Hypermobility Linked to Depression and Anxiety at Young Age

By Madora Pennington, PNN Columnist

The teenage years are difficult for almost everyone, but even more so for teens with hypermobile joints, a condition known as hypermobility. Their joints have an excess range of motion, and some can literally bend their arms, legs and fingers backward.

Jess — who asked that we not use her last name — had always noticed she was extremely flexible. By the time she was a teenager, Jess had crushing fatigue and anxiety. She obsessed over her grades like a perfectionist and developed an eating disorder. Her terrible anxiety caused her to self-medicate, as she did all she could to keep up with her peers.

Eventually, it all became too much. Jess had to leave high school and finish her degree through proficiency testing. Not until age 19 was she finally diagnosed with Ehlers-Danlos Syndrome (EDS), a disease that weakens connective tissue in the joints and causes hypermobility.

Hypermobility in early life is normal. All babies and children are highly flexible, which most eventually outgrow. But for some children, hypermobility is permanent. Excessively loose joints are a feature common to many inherited disorders like EDS, wherein the body cannot make connective tissue that is stable and strong. In essence, the "glue" holding their bodies together is flawed and weak.

Extremely flexible people like Jess may be seen by their peers as having interesting or cool skills, like doing contortionist tricks or excelling in yoga. But hypermobility is a severe health problem that can lead to mental health issues.

"Hypermobility affects one in four people in the UK. Like other musculoskeletal conditions, it can have a profound and far-reaching impact on life, causing daily pain, fatigue and often disrupted sleep," says Dr. Neha Issar-Brown, Director of Research and Health Intelligence at the UK-based charity Versus Arthritis. "Previous studies in adults have shown that you are more likely to suffer from anxiety if you have hypermobility, and that the daily toll of painful symptoms can lead to depression.”

A new study in BMJ Open found a similar link in adolescents. To see if there is an association between hypermobility, anxiety and depression in late adolescence, researchers at Brighton and Sussex Medical School evaluated over 14,000 teens with joint hypermobility.

At age 14, their hypermobility was scored, which is done by measuring the degree to which various joints hyperextend or bend past the point where they should. Then, at age 18, the participants answered surveys about depression and anxiety. Pain, a common complication of hypermobility, was also taken into account. The participants' heart rates were also studied.

"Many psychiatric problems, including depression and anxiety, start before the age of 25. It is therefore important to identify the factors that may increase the risk for these disorders. Being aware of the link between hypermobility and depression and anxiety means that we can work on developing appropriate and effective treatments," said lead author Dr. Jessica Eccles, Department of Neuroscience, Brighton and Sussex Medical School.

Eccles and her colleagues found that young people with joint hypermobility were more likely to have depression and anxiety, and their psychiatric symptoms were also more severe. Joint hypermobility was more common in females than males, but it was only among males that hypermobility increased the risk for depression.

"This study has highlighted the need for more targeted and bespoke support for hypermobile teenagers, particularly girls," says Lea Milligan, CEO of the UK-based advocacy organization MK Mental Health Research. "The findings don't just show the need for support for this group of individuals, but also demonstrate the importance of research that takes a whole mind, body brain approach to health and uses longitudinal studies to improve our understanding of which demographics are at higher risk of depression and anxiety.”

Why hypermobile people suffer from more psychopathologies may be due to a dysregulation of the nervous system called dysautonomia, which is very common among those with loose joints. Their poorly regulated nervous systems cause a rapid increase in heart rate when they rise to stand, when it should remain constant. This inability of the body to maintain a smooth and consistent heart rate when posture changes has also been associated with anxiety.

Rapid heart rates and poor cardiovascular regulation also occur when someone has anxiety and depression. The higher a person's resting heart rate, the more psychological symptoms tend to follow. Those with depression tend to have different skin temperatures, breathing rates, and a lower variability in heart rate.

Complaints from UK parents with hypermobile children motivated this study. They encountered healthcare providers that were ignorant of the complications and challenges of hypermobility and dismissed their concerns. More awareness and education are needed to help these families, and this study is a step in that direction.

Madora Pennington is the author of the blog LessFlexible.com about her life with Ehlers-Danlos Syndrome. She graduated from UC Berkeley with minors in Journalism and Disability Studies. 

Long-Haul Covid Draws Needed Attention to Dysautonomia

By Cindy Loose, Kaiser Health News

The day Dr. Elizabeth Dawson was diagnosed with covid-19 in October, she awoke feeling as if she had a bad hangover. Four months later she tested negative for the virus, but her symptoms have only worsened.

Dawson is among what one doctor called “waves and waves” of long-haul covid patients who remain sick long after retesting negative for the virus. A significant percentage are suffering from syndromes that few doctors understand or treat. In fact, a yearlong wait to see a specialist for these syndromes was common even before the ranks of patients were swelled by post-covid newcomers. For some, the consequences are life altering.

