Pierre Robin Sequence

It is vital that babies born with PRS get the care and supervision they need early on. This care should be provided by professionals who have experience of the condition, and it should be carried out (or at least coordinated from) a specialist hospital, especially during the first few weeks.

Ideally, this should be an established Cleft Team which includes a specialist paediatrician. This arrangement is much better for parents and children because of the expertise within the team, and this way appointments can be arranged as one long visit rather than several visits to individual specialists.

If for whatever reason your child is not under the care of a Cleft Team, you will need to see the following specialists at some point:

• Otologist
• Audiologist
• Dietician
• Specialist Health Visitor

You may need to ask for your child to be seen by these specialists. Do not assume that you will automatically be referred.

As Stickler Syndrome is so common in babies with PRS, your baby must also be seen by an ophthalmologist for frequent eye checks.

It’s best if you have a paediatrician who can look after all aspects of care, be alert for additional problems such as Stickler Syndrome, and refer you to various specialists when appropriate.

Seeing all these health professionals can be quite stressful for both parents and children. To make these experiences more positive and productive, try to make notes of points you wish to discuss and questions you’d like to ask, and make sure to ask for clarification if you don’t understand something that is being said. Keeping good records (such as copies of letters, dates of appointments, and names of specialists) will also make the entire process easier to manage.

All babies with Pierre Robin Sequence are assessed by a specialist nurse to establish the safest method of feeding.

Some babies need more assistance than others due to reflux and breathing difficulties. An individual plan will be put into place for your child which may include a nasogastric (NG) tube, a thin tube which goes in through the nose and into the stomach. It’s the most common method of feeding babies who, for whatever reason, can’t get enough nutrition orally.

Not all babies with Pierre Robin Sequence need a nasogastric tube, and for those that do it’s usually gone by the time they are a year old.

Establishing feeding with a bottle takes time, patience and experimentation depending on the degree of your baby’s difficulties. This includes finding a bottle and a position which suits your baby. All babies should be offered bottle feeding as early as possible (along with tube feeding where necessary) to establish an association between sucking and receiving food. Hospitals should establish a good feeding pattern before any baby born with Pierre Robin Sequence is discharged home. Parents need expert advice and help from specialist feeding nurses, specialist health visitors and speech and language therapists to help them once the child is home.

Breathing and feeding can require a big effort for many babies with Pierre Robin Sequence, so weight gain can be slow. To help with loss of calories, supplements can be added to feeds under the instruction of a dietician, or high calorie milk can be recommended.

Babies with a cleft palate can have a lot of issues with wind, and babies with PRS can gag and vomit easily, possibly due to the position of the tongue.

Feeding Equipment

There are various types of specialist bottles and teats available from CLAPA. Your Cleft Nurse or another medical specialist should advise you which bottles and teats to get.

In some cases, parents and carers are advised to try the ‘Haberman Feeder‘. This is a specialised bottle that was designed by a mother of a child that was born with Pierre Robin Sequence. It is designed to help any baby that has a weak sucking reflex. It is more expensive than other bottles but has been very successful for some babies whose degree of difficulty with sucking has been great. They are available from:

Athrodax Healthcare International Ltd
Hawthorne Business Park
Drybrook
Gloucestershire
GL17 9HP

Tel: 01594 544440

UK parents can get a reduced rate for this bottle as they can claim back the VAT. This exemption is because ‘your baby has a feeding difficulty’ and therefore this type of bottle has zero tax. On receipt of an order form, cheque and VAT form, Athrodax aim to send your order by return post. Credit card orders can be taken but they will be subject to VAT.

CLAPA holds a small emergency stock of complete Haberman bottles but we do not stock the replacement valves and teats, these will have to be purchased from Athrodax directly. Contact us if you need one of these bottles.

Your medical team will give you expert advice on feeding and weaning your baby. The information here is general only.

It is often recommended that babies with PRS start on solid food, such as baby rice, at around four months because they may find it easier to cope with food than with liquids.

As with all babies, solid food should be pureed initially and lumps introduced very slowly to enable you baby to learn to cope with them, otherwise they may gag.

It is important for you baby to experience chewing as it is thought to be a prerequisite for speech development. Teething rings may also be useful.

As always, discuss what is best with the health professionals looking after your baby. Whatever method is chosen, time, patience and a comfortable chair will be needed.

Some babies with Pierre Robin Sequence have very few problems with breathing. However, it is generally advised that babies with PRS are nursed on the side or towards the front rather than on their back.

This is contrary to the advice for most babies, but for babies with PRS it means their tongue is less likely to fall back and block their airways. Remember this is general advice only, and advice for your baby’s situation should always be sought from a specialist.

While travelling, it is recommended that your baby is positioned as upright as possible. If they need to lay on their side, they may need to travel in a carry cot.

You can also buy special car seats for babies that lay flat and allow your baby to travel in a prone position (lying on their front). These are similar to carry cots and can be quite heavy, but can also be adapted to a pushchair.

Occasionally a small tube needs to be passed through the nose, behind the tongue, to improve breathing. This is called a nasopharyngeal airway or nasal prong.

In severe cases, one option is to lengthen the jaw by a technique known as mandibular distraction which gradually moves the jaw bones into a different position over time.

Finally, a very small number of babies with major problems with their breathing require tracheostomies, which is when an opening is created at the front of the neck so a tube can be inserted into the windpipe.

It is very difficult for a baby with Pierre Robin to lay on their back as this can cause their tongue to fall back and block their airway.

The ‘Safe to Sleep‘ campaign to reduce cot death advises that babies sleep on their back and play on their front, but this does not apply to babies with Pierre Robin Sequence.

The exact right position for a baby with Pierre Robin depends on the severity of the breathing difficulties and how they are being managed; most babies with PRS are nursed on their side.

Advice should be sought from a medical specialist who will take all the factors involved into consideration.

If a cleft palate is present, a palate repair operation is needed. This usually happens between 6-12 months, but may be later depending both on the severity of the breathing difficulties and on the preferences of the surgeon.

There is a bit of a debate about the best time to repair a cleft palate with Pierre Robin Sequence. The problem is that repairing the palate may temporarily make breathing worse.

Some surgeons prefer to do the procedure at the usual time (from 6 months old) and accept that there may be some breathing issues which need to be managed until things get back to normal.

Other surgeons prefer to wait for the jaw to grow in the hopes that breathing will naturally get better and therefore reduce the chances of further breathing problems after surgery.

There are benefits and drawbacks to both options. Your surgeon will explain what they think is the right way forward for your baby, and will answer any questions or concerns you may have.

About the cleft palate repair operation

The procedure will usually take between 1 ½ to 3 hours, depending on the extent of the cleft, and babies will usually stay in hospital for at least two days afterwards.

Feeding may take some time to be re-established after cleft palate repair but in most cases should be re-established in one to two weeks.

Because the clefts in babies with Pierre Robin Sequence tend to be very wide, it is very often necessary for relaxing cuts to be made on the side of the palate, but these heal very quickly. There is also an increased risk of developing a small hole in the repair (fistula). These holes sometimes heal by themselves, but sometimes a further small operation is needed.

Learn more about cleft palate repair operations