The NZDSA is extremely pleased we can offer our members an invaluable new resource to learn about the needs of the older person with Down syndrome, as well as a follow-up workshop to answer your questions.

The digital resource The Golden Years, Ageing and Down syndrome is presented by expert Geraldine Whatnell and discusses how ageing may affect their health and wellbeing, as well as supports they may need if they are diagnosed with dementia.

The valuable video offers background on a range of subjects and demonstrate best practice when supporting people with Down syndrome. The subjects include:

(Click here to watch or download the full video).

  • Ageing and Down syndrome
  • Importance of health checks
  • Explores the connection between  Down syndrome and dementia
  • Looks at the myths about Down syndrome and dementia
  • Assessment and diagnosis of dementia
  • Understanding dementia and the stages of dementia
  • Strategies we can use
  • How we can help people with Down syndrome who live with dementia.

Presenter Geraldine (Dina) Whatnell is the Nurse Practitioner Mental Health and Addictions Service Palmerston North Hospital.

Geraldine brings with her nearly 40 years of professional skills and knowledge in the specialist area of developmental disabilities (intellectual disabilities and/or Autism Spectrum Disorder) and mental illness.

Alongside this, Geraldine has a wealth of personal lived experience as her younger brother Mark having a diagnosis of Down syndrome .

Getting older is associated with many changes, both biological and social. For someone with Down syndrome these changes can be particularly daunting and difficult.

There is evidence to suggest that some biological problems related to ageing can occur earlier in people with Down syndrome than in the general population.

The marked improvement in life expectancy for people with Down syndrome (an average of 60 -70 years, compared to an average of 9 years around 1900) also means that the problems relating to the condition and old age are only now being researched and addressed.

Unfortunately,  there are very limited few resources available for families and whānau who support and care for aging people with Down syndrome.

The NZDSA recognises this gap and decided to develop this resource to start filling this void.

In New Zealand there is no strategy or plan to address dementia for the general population therefore there is even less knowledge, information and support for people with Down syndrome.

If you are interested you can read and support this open letter to the Government to be more pro-active in this area.

https://mailchi.mp/alzheimers/stand-with-us-sign-our-open-letter-to-government?e=59bc99ddea

Click here to watch or download the video.

You do not want to miss this.

Make sure you check out the long list of world-leading presenters, who will cover most key topics that may be important to people with Down Syndrome as part of the NZDSA Virtual Down Syndrome Conference.

REGISTER HERE

The NZDSA this month will celebrate its 40th anniversary by hosting the largest Virtual Down Syndrome Conference ever organised in this country, to coincide with World Down Syndrome Month.

The virtual conference will offer a stunning variety of high-profile Kiwi and international expert speakers who will cover a wide range of topics including education, health, speech, relationships, ageing and legal issues.

The conference will have several presentations focused on health with Andrea Simonlehner from Natural Equilibrium doing two sessions on gut health and fussy eaters, while Geraldine Whatnell will share her expertise on growing old with Down syndrome.

One session that will be exciting to parents as well as younger adults with Down syndrome will be relationship expert Dave Hicks talking about the theory and practicalities of getting into a relationship. Dave is likely to become a familiar face on New Zealand television as he was the expert on the upcoming television series Down With Love.

One of the speakers from the United States will be Kavita Krell from Massachusetts General Hospital who will explain how you can develop an innovative online health plan from anywhere in the world, while her colleague Dr Brian Skotko will do a presentation about and for siblings of people with Down syndrome.

REGISTER HERE

The conference will also offer sessions wellbeing, speech therapy, advocacy and educations, so there will be there something for all stages of life and every member of our community.

The conference will also include a number of sessions specifically for people with Down syndrome, so for the full list of those events, please click here (all these sessions are marked in orange below).

The NZDSA has hosted many conferences, seminars and workshops during its history, but geographical and financial restraints often restricted the number of people who were able to attend.

All those barriers have now been removed by modern technology and the new digital skills we have learned during lockdown.

With modern video conferencing technology, there is no reason to miss out so keep an eye on our E-new and Facebook page for upcoming details how to register.

To get all this information, make sure we can send you our newsletters, so please register your details on the NZDSA website FOR FREE, at www.nzdsa.org.nz

Some sessions will be recorded as a future resource, but many others will not, so make sure you do not miss out on the webinars you are interested.

The NZDSA is non-for-profit charity that receives no Government funding and fully depends on grants and donations, so when you register or think the webinars are of value to you and your community, please remember to make a donation, so we can continue to offer these services to raise the awareness and knowledge around Down syndrome.

REGISTER HERE

 

THE FULL PROGRAMME OF THE VIRTUAL DOWN SYNDROME CONFERENCE

Tuesday 5th October, 7pm – 8pm

Bridget Snedden, President of Down Syndrome International(DSi)

Theme: Wellbeing and Rights

Title:  Getting to the good life – The importance of having a vision

Audience:  Everyone

 

Wednesday 6th October, 7pm – 8pm

Dr Mark Sinclair, Private Practice

Theme: Wellbeing

Title: Keeping an Emotional Balance (At home with children and teens)

Audience:   Parents, whānau, professionals – all ages

 

Thursday, 7th October, 7pm – 8pm

Trish Grant, Director of Advocacy IHC

Theme: Education and Rights

Title: Are we there yet?

Audience: Parents, whānau, professionals with a focus on school-aged children

 

Saturday, 9th October, 10am – 11am

Sir Robert Martin

Theme: Rights and Wellbeing

Title: From Institution to Knighthood

Audience: Everyone

 

Tuesday, 12th October , 7pm – 8pm

Erika Butters, Director, The Personal Advocacy and Safeguarding Adults Trust

Theme: Rights

Title: Supported Decision Making – a Human Right

Audience: Parents, whānau, professionals with a focus on youth and adults

 

Wednesday, 13th October, 7pm – 8pm

Dr Maree Kirk, Director, STPDS NZ

Theme: Education

Title: Individual Education Plan – The capabilities approach

Audience: Parents, whānau, professionals with a focus on school-aged children

 

Wednesday, 14th October, 12pm-1pm

Andrea Simonlehner, Natural Equilibrium

Theme: Health

Title: Brain and Gut Health for people with Down syndrome

Please note this session will view the recorded session hosted in September (there will be no Q&A)

It will be beneficial to view this for the session with Andrea on the 27th October

Audience: Parents, whānau, professionals – all ages

 

Thursday, 14th October, 7pm – 8pm

Who: Erika Butters, Director, The Personal Advocacy and Safeguarding Adults Trust

Theme: Rights

What: Supported Decision Making – a Human Right. 

