Pierre Robin Sequence
Jill Landis
Cincinnati OH USA
From: LEAVEN, Vol. 37 No. 5, October-November 2001, pp. 111-112.
It is eight o'clock in the
morning and you have just served breakfast to your children when the
phone rings. Answering the phone, you hear a tearful mother on the other
end: "My baby has just been diagnosed with Pierre Robin Sequence. Can
you help me? Will I still be able to breastfeed?"
As La Leche League Leaders
accustomed to helping mostly healthy mothers and babies work through
common breastfeeding difficulties, this question might leave many of
us speechless and scrambling for information. Although encountered rarely,
Pierre Robin Sequence has its own implications for breastfeeding and
infant care, making it important for Leaders to have a basic understanding
of the condition.
What is Pierre Robin Sequence?
Pierre Robin Sequence, first
identified in 1923, is the congenital combination of an abnormally small
lower jaw (micrognathia), cleft palate, and the posterior (or downward)
displacement of the tongue (glossoptosis). In isolation, Pierre Robin
Sequence (PRS) is not hereditary, however, since it occurs more than
80 percent of the time with at least one other genetic syndrome, it
is considered a genetic disorder. The severity of PRS varies considerably,
from some infants experiencing only feeding problems to others experiencing
serious difficulties with feeding, reflux, breathing, speech and hearing,
as well as complications from other syndromes (Pierre Robin Network
2001).
Feeding the baby with PRS
Nearly every baby with PRS
will experience difficulty with feeding and weight gain. Because of
his very small jaw, it is difficult for the baby with PRS to form a
seal with his mouth at the breast. If this were the only problem, his
mother would most likely be able to work around it by using different
nursing positions. Unfortunately, the baby with PRS also has a cleft
palate, which makes it difficult to create the negative pressure required
to draw the nipple back into the mouth and suck (Lawrence 1999). The
posterior displacement of the tongue can cause breathing problems, both
during eating and sleeping. When the baby's body is in certain positions,
his tongue can obstruct his airway (Pierre Robin Network 2001). Together,
all of these problems make breastfeeding, and conventional bottle-feeding,
nearly impossible. Eventually, through surgery of the cleft palate and
natural growth of the chin, the majority of babies with PRS will grow
into healthy children without the feeding issues that affected them
as infants. However, because PRS involves more than a straightforward
cleft palate, feeding at the breast is unlikely even as the baby gets
older.
Weight gain is a problem
for nearly all infants with PRS. Precious calories are lost during feeding
sessions when the baby struggles to move milk to the back of his mouth,
swallow it, and regulate his breathing. Unassisted by special technology,
it can take over an hour to ingest only a couple of ounces. Parents
and health care professionals commonly use one or more special feeding
methods to maximize the caloric intake of babies with PRS. For some
infants, the Mead Johnson Cleft Palate Nurser works well because it
allows the parent to gently apply pressure in coordination with the
baby's sucking and swallowing. However, this bottle only allows for
limited squeezing pressure and the infant still must struggle to suck
against the vacuum of air in the bottle. The Haberman Feeder, manufactured
by Medela, has a flexible, elongated nipple that allows the parent to
squeeze milk into the baby's mouth in coordination with his sucking
and swallowing. The nipple is separated from the bottle by a valve that
acts to keep the nipple full, thus eliminating the vacuum of air and
rewarding even a very weak suck. Many babies with PRS respond well to
the Haberman Feeder; however, some of the more severe cases require
aggressive intervention. A nasogastric tube (also called an NG-tube)
is a temporary feeding tube that is placed through the infant's nose,
down the esophagus, and into the stomach. This method allows gravity
to assist in feeding. An NG-tube can be used for up to 30 days. A gastric
tube (also called a G-tube) is surgically inserted into the stomach
and allows parents to give their babies any milk that wasn't taken orally
(Pierre Robin Network 2001).
Other Problems Associated with Pierre Robin Sequence
Some babies with PRS will
initially have feeding difficulties and move on quickly to a healthy,
typical childhood. Others will experience a variety of problems, depending
on the severity of PRS and the existence of other genetic syndromes.
Frequent ear infections are common in young children with PRS. This
can lead to surgery to insert tubes into the ear canals and if infections
are not treated or prevented, to hearing loss and speech delays. Other
problems with speech articulation can also occur. Reflux is common as
well, often showing up at two to three months of age. Children with
cleft palates are at risk of dental problems, and children with PRS
may have crowded lower teeth as a result of the small jaw (Pierre Robin
Network, 2001).
