Families What is Dravet syndrome?

Living with Dravet syndrome

Living with Dravet Syndrome

Adapting to live with Dravet syndrome is an immense challenge for those affected. The effects are all pervading and impact both the child/adult living with Dravet syndrome along with parents, siblings and extended family.

We hope this introduction and accompanying video provide all of those affected with a comprehensive overview into the many considerations of living with Dravet syndrome. You are not alone and Dravet Syndrome UK and our community are here to support you every step of the way.

 

Sleeping

There is an inherent relationship between sleep and epilepsy as sleep activates the electrical charges in the brain that result in seizures, which generally mirror the sleep-wake cycle. Sleeping difficulties in children suffering from Dravet syndrome are particularly common.

  • Sleep disorders and disturbances: Problems with sleep vary between children with Dravet syndrome but can include insomnia (20%), premature awakening (28%), nocturnal seizures (50%) and sleep apnea (52%). These can be attributed to a number of factors including side effects from medication and seizure activity during the night. With lack of sleep being cited as a key trigger for seizures, it is something that can cause many parents a restless night.
  • Nocturnal (night-time) seizures: Seizures are common when the brain is undergoing transitions (e.g. as your child is falling asleep or shortly before waking).

We all know that not getting a good night’s sleep can affect how we function during the day, leading to fatigue and an inability to function. Nocturnal seizures are no different as they significantly reduce the time spent in deep sleep and can be dangerous for children whose parents are unable to monitor them throughout the night.

  • Lack of sleep as a cause of seizures: Lack of sleep is often cited as a key trigger for seizures and can cause a vicious cycle, in which a child that becomes sleep deprived has more seizures, and the increase of seizure activity causes a child to become even more sleep deprived. It is therefore vital that you try to ensure your child sticks to a healthy sleeping pattern, where possible. Sleep disturbance could be a phase that your child will come out of once their bodies are used to the medication they are taking.
  • Importance of healthy sleep for parents: If you are the parent of a child with Dravet syndrome, it is very important that you make healthy sleep a priority for yourself too, so that you have the energy you need to function throughout the day and look after your child. Knowing your child is being monitored during the night may give you the peace of mind to have a good night’s rest. Respite, which is discussed in a later section, may be one way of ensuring you get some better quality sleep, while knowing your child is safe and cared for.

Eating

It is very common for patients with Dravet syndrome to have feeding difficulties. These can vary from child-to-child and can be caused by a number of things including side effects from medication, seizure activity and autism spectrum disorders (ASDs).

One of the first steps, if you are concerned about your child’s diet or eating patterns, should be to create a food diary. A food diary could shed some light as to why your child is over or under eating. By regularly recording your child’s eating habits you will be provided with useful information about their eating pattern and the potential triggers for their lack of appetite or their inclination to over-indulge at certain times.

It is important to identify if possible, from the food diary, whether it is the volume or type of food being eaten that is causing your child’s dietary issue. There are a number of factors that could be contributing to this, including:

  • Physical: your child may be over-eating as a result of lack of control and the ability to know when they are full.
  • Obsession: one feature of ASDs can be obsessive behaviours, so food and the consumption of it may have turned into an obsession or area of special interest, possibly leading to over-eating and weight gain.
  • Coping strategy: We all have different ways of dealing with stress and difficult periods in our lives. Your child may see food as a source of comfort, or they may be under eating as a way of dealing with stress.
  • Sensory: Your child may have a particular craving for a specific food, because it may be providing them with sensory satisfaction.
  • Medication side effects: You may have noticed a change in your child’s eating habits since altering their medications, even if the alteration is very slight.

If your child is having problems with eating too much or not enough we encourage you to get a referral to a dietician. They will be able to advise you on techniques to ensure your child is getting the nutrients they need. Your GP should be able to make that referral.

Gastrostomy/Tube Feeding

When eating problems become very severe having a gastrostomy may be an option. A gastrostomy is a surgical procedure for inserting a tube through the abdomen wall and into the stomach. The tube is then used for feeding. Having a gastrostomy can be a really difficult decision to make, as it is very hard psychologically to further medicalise your child. For some patients, it can be incredibly beneficial and impact, in a positive way, on all aspects of life. The procedure must be discussed with the patient’s medical team, including the consultant and dietician.

Gait

Gait refers to how a person walks; abnormalities relating to gait are typically associated with a physical disease, condition, or deformity. Any child with a condition that affects their motor and sensory development can develop gait problems. These problems are not necessarily specific to Dravet syndrome, as they have also been seen in other epilepsy syndromes, but they are strongly associated with it. For example, over time, a child with Dravet syndrome maybe unable to straighten their knees properly and this will affect how they stand, walk or run.

A number of studies have shown that children with Dravet syndrome typically exhibit progressive deterioration of gait after the age of ten, which can have a significant impact on their mobility and independence. As a rule, children usually start walking at a normal age but develop an unsteady gait as they become older.

There are a number of things you can do to try to manage and improve your child’s gait, including specifically designed footwear and strengthening muscle exercises. Speak to your healthcare professional for advice and request referrals to:

  • A physiotherapist
  • An orthotics clinic.
  • A specialist gait clinic.

