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9 Articles in Volume 17, Issue #3
Anxiety and Pain
Central Pain in Rheumatoid Arthritis
Imagine Dragons’ Dan Reynolds Educates People About Ankylosing Spondylitis
Letters to the Editor: Ehlers-Danlos Syndrome, Arachnoiditis
Managing Cancer-Related Pain: A Look at Alternative Approaches
Pain Management in the Elderly: Focus on Safe Prescribing
Painful Genetic Diseases
Responding to Women's Pain Early and Effectively
The 5 Most Misunderstood Terms in Pain Medicine

Letters to the Editor: Ehlers-Danlos Syndrome, Arachnoiditis

April 2017

Did Elvis Have EDS?

After I finished reading the article about Elvis Presley’s medical history by Forest Tennant, MD, DrPH,1 I wondered if he might have had Ehlers-Danlos syndrome (EDS)? The facts seem consistent with many of the medical issues common with this connective tissue mutation, including the stretched body parts, an inability to detox chemicals, self-medication, and an apparent need for greater pain relief. I understand that people with EDS are most often diagnosed at autopsy, where the mutation is found under a microscope.

Forest Tennant, MD, believes Elvis Presley may have had Ehlers-Danlos Syndrome,

I wonder if the head injuries that Mr. Presley experienced, plus his Chiari malformation symptoms, were related to EDS?

If Mr. Presley suffered from EDS, then it would be likely that his child and grandchildren may be gene carriers at risk for developing the disorder.

I also found it interesting that Dr. Tennant wrote about Howard Hughes’ medical case.2 I suspect that Mr. Hughes also suffered from EDS. He was intelligent and inventive, which are common traits found in people with this disease. The fact that Mr. Hughes survived a plane crash and was able to invent the modern hospital bed while he healed from broken ribs is truly amazing! 

Given that the health awareness ribbon for EDS is the zebra, I find it very intriguing that the movie he was known to have watched over and over was named Ice Zebra.

—Sonja D. Long
                    Homewood, Alabama

Dear Sonja,

Congratulations! You are the first person to contact me with the suspicion that Elvis Presley had EDS. His doctors not only did not suspect EDS, they never even considered it, as this genetic disease wasn’t regarded as a cause of severe pain and disability in Presley’s day. However, his primary doctor, George Nichopoulos, MD, who personally opined to me that Presley probably had “some sort of genetic disease,” as he had multiple “strange,” seemingly unconnected medical problems.

In a retrospective review of the Presley case, I too suspect he had EDS. The early age of onset of his multiple symptoms, pain patterns, pathologic conditions, and early death at age 39 are all compatible with a severe form of EDS.3 Beginning in his 20s, he experienced a wide variety of medical ailments, including respiratory, dental, headache, arthralgia, prostate, liver, spine, and gastrointestinal upsets.3,4 His medical symptoms involved various biologic systems and were migratory. This type of come-and-go pattern is typical of EDS. In the last years of Presley’s life, he complained of pain all over.

There are other compelling reasons to believe Presley had EDS. He developed severe constipation and mega-colon in the last few years of his life, requiring medical treatment including enemas. Large intestine problems are relatively common in EDS patients.3,4 Some EDS patients develop a condition known as visceroptosis, which is a disconnection and prolapse of the bowel from its abdominal, connective tissue attachments.5 Presley may have had this condition.

His unusual and exaggerated physical gyrations on stage suggest hypermobility of multiple joints. Some photos show him on the stage, and almost touching his fans in the first row when he bent over. Movements on stage are hardly grist for a clinical diagnosis, but his physicality was a marvel to those of us who watched his live performances.

Also, Presley’s eye and vision problems were typical of EDS. Physically, his eye sockets were deep and he had lax eyelids, or ptosis. He developed bilateral glaucoma, which often necessitated wearing sunglasses.

As for Howard Hughes, he may well have had a variation of Marfan’s syndrome, which is often seen in EDS. He was tall and developed osteoporosis, scoliosis, collapsed vertebrae, and a hip fracture that required surgery. These conditions are common causes of pain in Marfan’s patients who live past middle age, as was the case with Hughes. His physicians, as far as I know, never considered a genetic disease to explain his clinical state.

Presley and Hughes had something else in common. They both had multiple incidents of head trauma, leading to pituitary and autoimmune disorders as a result. These problems, on top of a painful genetic disease, manifested in complex, multiple medical ailments that befuddled their doctors, as these pathologic, clinical states were as yet unknown.

Today, we are better able to analyze and diagnose these painful genetic conditions. It is amazing that both Presley and Hughes were so accomplished despite horrific illnesses, significant pain, and suffering.  

Forest Tennant, MD, DrPH

Arachnoiditis Rx

I saw an article online about arachnoiditis by Forest Tennant, MD, DrPH.6 In it, he mentions a medication that can help reduce pain from this condition. I have adhesive arachnoiditis and take oxycodone, gabapentin, and a fentanyl patch. What medications would Dr. Tennant recommend for me?  

Johan Gustafsson 
      Karlskrona, Sweden

Dear Johan,

Adhesive arachnoiditis (AA) is an inflammatory condition of the central nervous system. When a diagnosis of AA is made based on MRI results, there would be evidence of inflammation in both the arachnoid layer of the spinal cord covering and the nerve roots of the cauda equina (spinal nerve bundle at the base of the spine). Adhesions may occur on the nerve roots and spinal lining as a result of the inflammation. When adhesions form, pain is excruciating and there is impairment of nerve connections to the legs, bladder, and bowel. In addition, spinal fluid flow is obstructed, which may result in headaches, tinnitus, blurred vision, and mental impairment.

Facebook postings have been a great help to suffering patients, but prescribing 1 drug is not the usual treatment for AA, or any other severe, centralized pain condition. Rather, a medical protocol, or regimen, which consists of multiple drugs must be used to obtain pain relief and foster recovery.

Unfortunately, patients have a tendency to expect “1 drug for 1 condition,” but that is not the recommended approach for treatment of AA. My guess is that the article you read suggests a corticosteroid (eg, methylprednisolone or dexamethasone). The experience at my clinic is that potent pain relievers such as those you are taking (oxycodone, gabapentin, and fentanyl) are not very effective unless the neuroinflammation, or underlying cause of the disease, has been simultaneously treated and controlled.

Consequently, we have found that for effective pain relief, regular administration of a low-dose corticosteroid, usually taken in the late afternoon, as well as an anti-inflammatory agent and a glial cell modulator are recommended.

The use of some neurohormones, such as oxytocin, human chorionic gonadotropin, and human growth hormone, may not only help control neuroinflammation and provide some pain relief, but they appear, in some cases, to produce permanent recovery.

I am aware of the great pain, suffering, and despair that befall patients with AA and other severe centralized pain problems. I also understand that the treatment regimen for AA is complex and, to date, has been able to offer only symptomatic pain relief with such drugs as opioids and neuropathic agents. You might want to read an article I wrote on AA that appeared in the June 2016 issue of this journal.7

If you so desire; we can send your physician an up-to-date, clinical protocol.

Forest Tennant, MD, DrPH

Last updated on: April 25, 2017
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Chronic Pain in the Elderly: Special Challenges

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