Audience: People with Down syndrome

 

Saturday, 16th October, 10am – 11am

Dr Brian Skotko,  Director of the Down Syndrome Program at Massachusetts General Hospital

and Susan Levine, Co-founder and social worker at Family Resource Associates, Inc.

Theme: Siblings 18+

Title: Adult brothers and sisters of siblings with Down syndrome: Exploring past experiences and current roles

Audience: Siblings 18+

 

Tuesday, 19th October,  7pm – 8pm

Fiona Kenworthy, Speech & language therapist

Clinical Director /Small Talk Therapy

Theme: Speech and Language Therapy

Title: Early literacy skills for children with Down syndrome – Families making a difference

Audience: Parents, whānau, professionals with a focus on early literacy skills (not age specific)

Hosted by UpsideDowns Education Trust and the NZDSA

 

Wednesday, 20th October, 7pm – 8pm

Dave Hicks, Academic Programme Manager – Unitec

Theme: Wellbeing

Title: Relationships – Theories and Practicalities

Audience: Parents, whānau, professionals – all ages

 

Thursday,21st October, 12pm -1pm

Gretchen A. Good, PhD, Senior Lecturer in Rehabilitation and Disability Studies, Massey University and Jane Lee, MSW, Tutor in Rehabilitation and Disability Studies, Massey University

Theme: Wellbeing and Rights

Title: Parenting during a pandemic:  Have any lessons been learned?

Audience: Parents, whānau, professionals – all ages

 

Thursday 21st October, 7pm – 8pm

Who: Dr Mark Sinclair, Private Practice

What:  Managing your feelings

Audience: People with Down syndrome

 

Saturday, 23rd October, 10am – 11am

Kavita Krell, Director of Customer Experience for Down Syndrome Clinic to You (DSC2U)

Theme: Health

Title: DSC2U: An online personal care plan for caregivers and primary care physicians

Audience: Parents, whānau, professionals  – all ages

 

Tuesday, 26th October, 7pm – 8pm

Andrea Simonlehner, Natural Equilibrium

Theme: Health

Title: Fussy Eaters

Audience: Parents, whānau, professionals – relevant for all ages

 

Wednesday, 27th October, 7pm – 8pm

Geraldine (Dina) Whatnell, Nurse Practitioner Mental Health and Addictions Service Palmerston North Hospital

Theme: Health

Title: The Golden Years: Ageing and Down Syndrome

Audience: Parents, whānau, professionals – focus on adults who are 25+

 

Thursday, 28th October, 7pm – 8pm

Who: Libby Hunsdale, Michael Holdsworth, Rochelle Waters, Amelia Eades, Luka Willems, Lily Harper, Carlos Biggemann, Bradley Lewis, Jacob Dombroski, Alfie Linn

What: Meet the Stars!

Audience: People with Down syndrome, but others are welcome to join

 

Saturday, 30th October, 10am – 11am

Dr Maree Kirk, Director, STPDS NZ

Theme: Education/Wellbeing

Title: Making visions work – Social skill development for identity across the lifespan

Audience: Parents, whānau, professionals with a focus on school-aged children

 

UpsideDowns, New Zealand Down Syndrome Association, and Small Talk Therapy, are proud to bring you this free webinar on speech and language therapy for children and young people with Down syndrome.

Are you looking for ways to support your child’s speech during lockdown?

Are you new to speech and language therapy?

Have you been engaging with speech and language therapy for a long time but are looking for new ways to engage with your child’s communication journey?

Are you frustrated or confused by what public speech therapy is being provided? Are you keen to start with speech and language therapy but don’t know where to begin?

If you’ve answered yes to any of these questions, then this webinar is for you!

Fiona Kenworthy, a specialist in speech and language therapy for children with Down syndrome, Sarah Paterson-Hamlin, CEO of UpsideDowns, and Zandra Vaccarino, National Executive Officer of the New Zealand Down Syndrome Association, are answering questions from over 100 families, followed by a brief presentation by Fiona on some tips and tricks for lockdown SLT.

 

NZDSA MEDIA RELEASE

The New Zealand Down Syndrome Association (NZDSA) urgently calls on the Government to invest in disability attitude training for health professionals.

A deeply disturbing article in the Sunday-Star Times yesterday clearly articulated and emphasised the traumatic discrimination many families experience during pre-natal screening tests, as well as the pressure to terminate their pregnancies by medical professionals.

In the story, one expectant mother explains that as she was heading into theatre to have her baby delivered by caesarean, she was asked if she would want the doctor to resuscitate her baby if it was required.

“The stories from these parents once again demonstrates how parents feel pressurised and that information is not presented in a balanced way, nor are expectant parents provided with up-to-date information about Down syndrome,” says NZDSA president Kim Porthouse.

Porthouse says that most health professionals have no first-hand experience of living with Down syndrome and may not even be aware of the unconscious bias they bring to conversations with expectant parents.

“Because they have never met any people with Down syndrome living amazingly full and rewarding lives, they often revert back to a clinical health discussion that presents a doomsday scenario of potential health risks that may never eventuate.

“The parents are in shock, and in many cases termination is presented as the only logical option before parents even have time to process all the information,” says Porthouse

The NZDSA has consistently called for the National Screening unit to invest into disability attitudinal training for health practitioners, as well as focused training on how to offer screening and share screening results in a non-directive manner and to provide accurate and up-to-date information on Down syndrome.

“We have been asking for this for many years, but it still has not happened. It is such a sensitive moment so it is also important to offer expectant parents non-directive counselling to help them make their decision, but this is not available,” says the NZDSA president.

Porthouse says that people with Down syndrome are valued members of every community they live in, but for some reason medical professionals are determined to ignore the social aspects of the discussions.

“Down syndrome in itself should not be a reason to terminate a pregnancy. The way a society thinks about people with disability underpins everything,” says Porthouse, who emphasises that the NZDSA is not challenging a woman’s right to make her own decision, “but we just want them to be able to make a fully informed decision”.

