The Washington Post - USA (2022-05-17)

E4 EZ EE THE WASHINGTON POST.TUESDAY, MAY 17 , 2022

A revelatory conclusion Early in March 2020, less than two weeks before the pandemic descended, Chiancone and her sister met with Toro. “Not only did they tell me they’d figured it out, but that they could fix it,” Chiancone said. “I was in shock.” She was advised to immedi- ately stop taking methotrexate and Enbrel and begin high doses of folinic acid, which counters the effects of methotrexate. It took weeks, but her symptoms slowly receded. Chiancone was able to walk without a cane, the pain in her calves and joints diminished, then disappeared, her severe fa- tigue lifted and her memory re- turned. Testing conducted six months later at NIH showed that the level of folate in her spinal fluid was normal. Her EMG remains abnormal but stable, and the white matter lesions in her brain are largely unchanged. “There could be further improvement but we don’t know if she’ll get all the way better,” Toro said. Adult onset folate deficiency is better known in other parts of the world, including Canada, Toro said, but remains underrecog- nized in the United States. “I sus- pect this disorder is much more common than we know,” he added. The irony, in Chiancone’s view, is that she doubts that she actually had rheumatoid arthritis, the reason she was prescribed methotrexate for nearly six years. She has not taken RA medication for more than two years, with no ill effects. “It could have just been Ray- naud’s and osteoarthritis all along,” Chiancone said, referring to the most common form of arthritis which is caused by “wear and tear” on joints and typically treated with over-the-counter pain relievers. Chiancone wonders what would have happened to her had she not stumbled upon the NIH program. “I feel very lucky that it was something they could find and something they had a solution to,” she said. “It makes me wonder how many people out there are struggling with this.”

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and a letter from her neurologist, Chiancone was accepted. She spent the last week of January 2020 at NIH. “It was amazing,” she said. Toro said his interest was piqued by Chiancone’s unusual presentation: a nerve conduction study that he said was “very suggestive” of ALS; extensive white matter brain lesions that are common in neurodegenera- tive diseases such as multiple sclerosis but not ALS; and her weight loss surgery. People who undergo such operations are warned of the dangers caused by malabsorption and related prob- lems and told they must take replacement supplements of vi- tamins and minerals for the rest of their lives. Toro said he wondered wheth- er the combination of weight loss surgery and methotrexate, which is known to deplete folate, had blocked the transport of the es- sential vitamin across the blood brain barrier, preventing it from reaching the central nervous sys- tem. The result can be cerebral folate deficiency, which can cause nerve damage, muscle weakness, dementia-like memory loss and white matter lesions. (In chil- dren, cerebral folate deficiency is caused by a genetic mutation.) Cerebral folate deficiency is detected through a specialized test for 5-methyltetrahydrofolate, which requires a spinal tap. Chiancone underwent the test, which confirmed Toro’s hunch: the level of folate in her spinal fluid was virtually undetectable, even though the level in her blood was normal. After ruling out other neuro- logical diseases, a rare cancer, various errors of metabolism and genetic mutations, Toro and his team concluded that Chiancone probably had severe adult-onset cerebral folate deficiency caused by weight loss surgery and methotrexate. “My feeling is that surgery alone or the medication alone would not have [caused] it,” Toro said. Her case, he added, is a reminder that surgery and medication can have unintended con- sequences: “In Nancy’s case, they converged to magnify the side effects of each other.” An uniden- tified genetic susceptibility may have played a role, Toro said.

ment account to make extra mortgage payments and travel with her sister to Mexico to look at art. But after six months, the ALS specialist expressed doubts: Chiancone’s condition had not worsened as expected. A few months later the clinic dis- charged her, suggesting that she might have an unspecified muscle disorder. A subsequent muscle biopsy was essentially normal. No closer to an answer after more than two years, Chiancone’s joint pain had been eclipsed by exhaustion, immobility and un- predictable falls. She broke a bone in her foot after falling on stairs in her home and wiped out in front of a crowd in the middle of Dulles International Airport. And her memory was faltering. “There were long conversations [about students] that I couldn’t remember having even if they had happened the week before,” she said. “I felt very much on my own and that no one could help me.”

