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Children’s Health – Struggling at school
Guidance and Resources – Struggling at school
In case Children’s Health – Struggling at school
Guidance
This week the structured cases surround the assessment and management of children and young people presenting with alterations in their conscious level, behavior and development. You are expected to review normal developmental milestones in childhood in order to refine your skills in identifying departures from the usual pattern, and to identify key points in the History, Examination findings and Red Flags which may indicate significant neurological and developmental conditions.
You will be encouraged to explore Differential Diagnoses for common neurological presentations from infancy to childhood with key symptoms including seizures, depressed conscious level, developmental delay and concerns regarding behaviour and learning.
You are expected to complete the online material prior to attending the Themed Case Discussions in order to be able to apply your learning during the teaching.
By working through this case you will:
· Develop the ability to obtain clinical information via history taking focussing on development and learning in a young child and to review relevant factors from the wider history
· Develop skills in the neurodevelopmental assessment of a school aged child and spotting red flags for neurological and developmental disorders.
· Describe the epidemiology (including disease occurrence and risk estimates), clinical presentations and prognoses of common neurological disorders and disorders affecting development, and understand how these may differ with time, place and person (including global health perspectives).
· Apply knowledge of the epidemiology, basic science, pathophysiology of common neurodevelopmental presentations in childhood and show the ability to synthesise appropriate differential diagnoses.
· Synthesise clinical findings from the history and examination and apply clinical reasoning processes to generate differential diagnoses of neurodevelopmental symptoms in childhood.
· Understand the scientific bases of appropriate clinical investigations to diagnose and manage neurodevelopmental symptoms in childhood.
· Detail emergency initial management steps and when to seek help for index neurodevelopmental symptoms
· Formulate, in conjunction with patient and, if appropriate, other health care professionals, plans for treatment and management, and recognise the impact on the patients, immediate family or carers.
· Communicate effectively with patients and, when appropriate, relatives about the nature of their illness, management options and prognosis taking into account the need for shared decision making and issues of capacity and consent.
eBooks
Illustrated Textbook of Paediatrics 4th edition. Lissauer (Mosby Elsevier)
Textbooks
Nelson Essentials of Pediatrics:, Karen Marcdante MD, Robert M. Kliegman MD
Web Links
These free US resources from the Centre for Diseases Control provide very useful revision regarding normal development and information for families:
Developmental Milestones – CDC
We recommend you also explore the additional and diagnostic features for common developmental disorders on this resource.
A review article on the care and outlook of premature infants:
Premature newborn care – BMJ Best Practice (open through university library) WWW.elibrary.taibahu.edu.sa/taibah
Assessment of learning difficulty and cognitive delay – BMJ Best Practice (open through university library) WWW.elibrary.taibahu.edu.sa/taibah
Global Developmental Delay – Contact a Family
A review article regarding the identification of inherited metabolic disease in childhood:
Developmental delay: when to suspect and how to investigate for an inborn error of metabolism – BMJ Journals (open through university library) WWW.elibrary.taibahu.edu.sa/taibah
CASE COMPONENT
Case Introduction – Struggling at school
In case Children’s Health – Struggling at school
Daniel is an 8 year old boy who has recently moved into the area, you are a foundation doctor and see him in Community Paediatrics clinic as his mum is worried that his school performance is deteriorating.
Mum explains that he has a complicated background history and has cerebral palsy. He has been having lots of difficulties at school recently.
Mum tells you that Daniel has been under the Paediatric teams in Surrey after a very difficult birth – he was born prematurely at 26 weeks and spent 15 weeks in the neonatal unit in the Royal Leafy Hospital in Surrey. He was diagnosed with Cerebral Palsy when he was 2 ½ and had started to make progress but since moving to the North West, he has been having a lot more difficulties especially with his behavior and doesn’t want to go to school. She feels he needs more help.
You decide that Daniel’s history is likely to be complex and as a starting point you need more information and to explore his current difficulties.
List three areas you would like to enquire about in turn:
1. Specific aspects of his behavior
2. His Learning / schooling
3. His Mobility
You could also ask about his activities of daily living, attention, relationships with others, attendance at school and previous development.
