Pierre Robin Syndrome: What is it? How do you deal with it?
Twenty five years ago I gave birth to my daughter who had Pierre Robin Syndrome. Nothing is scarier than to have an ill child. Pierre Robin is not deadly for which I was thankful, but it does require special skills to deal with. My daughter had to be tube fed, she was on an apneic monitor, and she had almost constant ear infections, and suffered several surgeries in the first year of her life. A new baby is a joyful event but also stressful. An ill new baby is a joy tinged with sorrow and the stress is magnified a thousand times. This article will describe Pierre Robin Syndrome and its treatment.
Pierre Robin syndrome occurs in one in every 2000 to 30,000 births depending on the criteria being used to define the disorder. Pierre Robin Syndrome is characterized by a short lower jaw and a large floppy tongue. A cleft palette is present but a cleft lip is not. The upper palette is very high and arched. These characteristics cause the tongue to tend to fall back and block off the airway. In my daughters case she has to have surgery at 7 days to tack her tongue to her lower lip to prevent asphyxiation. In addition, these children often have feeding difficulties. My daughter had an artificial palette formed by a children’s dentist, but could still not feed. In retrospect I believe her severe ear infections caused all sucking to be so painful she simply wouldn’t do it. She required tube feeding every four hours for her first five months of life. The first time she was able to suck a bottle was after having her first set of ear tubes placed. I’d recommend you have this done early as these children will have ear infections and the tubes decrease the pain associated with them.
This is a list of the symptoms of the disorder:
Cleft soft palate
High arched palate
Jaw that is small and receding chin
Large tongue
Teeth already present at birth
Recurrent ear infections
Chocking due to the cleft palate
Breathing problems caused by the large tongue
Because Pierre Robin Syndrome can be associated with other genetic problems your doctor will want your baby to have genetic testing. According to my doctors, if no other genetic abnormalities are found you are no more likely than the average population to have another child with this problem. Some doctors feel the syndrome is caused by the baby’s position in the womb.
Treatment for Pierre Robins Syndrome may include surgery to Tack the tongue to the lip if breathing difficulties are present. Around 9 months the soft palate repair will be done. The shortened lower jaw on these children usually begins to grow out within the first 6 months of life. In those it does not, there are surgical procedures that can be used to help.
The hardest part of dealing with the disorder for me was the recurrent ear infections and feeding problems. My daughter was sick constantly and only weighed 12 pounds at 10 months. She also required an apneic monitor because she asphyxiated in the nursery. The monitor went off an average of 3 times a night the whole 10 months she was on it, usually because a wire was loose. Dealing with three surgeries in less than a year was also very stressful, but you do survive and more importantly so does your baby.
My best advice would be to be your child’s advocate. Doctors generally do want to help but it isn’t a personal thing for them. This condition requires many painful procedures be done to help your baby and they are necessary for ultimate recovery. Even so, you have a right to expect things to be explained to you. Some pain can’t be avoided due to safety for your baby but you have a right to expect as much gentleness and pain relief as is reasonable and safe. Babies do feel pain. Don’t let your doctor tell you they don’t. In fact find another doctor if he tells you that. Don’t alienate a good doctor but don’t be afraid to speak up either. You’re the only advocate your child has.
The good thing about Pierre Robin Syndrome is children recover from it and go on to lead normal lives. So hang in there. It gets better.
<https://www.google.com/health/ref/Pierre+Robin+Syndrome>
<http://www.geometry.net/health_conditions/pierre_robin_syndrome.php>
<http://en.wikipedia.org/wiki/Pierre_Robin_syndrome>
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