What is stiff person syndrome, the condition Celine Dion is battling? | CNN



CNN
 — 

Complications from stiff person syndrome, a rare neurological condition that causes spasms and muscle rigidity, have caused singer Celine Dion to cancel her “Courage World Tour” dates through 2024, according to a statement posted Friday on the star’s social media account.

Dion postponed several performances in December after learning the reason for her muscle pain and difficulties with mobility. “While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms that I’ve been having,” Dion said at the time.

Despite undergoing daily physical therapy, “she is in a lot of pain,” a source close to Dion told CNN.

In the Instagram announcement of the tour’s cancellation, Dion is quoted as saying: “I’m so sorry to disappoint all of you once again. I’m working really hard to build back my strength, but touring can be very difficult even when you’re 100%.

“It’s not fair to you to keep postponing the shows, and even though it breaks my heart, it’s best that we cancel everything now until I’m really ready to be back on stage again. I want you all to know, I’m not giving up… and I can’t wait to see you again!”

Stiff person syndrome is characterized by muscle rigidity and spasms, heightened sensitivity to stimuli such as sound and lights, and emotional distress that can cause muscle spasms, according to the National Institute of Neurological Disorders and Stroke. Over time people with the condition can develop “hunched over postures,” the NINDS said.

The condition typically begins with muscle stiffness in the middle part of the body, the trunk and abdomen, before advancing to stiffness and spasms in the legs and other muscles, according to the Cleveland Clinic.

The muscle spasms can be “quite severe. These can cause falls, severe pain and significant disability,” said Dr. Emile Sami Moukheiber of the Stiff Person Syndrome Center at Johns Hopkins Medicine. “Falls from severe spasms are very common. These spasms can be precipitated by startle, severe emotions, cold weather.”

These spasms can be strong enough to fracture bone, and any fall can lead to severe injury.

Dion said in her December Instagram video that spasms affect “every aspect” of her daily life, “sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I’m used to. It hurts me to tell you this today.”

The syndrome can also cause anxiety.

“Many patients, if not all, have an anxiety that is intrinsic to the disease and that anxiety actually feeds on the physical ailments of the disease that people can have,” Dr. Scott Newsome, director of the Stiff Person Syndrome Center, said in a video on the organization’s website.

At times, people with stiff person syndrome may be afraid to leave their homes because “street noises, such as the sound of a car horn, can trigger spasms and falls,” the NINDS noted.

Stiff person syndrome is very rare. About 1 out of every 1 million people have the syndrome, and most general neurologists will see only one or two cases in their lifetimes, Moukheiber said.

The first case of stiff person syndrome was reported in the 1950s, according to Newsome, and the disease was historically referred to as “stiff man syndrome.”

Since then, it has been found to affect twice as many women as men, and the name was changed to stiff person syndrome to avoid confusion.

The condition can develop at any age, but symptoms most often begin in a person’s 30s or 40s, according to the Cleveland Clinic.

Stiff person syndrome is thought to have features of an autoimmune disease, according to the National Institute of Neurological Disorders and Stroke: “It is frequently associated with other autoimmune diseases such as type-I diabetes, thyroiditis, vitiligo, and pernicious anemia.”

Although the exact cause isn’t clear, according to the institute, research shows it may be due to an autoimmune response “gone awry” in the brain and spinal cord.

“People with SPS have elevated levels of GAD, an antibody that works against an enzyme involved in the synthesis of an important neurotransmitter in the brain,” the institute noted on its website. “A definitive diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies.”

Other tests include an electromyography (EMG), which measures electrical activity in the muscles, and a lumbar puncture, commonly called a spinal tap.

Because of the rarity of the disease and the ambiguity of its symptoms, people will often seek care for chronic pain before they get neurological care. The condition can be misdiagnosed as anxiety, fibromyalgia, multiple sclerosis, Parkinson’s disease, psychosomatic illness or even a phobia, the NINDS noted.

On average, it takes about seven years for someone to receive a diagnosis of stiff person syndrome, Newsome says.

“Sometimes, [patients] get labeled crazy,” he said, “because on exam early on, there aren’t the hallmark features of stiff person syndrome.”

There is no known cure for stiff person syndrome, but medications can ease the symptoms. Immunoglobulin medications can help lower sensitivity to light or sound triggers, potentially helping head off falls or spasms.

