Ehlers-Danlos Syndrome (EDS) Demographics & Characteristics

A recent study released by Rheumatology takes a look at the demographics of 280 patients previously diagnosed with hypermobility syndrome. They gathered a cohort of 280 patients – 90% female and 10% male, between ages 18 and 66 (it is generally accepted that females have a higher rate of hypermobility than males, though it is not clear why). 95% of these patients had what was previously known as EDS type 3, now known as hypermobile Ehlers-Danlos Syndrome (hEDS). They proceeded to analyze a comprehensive medical history.

The average age of diagnosis of these patients was 29. 66% of patients had a family history of hypermobility, which corresponds with what we know about the genetic nature of Ehlers-Danlos syndrome. A family history of hypermobility and/or EDS is one of the accepted criteria to be diagnosed with hEDS.

66% of the patients had orthostatic intolerance, meaning their vitals changed dramatically when standing up and returned to “normal” when lying down. This includes drastic increases in heart rate and decreases in blood pressure due to the body’s inability to properly compensate for the challenges of standing upright. Almost half of those with orthostatic intolerance had bowel problems, compared to only 19% without intolerance. 45% had gastrointestinal problems, and 39% had bladder dysfunction. Chronic joint and muscle pain were reported in 91% of the patients. Approximately half of these patients with chronic pain used opioids long-term to relieve their pain. 43% patients reported being on work disability.

While the official clinical diagnoses for hypermobile EDS focus on joint hypermobility and skin hyperextensibility, the study confirms that this particular condition is systemic and also has a high correlation to orthostatic intolerance and chronic pain. Therefore it is important to look at a holistic, overall approach of each patient suspected to have hEDS in order to properly assess how to improve the quality of life of each person living with hEDS.

Ref: Dojcinovska, M., Cohen, H., & Wolman, R. (2019). 200 Demographics and clinical characteristics of a national cohort of 280 patients with hypermobility syndrome and impact on their work disability. Rheumatology, 58(Supplement_3), kez107-016.

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Updated Ehlers-Danlos Criteria

In May 2017, there were several changes in the criteria for the diagnosis of what used to be Ehlers-Danlos Syndrome Type III, hypermobility type. First, the name has been changed to hypermobile Ehler-Danlos Syndrome or hEDS. Instead of six types, there are now 13 types.

 

  Clinical EDS subtype Abbreviation IP Genetic Basis Protein
1 Classical EDS cEDS AD Major: COL5A1, COL5A1 Type V collagen
        Rare: COL1A1 Type I collagen
        c.934C>T, p.(Arg312Cys)  
2 Classical-like EDS clEDS AR TNXB Tenascin XB
3 Cardiac-valvular cvEDS AR COL1A2 (bilallelic mutations that lead to COL1A2 Type I collagen
        NMD and absence of pro α2(I) collagen chains)  
4 Vascular EDS vEDS AD Major: COL3A1 Type III collagen
        Rare: COL1A1 Type I collagen
        c.934C>T, p.(Arg312Cys)  
        c.1720C>T, p.(Arg574Cys)  
        c.3227C>T, p.(Arg 1093Cys)  
5 Hypermobile EDS hEDS AD Unknown Unknown
6 Arthrochalasia EDS aEDS AD COL1A1, COL1A2 Type I collagen
7 Dermatosparaxis EDS dEDS AR ADAMTS2 ADAMTS-2
8 Kyphoscoliotic EDS kEDS AR PLOD1 LH1
        FKBP14 FKBP22
9 Brittle Cornea Syndrome BCS AR ZNF469 ZNF469
        PRDM5 PRDM5
10 Spondylodysplastic EDS spEDS AR B4GALT7 β4GalT7
        B3GALT6 β3GalT6
        SLC39A13 ZIP13
11 Musculocontractural EDS mcEDS AR CHST14 D4ST1
        DSE DSE
12 Myopathic EDS mEDS AD or AR COL12A1 Type XII collagen
13 Periodontal EDS pEDS AD C1R C1r
        C1S C1s
           
IP, inheritance pattern; AD, autosomal dominant; AR, autosomal recessive; NMD, nonsence-mediated mRNA decay

 

 

hEDS continues to have no obvious known genetic testing and is only diagnosed through clinical testing.

As mentioned earlier, the Beighton scale is used to determine if a person is hypermobile. This continues to be an important determinant for hypermobility. A 5/9 score in adults is needed in order to meet this requisite. However, because age has an effect on flexibility (ie with age, you tend to become stiffer), there is a Five Point Questionnaire that determines if there is a history of hypermobility. The five points are the following:

  • Can you now (or could you ever) place your hands flat on the floor without bending your knees?
  • Can you now (or could you ever) bend your thumb to touch your forearm?
  • As a child, did you amuse your friends by contorting your body into strange shapes or could you do a split?
  • As a child or teenager, did your shoulder or kneecap dislocate on more than one occasion?
  • Did you consider yourself “double-jointed”?

If you answer yes to two or more questions, then you have met the criteria for hypermobility (with 80-85% sensitivity and 80-90% specificity).

 

The Beighton Scale and Five Point Questionnaire are the first part of three criteria that need to be met in order to qualify for the diagnosis of hEDS. This criterion also meets the diagnosis of hypermobility (you can have generalized joint hypermobility without a diagnosis of hEDS).

