Monday, 28 July 2014

How Important Is Endostatin To The Nervous System?

Endo...what? Today's post from (see link below) talks about a protein called endostatin and its potential value in the workings of the nervous system. Sometimes discoveries are made by accident and endostatin which was previously thought to be able to cut off the blood supply to cancers, has been found to be a key protein in maintaining healthy synapses, which are places where nerves communicate. Now I can't pretend to understand how this works, or what benefits may result form this discovery but congratulations if you can after reading this article. In short, it seems that this protein may be able to stabilise nerve pathways and one thing neuropathy patients do not have, are stable neural pathways. Maybe we'll be hearing the word 'endostatin' much more in the future.

UCSF Researchers Uncover an Unexpected Role for Endostatin in the Nervous System
Protein Once Seen as a Promising Anti-Cancer Compound Helps to Stabilize Neural Circuits
By Pete Farley on July 24, 2014

Researchers at UC San Francisco have discovered that endostatin, a protein that once aroused intense interest as a possible cancer treatment, plays a key role in the stable functioning of the nervous system.

A substance that occurs naturally in the body, endostatin potently blocks the formation of new blood vessels. In studies in mice in the late 1990s, endostatin treatment virtually eliminated cancer by shutting down the blood supply to tumors, but subsequent human clinical trials proved disappointing.

“It was a very big surprise” to find that endostatin, through some other mechanism, helps to maintain the proper workings of synapses, the sites where communication between nerve cells takes place, said Graeme W. Davis, PhD, Hertzstein Distinguished Professor of Medicine in the Department of Biochemistry and Biophysics at UCSF and senior author of the new study. “Endostatin was not on our radar.”

The findings were reported online July 24 in the journal Neuron.

Synapses are continually shaped and reshaped by experience, a phenomenon known as plasticity. But for those changes to be meaningful, said Davis, they must take place against a stable background, which paradoxically requires another form of change that he and colleagues call “homeostatic plasticity.” Just as we change our pace, slowing down or speeding up, to keep abreast of a running partner, neurons adjust aspects of their function at synapses to compensate for changes in their synaptic partners brought on by aging, illness, or other factors.

In an example of homeostatic plasticity, in the neuromuscular disease myasthenia gravis, as muscle cells become less responsive to the neurotransmitter acetylcholine, nerve cells ramp up their secretion of the neurotransmitter to keep the system in balance for as long as possible. Some researchers believe that in other disorders, including autism and schizophrenia, a failure in such homeostatic mechanisms keeps synapses from functioning properly.

In previous research Davis noticed that applying a toxin to a muscle cell in the fruit fly Drosophila melanogaster triggers homeostatic plasticity in the neuron that forms a synapse on that muscle cell: the neuron--which is called presynaptic, because it is “before” the synapse with the muscle cell--reliably releases more neurotransmitter, just as happens when muscle cells begin to malfunction in myasthenia gravis.

Davis has since built on this model of homeostatic plasticity by painstakingly knocking out Drosophila genes one by one and recording from presynaptic neurons to see which genes are necessary for the homeostatic response, because it is these genes that may be compromised in diseases affecting the process.

“So far we’ve tested about 1,000 genes this way, which has entailed close to 10,000 recordings,” Davis said.

Using this technique Davis and colleagues observed at one point that knocking out a gene called multiplexin significantly hampered homeostatic plasticity in presynaptic neurons. But because that gene helps to form a structural protein known as collagen—which in humans is a component of ligaments, tendons, and cartilage—the finding wasn’t immediately considered relevant to synaptic function.

The team learned that the multiplexin protein can be snipped by an enzyme to produce endostatin, so in experiments led by postdoctoral fellow Tingting Wang, PhD, they tested whether endostatin might play a role in homeostatic plasticity.

“Nobody picked up multiplexin to work on for a couple of years, because we didn’t think a collagen could be that interesting,” Davis said. “Then, when a new postdoc, Tingting Wang, came to the lab, we started thinking about it harder.”

When the group genetically deleted the portion of Drosophila multiplexin that forms endostatin, presynaptic neurons behaved normally, but homeostatic plasticity was severely compromised when toxin was applied to postsynaptic muscle cells. On the opposite side of the coin, when the team overexpressed endostatin at Drosophila synapses lacking multiplexin, homeostasis was restored, whether endostatin was expressed in muscle cells or presynaptic neurons.

The research team is unsure precisely how and where endostatin exerts its effects on homeostatic plasticity, but they believe that multiplexin is cleaved at the postsynaptic site to form endostatin, and that the endostatin signal is conveyed to the presynaptic neuron to alter its function. “Because so many people in the cancer world have studied endostatin, there is a great set of tools available” to study the protein, Davis said, so he expects his group to make rapid progress in addressing these questions.

“Despite its checkered history in cancer, we know endostatin is a signaling molecule and we know that the brain has a great deal of collagen—we just haven’t known what it does, and we certainly don’t know what endostatin’s receptors in the brain might be.” Davis said. “But it’s pretty exciting to think about a new signaling molecule with a profound role in the stabilization of the function of neural circuits.”

In addition to Davis and Wang, the research team also included Anna G. Hauswirth, a student in UCSF’s Medical Scientist Training Program; research specialist Amy Tong; and postdoctoral fellow Dion Dickman, PhD. The research was supported by grants from the National Institutes of Health.

UCSF is the nation’s leading university exclusively focused on health. Now celebrating the 150th anniversary of its founding as a medical college, UCSF is dedicated to transforming health worldwide through advanced biomedical research, graduate-level education in the life sciences and health professions, and excellence in patient care. It includes top-ranked graduate schools of dentistry, medicine, nursing and pharmacy; a graduate division with world-renowned programs in the biological sciences, a preeminent biomedical research enterprise and two top-tier hospitals, UCSF Medical Center and UCSF Benioff Children’s Hospital San Francisco.

Sunday, 27 July 2014

How To Cope With An Invisible Illness

Today's post from (see link below) looks at the frustrations encountered when you're faced with vague symptoms, sometimes linked with chronic pain, that have not been properly diagnosed and identified. Many people with neuropathy are in exactly this position and feel that they're invisible to the rest of the world. This can lead to isolation and a feeling that it's not only others who think that it's all sitting between your ears. Very often neuropathy patients are having to deal with problematic, autonomic symptoms (autonomic means the involuntary functions of the body - breathing, digestion, sexual function, sweating etc etc) and this can be both alarming and virtually impossible to explain to family and friends, never mind doctors. This article gives some useful support for those in that situation. Autonomic disorders are some of the worst side effects of nerve damage in your body. Don't give up and listen to your body - the doctors and people in your circles will catch up in the end.

Invisible Illness: How to Sustain Hope?
Deborah J. Cornwall Become a fan Author, 'Things I Wish I’d Known: Cancer Caregivers Speak Out' Posted: 07/11/2014 1

Think about your everyday life -- how taking a run, or walking your dog on the beach, or socializing with friends are just part of a normal day. How you share daily responsibilities with others. How you set long-term goals and arrange your life to pursue them.

Then imagine never knowing each morning whether today you'll be able to do those things you take for granted, or if today you'll be unable to get out of bed. Today it might be vertigo and stomach pain. A couple of days ago, it could have been crippling fatigue and muscle pain, or almost fainting. Tomorrow or the day after, it could be a migraine, or a racing heartbeat, or severe insomnia, or all of these at once.

Imagine not knowing which symptoms will hit, and when, and never being able to plan for tomorrow, let alone for your whole future. And imagine a world in which it's extremely hard to find doctors who take seriously that your discomfort is really physiological, a world in which the right treatment for you is elusive.

