Special needs

Question 1

what is Down syndrome ?

Answer 1

• Most frequent genetic cause of a learning disability.
• Error in cell division resulting in an additional third chromosome 21 (trisomy 21)
• Trisomy 21 – 92%
• Mosaic (mixture of some cells with 3 and some a pair) Trisomy 21 – 2-4%
• Translocation (where one chromosome breaks off and watches to another) Trisomy 21 – 3-4% (carries increased likelihood of DS in future pregnancies)

Question 2

what are the characteristics of someone with downs syndrome?

Answer 2

 Short Stature
 Bradycephaly
 Widely spaced eyes
 50% have a congenital heart defect
 Ten-fold increased risk than the general population of
developing a seizure disorder
 Increased risk of early onset Dementia or Alzheimer’s
Disease
 Atlantoaxial instability – meaning the neck must be handled (should reconsidered when on dental chair)
correctly as there is a risk of spinal cord depression
 90% have hearing deficits
 Children with DS are 15 more likely to develop Leukaemia
 Multiple immune defects putting them at increased risk of
developing periodontal disease.
- delay in wound healing

Question 3

what is fragile x syndrome ?

Answer 3

• affects 1 in 1500
• second most common genetic defect associated with learning disabilities
• tip of the X chromosome is fragile and susceptible to breakage
• sex linked dominant trinuleotide repeat disorder on fmr1 gene
• other eg are Huntingtons disease and freidrichs ataxia

Question 4

what are the characteristics of someone who has fragile X syndrome ?

Answer 4

• learning disability
• long face
• prominent ears and forehead
• hypotonia
• high arched palate/ malocclusions
• large testes
• seizures in 10% hyperactivity and attention span disorders eg. ADHD or autism in 1:3
• behavioural disorders

Question 5

what is Williams disorder ?

Answer 5

• A rare genetic disorder affecting 1 in 18,000 people in the UK.
• Deletion of elastin gene on Chromosome 7
• Non-hereditary genetic abnormality
• Commonly associated with a LD

Question 6

what are the characteristics of Williams syndrome?

Answer 6

 Facial characteristics: wide mouth, pronounced bottom lip, flattened nasal bridge, high rounded cheeks, irregularly spaced dentition, starburst eyes.
 Commonly have a congenital heart defect (narrowing of the aorta)
 Delayed growth and failure to meet development targets in life.
 Excessive talking and hyperactivity with uninhibited behaviour and over friendliness with strangers including a compulsion to talk to adults (often inappropriately).
 Exaggerated reactions to happiness/sadness/fear.
 Can develop depression/mental health issues in later life
- dentally = small mouth and teeth
- can have inappropriate convos with adults

Question 7

what is triple x syndrome ?

Answer 7

• Trisomy X or XXX Syndrome where females have an extra chromosome X.
• Is relatively common affecting 1 in 1000 live births in the UK
• Many don’t have symptoms or will have mild symptoms and will go on to lead a normal life.

Question 8

what are some symptoms of triple x syndrome?

Answer 8

 Delayed speech development and motor skills, failure
to meet developmental targets
 Learning Disabilities of varying degrees.
 Behavioural problems such consistent with ADHD or
Autism
 Mental Health issues are common such as depression
and anxiety
 Epicanthal folds with upward slanting palpebral
fissures (as in DS).
 Classic features: Hypertelorism, Tall, Early menopause,
weak muscle tone, dental abnormalities.
 Increased risk of developing sleep apnoea, asthma,
scoliosis, lupus and Sjogren’s.

Question 9

what is prader will syndrome ?

Answer 9

 Genetic Condition related to partial chromosome 15 deletion (paternal).
 Prevalence in the UK is estimated to be between 1:10-30,000

Question 10

what are does of the symptoms of prader willi syndrome?

Answer 10

 Most will have mild to moderate learning disabilities.
 Hyperphagia (over-eating/insatiable appetite) resulting in diabetes and obesity.
 Mental Health issues such as temper outbursts, stubbornness, compulsive behaviours, controlling and manipulative behaviour, have difficulty changing their routine.
 Delayed development and poor growth as a result;
short stature, small hands and feet.
 Poor muscle tone, feeding difficulties as a child
 Narrow forehead and almond shaped eyes with a
triangular mouth
- because theyre obese may have to refer to bariatric dental service
- if they have diabetes may be more prone to perio, post extarction healing

Question 11

what are some dental aspects to consider that re relevant to DS?

Answer 11

Question 12

dental summary for DS

Answer 12

Question 13

what are the 5 principles of the MCA

Answer 13

1. assume capacity (unless they lack)
2. make reasonable adjustments (make things easier for them to understand eg. removing jargon)
3. unwise decisions (just because they make unwise decisions doesn’t mean they lack capacity)
4. best interests decision ( the decision must be in the pts best interest)
5. least restrictive ( managing the pts care in the least restrictive way possible eg. instead of using GA where they are fully unconscious give LA where they are fully conscious however if the pt lacks capacity and has severe learning disabilities and GA is the only way you can safely provide care

Question 14

what test do we do to assess capacity ?

Answer 14

2 stage test

1) do they have an impairment or disturbance in the functioning of the mind or brain

2) is this affecting their ability to
a) Communicate
b) Understand
c) Retain
d) Evaluate

Answering Yes to any of the above (a-d) indicates a lack of capacity

Question 15

what is learning disability ?

