Foreword

As a mother or father, you are probably feeling a great deal of conflicting emotion at present, perhaps disappointment, shock, fear, confusion, isolation, as well as the love and protectiveness you feel towards your new baby.

Take heart. As you read information written by the various professionals working with children who have a cleft lip and/or palate, you will realize that a great deal can be done to help your child.

Take your time to read through any information you find, you don't need to know everything at once. Some information will be more helpful to you than others at present and there may be information that you will not feel like delving into right mow. There will be time for that at a later stage.

Take in as much as you need or wish for the moment and remember, you are not alone. There are many professionals and friends available to help you and your new baby.

MoonDragon's Homebirth Pictures & Stories: Keith's Homebirth

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Development of the Lip & Palate

The mouth and nose of a baby develop between the fifth and twelfth weeks of life inside its mother's womb. The palate is the roof of your mouth. You can feel your own palate by running your tongue over the top of your mouth. If you open your mouth and look into a mirror you will see that the palate extends from your teeth all the way back to the little dangling extension, called the uvula, in the middle of the back of your mouth. The palate is made of bone and muscle and is covered by a thin, wet skin that forms the red covering inside the mouth. It's purpose is to separate your nose from your mouth much like a floor separating the basement from the ground floor. The palate has an extremely important role during speech because when you talk, it prevents air from blowing out of your nose instead of your mouth. The palate is also very important when eating. It prevents food and liquids from going up into the nose. During swallowing, the tongue presses up against the palate and pushes the chewed food to the back of the throat where it then goes down into the stomach.





The lips and palate originate from three areas of the baby's developing face. These areas are also called prominences. They are the Central or "Frontal Nasal" prominence, the Left "Maxillary" prominence, and the Right "Maxillary" prominence. The Central prominence will grow and become the infant's forehead, nose, middle portion of the upper lip (called the philtrum or Cupid's bow) and the primary palate, which is the part of the upper jaw that holds the middle four teeth. The Left and Right "Maxillary" prominences will grow and become the lower face, the lower lip and jaw, all but the middle portion of the upper lip and jaw, and the "secondary" palate (this is the part of the palate from behind the middle four upper teeth to the back of the mouth).



Normally, the three prominences on the child's developing face will grow towards the center of the face and fuse together during the sixth to thirteenth weeks of pregnancy. When this happens correctly the lips, mouth, and palate of the child develop normally. However, sometimes this growth process is disturbed in some way, preventing the prominences from meeting. When growth is disturbed, the lips and mouth do not form properly, leaving a cleft in the lip.

How Does The Normal Palate Form?

As demonstrated above, the prominences grow and come together, fusing to create the nose, the mouth, the lips and the front part of the palate. Next to the prominences are the palatine shelves which start out as ledges on either side of the mouth. As the fetus grows, these ledges lengthen and join in the middle to form the back of the palate in the same way that a zipper closes. The joining process, or "closing of the zipper" starts up front by the teeth and moves backwards towards the throat. If the process of growth and joining is interrupted at any stage, a gap or split will develop, resulting in a cleft of either the lip or of the palate. The type of cleft that develops in the lip and/or palate depends upon when the joining process is interrupted.

Click here to see a short QuickTime movie showing development of the palate (592K).

MoonDragon Images - QuickTime Movie: Closing of Palate

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What is a Cleft Palate?

A cleft is defined as a gap or split between two objects. A cleft palate is a palate that has not formed properly during the baby's growth inside of the mother. There is a split in the palate leaving a hole between the nose and the mouth.

If the split is not fixed, the child may develop problems with eating, swallowing, talking, and sometimes with hearing. Examples of such problems include food or liquids coming out from the nose while eating, repeated sinus and ear infections, and poor speech with a nasal sounding voice that is very hard to understand. If the cleft involves the bone of the upper jaw, the child's teeth may also be affected which can alter the child's ability to chew.

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DETAILED DESCRIPTION



A cleft lip (CL) is a separation of the two sides in the upper lip. The separation often includes the bones of the upper jaw and/or upper gum. A cleft palate (CP) is an opening in the roof of the mouth in which the two sides of the cleft palate can occur on one side (unilateral cleft lip and/or palate), or on both sides (bilateral cleft lip and/or palate). Because the lip and the palate develop separately, it is possible for the child to have a cleft lip, a cleft palate, or both cleft lip and cleft palate.



Clefts result from incomplete development of the lip and/or palate in the very early weeks of pregnancy. Cleft lip and cleft palate are congenital defects, or birth defects. During this time the face is being formed - the top and the two sides develop at the same time and grow towards each other, finally fusing in the middle. The lip and primary palate develop at 4 to 6 weeks of gestation, while the secondary palate develops at approximately nine weeks.

Cleft lip and cleft palate comprise the fourth most common birth defect in the United States. One of every 700 newborns is affected by cleft lip and/or cleft palate.

The majority of clefts appear to be due to a combination of genetics and environmental factors. The risks of recurrence of a cleft condition are dependent upon many factors, including the number of affected persons in the family, the closeness of affected relatives, the race and sex of all affected persons, and the severity of the clefts.

A child born with a cleft frequently requires several different types of services, e.g., surgery, dental/orthodontic care, and speech therapy, all of which need to be provided in a coordinated manner over a period of years. This coordinated care is provided by interdisciplinary cleft palate/craniofacial teams comprised of professionals from a variety of health care disciplines who work together on the child's total rehabilitation.

In the instance of a cleft forming, the final closing does not fuse properly and an opening remains. The cause or causes for this failure to close are as yet not clearly understood, although research has been and continues to be undertaken. Genetics and environmental factors are both considered instrumental in causing clefts.

A. Cleft lip before surgery, age 2 months 8 days. C. Cleft palate before surgery, age 2 months 8 days.	B. Cleft lip after surgery, age 5 months 22 days. D. Cleft palate after surgery, age 5 months 22 days.

A submucous cleft palate is where, although the surface layers of the soft palate (mucous membrane) are complete, the underlying muscle is incomplete. A submucous cleft of the hard palate is where the bony element is incomplete. In its most minor form only the uvula is cleft, but even this leads to an abnormality in the muscles in the palate and, if the speech is affected, a repair will be required. Normal speech production is the primary goal of any surgical repair of a submucous cleft. A submucous cleft palate can prove difficult to identify, the palate appearing normal in some children. Special tests may be necessary to properly identify it. For more on submucous cleft palate, see the Cleft Palate Foundation.

A. Cleft palate before surgery, age 0 months 15 days. C. Cleft palate after surgery, age 2 years 12 days.	B. Cleft palate after surgery, age 5 months 21 days. D. Cleft palate after surgery, age 2 years, 10 months 28 days. Healing is complete.

A cleft lip or cleft palate can be either unilateral (one-side only) or bilateral (both sides). A cleft can be either complete or incomplete. A complete palatal cleft involves both the primary and secondary palate, while an incomplete cleft involves the secondary palate only. A child may be born with either a cleft lip or cleft palate or both. Combined cleft lip and palate (CLP) represents approximately 50% of incidents, cleft palate alone is about 30%, and cleft lip alone is about 20% of incidents.

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What Can Be Done?

Cleft lip and cleft palate are correctable birth defects. Treatment of cleft begins within months of birth with corrective surgery, and continues in one form or another until the person reaches their late teens.

