Children – congenital or inherited conditions

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• Symptoms
• Treatment

The incidence of children being born with this condition increases with the age of the mother. In the UK, the NHS estimates that for example, a woman who is 20 has about a 1 in 1,500 chance of having a baby with Down's, while a woman who is 40 has a 1 in 100 chance. This study https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3746270/ estimates 37 090 people had Down's syndrome in England and Wales in 2011.

This year 1200 babies will be born with a cleft in the UK according to the Cleft Lip And Palate Association https://www.clapa.com

These children often have difficulties with facial and mouth functions. Common symptoms are for example difficulties in swallowing, difficulties in closing the mouth, drooling, or weakened musculature in the face and oral cavity. This affects the child’s face-, bite- and jaw development, and also the child’s speech ability.

In this section we will discuss several congenital or inherited diseases which usually exhibit symptoms and problems in the face’s and mouth’s functions. ^{[2, 6]} These difficulties need to be investigated fully in order to design an individual treatment plan where IQoro^® can be a complement; with the aim of reducing or eliminating such problems, and overall to contribute to a better quality of life for the child and its family. IQoro^® is a new and unique neuromuscular treatment method that requires just 30 seconds’ exercise, three times per day.

Difficulties with the mouth functions - common in children with the following diagnoses and abnormalities: ^[1–11]

• Down syndrome. ^[2–5]
• Cerebral palsy. ^[11]
• Hiatus hernia-like symptoms because of illness, or a delayed maturity in the digestive tract.
• Hiatus hernia, which may have been wrongly diagnosed as an unwillingness to eat, or anorexia in children and youths.
• Receding, short upper lip.
• Breathing through the mouth, or snoring.
• Dribbling or drooling.
• Indistinct speech.
• Abnormal face-, bite- and jaw development.
• Cleft palate. ^[1]
• Acquired injuries, birth injuries or sicknesses.
• Prader Willis-syndrome.
• Möbius syndrome. ^{[7, 9]}
• Sotos syndrome.
• Ehlers-Danlos syndrome.
• Angelmans syndrome.
• Chri-du-chat syndrome.
• Charges syndrome. ^[10]
• Congenital muscle dystrophy, myotonic dystrophy. ^[8]
• Brain haemorrhage, brain infarction.
• Cancer of the face, mouth, head or neck region.

Common symptoms, abnormalities and difficulties.

All children, whether healthy or diagnosed with a condition, that breathe through their mouths lose the contact between the rear of the tongue and the soft palate. This results in the gag reflex being dominant, and therefore makes it difficult to swallow food of solid consistency. The contact between the soft palate and the rear of the tongue is a precondition for an optimally functioning swallowing process, which normally begins with the child closing his or her lips.

As a rule, children that are born with a functional handicap, or are victims of an acquired injury or illness, often have a problem with the mouth’s function, with the following symptoms:

• Open mouth – difficulty in closing the mouth.
• Breathing through the mouth, and increased sensitivity to infections.
• Swallowing difficulties.
• Vomiting due to a dominant gag reflex, or a delayed maturity of the digestive tract.
• Eating- and chewing difficulties - extended mealtimes.
• Must be fed by enteral nutrition or by PEG.
• Reflux, acid indigestion, heartburn.
• Dry, or phlegmy cough.
• Over-relaxed, or slack musculature in the lips, tongue and jaws.
• Dribbling and drooling.
• Tongue protruding between the lips, or hanging over the lower lip.
• Indistinct speech.
• Delayed, or non-existent verbal ability.
• Difficulty in showing emotions via facial expressions.
• Abnormal teeth-, bite- and jaw development.
• Paralysis problems, e.g. in the face and pharynx.
• Function abnormalities in the esophagus.
• Facial asymmetry.
• Short tongue web lingual frenulum which hinders normal tongue movement and speech development.

Poor muscle tension gives rise to disabilities

A child’s functional impairment or inactivity as a result of congenital illness or an acquired injury or sickness, often results in a relaxation of various muscle groups in the body. This can cause various mouth- and face related, orofacial, difficulties.