You could also ask about his activities of daily living, attention, relationships with others, attendance at school and previous development.
Why is it helpful to use this structured approach?
This allows parent and child to express their concerns in their own words and for you to identify the most pressing issues for them. (ICE Approach)
To identify these key issues and discuss them in detail in turn helps each symptom to be explored in detail. Where it is possible for one diagnosis to explain multiple symptoms on some occasions, in patients with complex of lengthy histories an approach using a problem list is helpful to ensure that each concern is addressed.
Within the consultation - It helps to structure discussions.
At the end of the consultation - It facilitates summarizing and the generation of action plans in a systematic manner.
Which questions could you ask in these areas, and to whom?
To Daniel: Ideally initial questions should be directed to Daniel asking about
1. Any concerns he has about school; what are his like and dislikes?
2. His friends.
3. What are the things he finds easy, then are there any things he finds difficult?
To Mum:
1. What are your main concerns about his behavior? Please give some examples.
2. What have school reported to you regarding his progress? What support is he receiving at the moment?
3. Does he have any mobility difficulties at present? What additional support is in place at the moment?
It is important to ask as open questions as possible initially to allow Daniel and mum to express her concerns, you are looking to develop a picture of his strengths and difficulties in this way with significant amounts of information likely to be given freely.
You will then need to explore specifically aspects of the information you are given.
In discussions of children’s difficulties and behaviour it is very important that the consultation does not represent a list of complaints about him. This can potentially disturb his ability to engage with professionals and is particularly the case during adolescence.
Should Daniel not wish to talk to you, explaining how much his views and experience are important in trying to address some of these issues, then returning later in the consultation to this may be helpful.
Why is it important to involve Daniel in the consultation?
In discussions of children’s difficulties and behaviour it is very important that the consultation does not represent a list of complaints about him. This can potentially disturb his ability to engage with professionals and is particularly the case during adolescence.
Should Daniel not wish to talk to you, explaining how much his views and experience are important in trying to address some of these issues, then returning later in the consultation to this may be helpful.
History Given:
The key phrases which are helpful in guiding your reasoning regarding the causes of Daniel’s difficulties are highlighted in bold.
Mum states that Daniel has always had difficulty in new situations and He often says he doesn’t want to go to school. he hasn’t really made any friends.
Mum says that school have requested support for him as he has been falling behind in reading and maths, and had been keeping up before. He had been having one to one support in his previous school and is awaiting an Educational Healthcare Plan to be completed at his current school
Daniel’s cerebral palsy affects his legs. Daniel does very well with his mobility, he usually walks but tends to walk on his toes and gets very tired, he often falls. He has been using a wheelchair for long distances. His mum is not concerned about his writing.
The symptoms specifically surrounding his motor development and relating to the previous diagnosis need to be explored further to ensure they are consistent with the given diagnosis.
His progress is particularly important although it would seem that he is making progress; any definite loss of skills may represent developmental regression; this is a red flag for a serious degenerative or destructive condition warranting urgent investigation.
Differential diagnoses for Daniel’s motor difficulties:
· Cerebral palsy affecting the lower limbs; spastic diplegia; this is the most likely given the information surrounding his birth.
· Disorders of muscle have to also be considered as at this stage it is not clear whether his difficulties with motor function are due to a loss of power and tone or imbalances.
The VITAMIN D approach may be helpful; some of these areas will highlight risk factors or underlying causes for brain injury and cerebral palsy; rarer conditions such those in the neurometabolic group have to be considered is if there are features of deterioration.
Please list at least 3 aspects of his past history which may be helpful?
· Pregnancy History; any indication of infection or placental dysfunction, drug or alcohol use
· Birth history; prematurity of difficult delivery
· Postnatal health and progress
· Developmental history
Please read through the following additional history:
Daniel was born by emergency Caesarian section at 26 weeks gestation following antepartum haemorrhage. He was initially very ill and was ventilated for 10 days. He was then on Nasal CPAP for 2 months and oxygen for a further 2 months and was discharged from hospital just after his original due date. He was feeding well.