 

The second criterion has three parts (A, B, and C), of which two must be met to qualify. Part A is a series of 12 symptoms, of which five must be met in order to quality:

  • Unusually velvety skin
  • Mild skin hyperextensibility
  • Unexplained striae on back, groins, thighs, breast or abdomen in adolescents, men or prepubeteral women without a history of significant gain/loss or weight.
  • Bilateral piezogenic papules of the heel
  • Recurrent or multiple abdominal hernias (umbilical, inguinal, crural)
  • Atrophic scarring with papyraceous or meosideric scars
  • Pelvic floor, rectal and/or uterine prolapse in children, men or nulliparous women without a history of morbid obesity or other known predisposing medical conditions.
  • Dental crowding or high or narrow palate
  • Arachnodactyly (fingers & toes, long and slender), Steinberg Sign (thumb beyond palm), Walker-Murdoch sign (thumb overlapping fifth finger around wrist)
  • Arm span to height ≥ 1.05
  • MVP
  • Aortic root dilation

 

Part B is a positive family history of one or more first degree relatives, independently meeting the criteria of hEDS.

Part C is musculoskeletal complaints; a patient must have at least one to qualify:

  • Daily musculoskeletal pain at least three months in two or more limbs
  • Chronic widespread pain for more than 3 months.
  • Recurrent joint dislocation or frank instability in the absence of trauma (A or B)
    • 3 or more atraumatic joint dislocations in the same joint or two or more atraumatic dislocations in two different joints occurring at two different times.
    • Medical confirmation of joint (2 or more) instability

 

The third criterion contains three pre-requisites, all of which must be met:

  1. Absence of unusual skin fragility which should prompt consideration of other types of EDS.
  2. Exclusion of other heritable and acquired connective tissue disorder with autoimmune connective tissue disorder (ie Lupus, RA).
  3. Exclusion of alternative diagnoses that may also include joint hypermobility by means of hypotonia and/or CT laxity. Alternative diagnoses and diagnostic categories include, but not limited to, neuromuscular diseases, myopathic EDS, Bethlem myopathy, or HCTD (ie other types of EDS), Loeys-Dietz Syndrome (Marfan), and skeletal dysplasias (ie OI).

 

References

American Journal of Medical Genetics, Part C. (Seminars in Medical Genetics), March 2017.

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Ehlers-Danlos (EDS)

It is known that physically active patients who have stiff muscles frequently develop pain.  What is less known is that patients with overly flexible joints will also frequently develop muscle pain.

Congenital diseases, such as Marfan Syndrome and Ehlers-Danlos Syndrome (EDS), may cause joints to repeatedly dislocate, resulting in stiffness in the muscles surrounding the joint – a way that the body attempts to help the unstable joint.  The reflexive stiffness and spasms in the muscles surrounding the unstable joint cause pain.

Treatment to relax the muscles such as injections into trigger points or into the muscle attachments may do too good a job – that is, if the muscles become too relaxed, the joint may become too loose and  continue to dislocate.

An alternative treatment we have used is low level laser therapy (LLLT).  LLLT uses light energy in a narrow wavelength (810-980nm) to deeply penetrate through the skin into the muscles and tendons.  LLLT produces an increase in chemicals in the body (Cytochrome C Oxidase) that enables the production of ATP (adenosine triphosphate) which can aid in healing damaged tissue. Using LLLT to reduce pain in EDS patients has resulted in ~40% decrease in pain lasting for 3-4 weeks (or even longer!) without making the underlying muscle too loose.

Read more about EDS HERE

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The Start of Baseball Season

The baseball season just opened and already players are calling off their first few games due to “tweaked knees” and “sore backs”. As the sun is starting to come out, and the snow is (FINALLY!) melting, many of us are anxious to get outside and start playing sports again. However, this is also a time where pain can start rearing its ugly head. Many people forget to limber and stretch properly before AND after playing sports, causing strains and sprains of muscles.baseball

So keep in mind these important tips to get moving again this spring:

  1. Do gentle limbering stretches every day to help maintain flexibility of your muscles.
  2. Stretch before and after any exercise.
  3. Start slowly – if you haven’t moved all winter, start with simple exercises, like walking, before starting anything more difficult.
  4. If you’re feeling extremely sore the following day, it means you may have done too much. Cut back on the difficulty and amount of time the next time you exercise. Slowly increase the difficulty and the length of the session. A safe rule of thumb is to start out after a long layoff at 50% of your prior routine and then increase 15-20% every 5-7 days as long as you feel okay at any level before going to the next.
  5. Drink lots of water!
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Is access to your medical records a good thing?

More and more doctors are transferring their records to an electronic format. One electronic medical records software, OpenNotes, allows patients to view their medical records online as well. Does having this information mean better care for a patient?

The New York Times published an article about one patient, Steven Keating, who benefited from the ability of seeing his medical records. In fact, because of this, he was able to push his doctors to perform an MRI of his brain, which revealed a brain tumor the size of a tennis ball.

In other cases, having this unfiltered information may create unnedatacessary concern and anxiety. For example, if one of your blood levels isn’t within the “normal range,” what does this mean? Most people would probably run to their computer and search “abnormal blood level for _________” and get countless different possible diagnoses….most of them serious, and probably not related to your “abnormal” blood levels. Results of imaging studies, such as x-rays and MRIs may also appear to be serious when they are actually only reflecting the results of age and normal wear and tear.