Common challenges for children with such problems are indistinct speech, eating-, chewing-, and swallowing difficulties, which can lead to extended, time-consuming, mealtimes. Many children are advised to eat mashed food, and drink drinks containing a thickening agent in order to ease swallowing. It is not unusual that nutrition is provided via the nose or directly into the stomach through a so-called PEG Percutaneous Endoscopic Gastrostomy.

Difficulties with open mouth and protruding tongue ^[2–4]

Children, for example with Down syndrome, can have a problem with a protruding tongue as well as difficulty in closing their mouths, which also causes drooling and swallowing difficulties. The swallowing process begins with the lips closing and a low-pressure being created in the oral cavity. If a child cannot close his lips, it is difficult to begin to swallow. You can try this yourself; open your mouth a little and try to swallow – it’s difficult!

 Read more about:

“Swallowing difficulties – dysphagia, what is it?”

“The swallowing process – when it works as it should”

Weakened musculature affects the preconditions to be able to make speech sounds

Children that are suffering from relaxed, weakened and imbalanced musculature in the face and oral cavity are often also affected by a delayed verbal speech development. Weakened musculature in the tongue and lips, and abnormal tooth-, bite- and jaw development affect the physical and anatomical preconditions to be able to articulate clearly ^[2]. Another factor that can affect speech development is a short tongue web or lingual frenulum. This results in the child’s not being able to use the tongue properly: either at mealtimes, or for optimal speech development.

Not only a problem physically - but also psychologically and socially

It can be said that in general, apart from the physical and anatomical aspects of problems with the face’s and mouth’s functions, that the child is often affected psychologically and socially too. To not be able to eat and swallow normally, have a reduced ability to express one’s self verbally, suffer from drooling problems, and also have an abnormal appearance – for example with a cleft palate – often restricts an individual’s abilities in many social situations. Ultimately, this can create a problem that makes it more difficult to be integrated in society. These factors can affect not only the child's, but also the family’s quality of life^{. [2]}

Children – examining the face and mouth

It is not always easy to examine children with functional abnormalities in the face and mouth. These children can, partially because of their diagnosis or acquired condition, be reserved in contact with strangers, or feel a deep frustration that they cannot themselves describe their situation. Younger children, and children with understanding difficulties, may find it difficult to comprehend the reason for an examination of the mouth, bite and pharynx.

From playing to examining

It is very important, whatever the treatment, to approach children on their own terms. It should be added that all people with mouth-related problems can be sensitive to pain or find it unpleasant to have their faces touched at all. This is doubly true of children. To make an examination easier proceed as if it were a game, and with the cooperation of the parents. In this way children are tempted to make various movements on which the judgement and diagnosis can be based. An example is the tongue’s mobility: jam can be placed in the corners of the mouth and on the top and bottom lips, and the child will be tempted to lick it off.

The examination is important in deciding a treatment strategy

An examination is important to define the child’s difficulties; partially to establishing an individual treatment plan, but also in order to be able to establish a start position from which to measure the progress of the treatment.

If the child breathes through its mouth, snores, or often has its mouth open, it can be because the tonsils or the adenoids are enlarged. This makes it more difficult to breathe via the nose, and to swallow. Tonsils and adenoids should therefore be examined and judged, and possibly be the subject of an intervention, by an Ear, Nose and Throat (ENT) specialist.

Treatment strategy – for effective and optimal interventions

The treatment strategy can be thought of as a pyramid, where the ultimate goal at the top is to reach optimal nose breathing, and swallowing with a closed mouth. To reach the top of the pyramid it is essential to train the different muscles in the correct sequence. The body is all one complex unit and intervention can be required from many different professionals, for example doctors, dentists, speech therapists, speech pedagogues, physiotherapists, work therapists, nurses and others. The first stages cannot be avoided or skipped. See below:

1.  Postural control.

Aim for an optimal upright and balanced posture and sitting position for the child in a good chair, or wheelchair

2.  Chewing muscles

The strength of the chewing muscles is important to enable complete lip closure and to process the food in the mouth, and they are the only muscles that are active in all levels of the swallowing process. In addition, the chewing muscles stimulate the swallowing reflex.