That's the world of autonomic disorders, like POTS (Postural Orthostatic Tachycardia Syndrome, sometimes called simply Postural Tachycardia Syndrome) and Neurocardiogenic Syncope. In these conditions, body systems that are supposed to work automatically -- controlling your heartbeat, blood pressure, breathing, thinking, temperature, digestion, vision, and more -- don't work as they should and create a plethora of potentially disabling symptoms. This world could force you to give up your career dreams, narrowing your total focus to just getting through today. Such conditions are called "invisible" because you may not actually look sick when you are most displaced from normal living.

Diagnosis and Treatment Planning Can Take Years

Researchers and clinicians don't yet understand the causes for the wide variety of autonomic disorders, why symptoms present differently for every patient, or why each patient's condition varies so greatly from day to day. As a result, treatment for each patient focuses on managing symptoms and may involve a process of trial and error.

A prior article on this topic, "5 Things to Do When Symptoms Are Not 'All in Your Head,'" described Val's multi-year effort to figure out what was wrong with her body. She is not alone in her struggle. Clare's husband described that 18 different physicians took them through nine different diagnoses over 15 years before concluding that she too had POTS. He says it was an unpleasant process:

The treatment that Clare has received has been so degrading and appalling that when we actually found a doctor who was willing to listen to the full story and believe that there was something physically wrong, we were overjoyed. Previously, when blood work and tests kept coming back as normal, many doctors would actually ask me if she was "that fragile type of woman who gets hysterical or over-reacts." Some would ask me that right in front of her, as though she couldn't hear us or her opinion didn't matter.

Kelly's mother described another example of what that kind of interaction with a doctor looks like:

I watched Kelly evolve from being an executive powerhouse and "super Mom" to experiencing severe physical crashes that required emergency hospitalization. When five days of tests all came in as normal, the doctor in charge announced, "We can't find anything wrong with you. You have panic attacks, so I'm sending you home with anti-anxiety drugs and a recommendation to see a counselor." I tried to explain that her heart is jumping out of her body, with a sky-high heart rate one minute and a blood pressure so low that she nearly faints, and then it goes back down when she lies down. When I insisted that those symptoms were real and not psychological, he actually said, "You're not helping your daughter one bit," and stormed out of the room.

Val, Clare, and Kelly represent only the tip of the iceberg.

Patients Are Frustrated, PCPs Are Challenged

Dr. Satish R. Raj, M.D., MSCI, FACC, is a clinician, researcher, and associate professor of cardiovascular sciences at the University of Calgary and an adjunct associate professor of medicine and pharmacology at Vanderbilt University. He is one of the world's experts in the research and clinical aspects of autonomic disorders. He explained that diagnostic delays, especially at the PCP gateway, may occur because symptoms like lightheadedness, chest pains, shortness of breath, and GI upset can be caused by many other medical conditions.

"When you have vague and nebulous but real and sometimes disabling physical symptoms, like crushing fatigue," he says, "and your tests come back negative, it may be hard for a PCP to determine what's wrong. Multiple simultaneous symptoms make it difficult to predict a single course of investigation to reach a definitive diagnosis." As a result, physicians gravitate toward the more prevalent conditions first as the possible causes and look for factual data in test results to rule those in or out.

A Web-based survey implemented by Dysautonomia International in December of 2013 and released on July 11, 2014, reveals stunning statistics about the proportion of such patients who spend years trying to learn why they can no longer function "normally." Nearly 700 respondents with POTS told a shocking story:
Almost six years: The average time from first symptoms to diagnosis. For over 20 percent, it took more than 10 years. This didn't include time to determine (by trial and error) what treatments might relieve symptoms.
83 percent: Proportion of respondents who received a psychological diagnosis before eventually being diagnosed with POTS: In fact, research has shown that POTS patients are actually less anxious than the general population.
12 percent: Proportion diagnosed by a pediatrician or primary care physician.
27 percent: Proportion who saw more than 10 different doctors before being diagnosed. Some 8 percent saw more than 20 doctors.

Given prevailing estimates that these conditions affect between 500,000 and 3,000,000 individuals (disproportionately young women) in the United States, perhaps these patients deserve more timely support from the medical community.

Saturday, 26 July 2014

Dealing With Neuropathy

Looking at the title from today's post from (see link below) may suggest that it has nothing to do with you and you shouldn't read on (unless you also have diabetes of course). However, because diabetes is by far the most common cause of neuropathy, you tend to find many more articles related to that disease than anything else. If you have neuropathy, the cause is just one element in the puzzle; once you have it, you share the same symptoms (generally) with everybody with neuropathy and therefore all articles can be useful and maybe teach you something you didn't know. This particular article is fairly general and many people will recognise the content and already be aware of it but for people just trying to establish the basics of what's happening to them, it may be helpful.

Dealing with Diabetic Neuropathy
Posted on February 7, 2014 by Staff in Diabetes Basics

According to a recent national survey by the American Diabetes Association (ADA), the majority of people with diabetes have symptoms of diabetic neuropathy (nerve damage). But, only a small minority has been diagnosed with this condition. Diabetic neuropathy is a complication of diabetes that leads to sensations of pain and/or numbness, tingling or “pins and needles” in the feet and hands. According to study findings, a staggering 56 percent of people with diabetes have never even heard of the condition.

This lack of awareness is unfortunate for the 18.2 million people in the United States living with diabetes. The symptoms can make it hard to function, and because the pain of diabetic neuropathy usually is worse at night, many have difficulty sleeping. The nerve damage can make a person extremely sensitive to even the lightest touch, and simply wearing socks or having a foot touch a bed sheet can cause pain. People with this condition may have difficulty walking, working or socializing. And diabetic neuropathy is a major risk factor for foot injury, infection and amputation. Left untreated, diabetic neuropathy always progresses.


With attention, management and treatment, diabetic neuropathy can be prevented or delayed. Awareness is the first, critical step in taking care of yourself and preventing serious problems in the future.

That’s why the ADA has embarked on an awareness campaign to educate people with diabetes about diabetic neuropathy. The campaign focuses on the seriousness of this complication, recognizing the symptoms and most important, the things people can do to prevent, reduce or manage these symptoms. There is a patient “pocket checklist” so that you can review a list of symptoms. If any of these symptoms apply to you, take this list to your health care provider; ask if you have neuropathy and what you can do about it. For more information or to get your free copy of All About Nerve Damage & Diabetes, call the ADA at 1-800-DIABETES (1-800-342-2383), or visit


The most common type of nerve damage in people who have diabetes is called peripheral neuropathy, which affects the feet and hands. Reporting symptoms to your health care provider can help prevent future complications, such as amputations. Take this list to your next office visit. Symptoms Place a check mark next to any symptom you have:

My feet tingle.
I feel “pins and needles” in my feet.
I have burning, stabbing or shooting pains in my feet.
My feet are very sensitive to touch. For example, sometimes it hurts to have the bed covers touch my feet.
My feet hurt at night.
My feet and hands get very cold or very hot.
My feet are numb and feel dead.
I don’t feel pain in my feet, even when I have blisters or injuries.
I can’t feel my feet when I’m walking.
The muscles in my feet and legs are weak.
I ’m unsteady when I stand or walk.
I have trouble feeling heat or cold in my feet or hands.
I have open sores (also called ulcers) on my feet and legs. These sores heal very slowly.
It seems like the muscles and bones in my feet have changed shape.
Other symptoms I have include: ____________________________


Talk with your health care provider about treatments available to relieve pain and reduce burning, tingling and numbness.

Friday, 25 July 2014

Exercise Can Help Neuropathy (Vid)

Today's post from (see link below) is primarily aimed at people who are living with neuropathy as a result of cancer treatment but it applies to all neuropathy patients and is worth looking at to see if there is anything that may help you personally. It's another post offering exercise as an option for reducing the impact of symptoms and most neuropathy patients will run screaming to the hills at the very idea. However, it proposes low-impact routines which are designed to help with blood flow, sensation, strength and balance and these can be very useful. You need to listen to your own body and not go too far but pushing yourself just a little will reap benefits. Browse through the slides and see what you think.