The discrimination and stigma in society does not stop once the baby is born, judging by the recent experiences of Bridie Allen, parent of two-year-old Sienna.

“After the birth of my daughter, some people asked if I didn’t get the screening, as if there had been some mistake,” says Allen.

Porthouse says that this type of question confirms the discriminatory assumption by many in our society that expectant parents would terminate.

“And let’s not forgot how hurtful this unconscious bias in society must be to people with Down syndrome, who are told that their lives don’t really matter,” says Porthouse.

Vincenzo Vaccarino is a young man with Down syndrome from Palmerston North and he says the pre-natal screening discussions make him feel “very bad and uncomfortable”.

“It makes me feel very sad to hear that parents choose not to have babies with Down Syndrome,” says Vincenzo, who married his girlfriend Rachel two years ago.

“I think it is cruel. People should consider the rights of people with Down Syndrome, we have the same human right to live, like every other person.”

 

 

 

 

 

The New Zealand Down Syndrome Association has launched a wonderful new resource to fill a “huge void” for health professionals when dealing with new parents of a child with Down syndrome.

“We have received overwhelming feedback from parents and whānau that the health professionals they deal with in those whirlwind first days, often do not have the information they need, so we hope that this will help both parties fill this huge void,” says Zandra Vaccarino, the NZSDA’s National Executive Officer.

Vaccarino says that new parents deal with a variety of health professionals, from their midwife, early intervention team and paediatrician to the Plunket nurse and GPs.

“Often these professionals seem to think that one of the other will have given given parents and whanāu specific information on Down syndrome, but often the parents fall in a big information-free abyss,” says Vaccarino.

The National Executive says that the Down syndrome diagnosis is a surprise for many parents so they have lots of questions and need to learn more about Down syndrome as well as navigate where and how to access  support.

“Parents are looking for answers and support, but often the first conversations are unnecessarily stressful and very upsetting, when the health professionals have no information – or worse – provide misinformation,” says Vaccarino.

The new brochure, Tips For Health Professionals, provides professionals with resources, key contacts and useful tips on how to approach these vulnerable parents in a respectful and sensitive manner.

“In many cases, doctors, nurses or other support workers will have limited knowledge of Down syndrome and may not be aware of the amazing resources and support organisations available to the parents.”

A large section of the brochure covers the language professionals are suggested to use and what phrases to avoid, as they may be considered offensive or archaic.

The brochure also includes a milestone map specifically for children with Down syndrome.

“Usually parents are given a milestone list for typical children. Having a specific Down syndrome road map will provide parents with realistic expectations about their child’s development and hopefully avoid unnecessary concerns and frustration.”

Health professionals and anyone interested in people with Down syndrome can download Tips For Health Professionals, and other resources on the NZDSA website. The resources are free to download once you have registered your contact details.

 

 

Actor Duncan Armstrong stars in a series of new videos released by the Health and Disability Commissioner and the Nationwide Health and Disability Advocacy Service to help people with learning disabilities think about their own experiences with using disability services and their rights under the Code.

There are five videos, each with a different story. The videos look at how Sam, played by Duncan, and his friends use disability support services and how they resolve any concerns that they have.

The videos were produced by Film for Change Aotearoa and made locally in Wellington with Wellington actors, including people who use disability services.

Each video has a closed captions option, and the closed captions and slides have been transcribed into Word documents. Deaf Aotearoa has created New Zealand Sign Language (NZSL) for each video.

 

Dementia is more common in people with Down syndrome than the general population. Liz Evans and Tanya Duckworth review research on why this is the case, along with recommendations for how families can support a loved one with dementia.

The term dementia doesn’t refer to one specific disease but a set of symptoms caused by a number of different brain disorders. Dementia results in a decline in a person’s mental abilities–their capacity to think, reason, and remember.

Most people with dementia will experience changes like:

  • declines in memory, with more recent information or events being harder to remember
  • difficulty concentrating
  • difficulty finding the correct words to say
  • reduced capacity to plan, to pay attention, and poorer judgement
  • feeling less motivated
  • personality and behaviour changes.

When dementia occurs in people under the age of 65, it is called ‘younger onset dementia’.

There are many different types of dementia with different patterns of symptoms. The most common form is Alzheimer’s disease. People with Alzheimer’s disease show progressive memory loss and a gradual decline in other skills. Their brains show changes in the form of a build-up of sticky plaques between the brain cells and tangles within the cells1.

Dementia is not a normal part of ageing.

Normal ageing does mean that the brain slows down, and it is common to find it harder to remember things as we age. However, forgetting recent events and conversations, forgetting the names of family members, and losing skills we once had are not normal at any age.

 

But changes in a person’s memory and thinking skills can also be caused by other medical conditions, many of which can be successfully treated.

Examples include:

  • a vitamin or mineral deficiency
  • a mental health problem such as depression
  • problems with sight or hearing
  • a side effect of new medication, or even a change in how their body deals with existing medications
  • an underactive thyroid (hypothyroidism)

 

Some of these conditions are more common in people with Down syndrome than the general population. So, any time a person is showing a decline in their thinking or memory, or changes to personality and behaviour, it is important to talk to their doctor about it straight away.

 

How common is dementia in people with Down syndrome?

Dementia, particularly Alzheimer’s disease, is much more common in people with Down syndrome than the general population and it tends to occur at a younger age. Scientists believe this is because a gene on chromosome 21 called the amyloid precursor protein (APP) gene plays a major role in the brain changes associated with Alzheimer’s disease. Genes are a code for proteins, and because most people with Down syndrome have three copies of this gene, they get more of its protein1.

 

Different studies have found very different rates of dementia in people with Down syndrome, ranging from under 10% up to 49 years of age, to around 30% for those in their 50s, and over 50% for those aged over 602. One recent study found a rate of just over 50% in those over 60 years3, but another recent study found a rate above 80% in those over 654. he average age for diagnosis is in the mid 50s4.

 

he outward symptoms of dementia do not start for some years after this and somelive into old age without developing symptoms3. So, it is not inevitable that a person with Down syndrome will get dementia but, due to the increased risk, it is still likely.

 

What are the signs of dementia in people with Down syndrome?