Mum was told when he had ultrasound scans of his head that small bleeds were present in the fluid spaces of the brain.
He was seen in clinic for monitoring after discharge; he sat up alone when he was 15 months and walked by himself at 2 years 4 months; he walked on his tiptoes and she remembers that he had been stiff and had exercises to do which seemed to help. He talked quite late and after going to nursery by 3 years had made progress, talking in short sentences, he could feed himself and liked to play with toys pressing small buttons and with mum’s phone.
Mum remembers being devastated when she was told that he had cerebral palsy.
He had an MRI of his brain under sedation that showed Periventricular Leukomalacia.
Looking at the history above, what contributing causes for Cerebral Palsy can you identify?
‘antepartum haemorrhage’; causing fluxes in fetal circulation
‘born at 26 weeks gestation’ ‘initially very ill and was ventilated for 10 days’; this would suggest a degree of instability in cardiorespiratory systems.
‘small bleeds were present in the fluid spaces of the brain’.intraventricular haemorrhage supports the fluxes in circulation in the perinatal period.
‘Periventricular Leukomalacia’ - this is defined as cystic change occurring in the periventruclar white matter
Please follow the link below and focus on the flow diagrams that detail:
· Risk factors for development of cerebral palsy
· Pathogenesis of priventricular leukomalacia (PVL)
Cerebral Palsy – McMaster Pathophysiology Review
CASE COMPONENT
Further Case Information – Struggling at school
In case Children’s Health – Struggling at school
You are aware that to assess Daniel’s educational ability in a clinic setting is challenging, please list at least 2 ways you could approach this?
1. To ask Daniel and his mother regarding his strengths and difficulties.
2. To ask mum if she has any previous reports regarding Daniel’s progress either from his previous school and the current school.
3. To ask mum and Daniel to consent to contacting the school directly to request a formal report; further information could also be formally requested regarding his behavior and interactions with other children.
4. Given there are several needs to be explored the school nursing team may assist in gathering together health and education information.
5. There are concerns expressed about attendance and Daniel not wanting to go to school, he may be at risk of school refusal; the school attendance team and school nursing team will have access to his attendance records.
Daniels mother mentioned in addition that he did not want to go to school; prior to discussing this in more detail with mum, you review your knowledge regarding poor school attendance.
Background Science – Struggling at school
In case Children’s Health – Struggling at school
Please revise your functional neuroanatomy of the motor and sensory cortex and the cerebral circulation.
Please revise the difference between the physical signs in upper and lower motor neurone lesions.
Please review the history given up to this point again. What is your assessment of Daniel’s early development in the four areas?:
· Gross motor
· Fine motor/vision
· Hearing/speech
· Social
List additional questions you would ask in each of these areas.
Gross Motor:
Sat alone at 15 months and walked at 2 years 4 months; even corrected for 14 weeks (3 ½ months) of prematurity, represents gross motor delay.
Additional question: What prompts the use of the wheelchair?
Fine motor/ vision:
As a toddler he was able to feed himself and described as liking toys with buttons mum’s phone. Not suggesting fine motor or visual difficulties. Information from the initial history of no concerns regarding his writing, delay in this area is not likely.
Additional question: Has he had a visual assessment? Has he got a squint?
Hearing/speech:
Speaking in sentences by the age of 3 years represents a degree of speech delay; a hearing assessment would be useful, this represents mild speech delay.
Additional question: Has he had a hearing assessment? Describe his early speech.
Social:
Daniel showed some early self care skills; and mum expressed no concerns about his early social development.
Additional question: Describe his early speech, and interaction including eye contact.
As mum has expressed concerns about his current social skills therefore further assessment of his social skills will be needed
In conclusion:
Daniel has early gross motor developmental delay and some concerns regarding his early speech and language development. Further information and formal assessment may be necessary.
What is the definition of global developmental delay?
A child has global developmental delay if they have a significant delay in milestones in two or more areas (2 or more standard deviations below the mean).
What are the main causes of profound disability?
