Couple loses 3 children in 3 days to rare disease that robs children of their sight, memory and …


The parents of 10 children had to make the brutal decision to take two of their kids off life support and let a third child die during one weekend last July.

Les and Celeste Chappell lost Christopher, 20, Elizabeth, 19, and James, 15, to a rare illness called Batten disease in the span of three days this past summer.

And one of their younger sons, Samuel, is still living with the genetic disorder, which usually kills children by their early 20s.

Batten disease, for which there is no treatment, causes children to lose their ability to see, speak and remember things, and the rare ailment eventually renders them unable to swallow, which chains them to life support.

Les and Celeste first thought about the decisions they would have to make in regards to their children’s care over ten years ago, according to the Washington Post.

Elizabeth, James and Christopher were in bad health, and the pair had taken them to a number of specialists, which is when they first learned about Batten disease.

They knew that a Batten disease diagnosis meant an early death but the symptoms of the illness matched what they were witnessing: Christopher and Elizabeth had bad vision and their children were having seizures.

This led to a conversation one evening in 2005 during a date night, in which Les and Celeste confronted the harsh reality they were being presented with.

Les admitted that he had been researching the disease they had been told about and that he thought that it was what was plaguing his family, and Celeste agreed.

Then, together, they discussed the fact that, if their suspicions about their children’s health were correct, they would eventually have to make a decision to either keep their children alive with tubes or let them die.

Soon after this conversation, genetic testing confirmed their worst fears, and Elizabeth, James and Christopher were diagnosed with Batten disease.

And the family’s heartbreak over the fatal diagnosis did not end there: at the time, Celeste was pregnant with another son, Samuel, who would eventually be diagnosed with Batten disease.

Les and Celeste Chappell (pictured left to right) have a younger child named Samuel who has the same genetic disorder that killed three of his older siblings

Les and Celeste Chappell (pictured left to right) have a younger child named Samuel who has the same genetic disorder that killed three of his older siblings

Les and Celeste Chappell (pictured left to right) have a younger child named Samuel who has the same genetic disorder that killed three of his older siblings

The Chappells, who live in Springville, Utah, had to adjust to the excruciating truth that their children would never grow up to lead the lives their parents had imagined them living.

Watching their children grow up, they had predicted that Christopher would be a doctor, Elizabeth would be a gymnast, and James a ballplayer.

Instead, they watched the trio’s health worsen overtime.

The children’s quality of life deteriorated rapidly, and they lost their ability to swallow, move, see and recall memories.

And when the time came for them to decide whether or not to let Elizabeth and James be tube fed, they decided they would.

But as Les and Celeste witnessed their bodies deteriorate before their eyes, they decided they would not tie Christopher to a similar fate.

WHAT IS BATTEN DISEASE?

Batten disease, also known as Neuronal Ceroid Lipofuscinoses, is a rare genetically-inherited disease.

Sufferers tend to live for 10 years after diagnosis.

It is caused by waste buildup in the cells which creates neurodegenerative effects including:

  • Blindness
  • Seizures
  • Personality changes
  • Dementia
  • Loss of motor skills and the ability to walk, talk and communicate

Because it is a rare disease, it is often difficult to diagnose and is untested at birth. 

Ways to diagnose: 

  • Blood or urine tests
  • Skin or tissue sampling
  • DNA analysis
  • Electrical studies of the eyes 

There are 14,000 children worldwide that are diagnosed with the disorder.

There is currently no known cure or treatment available for Batten disease.

And once that decision was made, the couple said it made sense to take their other two children off life support, as the tubes were only prolonging their eventual death. 

On the day that the children came off life support, their three hospital beds were lined up side by side in a sunny area of the Chappell’s living room.

James, Elizabeth and Christopher were given morphine and lorazepam so that they would die comfortably.

A hospice team warned Les and Celeste that it could take weeks for their children to die, which they told the Washington Post they were not prepared for.

But they were done with the gut-wrenching waiting period in a few days, as the children passed away quickly after they were taken off life support on Friday the 14.

The first of the Chappell’s children to die was Elizabeth, who died that day. James passed away the following day, and he was followed by his older brother Christopher, who died on Sunday the 17.

On Saturday night, the evening before Christopher, who survived the longest, died, Les and Celeste struggled to stay awake as they stuck by their son’s bedside.

Celeste told the Washington Post about their experience that evening, saying: ‘How can I sleep when my babies are dying?’

‘He’s gone!’ she had said after realizing that Christopher was no longer living. 

After each of the deaths, the children’s family members sat around their bodies and told stories remembering good times they had had with them.

One obituary was written for the three of them, who were buried together after their funeral service on Saturday, July 22.

It states that Elizabeth loved princesses, dolls and being outdoors, while James enjoyed sports, baseball especially. Christopher liked hanging out with his older brothers.



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