One Child's Journey With Pierre Robin

In January of 2014 our family had one more "new beginning" after twenty months of "new beginnings." That little girl there on the right took her first normal breath in almost two years. If you look carefully you can see the hole in her throat where the trach was just moments before. After twenty months, she had her trach removed and could finally do many things that most people take for granted.

For instance, for the first time she could take a bath without having two adults in the house. Prior to that day, bath day meant trach tie change day which requires two trained adults. Additionally, this liberation opened the door to an ocean of folks who could babysit when Mom needs a day off. For the first 20 months of her life only her two grandmas were trained to do all the medical stuff necessary for a baby with a trach.

A trach requires suctioning several times a day and cleaning around it often. January 2014 brought our granddaughter's first days of not being suctioned! Her bedtime routine became immediately simpler and the amount of "stuff" she had to take with her when she traveled dwindled to a mere diaper bag. The suction machine and the oxygen could be left at home (in fact, they were been donated to the hospital to give to another family who may not have insurance). Yes, removing the trach was our little blessing's best "beginning." But let me share with you a few more firsts we had with the birth of our third grandchild . . .

It was a Really Rocky Beginning

That picture on the right was taken just after our granddaughter arrived. Our precious baby came two months early weighing in at only a little over three pounds. Her early arrival was due to a relatively rare condition known as Pierre Robin (pronounced Pee-air Roe-Ban) Sequence.

From the research I've done, I've learned this condition is caused when the collarbone and jaw don't separate probably during the first trimester of pregnancy. (Did you know they started out connected? I certainly didn't!) They discovered her problem during an early sonogram, so the doctors were prepared. She didn't have any liquid in her stomach when they looked at the pictures, an indication that she couldn't swallow properly.

Because the jaw doesn't separate early enough, it gets held back and doesn't leave enough room in the baby's mouth for the tongue. With the tongue in the way, baby can't really swallow properly. Additionally, because the tongue ends up in the back of the mouth, most Pierre Robin babies are born with a cleft palette or worse. Our wee one had a very mild case of the "sequence," so her side effects were limited. The condition can be a "syndrome" which can include a full cleft palette and/or lip, facial deformities and missing or extra chromosomes. We were and are very blessed. Let me share with you another of our firsts.

First after First

From the beginning life was rocky for our three and a half pound champion. Before she was two days old she was rushed to Children's Hospital for surgery. Someone had inadvertently intubated wrong, causing a perforation in her belly. The surgeons took care of her quickly, but it was still a scary few days.

Within a week, they decided to take out the ventilation tubes and try to let her breathe on her own, but unfortunately, her little jaw just wasn't big enough. Her tongue just couldn't get out of the way. So they put the tubes back down her nose to help her breathe and give her nourishment.

During those early months there were more "firsts" than we can possibly count. But the "first" that we remember the most was the day that her mommy and daddy finally got to hold her for the FIRST time. It seemed like forever, but it was only 2 weeks. Because of her severe condition, no one got to hold her when she was born. We were able to reach into the "pod" (her crib type incubator), to touch her and talk to her. But let's face it, there's nothing like holding a newborn baby!

Finally, after almost two full weeks, a nurse noticed that mom and dad hadn't had an opportunity to hold her and took her out of her warmed private room long enough for parents and firstborn to begin the bonding process. Those pictures were some of my favorite for a good while!

First Baby of the House

Within a month or so this beautiful newborn had finally gained enough weight to undergo the procedure to have her trach put it in. It was nice to be able to see her without the tubes in her nose. However, even with the trach in, she had a long road before she was even going to be able to come home. Her next few months included the addition of a g-tube and being allowed to wear clothes! But most importantly, after getting down to about two and a half pounds she spent most of her time growing.

Finally, after more than three months in NICU, my youngest daughter's first daughter got to meet her puppy (who would later become her best friend). She still seemed so fragile. Completely vent dependent, I was often fearful that I'd take too long getting the suctioning done or not turn the oxygen on to the right level. However, she was really quite tough and withstood all of my faux pax and more!