Defying long odds Chiancone’s discovery of the NIH program made her feel hopeful for the first time in months. Earlier in her life, she had spent the better part of a year crushed by severe postpartum depression until, with her mother’s help, she found effective treatment. That ordeal “definitely influenced my persistence in finding someone who could help me,” she said. The NIH program, launched in 2008, is the original site of the pioneering Undiagnosed Diseas- es Network, which now operates at 12 medical centers around the country. Since its inception, about 4,500 people have applied to the UDP and 1,500 have been accepted. Care is free and patients spend a week at the NIH Clinical Center undergoing a meticulous work- up conducted by experts from multiple specialties. Approxi- mately 22 percent receive a diagnosis, sometimes years later. The program, which has iden- tified 25 new diseases, attempts to tease out possible genetic un- derpinnings of patients’ illnesses and to provide useful advice to those who do not receive a diagnosis. Two months after submitting her application, medical records

gle with her weight, Chiancone decided to undergo gastric sleeve surgery, which removed about 80 percent of her stomach. The operation was successful (she has since lost 115 pounds) but six months later the pain in Chian- cone’s hands worsened and spread to her hips and legs. Her rheumatologist suspected that the weight loss surgery had impaired her ability to absorb methotrexate and switched her to an injectable form of the drug. Chiancone didn’t notice much improvement. For months she felt exhausted, achy and generally ill. In December 2017, she began having severe cramps in her calves which intensified after the mile-long walks she frequently took with her sister Janet. Chian- cone’s family physician sent her for a sonogram, which ruled out a blood clot. Her rheumatologist then prescribed a muscle relaxant and a steroid; neither helped. A week later, Chiancone was trying to reach something on a shelf in her office when she realized she could no longer stand on tip toes. “It was just really weird,” she said.

Dire diagnosis She was referred to a neurologist who quickly ordered blood tests, MRI scans of her brain and spine as well as an electromyogra- phy (EMG) test and a nerve conduction study, which assess the functioning of nerves and mus- cles. Both were abnormal. On Feb. 1, 2018, the neurologist told Chiancone she had ALS, a rare, progressive and fatal disease that destroys motor neurons in the brain and spinal cord. “I knew something was wrong but I never expected that,” Chian- cone recalled. “I kept thinking, ‘There’s no way I have this.’ But then I’d think that’s probably what other people think when they get this diagnosis.” The neurologist sent her to a specialized ALS clinic. Chiancone said she was certain the ALS expert would tell her that something less dire was to blame. But he concurred with the diagnosis and scheduled follow-up appoint- ments. Chiancone decided that if her life was going to be drastically cut short, she wanted to maximize the time she had left. She bor- rowed $10,000 from her retire-

Cold numb hands
Chiancone’s difficulties started
when she was 46. In 2013, she
experienced periodic episodes in
which her fingers would become
numb and painful and turn white.
Her primary care doctor sent her
to a rheumatologist who diag-
nosed Raynaud’s disease, a typi-
cally self-limiting condition in
which small arteries that supply
blood to the skin overreact to
stress or cold temperatures.
Because Raynaud’s can accom-
pany certain autoimmune diseas-
es, the rheumatologist ordered a
blood test for lupus, which causes
joint pain. When the test ap-
peared to be positive, Chiancone
began taking the immunosup-
pressive drug plaquenil (better
known by its generic name hy-
droxychloroquine) but stopped
after a second lupus test was
normal.
After Chiancone’s hand pain
worsened, the rheumatologist or-
dered blood tests for rheumatoid
arthritis (RA), a chronic inflam-
matory disorder that attacks
joints. Although tests for RA were
negative, her erythrocyte sedi-
mentation rate and other inflam-
matory markers were elevated.
Inflammation has many causes,
including other forms of arthritis,
certain bowel diseases and some
cancers. It has also been linked to
obesity.
In 2014, the rheumatologist di-
agnosed possible seronegative
rheumatoid arthritis, an uncom-
mon form of the disease. She
prescribed medicine for RA and
told Chiancone that if her pain
improved, that would probably
validate the diagnosis, Chiancone
recalled.
Chiancone began taking meth-
otrexate, a powerful drug used in
chemotherapy and to treat RA
that has not responded to other
medications. She also began giv-
ing herself injections of Enbrel, a
medicine used to treat moderate
or severe RA. Chiancone was told
to take folic acid supplements;
methotrexate is known to reduce
levels of folate, a vitamin essen-
tial to the formation of blood cells
among other functions.
The pain in her hands initially
improved once she began taking
the medications, but then re-
turned.
In 2016, after a lifelong strug-