How Exercise Can Help Neuropathy
Dana-Farber July 23, 2014

For many patients treated with chemotherapy, peripheral neuropathy can be an uncomfortable and sometimes dangerous side effect. The condition, which includes tingling or loss of sensation in the arms or legs, can increase risk for falls and fall-related injuries.

To help prevent and ease these problems, Dana-Farber exercise physiologist Nancy Campbell, MS, recommends patients use low-impact exercise routines like finger taps, calf stretches, and ankle rolls. These exercises help increase blood flow to the peripheral nerves, restoring feeling in the extremities. The routines also build strength and improve balance, which can lead to fewer falls.

View Campbell’s presentation below for more information on how exercise may help ease or prevent neuropathy, including step-by-step instructions for helpful exercises:

How Exercise Can Help Neuropathy from Dana-Farber Cancer Institute

Thursday, 24 July 2014

Nerve Damage: What's Going On?

Very short and simple but very useful for people new to neuropathy, todays post from (see link below) sums up nerve damage and which nerves are involved in a nutshell. Thanks to the complex nature of neuropathy, it's so easy to forget the basic details of what's happening to your body. This short article is a useful refresher for everybody living with neuropathy.

Neuropathy and Nerve Damage 
Info from WebMD: Never Damage
Your nervous system is involved in everything your body does, from regulating your breathing to controlling your muscles and sensing heat and cold.
There are three types of nerves, or neurons, in the body: 

Autonomic nerves.
These nerves control the involuntary or partially voluntary activities of your body, including heart rate, blood pressure, digestion, and temperature regulation.

Motor nerves. These nerves control your movements and actions by passing information from your brain and spinal cord to your muscles.

Sensory nerves.
These nerves relay information from your skin and muscles back to your spinal cord and brain. The information is then processed to let you feel pain and other sensations.

Nerve pain and nerve damage can be mild. But, because nerves are essential to all you do, nerve pain and damage can seriously affect your quality of life.
What Are the Symptoms of Nerve Pain and Nerve Damage?

With nerve damage there can be a wide array of symptoms. Which ones you may have depends on the location and type of nerves that are affected. Damage can occur to nerves in your brain and spinal cord. It can also occur in the peripheral nerves, which are located throughout the rest of your body.

Autonomic nerve damage may produce the following symptoms:

inability to sense chest pain, such as angina or heart attack
too much sweating (known as hyperhidrosis) or too little sweating (known as anhidrosis)
dry eyes and mouth
bladder dysfunction
sexual dysfunction

Damage to motor nerves may produce the following symptoms: 

muscle atrophy
twitching, also known as fasciculation

Sensory nerve damage may produce the following symptoms:

tingling or prickling
problems with positional awareness

In some instances, people with nerve damage will have symptoms that indicate damage to two, or even three, different types of nerves. For instance, you might experience weakness and burning of your legs at the same time.

Wednesday, 23 July 2014

How Do You Describe Your Neuropathy Self?

Today's post from (see link below) is a sort of self-help article, the like of which many neuropathy patients are used to and generally find that 'it's easy for them to say!' However, there are some good points here and it does show how easy it is to talk yourself into feeling worse than you actually do by your choice of words. When you describe your symptoms to your doctor, or others, do you immediately go for the worst case scenario and use language to try to convince the listener that you're not faking it? It may be perfectly understandable (we face enough disbelief and incomprehension as it is) but you may not be doing yourself any favours because you may end up believing your own descriptive language. Talking your pain and discomfort down a bit may help to reduce the impact psychologically. After all, what you're feeling is bad enough without exaggeration of any sort. Certainly something to think about.

The Words You Choose Make a Difference  
Posted by Dionetta Hudzinski | July 16, 2014

Words are the building blocks of language. We use words to communicate our thoughts, ideas, beliefs and emotions. Words can have specific meaning or a variety of meanings depending how they are used in conversation. Words can convey a clear message or a confusing message. Some words are gentle and comforting while others are harsh and distressing to hear. It is HOW we use words that matters most.

Words can explain, define, label, clarify or confuse. Words can influence (positively or negatively) our beliefs about us and our beliefs about others. Words can influence how we think; how we think can influence how we feel.

So what does this have to do with pain management, you might ask? Lots! First our self-talk and the words that we use can either build us up or tear us down. I know, I was the worst offender in this area. The words I chose to speak to myself were harsh, condemning and not nice at all. I would never use those same words to talk to friends or family but I was using them on myself. Words like lazy, worthless, failure, you’re not enough, you’ll never succeed at that…these and so many others; no wonder I was feeling less than everyone else, feeling like a failure with no motivation to change. Why change? My self-talk told me it was not even worth starting so why bother? At one of my lowest points I decided to take an online course which changed my life…in fact the course was called “Change Your Life In 30 Days” with Rhonda Britten famous TV Life coach of the Starting Over TV reality show. I learned that words carry energy and that I need to choose my words carefully and wisely. Slowly over 30 days I stopped the negative self-talk and replaced it with positive thoughts. I learned how to be gentle with myself and how to practice self-compassion. In fact, I came up with a little affirmation that I say even today when a negative thought pops up…”I forgive myself for having that thought; I choose love instead”. My overall vocabulary changed and with it my attitude and motivation.

Vocabulary matters! Think of words that people use to describe people in pain. Words that make you cringe when you read the latest news article about prescription drug abuse. Words like – narcotic, addict, doctor shopper, pain victim and pain killer- are coated heavily with negativity and prejudice. Words that ought to be used are opioid, substance use disorder, pain relief seeker, person living with pain or pain medication.

For example, the word narcotic is a legal term used to denote illegal drugs such as heroin and cocaine. When used in that context, it is appropriate and clearly states what the issue is at hand. When used in the health care system, it unjustly labels individuals who need to take a class of medication called opioid analgesics. The term “addict” is derogatory in nature, whether referring to someone who is physically dependant on opioids treating their pain as well as when referring to someone with a substance use disorder. Addict inappropriately labels someone to a lower station in life as if they are of lower value as a human being.

Even the word DRUG can carry with it a negative overtone—drug as in street drugs? People with pain take medications not DRUGS. What about if you find yourself calling your Oxycodone “OXY’s”? Oxycodone is the medication and OXY, is a street name. How about choosing to call Percocet (oxycodone with Acetaminophen) “PERCS” which is again, a street name? How does that label you and others who use these medications for the legitimate treatment of pain? How do you feel when you hear those words used when directed at you?

How do the words “drug seeking” strike you? Wouldn’t you rather hear relief seeking? How about being called fat or obese compared with over-weight? How about crippled vs disabled, impaired or handicapped? I looked up the word disabled – other words with similar meanings are incapacitated, inactivated, deactivated, Immobilized, hindered, or rendered useless. Words are powerful in defining ourselves. Think about how you feel when you read “pain sufferer” vs. a person experiencing or living with pain? One is powerless and helpless while the other is empowering and uplifting. After all, not all people living with pain are pain sufferers especially when they have secured an effective pain treatment plan. Many thrive and show their strength in character every single day. What about the use of the word “victim” compared to survivor—one shows helplessness and the other strength? Which would you prefer?

Words are energy. A Japanese researcher, Dr. Masaru Emoto, who wrote Hidden Messages In Water, says that water and its crystals are influenced by the environment they are in. He has shown through his unique photographic process how certain words shaped beautifully awesome crystals while other words produced ugly and unorganized crystals. Words like “I love you. You are beautiful” form gorgeous well organized crystals while words like “I hate you. You are ugly” form distressing unorganized forms that make you turn away. We are composed of 70% water so what do words do to our water?

Another aspect to consider is that your brain believes what you tell it…and makes no distinction between whether it is a true statement or not. So what words do you use to talk to yourself and others?

Choose wisely. I choose love, gentleness, compassion and patience.