Primary surgery begins with the lip repair at about 3 or 4 months and palate repair between 6 and 12 months. Cosmetic surgery on the nose may occur before the child attends school. A corrective rhinoplasty may be performed on the nose in the teenage years. Orthodontic surgery begins with maxilla expansion at around 9 to 10 years followed by bone grafting. Further corrective surgery (an osteotomy) may be carried out at about 18 years.

Management Team

The treatment of Cleft Lip and Palate is carried out by a team of Specialists. These are:

• Plastic Surgeon


• Speech and Language Therapist


• Orthodontist


• Maxillo Facial Surgeon


• Ear Nose and Throat Specialist


• Audiologist


• Pediatric Dentist

The psychologist, the social worker and a dietitian may sometimes be involved. Where there are any psychological or other personal or family problems, these specialists can give tremendous support.

Some Basic Points For Parents About Cleft Lip & Palate

The condition affects approximately 1 in every 700 babies.

It is the most common birth defect of the head and neck region.

It occurs in the early weeks of pregnancy. During this time the face is being formed - the top and the two sides develop at the same time and grow towards each other, finally fusing in the center. For some reason in a child with a cleft lip and/or palate this final closing does not fuse properly and a cleft remains open.

The cause or causes for this failure to close are as yet not clearly understood although much research has been and is being undertaken.

In some cases there is a hereditary factor but cleft lip and/or palate can suddenly appear in a family with no known history of the defect. When this happens it may not appear again for several generations.

Teeth are important. The greatest of care should be taken of all children's teeth but this is especially important in the case of a cleft lip and palate child. The first teeth and later the permanent teeth are very important for the success of the Dental Specialist's work - neglected teeth make the task of the Orthodontist more difficulty.

Hearing is important. In some children there can be a hearing impairment due to the cleft lip and palate condition. This does not always happen, but the possibility is there and the child's hearing should be checked at approximately 12 months of age. Bad hearing can hinder the easy development of good speech.

Speech and Language is also important. Parents should talk to their children in the normal way. They should speak clearly and should never allow other children to make fun of their child's early speech. They should read stories to the child and increase his/her vocabulary at every available opportunity.

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CAUSES & RISKS

What Causes a Cleft Lip & Palate?

Family History

Some families have a history of clefting. There may be a grandparent, parent, cousin, brother or sister, or another relative who has had a cleft palate. This may be passed on from generation to generation. However, only 1 out of every 5 clefts are inherited. There are many children born with cleft palates who have no family history of clefting.

Environmental Factors

Most cleft palates seem to be caused by environmental factors that increase a mother's risk of giving birth to a child with a cleft palate. Such factors include exposure to German measles (Rubella) or other infections, certain medications, alcohol and drug usage, cigarette smoking, and certain vitamin deficiencies, especially during early pregnancy.

Since the baby's face develops so early in the pregnancy, even when these factors are minimized through proper prenatal care, the damage may have already occurred to the child before the mother was even aware that she was pregnant.

The number of children born each year with cleft palates is growing. Some health care providers and scientists believe this rise is caused by the recent increase in teenage pregnancies and the unavailability of proper prenatal care to many pregnant women. Good nutrition prior to becoming pregnant and during pregnancy when combined with good prenatal care is one of the best ways to increase the chances of giving birth to a healthy baby.

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How Often Do Cleft Palates Occur?

The chances of having a baby with a cleft palate (without a cleft lip) is thought to be 1 in every 3000 live births. It is more common in Asian and Asian-American populations and less common in Africans and African-Americans. Out of one hundred children born with a cleft palate, sixty of them will be girls and forty of them will be boys . An important fact to remember is that a baby born with a cleft palate is more likely to have other associated birth defects than babies not born with a cleft palate. Also, infants with a cleft palate only are twice as likely to have associated birth defects than infants born with a cleft palate and a cleft lip.

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What Are My Risks For Having A Baby With A Cleft Lip or Palate?

In general, the risks of giving birth to a child with a cleft palate are highest when both parents have clefts themselves. If only one parent has a cleft palate, the risk of having a baby with a cleft palate is 1 in 20 (5%). If a set of parents, neither of whom have a cleft, gives birth to a child with a cleft, the chances of them giving birth to a second child with a cleft palate is between 2 to 4 percent.

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Can A Cleft Lip & Palate Be Prevented?

The major issue in prevention of cleft palates is public education of possible environmental and genetic dangers to their children. Increased access to prenatal care for mothers and an effort to decrease teen pregnancies are important goals. Women older than 35 must be counseled regarding the maternal age risk factors because older women are more likely to give birth to a child with a birth defect. Also, families with a history of cleft palates should seek genetic counseling to determine their relative risks for giving birth to a child with a cleft palate.

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Genetic Background & The Genetics of Cleft Lip and Palate

The human embryo has a recognizable face at around eight weeks, when it measures 28mm from head to rump. At this stage the nose and lips have already been formed, probably as a result of cells migrating from the direction of the forehead and cheeks into the face. The primary palate is formed at the end of week seven, and results from growth from the inner sides of the upper jaw towards the midline, and their subsequent fusion. Fusion proceeds from front to back and is not completed until the 11th week. The primary deformity in clefting of lip or palate occurs if this process of fusion is not completed.

Cleft lip and palate occur among all races and has been recognized for many years. In the general population, i.e. from couples with no history of clefts on either side of the family, the incidence varies with populations, between 1/5000 and 1/1000 for cleft lip and/or cleft palate, and about 1/2500 for cleft palate alone. Asians are at higher risk than Caucasians or Blacks. Data from Denmark, where centralized data has been kept for decades, suggest a slow increase in the incidence in recent years, but this could be due to reporting of milder cases.

The causes of cleft lip and palate remains unclear, although there is agreement that heredity plays a major role as a cause. The most recent surveys indicate that in some families there may be just one change in one of the units of heredity, or genes; however, cleft lip or palatein different families may be due to different changes, or mutations, in different genes that have very similar effects in the development lip. in these cases genetic risks should be reasonably easy to determine. In other families, cleft lip may be due to the combined effects of more than one gene. Possibly more than 3% of families. Lastly, animal models suggest that maternal exposure to substances that alter the normal composition of the hereditary material during the critical period of development cooperate with the genetic make up of the developing embryo in producing the cleft lip. Manifestation of the trait would depend then, not only on genetic make up, or genotype, but also on exposure to these unspecified harmful substances called mutagens.

The genetics of cleft lip and/or cleft palate is complex to say the least, and an accurate clinical diagnosis is essential before giving recurrence risk figures. The first thing to be considered is that cleft palate as an isolated malformation behaves as an entity distinct from cleft lip with or without cleft palate. One line of evidence suggesting that this is the case comes from twin studies, one third to one half of identical twins are concordant, i.e. if one shows clefting the other one shows it also, although the degree of severity observed may be quite different between both twins, however, one twin with cleft lip and the other with cleft palate have never been observed.

