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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/