Pain relievers, anti-anxiety medications and muscle relaxers can be a part of treatment for this disease. The Stiff Person Syndrome Center also uses botulinum toxin injections.

Additional benefits may come from the use of acupuncture, physical therapy, heat and water therapy, the Cleveland Clinic noted.

“If left untreated, the disease can cause severe impairment of daily living,” Moukheiber said.

Dion, who previously said she has struggled with her health for a while, said she has a great team of medical professionals and her children’s support.

“I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again,” she said. “But I have to admit it’s been a struggle.”

For a performer like Dion, loud noises and bright lights could act as triggers of muscle spasms.

“This is a very challenging illness that might take a toll on her if it is not treated aggressively, timely and appropriately,” Moukheiber said.



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US cigarette smoking rate falls to historic low, but e-cigarette use keeps climbing | CNN



CNN
 — 

The percentage of adults who smoked cigarettes in the United States fell to a historic low last year, the US Centers for Disease Control and Prevention found. However, e-cigarettes are becoming even more popular.

About 11% of adults told the CDC last year that they were current cigarette smokers, according to the latest preliminary data from the National Health Interview Survey, a biannual survey that provides general information about health-related topics. The survey includes responses from 27,000 people age 18 and older. In 2020 and 2021, about 12.5% of adults said they smoked cigarettes.

This is a significant drop from when surveys like these started. Surveys of Americans in the 1940s found that about half of all adults said they smoked cigarettes. Rates began to decline in the 1960s, and more recently, in 2016, 15.5% of adults said they smoked cigarettes.

Recent studies have shown some groups are still at higher risk. While the latest CDC survey doesn’t capture this level of detail, cigarette smoking rates among some communities – including Native Americans, Alaska Natives and members of the LGBTQ community remain “alarmingly high” according to the 2023 State of Tobacco Control report from the American Lung Association.

The general drop in cigarette smoking among adults should have a positive impact on public health.

Cigarette smoking is still the leading cause of preventable death and disability in the US. So many people have died from smoking, the CDC finds, that more than 10 times as many US citizens have died prematurely from cigarette smoking than have died in all the wars fought by the US.

Smokers are 90% of the lung cancer cases in the United States, but smoking can also cause someone to have a stroke, coronary heart disease, and COPD, as well as other cancers including bladder, colon, kidney, liver, stomach and other cancers. People who live with smokers also are at a greater risk of death, because of secondhand smoke.

This latest survey does not capture why fewer people smoked cigarettes, but the number has been on the decline since the 1960s, after the US surgeon general released the first report on smoking and health that concluded that smoking causes serious health problems.

Experts credit a variety of efforts for the decline in cigarette smoking – anti-smoking campaigns, programs that educate children about the danger of smoking, laws that severely restrict where people could smoke and where cigarette companies could advertise, as well as better access to smoking cessation programs and higher taxes that make cigarettes expensive.

However, Congress hasn’t raised federal tobacco taxes in 14 years. The federal cigarette tax remains $1.01 per pack, and taxes vary for other tobacco products. No state increased its cigarette taxes in 2022.

The pandemic may also have had an influence. Smokers were much more vulnerable to the severe consequences of Covid-19 and that gave some people the extra motivation they needed to quit – and may have given doctors the extra motivation they needed to help them too, according to Dr. Panagis Galiatsatos, a volunteer medical spokesperson with the American Lung Association. At some level, the pandemic also made the medical establishment easier to access.

“The pandemic, I think, really allowed physicians time they never probably had in the past to conduct these telemedicine visits that were appropriate just for smoking cessation strategies, helping them help patients quit and stay quit,” said Galiatsatos, who is a pulmonary and critical care medicine physician and is director of the Tobacco Treatment Clinic with Johns Hopkins Medicine.

Galiatsatos points to the US Surgeon General report released during the Trump administration, just prior to the start of the pandemic. The report detailed that of the patients they have now that smoke, the few that are left are going to be the most resistant to quitting. Then-Surgeon General Jerome Adams’ report encouraged more doctors to help their patients quit. The report found that 40% of smokers are not routinely told by their doctors to stop.

Still, the culture has changed. Smoking is much less socially acceptable in some cultures in the US.

E-cigarette use, though, seems to be more socially acceptable, especially among younger people studies show, and that may explain why those numbers are up.

The current survey found that e-cigarette use rose to nearly 6% last year, that’s up from about 4.9% the year before.