Just having raw data and unfiltered notes is not generally useful and can be harmful. If you are going to get your notes, make sure to discuss your concerns regarding what you read with your physician.

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Acetaminophen

The BMJ (what used to be called the British Medical Journal) just published an article stating that acetaminophen (ie Tylenol) has been found to provide no relief in low back pain (compared to a placebo). Acetaminophen was also related to a risk of having an abnormal liver test. The article included data from 13 randomized controlled trials (RCTs).

An RCT to evaluate a drug is a research study where one group of people receives the actual drug (in this case, acetaminophen) and the other group receives a placebo (a pill with no active ingredient, generally a sugar pill). Then, data is collected from both groups to see if either achieved any painTylenol relief. In this analysis, it shows that whether you receive acetaminophen or a sugar pill, you had the same amount of pain relief – meaning that active drug had no real pain relief effect.

Although most of the patients I see find no relief from acetaminophen, a small percentage of patients do, so don’t completely dismiss this drug when it comes to helping your pain. However, if you do take acetaminophen, make sure that you’re not taking too much. The FDA recommends taking less than 3000mg a day. This means if you’re taking Extra Strength Tylenol (500mg), you can only take 6 pills a day, or regular strength, 300mg, 10 pills a day. Higher amounts can lead to liver damage that could even be life-threatening.

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Smoking and Back Pain – is it related?

We all know that smoking is bad for you. In fact, since 1965, the Surgeon General’s Warning posted on every package of cigarettes says: Smoking Causes Lung Cancer, Heart Disease, Emphysema, And May Complicate Pregnancy. But did you know that in addition to the myriad of illnesses that are related to tobacco and smoking, recent studies show that smokers have a higher prevalence of back pain than non-smokers? It’s true.

Several studies in the 1980s and 1990s have shown a correlation between smoking and increased back pain.  More recently, a smokingmeta-analysis was done in 2010, which basically means a large number of studies (in this case, 40) were put together, and the results were compared to see what pattern, if any, existed between back pain and smoking. This meta-analysis concluded that current and former smokers have a higher prevalence of back pain. It even made the comparison between adult smokers to adolescent smokers; adolescents showed a higher prevalence of back pain. Therefore, we can conclude, the earlier one smokes, the greater risk to your back.

Is there a technical or medical reason behind this connection? Indeed. Smoking causes constriction of blood vessels, which reduces the amount of blood going to your muscles. Decreased blood results in decreased oxygen, which is one of the most important factors in initiating muscle pain. Some smokers actually had parts of their arms and legs amputated because of dead tissue caused by too little oxygen.

Do you want be free of back pain? Put out that cigarette and quit smoking!

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Spinal Surgery for Lumbar Stenosis

A recent study was published in the Journal of the American Medical Association, analyzing spinal surgeries done on Medicare patients who were diagnosed with lumbar stenosis.

Let’s rewind – what is lumbar stenosis? Lumbar refers to the lower part of the spine, where we would normally identify as low back. Stenosis refers to the narrowing of the bones in the spine, often pushing or compressing the nerves that being in the spinal cord and extend into your legs. Spinal stenosis is often associated with pain and numbness in the leg.

When a patient is diagnosed with lumbar spinal stenosis, a wide variety of treatments are offered. If the spinal stenosis is actually causing pain and difficulties functioning, such as walking, you would imagine that exercise and physical therapy would have little or no effect. However, pBack Painhysical therapy and exercise are frequently prescribed and may reduce symptoms. This means that at least some of the pain is from muscles and that the picture seen with the CT or MRI showing the nerves being compressed wasn’t accurately showing the reason for all the pain. Without a thorough examination of the muscles in the lower body, we may overlook an easily treated source of pain.

Overreliance on imaging studies can lead to unnecessary, costly, and sometimes damaging treatments, which may include various pain medications, injections (such as epidural injections), and surgery. When surgery is recommended, there are different complexities of surgeries that can be performed.

In this recent study, the surgeries were divided into three categories: 1. Decompression by itself (removing a small piece of bone from the spine to relieve pressure on the spinal cord or nerves), 2. Simple (only 1-2 levels) fusion (fusing the bones in the spine using metal and or bone that may be harvested from the hip or provided by a bone bank), or 3. Complex Fusion (more than 2 levels and/or anterior [front] and posterior [back] approach). From the years 2002 to 2007, the number of decompression and simple fusion surgeries decreased while the number of complex fusion surgeries increased 15-fold. With increasing complexity of the surgery, the odds for complications and rehospitalization significantly increase without a significant demonstrated improvement in outcomes.

We should all be concerned that the number of complex surgeries is increasing, causing more severe side effects and death. Hospital charges alone for a less complex surgery can cost around $23,000 while the complex surgeries may cost over $80,000.

 

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In today’s world we are bombarded by information and ways to obtain it. Do you want to know how many steps you take a day? Just buy a device and it will calculate it for you. Care to know what your cholesterol levels are? Simply go get a kit at your nearest drug store. Is it possible we are gathering too much information for our own good?

