The Ehlers-Danlos Blog

Disabled Britons Harness Twitter to in Effort Stop Welfare Cuts

2012-01-20T03:30:00.000-05:00

LONDON — Writing in The Guardian, Patrick Butler's "How the Spartacus welfare cuts campaign went viral" profiles the successful social media campaign of several disabled activists who are forcing Great Britain's conservative government to rethink plans to slash welfare and health funding.
EDS-survivor Kaliya Franklin and co-founder of the "the #spartacusreport Twitter storm" argues:

...it was the internet, blogs and Twitter that enabled disabled people to get their voice heard, unmediated by traditional media. "None of this would have happened without social media. The campaign has been done by people mostly from their beds. We would not have been able to find each other had we not had access to social media."

Butler's profile of Franklin and her fellow activists reveals:

Franklin also hosts a successful blog – Benefit Scrounging Scum – where some of her made-for-YouTube videos have become cult viewing (her "shame on you..." message to David Cameron marked the beginning of the Spartacus campaign in October 2010). One clip records her putting the Labour leader Ed Miliband on the spot at last year's Labour party conference, with Franklin's eloquence about politicians' toxic use of "benefit scrounger" rhetoric contrasting with the awkwardness of a surprised Miliband.

....Law graduate Franklin, 36, was not disabled as a child. She had planned to join the army before an accident, while teaching in the US after university, exacerbated existing inherited health problems. At 28, she was diagnosed with Ehlers-Danlos syndrome. Symptoms include joint hypermobility and arthritis, and she has serious and persistent health and mobility problems. "Since October, I do not think I have been out of my pyjamas for more than a handful of days," she says.

The importance of DLA for Franklin is that it helps her to live independently. Despite her mobility and health difficulties, she is not eligible for social care support, having been assessed as requiring only "moderate" needs. She has been refused an NHS wheelchair, and relies heavily on a support network of friends and neighbours to help her.

One of the problems, she says, is that despite years of underfunding for adult social care, people assume the state's support for disabled and chronically sick people in receipt of DLA is much more comprehensive than it is, and that "we all have a nice bungalow and an adapted car".

Despite their success, the Spartacus campaigners are already counting the personal cost to their health. Some, already ill, have retired exhausted.

Our kudos to Patrick Butler and The Guardian for strong coverage of government efforts to balance the UK's budget on the broken backs of its most vulnerable citizens.

Ehlers-Danlos Survivor Doing 'God's Work'

2012-01-20T02:47:00.001-05:00

Talladega, AL — Reporter Elsie Hodnet of The St. Clair Times does an excellent job profiling EDS survivor Lexie Smith in truly comprehensive story of personal triumph in challenging physical circumstances.
Ms. Smith, who suffers from a combination of the both the classical vascular types of EDS, appears to have made phenomenal use of service dogs, deals with chronic pain through extensive online ministry to fellow sufferers.

No Fear: Britons Fight Back Against Prying, Misinformed Eyes

2011-12-13T08:51:00.001-05:00

London — In an effort to resolve its budget crisis, the UK has instituted a policy which encourages citizens to report disabled people who "appear" to be doing more than their disability might allow, never forgiving that a wheelchair-bound EDS sufferer might, occasionally enjoy a brief walk or a breezy trip down a slide. Writing in The Guardian, advocate Lucy Glennon posits that the policy is narrow-minded and deeply misinformed. We agree.

Wisconsin Considers Pot to Ease the Pain

2009-11-16T23:27:00.000-05:00

Wisconsin Democrats seek to ease the pain of cancer patients, and others with serious chronic pain, by legalizing medical marijuana.

Wisconsin Democrats support medical marijuana

By SCOTT BAUER
Monday, November 16, 2009 5:15 PM CST

Legalizing medical marijuana will ease the cancer patients' pain and help others who are suffering, supporters of legalization argued Monday.

