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Sofia – Born 19 June 2010

Hi my name is Andrea, I’m Sofia’s mom I guess all PRS stories are different but we all share a common feeling of not know at the beginning what our children have since PRS is not very common, that’s why I would like to tell you Sofia’s story. Sofia was born June 19,2010 she is my second daughter and imagine our surprise when we found out that she had PRS and cleft palate, my pregnancy was very normal, didn’t have any issues but during my 6 month I had to go for that months ultra sound and during that visit I had a small scare because the lady that did the ultra sound could not see her jaw and neck developed, after a very long time trying to see if that was true they finally told me that everything was fine, that she just had her little hand on her chin and that’s why they couldn’t see it. After that visit I was still a little worried and had 2 more ultra sounds during the remaining months of my pregnancy and they all were the same she had her had on her chin.

When Sofia was born she was breathing very little, she was taken immediately to NICU and was coughing up blood, the doctors had to make a small incision on her side to her right lung to take out the liquid she had in her lung (I’m not sure what’s the name of that procedure), my husband was with her all the time while I was being taken care off. After she was stable I was able to go see her and was very sad to se my little girl like that and very scared. We didn’t know what was wrong with her and we were preparing for the worst. that same day she was transferred to Joe Dimaggio Children’s Hospital in Hollywood FL, she went directly to NICU where she was taken care off by all the great nurses and doctors there, my husband went with her while I stayed in the hospital where she was born. The next day I went directly to see her and she was with all kinds of gadgets, machinery was beeping everywhere and was a very scary and stressful day. finally a pediatrician on duty that day told us what was happening and that she had Pierre Robin Sequence that is not very common and started explaining what it is, thankfully my mother was with us, she came from Charleston SC, she works in the University of South Carolina and she has access to medical encyclopedias and other medical references including a doctor that knew what PRS is and the procedures that are available.

Sofia during the First few days since her birth had an NG tube and that’s how we fed her, she was almost always placed on her belly since her tongue always obstructed her breathing, and was connected to all kinds of machinery. after a week and a half of being in the NICU we where introduced to the surgeon that was going to do her jaw distraction, at first my husband and I did not know what decision to make if it was worth putting our little Sofia through such a big surgery and at such a young age and also putting her through all that pain. we searched the Internet for all kinds of information about this surgery and the pros and cons of doing it and also not doing it, finally we had a great support group and a friend of my husband told us something that will always stay with us, he said “yes it will be hard for you and your family to see her in all that pain but remember that at the end of all of this she will be healthier and her life and yours will improve”. So after much deliberation we decided to go ahead with the surgery.

In June 28 2010 Sofia had her Mandibular Distraction and G-tube it was a 4 hour long surgery we waited a while to see her, but finally when we went to her room and saw her we were relieved that everything came out fine and she was fine. My initial shock in seeing her wasn’t that bad I thought I would start crying but I didn’t, just wanted to be strong for her, my husband and my older daughter. Sofia was very swollen after her surgery se had the distractors that you could see from the out side. She was on a ventilator and could not move, they had to keep her very still dew to the fact that the surgeon had to break her jaw in 4 places to insert the distractors. During the first week the nurses had to move her distractors (I don’t remember very well how much they had to move them). And she had to keep very still during that time, that’s why the surgeon ordered to give her a medicine call vecuronium witch is a muscle relaxant and is also classified as a paralyzing agent. When I first heard what they were injecting her I thought I would loose it. It is a dangerous anesthetic and she was so young to be receiving that. But after several nurses confirming that it was necessary for her to stay still and have a successful distraction I started to calm down. She was on a ventilator for a week and a half, the vecuronium was only administered for a few days. And finally after the first week and half the ventilator was removed and she started breathing on her own and was also starting to receive formula through her G-tube a few days after her surgery. the next weeks we where taught by the nurses how to take care of her wounds and how to feed her through the G-tube and also trying to feed her with the Haberman bottle, it was a whole new learning experience and we where really nervous to take her home. But finally in August 2010 we finally got to take her home!!. It was very exciting and very challenging at the same time we had long nights, we had her on a schedule where my husband and I took turns cleaning her wounds and feeding her 24/7. 3 or 4 months later (don’t recall the exact month) her distractors were removed! We were so excited, we just couldn’t believe that we made it and that our little Sofia was such a trooper and so strong. We thank God for all the strength and patience he gave us especially to Sofia.

In January 2011 Sofia had her palate repair; this surgery wasn’t as long as the first one but she was very cranky when she came out of surgery. We stayed in the hospital for a few days, and then went home. with this surgery we didn’t have to worry about a lot like before just make sure she didn’t put anything in her mouth and feed her a liquid diet for a few days until the surgeon gave a good to go to start feeding her the baby food. I forgot after her distractors were removed her G-tube was removed too, she drank her formula from the Haberman bottles until her palate was repaired and if we tried a little we gave her cereal.

After her palate repair she started going to speech therapy and she’s been going since it has helped her a lot. She has problems with some sounds dew to the fact that she has a very small tongue and can’t move it very well or stick it out.

Her last surgery was her frenulum being cut so she could move her tongue a little better and we believe it worked. She doesn’t stop talking :). Now she is a very smart, funny, beautiful 3 year old and she is doing great she snores a little but nothing to be concern about, she doesn’t have feeding problems. And now the only thing that concerns us are her teeth since she was born with her two bottom teeth and where removed a few days after she was born. We have been to a pediatric dentist and they tell us that we just have to wait for all her baby teeth to come in and fall out and her big girl teeth to come in and see what we can do about them. So we have some time.

We have been very fortunate that Sofia has had great doctors, nurses, and speech therapists that have helped her to live a much better life. We also have to thank God for giving us this experience and making us stronger, patient, and be a support for one another in difficult times. We have not regretted making any decision that has involved Sofia’s well being and I think the best decision we have made is the one for her Mandibular distraction. It is true that her life and ours has been better.