fatal.  She was going to make it!!!  He said that it was too early to tell the degree of exstropy she was born with as everyone was still examining x-rays and ultrasounds but that she was going to make it.  That was another time where I felt myself let out my breath when I hadn’t even realized I had been holding it.  I remember him telling me, the concern should not be whether or not she is going to have a life because she will.  The question was what quality of life she will have based on the severity of her condition.  I made him repeat it again.  I don’t really know why other than to re-enforce in me that my daughter was going to have the best quality of life that I could provide her with her current condition.  We were not going to be beat down by this. We were going to learn and grow with this and make the best we could out of it.

Did I still have those “lost” dreams of a healthy child?  Yes, they crept up here and there especially as we entered more and more surgeries but I didn’t dwell on them.  My job, as I saw it, was to arm myself with the strength, knowledge and the tools necessary to provide my daughter with the love, support, knowledge and tools in order for her to have the best quality of life she could have in spite of her condition.

The other memorable moment I remember about talking with the genetics Dr. was the question of why did this happen?  I hounded the man with everything I could think of.  I asked about the foods I ate, the medications I took, the work environment I was in, the water,  the air, the area I lived in, what I used to clean the house, the activities I participated in, the color of the sky, whatever entered my mind as to what I possibly did to cause this.  He assured me that NOTHING environmental “caused” this condition.  IT WAS NOTHING I DID.  AND THERE WAS NOTHING I COULD HAVE DONE TO PREVENT IT.

Birthday!

Welcome

Hello, if you are reading this, you may have just discovered that your child, or a child in your life, was born with bladder, cloacal or another of the various forms of exstrophy.  Well, I am writing this because I am the parent of a daughter born with cloacal exstrophy over 10 years ago.  Exstrophy is a rare condition requiring specialized medical care and expertise.  It does not go away, your child will not “grow out of it” and while it can “repaired” through staged surgeries, it will affect your child for a lifetime.

At this point, you may be looking for information about this unique condition.  “Ignorance is bliss” may or may not be comforting to you at this point but it will not carry you very far into this journey.  So get up, dust yourself off and get back in the saddle.  My aim is to arm you with some information.  As this journey continues YOU will be the source of information concerning your child and exstrophy.

The diagnosis of bladder exstrophy is frightening, in the beginning.  Many parents first learn about exstrophy soon after the birth of their child, as we did.  The news came as a surprise even though I had a difficult pregnancy.  I had a feeling through my whole pregnancy that something was wrong with my daughter, but not something as drastic as exstrophy.  I had been through several prenatal ultrasounds because of my insistent urging to my obstetrician, but everything “looked good”.  He wrote me off as a nervous new Mom – to – be.  After my daughter’s birth, I, like other parents, was catapulted into the world of not only having the normal hopes and dreams for my newborn daughter, though at that particular point in time I questioned them, but also into a maze of a rare, complex and chronic health condition.  I was disoriented.  It was one of those times in my life where everything looked different, sounded different, felt different and even smelled different with the accompanying feeling of when am I going to wake up to realize this is not real.  Later I found that to maneuver through the maze I had to learn a new language (medicalese) and start drawing a new map.

After months of reading pregnancy and baby books, counting the weeks on the calendar and comparing what my daughter might look like at any given week with the in utero drawings in the books, this was not the birth experience that I dreamt of.  My feelings and emotions were already heightened due to a difficult pregnancy, anticipation, worry, having to be induced and then finally going through labor.  Yep.  I delivered naturally and with no epidural.  With the induction, my daughter and my body decided that it was going to sluggishly go through the whole day with me only being able to dilate to 3.  Then within ½ I went from 3 to PUSH!!! 

When my daughter finally arrived the whole atmosphere of the delivery room changed.  Everything went quiet.  My daughter wasn’t crying and no one was saying anything.  I began to worry.  Were my instincts throughout the pregnancy correct?  After a few seconds my daughter let out a howl and then never stopped crying.  I let out my breath (I hadn’t even realized I had been holding it).  Now I waited for news from the Dr.  Nothing.  Wasn’t there supposed to be the … BIG ANNOUCEMENT… The congratulations it is a boy or girl?  Nothing.  So I finally asked?  Is it a girl or a boy?  Is everything alright?  A nurse came to hold my hand as the Dr. announced there is a problem with the baby.  My whole line of vision went black, and then, as the images in the room started to reappear I asked again, “Is it a girl or a boy?”  My Dr. simply said, “We don’t know.  There is an area of the abdomen where the internal organs are on the outside and the “deformity” extends down to the organs of gender and we cannot tell.”  What?  You cannot tell?  I had never heard of such a thing.