A second consideration is that cleft lip, with or without cleft palate, may occur as one of a group of malformations in many syndromes of complex appearance. A syndrome is a group of small abnormalities that are often seen together in children of unusual appearance, giving them a resemblance or "family air" as it were. So cleft lip and/or palate are often associated with other abnormalities, forming part of several syndromes in a person of otherwise normal appearance. In some of the syndromes where cleft lip and/or palate are seen, the other abnormalities may be obvious and more threatening, as in the case of some chromosomal syndromes. It could be worthwhile to make here some brief comments about chromosomes. Chromosomes are like packages where the cell organizes the hereditary material when it is going to divide. These packages are normally found in an even number, and can be matched in pairs, reflecting the fact that half of the hereditary material of the cell comes from the father and the other half from the mother. There are 46 chromosomes (23 pairs) in the human cells, except in the germ cells where the members of each pair have separated and are found now in "single dose." Twenty two of these are called "autosomes", and they contain the information for many varied traits, some of which will be noticeable in the offspring. For the purpose of identification, these chromosomes are assigned a number, from 1 to 22, so that when we refer to chromosome 6 for instance, we all know what chromosome we are talking about. The remaining chromosome is called the sex chromosome, because among other genes it contains the genes that determine the sex of the offspring. Sperm carrying the Y sex chromosome is bound to produce a male offspring, while sperm carrying the X sex chromosome will produce a female.

The chromosomal syndromes are associated with abnormalities in the distribution of the chromosomes among the germ cells, so that some of these germ cells or gametes end up having more hereditary material than normal, and others less. These events, when they happen, cause very characteristic abnormalities, easy to identify, like for instance Downs syndrome. In other cases where cleft lip and/or palate is observed, the accompanying clinical features may be more subtle and could go unnoticed in a superficial examination. For instance, in Van der Woude syndrome the clefting of the palate or upper lip is accompanied by symmetrical lumps or pits on the lower lip. Van der Woude syndrome is diagnosed in the presence of cleft lip, or cleft palate, or both, together with the lower lip pits, and it is inherited from one of the parents to the child with the probability of one in two.

In some families, clefting of the secondary palate or bifid uvula are inherited following an "X-linked recessive" pattern, i.e. the problem is observed in 50% of the male offspring of unaffected couples, where the mother is in most cases the carrier. In these families, the daughters are hardly ever affected, but 50% of them on average are carriers. In one of these families from Iceland the single gene has been mapped to the long arm of chromosome X.

In its "pure form", i.e. unaccompanied by any other malformation (the so called non-syndromic cleft lip with or without cleft palate) it appears to be an autosome; dominant in some families, where it has been located on the short arm of chromosome 6. Other pedigrees show an autosome; recessive pattern of inheritance, not mapped so far. A detailed family history may help to establish the mode of segregation, and hence the recurrence risk factor. However, most families may not show a pedigree congruent with the autosomal dominant model, and if other diagnoses have been excluded, it would be proper to quote recurrence risk figures based on empirical data compatible with a multifactorial model. The following table may be useful in these cases.

GENETIC RISKS IN CLEFT LIP AND PALATE

Relationship to index case	Cleft lip/palate	Cleft palate
Siblings (overall risk) Siblings (no other affected) Siblings (2 affected siblings) Siblings & affected parents Children Second degree relatives Third degree relatives General population	4% 2.2% 10% 10% 4.3% 0.6% 0.3% 0.1%	1.8% - 8% - 6.2% - - 0.04%

Notice that if it can be established that there are no other affected relatives, the risk to siblings (2.2%) is less than the overall risk (4%). The higher figure should be used if the history is unreliable or unavailable.

From a subjective point of view, it is essential to emphasize that cleft lip occurs as an accident of nature, and not through any fault of any of the parents. happily, as commented in other sections of the brochure, it is a problem amenable to correction.

We can not but speculate on the reasons why the genetics of cleft lip and palate are so frustrating. It could well be that we are looking at the wrong trait, that is to say, at a trait that is too far removed from the error in the development of the embryo. There are many processes which could play a role in the formation of the lip, and each of these requires different key molecules to be made normally. Many genetic mutations could alter the function of any of these molecules, and any of these could lead to cleft lip. This model would explain the high incidence of cleft lip and the low recurrence in the same family. But several other models are possible.

In brief, when counseling a family with cleft lip and palate it is essential to ascertain whether this is the only malformation (non-syndromic cleft lip) by examining the patient in minute detail. Then a careful family history should be taken to ascertain whether there is a recognizable pattern of segregation, i.e. whether it can be said that in a particular family the clefting is inherited as a sex-linked or an autosomal trait, etc., and if all of these have been excluded the figures given in the table above could be mentioned. The therapeutic requirements and possibilities should be mentioned in the same session, if possible with illustration of real life corrections.

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CLEFT TREATMENT

Cleft Lip Repair

The surgical repair of the cleft lip is carried out at about three to four months of age when the baby is about 10 lbs in weight. It is carried out under full anesthetic. There are various opinions as to whether it is better to do a repair earlier or later than three months, but most surgeons opt for the middle ground of about 3 to 4 months.

The appearance of the protruding premaxilla is very worrying to the parents or other non-medical person who cannot see how the defect can possibly be rectified. The whole emphasis in repairing the lip is on the muscle repair, in order to mould the distorted premaxilla back into its proper position.

The premaxilla in a baby with cleft palate is rotated forward because the gums are divided or cleft and the normal restraining action of the muscle in the lip is broken. The emphasis is on the muscle repair at the time so that the premaxilla is gently moved back into position by the repaired lip.

The repair involves making incisions and bringing the pieces of lip together to form a full lip. There are a number of different techniques from which the surgeon can choose depending on the nature of the cleft. Your child will be given pain killers after the procedure, and the stitches will be removed after approximately one week, again under general anaesthetic.

Cleft Palate Repair

Palate repair is carried out between 6 and 12 months of age and likewise is carried out under full anesthetic. The premaxilla has been moulded back into place and the repaired muscle has helped to close the cleft palate partially. The surgery involves making a number of incisions in the palate and using the tissue and muscle present in the roof of the mouth and joins these together to close the cleft in three layers, namely the roof of the mouth, the floor of the nose and the muscle in between. Like the lip repair, there are a number of different techniques the surgeon can use. However, as long as the operation is carried out by a surgeon experienced in management of Cleft Lip and Palate, there need not be too much concern about the various types of surgery or the timing of it.

In observing babies after surgery, some surgeons felt that it was very unkind to restrain them with elbow splints or mittens to keep their hands away from their faces.

A test study was carried out by an Irish Plastic Surgeon on twenty babies, using adhesive strips to cover the stitches on the lip repair and putting no restraints on the babies' hands. Not one case went wrong. A paper on the findings was sent to the Plastic Surgeons Journal in Britain. The editor of the journal was very interested in the paper and drew attention to existing evidence which showed that babies do suffer emotional and psychological damage through being restrained. The paper was well received and it appears that restraints are not now as widely used as before. However, this is still a matter for the individual surgeon.

The above point is made to illustrate the need to have an open mind on new methods and also to show how important it is to keep the child's confidence during the overall approach to the treatment of cleft lip and palate.

In about 10% of palate repairs the center of the repair does not heal, leaving a hole (fistula) through which there may be excess leakage. This hole usually needs to be closed surgically at a later stage (between 3 and 10 years of age).

Pharyngoplasty

Some children will have to have a special procedure called a Pharyngoplasty to improve the quality of their speech. This is because in some children, the soft palate is not functioning properly and allows air to escape into the nasal passages during speech, giving a nasal tone. A pharyngoplasty, performed by the plastic surgeon creates an improved seal between the soft palate and the back of the throat to reduce the air escape and hence the nasal tone.

Not all children with nasal speech will require this operation. A detailed assessment by the speech and language therapist and plastic surgeon, including x-rays and videos of palatal movement will determine what treatment is required.