Toileting

It is not unusual for patients with Dravet syndrome to toilet train later than an average child, and depending on the level of learning disability some patients may remain incontinent. If you have access to an occupational therapist they may be able to give you ideas on toilet training, alternatively you should have access to an incontinence service who will also be able to offer advice and experience. The incontinence service also provide and supply nappies and pads for children and adults who are incontinent. You can get referred by your health visitor, community nurse or GP. At the time of going to print the incontinence service provides free nappies and pads to all children and adults aged over 4 years of age.

Schooling

Every child with Dravet syndrome will have different educational needs and it is important for you to understand the options available to support your child’s learning. This will help you to ensure that you make the right decision for you and your child and that their special educational needs (SEN) are met. This may be in a mainstream school or nursery with extra support, or in a special school.

a) Your Child’s Rights to Additional Support

If you are considering sending your child to a mainstream nursery or school, make sure you are aware of the additional support that you can receive. This will vary from school-to-school so it is important that you research your local schools and arrange a visit to understand what extra support is available at each. All state-funded schools (including nurseries and playgroups) should follow the Special Educational Needs Code of Practice Guidelines which provides information on how schools should assess and meet SEN.

Your child should be allocated a Key Worker or Special Educational Needs Co-ordinator (SENCO) who can help you to work out what additional needs your child might require. This could be a teaching assistant with them in the classroom, a specific method of teaching or additional help outside the nursery or school, such as a speech and language therapist.

b) Responsibilities of Mainstream Schools

It is important to remember that the Government’s education policy is focused on helping all children achieve their potential and have equal opportunities within their learning environment. It is also illegal for any child to be discriminated against due to a disability or special educational needs. This is reinforced by a law known as The Equality Act 2010 and The Special Educational Needs Code of Practice which plays an important role in ensuring that schools are aware of their role in ensuring this is realised.

The focus of The Special Educational Needs Code of Practice is ensuring that children’s special educational needs are identified as quickly as possible and that early action is taken to meet those needs. It also places emphasis on monitoring the progress of children with special educational needs towards identified goals.

c) Early Years Action and Early Years Action Plus

The Early Years Action plan is offered to children at an early age with additional educational needs. This may be due to them not progressing as expected or due to behavioural, sensory or physical problems they are experiencing.

A Key Worker or Special Educational Needs Co-ordinator (SENCO) will carry out an assessment of your child’s needs to decide what support they require and develop an Individual Education Plan (IEP). This will highlight what special help will be provided at school/nursery, what you can do at home, and include targets for your child to achieve.

You should meet regularly with your Key Worker or SENCO to discuss your child’s progress and if your child is not making enough progress through the Early Years Action programme, they may be able to receive additional support. This is referred to as Early Years Action Plus.

d) Education, Health and Care Plan (EHC Plan)

You or your nursery/school can ask the local authority for a formal assessment of your child’s special educational needs to help ensure they receive additional support. Based on your application, the local authority will decide whether or not to assess your child or issue an Education Health and Care (EHC) plan, which brings your child’s education, health and social care needs into a single, legal document (this document replaces Statements of SEN and Learning Difficulties Assessments for children and young people with special educational needs).

The assessment will be based on conversations between the local authority and a number of people including you and your child, their school or nursery, doctor and an educational psychologist. Once the authority has collected all the information it needs for the assessment, it will decide whether or not an EHC plan is necessary (if they decide not to proceed with a plan, they must inform you within 16 weeks of the initial request).

When drafting the EHC plan, your local authority should work closely with you and your child to make sure the plan takes full account of your views, wishes and feelings. Once the plan has been written, a draft will be sent to you. You will be given 15 days to comment on the draft and you can ask for a meeting to discuss it if you want one. At this stage, you are able to request a specific school, or other setting, that you want your child to attend. Your local authority has 20 weeks from the request for the EHC needs assessment to issue the final plan to you. Once an EHC plan has been finalised, your local authority has to ensure that the special educational support in section F of the plan is provided, and the health service has to ensure the health support in section G is provided. This should help to enable your child to meet the outcomes that you have jointly identified and agreed.

Your local authority has to review your child’s EHC plan at least every 12 months. That review has to include working with you and your child and asking you what you think and what you want to happen, and a meeting which you must be invited to. It is also an opportunity to update the plan to assist your child’s development and ensure it is relevant for their progression. It is important that you provide an honest account of how you feel your child is progressing and discuss any concerns you have. You can also actively suggest ways you feel this can be improved or any further support you feel would benefit your child. If your child has an EHC plan you may be entitled to a personal budget, which allows you to have a say in how to spend the money on support for your child.

You can use your personal budget in three ways (or as a combination of two or more of these options):

  • direct payments made into your account – you buy and manage services yourself
  • an arrangement with your local authority or school where they hold the money for you but you still decide how to spend it (sometimes called ‘notional arrangements’)
  • third-party arrangements – you choose someone else to manage the money for you

You have the right to appeal if you disagree with your child’s final EHC plan (or if you disagree with the local authority’s decision not to provide an EHC plan if this should be the case). Your local Parent Partnership Scheme will be able to guide and support you through the process.