Two Democratic state lawmakers, advocates and those fighting chronic diseases said at a news conference there is momentum nationwide to decriminalize the use of marijuana for medical reasons.
They pointed to Gov. Jim Doyle's comments last month in support of legalizing medical marijuana for people who have a doctor's prescription. Also, the American Medical Association called last week for a federal review of marijuana's status as a controlled substance to make it easier to do research that could lead to development of marijuana-based medicines.

Everyone knows someone who would benefit if the law were changed, said Jacki Rickert, founder of "Is My Medicine Legal Yet?" She suffers from Ehlers-Danlos syndrome and reflexive sympathetic dystrophy, bone and joint diseases that limit movement and lead to painful muscle spasms. Marijuana eases the pain, she said.

Rickert, 58, has lobbied more than a decade to legalize medical marijuana in Wisconsin. She was arrested in 2000 when Mondovi police raided her home and confiscated marijuana. The district attorney later declined to press charges.

"We're not criminals, we're just people trying to get on with our lives," said Gary Storck, who said he starting using marijuana in 1972 to treat his glaucoma and arthritis.

A similar bill was introduced in the Legislature in 2002 but did not pass.

Under the measure co-sponsored by Rep. Mark Pocan, D-Madison, and Sen. Jon Erpenbach, D-Waunakee, a person would need a prescription from a doctor to receive marijuana, which could either be grown at home or obtained through a licensed nonprofit dispensary. The state would keep a registry of both those who can receive and dispense marijuana.

The Department of Health Services could not estimate how many people would qualify for marijuana prescriptions, according to the fiscal estimate for the bill. Seventeen of 132 lawmakers have signed on in support.

"This law needs to be changed," Rickert said. "We can't wait any longer."

Thirteen states have legalized medical marijuana, according to the National Organization for the Reform of Marijuana Laws. The Wisconsin bill is up for a hearing Dec. 15, and Erpenbach said the goal was to have it voted on sometime in January.

The governor said last month that he had no problem with the use of marijuana to treat severe pain and other medical conditions, if it's prescribed by a doctor. Restricting the use of medical marijuana makes no sense when doctors can already prescribe more dangerous drugs, such as morphine, he said.

Doyle's comments come after a decision by the Obama administration not to prosecute users and suppliers of medical marijuana in the states where it's been legalized. The decision is a clear break from the policies of the Bush administration and another sign pointed to by backers of Wisconsin's bill that the attitude toward medical marijuana is changing.

Teen Gets Relief From Ehlers-Danlos

2009-11-15T04:44:00.002-05:00

Finally, a success story!

South Carolina Teen Gets Real Treatment

Two years ago, Mathis began having nonstop headaches that couldn't be explained.A year later, her legs started to feel numb and she walked with short, slow steps.
Two surgeries later, Mathis now walks with a normal stride, drives and dances. She's taking a new approach to life.
"She's a bit of a miracle child," said her grandmother Pat Mathis. "A lot of prayers went up for her, and the Lord answered them."
Mathis, 19, was diagnosed with Ehlers-Danlos syndrome, a neurological condition, last December. She visited several doctors who couldn't explain the headaches or what caused her legs to give out. It was frustrating; at times, she felt as if people doubted what she complained of feeling.
"It was very difficult," Mathis said. "It was a lot of going to doctors who said, 'Maybe it's this,' and they'd do tests and my tests would come back normal. We could see how things were getting worse, but no one could do anything about it."
She visited family doctors, neurologists, ear, nose and throat doctors and a chiropractor. Mathis was told she might have had migraines, lupus or fibromyalgia.
"It was frustrating," said her mom, Lisa Mathis. "There was never an option to not follow up on what was going on."
Once Mackenzie had difficulty walking long periods of time and needed wheelchairs at the mall and the airport, the family started searching harder.
A family friend got Lisa Mathis in touch with a family whose children were diagnosed with Ehlers-Danlos. The symptoms that family's children experienced matched what Mackenzie was dealing with. Lisa Mathis said this helped get the family on the right track to find a diagnosis.
Ehlers-Danlos is a collection of genetic disorders that affect collagen, a protein that adds strength and elasticity to connective tissue. Patients often experience a downward pull of the spinal cord, which causes Chiari malformation.
According to Mackenzie's doctor, that's when the cerebellum, which controls balance, and the brainstem push downward. The pressure creates a range of problems, including the headaches and balance issues she experienced.
Changing her life ... and others'
She underwent two surgeries that have returned her life to normal.
One surgery removed some of the ligament that pulled her spinal cord downward. The second surgery was a cranial spinal procedure, which improved the relationship between her skull base and her upper spine to eliminate the brain stem's deformation.
"Those two surgeries completely changed my life, and I can now do things I didn't think I would do again," Mackenzie said.
Lisa Mathis said her daughter's recovery was quick compared with some who have been diagnosed with Ehlers-Danlos.
"Her story is not typical of these patients, probably because we moved quick compared to others," said Lisa Mathis, who said it sometimes takes people 10 to 20 years to receive an Ehlers-Danlos diagnosis.
Mackenzie Mathis is helping other Ehlers-Danlos families. She's learned that about 50 families in North and South Carolina are dealing with the condition. In January, she'll host a support group for those families in Spartanburg. Her neurosurgeon is based in Bethesda, Md., and she serves as a spokeswoman for the hospital and answers questions from Ehlers-Danlos patients through e-mail and Facebook. She's also blogging about her recovery, and the blog has been read by people as far away as the United Kingdom.