The same question can be asked about medical testing. Is it useful or even helpful to know certain things about our bodies? Many of us have undoubtedly had the experience where we have gone to the doctor because we had pain and an imaging study was ordered. MRI, CT, and ultrasound can give us information about our bodies that would have been unimaginable in the past.

While technology in general is of course a huge benefit, at the Norman Marcus Pain Institute, we believe it is necessary to proceed with caution as we navigate through it all. At NMPI, one common problem we find in back pain, neck pain, and shoulder pain is the overuse and overreliance on imaging studies. “Abnormalities” are often found on imaging studies without any relation to one’s pain. In fact, seeing abnormalities is more common than seeing a “normal” spine!   For example, up to 40% of people without back pain can haveBack Pain herniated discs and as many as 70% may have degenerated discs. That is why the US Institute of Medicine suggests not getting an MRI too quickly since you are likely to see one of these problems and assume it is the cause of your pain. This can lead to unnecessary nerve blocks and surgery.

At NMPI, we often see patients who have a herniated disc, yet they only have pain in their back and buttock. They have not experienced any radiation into the leg(s) at all. In these patients, the pain generally has nothing to do with the disc herniation since disc herniation pain generally radiates down the leg and into the foot. Even when the patient is experiencing pain down the leg with a herniated disc found on MRI, muscles in the low back and buttocks may be the cause of the pain. If muscles are not examined as a potential cause, you may undergo an apparently reasonable surgery, without achieving relief of pain. Some studies show that up to 50% of spine surgeries fail (resulting in failed back surgery syndrome) and one of the reasons is the failure to identify muscles that were the true source of the pain.

Most back pain and neck pain is caused by soft tissue such as muscles and tendons. This is confusing because you may have been told your pain is from your spine, discs, or nerves. The problem is most people as they get older have signs of wear and tear on their x-rays and MRIs, but these common signs of aging may not explain your pain if the pain actually originates in your muscles. That’s why we say, when diagnosing persistent pain it’s not having “more” information at hand, it’s having the “right” information at hand.

At NMPI, we often see patients who experience persistent pain even after multiple spine surgeries. Our non-surgical, non-invasive treatment program has most of our patients leaving our office free of long standing back, neck, shoulder, and headache pain.

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What To Do When Back Pain Causes Overdose?

“We don’t appreciate what we have until it’s gone.” If only we had a dime for every time we heard this. Perhaps the reason it is such a common expression is the simple truth in it. This certainly applies to our health, but specifically our backs. We probably all take our good health in vain, until something goes wrong. By the time we are in our forties over twenty percent of us experience some form of back pain. And what do we do when we experience pain? Well, unfortunately many people will turn to strong painkillers. This means opioids, morphine-like painkillers. And, while we have written about this topic in the past, there is something new on the horizon. Evzio, the brand name of injectable Naloxone, is a prescription medicine that can block the effects of morphine and related painkillers. Approved by the FDA in April 2014, it allows a patient to quickly treat themselves or be treated by a family member if the patient has overdosed on opioids.Evzio

In the past, Evzio was difficult to obtain due to its high cost. However, recently The Clinton Foundation announced that it has negotiated a lower price for Evzio (see NYT article). This will allow municipalities to more easily purchase this medication, making it more available to those who need it.

It is a sad reality that many people will turn, in desperation, to painkillers as an answer to their aching backs. We, at the Norman Marcus Pain Institute, only use opioids as a last resort. Our method of finding the source of the pain and treating it has eliminated back pain for thousands of patients.. Nevertheless, with the rise of overdoses each year, the increased availability of naloxone to non-medical personnel will allow lives to be saved.

 

 

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Is Looking Good Worth Having Back Pain?

There is an old expression, “beauty is pain.” Does it need to be? Is it worth risking your long-term health? Why do women wear high-heeled shoes when they know that looking beautiful in the moment may cause them pain down the road? Scientists from the Universite de Bretagne-Sud recently conducted experiments, which studied the effects of high-heeled women on men. Women who wore higher heels (3-4 inches vs. 2 inch heels) were able to get more men to answer survey questions on the streets and were picked up faster in bars and clubs. These studies show some type of empowerment in women with high-heeled shoes.  heelsKnowing that this empowerment exists, and as fashion has increased the height of heels from 4 inches to 5 inches, we would like to remind you that walking for extended periods in high heels can cause calf tendons to shorten and possibly result in an increase in low back pain.

Here at the Norman Marcus Pain Institute, we see patients that run the gamut of factors causing their back pain. Prevention is always the best treatment; since low back pain affects so many of us a simple intervention is being more conscious of our shoes! While those Manolo Blahniks and Jimmy Choos may be stunning, they may be causing a problem for you down the line. At NMPI, we won’t say don’t wear them, just please, wear them in moderation.

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Why Health Insurances Carriers Should Not Be For Profit Enterprises

In November, I read this article in The New York Times on How Medical Care Is Being Corrupted. In a nutshell — Insurance carriers have been incentivizing care for more than 20 years by selectively paying for some services whilst denying others.

To successfully address a complicated problem, such as long-standing pain, more than one approach is often most effective albeit initially more expensive. As a Pain Medicine physician, I have witnessed the defunding of the only approach that has been found to be consistently successful (by the Cochrane database for systematic reviews) in addressing patients disabled by chronic back pain: comprehensive multidisciplinary pain treatment centers.

