On April 19, 2006 my husband and I went in for our routine 20 week ultrasound.  Long story short and many tears later, we were told our daughter would be born with an Omphalocele, basically a hernia at the umbilical cord.  A few days later we had an amniocentesis performed.  Ten days later, I received the call that Grace had no detectable genetic abnormalities.

Fast-forward to September 6, 2006.  The day our precious daughter was to be delivered via scheduled c-section due to the predicted size of the Omphalocele.  They assumed it would be large to giant in size, so at least the size of a large grapefruit.  Grace was born at around 10:40 a.m. with no visible complications other than the Omphalocele.  She was

rushed to the NICU where they placed a tube down her throat to suction out anything she had in her stomach.  She weighed in at 8 lbs 4 oz and was 20 1/8” long.  She was a BIG baby for the NICU.

Her surgeon came to visit that evening and take a look at her abdomen.  Upon examination, he realized she had a considerable amount of excess skin surrounding the Omphalocele.  He came and discussed with us the awesome news that he would be able to close her abdomen the following day.  It appeared to him that the Omphalocele only contained part of her liver and maybe her gal bladder or appendix and with the addition of some Alloderm to allow him to close her muscles, he believed he would be able to fit everything back inside.

The next day, prior to her surgery she was x-rayed from head to toe, as well as numerous other tests, so that the surgical team could know as much about Grace’s anatomy as they could before going in.

Following the surgery, her surgeon returned to my room to let us know that the surgery was a success.  While there, he asked if anyone had told us that Grace had an extra vertebra and a few extra ribs.  We were very surprised as no one had told us that.  He said it was probably nothing, that a lot of adults walking around have extra of both and never even know it.  However, once we were released from the hospital, we would have a referral to the pediatric orthopedic clinic for follow-up.

Grace made an awesome recovery and surprised everyone when she was discharged at only 9 days old.  It was estimated that she’d be in the hospital for 3-4 months.

At 6 weeks old, we went to our first orthopedic appointment.  Grace’s spine was fully x-rayed.  When the orthopedist came in, he showed us the x-ray.  It was pretty obvious that Grace had something wrong with her back.  He pointed out the very small partial vertebra in the thoracic area of her spine as well as an extra full vertebra in her lumbar region.  He could find no evidence of extra ribs.  The curve in Grace’s spine at the point of the hemi-vertebra was measured at approximately 23 degrees.  He explained that worse case scenario the hemi would grow and Grace would have to have a fusion surgery at around 5 years of age.  Best case scenario, the hemi wouldn’t grow.  We were officially in the “wait and see” phase.  He sent us on our way with an order to return for a new set of x-rays in 3-4 months.

This went on for about the next 18 months.  We noticed Grace was having a bit of an issue climbing up on chairs.  She would mainly pull herself up using her arms and once she was mostly up, she would then pull her legs up behind her.  She didn’t really use her legs in climbing.  Her doctor was under the impression that it may just be “her” way of doing things, but decided to have her get an MRI to rule-out any other spine/spinal cord problems.  At this point, Grace’s curve measured just above 40 degrees and no other abnormalities were noted about her spine.

Over the course of 18 months, every time we were seen by the orthopedist, he would tell us that her curve hadn’t grown.  His rule of thumb was that if a number measured within 5 degrees (give or take) of the last number, there was no change.  So every time he measured, it was always within 5 of the previous visit, therefore, by his rules, the curve was the “same” when in actuality, her curve had almost doubled in size.

At this point I also began to notice that her lower spine was “sticking out”.  When I pointed it out to her orthopedist, he said it was just muscle building up.  He also continued with his stand that he would do nothing but fuse her where she was at, when the time was right.

I didn’t like that answer.  I didn’t like that my daughter would have to live her entire life with a crooked spine, pain, potential respiratory issues, repeated surgeries and a deformity.   At the last view of her x-rays, I could now see her spine was becoming a backwards “S”.  Before the area of her spine above the hemivertebrae was straight and the lower angled out from hemi and was also straight.  I searched many days on-line to find answers and came across a few scoliosis boards.  It was on one of these boards that another mom recommended I get a second opinion.

A few weeks later I was discouraged when all I could find through my insurance was another doctor, who happened to be in the same clinic as our original doctor.  I didn’t like the idea of basically going to the same place for another opinion and I felt they would be very biased towards the plan the other doctor had laid out.