This procedure involves repositioning some of the tissue from the palate and the back of the throat so as to prevent air escape through the nose, the escape having resulted in nasal speech. This escape of excess air is referred to as Velapharyngeal incompetence (VPI). This procedure is required in approximately 10-20% of all palate repairs. VPI cannot be corrected by speech therapy alone.

Normal Soft Palate	Soft Palate Insufficiency

Alveolar Cleft Bone Grafting

The expanded anterior (or front) part of the hard palate contains the teeth, both baby teeth and permanent teeth. When this part of the palate is cleft - i.e. in a complete unilateral or bilateral cleft lip and palate - the alignment of the teeth will be distorted and orthodontic treatment will be necessary at a later date.

Alveolar bone grafting is the replacing of missing bone in the front and roof of the mouth. This procedure is carried out when the second, or permanent, teeth are beginning to erupt, which is usually at around 9 to 11 years of age. The orthodontist determines the timing while the procedure is carried out by either a maxillo-facial surgeon or plastic surgeon. The bone graft ensures that teeth have a stable support to erupt through. The bone for the graft is taken from the iliac crest (the top of the hip). See Orthodontics (9-13 years) A lip revision can sometimes be carried out at the same time as the bone graft if the initial lip repair is deemed to be some way unsatisfactory.

Rhinoplasty

Rhinoplasty, or nasal surgery, is designed to improve the appearance of the nose. It may be carried out because the nose appears either too broad or flat or asymmetrical. Often in the repair of a bilateral (two-sided or double) cleft lip, the tip of the nose is pulled down. Ear cartilage is used to build up the flat nose. This surgery is carried out upon the recommendation of the surgeon and can take place anytime from just prior to your child attending school right up into his/her teenage years. Early repair may not prove sufficient as the shape of the nose changes constantly throughout the teenage years requiring further intervention. For this reason you may find the surgeon reluctant to recommend such early nasal surgery. Indeed this surgery is often left until the age of 16-18 years.

Good Speech



A cleft lip is serious but can be dealt with by careful surgery so the child will look well and the stigma of cleft lip will be minimal, however the problems of the cleft palate are far more significant and can affect speech and language development. This may require not only speech and language therapy but, in some cases, further surgery. This is why regular recalls are essential to monitor the child's speech. The child should first see a speech and language therapist at six months and be reviewed regularly until the speech and language therapist feels that he is ready to be discharged.

Unilateral Cleft Lip & Palate Samantha at 3.5 months Samantha at 6 months Samantha at 22 months Bilateral Cleft Lip & Palate James at 12 months James at 3 years James at 7 years

So, the plastic surgeon who began the repair work when the child was three months old finally discharges him/her at the age of 18-20 years. This is why it makes sense to have a co-ordinated team to ensure that all the recall appointments are made for the proper time of treatment and that nothing is left to chance.

Nonetheless, parents should ensure that their child is seen by the plastic surgeon until the child is discharged.

Factors Affecting Surgery

An extensive survey into the various factors which might give the best results in cleft lip and palate repair was carried out in a reputable Cleft Palate Unit in England. Everything was examined:

The type of surgery, i.e. operations carried out following the examples laid down by different surgeons over the years

The type of sutures

The timing, i.e. the age of the child

Hemoglobin (blood iron level)

Parental influence

There was some evidence to suggest that early rather than late repair was better - certainly before speech would normally begin between one and two years. But, of all the factors considered in this survey, there was no evidence that one factor or decision was better than another except in the area of parental influence. There was overwhelming evidence to show that environment factors and parental influence made a significant difference. The development of the child's language skills and confidence were much greater when the parents were well motivated towards treatment. When they co-operated fully with the specialists and had a healthy, balanced, caring attitude to their child, he/she was much more likely to benefit maximally from treatment.

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FEEDING

Many children with clefts feed without difficulty so you should not automatically expect problems. The most immediate concern for a baby with cleft palate is good nutrition.

Sucking for children with a cleft palate is difficult because of the poorly formed roof of the mouth and without a properly functioning palate, it is difficult to gain and maintain suction of the mouth. There may also be some problems of liquid coming down the nose. Children with just a cleft lip (without a cleft palate) do not routinely have feeding difficulties. The following are suggestions to help aid in the feeding of your infant:

• In normal feeding the upper lip does relatively little work so babies with cleft only do not usually have a difficulty.



• Breastfeeding is allowed. It will take extra time and patience. Be open for alternatives if this is not providing adequate nutrition for your infant. You may still pump your breast and feed your infant breast milk through other techniques.



• Small, frequent feedings are recommended. This can be a frustrating and slow process, however, your infant will receive more calories, and therefore, gain weight.





• Positioning your baby differently may also help with feeding. You may find that using a more upright position prevents fluids coming down the nose. Whatever the position, it should be comfortable for both parent and baby. Referral to a dietitian is recommended for advice on dietary requirements.

There may be temporary feeding difficulties following palatal repair. Feeding patterns pre and post operative should be checked with the hospital where surgery is due to take place.

It is important to remember that aside from the mechanical difficulty of the cleft your child is like any other baby and, given some help and direction, you can develop a workable feeding pattern. If your child is having difficulty it is important to acknowledge that the fault lies with the cleft, not with your child or with you. You cannot be expected to automatically know the most suitable way to feed your child.

Parents should not have to feel stressed or alone when coping with feeding difficulties. If your baby is having problems you should get in touch with a speech and language therapist. An early referral to speech and language therapy is not automatic since not all pediatricians or plastic surgeons are aware that speech and language therapists help with feeding. You may have to request a referral.

Since cleft lip and palate is such a specialist area, not all speech and language therapists will be experienced in helping with feeding difficulties but will always be able to refer you to a therapist who specializes in children with cleft lip and palate.

Feeding Supplies

There are many types of bottles and nipples on the market that can assist with feeding an infant with cleft palate. Consult your child's health care provider regarding which type is most appropriate for your child. The following are a few examples:

• NUK Nipple: This nipple can be placed on regular bottles or on bottles with disposable bags. The hole can be made larger by making a criss-cross cut in the middle. An ordinary bottle with a large cross cut in the nipple allows a free flow of milk.



• Mead Johnson NurserŽ: This is a soft, plastic bottle that is easy to squeeze and has a large crosscut nipple. You may use any nipple that the infant prefers with this system.



• Haberman FeederŽ: This is a specially designed bottle system with a valve to help control the air the baby drinks and to prevent milk from going back into the bottle.



• Rosti Spoon Bottle: This is a specially designed bottle that seems to be found in United Kingdom, Ireland and Australia.



• Syringes: These may be used in hospitals following cleft surgery and may also be used at home. Typically, a soft, rubber tube is attached on the end of the syringe, which is then placed in the infant's mouth.

In some cases, supplements may be added to breast milk or formula to help your infant meet his/her calorie needs. Consult your child's physician regarding other home devices (e.g., small paper cups) for feeding your child with a cleft palate.

Feeding your baby is not only a matter of giving him/her sufficient nourishment. In usual circumstances feeding is a relaxed enjoyable time for parent and baby. Close physical contact encourages a good relationship and eye contact and turn-taking form the foundations for early communication.

If feeding is stressed and frustrating it is a negative experience and the opportunity for early interaction is missed. The main point to remember is that help is at hand if you seek it out. Given help and direction, although you may have to make adjustments to make feeding successful, you can become relaxed and confident in feeding your baby.

