Foot Surgery - Finally!

Some of you may recall that we have been trying to pursue foot surgery for Rebekah for some time. In fact, we were all set to travel back and forth to St. Louis last fall to have her feet casted and corrected for rocker bottom feet/vertical talus. However. Rebekah got a bowel obstruction last September and spent 2 weeks in the hospital. Although it resolved itself, we have been leery to travel so far for orthopedic services since she is likely to have another repeat bowel obstruction at some point.

Rebekah's right foot bends outwards to the right. The toe bones all bend to the right. Her toes on both feet look small because they are all webbed from the first joint. Her feet are more than "flat", they are actually convex from an anomaly related to Trisomy 18 called Rocker Bottom feet. 

Another look at her feet from the bottom. you can see the outward curve of the right foot and the bony protrusion on the inside of that right foot. This is due to her talus bone in the foot appearing vertical and diagonal.

Given Rebekah's genetic diagnosis, we have met many obstacles (doctors and support from others) in seeking to fix Rebekah's feet. In fact, there are many people who believe we would just be subjecting our child to unnecessary and senseless surgery and "pain and suffering" because we cannot "fix" trisomy and "everyone knows" she cannot (and will not ever) walk.

You can see the prominence of the heel bone being pulled up. This is the sign of "rocker bottom feet" and results in her heel sticking way out. Shoes do not fit this girl! You can also see the convex action of the foot where it should be concave into an arch. This foot defect causes her weight to be distributed on the inside front right of her foot during weight bearing - a balancing act at best to maintain a standing position, let alone walk.

A shot of Rebekah's bones from the top of her foot. you can see how her right toe bones all curve outwards to the right. This anomaly will NOT be corrected with her surgery as this is bone work. If and when we address this, Rebekah will be much older.

Well, Rebekah didn't read those textbooks and we claim victory over the effects of trisomy in her life. We believe she WILL walk one day, but her feet need to be fixed in order to help her reach this goal. And, while surgery of this nature would be "elective" in that it is not necessary for her health, her foot anomalies create feet that cannot be properly balanced on for walking. Anyone WITHOUT TRISOMY would not be able to walk independently on them. So we choose HOPE and FAITH and will give her every chance to overcome the effects of Trisomy on her body.

Rebekah bright and early at Shriner's waiting for check in.

On Tuesday, May 14, at 6am, Rebekah checked into the Shriner's Children's Hospital in Greenville, SC for surgery on both of her feet. Rebekah was born with "rocker bottom" feet and has some bone anomalies caused by tight tendons and mis-angled bone. This is how the procedure was described: The surgery consists of releasing the heel cord tendon to drop the back of the foot bone down, allowing the foot to be more flat. He right foot will also have the outside tendon released so that it will not pull the foot outwards. A pin will be placed through soft tissue to try to hold some of the mis-angled bones into better alignment. Both feet will be casted up to her thigh for 6 weeks. Then the casts will be removed, the pin taken out, and her foot molded for AFOs (ankle-foot orthodics). Then lower leg casts will be put on for another 3-4 weeks while the AFOs are being made. This is all soft tissue work and no bone work will be done (directly).

Once the surgery began, the doctor discovered that Rebekah's calf muscles on both legs actually extended all the way down to her feet as well, and they were released in addition to the Achilles tendon. On her right foot, two tendons on the outside had to be released (instead of just one) and two pins were placed into her foot (versus one) - one from the back and one from the front - through soft tissue to help hold the foot in proper position.  Her bones in the front of her foot all curve to the right and, unfortunately, none of this soft tissue surgery will correct that. But hopefully the more neutral position of her foot will correct the growth pattern.
 
The surgery was successful. Rebekah spent the night in the hospital and it took a few days to get her back to eating. I have to admit, watching her those few days I was really concerned that maybe we had made the wrong decision. She vomited and would not tolerate anything until we gave up on narcotic pain meds and went with just motrin or tylenol. As soon as we dropped the narcotics, she perked up and started acting like her old self!

Rebekah's pain response causes her whole body to go into a flushing mode. Look at how red her cheeks and arm is.  It was also on her chest and upper legs. 

A closer look at her vasodilation reaction to pain.

Her white casts were overwrapped post-surgery in the color of our choosing. I went with the bright tie-dye/camo colors to match the bright colors that were in all the stores for girl's clothes. :-)  Sitting on Rebekah's lap is a new Build-a-Bear (well, dog). Shriner's has a room with all kinds of build-a-bears and each child after a surgery is allowed to pick out a bear and outfit/accessories. Ours has a princess crown and wand and silver shoes. She has a princess t-shirt on. There weren't any really cute princess dressed that didn't clash with the pink on the poodle.

The casts add a LOT of weight and we are supposed to maintain her feet in an elevated position where the heels do not touch anything in order to minimize pressure sores. Well, let's just say after about a week in the casts, Rebekah got tired of lying in the same position and unable to move. So three weeks into the 6 week full leg cast, she is easily lifting those heavy casts and flinging them all around. She has figured out how to finagle her body back and forth and move across the floor. She is delighting in brutal leg whacks against her family's extremities, and all of us have at least one black and blue mark to show for it!  This girl has to have abs of steel by now!