3. Lip closure

Complete closure of the lips is a precondition for the tongue’s being able to take up its correct position against the roof of the palate, and for breathing through the nose. Lip closure is also essential to effectively transport food backwards towards the pharynx and to start the swallowing reflex.

4. The tongue’s backwards and upwards movements

The tongue moves the food to the right and left in the mouth and backwards towards the pharynx. The rear of the tongue’s, and the food bolus’ contact with the hard palate initiates the swallowing reflex. Immediately after this, the tongue’s forward movement starts and at the same time the epiglottis moves down and tightly closes the air pathway.

5. The soft palate and gag reflex

When it functions normally during swallowing, the soft palate should close tightly against the rear walls of the pharynx and up against the nasal cavity to prevent leakage of food to the nose. After this, the esophageal phase of the swallowing process is set in action. The contact between the rear of the tongue and the soft palate are important to activate the esophagus, stomach, intestines and other vital organs. The tongue’s contact with the soft palate is also important to oppose a dominant gag reflex: gaging or cascade vomiting, a dominant gag reflex makes it difficult to swallow solid consistencies.

6. Breathing through the nose, and swallowing

Breathing through the nose and a closed mouth are essential for a safe and effective swallowing ability.

Treatment with IQoro^® can improve the mouth’s functions

IQoro^® is a new and unique neuromuscular treatment method that requires just 30 seconds’ exercise, three times per day. IQoro^® acts on and regenerates the entire swallowing process. It stimulates the sensory nerves in the mouth, and by doing this reaches the brain’s control system for the swallowing process. This control system is closely linked to the control systems for other bodily functions, including breathing, the ability to make speech sounds, facial expression, postural control, stomach and intestine functions, and more. IQoro^® activates the body’s own pre-programmed systems – a natural chain of nerve pathways and muscles from the face, oral cavity, pharynx, esophagus, and down to the diaphragm. Our basic, vital functions of breathing, eating, speaking and smiling use the same muscles and nerves – but in different ways. This explains why exercising with IQoro^® can have a positive effect on so many different functions.

In total this creates good conditions for improvements for children with congenital or acquired sicknesses as described above under,”Difficulties with the mouth functions - common in children with the following diagnoses and abnormalities”.

It is important to emphasise that a congenital sickness, or one acquired at birth or later, cannot be cured by training with IQoro^® On the other hand many of the symptoms that arise from these conditions can be lessened or eliminated. The success in doing this is also dependent on the degree of support offered by the health care services.

Exercising with IQoro^® has the following effects:

• Optimises eating, chewing and swallowing ability.
• Strengthens the musculature in the chewing muscles and lips, which counters drooling.
• Enables proper lip closure.
• Improves the preconditions for distinct speech.
• Gives improved symmetry and mobility in a face which was previously slack or paralysed.
• Optimises the preconditions for normal bite-, jaw- and face development, as the training creates improved muscle strength and balance in the function between the tongue, cheeks, lips and pharynx.
• Encourages the tongue to develop a natural backwards- and upwards movement towards the roof of the palate. The tongue takes up a normal position in the mouth and does not protrude: which is a precondition for a normal development of the roof of the palate and the middle section of the face.
• Improves the contact between the tip of the tongue and the soft palate, which is essential for activating the brain nerve Vagus, which controls all the body’s inner organs except the sex organs. This is also important for activating the musculature in the pharynx, esophagus and stomach. It can also reduce the problems of infrequent bowel movements. Finally, the contact between the rear of the tongue and the soft palate is important to counter the development of a dominant gag reflex.
• Children with extended and complicated mealtime situations that require modified, pureed food, or nutrition delivered nasally or via a PEG, can achieve an improved, or normal ability to eat via the mouth.