Before fall, Dawson, 44, a dermatologist from Portland, Oregon, routinely saw 25 to 30 patients a day, cared for her 3-year-old daughter and ran long distances.

Today, her heart races when she tries to stand. She has severe headaches, constant nausea and brain fog so extreme that, she said, it “feels like I have dementia.” Her fatigue is severe: “It’s as if all the energy has been sucked from my soul and my bones.” She can’t stand for more than 10 minutes without feeling dizzy.

Through her own research, Dawson recognized she had typical symptoms of postural orthostatic tachycardia syndrome, or POTS. It is a disorder of the autonomic nervous system, which controls involuntary functions such as heart rate, blood pressure and vein contractions that assist blood flow.

It is a serious condition which affects many patients who have been confined to bed a long time with illnesses like covid as their nervous system readjusts to greater activity. POTS sometimes overlaps with autoimmune problems, which involve the immune system attacking healthy cells. Before covid, an estimated 3 million Americans had POTS.

Few Doctors Treat Autonomic Disorders

Many POTS patients report it took them years to even find a diagnosis. With her own suspected diagnosis in hand, Dawson soon discovered there were no specialists in autonomic disorders in Portland — in fact, there are only 75 board-certified autonomic disorder doctors in the U.S.

In January, Dawson called a neurologist at a Portland medical center where her father had worked and was given an appointment for September. She then called Stanford University Medical Center’s autonomic clinic in California, and again was offered an appointment nine months later.

Using contacts in the medical community, Dawson wrangled an appointment with the Portland neurologist within a week and was diagnosed with POTS and chronic fatigue syndrome (CFS). The two syndromes have overlapping symptoms, often including severe fatigue.

Dr. Peter Rowe of Johns Hopkins in Baltimore, a prominent researcher who has treated POTS and CFS patients for 25 years, said every doctor with expertise in POTS is seeing long-haul covid patients with POTS, and every long-covid patient he has seen with CFS also had POTS. He expects the lack of medical treatment to worsen.

“Decades of neglect of POTS and CFS have set us up to fail miserably,” said Rowe, one of the authors of a recent paper on CFS triggered by covid.

The prevalence of POTS was documented in an international survey of 3,762 long-covid patients, leading researchers to conclude that all covid patients who have rapid heartbeat, dizziness, brain fog or fatigue “should be screened for POTS.”

A “significant infusion of health care resources and a significant additional research investment” will be needed to address the growing caseload, the American Autonomic Society said in a recent statement.

Lauren Stiles, who founded the nonprofit Dysautonomia International in 2012 after being diagnosed with POTS, said patients who have suffered for decades worry about “the growth of people who need testing and treating but the lack of growth in doctors skilled in autonomic nervous system disorders.”

On the other hand, she hopes increasing awareness among physicians will at least get patients with dysautonomia diagnosed quickly, rather than years later. Dysautonomia International provides a list of a handful of clinics and about 150 U.S. doctors who have been recommended by patients.

Congress has allocated $1.5 billion to the National Institutes of Health over the next four years to study post-covid conditions. Requests for proposals have already been issued.

“There is hope that this miserable experience with covid will be valuable,” said Dr. David Goldstein, head of NIH’s Autonomic Medicine Section.

A unique opportunity for advances in treatment, he said, exists because researchers can study a large sample of people who got the same virus at roughly the same time, yet some recovered and some did not.

‘Huge Influx of Patients’

Long-term symptoms are common. A University of Washington study published in February in the Journal of the American Medical Association’s Network Open found that 27% of covid survivors ages 18-39 had persistent symptoms three to nine months after testing negative for covid. The percentage was slightly higher for middle-aged patients, and 43% for patients 65 and over.

The most common complaint: persistent fatigue. A Mayo Clinic study published last month found that 80% of long-haulers complained of fatigue and nearly half of “brain fog.” Less common symptoms are inflamed heart muscles, lung function abnormalities and acute kidney problems.

Larger studies remain to be conducted. However, “even if only a tiny percentage of the millions who contracted covid suffer long-term consequences,” said Rowe, “we’re talking a huge influx of patients, and we don’t have the clinical capacity to take care of them.”

Symptoms of autonomic dysfunction are showing up in patients who had mild, moderate or severe covid symptoms.

Yet even today, some physicians discount conditions like POTS and CFS, both much more common in women than men. With no biomarkers, these syndromes are sometimes considered psychological.

The experience of POTS patient Jaclyn Cinnamon, 31, is typical. She became ill in college 13 years ago. The Illinois resident, now on the patient advisory board of Dysautonomia International, saw dozens of doctors seeking an explanation for her racing heart, severe fatigue, frequent vomiting, fever and other symptoms.

For years, without results, she saw specialists in infectious disease, cardiology, allergies, rheumatoid arthritis, endocrinology and alternative medicine — and a psychiatrist, “because some doctors clearly thought I was simply a hysterical woman.”

