One On One

Q & A – Dr Jooste

The Free State this week conducted a mini Smile Week event at the Universitas Academic Hospital where 12 children born with cleft of the lip and cleft of the palate underwent free facial reconstructive surgery. The two conditions present numerous challenges to children including difficulties in breathing and eating. The Weekly’s Martin Makoni spoke to the Head of Department for Plastic and Reconstructive Surgery at the Universitas Academic Hospital, Professor Johannes Jooste, about some of the underlying causes for the problem and what can be done to address them. Makoni also asked Jooste how widespread the problem is in South Africa and how the country compares with the rest of the world. Excerpts:

How would you define a cleft lip?
I want us to talk about both the cleft lip and cleft palate. There are two fixtures in the face that we call the primary palate and the secondary palate. The primary palate comprises the lip, the alveolar, where the four front teeth come in, and the small part at the back of that but in front of the hard palate. The secondary palate is from behind the teeth, to the back, where the soft palate is. So, there are different reasons for developing clefts in these two different areas. The palates usually start developing at around 6-10 weeks of pregnancy. It is a developmental problem because the central features your face – the nose, the lips, the teeth, the palate and everything – grow from the sides, inwards and unite when they come together in the middle line of the face. So, the cleft lip or cleft palate could be a result of something that stopped them from growing together and the cleft was left open. It means the lips are not continuous because from the initial stages, they did not grow together and fuse.

So, what causes the two sides not to grow together and eventually become a solid lip or palate?
It’s difficult to say what exactly causes it but there is a genetic input that we can find. If one of the parents has a cleft lip or cleft palate, there are about 4-7 percent chance that their first child would have a cleft lip or palate. And if there is one parent and one child with cleft lip or palate, the percent goes up a little bit to 12-13 percent. So, there is definitely a familial or genetic input. But anything that can happen during the pregnancy at that specific time when (the two sides of the lip or palate) need to fuse together can stop it. This could be a case of the mother having to take some medication because she is ill. So, that could have an impact on the fusing of the lip or the palate.

But what exactly causes cleft lips or a cleft palate?
It’s not really known. The actual cause has not been completely worked out. It remains a mystery. We are looking at different areas of causes but there is no one that we can specifically go to and say, if we stop doing this, there will be less cases. We are not at that point yet.

Is it possible to detect the possibility of a cleft lip or cleft palate when the baby is still in the womb?
Yes it is. That can be done by doing sonogram investigations. Today’s sonograms are much more comprehensive than in previous years. They can diagnose the cleft lip pre-birth. The palate is not always easy to see but because you can see the features on the baby, you can see that there is a cleft lip, that it’s not together. And they have started trying to operate on the intrauterine to try and have the baby born normally. There has been some success, not in people yet, but in animals. So, they are working on that, but usually this is found at birth and then we – as reconstructive and plastic surgeons – are called to see how we can go forward. We always have to speak to the parent of the child, first to get their consent for any procedures to correct this.

What sort of problems do children born with cleft lip or cleft palate face?
At birth, the main problem there is usually feeding, especially if there is a cleft in the palate as well. This is because your palate is normally like a shield between your nasal cavity and your oral or mouth cavity. The soft palate at the back closes off the back of the throat. So, if you are swallowing, you can close off that, and then you can clear negative pressure inside your mouth. A baby has to do this in order to suck milk into its mouth and swallow if they don’t have problems swallowing. But if they have problems creating this negative pressure for sucking on the nipple… because the air comes through the nose and they can’t shut-off the nose. So, the baby can’t actually suck. If the baby as a curved palate, the mother has to make the tit of the bottle softer and have a bigger hole for the milk to come out easily. In this case the baby has to use its gums to gnaw on the tit and when the milk drips out into the mouth, the baby can swallow. The baby can’t suck. And those babies with cleft palates have lots of problems with breastfeeding because they can’t suck the breast to get the milk out. So, the mother has to express the milk from the breast, put it into a bottle, and then feed the baby with her own breast milk. But that is something the mother has to learn from an early age.

And socially, what sort of challenges can be faced?
Because children with cleft of the lip and cleft of the palate cannot feed easily, that places a burden on families and caregivers who often do not know how to deal with such a situation. Those with the cleft of the lip are often denied their right to smile and become miserable due to the social stigma they face. This burden is shared with their parents and families as some communities are not unfamiliar with such deformities.

But it will obviously be a problem when they grow up, at what stage can this be fixed?
Obviously, as they start growing, the cleft lip will be on the outside and everybody sees that the lip is not continuous. This can be a problem for the mother, so we usually repair the lip at about three months of age. We want the baby to be a little bit bigger to allow the lungs to be a bit stronger and the baby will have to get an anaesthetic, so it works better that way. As for the palate, we look at about 9-12 months before we close that off. That’s just before the baby starts talking, because the baby needs a closed palate to make sounds and meaningful words. After that, the palate should be able to fully close in the second year. But it doesn’t always work like that. At times we come across challenges with speech. So, those are the two main problems, initially feeding, and then speech. This is very long term work, we don’t normally get finished with these children until they are about 18-20 years old. Over that period, if the initial operations were done properly, a person might undergo 3-5 operations. If it wasn’t properly from the beginning, you could be looking at 10-20 operations.

How prevalent is this condition in South Africa?
It’s difficult to say the exact figure but it’s estimated that among the African American population in the US, it occurs in about one in every 2 500 live births. In Malawi, the occurrence is 0.7 per 1 000 live births. Among the Caucasian, it’s about 0.6 per 1 000 live births. But it must be noted that there is no particular group that may be viewed as more vulnerable to the condition than others. There are no conclusive studies on that.

In the event that one cannot afford to the reconstructive surgery, what’s the worst that can happen to their lives?
When the baby grows up to be an adult, they will still have that cleft lip or palate, which may be more visible because they are grown up. A cleft lip might not pose much problems in adulthood because they can still eat and talk. But if the palate stays cleft into adulthood, the person will always talk abnormally. This is because, to speak normally, you need to have an intact palate. If the palate is not intact, the air that you breathe out when you are speaking or talking, goes through the nasal cavity, resulting in a person sounding like they are speaking through the nose, nasal speech. The person will not get ill because they have a cleft lip or palate. They will only have problems with speech and food may come through the nose. In the Free State, parents can visit their local clinics so that the condition of the child can be assessed and referred to a major hospital like Universitas for reconstructive surgery.