Improvements in some or more of the above described areas can contribute to an increased physical capacity and an improved quality of life. Every small degree of improvement can contribute to the child developing his or her independence.

This is how you exercise with IQoro^®

IQoro^® - just as effective for otherwise-healthy
children with eating, speech and swallowing difficulties

It is important to emphasise that IQoro^® is, of course, just as effective for children that are otherwise healthy who, for reasons other than congenital or acquired illnesses, have a problem with their eating, speaking or swallowing ability. It can be worth pointing out that a healthy child, or indeed an adult, with eating and swallowing difficulties far too often have their symptoms classified as an eating disorder caused by psychological issues, when the real issue has been esophageal dysphagia caused by a hiatus hernia.

Read more about hiatus hernia here

When can a child begin to train with IQoro^®?

Treatment with IQoro^® can begin soon after a child’s first birthday. IQoro^® in its ’small’ size suits children between one and a half and 12 years old and has the same form and function as the adults’ model.

The treatment period for children will vary depending upon the severity of the condition and may be necessary through all the growing years.

Create safe and secure conditions for training

To get good acceptance of the treatment method it is important that those that help the child: parents, speech therapists, assistants, etc. allow the child from the start just to get familiar with its IQoro^® - to feel it, to taste it. Dipping it in something tasty that the child can suck or lick off can ease the introduction. Playing at training can help, for example by hiding the IQoro^® inside the lips, or having a play ’tug-of-war’ together, preferably in front of a mirror. In this way you can create safe conditions for the child to become used to IQoro^® at his or her own pace.

When the training routine is established, it is important that exercising with IQoro^® becomes a natural part of the daily routine. The child should exercise, with or without help, three sessions per day as described in the manual.

This is how you train with IQoro^®  – see the film

IQoro^® – long clinical experience

Research with the IQoro^® treatment method has not been specifically carried out on children. However, Dr. Mary Hägg has many years of clinical experience stretching back to 1979, of treating children from premature birth to late teenagers with both common, and less well-known conditions.
 

Research shows that IQoro^® acts on, and regenerates, the entire swallowing process. It stimulates the sensory nerves in the mouth, and by doing this reaches the brain’s control system for the swallowing process, which is closely linked to the control systems for other bodily functions^[13], including breathing, the ability to make speech sounds, facial expression, postural control ^[12], stomach and intestine functions, and more. This explains why exercising with IQoro^® can have a positive effect on so many different functions.

Read more about ”Why IQoro^® works” here

 

IQoro® makes a difference for a chronically ill patient

Maja Eriksson, 22 years old, from Gävle in Sweden suffers from Myotonic Dystrophy, a chronic illness which in many ways is reminiscent of ALS (Amyotrophic Lateral Sclerosis - sometimes called Lou Gehrig's disease). After years of aching muscles, tiredness and periods of swallowing difficulties, Maja at last got a diagnosis.  In her fight to maintain her muscle strength the neuromuscular trainer IQoro® has made the difference in Maja’s everyday life.
 

 Read patients' own stories here

 

Matteus -  a contented 10 year old

Matteus and Arion Hylander, 10 years old, are twins, but not especially like each other. This is not only because they are non-identical twins, but because Matteus was born with Prader Willis Syndrome which makes circumstances for the two brothers look very different. However, Matteus’ life now looks fairly positive, partially because he has got help with oral training from the Speech- and Swallowing Centre at Hudiksvall hospital in northern Sweden.
 

 Read patients' own stories here

Text by:

Dr. Mary Hägg, Doctor of Medicine, Post-doctoral researcher at Uppsala University specialising in orofacial medicine, Head of Department at the Speech and Swallowing Faculty: Ear, Nose and Throat Clinic at Hudiksvall Hospital, Sweden. Registered Dentist. In cooperation with MYoroface.

October 2016. (Translation from the original Swedish text by T.W.Morris, MYoroface).