Like most newborns, our infant slept most of her days and because of her g-tube, she didn't even really have to wake up to eat! In fact for those first few months she had to be connected to the g-tube 24/7. Add to that the Pulsox cord and the vent tubing and you can imagine that getting her out of bed was a challenge all by itself. Those first few months at home went by very quickly. I never thought I'd ever be a nurse in any small sense of the term, but being at her house two or three days every week being one of her two primary caregivers (outside of her parents) was truly an experience.

Detractors Move her Jaw 17mm

She was six months old when she had her second hospital stay. In order to make room for her tongue, the surgeons had to split her jaws and put metal pieces in that would stretch, "detract," her jaw. They protruded behind her ear about 1/2 inch, and every day for a week or more, her father would turn them one full turn in the morning and another full turn in the evening taking her jaw out an extra 1mm every day. The detractors are pictured there on the left. They stayed in her jaw for about five months giving her jaw time to heal around them. And another surgery when she was almost a year old extracted the detractors.

That photo shows the detractors, but they are actually much smaller than they appear in the photo. They might be 20-25 mm long each at best.

Vent Free

By her first birthday, our beautiful brunette was vent free for a few hours every day. Increasing the time by 1/2 hour every day, she had worked up to be able off the vent for her entire first party. It was so nice to be able to pass her around from person to person without that leash! She was a little slow at moving around being attached to that machine. In fact rolling over was a bit of a challenge because one way or the other, the roll always involved rolling over the vent tube. But by her first birthday she'd been able to learn how to crawl and she was having a blast. Knowing that she didn't really need the vent, she also began to pull it off by herself! We'd hear the alarms go off and run in to check on her. There she would be, sitting and smiling just waiting on us to hook her back up. Her life was full of firsts, but even after her first birthday, the less than normal firsts weren't done yet.

But my favorite may have been her first laugh

Crying and laughing are things that most parents simply take for granted. I hope I never will, ever again. It was Mother's Day (just before her first birthday) when I heard her first cry and just a month after her first birthday when we heard her first laugh. It's hard to imagine a baby that can't cry or laugh, but a trach requires a lot of air rushing past it in order for a baby to make noise.

Finally after a year she had the strength to pass enough air to begin to make sounds. She began to say "mom" shortly after her crying could be heard, but we discovered that without her palette most words would be very difficult. Consonants are relatively impossible when you don't have a full palette, so even though she was over a year, we were still going to have to wait to hear her first real words. Fortunately, she had picked up sign language quickly and communicated well! Our oldest grandson learned sign language too, even though he had no physical handicap. I highly recommend it for every child.

But, I digress . . . are you ready for the last of the firsts . . . .?

Photo by Monica Lynne Photography

Here's the video I took with my phone when she began laughing that very first time!

Fixing the Palette

We are so blessed! Only the soft palette (the part you find furthest back in your mouth) was missing, so there was no apparent evidence that she had any problems at all. By this time our little beauty was completely vent free, but they'd left the trach in to make the healing process of the palette go faster. With the palette and the g-tube, she was able to get nourishment as well as air without anything touching the surgeon's work. The first thing I noticed after the palette surgery (besides the splint type wraps on her arms so she couldn't stick her fingers in her mouth) was her ability to "click clack" with her tongue on the roof of her mouth. And within days she was starting to make a "b" and a "d" sound. She'd been wanting to talk for so long and with the palette restored, she could finally begin to make those beautiful, melodious sounds we'd been waiting for so long to hear.

After the palette surgery, it was only a matter of weeks before the trach came out. Then finally in June 2014, 25 months after the journey began, the g-tube mickey button was removed. We are at the end of all of these unusual firsts now. Those first two years were a real learning experience. We had a lot of support, people who have prayed and helped as we walked this long road. We are truly grateful for each and every one, for all the love and for Jesus Christ who brought us through.

Below I'll share a photo of our little beauty today . . . Now we're doing the regular firsts . . . first day of pre-school, first day of Kindergarten . . . first time to swim without water wings . . . yes, we're blessed.

If you have more questions about Pierre Robin Sequence or would like to leave a comment, please do! I'd love to hear from you!