While cost should be a consideration in choosing treatment – it should not be the primary deciding factor. For-profit insurance companies can be effective in helping policyholders fund their medical care but they are constrained by conflicting interests

  1. A moral and ethical obligation to care for policyholders who have purchased a policy to protect their health.
  2.  Shareholders who have invested in the company and expect each quarter to produce a reasonable profit.

If you believe, as I do, that health insurance not be allowed to be a for profit enterprise, join in a national discussion to legislate that all health insurance be not-for-profit. Insurance companies should only exist for the protection of the individual patient.

 

 

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Earlier in November, Rafael Nadal, the 14-time Grand Slam winner, announced he would receive stem cell treatment to help heal his ailing back, the same type of treatment he received for his knee. His doctor in Barcelona, Dr. Angel Ruiz-Cotorro, who has been treating Nadal for 14 years, said, “Nadal’s back pain is ‘typical of tennis’ players in that the treatment is meant to help repair his cartilage.” Stem cells were recently extracted from Nadal for a cultivation process to “produce the necessary quantities,” said Ruiz-Cotorro. Once cultivated, the stem cells will be placed into the joints of his spine with the goal of regeneration of cartilage as well as for an anti-inflammatory effect. Dr. Ruiz-Cotorro predicts that Nadal can return to training in early December.

Will stem cell treatment work for Nadal’s Back Pain?

Stem cell treatment may seem logical in certain situations – for example, if you have a mechanical problem where a knee has worn out cartilage, causing bone to rub against bone, it makes sense to use stem cells to grow new cartilage to have a cushion to protect the bone and cause the knee to be less painful. As much as we may want to see him back on the courts tennisgrabbing more grand slam titles, if Nadal’s stem cell treatment is being used to eliminate his pain by repairing his joints or discs, the actual cause of his back pain may not be addressed.

Where does back pain originate?

The number one reason for back pain is muscular and other soft tissue, yet muscles are rarely evaluated as the cause of back pain. The only way to determine if Nadal’s back pain is from soft tissue and similar to most people with back pain would be a physical examination of Nadal’s back that included identifying possible muscles as the cause of his pain.

Some doctors believe that the disc, the cushion between the bones of the spine (the vertebra), is a major cause of back pain. They believe that surgeries to correct the flattening or herniation of the disc will decrease or eliminate back pain. Sometimes they are right, but they are just as likely to be wrong. The truth is that there is as high as a 50% failure rate for spine surgeries that were done to eliminate back pain thought to be related to disc problems. There are other joints in the spine that are thought to cause pain; one of them is the facet joint, which could also be a target for stem cell treatments.

When doctors rely on an MRI or CT scan to determine the source of the pain, the information obtained is often confusing. If a surgeon sees an abnormality on an MRI, he will often point to that abnormality as the cause of the pain; in my experience the abnormality found on an MRI or CT scan frequently is not the cause. In fact, if you randomly selected 100 people off the street, and perform an imaging scan, 40 may present with a herniated disc and have no pain and absolutely no awareness of their herniated disc; 70 may have degenerated (worn) discs with no pain, and a large number will have facet joint abnormalities. Therefore, finding an “abnormality” is more common than not. One, then, can deduce that the abnormality is more likely NOT the source of the pain. So treating the abnormality (with steroid injections, surgery, or stem cells) may therefore not relieve the pain.

Stem Cell Treatment and Sports Stars

Nadal, currently ranked as the number 3 professional tennis player in the world, is not the first sports star to chase after a “miracle cure.” The Denver Bronco quarterback Payton Manning and Yankees pitcher Bartolo Colon both went abroad to seek out stem cell treatment as a quick fix to get back in the game. (They both seem to be doing better overall, but it is inconclusive if the stem cell treatment was the cause of their recovery).

Will it work?

In the laboratory, it has been possible to demonstrate the ability of stem cells (most commonly found in the developing embryo and newborn) to grow new tissue. These cells are like silly putty; they can turn into, or adapt, to become any type of tissue. For example, a stem cell in the right environment in the body could become bone, cartilage or some organ (for example, liver or pancreas). But, it hasn’t been as easy to grow tissue in an actual person. There are some early studies that show that stem cells “may” relieve back pain, but both the doctors who are testing the technique and outside experts say much more research is needed before they can say whether the treatment offers real relief.

The use of stem cell therapies continues to be a hot topic for debate in the sports medicine and orthopedic surgery worlds. There is no current evidence-based research to prove that it works.

Sir William Osler, a famous physician, once said: “Use every new treatment as quickly as possible before it stops working.” Stem cell treatment needs to be further investigated to determine if stem cell treatments indeed work, and if so, for what conditions?

 

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Medical Mystery

For seven years, searing pain with no relief – that was the title of The Washington Post story that peaked my interest. So I read the article and discovered that Charon Wicker had been experiencing a burning, throbbing, excruciatingly painful sensation in her index finger – for seven years – even though she had:

  • Repeatedly consulted pain specialists and orthopedic surgeons, internist and endocrinologist; and even a hand surgeon;
  • Undergone two operations to replace the herniated disks in her neck;
  • Taken all sorts of painkillers and become dependent on the sleeping pill Ambien;
  • Received X-rays, MRI scans, and a nerve conduction test all negative);
  • Spent months in physical therapy;
  • Took a variety of anti-inflammatory drugs, increasingly strong painkillers, including oxycodone.