If more people know, more people will be diagnosed!

Teen Dies From Ehlers-Danlos: Classmates Plant Tree

2009-11-15T02:45:00.000-05:00

We are saddened by the death of Forrest Goetsch, a fourteen-year-old athlete, whose courage in the face of Ehlers-Danlos Syndrome inspired his classmates to plant a tree in his honor.

It was just an honor to be able to call him one of your friends," Nick said of Forrest.Beyond physically grueling sports, Forrest endured multiple surgeries for a bilateral facial cleft that affected his appearance, Steve Goetsch said.

We are a bit puzzled by broader story, however. It appears that Ehlers-Danlos was not originally listed as his cause of death.

An autopsy showed that a D.C. Everest Junior High student who died Sept. 21 during cross country practice suffered from a genetic connective tissue disorder, according to the Marathon County Medical Examiner’s office.

Fourteen-year-old Forrest Goetsch died from complications of Ehlers-Danlos Syndrome (EDS), Marathon County Deputy Medical Examiner Jean Finley said today. A preliminary autopsy done shortly after his death indicated Goetsch’s death was from cardiac-related natural causes.

We can't help but wonder how many similar instances might be the result of underdiagnosis, due to a lack of general awareness of this disease.

Playing Through Pain

2009-11-15T02:11:00.000-05:00

Playing through pain, this pianist sings with his fingers.

"If there's a moment of trauma, or a moment of triumph, hopefully [listeners] will feel they've been taken on a compelling emotional journey and can attach that to whatever resonates with them," he said. "As I was writing, I was able to get pretty deep into some of the specific things that I was processing and, at the same time, not have to have that manifest in the finished product in a way I had to be too concerned about."

Baerman tends to downplay some of his accomplishments, most notably the very act of playing piano, as a person with a painful, degenerative condition known as Ehlers-Danlos Syndrome. Therefore, the following statement bears particular attention: "Ultimately, I'm really happy with ["Know Thyself"]. I think I've managed to come up with something that's emotionally substantial, certainly the most intense and most genuine work I've produced to date."

SNAP, CRACKLE, POP! A profile in courage

2008-08-02T05:20:00.000-04:00

SARASOTA–"Snap, crackle, pop." Familiar sounds to the EDS sufferer. WWSB, Sarasota's ABC affiliate reports on an EDS-afflicted teen, whose internal strength keeps her going.

Knowing you're not alone!

2008-07-08T08:58:00.000-04:00

As with many rare disorders, the toughest challenge for those confronting them, aside from the disorder itself, is sense of being alone. "Our Journey With Ehlers-Danlos" is a sharp, hearfelt "web log" in the best sense. Filled with practical resources, "Our Journey" offers real hope in seeking to confront Ehlers-Danlos as a challenge, rather than letting the disease become an insurmountable obstacle. To its writers, we offer thanks!

