How devastating is it for parents to find out their newly born child has congenital defect? It must be very traumatic, they must feel very distraught. I cannot entirely speak for them but I do partially understand that it is a very painful reality. I will never be able to quantify the pain that they are experiencing at that very moment seeing their child with abnormality. They must be having mixed emotions and their thoughts are running awry. Feeling depressed, mixed with guilt, questions on what did they do wrong to deserve such an abnormal child, why them of all people, among other else.
Cleft lip and palate are one of the most devastating abnormality a child could have. Not only because it is aesthetically displeasing, but it also brings along with it several medical conditions. A child can have recurrent upper respiratory infection, obviously from the defect. In addition, if the cleft palate also affects the opening of the eustachian tube, a child can also have a frequent ear infection and worse deafness.
(image courtesy of http://plasticsurgery.about.com/od/Cleft-Lip-And-Palate/ss/Goals-And-Timing-Of-Cleft-Palate-Repair_3.htm)
Eustachian tube is a communication that clears and drains fluid and debris from the middle ear to the nasopharynx. In a child it is almost horizontal relative to its opening at the nasopharynx, hence the easier access for infection. It is naturally closed at rest, but the muscular opening at the nasopharynx end is disrupted by cleft. This does not allow the muscle opening to open thus fluid accumulates in the middle ear, followed by infection and eventual hearing loss. The incidence of hearing loss is decreased if repair is done early (before 12 months old).
Another problem that cleft palate imposes is feeding difficulty. For one, there is a contiguity of the oral and nasal cavity. This does not allow the baby to create a sufficient suction pressure needed to extract milk from the breast. If baby swallows, he tends to swallow more air than food, and food may regurgitate through the nasal cavity. This is a risk factor for malnutrition.
Speech is another important aspect that must be considered in children with cleft palate. Between two and three months of age, infants will start making sounds. Between six and ten months, babble such as “dadada” and “bababa “will occur. The prelinguistic babbling of infants is ultimately crucial in the development of normal speech. Most children speak their first words (mama, dada) by 13 months of age. True speech — characterized by the ability to put words together to convey meaning and communicate with others – develops by two years of age.
All of these steps on the pathway to intelligible speech require a normal velopharyngeal mechanism. The velopharyngeal mechanism includes the palate and the back of the throat. During speech, the goal is to have air flow out through the mouth instead of out through the nose for most speech sounds. The exceptions are m, n, and ng. In order for air to be directed out of the mouth and not the nose, the soft palate lifts and moves toward the back of the throat. You can see this motion of the palate when you open your mouth and say “ahhh.” This movement closes off the mouth from the nose. If this movement is impaired due to a cleft in the palate, too much air will escape from the nose during speech and the speech becomes hypernasal. Hypernasal speech is speech that sounds overly nasal in quality as if the person is talking through the nose.
In summary, when there is a cleft in the palate, air escapes out from the nose instead of from the mouth when certain sounds are made. Repair of the cleft palate is necessary for the development of normal speech. Speech therapy may still be required after repair of a cleft palate. This is especially true if a cleft palate is closed later in life, after a person has established compensatory habits.
Timing of Repair
The optimal timing for cleft palate repair is controversial. Repair is considered early when it is done before 12 months of age. Early repair is associated with better speech development and less hearing loss. On the other hand early closure of the palate can impair facial growth. Specifically both nasal growth and palatal growth can be restricted with early repair. Delayed repair is associated with better facial growth, but at the cost of better speech and hearing outcomes.
In order to glean the advantages of both early and late repair while minimizing the disadvantages, a two-stage repair has been proposed. A two-stage repair involves closure of the soft palate between three to six months of age and closure of the hard palate between 15 to 18 months. With this approach, the part of the palate necessary for normal speech development is intact, but without the growth restriction of closing the hard palate.
I have a newly born baby who presents with cleft lip and palate. At the same time, the baby is small for gestational age (the weight is below 10th %ile rank for gestational age). At present, my dilemma is how to feed the baby. There is no pediatric orthodontist around whom I could refer the baby for denture. (When I was still a fellow, we had a similar case. We referred this to a pediatric orthodontist who created an artificial palate that we put on the baby every time he feeds. This creates a barrier between oropharynx and nasopharynx thus milk do not regurgitate into the nasal cavity. While it may not be able to create the same suction pressure as a normal child does, it still enables the baby to suckle well).
There is no available special nipple, specifically designed for cleft palate babies, locally. Sure, I could try breastfeeding but as stated above, the baby might not be able to create enough suction pressure to extract milk from his mother’s breast. This may bring about malnutrition and remember the baby is already small for gestational age. This will present a big risk indeed for baby to be malnourished. Babies who are small for gestational age need their weight to be corrected before the reach 6 months old so as to avoid their risk of acquiring metabolic syndrome later during adult life. Meanwhile, I have inserted an orogastric tube but I will have to let baby suckle before going home soon.