“Childhood” and “dementia” are two words we wish we didn’t have to use together. But sadly, around 1,400 Australian children and young people live with currently untreatable childhood dementia.

Broadly speaking, childhood dementia is caused by any one of more than 100 rare genetic disorders. Although the causes differ from dementia acquired later in life, the progressive nature of the illness is the same.

Half of infants and children diagnosed with childhood dementia will not reach their tenth birthday, and most will die before turning 18.

Yet this devastating condition has lacked awareness, and importantly, the research attention needed to work towards treatments and a cure.

More about the causes

Most types of childhood dementia are caused by mutations (or mistakes) in our DNA. These mistakes lead to a range of rare genetic disorders, which in turn cause childhood dementia.

Two-thirds of childhood dementia disorders are caused by “inborn errors of metabolism”. This means the metabolic pathways involved in the breakdown of carbohydrates, lipids, fatty acids and proteins in the body fail.

As a result, nerve pathways fail to function, neurons (nerve cells that send messages around the body) die, and progressive cognitive decline occurs.

What happens to children with childhood dementia?

Most children initially appear unaffected. But after a period of apparently normal development, children with childhood dementia progressively lose all previously acquired skills and abilities, such as talking, walking, learning, remembering and reasoning.

Childhood dementia also leads to significant changes in behaviour, such as aggression and hyperactivity. Severe sleep disturbance is common and vision and hearing can also be affected. Many children have seizures.

The age when symptoms start can vary, depending partly on the particular genetic disorder causing the dementia, but the average is around two years old. The symptoms are caused by significant, progressive brain damage.

Are there any treatments available?

Childhood dementia treatments currently under evaluation or approved are for a very limited number of disorders, and are only available in some parts of the world. These include gene replacement, gene-modified cell therapy and protein or enzyme replacement therapy. Enzyme replacement therapy is available in Australia for one form of childhood dementia. These therapies attempt to “fix” the problems causing the disease, and have shown promising results.

Other experimental therapies include ones that target faulty protein production or reduce inflammation in the brain.

Research attention is lacking

Death rates for Australian children with cancer nearly halved between 1997 and 2017 thanks to research that has enabled the development of multiple treatments. But over recent decades, nothing has changed for children with dementia.

In 2017–2023, research for childhood cancer received over four times more funding per patient compared to funding for childhood dementia. This is despite childhood dementia causing a similar number of deaths each year as childhood cancer.

The success for childhood cancer sufferers in recent decades demonstrates how adequately funding medical research can lead to improvements in patient outcomes.

Another bottleneck for childhood dementia patients in Australia is the lack of access to clinical trials. An analysis published in March this year showed that in December 2023, only two clinical trials were recruiting patients with childhood dementia in Australia.

Worldwide however, 54 trials were recruiting, meaning Australian patients and their families are left watching patients in other parts of the world receive potentially lifesaving treatments, with no recourse themselves.

That said, we’ve seen a slowing in the establishment of clinical trials for childhood dementia across the world in recent years.

In addition, we know from consultation with families that current care and support systems are not meeting the needs of children with dementia and their families.

New research

Recently, we were awarded new funding for our research on childhood dementia. This will help us continue and expand studies that seek to develop lifesaving treatments.

More broadly, we need to see increased funding in Australia and around the world for research to develop and translate treatments for the broad spectrum of childhood dementia conditions.

Dr Kristina Elvidge, head of research at the Childhood Dementia Initiative, and Megan Maack, director and CEO, contributed to this article.

Kim Hemsley, Head, Childhood Dementia Research Group, Flinders Health and Medical Research Institute, College of Medicine and Public Health, Flinders University; Nicholas Smith, Head, Paediatric Neurodegenerative Diseases Research Group, University of Adelaide, and Siti Mubarokah, Research Associate, Childhood Dementia Research Group, Flinders Health and Medical Research Institute, College of Medicine and Public Health, Flinders University

This article is republished from The Conversation under a Creative Commons license. Read the original article.

The holiday season is meant to be filled with joy, connection and celebration of rituals. Many people, however, are starkly reminded of their grief this time of year and of whom – or what – they have lost.

The added stress of the holiday season doesn’t help. Studies show that the holidays negatively affect many people’s mental health.

While COVID-19-related stressors may have lessened, the grief from change and loss that so many endured during the pandemic persists. This can cause difficult emotions to resurface when they are least expected.

I am a licensed therapist and trauma-sensitive yoga instructor. For the last 12 years, I’ve helped clients and families manage grief, depression, anxiety and complex trauma. This includes many health care workers and first responders who have recounted endless stories to me about how the pandemic increased burnout and affected their mental health and quality of life.

I developed an online program that research shows has improved their well-being. And I’ve observed firsthand how much grief and sadness can intensify during the holidays.