· Chromosomal abnormalities; genetic factors including micro-duplications and deletions are being increasingly identified as important in this group of children
· Cerebral Palsy,
· Inborn errorrs of metabolism
Please list at least five effects of profound disability on the different body systems?
People with profound and multiple learning disabilities: all areas of their daily life are likely to be affected; they may have:
· have more than one disability
· have a profound learning disability
· have great difficulty communicating
· need high levels of support in all activities of daily living
· may have additional sensory (vision and or hearing) needs
· may have an inability to protect their airway and be at risk of recurrent chest infections.
· May be unable to regulate their own intake require gastrostomy nasogastric or other interventions.
· may have challenging behaviours and mental health difficulties
are at greater risk of other neurological disorders such as epilepsy and early puberty for example.
Which other professionals may be involved in a care package for the family?
· Speech and language therapist
· Physiotherapist
· Occupational therapist
· Neurologist
· Community Paediatricians
· Community nurses
· Early support needs team
· ENT and audiology
· Ophthalmologist
Please review the following article regarding cerebral palsy and answer the following questions:
Managing common symptoms of cerebral palsy in children – BMJ Clinical Review (open through university library) WWW.elibrary.taibahu.edu.sa/taibah
What is the definition of Cerebral Palsy?
A disorder of tone, posture and movement, caused by a non-progressive brain lesion in a developing brain
Can you think of differing types of Cerebral Palsy?
· Spastic – most common presentation – upper motor neuron findings on clinical examination.
· Ataxic
· Dyskinetic (also called choreo-athetoid)
In reality many children with cerebral palsy have a mixed clinical presentation.
With regards to cerebral palsy, which of the following statements are TRUE?
A) The neurological functioning of the child deteriorates steadily over time.
B) Intellectual development can be predicted by the degree of physical disability.
C) In most children with cerebral palsy, the cause is post-natal
D) Prematurity and low birth weight are important risk factors for cerebral palsy
· The neurological functioning of the child deteriorates steadily over time.
. a common definition used is:
‘the term used for a group of nonprogressive disorders of movement and posture caused by abnormal development of, or damage to, motor control centers of the brain. CP is caused by events before, during, or afterbirth. The abnormalities of muscle control that define CP are often accompanied by other neurological and physical abnormalities.'
The key information part of this definition is that the damage / lesion in the brain is non-progressive; the manifestations of the changes in muscle tone and function can change over time due to the evolution of spasticity in the first year of life then a temporary increase in the imbalance of muscle tone surrounding a joint and therefore a temporary deterioration in gait during growth spurts for example
· Intellectual development can be predicted by the degree of physical disability.
Marked effects can occur on the motor cortex due to vascular instability as this is supplied in the ‘watershed’ zone, without global effects on the brain, therefore children and adults with severe disability may not uncommonly have normal intellect. It is therefore essential that each child and adult is treated and assessed as an individual within consultations and by the multidisciplinary team to tease out strengths and difficulties and allow communication and independence where possible.
· In most children with cerebral palsy, the cause is post-natal
It is thought that the majority of children with cerebral palsy have a prenatal cause but commonly a clear cause cannot be identified althoufgh this is improving with improved imaging techniques.
· Prematurity and low birth weight are important risk factors for cerebral palsy
.Correct answer.
The most common abnormalities of muscle tone is spasticity
What do the terms hemiplegia, diplegia, quadriplegia mean?
Hemiplegia – unilateral arm and leg involvement
Diplegia – symmetrical paralysis (may be of the legs, arms, face or combined thereof)
Quadriplegia – all four limbs involved.
Can you think of any medications which can be used to help manage increase tone in children with cerebral palsy?
· Baclofen
· Diazepam
· Botulinum toxin
Examination:
You carry out a full General and Neurological examination. Why would you do this?
Examination focus; looking for physical signs to confirm the diagnosis of cerebral palsy and to look for any evidence of complications or an additional underlying cause for his difficulties, in particular any signs of an underlying genetic cause. In addition, contact with children is regarded as a health screening and monitoring opportunity.