Posted in Communication

Tuesday, 22 July 2014

Has Compassion For Chronic Illness Disappeared? (Vid)

Today's excellent video from (see link below) is a must see for anybody living with chronic neuropathy and feeling somehow 'judged' for being ill. Similarly, friends and family will maybe recognise many of the things said here and be better able to understand and support their loved ones. As Ken McKim says; it's 32 minutes long but not as long as anything with a Kardashian in it but it's never boring, or too technical and is definitely worth a half hour of your time. An important video - don't miss it!

The Slow Death of Compassion for the Chronically Ill
Ken McKim
Published on 3 May 2014  
UPDATE: New video up on my channel, "Feel This Pain!" Check it out.________________________

This video, while 32 minutes long, is shorter than anything with a Kardashian in it, and much more important. Please make the time to watch.

A quick summary:

Society has been programmed to view the chronically ill as lazy and not trying hard enough due to an overwhelming amount of inspiring Internet stories highlighting incredible things a minority of people suffering from disease and/or disability have accomplished.

The media has a bias against pain medications, referring to them as "drugs" instead of "medications" more often than not in a concerted effort to stigmatize the medications and by extension those who take them.

Alcohol killed more people in 10 years than died of opioid overdoses: 139,093 deaths attributed to alcohol from 2000-2010 The CDC number puts opioid overdoses at 125,000 deaths over 10 years

The DEA now wants to reclassify any medications containing hydrocodone as Schedule II instead of Schedule III. This will make them harder to prescribe and harder to obtain; in many cases doctors will require more in-person visits before re-prescribing which costs more money for those patients (not everyone has insurance).

The public is okay with this because they now view the chronically is as people who are somehow lacking in motivation and who abuse drugs.The have this viewpoint because they don't truly understand the kind of pain these people are in, every hour of every day.

Making it harder for those with chronic illnesses such as Crohn's and Fibromyalgia to obtain the pain medications they need to sustain any kind of tolerable quality of life does nothing to prevent accidental overdoses. It increases costs for the sickest of our citizens, and has the effect of stigmatizing both the medications and those who take them. Oxycontin has always been Schedule II and it has done nothing to prevent its abuse. People who want to misuse drugs will do so no matter what their classification; punishing the millions who take these meds responsibly, and who need them to live is abuse. The chronically ill have already been betrayed by their own bodies, and now their government as well. The DEA must not reclassify these medications as Schedule II.

Of course people always want to know "where do you get your stats?" so here are some of the pages I looked at when putting this together:

Monday, 21 July 2014

Neuropathy And High Blood Pressure

Today's post from (see link below) looks at neuropathy and sleep apnea leading to higher blood pressure for patients at night. Many people with neuropathy suffer from disturbed sleep patterns brought on by sleep apnea (stopping breathing a number of times during the night). According to this short but complex article, this can also cause concurrent high blood pressure. Neuropathy is seen as a stress factor which inhibits the body's normal lowering of blood pressure during sleep. The consequences if true are obvious and the study suggests that more attention should be paid to the patient's cardiovascular system if they are living with severe neuropathy. Changes in blood pressure are a known factor in autonomic neuropathy (where it affects involuntary functions in the body) and maybe this should be taken more seriously than it currently is.

Painful Neuropathy and Increasing Blood Pressure 

This article originally posted 18 July, 2014 and appeared in Cardiovascular, Neuropathy, Issue 738

Painful diabetic neuropathy (PDPN) has been associated with higher nocturnal blood pressure in patients....

PDPN can cause obstructive sleep apnea (OSA) and affect one's quality of life. As this condition is often underdiagnosed, researchers conducted a study focusing on the increasing cardiovascular risk associated with neuropathic pain.

The study included a total of 113 diabetes patients with PDPN (n=34), painless diabetic polyneuropathy (n=33), and without diabetic polyneuropathy (n=46). Neuropathic pain, risk of obstructive sleep apnea, autonomic function, and blood pressure were all assessed in the study with the use of the Douleru Neuropathique en 4 Questions (DN4). Nocturnal systolic blood pressure was significantly higher in patients with PDPN (130.4 ± 15.6 mmHg) than those without diabetic polyneuropathy (119.9 ± 10.6 mmHg; P less than 0.0001) and those with painless polyneuropathy (124.2 ± 12.3 mmHg; P less than 0.05). The PDPN group also experienced less change in systolic and diastolic blood pressure overnight when compared to those without diabetic polyneuropathy (p less than 0.05). The "nondipping" decrease in blood pressure overnight was seen in 8 patients, which was highly correlated to PDPN status (p=0.007).

Researchers concluded that PDPN is associated with higher nocturnal blood pressure that is independent of pain-related sleep problems and other diabetes-related comorbidities. The theory is that neuropathic pain acts as a stressor, which induces sympathetic response during the night and inhibits the blood pressure from falling during the night. This highlights the importance of managing the patient's cardiovascular risk more closely while attempting to treat the neuropathic pain at the same time.

Practice Pearls:
  • The theory is that neuropathic pain acts as a stressor, which induces sympathetic response during the night and inhibits the blood pressure from falling during the night.
  • Nocturnal systolic blood pressure was significantly higher in patients with PDPN than those without diabetic polyneuropathy and those with painless polyneuropathy.
  • The PDPN group also experience less dip in systolic and diastolic blood pressure overnight when compared to those without diabetic polyneuropathy.
D'Amato C, Morganti R, Di Gennaro F, et al. A Novel Association Between Nondipping and Painful Diabetic Polyneuropathy. Diabetes Care. 2014 July 10.

Sunday, 20 July 2014

Nerve Assessments In The Eye For HIV Neuropathy

Today's post from (see link below) looks at a new way of assessing small fibre neuropathy by looking at the density of nerve fibres in the cornea. The cornea is one of the most densely packed areas of nerves in the body and it is relatively easy to see whether that nerve density has been diminished through nerve damage. If your doctor is conscientious enough and funds are available, you may undergo a skin biopsy to establish small fibre neuropathy or not. However, it's often inconclusive as well as being invasive and requires repeat surgery to make comparisons. Investigation of the cornea is non-invasive and by definition much cheaper and may well be the way forward in the future of neuropathy diagnosis. You can read about the relationship with HIV in the article, (which may require some concentration) but is definitely interesting and worth the effort.

New research sets stage for noninvasive monitoring of HIV-induced peripheral neuropathy
Posted By News On May 12, 2014 - 4:30am

Philadelphia, PA, May 12, 2014 – Corneal nerve fiber assessment has great potential as a tool to diagnose and monitor peripheral neuropathy induced by HIV, say scientists at the Johns Hopkins University School of Medicine. The results of their study are published in The American Journal of Pathology.

Although corneal nerve assessments have shown increasingly valuable as a replacement for epidermal nerve fiber evaluation in diabetic peripheral neuropathy, the evaluation of corneal alterations in tracking HIV-induced neuropathy has yet to be explored.

"The cornea is the most densely innervated tissue in the body, so corneal nerve assessment is extremely sensitive for detecting small sensory nerve fiber damage as compared to other tests including measurement of intra-epidermal nerve fibers in the skin," notes lead investigator Joseph L. Mankowski, DVM, PhD, who is Professor of Molecular and Comparative Pathobiology, Pathology, and Neurology at the Johns Hopkins University School of Medicine, Baltimore, MD.

Although not life threatening, HIV-induced peripheral neuropathy greatly compromises patient quality of life. Currently, skin biopsy is the accepted standard for measuring the loss of small, unmyelinated C fibers in the epidermis, one of the earliest detectable signs of peripheral nerve damage. However, skin biopsy is an invasive procedure, and ongoing assessment requires repeated surgical procedures. Electrophysiological testing to measure peripheral nerve conduction properties is not a viable alternative because current methods lack the sensitivity required to detect damage to small, unmyelinated fibers, especially in early stages of disease. Therefore, new sensitive, noninvasive methods of assessing small fiber nerve damage are urgently needed to detect and monitor peripheral neuropathy in HIV-infected individuals.