Some Feeding Products & Information:

Feeding Products

Feeding Products

Feeding Protocol

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SPEECH


Speech & Language in Cleft Palate

It is important to realize that not every child with a cleft palate will experience speech difficulties. However, because of the nature of the condition, the child with cleft palate is more at risk of having a speech and/or language problem and both parents and professionals should keep a close eye on speech development.

Speech and/or language problems in children with cleft palate are usually associated with;

• Deficits in hearing.


• wrong patterns of tongue movement.


• Problems of palate function.


• A combination of these factors.

A child with a cleft palate should be seen by a speech and language therapist at about six months of age or earlier if there are feeding difficulties. Feeding and speech are closely linked so a speech and language therapist can help with both aspects. Obviously a child this young will not be able to co-operate with the therapist, but the therapist can learn much from listening to the child's babbling pattern and observing how he/she interacts with the parents. At this point the speech and language therapist can work with parents and advise on important aspects of speech and language development. Also, once in the system, the child can be reviewed regularly by the speech and language therapist to ensure everything is developing along normal lines.

Helping Normal Speech & Language Development

As with any child, there is much that parents can do to encourage development of good speech and language. Remember, in order to be able to speak, the child must first be able to listen and understand words and sentences, so it is important to help in this process. The importance of parents helping with early speech and language development cannot be over emphasized, since the child will spend most of his/her time with the parents in the early years. However, activities should be done in a natural and fun way where possible and parents should not place the child under pressure.

Here are some guidelines:

Talk to your child from a very early age. Do not just do an activity; talk your child through it, e.g. at bath time, "here's the soap/water/towel etc.", "Mammy is washing", "washing hands/face/toes etc.".

Name everyday items such as cup, spoon, plate, car, door, dog, bricks etc. for your child to help him/her to understand and use vocabulary.

Going through simple picture books and naming the various items helps vocabulary.

Pretend play with tea sets, dolls, shops, dressing up, etc. is a great fun activity in which your child can learn lots of simple vocabulary and sentences. Again talk your child through the activity, such as "dolly is eating", "eating cake", "pouting the tea", "here's the cup etc".

Telling simple stories from picture books is very helpful and enjoyable. Adapt language suitable to the child rather than using the book rigidly.

Singing little songs and nursery rhymes also helps to develop language. Children with a cleft palate may tend to use the back of their tongue too much, resulting in many sounds being made far back in their mouth. The speech and language therapist can advise on this. It is important, however, to encourage the child's attempts at babbling, and praise effort even if it does not sound totally correct.

Speech & The Soft Palate

The length and movement of the soft palate (at the back of the mouth) are important factors in good speech. During speech the soft palate moves back and up to close against the back wall or the throat preventing air from escaping into the nasal passages.

If the palate is too short or the movement is poor, this closing action does not take place and air escapes into the nasal passages, causing nasal sounding speech.

There are exercises which promote good palatal movement which you can practice with your child as part of a game:

Any blowing exercise eg. blowing bubbles, blow football, blowing through a straw, although your child may learn to do this until he or she is 2 or 3 years of age.



Sucking exercises such as using a straw to pick up small paper squares (make sure they are larger than the width of the straw so that the child cannot suck them through the straw). Sucking up juice with a straw is also good exercise.

If nasal speech is a problem a detailed assessment will be carried out by the plastic surgeon and speech and language therapists to determine the best course of treatment. This can include speech recordings, x-rays and videos of palate movement. The plastic surgeon and speech therapist can then decide if the child requires:

1. A course of speech therapy.
2. A course of speech therapy and an appliance in the mouth.
3. Surgery to correct the difficulty, followed by speech therapy if required.

The child may require a combination of these approaches. The majority of children with cleft lip and palate who have initial speech problems will grow up to have normal speech if they are tended to at the appropriate time and have all the necessary speech therapy and surgical and orthodontic treatment.

Older children or adults who have difficulties should also attend a speech and language therapist, even if they did not do so when they were younger, since many problems are correctable with therapy.

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HEARING PROBLEMS & TREATMENT

Hearing & The Cleft Palate Child



The part of the ear which is usually affected by deafness in a child with a cleft palate is the middle ear. If sound cannot travel freely through the inner ear the person cannot hear properly. The middle ear is the structure inside the eardrum which transmits sound into the inner ear. It is in communication with the back of the nose via the eustachian tube. Air must be able to get through the back of the nose, into the middle ear and out again, otherwise deafness will occur.



When a child has a cleft lip only, hearing problems do not arise. However, when there is a cleft in the palate, hearing problems can arise for the following reason. The soft palate at the back of the mouth has muscles connected to the eustachian tube. When we yawn, eat or swallow the palate moves and this pulls the tube open and allows air to pass in and out of it. In a child with a cleft palate, just as there is in an abnormality of the palate muscles, so is there an abnormality of the eustachian tube muscles as both palate and eustachian tube form part of the same muscle group. Thus, if the eustachian tube doesn't work properly, air cannot get into the middle ear as readily as in the normal child and it's place is taken by fluid. When a cleft palate is being repaired by the plastic surgeon some of the muscles connected with the eustachian tube have to be divided to bring them into the middle of the mouth. They are then sewn down the center to close the palate. The presence of fluid in the middle ear interferes with the conduction of sound through it and the child becomes "hard of hearing". He is not deaf but has a moderate degree of deafness. possibly 30-60 decibels on average. It is not known without keeping a national register of cleft palate patients how many children may be affected in this way but it could be as high as 90%. It is therefore important that all children with cleft lip and palate should have a full hearing assessment carried at approximately twelve months.

The Effect of Deafness

Being hard of hearing in early years will mean poor speech, because a child can only imitate the sound it hears. This has improved enormously in recent years because there is continuous testing of hearing in infants. Parents must be aware of the possibility of poor hearing in a child with cleft palate so that he/she is not deprived of full learning ability in the early years.

Usually at the age of 6 or 7 the child's face becomes longer and the eustachian tube becomes more sloped in position. Infection does not then get in so easily and therefore the problems of middle ear infection and blocked eustachian tube tubes tend to decrease after that age. In the younger child, however, the tube is in a more horizontal position and is more readily blocked and infected. This is when the problems more commonly arise.

The Signs of Deafness in Children

Every child with a cleft palate should have extra attention with regard to his/her hearing. Parents and teachers are usually the first to realize that a problem may exist. Turning up the sound of the television or having to repeat oneself are often the first clues of this!

In a very young child it is slightly more difficult to detect hearing loss. If the child appears unresponsive and frustrated or withdrawn, this behavior could be because he is suffering from ear infection resulting in poor hearing. Poor hearing can result in poor or delayed speech. If you are unsure you should bring your child to the ENT consultant to have his ears checked.

Treatment of Hearing Problems

Treatment involves day case surgery, i.e. admission of the child to hospital for a day, the administration of a general anaesthetic and drainage of the middle ear fluid (sometimes known as "glue") with the insertion of a grommet or ventilation tube. Grommets act by draining the fluid, but more specifically by allowing air into the middle ear, thus helping to dry up the secretions and bring the hearing back up to normal. They generally last 6-9 months after which they are usually naturally extruded.

Parents will generally notice an improvement in the hearing within a few days. Unfortunately grommets may need to be inserted on a number of occasions until the child's eustachian tube starts to work properly; in many cases such children will need regular supervision by the ENT surgeon until they are 10 years of age. Some children may actually continue into adolescence with continuing ear problems and a small number will ultimately require more major ear surgery.