Your findings are as follows:
Well looking 8 year old boy;
Height 128cm; Weight 27kg;
Head circumference 53.5 cm (50th-75th centile)
No dysmorphic features noted
Transient eye contact and good interaction with mum, responds appropriately speech fluent.
HS normal; pulses normal; BP 96/62
Resp; Chest clear, no Harrisons sulci, no scoliosis
Abdomen soft non-tender no organomegaly; palpable hard stool in the left iliac fossa
No cranial nerve abnormalities, no squint
Peripheral nerves; tone;
Upper limbs normal; power 5/5; reflexes present
Lower limbs: increased tone with ankle clonus bilaterally, ankles dorsiflex just to the neutral position. Power 4/5 in flexors and extensors. Reflexes increased bilaterally. Plantars upgoing. No calf hypertrophy noted
Gait is observed: tendency to tiptoe walk, with an equinus gait, with circumduction, slight flexion of the knee and hip and increases adduction of the knees with a degree of scissoring. The trunk rotates slightly with each step.
What is the significance of calf hypertrophy?
With a history of mobility difficulties and tiptoe walking a neuromuscular disorder such as Duchenne or Becker muscular dystrophy is a possibility. Calf hypertrophy with proximal muscle weakness would be present with difficulty rising from a sitting.
Watch the short video below for more information:
https://youtu.be/0htikR11nU8
This resource discusses DMD in detail with a genetic focus. The website is also useful as a link for information on other conditions:
Duchenne muscular dystrophy - Genomics Education Programme.
Regarding muscular dystrophy, which of the following statements are TRUE?
· Boys with Duchenne muscular dystrophy often present with other neurological signs.
· Duchenne muscular dystrophy is the most common muscular dystrophy in northern Europe and is due to an abnormality of the dystrophin gene.
· A young man with Duchenne muscular dystrophy can pass the disorder to his son.
· Steroid treatment has been shown to delay the progression of the disease to retain mobility longer in Duchenne Muscular Dystrophy.
· The initial diagnostic test for muscular dystrophy is serum creatinine.
· Boys with Duchenne muscular dystrophy often present with other neurological signs.
.Correct answer.
Speech delay and delay in walking with tiptoeing gait is common prior to the development of perceptible weakness therefore muscular dystrophies need to be considered in any child presenting with developmental abnormalities.This is because various isoforms of the dystrophin protein are expressed in brain tissue.
· Duchenne muscular dystrophy is the most common muscular dystrophy in northern Europe and is due to an abnormality of the dystrophin gene.
.Correct answer.
The dystrophin protein provides a structural link between muscle fibres, lack of dystrophin leads to progressive deterioration of muscle structure and it is also likely that dystrophin is involved in intracellular functioning. The dystrophin gene is carried on the X chromosome.
· A young man with Duchenne muscular dystrophy can pass the disorder to his son.
Duchenne muscular dystrophy is an X-linked recessive condition as this is carried on the X chromosome and a father passes a Y to his son to make him male a father cannot pass the condition to his son.
· Steroid treatment has been shown to delay the progression of the disease to retain mobility longer in Duchenne Muscular Dystrophy.
.Correct answer.
This has delayed the loss of walking significantly (on average by 2 years) but is not curative and side effects remain problematic.
· The initial diagnostic test for muscular dystrophy is serum creatinine.
Creatinine Phosphokinase or CK is the investigation of choice in addition to genetic testing.
Can you think of any longer term problems caused by a progressive muscle weakness problem such as DMD?
· Reduced mobility, loss of ambulation
· Scoliosis – spinal surgery will be needed if scoliosis significant
· Feeding problems – may cause malnutrition – gastrostomy feeds may be required
· Cardiac involvement (depending on condition, but common in DMD)
· Respiratory weakness- nocturnal non-invasive ventilation may be required
What are the possible side-effects of steroids in children?
· Increased appetite – with weight gain
· Behavioural deterioration – especially if pre-existing problems
· Hypertension
· Growth failure
· Gastrointestinal irritation
· Bone thinning
Please give examples of other conditions inherited in this manner (X linked conditions).