Some argue that e-cigarettes are a good substitute for regular cigarettes, and in some countries they are even promoted as a smoking cessation devices, but the CDC says that “e-cigarettes are not safe for youth, young adults, and pregnant women, as well as adults who do not currently use tobacco products.”

A BMJ study published in February found that people who used e-cigarettes to quit smoking found them to be less helpful than more traditional smoking cessation aids.

The US Food and Drug Administration says there is not enough evidence to support claims that these products are effective tools to help people quit smoking. None are approved for this purpose. The FDA says there are no safe tobacco products, including e-cigarettes, vapes, and other electronic nicotine delivery systems.

“I always hold no stigma or judgment when anyone wants to smoke a traditional cigarette or use electronic cigarettes, but as a lung doctor, I will always promote only air to come into the lungs,” said Galiatsatos. “From my standpoint, I think we should still have a public health mindset around e-cigarette usage because for some individuals, they’re going to have health consequences from this product.”

He said people may choose to vape instead of smoke cigarettes, but clinicians should be prepared to help this population if they do want to quit.

E-cigarettes can, though, produce a number of chemicals that are not good for human health, including acrolein, acetaldehyde, and formaldehyde. Studies show these chemicals are known as aldehydes and can cause lung and heart disease, according to the American Lung Association.

Among teens, nicotine exposure can harm the developing brain, according to the US surgeon general.

E-cigarettes are much more popular than cigarettes among teens, so the adult e-cigarette user numbers will likely continue to grow.

About 14% of high school students said they used e-cigarettes, and 2% of high school students smoked cigarettes last year, according to separate CDC data.

The rate of kids that use e-cigarettesis high, the American Academy of Pediatrics says.

Specifically, in 2022, nearly 5% of middle school and about 17% of high school students reported some form of current tobacco use, according to CDC data from an earlier survey. In 2021, about 11% of middle schoolers and 34% of high schoolers said they had ever tried tobacco.

These “try rates” are important because most adult smokers started at young ages, according to the CDC.

The AAP continues to encourage pediatricians to screen for tobacco use as part of a child’s regular checkup. A talk about tobacco should start no later than age 11 or 12, the report says.

For adult smokers, the CDC encourages encourages people to call 1-800-QUIT-NOW where people can get free confidential coaching. The government also offers free online resources and even text programs that can help people quit.

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What older Americans need to know before undergoing major surgery | CNN



KHN
 — 

Larry McMahon is weighing whether to undergo a major surgery. Over the past five years, his back pain has intensified. Physical therapy, muscle relaxants, and injections aren’t offering relief.

“It’s a pain that leaves me hardly able to do anything,” he said.

Should McMahon, an 80-year-old retired Virginia state trooper who now lives in Southport, North Carolina, try spinal fusion surgery, a procedure that can take up to six hours? (Eight years ago, he had a lumbar laminectomy, another arduous back surgery.)

“Will I recover in six months — or in a couple of years? Is it safe for a man of my age with various health issues to be put to sleep for a long period of time?” McMahon asked, relaying some of his concerns to me in a phone conversation.

Older adults contemplating major surgery often aren’t sure whether to proceed. In many cases, surgery can be lifesaving or improve a senior’s quality of life. But advanced age puts people at greater risk of unwanted outcomes, including difficulty with daily activities, extended hospitalizations, problems moving around, and the loss of independence.

I wrote in November about a new study that shed light on some risks seniors face when having invasive procedures. But readers wanted to know more. How does one determine if potential benefits from major surgery are worth the risks? And what questions should older adults ask as they try to figure this out? I asked several experts for their recommendations. Here’s some of what they suggested.

Ask your surgeon, “How is this surgery going to make things better for me?” said Dr. Margaret “Gretchen” Schwarze, an associate professor of surgery at the University of Wisconsin School of Medicine and Public Health. Will it extend your life by removing a fast-growing tumor? Will your quality of life improve by making it easier to walk? Will it prevent you from becoming disabled, akin to a hip replacement?

If your surgeon says, “We need to remove this growth or clear this blockage,” ask what impact that will have on your daily life. Just because an abnormality such as a hernia has been found doesn’t mean it has to be addressed, especially if you don’t have bothersome symptoms and the procedure comes with complications, said Drs. Robert Becher and Thomas Gill of Yale University, authors of that recent paper on major surgery in older adults.

