Linda Mitchell-Storer is in the early stages of Alzheimer’s disease. For now, she is able to operate her business, the Vintage Finery clothing store in Orrington, and live a close-to-normal life with her husband, Tollef Storer, in their idyllic family home overlooking the Penobscot River.
But Mitchell-Storer, 68, notices her cognitive lapses. Sometimes she forgets what she is doing, how to say or do something. Her spelling is not as good as it used to be.
“It’s scary. I get flustered sometimes,” said Mitchell-Storer, who was first diagnosed with Alzheimer’s a few months after her mother, Rosie Mitchell, died of the disease in December 2015. At the time, after seeing her mother deteriorate and noticing she wasn’t quite as sharp as she used to be, Mitchell-Storer got tested for Alzheimer’s. Her tests came back positive – devastating news – but she also learned she had the disease in its very early stages, earlier than most patients.
So when the news broke in early June that a first-of-its-kind Alzheimer’s drug, aducanumab, was approved by the Food and Drug Administration, it gave Mitchell-Storer and millions of patients nationwide hope that scientists are finally learning how to treat the disease and slow its progression.
Mitchell-Storer said she’s ready to take the medication, although the drug has generated controversy over whether it should have been approved by the FDA. The flare-up over the drug includes three resignations in protest from an FDA advisory board that recommended against approval, concerns that it sets a poor precedent to give other drugs the green light, the high cost of the drug and its potential impact on health care costs.
But while aducanumab, developed by Biogen, is not a cure for Alzheimer’s, it’s the first new drug that could be effective at slowing the progression of the disease that robs people of their brain function. As the disease worsens, people with Alzheimer’s may not recognize family members as they lose the ability to take care of themselves.
Her physician, Dr. Cliff Singer, a geriatric neuropsychiatrist at Northern Light Acadia Hospital in Bangor, said Mitchell-Storer should be a good candidate for the new Alzheimer’s drug. But aducanumab is controversial because studies do not definitively conclude that the drug benefits Alzheimer’s patients. One study showed it can slow down the progression of the disease by 22 percent, but other studies have shown zero or unclear benefits to patients. About 29,000 people in Maine have Alzheimer’s disease, and more than 6 million nationwide.
Linda Mitchell-Storer at her business, Vintage Finery, in Orrington. She knows the aisles and inventory like the back of her hand. Shawn Patrick Ouellette/Staff Photographer
CONTROVERSY
Three members of the FDA’s advisory board – which recommended against approval – have resigned in protest in recent weeks, arguing that it sets a precedent for the regulator to OK medications that are not proven to work.
Singer said despite having “mixed feelings” about the approval process and prospects for aducanumab, the drug represents a positive step forward.
“This is a big deal,” Singer said. “This is the very first medication approved that has a potential disease-modifying effect.”
Mitchell-Storer said she knows the drug is not a miracle cure, or that it even might not work, but she wants to take the medication.
“I’m really excited about it,” she said. “I’m all for trying it. I want to try it and see if I feel better. I’m excited that there’s finally a treatment for Alzheimer’s after all these years.”
Linda Mitchell-Storer prepares dresses for a client at Vintage Finery, the clothing shop she has owned and operated near her home in Orrington for four decades. Though she has been diagnosed with Alzheimer’s disease, Mitchell-Storer says she wants to keep running the store while she can. Shawn Patrick Ouellette/Staff Photographer
But Dr. Aaron Kesselheim, a Harvard Medical School professor and one of the members of the FDA advisory board who resigned over the decision, recently told the Boston Globe that the decision to recommend it anyway was one of the agency’s worst recent decisions.
“It is important that the FDA maintains that trust by ensuring the drugs it gives validations to have reasonable science behind them,” Kesselheim said. “Maybe the resignations will bring attention to the fact that there is concern in the scientific community that in these cases, that is not happening.”
Northern Light Health is participating in the Phase 4 study of the new medication, which will involve patients taking the drug and close monitoring of brain function, but will not include a placebo.
HOW THE NEW DRUG WORKS
Despite decades of study, Alzheimer’s is still a mysterious disease for scientists, who are researching what happens to the brain as the disease advances. But scientists do know that dying nerve cells in the brain disrupt connections and cause “memory failure, personality changes, problems carrying out daily activities and other symptoms,” according to the Alzheimer’s Association.
Alzheimer’s is a form of dementia, which is a disease that attacks the brain cells. Dementia is a more general term, and some people who have cognitive loss from dementia do not have Alzheimer’s. The Alzheimer’s Association 2021 report says there are not good estimates for how many people have dementia, but it is projected that 11 percent of those age 45 and older have some form of cognitive impairment, and some of those people will develop dementia or Alzheimer’s later in life.
For an Alzheimer’s diagnosis, doctors look for telltale signs, such as large numbers of amyloid plaque and twisted fibers of a protein called tau inside brain cells.