· Haemophilia A&B
· colour blindness
Case Conclusion – Struggling at school
In case Children’s Health – Struggling at school
Patient: Daniel Smith – NHS No. 2689033376
Attends: All Saints Primary School, Bolton
Dear Ms Smith
Problems:
1. History of prematurity at 26 weeks (born in Surrey)
2. Antepartum Haemorrhage (APH)
3. History of abnormal cranial ultrasound in the neonatal period
4. Past history of stormy neonatal course
5. Cerebral Palsy (previous diagnosis; predominantly affecting the lower limbs)
6. Recent concerns regarding behaviour
7. Evidence of mild constipation
Follow up: 6 months
Action Plan; Referrals:
· Physiotherapy for assessment
· Speech and language therapy assessment
· To request further information from school regarding behaviour
· School Nursing Team; to contact you regarding attendance and any barriers to access to the school curriculum.
I was pleased to meet Daniel and your self following your move to the area. Daniel has a complex background history and you brought with you some information regarding his background history and scan findings.
To summarise, Daniel is the second of your two children, his older brother Sam (10Yrs) is well, and there is no additional family history of note.
He was born following a significant bleed in pregnancy by emergency caesarean at 26 weeks gestation. He had a stormy initial course and whilst on the neonatal unit the team had expressed some concerns that his development may be affected. He did however make excellent progress with initial feeding.
We discussed your current concerns in turn:
Cerebral Palsy;
Daniel was diagnosed with Cerebral palsy at the age of 2 years. He has responded well to physiotherapy input and daily exercises / stretches with previous use of splints to his ankles but you feel that currently his walking is very difficult and he appears to be again walking persistently on his toes and tiring easily. When he has walked long distances he complains of pain in his legs.
Daniel has no difficulties with eating or swallowing and tolerates a family diet although is fussy. He has no history of chest infections or other illnesses
Examination today revealed an increase in tone and reflexes in Daniels legs, the findings were consistent with his previous diagnosis of cerebral palsy and the findings on his previous MRI scan would suggest that this has arisen due to the events surrounding and prior to delivery in combination with his prematurity.
Today we have referred him to the physiotherapy team for formal assessment of his tone and gait, as he is likely to benefit from further intensive input given his recent growth spurt.
Concerns regarding Behaviour;
You described the difficulties Daniel is having surrounding his ability to concentrate particularly in a school setting . He has just started in his new school and as a first step I would like to obtain more information regarding his behaviour and progress in order to guide further formal assessments in this area.
Concerns regarding social interaction;
We discussed his play and interaction with other children, in addition to the previous concerns regarding his speech and language development. Again as a first step I would like to obtain more information regarding his behaviour and progress in order to guide further formal assessments in this area, and with your permission I have written to Daniel’s school today to obtain some detailed observations of his behaviour, progress and interaction in a school setting. I have also asked my colleague, a specialist Speech and Language therapist to assess him in a school setting
New problem identified today.
On examination signs of mild constipation was found today. I would like you to increase the amount of fluid, fruit and vegetables in Daniel’s diet and work on his sitting position when on the toilet using a step and sitting on the toilet at least once a day. Should Daniels stools remain hard (we looked at the stool chart today) I would be grateful if you could see your GP between appointment as treatment to soften his stools may be helpful.
In conclusion
Daniel’s underlying health problem is Cerebral palsy affecting his lower limbs due to difficulties surrounding his premature birth. He will need input from the physiotherapy team locally and additional support is likely to be available via the local SCOPE support group.
Your additional concerns today require careful assessment in particular surrounding his behaviour and progress at school. As he has just recently transitioned to a new school and this change coincides with a change in his symptoms we agreed that gathering further information from both school and the speech and language therapy assessment. We have written to school with your permission today. A formal meeting of the multidisciplinary team may be needed to ensure that Daniel’s needs are being met.
Please feel free to contact me at the clinic if you have any other concerns about Daniel before the next clinic appointment
Yours sincerely
Dr A Robertson
Consultant Community Paediatrician
Cc:
· Dr G P Smith,
· School Nurse,
· Physiotherapist,
· Speech and Language Therapist
Formative Assessment – Struggling at school
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