But nothing worked. Ms. Wicker’s pain continued.

The Case of Sore Fingertip looked as if it was a case for Sherlock Holmes (or his creator Dr. Arthur Conan Doyle) or Dr Gregory House.

It made me think — Be careful whom you ask for help!

And finally, Charon did get the right help from a hand surgeon who, listened to his patient; reviewed her medical history; and then examined her fingertip – where she had been complaining of pain for seven years, carefully. He saw a “slight bluish discoloration underneath her nail,” and found the cause of her pain — a rare benign vascular growth – smaller than the head of a pin – a tumor. He removed the glomus tumor and the pain was gone.

It’s uncommon to find that persistent pain is caused by a glomus tumor – but it is common that someone with persistent pain will be offered spine surgery to help them.

It is unfortunate that in Ms. Wicker’s case, she underwent TWO aggressive spinal surgeries and the pain continued. Even though her pain was in her fingertip (something that would clearly not be caused by a faulty disk or helped with insertion of an artificial disc in her neck) and there were no signs or symptoms that pointed to spine surgery to mitigate the pain, surgeons suggested two separate operations to replace the spinal disks. Whereas drugs have to go through extensive testing to show they are safe and effective, surgical procedures do not.  When the first surgery didn’t work, the suggested solution was another surgery to replace another the disc.

In my practice, I have seen many patients over the years who come to my office complaining of chronic neck, shoulder, low back and/or leg pain that continues after “failed back spinal surgery. The herniated disc that was discovered on an MRI didn’t explain the source of their pain.

MRIs can show doctors beautiful pictures of the bones and the material separating the bones (the discs) of our spines but interestingly an MRI of the spine will usually find some “abnormality” in most adults – the majority of the time without any pain complaint. Desperation is often one of the reasons that patients undergo very invasive procedures based on an MRI and not supported by the clinical picture.

Sometimes a surgery works but how could it in The Case of Sore Fingertip? It didn’t and as Sherlock would say, “It’s elementary, my dear Watson.” It just didn’t make sense.

You can read the article in full here.

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Men:
Where do you keep your wallet? Many of you probably answered, “your back pocket.” Did you know that this could be causing your back pain?

When you sit down with your wallet in your back pocket, it presses into your muscles, which can cause pain in your low back and buttock. The squeezed muscle can press your sciatic nerve causing the pain to travel down your leg.

If you’re suffering from back pain, try keeping your wallet in an external bag or a front pocket. Or simply decrease the thickness of your wallet by taking out any extraneous items, such as old receipts or credit cards that you no longer need or use.

 

Women:

Those fashionable Jimmy Choos or Manolo Blahnik’s, which are known for their height, may actually be increasing your low back pain. But don’t switch out those high heels for the increasingly popular ballet flats or stylish sandals because flats can stretch and strain the muscles in the calves and thighs causing pain in the buttock and low back.

You don’t have to forgo fashion for comfort. Wearing low, comfortable heels can save your back from unnecessary pain. Remember to wear shoes that fit properly as well – ill-fitting shoes can cause you to tighten your leg and buttock muscles causing low back, buttock, and leg pain.

 

Children:

Now that school is back in session, many kids are carrying heavy books in their backpacks. Often, the heavy, incorrectly worn backpacks can cause poor posture and strain on muscles. Parents, have your child(ren) pack their backpacks so that the heaviest books are placed towards the back of the bag. This way, the heaviest part of the bag is closer to the back of the child. (see picture) Also, remind your child(ren) to use both straps on both shoulders. This helps distribute the weight evenly, as opposed to placing strain on one shoulder. Another option is using backpacks with rolling capabilities.

Similarly, all you ladies who stuff your shoulder bags with everything from lipstick to workout weights, carrying a heavy bag on your shoulder every day can be the source of your aching neck and back. While those oversize totes conveniently contain everything you might possibly need in a day, the accumulated stress on your neck and spine from “pocketbook overload” can lead to serious strain on your muscles, tendons, nerves, and ligaments, causing serious body aches. So what does a fashionable woman on the go do?

  • If you absolutely must carry a tote, lighten the load – try to take out the items you don’t really use or need.
  • Switch the bag from one shoulder to the other often, minimizing the amount of time you strain the shoulder and neck muscles.
  • Try using two lighter bags instead of one big one. Splitting the load and carrying them on both sides distributes the weight evenly.

And don’t forget; no matter what you carry, having good posture helps reduce back pain.

children backpack

Parents:

Lifting your baby 50 times per day, or bending and lifting up to get your active toddler out of his/her sibling’s hair, are innocent movements that can indeed cause low back pain. Good posture and proper lifting techniques can help parents avoid the increased risk for low back pain. Some key pointers:

  • Keep your feet (shoulder width) apart, your back straight and bend your knees to reach for your child.
  • Use your leg muscles, which are bigger and stronger than your back muscles, to lift your child. After you bend over, bring your child to your chest, bring your back to the upright position, and then straighten your legs. If you straighten your legs before bringing your body upright, you’re using your low back muscles to lift your child. However, if you straighten your legs last, then you’re using your leg muscles.