In people without Down syndrome, the earliest signs of Alzheimer’s disease are usually memory problems. But in people with Down syndrome, the first signs noticed by carers are more likely to be changes in behaviour and personality, such as increased stubbornness and behaving inappropriately. Other early signs include difficulty paying attention and lower ability to plan, solve problems, and make judgements5, 6.

 

Other changes may include 5, 6:

  • apathy
  • social withdrawal
  • increased dependency
  • confusion
  • prolonged sadness
  • fearfulness
  • repetitive speech
  • getting lost or disoriented in familiar places
  • irritability or aggression
  • seizures for the first time in adulthood

 

 

What is the latest research on dementia in Down syndrome focused on?

Scientists are working in a number of areas to further knowledge about the link between Down syndrome and dementia. Two important areas of current research are regarding diagnosis, and possible future treatments.

Our own research team is conducting the Successful Ageing in Intellectual Disability (SAge-ID) Study. One of the aims of that study is to compare different screening tools and assessments that may be suitable for people with intellectual disability, including those with Down syndrome. A further aim is to look at the factors associated with a higher risk of dementia in this group. People with intellectual disability aged over 40 can participate in the study, including those with or without dementia. This is to ensure a good a mix of those who are healthy and those experiencing declines.

Other researchers are looking at different biological markers that might be able to identify the brain changes associated with Alzheimer’s disease much earlier, even before cognitive symptoms begin. Examples include protein changes in a person’s blood, new types of brain scans, and measuring brain waves through EEG. If such measures could reliably detect brain changes earlier, then this could one day aid in directing specific therapies during the window before symptoms start1, 7. It could also help researchers as they try to develop and test future therapies focused on preventing dementia.

Certain medications can help to slow the rate of cognitive decline in some people who have dementia. However, studies with people with Down syndrome have found inconsistent results about whether these medications are effective, though some case studies suggest they may be for some people8, 9. However, people with Down syndrome may have an increased risk of side effects from these medications9.

Newer research is trying to develop future treatments that could prevent or alter the course of Alzheimer’s disease7, not just address the symptoms. Much of the research on drugs and neurotransmitters (brain chemicals) is done initially using mice. The safety and usefulness for humans then needs to be established.

 

A handful of studies have also looked at whether antioxidants could prevent or slow Alzheimer’s disease in people with Down syndrome10. So far, the results have not found that antioxidants worked to prevent decline8. Instead, results have pointed to the highly complex nature of the brain changes that lead to cognitive decline in people with Down syndrome. Much more research will be needed before scientists can identify specific supplements that may reduce dementia risk in people with Down syndrome.

Are there factors that increase–or decrease–the chances that a person with Down syndrome will develop dementia?

 

There is only a small amount of research about risk factors specific to people with Down syndrome. A handful of case studies suggest that people with atypical forms of Down syndrome may have a lower risk of developing Alzheimer’s disease11. Other studies have looked at the role of particular genes known to influence risk in the general population, but results are not always consistent across studies. Results regarding the potential influence of gender, hormones, and level of intellectual disability have also varied between studies.

 

However, much of what is known about dementia in the general population could also apply to those with Down syndrome. There is a considerable amount of evidence from the general population to support the protective effects of a healthy lifestyle. Research regarding people with Down syndrome is lacking but the World Health Organisation12 recommends that people with intellectual disabilities should focus on similar targets.

 

A healthy lifestyle aiming to reduce dementia risk would include good nutrition, regular exercise, and not smoking12-14. People (in the general population) who regularly do moderate-intensity exercise have a lower risk of dementia. They also have a higher brain volume in areas related to memory, planning, and learning.

The Mediterranean diet has also been found to reduce dementia risk in the general population as has staying socially active and engaging in stimulating activities for leisure, work, or education.13

One of the most important elements of a healthy lifestyle is preventative health care including regular medical check-ups. Good physical and mental health throughout life is associated with a lower dementia risk in the general population13.

People with intellectual disabilities often have undiagnosed or untreated health conditions which could be treated.

Sensory problems and physical disabilities can also compound their health and quality of life.

In the general population, cardiovascular disease is a particularly important risk factor for dementia15.

In general, people with Down syndrome have an overall lower risk of cardiovascular disease than the general population. However, it is reasonable to assume that for those people with Down syndrome who do have risk factors for cardiovascular disease, these factors would increase the risk of dementia. Such risk factors include a family history of heart disease and stroke, having diabetes, low levels of physical activity, a diet high in saturated fats, and smoking16.

 

Obstructive sleep apnoea is also known to increase the risk of dementia in the general population and it is very common in people with Down syndrome. It is possible that this could be an important additional risk factor for people with Down syndrome15. Medical management of obstructive sleep apnoea is based on an individual sleep study.

 

How can families identify the early stages of dementia and differentiate those from mental illness or other problems?

 

Diagnosing dementia in people with Down syndrome can be difficult. The standard tools for assessing cognitive function in the general population are not suitable when someone has an intellectual disability.

What is needed is to compare the person’s functioning to what it was before symptoms began9, but the person’s typical level of function may not be well documented.

As a result, health professionals rely on information provided by family, carers and other people who know the person well, to help come to an accurate diagnosis6. So it is important for the people close to the person with Down syndrome to know the early signs of dementia and to consult a doctor about any changes observed or any other concerns.

The earliest noticeable signs in people with Down syndrome may be behavioural or personality changes. If a person with Down syndrome consults a doctor when these changes are observed, then memory and other cognitive testing can be carried out at regular intervals to help to determine if decline is also occurring5.

 

There are tools available such as the Early Detection Screen for Dementia recommended by the National Task Group in the US. This is a tool that can help you to track your loved one’s skills and any changes in their functioning time. At present, the tool does not provide a cut-off score: rather, it is designed to facilitate talking about any observed changes with a health professional.

 

While families and carers are critical to recognising changes in their loved one, consulting a doctor is essential to determining whether those changes might be dementia or something else. There are other conditions that may look the same as dementia, many of which can be tested for and treated.

 

What can parents/carers do to prepare for the management of dementia in their loved ones?

If your loved one develops dementia, the keys to supporting them will be early planning and working well with their doctor and other professionals. So encourage your loved-one to find an attentive doctor they feel comfortable with, and to continue to see that doctor for annual health checks.

