MANAGEMENT

Managing children with Angelman syndrome requires a multidisciplinary approach that focuses on managing medical problems and developmental delays, as well as addressing feeding & gastro difficulties and sleep issues. They also need support to participate in their community. Below is a more detailed overview of the management of children & adults with Angelman syndrome.

Key Considerations:

No Cure: Angelman syndrome is a lifelong condition, and there is currently no treatment or cure for the condition itself.
Lifelong Support: Individuals with Angelman syndrome (all genotypes) will require ongoing support and 24/7 care throughout their lives.
Individualized Approach: Management plans should be tailored to the specific needs of each person with AS.
Focus on Strengths: Emphasize the individual's strengths and abilities, and help them achieve their full potential. Always presume competence.
Positive Reinforcement: Use positive reinforcement to promote appropriate behaviours, rather than punishment.
Challenging Behaviours: These may be a result of anxiety, pain, frustration, lack of sleep, or illness. Understand that all behaviour is communication; address them with appropriate strategies. Avoid additional medication / sedatives, until the source has been identified.

Angelman Syndrome: What It Is, Symptoms & Treatment

Embrace a holistic view of life: see Person-centred Planning and the Enabling Good Lives Approach (EGL)

This site is for informational purposes only. For medical advice, please consult a professional.

LIFE STAGES & CARE PLANS

The clinical management for people living with Angelman Syndrome changes as they move through the different stages of life.

Standards of Care for Angelman Syndrome (2022) published by Angelman Syndrome Foundation, USA. This collaborative project documents common issues that accompany AS and the most common and established ways of providing care for the issues.
Angelman Syndrome Clinical Management Guidelines (DYSCERNE— Angelman Syndrome Guideline Development Group, UK) - a general resource for Health Care
Challenging behaviour (Angelman UK) - All behaviour is communication. It is very important not to ignore behaviours that challenge without additionally teaching an alternative communication strategy, otherwise the person has no way to communicate their needs.
Behaviour Training Series - a video based series to explore possible root causes of challenging and aggressive behaviors and identify possible solutions. Funded by the Canadian Angelman Syndrome Society (CASS) and the Angelman Syndrome Foundation (ASF), and developed by the ASF.

Listed below (and in the links) is information applicable to Atearoa NZ

Birth - 3 yrs
3 - 6 yrs
6 - 18 yrs
18 yrs - Adulthood

BIRTH - 3YRS

General Health

The diagnosis of Angelman Syndrome can be difficult in infancy because typical characteristics aren't usually apparent at birth and symptoms may appear gradually. Even though sucking, breast feeding, reflux, and sleep may be problematic, the infant can appear to be happy and healthy until they fail to reach certain milestones, such as sitting on their own.

When you and your GP have concerns and agree that your baby may be 'failing to thrive' or if there are other indicators that something is not as it should be, you should be referred to a specialist Paediatrician at your public hospital. The Paediatrician will do a detailed assessment and will have access to a variety of tests and other specialists in areas like gastroenterology, orthopaedics, neurology, etc.

In their role as care co-ordinator the Paediatrician can also involve other services such as Genetics Service - for specialist diagnostic tests for diagnosis of genetic conditions, explanation of the diagnosis, discussion of prenatal or pre-symptomatic tests for genetic conditions, and to discuss implications of a family history of genetic conditions. These services are based in Wellington and Auckland, but they have outreach clinics in other areas. Samples of blood will be sent to laboratories here in New Zealand, or overseas for further testing.

See SUPPORT & MANAGEMENT PLAN: BIRTH - 3YRS

3 - 6 YRS

Health & Community Participation

Your Early Intervention Team will assess your child and help you access appropriate equipment, housing, transport, and education - and help with day-to-day activity and participation in your community. A variety of government subsidies are available for equipment, house modifications and modified vehicles but the criteria are complex and tight. Details of these criteria are available from the two agencies that administer the programmes:

Accessable – for the Auckland and Northland regions. Freephone 0508 001 002
Enable NZ – for the rest of the country. Freephone 0800 17 1981

See information SUPPORT & MANAGEMENT PLAN: 3 - 6YRS

6 – 18 YRS

Education & School Therapists

During the school-going stage, many extra supports, eg. equipment, physio and communication, will come through specialist therapists working within the education system (MoE). These school therapists will monitor your child's developmental progress, and will refer you to specialists for follow-ups, if & when necessary. See Education for information on accessing the curriculum.

For optional private therapy options you may wish to explore, see Therapies.

Community Participation - Social Capital

Use the school years to work strategically on building more social capital for your child. Extend your child's 'social footprint' by getting involved in the school and your local community as much as possible. Join after-school clubs and activities - both mainstream and other. Creating solid, lasting friendships and relationships in your local community will help support your child as they prepare for their adult years.

More information and ideas for Social Capital here.

See SUPPORT & MANAGEMENT PLAN: 6 - 18YRS

18 - Adulthood

Transitioning Health Services

When your child turns 18, they are legally an adult. They will be discharged from all Child Services and will need to access their health needs through Adult Services. This process is not straightforward as different ministries and departments seem to use different transition processes. It is important to know that:

In general, from 18yrs, the regular health monitoring of your child will cease
All health matters will need to go through your GP
Health matters will be treated only when/if they occur
When necessary, the GP will then refer you back to the relevant specialists in the adult public system

Transitioning from School to Community

Your adult child may remain at school until their 21st year. Parents should start looking at Transition Services 18 months before they leave school. A funded Transition Coordinator will be asigned to support the planning process. Once a student leaves school, all school-based therapy programmes & services will cease. ORS funding will be transferred from Education (MoE) to the Ministry of Social Development (MSD) to fund a day programme or vocational service, usually only for 3 days a week. This is important to factor in to your plans for the future.

See more about creating a good life for adults with Angelman syndrome in Aotearoa NZ - SUPPORT & MANAGEMENT PLAN: 18 - Adulthood