Maggie Grace was born with a rare medical condition called Pierre Robin Sequence. Here you can find answers to some of the questions people have about this special condition. Please note that I post updates regarding her medical situation on the Latest News page, so some information here is not up-to-date with Maggie's current condition.
What is Pierre Robin Sequence?
Why is it called a "sequence"?
What kinds of problems can PRS kids have?
How is all of this affecting Maggie?
What about surgery? How will Maggie's jaw and palate be corrected?
Where can I learn more about PRS and cleft palate?
See pictures of Maggie's cleft, the Haberman bottle, and the apnea monitor "belt"
Pierre Robin (pronounced Roban) Sequence, or PRS, is a group of problems that occur together. This includes a small or recessed chin/jaw (called micrognathia or retrognathia), the tongue being placed further back in the mouth than normal (called glossoptosis), and usually a cleft palate. Some people do not consider it to be PRS if there is not a cleft palate, while others do. PRS never includes a cleft lip.
It is called a sequence because the 3 parts occur in a sequence as the baby develops in utero. First, something gets in the way of chin development (There is some controversy about what may cause this. Some say positioning in utero may play a part. Others say that the positioning problem, if it exists, is due to genetic factors which cause the fetus to malposition him/herself.). Because the chin does not grow enough, the space inside the mouth is smaller than it should be, and there is not enough room for the tongue, so it sits higher in the mouth and the palate does not grow together and close as it should. Finally, the crowded tongue tends to go to the back of the mouth because there is no room for it otherwise. These things happen in order, and thus it is called a sequence.
The big problems with PRS are feeding and breathing problems. Because the tongue is farther back in the mouth, it can block the airway partially or completely and make breathing difficult or impossible. In the worst cases, some babies have to have a trachiostomy (trach tube) so that they can breathe. Because breathing is harder for PRS babies, and because it is difficult (or sometimes impossible) to create suction with no palate to press the tongue against, PRS babies can have a hard time coordinating suck-swallow-breathe patterns. The babies may not be able to get much food through the nipple, may have trouble breathing or choke often, or may work so hard to eat that they burn more calories than they are consuming. In the most severe cases, the babies will not be able to eat by mouth at all and will require a feeding tube--either an NG tube (nasogastric, which goes down through the nose, down the throat, to the stomach), an OG tube (oralgastric, which goes through the mouth), or G tube (gastric, which is surgically implanted through the abdomen). The G in all of these abbreviations can also stand for "gavage." PRS also usually occurs with other syndromes (80% of the time), so often PRS babies have other problems that are not directly related to the PRS itself.
Maggie has a pretty mild case of PRS as far as we can tell. She does not require a trach tube and has not had any breathing problems since she came home from the ICU at 13 days old. At first she needed to sleep on her tummy for breathing purposes, but now we keep her on her tummy to control her reflux. She has an apnea monitor (You can see a picture of the apnea monitor "belt" at the bottom of this page.) she wears while sleeping, which would alert us if she ever stopped breathing (she never has, thank goodness!). We have not found any other syndromes in Maggie, though we are having genetic testing done now for two particular syndromes (one of which is Stickler's Syndrome, which you can read about on the PRN site.).
Because Maggie cannot eat as much volume as other babies her age, we supplement her feedings with extra additives so she gets more calories for the volume she's taking in. Even so, it has been a rocky road with her weight gain, and she is in the 10th-25th percentile for her age. Even with all the ups and downs though, she has not had to have a feeding tube and we hope she will not need one.
When feeding Maggie by mouth, we make sure that she does not eat for longer than 30 minutes at a time so that she doesn't become overtired or burn too many calories. She also uses a special kind of bottle, called a Haberman Feeder (You can see a picture of the Haberman at the bottom of this page.). The Haberman works because it has a longer nipple which goes past the cleft. It also has a one-way valve which makes sure she gets less air as she drinks and keeps the nipple from flooding her with too much formula at once (it will not leak out on its own as a traditional nipple would). Most importantly, the Haberman nipple has a reservoir in it which holds the formula, and can be squeezed to deliver the formula into Maggie's mouth. This way she does not have to rely on her weak suck to get enough food.
Unfortunately, Maggie has also had to deal with reflux (also called GERD--gastro-esophageal reflux disease). This is the same disease many adults have that you see the commercials on TV about. Her stomach produces too much stomach acid, and it comes back up her esophagus. This is very painful for her and sometimes can choke her, but is thankfully being controlled with medication. Other than all of this, Maggie is a normal, sweet baby who smiles and enjoys learning about the world around her just like all other babies her age!
Right now the surgeon is monitoring Maggie's jaw growth. Sometimes PRS babies experience growth spurts during the first year and the jaw grows enough on its own that nothing needs to be done to correct it. If not, the jaw would have to be fixed before her palate surgery could take place. Otherwise her mouth would still be too small inside to hold the tongue, and she would have more breathing problems. This type of surgery is called jaw distraction. For now her surgeon does not think she will need this type of surgery, but again, it depends on her growth. The surgeon, the feeding team, and we all think that her jaw is showing some growth so far! The palate repair surgery for PRS kids can occur anytime between 9-18 months or even sooner or later in certain cases. Her surgeon is thinking that she will probably have her palate repair when she is 8 months old, which would be January 2004. Maggie's palate repair is complete! Read more on the Latest News page or see pictures on the Surgery page!
These websites are great resources:
Pierre Robin Network http://pierrerobin.org - Lots of great info about Pierre Robin specifically, including information about other syndromes that may appear in PRS kids, and networking information to get in touch with other families of PRS kids. I HIGHLY recommend the PRN email list for ALL parents of PRS kids. It is a terrific resource no matter how mild or severe a case of PRS may be.
Stickler Involved People - This site addresses the "associated condition" called Stickler Syndrome. There is information about SS here as well as resources for supporting families dealing with SS.
Cleft Palate Foundation http://www.cleftline.org - Here you can order free pamphlets about various cleft palate issues and read personal stories from adults who have clefts or from families of children with clefts.
Wide Smiles http://www.widesmiles.org - This site has lots of information as well as a place to look at pictures shared by various individuals and families. Here you can see pictures of different types of clefts, surgeries, children before and after repairs, children with trach or feeding tubes, and whatever else you may be curious about. To see the pictures, click on Photo Gallery (about halfway down the front page), and you may want to click on "Cross Index" so that you can see the pictures indexed by type of cleft, etc. There is a section for PRS photos specifically that was helpful to me.