Swimming traditionally for children with grommets was not allowed but this has changed over the past few years. Children with grommets in place can swim provided certain precautions are taken. Please discuss this with your ENT surgeon at your post-operative visit with him/her.

Tonsillitis & Adenoids

Like any other child, the child with cleft palate may suffer from tonsillitis and adenoiditis, thus resulting in recurrent sore throats or upper respiratory tract infections. Removal of the adenoid pad at the back of the nose may have the effect of removing the buttress against which the reconstructed palate closes. When repairing a cleft palate the plastic surgeon sometimes takes tonsil tissue from each side to aid in closure of the gap at the sides of the pharynx. The ENT surgeon therefore is often unwilling to remove tonsils and adenoids, thereby compromising the palate repair. In cases, when a child who has had a cleft palate repair performed suffers from true recurrent tonsillitis (6 genuine attacks per 18 months, requiring antibiotics, etc.) surgery may have to be considered despite the risk involved. In such instances it is suggested if possible that the child be seen at a combined cleft palate clinic and a joint decision regarding surgery be made by the plastic surgeon, ENT surgeon, and speech and language therapist involved in the case. If such surgery is really required there is the risk that the child's speech may deteriorate post-operatively, and that even further palatal surgery may have to be considered in due course. The same risk also applies to the removal of the adenoids in such a child.

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ORTHODONTIC TREATMENT

Primary Surgery

This is the first operation carried out by the plastic surgeon who works in close consultation with the orthodontist and the other members of the team of specialists.

Dental Records

From about 2˝ years the dental records begin from which the later treatment is planned, X-rays, impressions and photographs may be taken at this stage.

Full Assessment

At about the age of 7 years a full orthodontic assessment is done. Any supernumerary teeth are removed (these are tiny teeth - denticles) which interfere with the proper development or eruption of second teeth. This treatment is usually provided by a maxillo-facial surgeon.

Preliminary Orthodontics

For some children simple orthodontic treatment involving braces to straighten the teeth can now begin and no further treatment may be necessary.

Rapid maxillary Expansion & Bone Grafting

In most cases, particularly bilateral complete clefts of lip and palate, unilateral complete clefts of lip and palate and some alveolar clefts (bone beneath the gum at the site of the cleft), expansion of the maxilla and the bone grafting is of great advantage.

In these cases the upper jaw appears to settle back a bit further than the lower jaw because the maxilla may not grow as well as it should, due the absence of tissue and bone. This presents a 'crossbite' with the upper dental arch collapsed inwards within the lower dental arch - this should be the other way around.

Expansion should start at about 9 to 10 years of age. It is achieved by fitting fixed appliances to the upper jaw which moves the dental arch back to its correct shape and in doing so, the underlying bone moves with the teeth.

Radiographs will show when this has been successful and the maxillo-facial surgeon then places a bone graft harvested from the top of the hip bone to replace the missing bony tissue.

The expanded position of the dental arch is maintained for 6 to 12 months by which time the graft should have taken successfully.

This procedure should be completed by the age of 10 to 11 years. The teeth will usually erupt through the graft and when all the permanent teeth, including canines and premolars have erupted (usually by the age of 13 years), full orthodontic alignment of teeth can start with the use of fixed braces.

The aim of orthodontic treatment is, where possible, to align all the teeth and close all residual spaces without the use of bridges or dentures. This can usually be achieved when there is only a lateral incisor tooth missing.

Osteotomies

When orthodontic treatment is completed, a significant number of cases remain unsatisfactory because the bones of the upper and lower jaws are incorrectly related to one another and consequently the dental arches are also poorly related to one another. This may be corrected by surgery at the approximate age of 18 years. In the majority of cases an osteotomy will give very good results.

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DENTAL HEALTH

It is important that the primary (baby) teeth and permanent (adult) teeth remain sound and that the gums are kept healthy. All teeth, including the primary teeth, in a child with a cleft lip/palate are important for future orthodontic treatment.

Your child should not sleep with a bottle in his or her mouth as this habit can result in rapid decay of the teeth - a condition known as "Nursing Bottle Decay". If a pacifier is used, it should not be dipped in sugar, honey or anything sweet as this can also cause extensive decay in a young child.

Dental decay is best prevented by avoiding sugary snacks such as bars, biscuits, chocolates, sweets and sugary drinks between meals and at bedtime. Young children are often hungry and sandwiches, fresh fruit, toast, cheese and yogurt are examples of healthy snack foods which may be eaten between meals and at bedtime. Milk, water or sugar-free drinks may also be taken.

Your child's teeth and gums should be kept clean and healthy with regular tooth brushing and this should be supervised by a parent. Daily brushing with a fluoride toothpaste helps prevent dental decay. Only a very small amount of fluoride toothpaste - the size of a pea - should be used for young children and it is important to supervise your child so that toothpaste is not swallowed.

Your child with a cleft lip/palate should attend a local dentist regularly with the first visit at about the first birthday. Dental treatment may be provided by your family dentist or specialist children's dentist.

Looking After Your Child's Teeth

Remember:

Prevention is the key to healthy teeth and gums.

Dental decay can be prevented by a combination of fluoride, diet, oral hygiene and regular dental attendance.

If fluoride is not in the water supply contact your local dental clinic for advice. Use only a pea-sized amount of fluoride toothpaste when your child is young. Tooth brushing should be supervised by a parent so that excess fluoride toothpaste is not swallowed.

When braces are on the teeth a special toothbrush is available and should be used. Fluoride mouthwashes are also of benefit when braces are on the teeth.

When buying soft drinks choose DIET (sugar-free) sodas/tonics as they are kinder to the teeth. When your child is on medication ask your health care provider to prescribe sugar-free brands, if appropriate. It is best, if possible, to avoid soft drinks as much as possible. The sugar-free types often contain artificial sweeteners (such as aspertame or saccharine) that can cause allergic reactions in certain sensitive individuals or other health issues.

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THE HOSPITAL

Prepare Your Child for Hospital

• Talk about hospital as a cheerful place where doctors and nurses help to make people better.


• Play doctors and nurses.


• Read hospital stories for him/her.


• Reassure him/her that you will be with him as much as possible, and that he will be coming home.


• Ask your health care providers about the treatment he/she will have.


• Explain about x-rays, injections, blood tests etc. Tell the truth!

Prepare Yourself

At your first visit with the plastic surgeon, he/she will discuss with you the details of the surgery, risks, complications, costs, recovery time, and outcome. At this time, your child's surgeon will answer any questions you may have. Make a list of questions to take with you.

• Find out as much as you can about the hospital. Check for its advice leaflet.


• Check accommodation, use of food services, use of phone and any costs involved.


• Organize relatives and friends to look after the rest of the family.

For Yourself

• Wear light clothes - hospitals are very warm.


• Bring something to pass the time (books, knitting).


• Bring coins for the phone.


• Be considerate of others, don't make noise at night.


• Don't give any child food or drink without permission.

For Your Child In Hospital

Bring his/her favorite toy or blanket, no matter how scruffy.

Tell the nurse his/her special names for food, the potty etc., and if he has a bottle.

Cuddle your baby.

Play with your toddler.

Help to care for him/her.

Explain what is going to happen during examinations and injections.

Your child will need you especially before and after an operation.

Reassurance and lots of love is often the most important medicine.

Encourage your child to get involved with the other children, and in the hospital play group.

It is important that you take a break.

