· Web viewA cleft lip can range from a little notch in the colored part of the lip to a...

INFORMATION PACK

This pack is designed to give you some basic information about different clefts of the lip and palate, some associated conditions, the medical treatment

pathway, and the various additional support services that CLAPA provides.

A cleft information pack for volunteers

Contents

What is cleft lip and palate

?.........................................................................................................................................................2

What causes cleft lip and palate?.................................................................................................2

How is it treated?............................................................................................................................2

Associated conditions ..................................................................................................................... .

Pierre Robin Sequence .........................................................................................................3

Stickler Syndrome..................................................................................................................3

Van der Woude Syndrome ...................................................................................................3

Treatment Pathway:........................................................................................................................ .

Antenatal.................................................................................................................................4

0 - 3 months............................................................................................................................5

3 - 6 months............................................................................................................................5

6 - 12 months..........................................................................................................................6

12 months - 5 years...............................................................................................................7

6 - 12 years..............................................................................................................................8

13 - 20 years...........................................................................................................................9

21+.........................................................................................................................................10

Volunteer Cleft Information Pack June 2016

WHAT IS CLEFT LIP AND PALATE?

In early pregnancy, different parts of a baby’s face develop separately and then join together. If some parts do not join properly, the baby is born with a cleft.

A cleft lip can range from a little notch in the colored part of the lip to a complete separation of the upper lip which can extend up and into the nose. This can affect one side of the mouth (unilateral) or both sides (bilateral), and can be complete (meaning the cleft goes up into the nose) or incomplete.

A cleft lip can also affect the gum where the teeth come through. Again, this can range from a small notch to a complete separation of the gum into two parts.

A cleft palate is a gap in the roof of the mouth. The back of the palate (towards the throat) is called the soft palate, and the front (towards the lips) is called the hard palate. A cleft can affect the soft palate or both the soft and hard palate.

You can have a cleft lip, a cleft palate, or a cleft lip and palate. A cleft palate by itself is often called an isolated cleft palate.

WHAT CAUSES CLEFT LIP AND PALATE?

There is no single cause of cleft lip and palate. Research tells us it’s often caused by a combination of different genetic and environmental factors.

Most of the time, a cleft is caused by genetic and environmental factors coming together in a way which can’t be predicted or prevented. Recent research suggests that when a baby is developing there are certain genes which are ‘switched on or off’ by things in the environment, but it’s very difficult to narrow down what these are.

Remember to stress: if someone has a child with a cleft, it is very unlikely to be because of something they did or did not do.

HOW IS IT TREATED?

Surgery is needed to repair the cleft. Cleft Teams aim to repair a cleft lip between 3-6 months, and a cleft palate between 6-12 months. Different teams may perform surgery at different times, and research is currently underway to see if this makes any difference to a child’s outcomes later in life.


Every cleft is unique, just like every child, so the exact treatment pathway will vary. It may include further surgery to help with issues such as speech and growth of adult teeth. It may also include speech therapy; help with hearing, orthodontic work, etc. A Clinical Psychologist (or similar) will also be available at most Cleft Teams to work with families or individuals at any point in the treatment pathway.

ASSOCIATED CONDITIONS

PIERRE ROBIN SEQUENCE - The condition is rare; UK statistics put it at 1 in 14,500 babies, or just under 50 babies per year. Around 50-80% of these babies have other associated conditions, with the most common being Stickler Syndrome.

Symptoms

Small Lower Jaw Breathing difficulties Cleft PalateA baby with a cleft palate is born with a jaw which is noticeably smaller and more receded than those of other babies. This is called mandibular hypoplasia or micrognathia.

The placement of the tongue means it is more likely to ball up and fall backwards into the throat, causing obstruction and therefore breathing difficulties. Breathing difficulties can be very difficult to manage, so it’s important the baby is under the care, or at least under the supervision, of a specialist hospital.

A cleft palate (or a high arched palate without a cleft) may be present which can affect the child in a variety of ways depending on its severity. Babies with a cleft palate often have issues with milk coming down their nose during bottle feeding, but this gets better as a feeding pattern is established, the child grows and the palate is repaired.

What does this mean? Most children with Pierre Robin Sequence grow up normally, even if they start their lives with quite severe problems. All babies with PRS will have some difficulties, but these will vary from child to child.

Some have no problems with breathing and only minor feeding difficulties. These babies can usually go home shortly after birth.

Others have moderate difficulties which may mean they have to stay in hospital until breathing and feeding patterns can be established. They may need to use a feeding tube (nasogastric or NG tube) for a while at home.

A smaller group of babies have major difficulties in both areas, and these can persist for several months and require the assistance of a nasal prong or even a tracheostomy.

All babies born with PRS need to be carefully investigated and monitored to make sure they can continue to take in enough oxygen.

