Saturday, May 29, 2010

I Love Prunes! (Not Really)

First of all, I promise that this kid does smile--just not for the camera!

At Sadie's last appointment, she was an incredible 13 lb. 6 oz. and 23 inches long!  She's come so far, but she still has a long way to go.  With persistent cloaca her bowel and bladder communicate.  So recently, Sadie has had urine coming out of her mucus fistula (the opening to the lower part of her bowel).  Dr. Rink told us that this can be expected.  He wanted to check some bloodwork to make sure her bowel wasn't reabsorbing the excess sodium, etc. that her bladder should be emptying.  That could impact her acid-base balance.  When she had her blood drawn we discovered that she is anemic, so she is now taking an iron supplement.  Her pediatrician recommended that she start drinking some prune juice to keep her from getting constipated.  Apparently, we have a potential picky eater on our hands!  She refused the prune juice and shot us a look like we were the worst family in the world.  We tried repeatedly, and she refused repeatedly.  So we tried apple juice with the same results.  I decided to try baby food prunes with a spoon.  You can see how that's going, though we try at least once a day.  (I don't think we're going to see any results, unless prunes can be absorbed through the skin.)  Otherwise, things are going great--we heard Sadie's first big belly laugh last week!

We have a surgery date:  Sept. 22 at 9 am.

Sadie will be admitted on the 21st for bowel prep.  This surgery is called a PSARVUP (posterior sagittal anorectovaginoureteroplasty).  The goals of this surgery are to put everything back together, achieve continence, and allow her the ability to concieve when she's an adult.  Basically the urologist and surgeon will go in together and see what her internal anatomy looks like and reform the channels that should be there.  The combined surgery will take about seven hours, and she'll be in the hospital for about a week.  She will go in a couple of months later and have her vesicostomy and colostomy reversed.  When it's all said and done she will have about six surgeries.  Then, when it's time to potty-train we'll be able to determine if anything further will need to be "tweaked."  She will also be having an MRI of her spine to determine how her syrinx will impact her development, or if it will have any impact at all. 

As for the rest of us, Elena's looking forward to the end of school--3 days left!  Chris is going to the Indianapolis 500 tomorrow, as we do every year.  One of his friends is taking my ticket, since I'm not sure how to make breastfeeding work while watching the greatest spectacle in racing.  Chris and I are just plugging forward, working, and preparing for round 2 at Riley.

Thursday, May 20, 2010

Progress in Pictures

Just a few pictures of Sadie's progress since she came home. Because of the H1N1 restrictions, her big sister was not allowed to visit her in the hospital. So, she didn't get to meet Elena until she came home.
We didn't leave the house much those first few weeks, except to return to Riley for appointments. (That's all we did for awhile!) After initially being told that Sadie's repairs would be done at about age 2, we were surprised in early February to learn that her surgery would be done after 6 months. She was cleared by pulmonology after her first visit. Dr. Rink, her urologist, was very pleased with her progress at her first appointment. Her renal ultrasound revealed two normal kidneys! After mentally preparing ourselves for the possibilty of permanent kidney damage and possibly dialysis, we were told that her kidneys were just fine. Such a huge answer to prayer--one of many. Sadie failed her first swallow evaluation in Feb. but was able to pass in March. After feeding her through an NG tube for about 10 weeks, Sadie was finally able to eat normally. Chris snapped a picture as I took out her NG tube for the last time. I was crying hysterically. It was like we were bringing her home for the first time. Except for her ostomies, she was a fairly normal baby with whom we could do normal things. Since that day Sadie has enjoyed tub baths, walks in her stroller, going to church, etc. We try to keep life as typical as possible, but despite her progress we get frequent reminders that Sadie still faces difficult times.

Thursday, May 13, 2010

A Diagnosis

This is how Sadie looked the first time I got to see her. Her belly had actually been quite a bit bigger. At this point she'd had a catheter in for almost twelve hours, which relieved a lot of the backup. Sadie was 4 lb. 15 oz. at birth and 17 inches long. It would be another week before I was allowed to hold her, but she was roughly the length from my elbow to my wrist. I didn't realize how tiny she was when I looked at her. It wasn't until I saw pictures of myself holding her that I realized how small and fragile she really was.

Shortly after her delivery the medical team at Riley Hospital for Children was able to diagnose Sadie with persistent cloaca. A cloaca is a common channel for the urinary, reproductive, and lower digestive system. All baby girls have it, but during their development in utero the common channel should divide into three separate openings. For an unknown reason Sadie's development stopped and this did not happen, leaving her with a cloaca. I've been an RN for eleven years, and this is something I'd never heard of. (No one at Riley knew what I did for a living, but I'll save that for another post!) As we later learned, this birth defect is seen in about 1 of every 20,000 live female births. Persistent cloaca is also associated with defects of the spine and heart. Sadie's heart was normal but we later learned that she had a small syrinx (fluid-filled area) at her 12th thoracic vertabrae.

Her diagnosis hit us so fast that we didn't have time to process what was happening. In order to allow her bowels and bladder to function, she had to have openings created surgically for her bowel and bladder. The morning after her birth she went to surgery for a colostomy and vesicostomy. Over several days her renal function slowly improved and she was able to take breastmilk through a feeding tube. She was off the ventilator in 5 days and only needed oxygen for a couple of days after that. She spent a total of 17 days in the NICU and another 4 days in the infant unit. After 21 days we were able to bring her home. And that's when the real adventure began!

Wednesday, May 12, 2010

Sadie's Rainbow

Thank you for visiting our blog! This site is intended to keep everyone up-to-date on our family, and to help other families facing similar issues to our own. But first, I'd like to explain why we chose this blog name.

I was 30 weeks pregnant with our youngest daughter, Sadie, when an ultrasound showed what our doctor believed to be a cyst in our baby's abdomen. We were referred to Maternal Fetal Medicine at Community Hospital North in Indianapolis. On December 9, 2009 our physician, Dr. D, gave us devastating news. Sadie's bladder was not draining, and the backup of urine had damaged our kidneys. She had almost no amniotic fluid, she appeared to have a clubbed foot, and she never opened her fists. These were all markers for a chromosomal abnormality. Dr. D's main concern was Trisomy 18, and she recommended doing an amniocentesis.

My husband and I were reeling from the news. After the amnio we sat with our pastor in our room on the 5th floor at Community. The weather was especially foreboding that day. The gray skies spat rain and snow off and on, and the wind was howling. While we tried to come to grips with what had just happened, someone else on the 5th floor was looking outside. A pharmacist named Rob Martin caught a glimpse of a rainbow and photographed it. We didn't see it that day, but the following day Chris saw the image on the Indianapolis Star website. Even though we had no idea what the results of the amnio would be, it was if God was sending us His promise that He was with us, even when we couldn't see Him. The image of that rainbow gave us hope despite our uncertainty.
We would learn three days later that our amnio was normal. However, our baby was still in danger. I was taken to Indiana University Hospital on Dec. 14 and 4 days later our daughter was born. She was 8 weeks premature and had surgery 24 hours after her birth.
I'll be posting frequently to get everyone caught up to where we are today. So check back often!