This page is meant to be a resource to moms and dads who have a child or suspect they have a child with Pierre Robin Sequence and/or Stickler Syndrome. However, no two journeys are alike, and I am NOT a medical doctor – I’m just a mom. A mom with a daughter named Mercy.
{Baby Mercy at 5 months old – prior to Stickler Syndrome diagnosis}
When Mercy was born, she did not breathe. It became readily apparent she had a cleft palate (not lip – these are two separate diagnoses) and her tongue was obstructing her airway. Intubation was the only way she was going to survive. At that time, her recessed chin was not readily apparent. My very first post on this blog about her birth came 10 days after she was born and I had some time to process all that had happened. You can read that post HERE.
{My husband sent me this photo from when Mercy first arrived at the Children’s Hospital. I was still a state away recovering from a c-section.}
Pierre Robin Sequence is a sequence of events (that has nothing to do with anything mom did or did not do while pregnant) that happens in utero early in the 1st trimester that causes a cleft palate and recessed chin (called micrognathia). The two combined make it difficult for the baby to breathe because the mouth is small and the tongue easily falls back into the airway. There are several ways to remedy this situation, but the current “gold-standard” of care is something called a jaw distraction.
These are the distractors that were placed in Mercy’s jaw at 5 days old. They are shown here completely extended. When they went into her jaw, they were much smaller. Essentially, a plastic surgeon breaks the baby’s jaw, places the distractors, and leaves the ends extending from the baby’s skull (Mercy’s were behind her ears – you cannot even see where they were now) so that a screwdriver of sorts can be inserted twice a day to slowly turn the pins and extend the baby’s jaw. You can read more about the surgery HERE.
Within 6 days, Mercy’s jaw was far enough extended for her to be extubated.
At 11 days old, I held her for the first time!
They continued to turn her pins for a total of 3 weeks as we worked HARD to teach her to feed from a bottle with a special valve for cleft palate babies. The bottles are from Dr. Brown’s and the valve allows milk to easily flow for a baby who has very little suction.
To learn more about these special Dr. Brown’s bottles click here:
A side note about these bottles – THEY LEAK! As in SERIOUSLY LEAK! As in BUY STOCK IN RECEIVING BLANKETS! But, I’m so thankful they exist! There are other kinds of bottles like the Haberman:
but, they do take more manipulation on the parent’s part.
Note: We did have to watch the nurses closely to make sure they put the special valve in (the blue disks you see in the photo above), and there were a few times we didn’t catch it and it set us back in feeding her and getting her home.
Another note on the Dr. Brown’s bottles – when Mercy was about 6 months old, I did some research and learned about the Y-cut nipples from Dr. Brown’s that DO NOT LEAK and work with the special valve! They are rated for 9 months +, but Mercy handled them just fine, so that might be something to look into when baby is a little older.
We were released from NICU when Mercy was 6 weeks old. Her plastic surgeon surgically covered her pins prior to us being dismissed. At home, we kept her on an Owlet monitor for heart rate and oxygen levels. You can read my review of it HERE. Very few PRS families are sent home with any sort of monitor, so this might be a good option if you are needing a little extra comfort.
At 3 months old, Mercy had her distractors removed. She was in the hospital one night and was super swollen! However, the surgery did not set her back eating or sleeping!
I will admit, from the moment she was born, I felt like something was different about her. Even without knowing the term Stickler Syndrome. I didn’t know what I was seeing in her, but I could definitely tell she was not like my other 9 children. You can find the story of all that led up to her diagnosis HERE. It was a very emotional journey, but one I had to take for my own peace of mind.
When Mercy was 6 months old, she got glasses.
Her myopia (near-sightedness) was diagnosed at -14.5. For those of you who have no idea what that means, it basically means, she can’t see diddly without her glasses. HOWEVER, she has surprised us over and over again by what she can see without them.
If you are wondering how a baby is diagnosed with myopia, you are not alone! First, they dilated Mercy’s eyes, and then with her resting in Daddy’s lap, they dimmed the lights and used a bright light and lenses to bounce light off her retinas and refract that light until they found the lens that refracted the light properly.
You can find the update about her glasses HERE. You can also watch her 7 month video below:
The end of May, right after Mercy turned 7 months old, she went in for her first retina check.
Anyone with high myopia, and especially those with Stickler Syndrome, are at risk for retinal detachments. A baby cannot tell you she sees “floaters,” so the surgeon must go in under anesthesia and check her eyes and repair any places that are weak right then and there with a laser. Praise the Lord, her eyes were doing great, and no laser was needed! She will continue to have this done every 6 months or so until she is old enough to be checked in the eye doctor’s office without being a squirmy-worm!
We also found out about this same time that the ENT does not believe she will need ear tubes at her palate repair (something most PRS babies do need because of recurrent ear infections). Mercy has not had an ear infection as yet, so we are very thankful for this! The ENT also said her ear anatomy looks perfectly normal, so praise the Lord for that as well!
In September of 2017, Mercy had another sleep study to be sure she was ready for her cleft palate surgery (closing that opening means less of an airway). Her original sleep study showed 11 apnea episodes an hour. The September study showed her down to just 1! So, in November of 2017, when Mercy was 13 months old, she had her cleft palate repair. You can read all about it HERE or watch the video below:
Some resources I highly recommend for post-op are:
No-No’s – the arm restraints that keep baby from sticking her fingers in her mouth:
Baby Leg Warmers – for keeping baby’s legs warm under the hospital gown and for helping hold the restraints on. You can get 5 pair for free (just pay shipping) at BabyLeggings.com if you use my code: RAISINGARROWS9
A 360° or low-profile sippy cup – only if your surgeon approves it! Baby will not want it for a while because even that small amount of sucking hurts.
Phone Holder – for binge watching Netflix! Obviously, not a necessity, but sure made the hospital stay more bearable for mama!
Laser Surgery for Preventing Retinal Detachment #1
When Mercy was 15 months old, she had an impromptu eye surgery to prevent her retinas from detaching. She had 2100 burns placed in one eye and 2600 in the other. After the surgery, one of her eyes was a bit swollen for a couple of days, but other than that, she acted as if nothing had happened! You can watch the video below that I did on her surgery, as well as see her Miraflex glasses with the built up nasal bridge. Getting the built-in bridge is crucial to keeping their glasses from fogging!
I will add to this page and to her story as she grows and I grow along with her. It’s not an easy journey, but there have been some resources along the way that have made me feel less alone. Here are a few of those resources:
Write Like No One’s Watching blog – the first upbeat blog I found by someone who has SS
PRS Facebook Group (really helps to get your questions answered – lots of SS mamas there too!)
Ava June & Mommy Too YouTube Channel – I actually “met” this mama on Instagram under the hashtag #pierrerobinsequence She shares a lot about her daughter’s daily life with PRS.
PLEASE REMEMBER – PRS and SS vary greatly from patient to patient! Don’t despair by reading worst case scenarios! Keep Your Chin Up, mama!
{Mercy and mama at 3 months}
{Mercy and mama at 5 months}