To study the pathogenesis of HIV-induced PNS disease, Jamie Dorsey, Research Technologist, and the research team led by Dr. Mankowski developed a simian immunodeficiency virus (SIV)-infected macaque model that closely reflects key peripheral nervous system (PNS) alterations seen in HIV patients with peripheral neuropathy. They sought to determine whether SIV infection leads to decreases in corneal nerve fiber density, and whether corneal nerve fiber density correlates with epidermal nerve fiber length counts, thereby setting the stage for follow-up investigation using corneal confocal microscopy.

"Moving to non-invasive and repeatable methods of nerve fiber measurements such as in vivo corneal confocal microscopy would enhance study of peripheral neuropathy by enabling early detection of damage, progression of nerve fiber deterioration, and enable assessment of therapeutic strategies in the SIV/macaque model," explains Dr. Mankowski. "Furthermore, adapting in vivo corneal confocal microscopy for use in tracking HIV-induced PNS damage in patients may be of great value to identify early PNS damage independent of performing skin biopsies."

To determine whether SIV infection leads to corneal nerve fiber loss, the researchers immunostained corneas for the nerve fiber marker βIII tubulin. They developed and applied both manual and automated methods developed by Jonathan Oakley, PhD, of Voxeleron to measure nerves in the corneal sub-basal plexus. These counting methods independently demonstrated significantly lower sub-basal corneal nerve fiber density among SIV-infected animals that rapidly progressed to AIDS as compared to slow progressors. Corneal nerve fiber density was also directly correlated with epidermal nerve fiber length.

Besides decreased corneal nerve fiber density, rapid SIV progressors had increased levels of SIV RNA, CD68-positive macrophages, and GFAP expression by glial satellite cells in the trigeminal ganglia, the location of the neuronal cell bodies of corneal sensory nerve fibers.

Together, these findings demonstrate that emerging noninvasive techniques to measure corneal nerve fiber alterations such as in vivo corneal confocal microscopy may be useful clinical tools to screen for and monitor progression of peripheral neuropathy in HIV-infected patients.
Source: Elsevier Health Sciences

Saturday, 19 July 2014

It's Like Lightning: Personal Neuropathy Blog

Today's post from (see link below) is another personal story of life with neuropathy and the various diagnoses that this person meets along the way. Personal stories like this are often the most powerful because they remind readers that they're not alone in how they're feeling, regarding the strange things that neuropathy does to their bodies. Well worth a read if you think you've got it bad!

It's Like my veins?? 

Posted by Davee Thursday, July 3, 2014

Good morning! I’ve decided to use my blog not only to writing, music, and other facets of entertainment, but, also to share my journey. I’ve had significant health problems for almost two years. Initially, my rheumatologist diagnosed me with systemic lupus (SLE) on June 19, 2013. However, the medication wasn’t working and I kept getting worse and developing new symptoms. So, after second, third, and fourth opinions, my neurologist believes instead I have fibromyalgia.

In April 2014 I visited yet another rheumatologist who I hoped would be familiar with autoimmune disorders. Following an extensive intake during my initial appointment, I found a place where the doctor also listened to me. Based upon my blood work, the doctor believes I have Sjogren’s Syndrome. She also made the diagnosis of Fibromyalgia and pre-lupus. I’ve never heard of pre-lupus, but, hopefully it stays in the “pre” category.

Thank you for listening, each week I will have a new installment chronicling my journey- Which is now more frustrating than ever. I test positive for ANAs in my blood, but, the lupus tests are negative. There are several varieties of autoimmune disorders, with different caveats and health variations. Learning to live with the unknown has become my routine.

It's like LIGHTNING struck my insides...


I also write fiction books and research at least one thing for each of my writings. I want to be as accurate as possible when talking about history, illnesses, dates, geographic areas....etc, but, you get my drift. In one of my recent books, I had characters who were struck by lightning. In order to get an accurate appraisal of the medical symptoms, longer term problems, or delayed symptoms, I turned to the internet.

Lightning is primarily an injury to the central nervous system, often with brain and nerve damage. Including muscle soreness, headaches, nausea, mild confusion, memory slowness, mental fog, and dizziness or balance problems.


How interesting that the majority of the issues occurring with me involve my nervous system. It seems that I was struck by lightning and didn't know it...NAH.

It did get me thinking about why this would happen . What is going on within my body that's causing my nerves to misfire? I had horrible neuropathy all over my body. At times it felt like pin pricks everywhere. My lips and tongue would also go numb. I would occasionally lose feeling in my shins and forearms as well. At one point, I thought I was having a stroke and my doctor even instructed me to go to the emergency room.
(Thankfully, I was not having a stroke.)

The longer term problems are even more ironically similar:
Problems processing new information
Difficulty accessing old information
Slower reaction time
Irritable and possible personality changes
Inattentiveness or forgetfulness
Headaches such as migraines
Chronic pain from nerve injury
Difficulty sleeping - either sleeping excessively or insomnia

I suffer from each item on the longer term problem list. This list further proved to me that maybe studies could combine persons with chronic nerve injuries for treatment possibilities. I'm not sure if this is already occurring, but, it might be a good start. I know I currently take 3 different meds to make life bearable, but, it would be great not to rely on such drugs.

Progressing further in research, I discovered the delayed symptom list:
Personality changes/isolation
Irritability and embarrassment at not remembering people, job responsibilities, and key information
Difficulty carrying on conversation
Chronic pain ad headaches


I can no longer multi-task and I still grieve over it. I used to be the go-to girl. I could juggle several projects at once and it was a piece of cake. Now, I can't even load a dishwasher and keep up with a conversation! no joke
I've experienced depression, anxiety, and anger at the limitations this auto-immune disease has forced upon my life. I'm not the same woman and it hurts, mentally.

I have to take meticulous notes and my co-workers know I will need everything in writing. Sometimes I miss critical elements of a project, and I've asked my team leader to review all my work.

It's debilitating and it worse than sucks.

Maybe I can make it more interesting and say I was struck by lightning.

It's worth a shot.

Friday, 18 July 2014

Tapentadol: A Promising Option For Neuropathic Pain?

Today's post from (see link below) talks about Tapentadol (Nucynta) being effective for diabetes-related neuropathy pain. Earlier articles on this blog (see alphabetical list to the right) have highlighted its efficacy for HIV-related neuropathy and other neuropathies also fall under its benefits (but as yet not approved for all). So why isn't this breakthrough semi-opiate ending up as a front-line treatment? The answer seems to lie in the cost and the competition between drug companies, which disregards the needs of the patients, who have been looking for an efficient chronic pain management drug with less side effects than its competitors. The FDA has approved it, which is the first major step with any new drug but doctors throughout the western world are possibly being pressured into not prescribing it due to pressure from health insurance companies who are always looking for cheaper options. It's symptomatic of a culture where economic cuts are paramount and the power of established drug company lobbies has never been stronger. However, if Tapentadol really is a better option for many patients living with neuropathy, then denying them access is at the very least, morally questionable.

Tapentadol-ER for the Treatment of Diabetes Associated Peripheral Neuropathy (DPN)

 This article originally posted 26 June, 2014 and appeared in Medication, Neuropathy, Pain, Issue 735

The FDA recently approved tapentadol-ER (Nucynta ER) for the treatment of peripheral neuropathy in diabetics.... 

With an increasing number of patients being diagnosed with diabetes, complications associated with the disease are becoming more common. 

Diabetic peripheral neuropathy (DPN) is the most common neuropathic pain seen in patients with diabetes, affecting up to 50% of patients. It is characterized as a burning or tingling sensation that affects the lower limbs. Painful DPN is associated with increased morbidity, mortality and substantial costs to the healthcare system.

