What is terminal Juvenile Batten disease?

What is terminal Juvenile Batten disease?

CLN3, often called juvenile Batten disease, is an ultra-rare, fatal, inherited disorder that primarily affects the nervous system and left untreated, is fatal. Children with CLN3 disease develop normally, even excelling in school until ages 5–6 years, when progressive vision loss becomes noticeable.

Can you survive Batten’s disease?

Children with Batten disease have a greatly shortened life expectancy. Children with infantile Batten disease often die in early childhood. Children with later onset forms of the disease may live into their teens to thirties, while those who develop the disease in adulthood may have a normal life expectancy.

Is there a cure for juvenile Batten disease?

There currently is no cure for juvenile Batten disease. Thus, available treatments focus on easing its symptoms. Seizures can be controlled with anticonvulsants. Because the types of seizures change over time, patients usually receive more than one kind of anticonvulsant over the course of their disease.

Why is Batten disease fatal?

The disorder affects the cells’ ability to break down and get rid of cellular waste. The body can’t dispose of proteins, sugars and lipids (fats), so they build up. This buildup causes problems with the nervous system that eventually leads to death. There is no cure for Batten disease.

What is the life expectancy of Batten disease?

The life expectancy is between ages eight to 10. Juvenile Batten disease occurs in children between ages five and 10. These patients usually live until their late teens or early 20s.

How do you treat Batten disease?

The only treatment approved by the U.S. Food and Drug Administration to treat Batten disease is Brineura (cerliponase alfa), an enzyme replacement therapy designed to slow the loss of walking ability in children with a type of Batten disease called CLN2.

What disease did Kennedy Hansen have?

Kennedy Hansen had already lost her vision and most of her motor skills to Batten disease by 2013, but when the head of her cheerleader squad went around a circle at practice one day asking each member to name their life challenge, Hansen astounded everyone, including her parents, by replying she had none.

What disease did love Kennedy have?

A teenage girl copes with her diagnosis of terminal juvenile Batten disease.

What is the treatment regimen for Battens disease?