Adult Care in Angelman Syndrome
Jessica Duis, director of the Angelman Syndrome Clinic at Children’s Hospital Colorado, discusses adult care for Angelman Syndrome. She covers topics such as transition and access to care, routine and activities, nutrition, behaviour, sleep, and gait and mobility. Duis emphasises the importance of transition programs, guardianship, and respite and day programs as individuals with Angelman Syndrome age. She also highlights the need for regular dental care, therapies, and the use of augmentative and assistive communication devices. Duis notes that individuals with Angelman Syndrome continue to learn and pick up skills as they age. She also discusses the importance of managing constipation, cyclic vomiting, sleep challenges, and non-epileptic myoclonus. Duis recommends the use of braces and orthotics for mobility issues and the management of periods for females with Angelman Syndrome. She concludes by sharing resources for further information and support.
Hello, my name is Jessica Duis. I’m the director of the Angelman Syndrome Clinic at Children’s Hospital Colorado, and I’m very happy to be with you today to be talking about adult care for Angelman Syndrome. Today, I’ll be discussing transition and access to care, routine and activities, nutrition, behaviour, sleep, and gait and mobility.
Transition and Access to Care
When you’re starting to think about transition of care as your loved one gets older, we always think first about transition programmes. In the state of Colorado, individuals can go to school, usually through the age of 21, and participate in transition programmes. These programmes focus on independence and vocational skills, which individuals with Angelman often enjoy. This can provide some prolonged routine and access to school services.
Guardianship and Trusts
Usually, guardianship occurs at the age of 18. When you start having conversations about transition programmes, it’s important to make sure that you’ve thought about this and that you’ve started the process for guardianship, thinking about trusts, which often come a little bit earlier than this.
Respite and Day Programmes
We also really emphasise the importance of respite and day programmes as your loved one gets older. This can be supportive to ensure that there’s some routine, but also that there’s some varying caretakers in your adult’s life.
Long-term Care Planning
Long-term care planning, talking early on with siblings or planning ahead for what happens with your loved one when you’re no longer around is also crucial. There are many resources for this available online at your convenience.
One of the things that we’ve been seeing in our clinic as well has been some nutritional changes for your loved one as they get older. We’re often seeing a presentation of something called Failure to Thrive, where the individual with Angelman has significant weight loss as they get older and into adulthood.
Behavioural changes are also common in adults with Angelman Syndrome. Anxiety is really high and can manifest a lot in adults as transitions are happening. Cognitive rigidity, where they have insistence on routine, and sometimes that routine includes a very preferred caretaker that they always want to be attached to, can be very challenging.
If there is a change in behaviour, you really want to rule out triggers. Severe constipation is a big trigger in adults for many of the things that we’re talking about. Pain, teeth issues, changes in environment that could be driving anxiety, mental health concerns that are contributing to sleep are all things to consider.
Gait and Mobility
One of the things that we observed about 10 years ago is that there is definitely a change in a good number of individuals as they get older and enter adulthood where they develop this pattern of walking where it’s called a flex knee gait pattern.
We continue learning about Angelman and this is what’s the beauty of our LADDER Learning Network, but also of our amazing multidisciplinary team that we have. One of the things I was incredibly thrilled to be a part of was putting together a multidisciplinary approach and standard of care for Angelman, and the algorithms that I showed today are within that paper. This is a free resource that anyone can access online. Take it to your physicians and your care providers and anyone your child interacts with in the community. There’s supplemental figures for therapists to help you. There’s amazing resources on the Angelman Syndrome Foundation website.
Thank you so much for having me. If there’s anything we can ever do for you, we’re here and we want to support this community as much as we possibly can.
- Title: Adult Care in Angelman Syndrome
- Author(s): Jessica Duis
- Author(s)’ affiliation: Children’s Hospital Colorado
- Publication date: 2023-07-07
- Collection: 2023 ASF Virtual Conference