Tapentadol-ER is a schedule II opioid analgesic that is commonly used for the treatment of moderate to severe chronic pain. It is unique in its dual acting mechanism in which it works as both a weak mu-opioid receptor agonist and a norepinephrine-reuptake inhibitor.

This distinct mechanism is said to represent a new pharmacological class of medications: MOR-NRI. Tapentadol ER is a weak mu-opioid receptor agonist, having an 18 fold decrease in receptor affinity when compared to morphine. Despite this decrease in affinity, tapentadol ER only produces a two to three fold decrease in analgesic effects when studied in animals. It also acts on the alpha-2 receptor located on the noxious nerve fibers in the spinal cord and throughout the central nervous system, inhibiting transmission of pain impulses. The dual acting mechanism of tapentadol-ER works to control the complex pathology that is neuropathic pain.

Until this approval the only options for FDA approved medications to treat diabetic peripheral neuropathy (DPN) were pregabalin and duloxetine.
Tapentadol ER provides sufficient pain relief with convenient twice daily dosing. The starting dose for opioid-naïve patients is 50mg twice daily and can be titrated to an effective dose of 100mg to 250mg twice daily. The maximum daily dose is 250mg twice daily. The maximum serum concentration is increased by 17% when taken in combination with a high fat breakfast but according to package labeling may be administered without regards to food.

Tapentadol-ER undergoes extensive hepatic metabolism and therefore must be adjusted in patients with hepatic impairment. The usual adjustment is extension of administration duration to once every 24 hours in patients with mild to moderate hepatic impairment (Child-Pugh Score of 7 to 9). It is contraindicated in patients with severe hepatic impairment. Tapentadol ER and its metabolites are excreted by the kidneys; however, no dose adjustments are required for patients with renal impairment.

The efficacy and safety of tapentadol ER has been studied in two DPN studies. Both studies were placebo controlled randomized studies in which patients had either type 1 or type 2 diabetes and painful DPN for a period of at least six months. Pain intensity was at least a 5 on an 11 point scale. In both studies approximately two-thirds of participants were opioid-naïve. Both studies came to the conclusion that tapentadol ER provided statistically significant improvement of pain intensity as compared to that of placebo.

Treatment related adverse effects were reported at 70.9% for those taking tapentadol ER and 51.8% for those taking placebo. The most common side effects were nausea, diarrhea, anxiety and dizziness. Most were mild to moderate and did not lead to discontinuation of the medication.
Practice Pearls:
  • The incidence of diabetes is on the rise as is the complication of neuropathy
  • The FDA approval of tapentadol ER for treatment of diabetic peripheral neuropathy provides practitioners an additional therapeutic option with a convenient twice daily dosing regimen.
  • Tapentadol ER has a unique dual mechanism suggesting that it may be particularly useful for complex pain associated with DPN.
Consult Pharm. 2013 Oct;28(10):672-5. doi: 10.4140/TCP.n.2013.672
Comment from Dr. Aaron Vinik, Diabetes In Control Advisory Board member, and study author:

Pain associated with DPN is a common complication of diabetes affecting 10-26% of patients for which the only approved drugs are Pregabalin and Duloxetine which at best reduce pain by 30% in about half the patients treated leaving a significant portion of the affected population without resort to significant pain relief. A paradox here is that third party payors insist that less expensive drugs such as the tricyclics should be used as first line despite the fact that they have cardiovascular cautions as well as not paying heed to the need for attention to the comorbidities of sleep loss, depression and anxiety as determinants of choice of initial therapies. However, the problem occurs when first line therapies have failed and the need for greater reduction of pain arises wherein a combination of drugs acting through different parts of the pain pathway have proved useful. The problem with this approach is that this increases the burden of drugs patients with diabetes must take with untoward effects, particularly cognitive impairment, which can have a disastrous impact on decision-making and is the cause of induced hypoglycemia and its consequences. Tapendalol represents a proposed new class of centrally acting analgesics combining two mechanisms of action: u-opiod receptor agonism and norepinephrine reuptake inhibition. Tapentadol extended release has been shown to be effective and well-tolerated for the management of severe, chronic pain in randomized, double blind, placebo-controlled and/or active comparator controlled phase 3 studies. Two studies (of which I am first author and coauthor of the second) were designed using a randomized withdrawal multicenter placebo control design and reached their primary endpoint and in August 2012 tapentadol ER received FDA approval for the mangement of pain associated with DPN in adults when a continuous around the clock opiod analgesic was needed for an extended period of time. To determine if subgroups would benefit, the data from the two studies were pooled. The results of this pooled analysis indicated that tapentadol ER is effective and well-tolerated for the management of neuropathic pain associated with DPN, improved measures of health related quality of life and had constent efficacy across different patient subgroups divided by age, gender, race, opiod experience and pain intensity. Sensitivity analyses showed that in the tepentadol arm ( n=360) compared with placebo ( n=342) the drug 54% achieved >30% pain reduction and 38.9%;50% pain reduction and the significant side effects were nausea, vomiting, dizziness, and somnolence. These figures argue in favor of using the drug as a second or third tier drug with the recognition that its effects are modest and there is the potential for habituation. Ultimately, what is needed is a drug that will reverse the pathogenic process and that the need for pain-relieving drugs will be the bandaid to be used in the interval before the damage to the nervous system has been resolved.

Thursday, 17 July 2014

Australian Breakthrough In How Pain Drugs Work

Today's post from (see link below) shows how modern research is getting closer to developing new and better-targeted chronic pain relief drugs to help manage conditions like neuropathy. This latest research has identified how pain drugs attach themselves to the proteins of nerve cells, thus preventing pain signals from being transmitted. The potential here is to be able to redesign drugs, so that they target the right cells in the right manner and just do the job they're meant to do without producing side effects. It's very detailed knowledge and way beyond most our our comprehension but this short article is not difficult to follow and shows how research into managing the side effects of our chronic pain condition is progressing. As so often with these things, there's nothing ready for the chemist's shelves yet but we have to be pleased with any progress - maybe the next generations will benefit from work being done today.

ANU scientists' new insight into pain medication 
Natasha Boddy, Health Reporter Date July 3, 2014

Scientists in Canberra have made a major breakthrough in unlocking the secrets of how pain relief drugs work, in a discovery that could lead to powerful new treatments for conditions such as chronic pain and epilepsy.

The Australian National University's Dr Ben Corry and Lewis Martin have discovered how drug molecules attach to proteins of the nerve cell, creating the potential to redesign drugs that do not have the side effects associated with current treatments.

Dr Corry said better understanding of how exactly pain relief drugs worked opened the possibility of discovering how pain relief could be better targeted to certain parts of the body.

"The two main things we've discovered is that although we knew which proteins local anaesthetics interacted with, what we've found is exactly how and where they bind to these proteins in the body," he said.

"That's the kind of detailed knowledge we need to be able to come up with the next generation of anaesthetic drugs."

Dr Corry said the discovery created the potential to develop drugs designed to selectively target the subtly-different proteins in specific locations, such as the heart or brain.

"One of the big ones is treatment for chronic pain, which is the biggest health cost to our economy. For people with ongoing chronic pain problems, there's no effective treatment and we really just try to manage it," he said.

He added there was also potential to design drugs to better treat conditions such as epilepsy and cardiac arrhythmia without side effects.

Using a supercomputer to simulate the drug's route into the nerve cell, the pair revealed for the first time how benzocaine, a local anaesthetic, and phenytoin, an anti-epilepsy drug, enter into nerve cells and prevent pain signals from being transmitted to the brain.

Pain signals are transmitted to the brain when proteins that act as tiny gateways in nerve cell walls open, allowing sodium and potassium ions to pass through. The simulation shows that the drug’s final binding site is inside the sodium gateway protein, which blocks it and prevents the signal from being transmitted.

Drugs that block sodium channels are also used to treat nerve-signal disorders such as epilepsy or heart arrhythmia. However, current drugs target sodium channels indiscriminately throughout the body, which can lead to side effects.