Early planning for any transitions begins with getting a diagnosis as early as possible. A baseline assessment of their skills when healthy is helpful. Use the free screening tool, and, if resources permit, arrange an assessment with a psychologist or psychiatrist.

Current recommendations are that people with Down syndrome have a cognitive assessment around age 30, to establish their normal level of functioning before declines begin and again at age 4017. But if they start to show declines, the assessment could be repeated annually5.

 

Build as much of a support network as possible around the person with Down syndrome. If dementia is diagnosed, talk with the person with Down syndrome about who is in their life and who they would like to invite to be involved in their care. Wherever possible, include the person as early as possible in the planning process. This may include discussions of end-of-life care18.

Support your loved one to understand their diagnosis so that they may participate in this planning. An easy-read fact sheet with pictures can help (see the resources links below). Find out what your loved one’s preferences are regarding care options, end-of-life planning, and what is important to them for their care18.

Families can facilitate holding onto items, such as photos and holiday souvenirs, which may one day serve a purpose in a memory box or life story. These are tools which can assist someone with dementia who is beginning to lose their memory. They can also aid communication between a person with dementia and others, and may help paid workers to understand the person better18.

Look into available services. People with Down syndrome have the right to access mainstream health services and aged care services. Those with younger onset dementia (before the age of 65 years) can also access aged care services if they have a diagnosis or suspected dementia. The National Younger Onset Dementia Keyworker Program can be accessed before a formal diagnosis is made. Of course, people with Down syndrome and dementia also remain eligible for disability-related supports. A range of allied health professionals may be involved in the care of someone with intellectual disability and dementia to promote their wellbeing.

 

As dementia progresses, the care goal needs to shift from supporting independence towards providing care and eventually palliative care 19, 20. Many people with Down syndrome and dementia may want to remain where they are living and their families may want this too21. However, if and when their care requirements can no longer be met in their current place, options will include transfer an aged-care facility or another disability service. Long-term planning for such transitions is helpful.

 

Dr Liz Evans is a NHMRC-ARC Dementia Research Fellow and Tanya Duckworth is a research assistant with qualifications in psychology and cognitive neuroscience. They are from the Department of Developmental Disability Neuropsychiatry (3DN), within the School of Psychiatry at the University of New South Wales in Sydney.

Resources

An easy read factsheet is available from the Alzheimer’s Society (UK) here:
https://www.alzheimers.org.uk/site/scripts/download_info.php?downloadID=1092

The screening tool recommended by the US National Task Group is available from this site: http://aadmd.org/ntg/screening

Alzheimer’s Australia has made a video about dementia in people with intellectual disability. It can be viewed here: www.dementia.org.au/videos/collections?playlist=IntellectualDisability

 

If you would like further information, or would like to talk to us about the SAge-ID study, please phone Tanya or Liz on (02) 9931 9160 or email us at [email protected]

 

 

References:

 

  1. Wilson, L., T. Annus, S. Zaman, and A. Holland, Understanding the process; links between Down Syndrome and dementia. Intellectual Disability and Dementia; Research into practice. London: Jessica Kingsley Publishers, 2014: p. 34-52.
  2. Sinai, A., T. Chan, and A. Strydom, The Epidemiology of Dementia in People with Intellectual Disabilities. Intellectual Disability and Dementia: Research into Practice, 2014: p. 24-33.
  3. Margallo‐Lana, M., P. Moore, D. Kay, R. Perry, B. Reid, T. Berney, and S.P. Tyrer, Fifteen‐year follow‐up of 92 hospitalized adults with Down’s syndrome: incidence of cognitive decline, its relationship to age and neuropathology. Journal of Intellectual Disability Research, 2007. 51(6): p. 463-477.
  4. McCarron, M., P. McCallion, E. Reilly, P. Dunne, R. Carroll, and N. Mulryan, A prospective 20-year longitudinal follow-up of dementia in persons with Down syndrome. J Intellect Disabil Res, 2017. 61(9): p. 843-852.
  5. Lautarescu, B.A., A.J. Holland, and S.H. Zaman, The Early Presentation of Dementia in People with Down Syndrome: a Systematic Review of Longitudinal Studies. Neuropsychol Rev, 2017. 27(1): p. 31-45.
  6. Nieuwenhuis-Mark, R.E., Diagnosing Alzheimer’s dementia in Down syndrome: problems and possible solutions. Res Dev Disabil, 2009. 30(5): p. 827-38.
  7. Castro, P., S. Zaman, and A. Holland, Alzheimer’s disease in people with Down’s syndrome: the prospects for and the challenges of developing preventative treatments. Journal of Neurology. 264(4): p. 804-813.
  8. Courtenay, K. and N. Eadie, Medication treatment of dementia in people with intellectual disabilities. Intellectual Disability and Dementia: Research into Practice, 2014: p. 62.
  9. Torr, J., Dementias, in Psychiatric and Behavioural Disorders in Intellectual and Developmental Disabilities C. Hemmings and N. Bouras, Editors. 2016, Cambridge Univrsity Press: Cambridge, UK.
  10. Ballard, C., W. Mobley, J. Hardy, G. Williams, and A. Corbett, Dementia in Down’s syndrome. The Lancet Neurology, 2016. 15(6): p. 622-636.
  11. Schupf, N. and G.H. Sergievsky, Genetic and host factors for dementia in Down’s syndrome. The British Journal of Psychiatry, 2002. 180(5): p. 405-410.
  12. World Health Organization, Ageing and Intellectual Disabilities – Improving Longevity and Promoting Healthy Ageing: Summative Report. 2000, World Health Organization: Geneva, Switzerland.
  13. Reppermund, S. and J.N. Trollor, Successful ageing for people with an intellectual disability. Curr Opin Psychiatry, 2016. 29(2): p. 149-54.
  14. Sisirak, J. and B. Marks, Health and wellness strand: recommendations from National Goals Conference 2015. Inclusion, 2015. 3(4): p. 232-249.
  15. Wilcock, D.M., F.A. Schmitt, and E. Head, Cerebrovascular contributions to aging and Alzheimer’s disease in Down syndrome. Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 2016. 1862(5): p. 909-914.
  16. Trollor, J., C. Salomon, J. Curtis, A. Watkins, S. Rosenbaum, K. Samaras, and P.B. Ward, Positive cardiometabolic health for adults with intellectual disability: an early intervention framework. Australian Journal of Primary Health, 2016. 22(4): p. 288-293.
  17. Dodd, K., S. Coles, T. Finnamore, T. Holland, S.K. Gangadharam, M. Scheepers, . . . S. Wilson Dementia and people with intellectual disabilities: Guidance on the assessment, diagnosis, interventions and support of people with intellectual disabilities who develop dementia 2015.
  18. Towers, C. and H. Wilkinson, Planning ahead: Supporting families to shape the future after a diagnosis of dementia. Intellectual Disability and Dementia: Research into Practice, 2014: p. 161-182.
  19. Carling-Jenkins, R. and C. Bigby. Supporting people with intellectual disability and dementia: A training and resource guide PowerPoint presentation for managers of disability organisations.; Available from: http://www.karingal.org.au/media/529677/day_one_-_dsws_-_karingal_theme.pdf.
  20. Jokinen, N., M.P. Janicki, S.M. Keller, P. McCallion, and L.T. Force, Guidelines for structuring community care and supports for people with intellectual disabilities affected by dementia. Journal of Policy and Practice in Intellectual Disabilities, 2013. 10(1): p. 1-24.
  21. Carling-Jenkins, R., C. Bigby, and T. Iacono, Family experiences of supporting a person with Down syndrome and dementia in Australia. Intellectual Disability and Dementia: Research into Practice, 2014: p. 145-60.