Schwarze, a vascular surgeon, often cares for patients with abdominal aortic aneurysms, an enlargement in a major blood vessel that can be life-threatening if it bursts.

Here’s how she describes a “best case” surgical scenario for that condition: “Surgery will be about four to five hours. When it’s over, you’ll be in the ICU with a breathing tube overnight for a day or two. Then, you’ll be in the hospital for another week or so. Afterwards, you’ll probably have to go to rehab to get your strength back, but I think you can get back home in three to four weeks, and it’ll probably take you two to three months to feel like you did before surgery.”

Among other things people might ask their surgeon, according to a patient brochure Schwarze’s team has created: What will my daily life look like right after surgery? Three months later? One year later? Will I need help, and for how long? Will tubes or drains be inserted?

A “worst case” scenario might look like this, according to Schwarze: “You have surgery, and you go to the ICU, and you have serious complications. You have a heart attack. Three weeks after surgery, you’re still in the ICU with a breathing tube, and you’ve lost most of your strength, and there’s no chance of ever getting home again. Or, the surgery didn’t work, and still you’ve gone through all this.”

“People often think I’ll just die on the operating table if things go wrong,” said Dr. Emily Finlayson, director of the UCSF Center for Surgery in Older Adults in San Francisco. “But we’re very good at rescuing people, and we can keep you alive for a long time. The reality is, there can be a lot of pain and suffering and interventions like feeding tubes and ventilators if things don’t go the way we hope.”

Once your surgeon has walked you through various scenarios, ask, “Do I really need to have this surgery, in your opinion?” and “What outcomes do you think are most likely for me?” Finlayson advised. Research suggests that older adults who are frail, have cognitive impairment, or other serious conditions such as heart disease have worse experiences with major surgery. Also, seniors in their 80s and 90s are at higher risk of things going wrong.

“It’s important to have family or friends in the room for these conversations with high-risk patients,” Finlayson said. Many seniors have some level of cognitive difficulties and may need assistance working through complex decisions.

Make sure your physician tells you what the nonsurgical options are, Finlayson said. Older men with prostate cancer, for instance, might want to consider “watchful waiting” — ongoing monitoring of their symptoms — rather than risk invasive surgery. Women in their 80s who develop a small breast cancer may opt to leave it alone if removing it poses a risk, given other health factors.

Because of McMahon’s age and underlying medical issues (a 2021 knee replacement that hasn’t healed, arthritis, high blood pressure), his neurosurgeon suggested he explore other interventions, including more injections and physical therapy, before surgery. “He told me, ‘I make my money from surgery, but that’s a last resort,” McMahon said.

“Preparing for surgery is really vital for older adults: If patients do a few things that doctors recommend — stop smoking, lose weight, walk more, eat better — they can decrease the likelihood of complications and the number of days spent in the hospital,” said Dr. Sandhya Lagoo-Deenadayalan, a codirector in Duke University Medical Center’s Perioperative Optimization of Senior Health (POSH) program.

When older patients are recommended to POSH, they receive a comprehensive evaluation of their medications, nutritional status, mobility, preexisting conditions, ability to perform daily activities, and support at home. They leave with a “to-do” list of recommended actions, usually starting several weeks before surgery.

If your hospital doesn’t have a program of this kind, ask your physician, “How can I get my body and mind ready” before having surgery, Finlayson said. Also ask: “How can I prepare my home in advance to anticipate what I’ll need during recovery?”

There are three levels to consider: What will recovery in the hospital entail? Will you be transferred to a facility for rehabilitation? And what will recovery be like at home?

Ask how long you’re likely to stay in the hospital. Will you have pain, or aftereffects from the anesthesia? Preserving cognition is a concern, and you might want to ask your anesthesiologist what you can do to maintain cognitive functioning following surgery. If you go to a rehab center, you’ll want to know what kind of therapy you’ll need and whether you can expect to return to your baseline level of functioning.

During the Covid-19 pandemic, “a lot of older adults have opted to go home instead of to rehab, and it’s really important to make sure they have appropriate support,” said Dr. Rachelle Bernacki, director of care transformation and postoperative services at the Center for Geriatric Surgery at Brigham and Women’s Hospital in Boston.

For some older adults, a loss of independence after surgery may be permanent. Be sure to inquire what your options are should that occur.

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