Dr Corry said the time spent on the National Computational Infrastructure's supercomputer conducting the research was equivalent to running a home computer for about 340 years.

The work from ANU's Research School of Biology has been published in the latest edition of PLOS Computational Biology.

"It's pretty exciting. We haven't come out with a new range of drugs or anything like that yet, but it's the first important step in trying to work out now how we can use this in designing new drugs," Dr Corry said.

Wednesday, 16 July 2014

Neuropathy: A Beginner's Guide

Today's post from (see link below) is short, simple and to the point; exactly what you want when meeting the term neuropathy for the first time. It will give you a good idea of what you're dealing with and what you might come to expect. Absolutely a worthwhile start to your neuropathy journey.

What is Neuropathy?
January 20, 2014 (no author mentioned)

The term “neuropathy” is a fairly broad one: it refers to nerve damage, typically to the peripheral nerves, which are those that connect the central nervous system (the brain and spinal cord), including sensory, motor, and autonomic (“automatic”) nerves, to the rest of the body. Damage to the nerves essentially corrupts communication between the nerves and the body, causing loss of sensation, pain, and weakness. Neuropathy can have any number of underlying causes, including overuse injury, traumatic injury, infection, diabetes, alcoholism, exposure to toxins, and certain diseases.

Mononeuropathy refers to a single nerve that has been damaged, usually by an injury or repetitive use or pressure. For example, the pain and numbness of carpal tunnel syndrome is caused by compression on the nerve that runs through the wrist, and the tingling, burning, and numbness can even extend into the arm and shoulder. Eventually, the repeated stress on this nerve can cause the muscles of the hand to weaken. Being excessively sedentary can also compress nerves to the point of damage.

Polyneuropathy is more common, and it affects multiple nerves in different places throughout the body simultaneously. It can have many causes, including poor nutrition (especially a vitamin-B deficiency), diabetes, shingles, certain medications, and complications from cancer and kidney disorders. It can also be a sign of certain rare diseases, such as Guillain-Barre disease, which causes the immune system to attack its own nerves.

Depending on the types of nerves that are damaged, neuropathy symptoms can vary, but the most common are tingling, loss of sensation, and burning pain, especially in the hands and feet. Those three accompany sensory nerve damage, whose other symptoms include sensitivity to touch, jabbing pain, and lack of coordination. Autonomic nerves (those that relate to involuntary actions, such as breathing and digestion) can result in extreme sensitivity to heat, dizziness from changes in blood pressure, and difficulties with digestion and bladder function. Neuropathy in motor nerves typically causes muscle weakness and paralysis.

The symptoms of neuropathy can be unpleasant, inconvenient, and painful in and of themselves, but they come with additional complications: people may burn themselves or injure their joints because they have less sensation, fall as a result of muscle weakness and lack of coordination, have ongoing digestive issues, etc. Neuropathy may also be a sign of a more serious condition, so if you experience symptoms, see your doctor.


Tuesday, 15 July 2014

Neuropathy Diagnoses: A Personal Story

Today's post from (see link below) is another chapter in the long-running neuropathy story of one patient in Colorado. It centers on the difficulties people have getting the right diagnosis and how meeting the right specialist at the right time can make things so much simpler. Knowing what you have and where you stand is an enormous relief for most neuropathy patients, even if the treatment outlook is not so optimistic or clear-cut. This person's experiences will resonate with many readers.

Living With Neuropathy The Journey of one PNer
P L Gerrard July 2013

Snow in Nashville

One of the things I learned after moving from Colorado to the South is that when it snows no one goes anywhere. Most times it’s a blessing, as I have the road to myself. Other times it can be infuriating. I have driven from Knoxville to Columbia in near white-out conditions only to arrive in Columbia and find everything shut down (even McDonalds) and my meeting cancelled. Here I had just made a 240 mile drive through snow, but the other meeting participants wouldn’t even drive 10 miles across town.

Snow in Nashville the morning of my Rheumatologist appointment was one of the times snow was a blessing. My appointment was at 8:00 and I was told to arrive at 7:30 to complete paperwork. While I completed the paperwork, sitting near the reception desk, I heard call after call come in to cancel appointments – no one goes anywhere when it snows.

Because there were so many cancellations, the Rheumatologist was able to spend a great deal of time with me. She was a fellow, and after her initial examination, she came back with the supervising rheumatologist. They went briefly over my examination, and then asked if anyone had ever suggested MS to me. They definitely believed my description of the pain was neurology-related, and I had made the comment that some mornings just taking a hot shower was exhausting. Evidently that can be one of the symptoms of MS.

With that question hanging over me, I was not going to wait another 3-4 months to get in with yet another neurologist. Instead I asked if the snow may have had the same affect on appointments in the Neurology Clinic at Vanderbilt, and if it would be possible for me to see a neurologist that day. They made a phone call to the Neurology Clinic, and Yes!! There was an appointment open in two hours.

Now, Vanderbilt is a huge complex, and just getting from the parking garage to the clinic itself is difficult for someone with constant pain in their feet. So, rather than attempt the walk back to the garage, only to return again, I settled in their food court with a magazine and waited for my appointment time.
My appointment was with Dr. G, head of the Neurology Clinic, and a truly caring and compassionate soul. After a thorough examination, he announced that I did not have MS, but was suffering from Small Fiber Neuropathy (SFN). My initial reaction was a huge let-down. After all, one neurologist had already conducted an NCS that was negative for neuropathy. But then Dr. G explained that an NCS is only truly effective for diagnosing LARGE fiber Neuropathy, and negative results are common with the Small Fiber variety. What I did have that is consistent with Small Fiber Neuropathy was an absence of reflexes in my ankles.

There are tests, like a painful skin biopsy, that can confirm small fiber neuropathy, but he felt confident in the diagnosis, based on the physical exam he performed, and believed it best to start me on medication and see if there was an improvement in my pain level. He spent about 20 minutes explaining neuropathy in general and small fiber specifically, and gave me several handouts that I could read later. Some of the things I remember he told me are:

- Neuropathy is not in and of itself a disease. It is a condition that is brought on by an underlying cause. The most common underlying cause of neuropathy is diabetes.

- The most common underlying causes of SFN are auto-immune diseases and cancer. But, in most cases it is idiopathic (no known cause).

- Neuropathy cannot be treated. It can be improved if the underlying cause is found and treated.

- Neuropathy can be progressive and irreversible. It will progress until the underlying cause is found and treated. Depending on the underlying cause the damage may be irreversible.

- Neuropathic pain cannot be treated with typical pain medications, because the pain is ghost or phantom pain. Think of people who continue to feel pain in a limb after it has been amputated. So, the object is to trick the brain into not “feeling” pain from the damaged/missing nerves.

- The most commonly-used medications for tricking the brain are anticonvulsants.

I had already been tested for diabetes, and some auto-immune diseases, but he ran more blood tests, and gave me a list of cancer screenings I should take to Doctor K to have scheduled. He also advised me to have the screenings repeated every 2 years, because it is not uncommon for either an auto-immune disease or cancer to present itself within 2 to 3 years of an SFN diagnosis.

All tests came back negative, so my SFN was now considered idiopathic. The only thing certain was that until an underlying cause was found it would be progressive, and most likely irreversible.

Monday, 14 July 2014

Fabry Disease And Neuropathy: A Personal Story

Today's post via (see link below) talks about life with Fabry's disease and neuropathy. Fabry's disease is a very rare genetic lysosomal storage disease, so most people don't have to worry about it but some of the symptoms may be familiar for people living with neuropathy. Lysosomes are sacs of enzymes within cells that digest large molecules and pass the fragments on to other parts of the cell for recycling. This process requires several critical enzymes.  If they are missing or defective, life can become very difficult. Fortunately, in this man's case, he was able to get a medication that replaced the necessary enzymes and restore some balance in his life. Not an every-day neuropathy story but one which shows how diverse and difficult to pin down, these symptoms are.