 

 

 

 

by Coen Lammers

As life expectancy of people with Down syndrome is increasing, the number of them developing dementia is also growing.

The extra chromosome that causes the developmental and health issues associated with Down syndrome also carries the Alzheimer’s genes, so people with Down syndrome often develop dementia at an earlier age.

Research shows that about one third of people with Down syndrome over 50 years develop dementia, which is creating new questions and challenges for families, support workers and health services.

Two leading Australian scientists analysed all the available research around this subject which they published last year.

Currently in New Zealand, there is no coordinated effort from the health authorities to prepare the sector for the ageing population with Down syndrome, so people on the frontline are left to come up with their own solutions, in their own community.

Hohepa Canterbury in Christchurch provides residential care and other services for people with intellectual disabilities and may be the only organisation in the country with a dedicated dementia unit for people with Down syndrome.

General manager Arnah Trelease explains that people with Down syndrome and dementia need specific care and staff need specialist training, which Hohepa originally was not able to offer.

“When the first people presented with dementia, they had to move to rest homes, where sadly they would not last very long and usually pass away within six months,” says Trelease.

Moving their clients away from Hohepa, however, was contrary to the commitment the organisation had made to its members that they could live out their days in their own environment.

“Dougie Brown was one of our original clients back in 1964 and when he started developing dementia, his mother Jendy refused to let him go to a rest home and reminded us of our commitment to our people.”

Hohepa considered its options and decided to turn Dougie’s house, Rose Cottage, into a dementia unit within Hohepa.

“We had to move Dougie’s house mates to different houses on our grounds, which got some resistance from the families, but they now tell us that we made the right decision,” says Trelease, who adds that the establishment of the dementia unit was only possible thanks to close collaboration with the Canterbury District Health Board.

“Tracey Hawkes from the CDHB has trained our staff in the Walking In Another’s Shoes programme to give them dementia related skills and helped us set up Rose Cottage.”

Juliet Nelson is the Integrated Support Manager running Rose Cottage and she says the big difference with a normal rest home are the staffing levels and the fact that people stay amongst their own people in a familiar environment.

The five clients who currently live at the cottage have two staff members, which is a significantly higher ratio than rest homes, which enables the staff to engage with the clients more one-on-one and find activities that fit the stages they are in.

“They remain part of our community and live as a family. They can watch the meals being prepared and sit around the table to have dinner,” says Nelson.

Hohepa has clients ranging from 6 to 82 years of age. “So they should not be moving out at the most vulnerable stage of their lives,” says Trelease.

“They should be able live out their lives in a familiar environment,” says Trelease who adds adds that the demand for dementia care is increasing and Hohepa are about to open a second unit.

“It feels like we’ve got a flood coming, and not just here. Other providers are contacting us because they may have one or two people getting dementia but are not able to set up their own units, so I think we will need a lot more collaboration in this space,” says Trelease.

Geraldine Whatnell on the other hand feels that the flood is more likely to be trickle.

Whatnell is part of the National Dementia and Intellectual Disabilities Steering Group and the first qualified Nurse Practitioner Dual Disabilties in New Zealand, specialising in intellectual disabilities and/or Autism Spectrum Disorder and associated mental health needs.

She says the most recent research indicates that the numbers of people with Down syndrome developing dementia may not be as high as assumed a few years ago.

Whatnell says he numbers may be inflated because many people with Down syndrome are incorrectly diagnosed as having dementia.

“Someone may present as being confused or off their food and doctors often go straight to dementia because they see someone with Down syndrome. In many cases, however, there could be other health reasons that can be easily reversed,” says Whatnell.

She says that people with Down syndrome are often diagnosed too late because they do not have the language skills that indicate any changes.  “So caregivers and health providers need to look for other indicators.”

The early warning signs for dementia is often epilepsy and starts presenting itself in the late 40s.

To help the doctors with their assessment, Whatnell hopes one day soon every person with Down syndrome will complete a baseline test when they are around 30 years, the Baseline Observation of Functioning For People with Intellectual Disabilities (BOFIID).

“And once you have that baseline doctors can compare those data when that same person presents later in life,” says Whatnell, adding that early diagnosis can lead to better support, better quality of life and less anxiety for the families.

To get a clearer picture if there is any onset of dementia in their 40s, caregivers can also use another effective screening tool, the Dementia Screen Questionnaire for Individuals with Intellectual Disabilities (DSQIDD).

Unfortunately, the Ministry of Health does not have data on how many people with Down syndrome in New Zealand have dementia.

Toni Atkinson, Group Manager Disability Support Services at the Ministry of Health, says the Ministry is aware of the misdiagnosis issue, but hopes the DSQIID baseline will help health professionals and support workers to notice any earlier decline in functioning.
“New Zealand has a growing and ageing population and we recognise that people with Down syndrome and their families will also be looking to the future and what supports are available.”