Ehlers-Danlos: A Rare Profile in the News

2008-07-04T22:57:00.002-04:00

Ehlers-Danlos is a very rare genetic disorder. Thus, it is rarely in the news. But, this profile from Salt Lake City's Deseret News really paints an extraordinary portrait of both the disease–and the courage of its sufferers. Here, writer Lois M. Collins captures the human struggle perfectly. Our kudos to her and the Deseret News.

Lacy Chambers, then 11, was playing volleyball when her shoulder slid out of its socket the first time. She had two surgeries over the next three years.
When she was 14, she was walking on the porch when her hip dislocated. That time, she was in a wheelchair for six weeks, able to leave it for only very small journeys. Two weeks after that, her other hip dislocated as she was getting up. She was placed in leg braces and a wheelchair for another six weeks.
Then her jaw dislocated, followed by her neck, her fingers, her wrists, her ankles and her knees. She was home-schooled for a while, deemed too fragile to risk public school. Every joint, it appeared, was at risk. Once, another student, jostling past in the hallway, grabbed her arm and the shoulder popped out again.
Kim Hansen was hospitalized for internal bleeding after a blood vessel ripped open when he was jogging. Another time, at work, a pipe hit him in the head and a blood vessel started leaking into his nasal cavity. Doctors had to do brain surgery, including tying off a carotid artery.
One day, playfully wrestling with his two young children, he sustained a little kick to the abdomen and was hospitalized for internal bleeding, which was expected to stop on its own. It didn't. He died from the damage to his organs. He was 36.
Though their symptoms appear to have nothing in common, both Chambers and Hansen had Ehlers-Danlos syndrome, an inherited condition caused by a defect in connective tissue. According to the national foundation that bears the syndrome's name, fragile skin and tissue and unstable joints are the result of faulty production of collagen, a protein that acts as "glue" in the body.
There are six major types of EDS, each designated by their symptoms. Chambers' hypermobility is an example of the most common form, which is also the most painful. Fully 95 percent of cases are hypermobile, or vascular, like Hansen's, or "classical," which is associated with scarring, "stretchy" skin, extensive bruising, slower wound healing and various internal-organ issues, according to Terri Keyes of the Ehlers-Danlos National Foundation. Vascular EDS is the least common of the three, but it is the one that can cause premature death.
"All types are woefully underdiagnosed," says Keyes. "Lack of diagnosis increases the health risks for people who have this, especially in surgery and pregnancy, as extra precautions need to be taken to reduce risk and increase the possibility of a successful outcome. Surgeries on undiagnosed vascular type are extremely risky," also true to some degree for all types of the syndrome.
The types do not change within families. A family with joint hypermobility EDS will not produce a child with vascular EDS, for instance. It's estimated that 1 in 5,000 people have EDS, which can afflict children or adults of any race and either gender.
Hansen's daughter, April Atwood, inherited his Ehlers-Danlos, while his son did not. But she has been affected in markedly different ways than her father. Her mother, Denise Groves, suspected the girl had inherited the condition because she bruised so easily. The first symptom appeared at age 14, when she got cramps that wouldn't go away. At Primary Children's Medical Center, doctors found that her area of vulnerability is her intestinal tract, which ruptures and bleeds.
"The doctor told me it was like sewing up Jell-O," Groves says.
At that time, she was hospitalized for six weeks fighting infection from the rupture.
But her mother says that in many ways Atwood has been lucky. She has been able to go everywhere. "She was on the swim team, because it's low-impact," Groves says of her daughter. "Running or team sports would have been out of the question."
Lacy Chambers, on the other hand, gave up gymnastics and violin because of the stress on her joints. With hypermobile joints, children are sometimes encouraged to stay home from school to avoid injury, and her mom, Dawn Lynn Heuer, briefly home-schooled her for that reason.
When Atwood was younger, she bruised so easily that her mother was afraid officials would "grab me for child abuse. It happens to families." Even a friendly touch on the arm can leave finger-shaped bruises.
