Jean’s Story

A diagnosis she feared, but one she expected 

 By Stephanie Dickrell

 Jean Micke has spent more than two decades witnessing the slow destruction of her family members. It started with her father’s diagnosis with ALS in 1996. Art Weber died three years later. 

 Then came her sister Peggy a few years later, diagnosed at age 42. Ten years later, her sister Cindy was diagnosed in 2012 as was her brother Jim. Then in 2019, it was Cathy and Richard. 

 Jean watched it all happen, caring for family members along the way.  

 “How do I explain it? It’s like cutting off one limb at a time,” Jean said. “It’s devastating.” 

 ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. As it progresses, people may lose the ability to speak, eat, move and even breathe. The ALS ultimately causes their death. It is degenerative and as of right now, there are no treatments to stop the progress or cure the disease. Some treatments can ease the suffering of patients.  

 About 90 percent of ALS cases in the U.S. develop spontaneously. The other 10 percent are known as familial ALS, the kind afflicting the Weber family. 

 Even before Peggy was diagnosed, the family knew ALS was a possibility. Her father Art donated blood for research purposes before his death. In 2000, doctors notified the Weber family that Art had died from a genetic form of the disease and all of his nine children had a 50/50 chance of carrying the gene. 

 So in May 2022, as Jean began to experience weakness in her fingers and arms, she knew the diagnosis was a possibility. 

 It was possible that a previous neck surgery could be the cause, she hoped. 

 “But I thought it was too much like ALS,” she said. 

 So she got tested. She knew the symptoms. She had seen them before. 

 “I was hoping it wasn’t,” she said. “But I knew it was.” 

Sharing the news

 The diagnosis itself was devastating. But worse was having to tell her five children and nine grandchildren ages 1.5 to 15 (16 in December). 

 “It’s the hardest thing I ever did in my life,” she said. 

 Understandably, they cried, she said. They knew what to expect. They were among the caregivers for their aunts and uncles who had lived and died with the disease. 

 “They’ve been there. They know what’s going on,” she said. 

 And they’ve shown up for Jean and Bill, too. 

 “Every time we ask for help, they’re all here,” she said. 

 The family is known for regularly hosting parties and events for their very large extended families. Jean says they will continue to do this. It’s part of who they are as a family. 

 “We get it done — together,” she said. 

 In a way, the ALS diagnosis has given the family a gift, a reminder not all families get: to be grateful for what you have today. Especially, that time with family. 

 “Oh my god. It’s priceless,” Jean said. “When people ask me how I’m doing, I say I’m blessed.” 

 She said this may surprise people. Other people would focus on the end, get angry at the unfairness of it all. But Jean has lived this before. 

 “My sister Peggy, she had it when she was 42. I’m 63. I got to see my kids grow up. … . I got to see my grandkids born,” she said. “I’m really blessed. My family is very close. I can’t complain about anything in life.” 

 Her blessings include her husband Bill. They’ve been married for 41 years, but have been together since they were in seventh grade.

 “Bill picks up all the slack,” Jean said. “He’s getting to be a really good cook.” 

 He’s always been good with the fryer, she explained, but recently tried his hand at chicken pot pie and pulled pork. 

 “The last while, I’ve been teaching him to cook. He’s a good student,” she said. 

 In her spare time, Jean lives in the moment: going for rides, watching the fall colors change, reading murder mysteries and sitting on the porch and listening to the birds, walking her dog and talking with family and friends. 

 “And I have the grandkids over all the time,” she said. 

The ideal guinea pigs

 There are a few ways to look at the Weber’s form of ALS. It’s slower moving than typical ALS. This gives those diagnosed more time with their families. 

 But it also means more time living on the decline. More time to live as a dependent, (as some may see it) as a “burden” to their family members, requiring constant care and straining resources like time, money and emotions. 

 The slow-moving progression makes it ideal for study. And the high inheritability gives researchers many test subjects. 

 Of the nine siblings, seven have the gene for this form of ALS and six have gotten a diagnosis. They have all become guinea pigs. 

 “We’re right at the front line,” she said. “We’ll let them do anything they want to us.” 

 In 2021, the family helped create NextGen ALS. As a nonprofit, NextGen focuses on funding research to develop treatments and cures for genetic forms of ALS. 

 There isn’t much to lose. Today, the decline of ALS is inevitable and irreversible. Anything they can try to stop or slow the progression is welcome. And any step they take could potentially move them closer to a cure — one that will secure the futures of their children and grandchildren. 

 Finding a cure is crucial to the survival of the family for generations to come. 

 The family has partnered with various medical institutions, to research their genetics and to find a cure. 

 As for Jean, she looks at it this way. 

 “We have hope,” she said. 

 She and her husband Bill have already traveled to New York for research. 

 “We’re hoping to have a break through to silence our gene,” Jean explained. Researchers hope a quarterly shot will stop the gene from being expressed, stopping ALS from developing.

 Stephanie Dickrell has been a journalist and writer in Minnesota for more than 15 years. She is also Jean Micke’s niece by marriage. While she doesn’t share the Weber family’s genetic predisposition to ALS, she has witnessed the toll the disease has taken on Jean’s extended family.