It took three years for her to be diagnosed with POTS. The test is simple: Patients lie down for five minutes and have their blood pressure and heart rate taken. They then either stand or are tilted to 70-80 degrees and their vital signs are retaken. The heart rate of those with POTS will increase by at least 30 beats per minute, and often as much as 120 beats per minute within 10 minutes. POTS and CFS symptoms range from mild to debilitating.

The doctor who diagnosed Cinnamon told her he didn’t have the expertise to treat POTS. Nine years after the onset of the illness, she finally received treatment that alleviated her symptoms. Although there are no federally approved drugs for POTS or CFS, experienced physicians use a variety of medicines including fludrocortisone, commonly prescribed for Addison’s disease, that can improve symptoms.

Some patients are also helped by specialized physical therapy that first involves a therapist assisting with exercises while the patient is lying down, then later the use of machines that don’t require standing, such as rowing machines and recumbent exercise bicycles. Some recover over time; some do not.

Dawson said she can’t imagine the “darkness” experienced by patients who lack her access to a network of health care professionals. A retired endocrinologist urged her to have her adrenal function checked. Dawson discovered that her glands were barely producing cortisol, a hormone critical to vital body functions.

Medical progress, she added, is everyone’s best hope.

Stiles, whose organization funds research and provides physician and patient resources, is optimistic.

“Never in history has every major medical center in the world been studying the same disease at the same time with such urgency and collaboration,” she said. “I’m hoping we’ll understand covid and post-covid syndrome in record time.”

Kaiser Health News is a national newsroom that produces in-depth journalism about health issues.

Tips on Living with POTS

By Ellen Lenox Smith, Columnist

I suddenly had my life come to a halt when “POTS” hit me in my sixties. Some of you may not be familiar with what that is.

Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia, a condition that affects the nervous system, causing light-headedness, fainting, unstable blood pressure, abnormal heart rates, malnutrition, and sometimes even death.

According to Dysautonomia International,  between as many as three million Americans have some form of dysautonomia, and over 70 million around the world.

I had always lived with low blood pressure, but as the instability of my neck, due to Ehlers-Danlos syndrome progressed, I suddenly felt that standing or even just sitting up in bed might cause me to faint.

I reported the symptoms to my cardiologist, who worked with me on finding treatments, but that journey was not a simple one.

The first thing he encouraged me to do was to salt my food, to keep my blood pressure elevated. When that was not enough, he prescribed salt tablets.

Suddenly, I started gaining weight. There was no evidence of swelling in the extremities, the weight retention for me landed in my chest. However, I continued to faint and feel horrible.  

I reported back to the doctor and was encouraged to add more salt. I got scared when I gained another nine pounds and decided to stop taking salt, assuming that was the culprit. Soon after, I passed out, was sent to the hospital, and diagnosed with congestive heart failure.

I came home after three days in the hospital and proceeded to melt out of my husband’s arms, hearing both legs snap as I fell to the ground. I woke up with terrible leg pain and now had to face two micro-fractures from the fall.  

When my cardiologist was contacted, he mentioned a medication that he thought he had prescribed for me, but I was certain he not ordered anything besides the salt. I admired his honesty in when he later admitted his mistake. He had confused me with another patient. Once I started the medication he intended for me to take, life started to return to me.

I wanted to share my story in hopes that a reader with POTS might find these tips helpful. It’s such a debilitating condition that I hate to think of anyone else suffering unnecessarily.

Here is what has helped to get my life back and blood pressure to almost normal:

1)  Sleep elevated at a 30 degree angle. You want to elevate the head of your bed under the frame, not just sleep with pillows to elevate. If you need to travel, you can take plastic containers often used by college students to elevate their beds to create storage. I use two containers on each side of the head.

2)  Keep hydrated. Drink plenty of water daily.

3)  Exercise to keep your circulation strong.

4)  My magic medication turned out to be Midodrine HCL, 5 MG tablets. Once I started taking them three times a day, I started to live with normal blood pressure! This specific medication may not be suitable for you, but there are other medications that may help. You should consult with a doctor before trying any of them.

I hope this might provide you with useful strategies to cope with POTS.  Don’t settle with being stuck in bed. Although finding the correct combination of medication takes time, I hope that it will bring some normalcy to your life.

Ellen Lenox Smith suffers from Ehlers Danlos syndrome and sarcoidosis. Ellen and her husband Stuart live in Rhode Island. They are co-directors for medical marijuana advocacy for the U.S. Pain Foundation and serve as board members for the Rhode Island Patient Advocacy Coalition.

For more information about medical marijuana, visit their website.

The information in this column should not be considered as professional medical advice, diagnosis or treatment. It is for informational purposes only and represents the author’s opinions alone. It does not inherently express or reflect the views, opinions and/or positions of Pain News Network.