Linda te Kaat  attended the New South Wales Down Syndrome Health Conference and reports how oral health is a major issue to consider for caregivers.

Oral health is the greatest health need for people with Down syndrome.  Oral disease shares common risk factors with cardiovascular disease, diabetes, cancer and chronic obstructive pulmonary disease. 

What is dental disease, and can it be prevented? 

 

  • Decaying teethA cavity can result in tooth enamel dissolving over time.  Early prevention can reverse tooth decay. 

 

  • Periodontal diseases This effects the gums and bone around the teeth.  Gingivitis is reversible and signs are red and swollen gums and bleeding on brushing.  There will be no signs on an X-ray and no pain and may be present in more than one tooth.  This develops more quickly in people with Down syndrome due to an altered immune reaction to plaque.  Bacteria invade below the gums to cause inflammation which can lead to bone loss around the tooth root if not treated.  X-rays are the only way to show if there is any bone loss. This also leads to bad breath and teeth can get loose or move and pain can occur on biting.    There is often no pain in late stages of this disease and it always requires dental treatment.     

 

  • Trauma to tooth or jaw.  Falls can cause dental trauma.  Any tooth that has been chipped, moved or discoloured needs immediate treatments.  Teeth that have been knocked out can be put back in again but never touch the root of the tooth and get the tooth to a dentist immediately.  Dead teeth do not hurt but can be infected.  Falls on the chin with problems opening the mouth may be a fracture to the jaw. 

 

  • TMJ (Temporomandibular joint & muscle disorders). This is caused by trauma to the jaw or TM joint or by grinding teeth from stress.  That symptoms to look out for are  pain or stiffness in the chewing muscles (often one sided), limited movement or locking of the jaw, painful clicking and popping or grating in the jaw on opening. This conditions tends to be more common in woman.  The treatment is often simple and in most cases the discomfort will go away with little or no treatment.  Eat small pieces of food, apply ice packs or heat packs.  Avoid extreme jaw movements like wide yawning and chewing gum.  Short term use of Nurofen may be useful. 

 

Without X-rays, up to 40% of decay can be missed and therefore it is vital that these are carried out routinely.   

Toothbrushing requires the same manually dexterity as handwriting and many people with Down syndrome find this difficult therefore regular dental care is required.   

Often cysts may not be painful, and antibiotics can stop the pain and infection, but infection can still be in the bone and not picked up without an X-ray.   

Chronic pain can lead to changes in behaviour and people with Down syndrome can have a high pain tolerance therefore once it reaches the level of pain it can sometimes be a major dental problem.  
Always use toothpaste with fluoride in it and never rinse the mouth after brushing.   

Savacol is good for plaque build-up and a mouthwash with fluoride is ideal.  This can also be used on a toothbrush to brush around teeth if they person is unable to swish and spit.    

The Oral-B electric toothbrush was also recommended as not only one of the cheapest but also the best to use but sometimes electric toothbrushes are not tolerated by our young onesso use gradually to get used to it and start at a young age.   

 

You should seek professional care when there is: 

  • Any swelling of the mouth, face or neck.   
  • Any ulcer that does not heal 
  • Any persistent burning of the mouth 
  • Any severe pain in the teeth – even if it stops 

 

For people that cannot explain their symptoms, watch out for a change in eating habits, oral behaviour or bad breath 

 Before going for treatment, discuss with the person with Down syndrome what is going to happen and use books to illustrate what they can expect. 

Always keep the language positive and if possible try to have a first visit as an introduction without any serious treatment. 

 

UNDER ATTACK

Is the Down syndrome community under threat?

New Zealand is on the cusp of introducing more advanced antenatal testing for Down syndrome. COEN LAMMERS investigates if the Down syndrome community should feel under attack and looks at the ethical, medical and social issues at the heart of this debate.

Pre-natal screening for Down syndrome and other genetic disorders has long been a contentious issue in New Zealand and abroad.

The introduction of more sophisticated and conclusive testing methods has increased termination rates and triggered a global debate around screening ethics, the value of a life with a disability and state-sponsored elimination of Down syndrome.

In some European countries, babies with Down syndrome have become a rarity.

Iceland has reportedly reached a 100% termination rate, closely followed by Denmark where in 2017 only four new babies were born with Down syndrome.
Whether you agree with the abortion policies in those countries or not, the undeniable fact is that in many countries young people with Down syndrome will soon become an uncommon sight, or worse, an unwanted anomaly.

A striking example of the growing sentiment that Down syndrome is a burden that some societies can do without, was highlighted last year in the Netherlands where some media commentators debated whether Dutch women had a moral duty to abort their babies with Down syndrome.

Their comments were based on a list published by the Dutch Ministry of Health of the most expensive diseases and conditions which rated Down syndrome as the most expensive condition to the tax payer.

This list and the articles were widely discredited, challenged and condemned as biased because it conveniently ignored major health areas like cancer.
This discussion triggered one Dutch physician to tweet that “We haven’t come this close to Nazi before,” referring to the systematic extermination of disabled people in Nazi Germany.

This quote may sound dramatic, but the overseas trends and policies in some of these so-called enlightened and modern societies have caused strong concerns in the global Down syndrome community about the value their own society puts on a life with Down syndrome.

Unfortunately, as they are introducing more effective screening programmes, most countries have not had a wide public discussion around this sensitive issue.
Statistics from the United Kingdom show that the termination rate of women who have had a positive screening test, has not changed over the past few decades and remains around a staggering 90%.

What has changed with the introduction of more sophisticated tests, is the actual number of positive tests and subsequent abortions in the UK, increasing from 482 in 2010 to 706 in 2016.

This year the National Health Service in the UK is rolling out the Non-Invasive Prenatal Testing (NIPT) which gives women a near conclusive result on whether their baby will have Down syndrome.

Some critics in the UK have argued against the introduction of the test, fearing Icelandic statistics.

The NIPT test is not yet widely available in New Zealand and Dr Jane O’Hallahan, Clinical Director of the National Screening Unit, says that a slower uptake can in this instance give New Zealand an advantage and the opportunity to have a debate on how to introduce the test in a responsible manner.

“We need to tread very carefully around the ethics and the management of introducing this test in New Zealand,” says Dr O’Hallahan.