Keep in mind; this is a good tip for lifting anything heavy!

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Eating forbidden foods, losing weight and helping your heart

I had dinner with Dr. Robert Atkins years before his untimely death. He ordered a juicy marbleized steak with a baked potato drowning in butter and sour cream. At the time I thought this wasn’t a healthy choice- I was wrong! A front page article in the NY Times earlier this week reports on a large year-long NIH funded study published in the Annals of Internal Medicine supporting the ideas of Dr. Atkins, viz. high fat/ low carbohydrate diets are better for your health than equal calories of high carbohydrate/ low fat diets. You not only lose more weight but your total cholesterol goes down and the good cholesterol (HDLs) goes up. This is a great example of showing us that what we firmly believe to be the truth (i.e. a low fat diet is best for your diet and health) may not be so. An important issue in this study is that both groups lost weight. Calorie restriction does work to lose weight- but it may be easier for some patients who have difficulty dieting, to limit their carbohydrates, still feel satisfied, and lose weight.

vegetables-and-fruits-farmers-marketAs I report in my book End Back Pain Forever, a previous NIH funded study also showed that calorie reduction consistently resulted in weight loss; this new study reaffirms that low carbohydrate/high fat diets are healthier for your heart. In my book I reported that eating plant rather than animal protein/fat was preferable if you were concerned about lowering levels of the bad cholesterol (low density lipoproteins-LDLs). If you’re thinking of becoming a vegetarian- eating a vegetarian diet has been shown to produce the lowest levels of cholesterol and in general to be associated with the lowest BMIs (Body Mass Index, which is a measure of body fat based on weight and height.). The carbohydrates that are most damaging are refined carbohydrates such as found in white flour and sugar. No matter what diet you choose you need to get the minimal amounts of essential minerals and vitamins, therefore always include healthy amounts of fresh fruits and vegetables.

To paraphrase Mark Twain, it’s very easy to diet and lose weight- I’ve done it twenty times; The trick is to keep it off once you’ve lost it. Here are some tips from my book:

1. Increase your physical activity. If you never walk anywhere, start to do that. If you walk two blocks a day, increase it to four. If you go to the gym once a week, increase it to twice a week. The idea is to begin where you are now and increase slowly and steadily. Don’t take on more than you can handle, but work your way up. Make yourself a winner by taking on an achievable goal.

2. Don’t attempt total deprivation. Avoid starvation diets. Your body senses that you are starving, and it adjusts by slowing your metabolism, the rate at which calories are burned. This makes it harder to lose weight. Being able to lose weight and maintain the loss requires changing the way you eat and increasing physical activity. Just as you changed your activity level gradually, do the same with diet. In other words, start slowly. Make one simple change. I recommend to my patients that they stop eating bread and pastries. Some love bread and can’t imagine living without it. They soon learn, though, that after a short while, the craving for bread diminishes and their weight starts to drop. Then stop eating pasta or only allow it (and bread) as a treat; let’s say on Sundays.

3. Learn how many calories are in the food that you eat. When patients tell me that all they eat is salad, but they’re gaining weight, I have to remind them that any sauce or dressing may contain high-calorie ingredients. When you are out at a restaurant, it is best to avoid foods that may hide unwanted calories.

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Pain is the most common reason that people seek medical attention. Pain affects tens of millions of Americans and costs the United States billions annually in medical care and indirect costs from missed workdays and loss of productivity.

ID-100202691As I mentioned in an earlier blog, “Concerns about the Growing Misuse of Opioids,” a common treatment for severe chronic pain is strong medication such as opioids. However, some patients who receive opioids (for example: morphine or oxycodone) frequently need more and more of the drug to get the same pain relief. This is called tolerance.

Some patients continue to have pain despite their taking increasing doses of opioids, and may actually experience an increase in pain as a result of their increased medication. Additional medication in such patients will lead to ever increasing pain. This phenomenon is called Opioid Induced Hyperalgesia (OIH). OIH is diagnosed when there is no added relief with higher doses of your pain medication and you observe:

  • Your pain becomes different than the pain you originally had.  It may become widespread, extending to new areas.
  • The quality of the pain may change.  For example, you may experience allodynia, a condition in which normal sensation, such as light touch or a simple movement becomes painful.

Although the mechanism by which this occurs isn’t fully understood yet, cells not previously thought to cause pain, such as microglia, appear to be involved. At the Norman Marcus Pain Institute, I use very small doses (referred to as ultra low-dose) Naltrexone, which can block the effects of the microglia. This can not only decrease opioid side effects but in some patients reduce or eliminate the drug completely. It is clear that long-term use of opioids can do more harm than good for some patients.  Therefore, it may be wise to periodically attempt to decrease the amount of opioids to see if this either results in no increase or an actual decrease in pain.

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Is Bad Weather To Blame For Your Lower Back Pain?

A New York Times article Weather May Not Affect Back Pain, printed mid-July, cited a study showing that weather may not affect back pain. The purpose of the study was to explore the often-heard complaint from patients diagnosed with persistent pain related to muscles and bones (musculoskeletal pain) that weather changes cause an increase in pain. The study was based on an analysis of 993 patients who had seen their physician in 2011-2012 complaining of an episode of sudden back pain. These were not patients with a history of back pain.