Always tell him/her when you will be back, and return when you say you will.

Give him/her news from home.

If you cannot stay with him/her all the time, organize other people he/she knows to visit.

After The Surgery For Cleft Lip

• Your child may be irritable following surgery. Your child's health care provider may prescribe medications to help with this. Your child may also have to wear padded restraints on his/her elbows to prevent him/her from rubbing at the stitches and surgery site.



• Stitches will either dissolve on their own or will be removed in approximately five to seven days. Specific instructions will be given to you regarding how to feed your child after the surgery. The scar will gradually fade, but it will never completely disappear.



• During the surgery, and for a short time after surgery, your child will have an intravenous catheter (IV) to provide fluids until he/she is able to drink by mouth. For a day or two, your child will feel mild pain, which can be relieved with a non-aspirin pain medication. A prescription medication may also be given for use at home.



• Your child's upper lip and nose will have stitches where the cleft lip was repaired. It is normal to have swelling, bruising, and blood around these stitches.

After The Surgery For Cleft Palate

• This surgery is usually more involved and can cause more discomfort and pain for the child than cleft lip surgery. Your child's health care provider may order pain medicine to help with this. As a result of the pain and the location of the surgery, your child may not eat and drink as usual. An intravenous (IV) catheter will be used to help give your child fluids until he/she can drink adequately.



• Your child will have stitches on the palate where the cleft was repaired. The stitches will dissolve after several days and they do not have to be taken out by the health care provider. In some cases, packing will be placed on the palate. Do not take the packing out unless you are told to do so by your child's health care provider.



• There may be some bloody drainage coming from the nose and mouth that will lessen over the first day.



• There will be some swelling at the surgery site, which will diminish substantially in a week.



• For two to three days, your child will feel mild pain that can be relieved by a non-aspirin pain medication. A prescription medication may also be given for use at home.



• Many infants show signs of nasal congestion after surgery. These signs may include nasal snorting, mouth breathing, and decreased appetite. Your child's physician may prescribe medication to relieve the nasal congestion.



• Your child will be on antibiotics to prevent infection while in the hospital. Your child's health care provider may want you to continue this at home.



• Your child may be in the hospital for one to three days, depending on your child's health care provider's recommendation.



• A small amount of water should be offered after every bottle or meal to cleanse the incision. You can continue to rinse this area gently with water several times a day, if necessary.

Diet After Surgery

Your child's health care provider may allow breastfeeding, bottle-feedings, or cup feedings after surgery. Your child should be placed on a soft diet for seven to 10 days after surgery. For older infants and children, age-appropriate soft foods may include strained baby foods, popsicles, yogurt, mashed potatoes, and gelatin. Note: your child should not use a straw or pacifier, as both could damage the surgical repair.

Activity After Surgery

Your child can walk or play calmly after surgery. He/she should not run or engage in rough play (e.g., wrestling, climbing) or play with "mouth toys" for one to two weeks after surgery. Your child's health care provider will advise you when your child can safely return to regular play.

Going Home From The Hospital

• Before leaving the hospital, make sure you know how to look after him/her.


• Write down instructions about food, medicine etc.


• Check when he/she has to return for a check up visit.


• Don't worry if he/she is difficult and demanding at home; this will soon pass.


• Talk about the hospital experience, and best of all - give lots of love.

Follow-up with your child's surgeon and the cleft team is very important. This will be discussed with you. Your child's health care provider will also be an important part of the child's overall health management after the surgery.

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Social & Psychological Aspects

The birth of a child with craniofacial malformation represents a crisis for parents. The parents, siblings, grandparents and other close relations may experience a range of feelings such as shock, numbness and grief. Many factors can contribute to these feelings.

Shock & Numbness

When a health care provider discusses a diagnosis with parents, initially parents feel that what they have been told is like a bad dream and one that they will wake up from and all will be well. It takes time for the reality of the situation to sink in. This is why it is important that information is stated and restated, because the amount of information actually taken in by parents in shock is very small.

Denial

"This cannot be happening to me, to my child, to our family". It takes some time to come to terms with stressful situations. This is a normal part of the accepting process and will be worked through eventually. However, when faced with the reality of the situation and given support and information, denial can be turned into acceptance.

Anger

Anger can color communication. It can be directed towards themselves, family, hospital, personnel, God/Goddess, etc. Parents can feel that life has dealt them a very hard blow and they may be angry about this. Again this is a normal reaction and part of the healing process.

Grief & Loss

Often the first reaction to the news is intense grief and sorrow. You may grieve the loss of the ideal baby you dreamed of plus the loss of your family life as you knew it before. You may not even know exactly what you are grieving. What you do know however is that sorrow sits on your chest like a ton of bricks. This feeling is normal for parents who have received such news. It will pass eventually with time. It is easy to forget the joyful experience of your baby's birth. At this time, you have to try and balance the positive aspects of the birth with your fears and anxieties.

Fear

People often fear the unknown. Parents fear that their child's condition will be the very worst it could possibly be. Their first thoughts are totally bleak. Guilt, especially if there is a hereditary element, confusion, sleeplessness and powerlessness are also emotions that parents can have. Gradually however, parents reach a stage of resolution i.e. being resolved to a situation they can't change, and build on the future. Not everyone feels all the reactions outlined. However, if they arise it is important to know you are not alone in these feelings.

How Do You Cope Through This Period?

Take one day at a time. Thinking too far ahead cam immobilize you.

Seek out information, ask questions. Learn and read as much as you can about the health issue and obtain knowledge about it. If you are anxious during interviews, make a list of questions beforehand. Ask you questions and expect direct, honest, clearly stated answers from your health care providers.

Learn the terminology. When you are introduced to new terminology you should not be hesitant to ask what it means. Whenever someone uses a word you don't understand, stop the conversation for a minute and ask the person to explain the meaning.

Do not be intimidated by professionals. They are there to provide a service to you, your child and your family. Don't let them ignore your concerns. If you don't like a certain health care provider or if you feel you are not receiving the best of care for your child, don't hesitate to find another that will work with you, your family and your child. Expect quality care and consideration from your health care providers and their support team. You do have the right to hire and fire them if you feel they are not providing the services you need.

Take care of yourself. In times of stress each person reacts in his or her own way. A few universal recommendations may help. Get sufficient rest, eat as well as you can, take time for yourself, reach out to others for emotional support. Do not be afraid to show emotion. So many parents, especially fathers, repress their emotions because they believe it to be a sign of weakness to let people know how badly they are feeling. Revealing emotion does not diminish your strength.

Support Groups

Find local support groups through your community, hospital, or online. Parents who have older children with cleft lip and palate can support new parents.

Self Esteem & Development of Identity

A child's sense of his wellbeing is developed from his interaction with his/her family, peers and society in general. The first place where a child learns about the world is within the family. Parents can develop a sense of wellbeing in their child by giving him/her love and cuddles and by gradually letting the child explore his/her world and develop independence.

Hospitalization

Hospitalization and surgery are on-going for a child with cleft lip and palate. Parents instinctive reaction is to protect their child from pain. Hospitalization for parents and children is a traumatic period. Children's anxiety can be reduced by parents explaining to the child what is happening in language appropriate to their age. Parents should spend time with their child in hospital so that the child can be reassured and comforted. The child needs to know that all is well. All children whatever their age need the reassurance of their parents while in hospital. This can be done verbally with the older child.