The NIPT is already privately available in New Zealand but Dr O’Hallahan says that the Ministry of Health is likely to make the test available, initially for women with a higher chance of having a baby with Down syndrome or other genetic disorders.

Dr O’Hallahan understands why the overseas statistics cause concern in the New Zealand Down syndrome community and is adamant that the Ministry of Health does not share the views of their European counterparts.

“There is no agenda to terminate lives with Down syndrome.”

Unfortunately, the Ministry of Health does not keep any records on positive tests for Down syndrome and terminations.

Dr O’Hallahan says that roughly the same number of children with Down syndrome have been born in New Zealand in recent years and from that she concludes that improved testing has not triggered a rise in terminations.

The National Screening Unit has created a working group of stakeholders to look at ways of improving informed choice for all ethnicities. This working group includes New Zealand Down Syndrome Association National Executive Zandra Vaccarino and board member Kim Porthouse.

Dr O’Hallahan says these stakeholders are providing valuable insights, beyond the medical discussions.

Mrs Porthouse says that the NZDSA wants the medical sector to understand that the screening debate is a social issue, not just a medical issue. “And the social implications need to be part of these discussions.”

She has an interesting perspective as a midwife and a mother of a son with Down syndrome and feels that there are two specific issues at stake in the screening discussions.
In her view, the pregnant women firstly need to be better informed before they decide to have any tests at all, and if they receive a positive test, the women need to get more balanced information about Down syndrome.

Mrs Porthouse says that most women think the tests are standard and don’t really consider the impact the results can have and the sudden life-changing decisions they may be facing.

“People think they are just going to take a picture when they get their first scan,” says Mrs Porthouse who feels that pregnant women should be better informed that the reason for the 12-week scan is for an NT (Nuchal Translucency) scan as part of the MSS1 (Maternal Serum Screening) screen.

The Ministry of Health has produced a brochure on Screening for Down syndrome, which clearly spells out the options and the choices women have, but Mrs Porthouse doubts if the brochure is used widely at present.

“Women should be offered the option to take this away to read through before deciding to screen, but often due to timing, most decide at the initial booking appointment.”
If they undertake the screening and it detects a higher risk or chance of Down syndrome, the families are forced to make quick, big decisions.

They need to make a call on having an amniocentesis, which carries a small risk of losing the baby, or in some centres they may be offered the NIPT test, which is expensive. They also need to consider what impact a baby with Down syndrome may have on their lives.

If the additional more-conclusive testing indicates the baby has Down syndrome, the medical provider will explain what Down syndrome is, but in most cases this talk will merely feature a long list of medical conditions the child may or may not develop during their lives.

“The doctors will cover themselves for everything, even if there is just a minimal chance the child will ever have those issues,” says Mrs Porthouse. Mrs Porthouse says the women are not being counselled on the wider social issue and positive impact a child with Down syndrome could have on their lives and community. “These families need to get the chance to talk to parents who are living with Down syndrome or other people who have faced the same decision.”

Instead, she is aware of anecdotal evidence that women often feel pressured to terminate their pregnancy. “It is a very emotional time for the families and it is hard to think clearly, so if you are only presented with a long list of all the things that can go wrong with your child, it is not hard to see that people opt for termination. “In many cases, it can be a decision they regret,” says Mrs Porthouse who personally knows of parents who after termination have struggled with their decision. “In some cases, the people get to know more about Down syndrome or get to know someone and they find out it was not at all what the doctors had made them believe. For some it has been devastating.”

Dr Jane O’Hallahan is aware of the cases in which women feel pressured to terminate their pregnancy. “But we don’t know how widespread this, but there should be no pressure.”
The Clinical Director of the National Screening Unit says that the Ministry of Health is aware of the issues and is continuously improving information going to women. “However the introduction of NIPT in the future will require an overhaul of the informed choice process to enable women to make the right decision for them and their families,” says Dr O’Hallahan, who admits that her colleagues often portray a future with Down syndrome in a negative light.

Dr O’Hallahan says that the information provided is “over-medicalised” and is not a social discussion. “It does not consider the value of people with Down syndrome or the fact that these people add value to so many lives of others.”

As an example, the Director mentions her own daughter who had the privilege of following a family with a newborn with Down Syndrome during her medical studies – a lovely experience that would benefit all medical students.

Dr O’Hallahan says that the feedback from the NZDSA and other stakeholders on the working group had been valuable to show the social side of Down syndrome and that the National Screening Unit is more focused on counselling and providing better education for medical professionals and medical students about Down syndrome. “We are currently putting a lot of effort into giving more holistic information to give a real picture of what life with Down Syndrome is like. We are investing to give women the right information, so they can make the right decision for them and their family.”

 

ANTENATAL SCREENING

What are all the tests and what do they mean.

NT scan: Nuchal Translucency ultrasound scan performed around 12 weeks (range 11 weeks to 13 weeks, 6 days). It measures the fluid in the nuchal space at the back of the foetal neck. The old test used to combine just the scan with maternal age to give a risk of Down syndrome. Risk results are no longer provided on NT scan alone as they are less accurate compared to MSS1 or MSS2 screens. The NT scan is now only used in conjunction with the MSS1 calculation.

MSS1 screen: Maternal Serum Screening (combined blood test – 2 markers – and NT scan) performed in the first trimester of pregnancy until 13 weeks 6 days gestation. Current government funded screen, offered to all women who engage with antenatal care in the first trimester.

MSS2 screen: Maternal Serum Screening (4 marker blood test, no scan) performed between 14 weeks to 20 weeks gestation. Accuracy levels are said to be about the same as MSS1 screen, offered to all women who engaged with services too late for MSS1 screening or those who prefer not to have ultrasound scans of their pregnancy. Government funded.

NIPT (or NIPS) test: Non-Invasive Prenatal Screening. Blood test only (new blood test which is different to MSS1), is said to be 99+% accuracy. Currently not government funded in New Zealand and expensive.

Amniocentesis: diagnostic test in which a needle under ultrasound guidance is used to draw fluid from around the foetus. This fluid contains foetal cells which are used to look at chromosomes to see if Trisomy 21 (Down Syndrome) exists. Carries a risk of miscarriage of 0.5 to 1.0% (1:100 – 1:200).