My child was born with a “deformity” as the Dr. announced.  He said it was very rare and he wasn’t sure if this is what the “deformity” was, but it was called exstropy.  I don’t remember too much more about what was said at that time.  All I remember is asking over and over again, “Is she going to be OK?”  I didn’t yet know for sure if my daughter was indeed even a girl but I had a gut feeling she was.  I remember the nurse patting my hand and telling me, “Everything is going to be OK.”  However, I wasn’t sure if she meant it or if she was just trying to keep me from becoming hysterical.  I don’t think she knew.

My daughter was over being “attended” to and she was wailing.  A testament to the feisty attitude she still holds on strong to today.  I heard the nurses say that her apgar scores were all 9’s and 10’s.  I thought to myself. 9’s and 10’s, that's good right?  From what I heard and read that was good.  She couldn’t be that bad.  My husband was over looking at her and then came to my bedside and said, “You were right, it is a girl.”  The nurse quickly reprimanded him and said, “We don’t know that.  What you are seeing is the birth defect; the absence of a penis does not indicate it is not a boy.”

They wrapped my daughter up tight and I got to hold her for a minute and then she was whisked away to the intensive care unit.  Well, what little one they had.  They informed me that she would be transported sometime that night to a bigger facility because they did not have the expertise to deal with that type of birth defect.

The Dr. continued working on me and a little while later the neonatologist came in and said that my daughter was born with bladder extrophy.  They were not sure of the extent, however, as exstrophy is a spectrum defect.  He said he tried to “feel” around and suspicion that my daughter was a girl but that genetic tests would have to be performed to be able to tell for sure.  He said that my baby was going to be sent to the Children’s Hospital of Wisconsin in Milwaukee, WI, about 40 miles away. 

I felt overwhelmed, sad, frightened and confused.  I was grieving the loss of my notion of the perfect delivery and healthy baby.  I feared what the future held for my baby, as well as, for my family.  All that was wrapped up in the guilt I felt of what did I do wrong? At this time, the professional were the experts and I was the bystander. 

Since exstrophy is a rare birth condition, there are few doctors experienced in the surgical repair of the newborn with exstrophy.  Accessing the best care for my daughter on my own, at this point, was impossible.  So I entrusted the care of my precious newborn daughter to people I didn’t even know.  It was a very difficult time to think about the types of questions that should be asked.  This was just the beginning of the many stages of my daughter’s development that would be challenging and altered because of the condition of exstrophy.

By the time I was able to get up and go to the nursery to see my daughter the whole extended family was there.  My, now ex- husband, in his panicked state called everyone and since everyone was local they all came to the hospital.  In a sense, it was nice to have them there for support and also that way I wouldn’t have to explain everything over and over again to everyone.  Everyone was already there.  However, in another sense it was overwhelming and stressful too.  I wanted time to see my daughter and be alone with her before they transported her and I had to stop and have everyone question me as to how I was feeling, what I should be feeling and how I was suppose to act.  My, now ex sister-in-law actually said to me, “Do you know what is going on?  You are acting so out of it.”  Then again that family is so overly emotional that if you are not wailing in the hallways, you are not acting “appropriately.”  To do it over again, I would not have had everyone there.

Some parents have found that by limiting phone calls and visits from family and friends in the first 24 hours they can save themselves explanations about the unknown.  Once you have solid information about your baby’s health status you can determine how you will disseminate this information.  You might want to set up an account with caringbridge.org.  This website allows families, who are experiencing health challenges, connect with family and friends, privately, to give regular updates.  This way you can control the information that goes out and don’t have to deal with well meaning but sometimes intrusive questions.

I got to go into the nursery for a few minutes before the ambulance came to transport my baby.  She was still wailing up a storm.  At first, I was afraid to even touch her.  Finally, I held out my finger and put it in her hand and started to talk to her and as soon as she heard my voice she stopped crying, grasped my finger tight, and looked me in the eyes.  She was lying in the bassinet with her diaper open and I could, for the first time, see her abdomen and the protruding red mass of internal organs slipping out the opening.  To be honest, I didn’t know what to think, I had half heard so much in the last hour or so, I was still trying to process all that was happening and then the transport personnel came to take her.  I do have to say they were a wonderful bunch of people.  They didn’t rush in and ram you over while busily going about their business.  They explained what they knew to me and they described everything they were doing to my baby and why.  After they had her situated for transport they let me have time to say good-bye and then took a couple of Polaroid pictures for me to hold unto.  They gave me a time estimate of what it would take to transport my baby to The Children’s Hospital in Milwaukee and get her settled in the Neonatal Intensive Care unit up there.  They also said that someone would call from the hospital to let me know how she did on the trip.

I took everything in and then watched as they wheeled her out of the nursery, down the hall and into the elevators.  I stood in the hallway, holding my Polaroid pictures and finally at that point allowed myself to cry.


LINKS:
http://www.caringbridge.org/