Please continue to pray with us that her surgery will make a difference on her balance and potential for walking, for the overall success of the surgery once the casts all come off, and for what the future holds for our baby girl.

Rebekah rockin' her multi-colored tie dye/camo casts as she leans into Daddy for some snuggling time.

A Nineteen Month Reprieve Ends... (PART I)

After going an amazing 19 months without a hospitalization, we finally broke our medical reprieve.  On May 30th, Rebekah started vomiting and was switched to Pedialyte to give her stomach a rest. On Memorial Day, she spent the day either sleeping or vomiting. She always tolerates pedialyte, so this was a different experience. I figured she had a virus. But after over 8 hours with no wet diapers, I called the pediatrician and they told us to head to the ER for IV fluids.

We spent 8 hours in the ER until they decided to admit Rebekah to the Peds Floor at Greenville Memorial Children's Hospital.  They started Zofran to stop her vomiting in the hopes we calm her stomach enough to restart at least Pedialyte. But after running standard blood and urine cultures, it was determined that Rebekah had a pretty significant UTI. We still didn't know the strain, so they put her on IV Rocephin, and we figured in a day or two she would be back to normal.  But it didn't go that way...

Because of the concern of the UTI extending to her kidneys, we did an abdominal ultrasound on 5/31.  It showed no major issues of Rebekah's kidneys or liver. By Wed morning, Rebekah's tummy was becoming visibly distended.  Rebekah continued vomiting despite the zofran, and was starting to vomit some bile here and there. All of this was a mystery since her blood work did not show anything significant (outside the UTI), and her white blood count numbers were actually going down. 



Michael kissing Rebekah during the Upper GI Study



An abdominal x-ray was ordered which showed a blockage in the smaller intestine. Because the doctors weren't sure if it was a complete blockage, they ordered an Upper GI Study to look at how things were digesting. To complicate matters, Rebekah had been on IV fluids since Monday evening, on IV Zofran for vomiting, and continued to vomit at least once every 3-4 hours. Her stomach was becoming more and more distended and the amount of bile backing up into the stomach was becoming significant.

Rebekah's distended tummy on Wed 6/1

For a period of about 12 hours, we attempted to administer multiple doses of barium to Rebekah for the upper GI x-ray series showing the progression of the barium through her tract. She kept vomiting the barium along with larger and larger amounts of bile. What little passed beyond the stomach was going no where. She was becoming more agitated and unconsolable. She absolutely did not want to be touched.

Rebekah's tummy on 6/2. The g-tube button was being pushed by so
much pressure that it was sticking out was past the stomach.

After the last xray at 7am Thu morning (6/2), her stomach was so bloated it was hard and her g-tube button was actually pushed up out of her skin. The pediatric surgeons decided that we were getting to a critical point and could not waste time trying different tests. We signed the paperwork, then I brought up a very sensitive issue with the surgeon.
I said, "I am sure you don't need to be told this, but if Rebekah codes during surgery, or there are any major issues, you are to do every thing the can for her."
Well, his response pleasantly surprised me. "When we cross the door into the OR, we do absolutley everything we can for our patient. Even if they had a DNR, it would stop at the door and be ignored."
(Rebekah does NOT have a DNR (Do Not Resuscitate order), but his point was in the event of an emergency, they treat everyone equally. How refreshing!.

Dropping Rebekah off at the OR.
Michael had to be at home for Isaiah's Kindergarten graduation.
It's tough having to 'choose' between your children. :-(

So Rebekah was taken to 'emergency' surgery Thu morning around 9:30 am. We met with the surgeon around 12:30p for a post op consultation before seeing Rebekah. She did great during surgery and they found and fixed the problem!

Rebekah's tummy when we picked her up post-op. Looks so much better!
It will continue to shrink as she recovers from major abdominal surgery.

Rebekah once again becomes a medical anomaly and defies statistics by having a Meckel Diverticulum present in only 2% of the population.  This extra section and about 3 inches of surrounding intestine were removed along with several adhesions. They threw in an appendix removal for free! LOL.  And to make things even more interesting, her Meckel Diverticulum didn't act 'normally' by bleeding, but inflamed the intestines enough to wrap around it causing a major obstruction. We had MAYBE another day before this would most likely have turned into periotinitis and/or necrotized (dead) intestine and become a major life threatening problem.

Sweet Rebekah looking much better after surgery.
Her face is relaxed and her lips actually have color.

Her GI doctor can't wait to see if this totally changes her digestive system issues! This may have been the culprit behind a lot of her early digestive system problems.

I want to end Part I of this story by sharing the story of Rebekah and her brother, Elijah.  Rebekah has been miserable for this past week. She hasn't smiled at Mommy, Daddy, or even her Nurse Becky. She has been very grumpy for the nurses. But when her big brother showed up Wed night, she cracked a grin, touched his face, and even held his hand. 

While that is a 'nice' story, I am telling it because there is a false assumption that trisomy kids are vegetables that are a burden to take care of and who will never know you. This is so far from the truth! Every trisomy 13 & 18 child I've met has a happy demeanor, a loving soul, and most definitely a recognition of their family. They are a special blessing, and sent to teach us about courage, hope, and unconditional love. I thank God so much for entrusting Rebekah to our family. It is where she belongs...