I went back to the support board, I was beyond upset.  We didn’t have the money to go outside of our insurance provider to be seen for another opinion.  Again, another mom suggested that I apply to the Shriner’s Hospital Network for a second opinion.  I had a Shriner’s right in my own city!  This I could do!   I applied.  It took 3 weeks to get the approval that they would see Grace and another 4 months to get in the door for our initial visit.

On July 31, 2008, we were seen for the first time at Shriner’s.  It had only been about 6 weeks since she had been seen by her previous doctor and her curves were now above 50 degrees.  They had grown almost 10 degrees.  I nearly jumped for joy when our new doctor said he could “fix” her spine.  However, Grace was still too small.  So, to buy time she was fitted for a Providence Night-time Brace.  She wore this brace from August 2008 until around January 2009 when she grew out of it.  At this initial appointment, we were put on the surgical docket for approximately one year out (this was the typical waitlist time for her doctor).

The orthotics department measured Grace for a new night-time brace.  She wore it off and on for about 2 months.  She had never complained about the first brace until it was too small.  However, from the first night, she complained and cried and fought us about wearing this one.  I knew something wasn’t right.  We went back in and saw the doctor.  They did many tests with Grace in the brace and finally realized that after about 15 minutes her lower spine would rotate so much that she wasn’t aligned correctly in the brace and therefore was making her rotation worse.  The braces had help hold the curves from progressing, but were basically encouraging her lower spine to twist outwards.  It was so far twisted that in some positions, I could almost grab hold of her spine.

Grace was then fitted for a regular TLSO brace and was required to wear it 20 hours a day.  In July 2009, our doctor said we couldn’t wait any longer, we had to schedule surgery.

On September 2, 2009, Grace was admitted to Shriner’s Hospital for her hemi-vertebra to be removed and vertebra’s T8 and T9 were fused together using hardware.  She was in surgery for 8 long hours.  She endured a collapsed lung, requiring a chest tube, she was placed on a ventilator, a foley catheter and had a blood transfusion as she lost a tremendous amount of blood.  They transferred her to the adjacent children’s hospital’s pediatric intensive care unit.  It was very hard to see her at first.  She was swollen beyond recognition.  She didn’t look like my sweet little girl in any way.  On top of it, she couldn’t talk or move without silently crying.  I would never wish anyone to have to see their baby in that condition.

The PICU nurses administered a medication to help the swelling go down.  By the next morning, she was able to have the ventilator removed and at 24 hours post-op, she was returned to Shriner’s for the remainder of her hospital recovery.

The first two days of her recovery she had a plaster splint we referred to as her tortoise shell, attached to her with a very wide ace-like bandage.  On the second day, she received her brace.  The fourth day was her 3rd birthday and was spoiled by family, friends and of course every shift of staff at the hospital.  This was the first day we finally moved her out of bed.  First on pillows in a wagon, we pulled her to the playroom.  Once she saw all the toys, she was much more willing to consider moving around.  On day five, late in the afternoon, she finally urinated on her own and we were allowed to take her home.

One week post-op, we returned to the clinic.  Grace’s curves were down to around 40 degrees.  Best case scenario had always been that the curves would just go away with surgery.  We discussed the options and the only one that made sense was to begin serial EDF (elongation, derotation, flexion) casting to reduce her curves.

April 2010, she received cast number 4.  Her curves are down below 10 degrees in cast and with T8 and T9 fused at 5 degrees (they were unable to make it zero because it caused mobility problems), this is Grace’s “best case scenario”.

May 25, 2010, Grace moved back to a TLSO brace for the summer.  This was our first opportunity to know her numbers out of cast.  She is still in the mid-20’s, but that is still an improvement from the mid-40’s she was 7 months prior.

We decided to give serial EDF casting a break because Grace is also experiencing some bladder issues.  In August 2010, Grace had a urodynamic test that showed her bladder isn’t emptying completely, is large in size for her age, and it doesn’t contract.  I believe they are referring to it as a “Neurogenic Bladder of Unknown Cause”.  We thought it was possibly due to an undiagnosed tethered cord, but a repeat MRI in September 2010 showed no anomalies other than her scoliosis that would cause her bladder to not work properly.

At this time, Grace is 4 years old.  She has never been able to void on the toilet, nor have a bowel movement on the toilet.  We are awaiting appointments for other opinions of her bladder problem, but as it stands, we will begin catheterizing her in the near future.  This isn’t something we want to do, but in the long-term, hopefully it will avoid other bladder issues.

That is Grace’s story to the present.

Submitted by:
Jane Bigler, Grace’s mom

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