MYSTERIES FROM E1

Deteriorating health made her desperate. NIH program made her hopeful.

FAMILY PHOTO Nancy M. Chiancone found help at the Undiagnosed Diseases Program at the

were peeved, though probably no one as much as Sandberg. “There is a common belief that everything at Taco Bell tastes the same, but the Grilled Stuft Nacho is the exception,” Sandberg said, explaining his appreciation for a specific sauce. “I want to make this clear,” he said. “The cheesy jalapeño sauce is different than their creamy jalapeño sauce. Without the cheesy jalapeño sauce, it is just not the same.” Sandberg found an online community that shared his taste in fast food. Someone started an online petition four years ago pleading with the chain to bring back the specialty item. The petition collected 792 signatures — including Sandberg’s. The appeal was not enough to revive the dish, so Sandberg decided to take mat- ters into his own hands. First, he began with at-home workouts, then incorporated weightlifting, high-intensity in- terval training and long-distance running into his regular fitness routine. “There are days that you want to give up,” Sandberg said. “On the days that you do want to work out, that’s when you push yourself physically, but on the days that you don’t want to work out, that’s

when you have to push yourself mentally.” Sandberg, who said he ate fast food “all the time” before begin- ning the challenge, believes tend- ing to his mental health as well as revamping his diet have been major contributors to his weight loss. “I used to think it was just about counting calories, but it’s actually about making your calories count,” he said, adding that he consumes lots of fruits and vegetables, lean protein and com- plex carbs. Once a week, Sandberg allows himself to dine on whatever his heart desires. “I have not had to give up anything I love, I just can’t eat it every day,” he said. “I feel the best I’ve ever felt.” Along with support from his fiancee, family and friends, his TikTok followers are also “a huge motivator,” Sandberg said. “Peo- ple have been very positive and encouraging.” “I have found a lot of people that don’t want them to bring back the Grilled Stuft Nacho because they want me to keep exercising,” he added. Over the past 16 months, Sand- berg has not only amassed a following of fellow Grilled Stuft

Nacho fans but also created a community of individuals hoping to get healthier. “A lot of people have reached out and told me that my videos have motivated them to start working out,” he said. The most important thing is “forging the habit itself,” Sand- berg, who currently weighs 188 pounds, tells people. “Make sure you stay consistent, even if it’s just walking for 30 minutes a day.” Although he is thrilled with his weight-loss results, Sandberg’s goal of bringing back the Grilled Stuft Nacho has yet to be achieved. He did, however, earn the attention of Taco Bell, which reached out to him in February and offered to make him a few Grilled Stuft Nachos. “We’ve been watching and cheering on Chris since day one,” said Nicole Weltman, Taco Bell’s head of social, in an emailed statement to The Washington Post. “When we saw his one year approaching, we wanted to recog- nize him and all he’s accom- plished. And what better way to do that than bring the Grilled Stuft Nacho back, just for him, just for one day.” “It was the best one I’d ever had,” said Sandberg, who coordi- nated with corporate headquar-

ters to go to his local Taco Bell, where they made several special Grilled Stuft Nachos for him, as well as a jar of cheesy jalapeño sauce, which Sandberg is saving for the right occasion. Weltman said “there are currently no plans for the return of Grilled Stuft Nacho, but just like all of our fan favorites, we never say never on what the future may hold.” Sandberg is not giving up hope. “I will feel very good if Taco Bell brings it back because I will feel like I did something for a lot of people,” he said, referencing the 792 people who signed the online petition, as well as other fans he has found on social media. “They want to eat it, and I want that for them.” Sandberg is often asked wheth- er he will continue to exercise daily if Taco Bell ultimately adds the Grilled Stuft Nacho back to its menu. “I can’t think that far ahead,” he said. “The important thing is to take it one day at a time.” Regardless of what the future holds in the taco department, though, “I never imagined that I would inspire so many others to want to live a healthier life,” Sand- berg said. “That’s reason enough to keep going.”

