Family Devastated After Brothers, 2 and 6, Both Diagnosed With Dementia

Following a terrifying diagnosis, one family is having to come to terms with the fact they may only have a limited number of years left with their two boys, who will struggle with seizures, slow development and childhood dementia due to a rare disease.

When Kristian Tucker, 31, and Bryden Tucker, 34, welcomed their third child in 2021, they relished life as a new family of five. But when their eldest child, Michael, now 6, started showing signs of delayed speech and seizures, the bubble began to burst.

Kristian Tucker, from Spokane, Washington, told Newsweek that Michael's seizures began at age 3 and "continued to get worse as he got older." Medication wasn't working, which left the doctors perplexed as to what the condition could be.

In February, the results of a genetic test led to a devastating diagnosis which resigned Michael to a short and difficult life, marred by development delays, loss of motor skills, and a cognitive decline resulting in dementia at an early age.

"After the genetic test, we were told that Michael has Batten disease type 2 in April 2023. We had never heard of it before, but I was horrified. When we first found out about it, we were told the average life span is about eight to 12 years," Tucker said. "I couldn't believe that I might only have a couple of years left with my baby and that he would suffer so much."

What Is Batten Disease?

Batten Disease is a group of nervous system disorders that start during childhood that can also be referred to as neuronal ceroid lipofuscinosis (CLN). The fatal disease has many forms, but it's categorized by the presence of a CLN gene which leads to the disorder. The symptoms of the disease can vary for each case, but they often include the loss of vision and eventual blindness, seizures, movement disorder and childhood dementia.

A child's development can be heavily impacted by Batten disease, according to the National Institute of Neurological Disorders and Stroke. Skills such as standing, walking, and talking may never be accomplished, or the child can lose their abilities over time.

'I Still Don't Understand How They Can Have This Disease'

The diagnosis was shocking for the Tucker family, but they had no time to think about the repercussions before they were hit by the next blow. Michael's two siblings, Talia, 4, and Oliver, 21 months, also had to be tested for the condition as it's not uncommon for the disease to affect multiple siblings.

It's estimated by the Cleveland Clinic that Batten disease affects about three in every 100,000 births in the United States, but siblings of children with the disease have a 25 percent chance of also having the disorder.

In May 2023, a month after discovering Michael's fate, the Tucker family was devastated once again to find out that Oliver also has the gene.

"I felt broken. These are my baby boys, the sweetest, happiest little boys. I still don't understand how they can have this disease. It has devastated us all, but we are trying to make sure the boys have the best life we can give them by filling their little lives with experiences and family," Tucker said.

"So far the only developmental issue they have had is with their speech; Michael seems to have more severe speech delays than Oliver. Oliver can only say a few words, and Michael was diagnosed with apraxia prior to his diagnosis, and he still struggles a lot with his speech. The only other symptom Michael has is the absence seizures," she said.

"Neither boy has shown any signs of dementia, and we hope to have years before that happens.

"We don't really know, but the doctor thinks that the boys will have more time than we usually see, based on how some of their test results have come back. We feel as if the ticking time bomb that is this disease just got a longer wick, but we're also reminded that it's still a bomb."

After the shock diagnoses revealed that two of their children have the cruel disease, the family is considering whether going through treatment would be the correct decision. While there is no treatment or cure for Batten disease, the Food and Drug Administration has approved the use of cerliponase alfa, which is an enzyme replacement therapy. The process involves inserting a port into the child's brain and then injecting the enzyme they're missing directly into the brain.

The treatment can be used on children over the age of 3 to slow down the loss of movement and physical ability. It isn't an easy call for the Tucker family to make, though, as they explained that it's an "invasive treatment" which isn't without its risks.

"It's such a struggle and it's something we are deciding with the doctors if the benefits out way the risk and potential harm," Tucker told Newsweek. "But if it gives them more time for a potential cure or something to stop these symptoms, I think that might be what is best for them. But we are still deciding.

"If we go forward with treatment, we hope Michael's seizures will stop getting worse and that we can slow, or even stop, the loss of mobility," she said. "However, it won't stop him from losing his eyesight or from the childhood dementia from happening. With Oliver, we hope it will prevent him from ever having seizures, but again, it won't stop the rest from happening."

'There Is Often Very Little We Can Do'

Dr. Josh Bonkowsky is the chief of the pediatric neurology division at the University of Utah Health and Intermountain Primary Children's Hospital. As the hospital covers a five-state region, Bonkowsky has come across cases of Batten Disease personally, telling Newsweek they see "one or two newly affected children."

"Unfortunately, for most types of Batten disease there are no treatments that have been proven to work," he said. "Families and their physicians are all very dedicated to finding options, but so far it is very limited.

"For Batten disease caused by CLN2, there is a treatment with an infusion of the missing enzyme, directly into the fluid space inside and around the brain. This is a complicated process and requires a pediatric neurosurgeon to perform a surgery to place a special port that connects to this fluid space where the enzyme is delivered.

"This infusion has to happen at least every other week. The enzyme treatment is expensive and time-consuming, has some risk of possible infections, and stops or slows the disease, but is not a complete cure."

Having seen the devastating effects that this "terrible disease" can cause, Bonkowsky added that until there is a cure, "there is often very little that we can do."

"The most important thing to help a family with the disease is to be there for them. The disease and treatment journey can be frightening, but knowing that you are not doing it alone makes a big difference," he said.

"We are at the point where the technology and science have advanced to make treatment and cures a real possibility. We have an opportunity, by supporting children and families, and by supporting children's hospitals and their physicians and scientists, to make it curable."

'We Want To Get The Word Out And Raise Awareness'

In June, the family opened up about their experience by creating a Facebook group called Michael and Oliver's fight against Batten Disease. They aim to create awareness for the condition, which very few people even know about.

Tucker said: "This is such a rare disease, so we want to get the word out and raise awareness. We are hoping that maybe we can get opportunities that may prolong and improve their lives.

"One of the most rewarding things about putting our story out into the world is that more people know about the disease than before. We do hope to connect and hear the stories from others going through this, but right now, hearing others' struggles, seeing what is waiting for our boys in the coming months and years is still too terrifying, too raw."

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