What is childhood dementia?

Childhood dementia is a rare condition that affects children and their families all over the world. Unlike adult dementia, which is well-known, childhood dementia is a relatively unknown condition. However, it affects approximately one in every 2,900 babies worldwide and is associated with over 100 rare genetic disorders. This frequently misrecognised condition, causes progressive brain damage and cognitive decline, leaving children with a difficult and debilitating condition that deprives them of their abilities and quality of life.

Overview of Childhood Dementia

Childhood dementia is not a single disease, but a term describing a group of childhood dementia disorders. These disorders result in a progressive decline in both the brain and the body that can impair the child’s ability to think, move, speak, and, ultimately, make sense of the world.

As a result, children can suffer from symptoms that are similar to those found in adults with dementia but the consequences for those children can be far more devastating. They often rob the child of the ability to develop, learn and grow into their own person in their formative years.

It is caused by many rare genetic diseases, including Sanfilippo syndrome, Batten disease and Rett syndrome. These genetic conditions cause progressive damage to the brain resulting in a gradual loss of mental and motor skills. In some children, the genetic disease has been present at birth, but, in others, it might not manifest itself until later in their development.

Childhood dementia is a challenging condition because it can manifest at any point from infancy through to adolescence. With around 700,000 children globally currently living with this condition, there must be an increase in awareness and support.

Symptoms and Early Signs

Recognising the symptoms of childhood dementia can be difficult because they vary depending on the underlying disorder and the child’s age. Common childhood dementia symptoms include memory loss, confusion, and a decline in cognitive abilities such as problem-solving, language, and reasoning.

Additionally, children may exhibit behavioural issues like hyperactivity, difficulty concentrating, and emotional disturbances such as anxiety or fear. Physical symptoms are also prevalent, including seizures, problems with motor skills, and loss of vision or hearing. In some cases, childhood dementia symptoms may be apparent from infancy, while in others, they may not manifest until the child reaches their teenage years. This variability in symptom onset makes early diagnosis challenging but crucial for managing the condition effectively.

As the disease advances, children lose their ability to talk, walk, read, and write. Eventually, they may become entirely dependent on others for their care as they lose the ability to perform simple tasks.

Causes of Childhood Dementia

Childhood dementia is linked to more than 100 rare genetic conditions, many of which are poorly understood and have received little attention from the medical community. These genetic disorders include, but are not limited to, Sanfilippo syndrome, Batten disease, Rett syndrome, and cystic fibrosis.

The underlying causes of childhood dementia are genetic in nature, meaning that children are born with these disorders, though symptoms may not become apparent until later in life. In some cases, genetic testing can identify these conditions before symptoms begin, allowing families to prepare for the challenges ahead.

Several categories of childhood dementia disorders exist, including:

• Inborn Errors of Metabolism: These are rare genetic disorders that affect the body's metabolism, leading to the accumulation of toxic substances that cause brain damage.
• Lysosomal Disorders: These are caused by the malfunction of lysosomes, the cellular components responsible for breaking down waste materials in the body. When lysosomes fail, waste accumulates, leading to progressive brain damage.
• Mitochondrial Disorders: These genetic conditions affect the mitochondria, the powerhouses of the cell. When mitochondria malfunction, cells cannot produce the energy needed to function, leading to widespread organ damage, including in the brain.
• Mucopolysaccharidoses: These are a group of metabolic disorders caused by the absence or malfunctioning of certain enzymes. The resulting accumulation of complex carbohydrates in the body leads to severe physical and neurological damage.
• Peroxisomal Disease: These disorders affect the peroxisomes, cellular structures involved in breaking down fatty acids and other compounds. Dysfunctional peroxisomes lead to the accumulation of harmful substances in the brain.
• Neurodegeneration with Brain Iron Accumulation (NBIA): A group of rare genetic disorders that cause iron to accumulate in the brain, leading to progressive cognitive and physical decline.
• Leukodystrophy: A group of disorders that affect the white matter of the brain, leading to a decline in motor skills and cognitive abilities.

Understanding the causes of childhood dementia is critical for developing treatments and supporting families afflicted by this debilitating disease. Unfortunately, due to their rarity, these genetic conditions have historically gotten little attention from the medical community. However, initiatives such as the childhood dementia initiative and the childhood dementia working group are attempting to change this.

The Role of Professional Carers

There is no cure for childhood dementia but carers can help families manage the condition and improve the quality of life for affected children. Although Tiggo Care does not offer services to children, the ideals of compassionate, tailored care that we advocate are vital in treating childhood dementia patients.

Professional carers can assist with personal care, medication adherence, and behavioural control. Carers offer emotional support to the child and their family as they manage the difficulties of living with the condition. Furthermore, care assistants support the physical signs of childhood dementia, such as mobility challenges and loss of motor skills, to keep the kid as comfortable and active as possible.

Professional carers in the United Kingdom are unable to manage or treat childhood dementia, but they can work closely with families and healthcare professionals to support the child's care plan. This collaborative approach provides the best possible care since it addresses all aspects of the child's condition in a thorough and caring manner.

Conclusion

Childhood dementia is a rare but serious illness that impacts the lives of thousands of children and their families around the world. Although this disorder is very much like adult dementia, the symptoms in young people have a more substantial impact. The effects are more rapid, with the condition quickly leading to cognitive and physical decline. Professional carers play a vital role in supporting people with this disorder, as they require special care and attention.

Although Tiggo Care works with adult care, the concepts of empathy, person-centred care, and holistic support apply to every care context; we hope that drawing attention to childhood dementia and the problems experienced by affected families might make a small contribution to improving the lives of those with this terrible disease.

If you have a child with childhood dementia, know that help is available. GPs, professional carers, healthcare providers, and support groups can offer information and assistance to help you and others look after your child and keep them safe.