So this article does not actually examine patients who already have back pain to see if the pain is increased with bad weather. In my practice, at NMPI, I have patients who can tell me a snowstorm is coming 3 days before it occurs. Although studies of the association between weather and pain are not uniformly supportive of the connection, a study done in 1995 of 558 patients with chronic pain found that 2/3 experienced an increase in pain and most of them prior to the occurrence of the bad weather.

bad weatherI have patients whose pain is so severe with bad weather that they can hardly get out of bed. Although there is no good scientific explanation for this association, some suggestions have been offered. When the barometric pressure falls, the air pressure in a painful joint may continue to be a little higher than the air pressure on the outside of the body, causing an increase in pain. Another explanation is that cold and/or inclement weather decreases the amount of time you are outside and active. Patients with musculoskeletal problems (i.e. muscle pain and arthritis) often feel worse with inactivity. In addition cold weather causes the blood vessels in the hands and feet to constrict, which can decrease the amount of available oxygen, resulting in increased muscle pain.

If your history indicates that bad weather causes an increase in your pain, it is likely that your flare up is most likely not an indicator of physical deterioration. Additional pain can be reasonably treated with increased medication for the brief period of weather related pain.

 

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Because of the growing problem of addiction, misuse, and diversion, 49 states have now adopted a state prescription drug database.  You may have read an article recently in The New York Times about Missouri being the only state that has not adopted such a database. In New York, as a prescriber of controlled substances, each time a patient is prescribed any type of controlled substance, I must log into the NYS website to confirm that a patient is not receiving other medications from other doctors.

prescriptionsI found a few patients who had not been honest with me and had received medications from other doctors. Unfortunately, the small occurrence of dishonest behavior has obliged all doctors to be alert for the possible misuse of medication.  At the Norman Marcus Pain Institute, I implement several rules for patients receiving any type of controlled substance from me. Here are a few of them:

•             Only one physician can prescribe all pain medications.

•             Only one pharmacy should be used to obtain all pain related medications.

•             All medications, including herbal remedies and over the counter medications, need to be reported since all medications can interact with one another.

•             Medications must be kept in a safe and secure place, such as a locked cabinet or safe.

 

Following these simple rules will help protect my patients and their families from improper use of pain medication.

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From 1991 to 2009, the number of prescriptions written for the strongest pain medications tripled. These medications are collectively named opioids and include morphine, oxycodone, and hydrocodone.Opioids are a type of pain drug that may cause serious side effects. From 2005 to 2009, the number of emergency room visits for nonmedical use of prescribed pain medication doubled. Therefore, the states and federal governments are acting to try to limit the amount of pain medication being prescribed.

At NMPI, we focus on finding the most effective treatment to relieve our patients’ pain – without surgery, steroid injections, or heavy painkillers. However, when a patient complains of severe pain and is not responding well to other pain medications, stronger, prescription opioids will be given.

 
Pain pills blog-Norman Marcus Pain Institute-blogSome opioids, such as oxycodone, are often combined with Tylenol (acetaminophen) in one pill. Some examples of these combination drugs are Lorcet, Lortab, Norco, Vicodin (are all hydrocodone and acetaminophen), and Percocet (oxycodone and acetaminophen). Most often, severe pain is related to a condition that will improve over time. Ideally, all prescription pain medications should be given only while severe pain persists. Like any medication, there are risks and side effects:

 

• Opioids can make you drowsy – which raises the risk of falling and severe injury
• Opioids when mixed with alcohol, anti-anxiety medication, seizure medication, muscle relaxants, or sleep-aids can be deadly.
• Opioids cause constipation and can lower sex drive.
• Patients can become physically and psychologically dependent on opioids.
• Overtime a patient with chronic pain can develop a tolerance for the opioid and need a higher dosage.

Keep in mind that not all pain requires such strong medication, and most patients with pain can be managed with drugs such as acetaminophen (Tylenol) and aspirin-like drugs, called non-steroidal anti-inflammatory drugs (such as ibuprofen and Naproxen). When taken as directed, these less powerful drugs may be all that you need. (There are potential serious side effects with acetaminophen and NSAIDs, which I will discuss in a future blog.) For certain pains, some drugs are better than others. Sometimes we find that a drug we have been using may not actually be effective. A New York Times article on July 23, 2014 reported that for treating low back pain, acetaminophen was no better than a placebo.

Physicians have a responsibility to properly care for patients in pain. Some of these patients may appear to be at a higher risk to abuse opioids. Occasionally, patients complain of non-existent pain to obtain opioids for its mood-altering affect, called a “high.” The fact is that physicians who had been writing too many prescriptions for pain medication are now wary of prescribing any potentially habit-forming pain drugs. This has resulted in a decrease in emergency room visits for drug overdose and deaths from overdose, but it has also resulted in depriving many patients of medication they legitimately need to function normally.

At NMPI, when we treat patients in pain who have a history of drug abuse or who test positive on a written test to determine the risk of abuse, I believe that these two basic American traditions should be the guiding principles:

1. Innocent until proven guilty; and

2. In the words of Ronald Reagan, Trust but verify. Those patients who have problems or are at risk to not properly use pain medication need extra attention, not condemnation. They may be more difficult to treat, but that is why there are specialists to deal with complex pain problems.

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