Answer 15

• combination of an IQ below 70 and a difficulty with performing everyday activities such as:
  ▪ Household Tasks
  ▪ Managing Money
  ▪ Socialising
  ▪ Self-care
  ▪ Developing new skills
  A learning disability is lifelong meaning it will affect that person for their whole life.

Question 16

what is a learning difficulty ?

Answer 16

Learning difficulty does not affect intellect and is not necessarily lifelong, it also does not hinder performance of
everyday activities. Examples include:
▪ Dyslexia
▪ Dyspraxia
▪ ADHD
▪ Dyscalculia

Question 17

what is a mild IQ?

Answer 17

IQ between 69-50 - many adults can maintain good social relationships and contribute to society

Question 18

what is a moderate IQ?

Answer 18

IQ between 35-49 (in adults mental age is from 6-9 years old) – marked developmental delays in childhood but most are able to develop some degree of independence with self-care and maintain adequate communication/academic skills but may need varying degrees of support in order to live and work in the community.

Question 19

what is a severe IQ?

Answer 19

IQ range from 20-34 (in adults mental age is from 3-6 years old) – they will need continuous support with all aspects of life including varying degrees of limitation in communication and self-care

Question 20

what is a profound IQ?

Answer 20

IQ range of <20 (in adults mental age is <3 years old) – they will have a severe limitation in self-care, communication,
mobility and continence.

Question 21

what are the causes of learning disabilities ?

Answer 21

• disruption of brain development either during pregnancy, labour/ childbirth or in early childhood

eg:
- Maternal illness in pregnancy
* Hypoxic injuries during child-birth
* Genetic disorders/conditions e.g. Trisomy 21
* Illness or injury in early childhood

Question 22

LD stats ?

Answer 22

Question 23

list some common conditions associated with a learning disability

Answer 23

• Downs Syndrome
• Williams Syndrome
• Autism
• Fragile X Syndrome
• Global Developmental Delay
• Cerebral Palsy
• Challenging Behaviour

Question 24

what is autism spectrum disorder?

Answer 24

• persistent difficulties with social communication and social interaction” AND
• “restricted and repetitive patterns of behaviours, activities or interests” (this
  includes sensory behaviour) AND
• present since early childhood, to the extent that these “limit and impair everyday
  functioning”.
  Autism is a Spectrum Disorder, meaning the severity can vary from person to person.
  Also important to note is that not everyone with Autism will necessarily have a learning disability.

Question 25

what is the autism traid?

Answer 25

1. Non-Verbal and Verbal Communication – literal interpretations of language with difficulty communicating with others and communicating their own thoughts.
2. Social understanding and Social behaviour – difficulty with empathy,
   dealing with and understanding social situations and inappropriate social
   responses.
3. Imagination and thinking/behaving flexibly – difficulty in coping with a
   change to routine, may pursue singular interests obsessively, excellent
   memory with regards to their own interests, prefer solitary play as they
   struggle with imagination.
   Additionally, may be sensitive to sounds, smells, tastes and textures.

Question 26

stats of autism in uk?

Answer 26

Question 27

what is ADHD?

Answer 27

• a persistent pattern of inattention or hyperactivity and impulsiveness that has a direct impact on daily living.
• In the UK, ADHD is recognised as a long-term health condition and therefore falls under the Equality Act (2010). This means reasonable adjustments should be made so that they are not placed at a disadvantage to receive dental care in comparison to a non-disabled person.

Question 28

characteristics of ppl with ADHD ?

Answer 28

▪ Careless mistakes and fails to give full attention when performing tasks.
▪ Seems to not be listening when spoken to directly.
▪ Trouble organizing tasks and activities.
▪ Easily distracted and forgetful.
▪ Often loses thing that are needed for activities (e.g. in school, pens, pencils,
books etc).
▪ Often fidgets, taps, squirms, inability to sit still.
▪ Talks excessively, interrupts or struggles to wait for their turn.
▪ May display challenging behaviours.

Question 29

what are some of the dental considerations when dealing with someone with ADHD?

Answer 29

• May display inappropriate behaviours, disruptive behaviours or need
  pharmacological adjuncts to enable dental treatment.
  ▪ Try to arrange appointments within their daily schedule and minimize disruption to routine.
  ▪ Avoid keeping them waiting, keep appointments short and use simple short sentences.
  ▪ LA is usually safe but avoid adrenaline if they are taking tricyclic antidepressants
  or monoamine oxidase inhibitors as there is a risk of tachycardia or prolonged hypertension.
  ▪ Inhalation sedation is generally safe, however intravenous sedation may cause further disinhibition of behaviours and not be successful

Question 30

which pts fall under special care dentistry?

Answer 30

Question 31

what is SCD?

Answer 31

Question 32

why is SCD important ?

Answer 32

• most need adjustments to be Abel to accept dental care
• eg. longer appts, being hoisted on dental chair, having things explained in different lang/ format, needing additional support in decision making etc
• GDP sometimes may not be enough for safe management

Question 33

what is community dental service ?

Answer 33

• usually based in multiple clinics within a defined region
• has additional equipment eg. hoists, wheel chair tippers, bariatrics, banana boards, other mobility aids
• combination of paeds and SCD
• provide care under NHS and CBT, acupuncture, sedation and GA