Post-pandemic holidays and prolonged grief

During the pandemic, family dynamics, close relationships and social connections were strained, mental health problems increased or worsened, and most people’s holiday traditions and routines were upended.

Those who lost a loved one during the pandemic may not have been able to practice rituals such as holding a memorial service, further delaying the grieving process. As a result, holiday traditions may feel more painful now for some. Time off from school or work can also trigger more intense feelings of grief and contribute to feelings of loneliness, isolation or depression.

Sometimes feelings of grief are so persistent and severe that they interfere with daily life. For the past several decades, researchers and clinicians have been grappling with how to clearly define and treat complicated grief that does not abate over time.

In March 2022, a new entry to describe complicated grief was added to the Diagnostic and Statistical Manual of Mental Disorders, or DSM, which classifies a spectrum of mental health disorders and problems to better understand people’s symptoms and experiences in order to treat them.

This newly defined condition is called prolonged grief disorder. About 10% of bereaved adults are at risk, and those rates appear to have increased in the aftermath of the pandemic.

People with prolonged grief disorder experience intense emotions, longing for the deceased, or troublesome preoccupation with memories of their loved one. Some also find it difficult to reengage socially and may feel emotionally numb. They commonly avoid reminders of their loved one and may experience a loss of identity and feel bleak about their future. These symptoms persist nearly every day for at least a month. Prolonged grief disorder can be diagnosed at least one year after a significant loss for adults and at least six months after a loss for children.

I am no stranger to complicated grief: A close friend of mine died by suicide when I was in college, and I was one of the last people he spoke to before he ended his life. This upended my sense of predictability and control in my life and left me untangling the many existential themes that suicide loss survivors often face.

How grieving alters brain chemistry

Research suggests that grief not only has negative consequences for a person’s physical health, but for brain chemistry too.

The feeling of grief and intense yearning may disrupt the neural reward systems in the brain. When bereaved individuals seek connection to their lost loved one, they are craving the chemical reward they felt before their loss when they connected with that person. These reward-seeking behaviors tend to operate on a feedback loop, functioning similar to substance addiction, and could be why some people get stuck in the despair of their grief.

One study showed an increased activation of the amygdala when showing death-related images to people who are dealing with complicated grief, compared to adults who are not grieving a loss. The amygdala, which initiates our fight or flight response for survival, is also associated with managing distress when separated from a loved one. These changes in the brain might explain the great impact prolonged grief has on someone’s life and their ability to function.

Recognizing prolonged grief disorder

Experts have developed scales to help measure symptoms of prolonged grief disorder. If you identify with some of these signs for at least one year, it may be time to reach out to a mental health professional.

Grief is not linear and doesn’t follow a timeline. It is a dynamic, evolving process that is different for everyone. There is no wrong way to grieve, so be compassionate to yourself and don’t make judgments on what you should or shouldn’t be doing.

Increasing your social supports and engaging in meaningful activities are important first steps. It is critical to address any preexisting or co-occurring mental health concerns such as anxiety, depression or post-traumatic stress.

It can be easy to confuse grief with depression, as some symptoms do overlap, but there are critical differences.

If you are experiencing symptoms of depression for longer than a few weeks and it is affecting your everyday life, work and relationships, it may be time to talk with your primary care doctor or therapist.

A sixth stage of grief

I have found that naming the stage of grief that someone is experiencing helps diminish the power it might have over them, allowing them to mourn their loss.

For decades, most clinicians and researchers have recognized five stages of grief: denial/shock, anger, depression, bargaining and acceptance.

But “accepting” your grief doesn’t sit well for many. That is why a sixth stage of grief, called “finding meaning,” adds another perspective. Honoring a loss by reflecting on its meaning and the weight of its impact can help people discover ways to move forward. Recognizing how one’s life and identity are different while making space for your grief during the holidays might be one way to soften the despair.

When my friend died by suicide, I found a deeper appreciation for what he brought into my life, soaking up the moments he would have enjoyed, in honor of him. After many years, I was able to find meaning by spreading mental health awareness. I spoke as an expert presenter for suicide prevention organizations, wrote about suicide loss and became certified to teach my local community how to respond to someone experiencing signs of mental health distress or crisis through Mental Health First Aid courses. Finding meaning is different for everyone, though.

Sometimes, adding a routine or holiday tradition can ease the pain and allow a new version of life, while still remembering your loved one. Take out that old recipe or visit your favorite restaurant you enjoyed together. You can choose to stay open to what life has to offer, while grieving and honoring your loss. This may offer new meaning to what – and who – is around you.

If you need emotional support or are in a mental health crisis, dial 988 or chat online with a crisis counselor.

Mandy Doria, Assistant Professor of Psychiatry, University of Colorado Anschutz Medical Campus

This article is republished from The Conversation under a Creative Commons license. Read the original article.