What is late infantile Batten disease, what are the symptoms of Ollie and Amelia Carroll’s condition and how is it treated?
Batten disease is a neurological disorder that occurs in children
LATE Infantile Batten Disease is a neurological disorder that occurs in children between the ages of 2 and 4 and is an early onset form of Batten Disease.
Here's everything you need to know about the disease, from the symptoms to the treatment.
What is Batten Disease and how common is it?
Batten disease is a disorder of the nervous system which results in death and typically begins in childhood.
(National Institute of Neurological Disorders and Stroke), the disorder will usually start to appear in children between the ages of 5 and 10, who may have been previously completely well.
There are many forms of the disease, but Late Infantile Batten Disease occurs begins between ages 2 and 4.
The first signs of the disease are are loss of muscle coordination and seizures. , this form progresses rapidly and ends in death between ages 8 and 12.
Batten disease is very rare: it occurs in five or six births in the UK per year, while research finds they're more common in Finland, Sweden, other parts of northern Europe and Canada.
What are the symptoms of Batten Disease?
Parents may notice early symptoms including vision problems or seizures.
Some early signs can be subtle and include personality or behaviour changes like slow learning, clumsiness, or stumbling.
Symptoms get worse over time and might include cognitive impairment, worsening seizures, and progressive loss of sight and motor skills.
Later on in the disease, children become blind and get dementia and batten disease suffers will typically die by their late teens or twenties.
How is Batten Disease treated?
There is no cure to reverse the symptoms of Batten disease or other NCLs (neuronal ceroid lipofuscinoses).
Seizures can be treated with drugs and physical and occupational therapy can be beneficial to sufferers.
Support groups and associations like (Batten Disease Support and Research Association) are available to support families with childen who suffer from the life-changing disease.
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Lucy and Mike Carroll recently spoke about .
Their children, Ollie, six, and Amelia, four, both have the disease.
Every two weeks Ollie and Amelia, who need round-the-clock care, travel to Great Ormond Street in London for enzyme treatment.