Groves learned vigilance with her daughter. It was hard to differentiate between a stomach bug and a crisis, but if intense cramping lasted very long, they'd head for the hospital. Groves remembers car rides where she was shaking hard, worried that the emergency room doctor would, like so many others, be unfamiliar with Ehlers-Danlos or know only of one type. "There are certain tests you have to be careful with," she says. "You can't shove things down her throat or start poking. The tissue is so fragile it can rupture easily. A little lack of knowledge could kill her."
During Atwood's first bout in the hospital, the doctors took a tissue sample and sent it to an EDS specialist out of state, who checked it for the genetic markers and confirmed the diagnosis, something only possible with vascular EDS. The other types are diagnosed based on family history and symptoms, says Dr. Mark Lavallee, a national expert on EDS from South Bend, Ind., who is board-certified in family medicine, sports medicine and nonoperative orthopedics.
Lavallee himself has the classical type of EDS. He likens it to having skin made of tissue paper, where others have construction paper. When the average person falls and gets an abrasion, he says, someone with classical EDS gets a much larger, more troublesome wound, difficult to sew up because the needle tears the skin. That form is also marked by noticeable scarring, bony protrusions and some dislocations of joints, though not to the extent of someone with hypermobile EDS. And while bleeding is a huge problem for those with vascular EDS, it's not insignificant in the other types, either.
There's no prevention for the genetic disease, and experts are trying to develop diagnostic tests for the nonvascular forms of EDS, Lavallee says. They're collecting blood and tissue samples, but the task is complicated because the body produces 11 different types of collagen from skin and nails to tissue and tendons, all found in multiple locations on various chromosomes.
There's also no disease-specific treatment.
"A lot of it is common-sense things like diet and exercise and not smoking and avoiding activities that will wear out the joint," he says. "The other is strength training. The muscles are fine, it's tendons that are problematic. If you keep the muscles strong, there's less laxity and dislocation." Lifting weights tends to strengthen tendons and ligaments, too, he says, and "a thicker and stronger tendon is better than a weaker, flabby tendon." But it has to be mild strength training.
An aortic complication is often present for those with classical or hypermobile EDS, so he urges caution with breath holding and strength training for those patients. "We monitor blood pressure diligently and get contrast to look at the baseline aorta and internal organs."
The foundation is making a huge effort to educate the public and physicians, Lavallee says. They've created medical reference guides and are doing outreach to conferences for rheumatology, for instance. They produced an educational CD, patient education resources and more. Their Web site, www.ednf.org, is packed with information.
Atwood hasn't had to rush to the emergency room since she graduated from the children's hospital. But she and her husband recently moved to Atlanta, which has left her mom feeling unsettled. "When she moved in September, I spent most of my time crying. I miss her, yes, but I'm scared to death."
EDS is something patients, properly diagnosed, learn to live with. They teach themselves to avoid as many of the episodes as they can. When Chambers feels like her ankles are particularly weak, she puts on ankle braces until they feel more stable. Some days she does well and on other days her joints are "really loose" and she has a lot of pain.
EDS did provide her with one bonus. She started receiving treatment at Shriner's Hospital for Children and along the way befriended a physical therapy assistant who was working there while he went to college. They stayed friends, though they drifted apart as her condition stabilized. Years later, they got reacquainted and they are now married with their own little boy.
Elijah is 10 months old and they're watching him for signs of EDS. The type she has confers a 50-50 chance a child will inherit it.
She's also maintained a sunny disposition, despite chronic pain. "There's nothing I can do about it, so why not be happy?" she tells her mom.
"I know my limitations and things I should avoid. That's how I control it."

Welcome!

2008-06-22T00:13:00.000-04:00

The Ehlers-Danlos blog is a tribute to all those who suffer in silence, struggling daily to overcome the challenges of their all-too-numerous afflictions. Here, we shall endeavor to offer news about this difficult family of diseases in the hopes that one day we shall be able to overome its ravages through research, diagnosis, and tremendous collective strength.