STICKLER SYNDROME - Stickler Syndrome is a progressive connective tissue disorder. Since one symptom of Stickler Syndrome is a cleft palate, and another is PRS it can be hard to diagnose, as other symptoms aren’t as obvious, the diagnosis may just be for cleft palate and/or PRS.

Between one in 10,000 persons & 3 in 10,000 may be affected. A person with this syndrome has a 50% chance of passing it one to any children.

o There have also been cases where it has occurred for the first time in a child. In some cases, parents only find out they have Stickler Syndrome when their child is diagnosed.

Symptoms, and how severely a person is affected, can vary from patient to patient, even within a family, and can be difficult to diagnose.


VAN DER WOUDE SYNDROME - Van der Woude syndrome is a genetic disorder which features lower lip pits combined with a cleft lip (with or without cleft palate), or cleft palate alone. While there are many syndromes that involve a cleft, this is the only one which can happen with an isolated cleft lip, an isolated cleft palate, and a cleft lip and palate.

The syndrome can vary greatly in how it affects people, so some may not even be aware of their lip pits, or they may have obvious lip pits and a very severe cleft lip and/or palate.

A person with this syndrome has a 50% chance of passing it on to any children. It affects between 1 in 35,000 & 1 in 100,000 people.

TREATMENT PATHWAY

There is a 20-year treatment pathway for cleft lip and/or palate which begins at diagnosis. Not all parts of this pathway will be relevant to all children, but it’s important to have a good understanding of what to expect over the years and how the services all fit together.

CLAPA’s Resources for Parents throughout the Treatment Pathway

Happy Faces Groups for new and expectant parents

Parent Contacts for parents who would like to speak to someone else who can relate to their situation and share experiences

Facebook Group for parents CLAPA has a variety of Facebook groups which can be a useful source of information

Photo Gallery of before and after surgery photos

Antenatal (before birth) Care

Local Care – Obstetric Unit or private Ultrasound Centre

Cleft Unit CLAPA’s Services

Diagnosis of cleft lip at 20-week anomaly scan, confirmed by specialist if necessary

Referral to local Cleft Centre within 24 hours of diagnosis if agreed with prospective parents

3D/4D ‘bonding’ scans are available for free or at a discount in certain areas with a referral from a Cleft Centre

Contact by Cleft Team within 24 hours of referral, usually by the Clinical Nurse Specialist (CNS)

Parents will be offered a visit from the CNS at an appropriate time and place

Online: Information on birth and diagnosis


0 – 3 Months

Cleft Unit Local Cleft Clinic Local Care CLAPA’s Services

Visit from Cleft Nurse within 48 hours of birth, and ongoing support, assessment and advice following discharge home

Specialist feeding assessment, advice and support in the use of special feeding bottles or a nasogastric (NG) feeding tube if needed

Preventative dental advice and treatment in consultation with Cleft Unit. The aim is for the child to have dental health just as good as other local children without a cleft

Newborn Hearing screen for all babies. Referral for ongoing hearing assessment and management for babies born with a cleft palate.

Referral to Ear, Nose & Throat (ENT) and/or audiology if needed

Clinical Psychology Support – parents will have access to a psychologist, counsellor or nurse who can help them cope with any feelings or concerns they have related to their child’s cleft. This is available at all points along the care pathway

Monitoring of child for signs of syndromes associated with cleft.

Genetic counselling will be offered to parents to help them understand why they had a child with a cleft and what the chances are of this happening again. This is available at any time, including when the child is grown up.

Diagnosis of cleft palate within 24 hours of birth.

Contact Cleft Unit within 24 hours of birth and/or diagnosis.

Dental Health education and advice (in liaison with the Cleft Units)

Welcome Pack – a set of specialist feeding bottles and teats as well as information is sent out to parents when required. These bottles and teats are also available to buy online or over the phone.

Information on Feeding and Repair Surgeries


3-6 Months

Cleft Unit CLAPA’s ServicesLip Repair Surgery

The lip repair usually takes place when a child is at least 3 months old. The child will be assessed before any surgeries by the multidisciplinary

team to ensure they are fit enough to have the surgery and to help monitor the ways in which the child will change afterwards. Parents will be able to ask any questions about their child’s care at this stage.

Timings of surgeries may vary, and the surgery date can be pushed back for a number of reasons – parents should always talk to their Team if they have concerns.

The Cleft Team will be able to help parents prepare for surgery, as well as give advice and support regarding aftercare.

Information on Repair Surgeries

6-12 Months

Cleft Unit Local Cleft Clinic CLAPA’s ServicesPalate Repair Surgery

The palate repair usually takes place between 6-12 months.

The child will be assessed before any surgeries by the multidisciplinary team to ensure they are fit enough to have the surgery and also to help monitor the ways in which the child will change afterwards. Parents will be able to ask any questions about their child’s care at this stage.

Timings of surgeries may vary, and the surgery date can be pushed back for a number of reasons

The Cleft Team will be able to help parents prepare for surgery, as well as give advice and support regarding aftercare.

