Skip to main content

Parents of children with juvenile Batten disease face many challenges, but can find value in small moments of joy, a new study points out.

Juvenile latte, also called CLN3 disease, is the most common type of inherited neurodegenerative disorder. Typically beginning between the ages of 5 and 7 with vision problems as the first symptom, patients experience cognitive difficulties, speech impairment, loss of motor skills and seizures as the disease progresses.

Illnesses like Batten impact not only the sufferers, but also their families. However, there has been little formal research on the experiences of parents and other family members affected by juvenile Batten disease.

To find out more, a team of scientists in Sweden interviewed eight parents – five mothers and three fathers – of five children with juvenile Batten. The parents were all in their 40s or early 50s, with children in their teens or 20s.

“This study is the first to our knowledge to examine the experiences of parenting a child with” juvenile Batten disease, the researchers wrote, noting that they focused on “its impact on the family system as well as on the concept of family resilience”.

recommended reading

The study, “Parental experiences of having a child with CLN3 disease (Juvenile Batten disease) and how these experiences relate to family resiliencewas published in Child: care, health and development.

Many parents reported feeling recurring feelings of loss and grief throughout the illness, from the time their child was diagnosed.

“We walked into a room with two healthy children and left with one dying,” a parent recalled after learning of the child’s diagnosis.

The sense of loss often persisted, and sometimes worsened, as the disease progressed, causing some parents to feel estranged from their children.

“He had a lot of psychotic symptoms which were horrible, it completely took over him, which made us lose more and more,” said a parent.

Many parents expressed the feeling that they had no opportunity to “recharge” or take time and space for themselves, feeling obligated to always be available for their children. These constraints often put a strain on the romantic relationships of the parents.

“Suddenly [a care] the assistant gets sick, and you can’t find a replacement, and then we have to step in, so you’re like a prisoner, in your own home, and you never know if you can take that trip or that dinner or go home to somebody a or something,” a parent said.

Another common theme was frustration with societal institutions that are not set up to support people with progressive diseases or with increasing care needs. Parents said they often sent in new insurance claims before previous ones had been processed.

‘These children should be given priority in the system and not have to wait the same way as others,’ one parent said. “Because this hierarchy doesn’t exist, things happen all the time with deteriorations (in function), so that when a thing is done, it’s already too late.”

Among parents with more than one child, a universal theme was feelings of guilt – believing that they had not been fully present as parents for their children without Batten.

“I have my grown son, he is my ‘rock’, it’s awful to say I have my son for that,” a parent said.

“Some parents described how the siblings yearned for a more ‘normal’ existence,” the investigators wrote.

Parents stressed the importance of trying to accommodate siblings’ feelings, even when they are complicated. Some parents said they felt growing up alongside a child with a rare disease helped their other children develop a deeper sense of empathy than their peers.

“This part is difficult, [the healthy sibling] sometimes hated [the ill sibling] and that it has to be allowed, that he can have those emotions and express those things, and I have to respond to that in an accepting way,” one parent said.

Another said the healthy brother ‘has become a much more mature and whole human being compared to his peers… It seems to have made him more responsible, more caring’.

Faced with these many challenges, some parents said they learned to derive great value from small moments of simple joy with their young Batten children.

“Several parents recounted occasions when they felt a deep connection with their children during the moments they shared,” the researchers wrote. “These moments could be, among other things, small pauses of calm in the midst of an intense outburst of anxiety, a hug, a smile or a laugh that communicates more than words.”

“I knew he wasn’t with me forever and I was his eyes, I was his voice, I was his, like it was my duty that he had the best during his time on earth is what led me, the love of [the child] … That was really the purpose of my life,” said one relative.

A number of parents spoke of the importance of finding a support group with others whose children also had juvenile Batten. Many have called for improvements to health insurance and more easily accessible support services.

“The results also indicate a need for consistent adult health care, with improved communication with families and caregivers, as well as increased knowledge of the disease and its impact on the family,” the researchers wrote.

The team said their study showed most parents were “resilient” to their child’s juvenile Batten diagnosis.

“Parents felt that the illness forced them to reconsider what was important in life, to value small moments of joy, and to create deep bonds by involving themselves in family routines and rituals,” wrote the team.

“Despite the challenges to the family system, it was possible to identify examples of family resilience,” they concluded.