From Growing Pains, to Neuropathy, to Fabry Disease
By David Gibson 2014

"Pain was the simple explanation, but pain without an underlying cause is hard to understand. I was experiencing pain in my hands … just gripping the handle bars on my bike was hard."

My childhood was typical: I had friends in school, I played some sports, I rode my bike a lot… Then, one spring day my bike rides started to change.

I tried telling my mom what was happening, but it was hard to explain.

Pain was the simple explanation, but pain without an underlying cause is hard to understand. I was experiencing pain in my hands…just gripping the handle bars on my bike was hard. I didn’t used to have this pain, and I didn’t do anything different…so why did it hurt so much? I found myself taking breaks from riding because of the pain; I would stop at the side of the road on my way to my friend’s house and rest – that would help the pain go away. And then I would make my way to my friend’s house, we would have a good time, and I would make it back home. Maybe it was a little late getting home, but that was how I coped with the pain.

When the pain started in my feet, things got tougher. I couldn’t always just stop and sit to rest. The summer heat would make it worse, or make it happen more often anyway. I tried to keep going, but often couldn’t. My mom took me to the doctor and after taking x-rays of my feet he explained the cause of my pain: “growing pains.” The doctor explained it all on the x-rays and pointed out the source of the pain -- my joints. It was just one of those things kids go through and some had it worse than others. The good news: it would go away in time.

Ok, growing pains, it would stop—that’s what I held on to. Complaining didn’t help, so I stopped talking about the pain, and when the pain came back I would go home and rest. After all it would go away in a couple hours and I could return to what I was doing, as good as new again. “Lots of kids must go through this; if they could deal with it so could I,” I told myself.

When I was 12, we moved to Marion, OH. My new school was okay. I made new friends and I started playing on the basketball team. I had learned to deal with the pain, and when it was too much to bear resting did the trick; but my stomach issues were another matter. My stomach started to hurt as well, and I couldn’t hide the number of bathroom breaks I needed: the diarrhea was terrible forcing a dash to the toilet 10, 15, 20 times a day. Thankfully these stomach issues didn’t happen a lot, but when it did it would last weeks.

My mom was concerned about these stomach symptoms, and so was the school nurse. So we went back to the doctor. Crohn’s disease was suspected and ruled out. This time, no explanation was found. Again, not complaining was the best answer for me. The ‘just grin and bear’ approach kept me out of the of the doctor’s office and free from undergoing more tests. Do your chores and be stoic--that was the way things were handled in my family.

One of the chores I hated the most was mowing the lawn. We lived in the country and had a big yard. The vibration from the mower would drive my hands crazy. For some reason, it would make my hands ache and the pain would go up to my forearms. I would go in the house, get a cool glass of water, and rest until the pain stopped. Unfortunately, my hands would keep feeling like they were vibrating. We also had a wood stove and I had to help my dad carry the wood and split it into smaller pieces to fit in the stove. Dad would often find me sitting on the wood pile, but he didn’t understand why I needed to rest. After all, I didn’t get hurt and I hadn’t broken anything.

I was 14-years-old when I learned that there was this health problem some of my family suffered from. My grandpa and his brothers had it, and some cousins had it as well. My grandpa died at 53 from it. My cousins with the condition -- called Fabry disease -- would get really sick and sometimes need to go to the hospital. They would suffer terrible pain and be unable to do anything for days.

I wondered if I had Fabry disease. I knew my family members who had Fabry had to be very careful about going out in hot weather because they perspired very little (hypohidrosis) or not at all (anhidrosis) and it put them at risk of overheating, getting sick, and triggering terrible episodes of pain.

The not sweating part didn’t sound so bad to me; I didn’t like getting all sweaty, but getting sick and being in pain sounded much worse. Also, they couldn’t play sports and exert themselves because it triggered episodes of pain – they had a note from the doctor that gave them permission to sit out Phys Ed class. I was told they would suffer like grandpa and his brothers did when they were younger. They would have terrible episodes of pain in their hands, feet, and sometimes all over.

I could deal with my pain. And I could play sports, and I was strong and could lift weights better than the football players at school. The football guys didn’t like that, but I didn’t care. I had a girlfriend. I was like the other kids. I didn’t have Fabry disease; my mom would have known if I did.

After graduating from high school and getting a job, my girlfriend and I got married. We had a wonderful little baby girl and were a family now. Unfortunately, my health issues persisted. Those “growing pains” I was supposed to grow out of were still with me, and they seemed to be worsening. I needed to take breaks at work sometimes because my hands and/or feet would hurt. I was still having episodes of stomach pain and frequent diarrhea. Sometimes, when it was really bad I would lose weight and my appetite would be off for a while. The cramping and stomach pain could get pretty extreme and they brought on bouts of vomiting too. After these spells of gastrointestinal problems passed, I was always able to gain the weight back. Even with these problems, I thought I got by pretty well most of the time.

I didn’t complain or say much to my family, but my mom knew I was still struggling; she started to question if I too had Fabry disease. One of my cousins was going to a hospital in New York City to be evaluated by health experts who were going to run a full battery of tests. He told the doctors about me and my symptoms, but they told him I didn’t have Fabry disease because I could sweat normally. After repeated visits and meeting another patient who was able to sweat and whose symptoms were similar to what I was experiencing, we decided that I get tested for Fabry disease. A blood sample was drawn at a local doctor’s office and was sent off to New York. Finally, after three weeks, the results came back: I was positive for Fabry disease. My mom cried and said, “Well, now we know.”

I was 22 when the health experts thought someone who sweats normally couldn’t have Fabry disease, and five years later I learned different. Even though my symptoms were somewhat different from what my relatives with Fabry experienced, we all had Fabry disease and the exact same mutation. I learned that Fabry disease is an inherited lysosomal storage disease that results when the body can’t produce enough of a certain enzyme called Alpha-Galactosidase A (or alpha-Gal). Lots of different mutations on the gene for this enzyme have been found and they usually run in the family. I also learned that my symptoms weren’t really all that different from my relatives’; my symptoms weren’t quite as obvious, and I didn’t help matters by not speaking out about my suffering. It turns out the pain in my hands and feet were caused by neuropathy; even my episodes of stomach pain and diarrhea were the result of my nervous system being impacted by Fabry disease.

Normally, the enzyme alpha-Gal is supposed to break down a fatty lipid named globotriaosylceramide (or Gb3). In people with Fabry disease (whose bodies do not produce alpha-Gal), Gb3 builds up in tissues such as the lining of blood vessel walls, the heart, the kidneys, and the brain. The build-up can make the blood vessels narrower, making it difficult for blood to pass through. And, over time, it can cause life-threatening complications such as kidney failure, heart attack, and stroke. It was the kidney failure that my grandfather and most of his brothers succumbed to. Fabry is on the X chromosome and it was thought that women would not suffer from the disease, but now we know women can suffer as well – sometimes, just as severely as the men. My mother and all her sisters have the disease and they suffer heart and other problems.

Fortunately, when I was learning about Fabry disease, there was a clinical research study evaluating a possible treatment -- called Fabrazyme -- that replaces the missing enzyme in patients with this progressive disease, and I was able to get in to the study. There were lots of trips to the hospital in New York and lots of tests.

It was five years before the FDA finally approved the treatment. I was fortunate to be receiving this treatment during most of that time. Fabrazyme is basically the same enzyme, alpha-Gal, that my body is not able to produce. I receive the treatment via an intravenous infusion over a span of two hours every two weeks...and it helps tremendously.

I still have health issues, but my organ functions have stabilized because of the treatment. And, unlike many of my family members who suffered terribly and died from complications like kidney failure, I have a fighting chance. Researchers and drug companies continue to work on developing new treatments, and gene therapy is being investigated as well. Perhaps a cure will be possible soon. The future is full of hope, so maybe this disease can be beaten before too long!