COURTESY OF CHRIS SANDBERG Chris Sandberg lost over 85 pounds in 16 months. LEFT: He was 280 pounds in 2019. RIGHT: What he looks like now.

BY SYDNEY PAGE

When Taco Bell removed the
“Grilled Stuft Nacho” from its
nationwide menu in 2015, Chris
Sandberg was crushed.
The specialty item — a triangle-
shaped tortilla filled with beef,
crunchy red strips, sour cream
and cheesy jalapeño sauce — was
“the best thing I ever had at Taco
Bell,” Sandberg said.
The day he discovered the dish,
he liked it so much, “I had it every
day for a week,” Sandberg said.
“The following week, it was dis-
continued.”
After it was taken from the
menu, he moved on and tried
other items, but none measured
up for him. It stayed fresh in his
mind. And on Jan. 4, 2021, he
decided to start a daily exercise
challenge on TikTok, in the hope
of getting Taco Bell’s attention.
Sandberg shared a video of
himself, breathless in his bath-
room mirror, saying, “Day one of
exercising every day until Taco
Bell brings back the Grilled Stuft
Nacho.”
The seven-second clip went vi-
ral with hundreds of thousands of
views. Sandberg doubled down
on his unusual — and somewhat
illogical — effort to apply pres-
sure to the restaurant chain
through physical activity. He
posted another mid-workout vid-
eo the following day, and every
single day thereafter. In the
roughly 470 days since he started
chronicling his daily exercise rou-
tine on TikTok, Sandberg has
shed more than 85 pounds.
“I’ve put in an unbelievable
amount of physical work,” said
Sandberg, who lives in San Fran-
cisco, is self-employed, and pro-
duces social media videos and
short films. He emphasized that
he does not work for Taco Bell and
never has.

The goal of his workout antic is twofold: While his primary objec- tive is to bring back his beloved Grilled Stuft Nacho, when he began his Taco Bell challenge more than one year ago, Sandberg — who weighed close to 300 pounds before the pandemic — was also seeking to lose weight. He decided to merge the two missions. “I do understand the irony in exercising every day for a fast- food item,” he said. “It started as a joke that has clearly gone way too far, but I can’t stop now.” Although Sandberg initially ex- ecuted the idea in jest, he quickly realized “it resonates for people,” as losing weight can be a stressful struggle for many. He wanted to find a way to make his experience lighthearted. Sandberg grew eager to get in shape after “I read that people who were overweight were more vulnerable to covid, so that had been at the back of my head for a while,” he said, adding that he was 275 pounds when he started the exercise challenge, after losing 15 pounds over the preceding few months. Sandberg thought that public- ly sharing his weight-loss journey on social media might help hold him accountable while also po- tentially motivating others. He wanted something to set himself apart, a cause that might be comi- cal enough to compel Internet strangers to cheer him on. “I didn’t want the journey to be about a number on a scale,” Sand- berg said. “I wanted it to be fun. I wanted the goal to be something that had nothing to do with my weight.” Enter the Grilled Stuft Nacho. “Everyone loves Taco Bell,” Sandberg said in a bold culinary statement. “That’s just what came to me.” Plus, he believed his efforts might actually work. According to the Taco Bell Fan- dom Wiki, the Grilled Stuft Nacho first appeared on the menu in 2013 but was discontinued in March 2014. It made a brief reap- pearance in 2015 before it was pulled for good. Some people

Love of this fast

food made him

drop 85 pounds

TikTok exercise challenge was aimed at return of Taco Bell item

“I feel very lucky

that it was something

they could find and

something they had

a solution to. It

makes me wonder

how many people

out there are

struggling with this.” Nancy M. Chiancone

The Washington Post - USA (2022-05-17)

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