More than 80 percent of people
with PRS have at least one of over 20 genetic syndromes. The two most
common are Stickler's Syndrome and Velo-cardio- facial syndrome. Twenty-five
to 30 percent of people with PRS have Stickler's Syndrome, a disorder
that can affect the joints, eyes, palate, heart, and hearing. They may
suffer from retinal degeneration, sensor neural hearing loss, prolapse
of the heart's mitral valve, joint pain, and cleft palate. Velo- cardio-facial
syndrome is the syndrome most commonly associated with congenital heart
disease and cleft palate. There are over 180 other abnormalities associated
with Velo-cardio-facial syndrome (Pierre Robin Network 2001).
How Leaders Can Help
Typical breastfeeding may
be impossible for babies with PRS, but that does not mean that they
cannot enjoy the benefits of human milk, especially when the condition
itself presents specific health risks. The presence of a cleft palate
increases the risk of recurrent ear infections in infants. Research
has repeatedly shown an association between fewer ear infections and
human milk (Duncan 1993 and Paradise 1994).
For the baby with a compromised
respiratory system, exclusive human-milk- feeding offers significant
protection against infection. Typically, babies with PRS will spend
more time in the hospital for treatment and observation, all the while
exposed to various infections against which human milk can provide important
passive immunity. All of these reasons, and many more, make human-milk-feeding
the superior option for babies who are physiologically unable to breastfeed.
(THE WOMANLY ART OF BREASTFEEDING 1997).
Leaders can be an important
source of information and support to mothers who wish to human-milk-feed.
They can provide accurate information about pumping, storing and using
human milk, and avoiding breast infections. Leaders can also help mothers
problem-solve ways to incorporate full-time pumping into the already
stressful routine of caring for a special-needs baby. Mothers of-babies
with PRS may need support and encouragement from Leaders for their decision
to human- milk-feed in light of criticism from family, friends, and
health care professionals. The Leader can serve as a listening ear for
the grief that comes with the loss of breastfeeding (Landis 2001).
While the baby with PRS might
not be able to nurse at his mother's breast, he can still find comfort
there. Leaders can encourage the mother to develop a nursing relationship,
which may or may not include the baby at the breast (Good Mojab 1999/2000).
Baby-wearing, cosleeping, and skin-to-skin contact are opportunities
for all mothers and babies to bond and enjoy each other. It is important
to remember that one of our most valuable roles as Leaders is to help
and support each mother as she establishes her own unique and loving
relationship with her baby.
References:
Duncan, B. et al. Exclusive
breast-feeding for at least 4 months protects against otitis media.
Pediatrics 1993; 91(5):857-872.
Good Mojab, C. Congenital
disorders: Implications for breastfeeding. LEAVEN Dec 1999/Jan 2000;
123-28.
Landis, J. Supporting the human-milk-feeding mother. LEAVEN Feb/Mar 2001; 3-6.
Lawrence, R. and Lawrence,
R. Breastfeeding: A Guide For the Medical Profession, 5th Edition.
St. Louis, Missouri: Mosby, Inc., 1999; 489-91.
Mohrbacher, N. and Stock,
J. THE BREASTFEEDING ANSWER BOOK, Revised Edition. Schaumburg, IL:
LLLI, 1997; 296-303.
Paradise, J. et al. Evidence
in infants with cleft palate that breast milk protects against otitis
media. Pediatrics 1994; 94(6):853-860.
THE WOMANLY ART OF BREASTFEEDING, 6th Ed. Schaumburg, IL: LLLI, 1997; 345, 349.
Web site: Pierre Robin Network:
www.pierrerobin.org
Author's Note: I would
like to thank everyone who contacted me after the publication of my
article about human-milk-feeding in the February-March 2001 issue of
LEAVEN. I was especially heartened by the stories of Leaders and other
mothers who have experienced human-milk-feeding firsthand. It came to
my attention that when I described conditions that might prevent breastfeeding,
the statement, "Babies with bilateral cleft palates, babies with Pierre
Robin Sequence, and babies with severe neurological impairments" was
too exclusive. I want to clarify that there are many reasons why a baby
may be unable to nurse, temporarily or permanently, and that each case
is highly individual. I in no way intended to establish criteria for
"acceptable" human- milk-feeding situations.
Jill Landis is a Leader
in Cincinnati, Ohio, USA with the Blue Ash-Kenwood Group. She has two
children: Emma (5) and Avery (2). Avery was born with Pierre Robin Sequence
and received his mother's milk via a Haberman Feeder for 23 months.
Last updated Friday, October 13, 2006 by njb.
Page last edited Sun Oct 14 09:31:08 UTC 2007.