Hearing test at 7-10 months for babies with a cleft palate, as well as annual hearing tests up to 5 years or as long as is required. Treatment as necessary

Information on Repair Surgeries


12 Months – 5 Years

Cleft Unit Local Cleft Clinic Local Care Speech and Language Therapy

Assessment – This usually takes place at around 18 months. Treatment will be offered if necessary, but the need may not become apparent until the child is older.

o A further assessment takes place at around 3 years old, and treatment is recommended if it’s needed. Assessments for speech problems at this and later stages may involve a nasendoscopy or videofluroscopy.

Psychological Support for families if requested prior to school entry.

Paediatric Dentistry advice and/or treatment if necessary. This is available along the entire treatment pathway.

Full assessment by Cleft Team – a child with a cleft should receive a full assessment by all specialists in the cleft team at age 5, to ensure that any ongoing or emerging issues are being dealt with appropriately.

Surgery to revise lip, or speech (velopharyngeal) surgery if necessary. These are available throughout the entire pathway when needed.

Audiology assessment of children with a cleft palate at regular intervals up to and including 5 years (5 year assessment may be at Cleft Unit).

ENT assessment if necessary

Speech and Language Therapy – This will be provided by local therapists in consultation with the specialist from the Cleft Team. The aim is for all children to have good quality, intelligible (understandable) speech by age 5-6.


6 – 12 Years

Cleft Unit Local Cleft Clinic Local Care CLAPA’s Services Orthodontic Assessment

and Treatment – An orthodontist will monitor the position of the child’s teeth, including their adult teeth when they start to come through. Treatment may be needed to correct their position, which often includes fitting braces.

Speech and hearing checked and managed for children with cleft palate.

Psychological Support for families if requested prior to school transfer, or to help with any issues such as bullying or self-confidence.

Full assessment by Cleft Team at age 10, including for orthodontic treatment.

Alveolar Bone Graft (ABG) Assessment and Surgery

This involves taking a piece of bone from the hip and placing it in the gap left by the cleft so adult teeth can come through properly. Between the ages of 7 and 9, a child will be assessed by the team’s orthodontist, paediatric dentist and the surgeon responsible for ABG surgery to see if it is necessary.

If it is necessary, the preparation for the surgery as well as the surgery itself will usually be completed by 12 years of age, with a follow-up 6 months later.

Ongoing management of speech and hearing issues

Dental and Orthodontic treatment

Speech and Language Therapy from local therapists in conjunction with the specialist from the Cleft Team

Residential Weekends for 9-15 Year Olds

Private Facebook Group for under 17s

Children and Young People’s Council (CYPC) for 9-17 Year Olds


13 – 20 Years

Cleft Unit Local Cleft Clinic Local Care CLAPA’s Services Offer of genetic counselling for

patients interested in the causes of their cleft and how likely they are to pass it on to any children they have (‘hereditability’)

Full assessment by Cleft Team at ages 15 and 20 (if necessary).

Discharge from Child Cleft Services between ages 16-20, including final discharge appointment. Patients may be referred on to adult services to continue their treatment if necessary, or they can get a referral later in life from their GP or dentist.

Orthognathic Assessment and Surgery

Orthognathic surgery involves re-aligning the jaws to change the appearance of a person’s profile. This is usually offered to patients who have an underbite, and is usually completed after their face has finished growing at 16-18 years old.

There is a lot of planning, assessment and preparation involved with this surgery, including orthodontic care before and after. The Cleft Unit can offer more information.

Surgery as Required

Surgery may be required or requested for a number of different reasons, e.g. rhinoplasty (surgery to change the shape of the nose), dental surgery, lip revision, speech revision, palatal fistulae closure

Ongoing management of speech and hearing issues

Paediatric and restorative dental care in liaison with Cleft Unit

Ongoing orthodontic treatment

Residential Weekends for 9-15 Year Olds

Private Facebook Group for under 17s with a cleft

Children and Young People’s Council (CYPC) for 9-17 Year Olds

Adult Voices Council for 18+ Year Olds

‘Adult Voices’ Facebook Group for over 18s with a cleft

Peer Contacts

Information aimed at teenagers with a cleft

Information aimed at adults with a cleft


(closing small holes in the palate), etc.

21+ Years

Treatment as required

Adults may re-enter the cleft service at any time for a consultation about any aspect of their care, including psychological support, genetic counselling, and specialist dental treatment, all of which is available on the NHS if it is deemed necessary.

Adults can get a referral to the Cleft Unit from their GP or Dentist.

CLAPA’s Services

‘Adult Voices’ Facebook Group for over 18s with a cleft Adult Voices Council for 18+ Year Olds Peer Contacts Parent Contacts who have a cleft themselves

If you would like more information on different types of clefts, associated conditions, the treatment pathway and anything else not covered on this information pack please visit www.clapa.com


http://www.clapa.com/

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