Comments From Other Children

Children are very curious people. They learn about their world through constant questioning of everyone around them. They constantly ask why? They are always curious about people and things that they perceive as different. They ask direct questions. Unlike adults they do not avoid the obvious. Once they are given a reasonable answer they continue what they are doing and nothing more is thought about it. If parents of children with a disability give the children a reasonable explanation about the disability, that they can tell their friends, then teasing and name calling can be reduced.

Child Development

Humans are dependent on their care givers for a much longer period of time than any other grouping. As an infant, a baby learns about the world through its needs being met e.g. being loved and nurtured. Through its parents a baby learns to trust and to explore his/her world. gradually as he/she matures, the child gains more independence but returns to the parent when he/she needs security an reassurance.

How? What? Why? are questions asked continuously, especially at 4 years of age. They are eternally asking Why? How? and What? and the meaning of words etc.

Singling out a special friend (5 years).. At 5 years, a child chooses his/her own friends. At 5 years old a child is also into magical thinking. This includes the idea that "wishes come true".

Sensitivity to criticism. At 8 years, a child is sensitive to criticism especially in front of others. This also happens at the adolescent stage.

Adolescence. Teenage years are always years of great uncertainty. Physically there is the development of sexual organs etc. Teenagers take on the adult physique. Their emotional growth is often slower than their physical growth. It is a time of great stress and uncertainty.

The adolescent who has a cleft lip and/or palate, or indeed any physical difference, may not realize that all teenagers go through feelings of uncertainty and insecurity in new and unfamiliar social conditions, and may attribute such feelings to have a cleft.

At this difficult stage, all teenagers need support and reassurance that it is normal to feel a certain amount of self consciousness and insecurity when entering the "social scene" for the first time.

All teenagers require that certain boundaries are set down by parents or guardians and should be encouraged to take responsibility for their actions. Given support, guidance and encouragement, adolescents learn to feel at one with themselves and to enjoy successful friendships at college, work and socially.

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Pierre Robin Syndrome

This syndrome also referred to as Pierre Robin Sequence is a common variation of simple cleft palate. The baby has a combination of cleft palate, a small underdeveloped lower jaw (retrognathia) and a falling of the tongue into the back of the mouth (glossoptosis). This never occurs in combination with cleft lip and is never inherited or passed on.

The incidence is one child in every 30,000. The milder form of the syndrome is relatively common whereas the more developed form is quite rare where the baby may have some problems breathing due to the tongue partially obstructing breathing because of its position at the back of the mouth.

It is commonly accepted that the cause of this syndrome is that the baby has been cramped in the womb with the head pressed more firmly onto the chest than usual. This physically holds back the lower jaw growth and pushes the tongue up into the roof of the mouth, stopping the natural closure of the palate in the early days of development.

This would explain the fact that almost all the babies' jaws catch up in growth over the first year of life and that this is not an inherited condition.

Repair of the cleft palate is often carried out at 9 or 12 months of age when the jaw has grown forward. This is to diminish any chance that if the operation is carried out earlier it might push the tongue back further into the back of the throat bringing on breathing problems.

A small number of babies who have breathing problems at birth because the jaw is so small may need nursing on their face in a special frame for some time while the jaw develops and normal breathing is restored.

It is important that babies with breathing problems are seen by a pediatrician and a plastic surgeon.

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Speech & Feeding

The child may see a speech and language therapist in the early stages if there are feeding difficulties. Some Pierre Robin babies have difficulty in getting their feeding established due to their cleft and small mandible. If this is not the case, parents should ensure that the child is taken to the speech and language therapist at about the age of 8 months. The therapist will not be able to do much direct work with the child at this stage but can advise parents on how best to develop communication directly (See section on Speech and Communication). A referral to a speech therapist is not always automatic and often the parent will have to request one.

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Hearing

Often a child with Pierre Robin Syndrome will have a hearing problem which can have an effect on speech and language development. It is important to have the child assessed for hearing loss at an early age so that the appropriate treatment can be carried out.

With the help of the plastic surgeon, pediatrician, speech therapist, audiologist, ENT surgeon and the dentist, the child with Pierre Robin Syndrome can look forward to a bright future.

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RELATED DISORDERS

CHROMOSOME DISORDERS

Humans have 23 pairs of chromosomes, with two sex chromosomes that decide gender and 44 chromosomes that dictate other factors, such as growth and function. A chromosome disorder is caused by an alteration in the number or genetic structure of chromosomes. A trisomy ('three bodies') is a chromosomal disorder characterized by an additional chromosome, so the person has 47 instead of 46. Down's syndrome (Trisomy 21), Edward's syndrome (Trisomy 18) and Patau's syndrome (Trisomy 13) are the most common forms of trisomy. Children affected by trisomy usually have a range of birth defects, including delayed development and intellectual disabilities.

Trisomy 13 and 18 births are as common as cystic fibrosis and more frequent than muscular dystrophy or neurofibromatosis. Although trisomy 13 and 18 births are considered to be rare, together they number 1 in 1,800 pregnancies and they are the most common autosomal trisomies after Down's syndrome.

Sometimes, signs of trisomy disorders may be evident during the pregnancy. Some of these signs may include:

• Too much amniotic fluid surrounding the baby.


• Only one umbilical cord artery.


• A smaller than expected placenta.


• The baby is small for its gestational date.


• The baby is less active than expected.


• Congenital defects are picked up during ultrasound scans.

MoonDragon's Pediatric Information: Down Syndrome Health Information

MoonDragon's Pediatric Information: Down Syndrome Alternative Approaches & Nutrition

MoonDragon's Pediatric Information: Down Syndrome Overview - A Genetic Disorder

MoonDragon's Pediatric Information: Trisomy 18 (Edwards Syndrome)

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RELATED LINKS

The Cleft Palate-Craniofacial Journal

The Cleft Palate Foundation

Links to Related Professional Organizations

10th International Congress on Cleft Palate & Related Craniofacial Anomalies, Durban, South Africa

American Academy of Facial, Plastic and Reconstructive Surgery

American Academy of Otolaryngology - Head and Neck Surgery (AAO-HNS), Alexandria, VA

American Academy of Pediatric Dentistry (AAPD), Chicago, IL

American Academy of Pediatrics (AAP), Elk Grove Village, IL

American Academy of Audiology

American Association for Dental Research/International Association for Dental Research

American Association of Neurological Surgeons (AANS)

American Association of Oral and Maxillofacial Surgeons (AAOMS), Rosemont, IL

American Association of Orthodontists, (AAO), St. Louis, MO

American Dental Association

American Society for Aesthetic Plastic Surgery

American Society of Plastic Surgeons (ASPS), Arlington Heights, Illinois

American Speech-Language Hearing Association (ASHA), Rockville, MD

Coalition of California Cleft and Craniofacial Teams, Santa Monica, CA

The Craniofacial Outcomes Registry, Chapel Hill, NC

The Craniofacial Society of Great Britain and Ireland, Stourbridge, West Midlands, U.K.

International Consortium for Oral Clefts Genetics, Philadelphia, PA

International Society of Craniofacial Surgeons, Dallas, TX

Florida Cleft Palate-Craniofacial Association, Gainesville, FL

National Institute for Dental and Craniofacial Research, Bethesda, MD

Online Mendelian Inheritance in Man (OMIM), Bethesda, MD

Velo-Cardio-Facial Syndrome Educational Foundation, Inc., Syracuse, NY

Resources: MedLine Plus: Cleft Lip and Palate

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