The new medication removes amyloid plaque, which disrupts cell functioning in the brains of Alzheimer’s patients. Singer said for more advanced stages of Alzheimer’s, the amyloid plaque has already caused so much damage in the brain that removing it likely will not help, which is why they are focusing on patients with early-stage Alzheimer’s.
Dr. Eric Dinnerstein, a neurologist with MaineHealth, said in later-stage Alzheimer’s patients, removing amyloid plaque is like extinguishing a match after a forest fire has been set.
“Once you start the fire, removing the match will not stop the progression of the fire,” Dinnerstein said.
Dinnerstein said aducanumab could help, and it’s historic that it was approved by the FDA. But it’s not clear how effective it will be. Side effects of aducanumab include swelling of the brain and persistent headaches.
“This is a very exciting time, as we are moving forward and there is now a drug that might work,” Dinnerstein said. “But there is a degree of uncertainty.”
Dinnerstein said despite the potential side effects and modest benefits, he will use the drug in his practice with his Alzheimer’s patients.
“It gives us yet another tool to try,” Dinnerstein said. “I will prescribe it myself to patients once it becomes available. We need to use whatever we’ve got to try to better the lives of patients and families. I’m in the trenches with these families and I feel their pain and distress and I want to help them.”
Linda Mitchell-Storer, right, helps Jean Miller of Castine pick out a hat for an upcoming party. Shawn Patrick Ouellette/Staff Photographer
Singer, the Northern Light doctor, said “on balance” he’s glad it was approved, but he cautions that any benefits will be modest.
“One of my biggest worries is people will have unreasonable expectations, and expect a huge impact in slowing the disease down,” Singer said. “It’s also expensive. But my greatest concern is it will be much harder to recruit patients into clinical trial research using placebo controls with this drug being available.”
Patients may be reluctant to participate in studies and have a 50 percent chance of receiving a placebo when they could take an FDA-approved drug, Singer said. Northern Light has recently partnered with Jackson Laboratories, Massachusetts General Hospital, MaineHealth and the University of Maine on Alzheimer’s studies.
COSTLY MEDICATION
The drug’s expense – about $56,000 per year – and controversy over its approval may mean that some insurance companies will balk at paying the cost, leaving patients footing more of the bill. Medicare is expected to cover the medication, but many patients would still be on the hook for thousands of dollars per year in co-pays, depending on whether they have “gap insurance” used to cover costs that Medicare doesn’t cover, and whether that insurance will pay for the new drug.
Sen. Susan Collins, R-Maine, has advocated for increased Alzheimer’s research, but she expressed concern with the cost of the medication.
“Alzheimer’s is a devastating disease that exacts a tremendous toll of those living with it and their families,” Collins said in a statement. “This newly-approved drug offers hope to some people at the earlier stages of Alzheimer’s. The price of the drug is, however, projected to be $56,000 a year, raising serious concerns about its affordability. I hope that the manufacturer will lower its price so that it is more readily available to those patients whom it may help.”
Orrington resident and businesswoman Linda Mitchell-Storer gets a hug from a client, Jean Miller of Castine, after Miller arrived last week to look for a hat for an upcoming party. Shawn Patrick Ouellette/Staff Photographer
Tollef Storer, Linda Mitchell-Storer’s husband, said the cost of the drug is worrisome.
“How do you tell if it’s working? Is this just going to be ‘hokey-pokey’ juice that people are going to be paying big money for?” Storer said.
There is also the cost to the health care system. The medication and related expenses could cost the U.S. health care system $57 billion per year, according to a Kaiser Family Foundation analysis, if one out of every six Alzheimer’s patients is prescribed the new drug.
Another complication, Singer said, is that one of the most common ways to diagnose Alzheimer’s disease in the early stages, a PET scan to detect the presence of amyloid plaque, is not in wide use for most patients.
“These PET scans are mostly in use for research modalities,” Singer said. “But one day in the near future we may have blood tests that can do these scans.”
Despite all of the controversy and concerns over the new drug, it is still a positive step forward, said Drew Wyman, executive director of the Alzheimer’s Association, Maine chapter.
“It’s not going to cure the disease, but it’s a big step. History has shown that the approval of a first drug in a new category will invigorate the field, open the door to more treatments and clinical trials,” Wyman said. “If this can buy you more time with your loved one, then that is worth a lot.”
Just a few-minute walk down the road from the family home in Orrington, Mitchell-Storer meets clients for appointments at Vintage Finery, the shop she has owned for 40 years. She wants to keep running the business while she can, and she knows the store’s winding pathways intimately. Wedding dresses are on the second floor, and clothing, hats, masks, boots and shoes, some hundreds of years old, are jammed into all corners of the store. There’s enough clothing of different styles, such as 1920s flapper outfits to fur coats, that someone planning a play or movie could likely find what they need for costumes.
“There’s a mermaid outfit here somewhere,” Mitchell-Storer said, and a minute later she pulled out a large mermaid costume.
Tollef Storer said he hopes the new drug will be effective.
“If it works, it means we would be able to prolong her life, and the time we have together,” he said.
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