When parents first learn their child has a birth defect, they are often overwhelmed and unsure about the right course of action to treat their child, until they have had time to process the information and do research to learn about the child’s condition.
Parents of children born with Pierre Robin Sequence (PRS) often find out about the condition after a child is born. This gives them minimal time to learn about the anomaly and limited time to research and determine the right course of action to treat their child. When PRS is severe, meaning the child cannot breathe or eat on his own, it is critically important that a decision is made immediately. Time is of the essence.
One option for treating PRS is with jaw distraction. Recent studies have determined that children who have a severe upper airway obstruction have a better set of outcomes if treated with jaw distraction as a newborn.
About Pierre Robin Sequence
The specific characteristics of Pierre Robin Sequence involve micrognathia (small jaw with receding chin), glossoptosis and cleft palate. The causes of the condition are unknown and infants with this condition have both eating and breathing difficulties, the most necessary functions infants need to live.
Often children diagnosed with PRS have another condition present. For more information about associated conditions, such as Sticklers Syndrome, the most common syndrome associated with PRS, go to Pierre Robin Network for additional information.
Treating Pierre Robin Sequence Through Conventional Means
There are conventional means to treat infants with severe upper airway obstruction that have been effective. Infants with conditions such as Pierre Robin Sequence, Stickler's Syndrome, Treacher Collins Syndrome, Nager Syndrome, and other craniofacial deformities often have a tracheostomy in order to breathe and are fed through a stomach tube (g-tube). Depending on the severity of the micrognathia, the child could have the tracheostomy anywhere between 1 and 5 years and some throughout their life.
Certainly there are some drawbacks for having a tracheostomy and a g-tube. Though a children with a tracheostomy can lead a normal life, they do experience many difficulties. A child with a tracheostomy is unable to cry for help and typically communicates via sign language. There is not an option to breast feed, feed with a bottle, or begin to eat regular food later in life.
These certainly are not life threatening issues, but they can be quite difficult to manage on a daily basis and there is often a need for 24-hour care in the first few years. Also, whether the child has the tracheostomy removed in the toddler years or later, he will continue to struggle to communicate verbally most of his life due to the lack of strengthening of the muscles for speech during crucial developmental years.
What is truly of concern is the significant possibility of infection with two “man made” holes in the child’s body.
Another Option- Jaw Distraction
There is an option for some children that provides a better quality of life, free of complications that the other modalities bring. Mandibular distraction osteogenesis or jaw distraction can be a better alternative.
Jaw distraction is a surgical technique that involves a gradual lengthening of the bones in the jaw. The process reduces the airway obstruction by advancing the tongue and opening the airway, allowing an infant to breathe on its own. Once the infant is breathing comfortably, feeding is usually much improved.
Dr. James P. Bradley, the Bernard Sarnat Craniofacial Chair and the Chief of Pediatric Plastic Surgery at the UCLA Division of Plastic Surgery, an expert in this field, has performed this surgery for numerous years. Dr. Bradley and his colleagues conducted a study of neonates with upper airway obstructions during a 10-year period in two different academic centers.
The results determined that infants who underwent jaw distraction had fewer hospital days, fewer emergency department visits, fewer respiratory infections, and less reflux. These infants had improved feeding, growth, and speech. During the study, no deaths occurred for the children who received jaw distraction and 3 deaths occurred for children who were treated with conventional methods.
What Should You Do if Your Child Is Born With PRS?
The first step after your child is born is to request a meeting with a team of doctors in your hospital. Together, determine how severe the upper airway issues are and with the team of doctors determine whether jaw distraction is a viable option. Do some research and locate the most experienced surgeon to perform the surgery.
Also, if your child is older and has already experienced a tracheotomy, jaw distraction is still a viable option. Contact your child's craniofacial team and talk with them about this option. Together, search for an experienced surgeon for this surgery.
Interview each of the possible surgeons and determine how many jaw distractions they have performed on infants, how many have been successful, and how many have failed. If there have been issues, ask why they have failed. Again, time is of the essence. Doctors in the field realize this and will communicate with you as soon as possible.
With research in hand and with advice from the team of doctors, you have the power to make the best decision for your child and give him or her the best quality of life possible.
