Lidocaïne perfusions

General
1

Hello everyone

I had an appointment with the neurologist last Wednesday of the Centre Anti-pain Nice (France): he offered me infusions of an anesthetic called Lidocaine. These infusions will be made in sets of three days a week for several weeks. I had already had there ten years and benefit lasted about three months.

But what was most important to me: he said that the disease was genetic, we know the gene and a drug was developed and now we were waiting the authorization to be placed on the market.

It would be wonderful: over burns and live like everyone ....

2

Hi Melody,

I think the new drug is from XENON. I've heard about this. But is this only for genetics EM ? Mine is secondary, I wonder if this new drug can be effective on it.

Carine

3

Mexiletine (the drug i'm on) is the oral analog of Lidocaine. Both Lidocaine and Mexiletine work by blocking sodium channels. The new drugs in development are also sodium channel blockers. Funapide (under development by a partnership of Teva and Xenon Pharmaceuticals), Raxatrigine (under development by Convergence Pharmaceuticals), Ralfinamide (under development by Newron Pharmaceuticals), and PF-05089771 (under development by Pfizer) are all selective Nav1.7 sodium channel blockers.

My doctor states unequivocally -- Erythromelalgia is caused by defects in sodium channels.

However, some people here have not seen improvement after taking Mexiletine or with Lidocaine infusions. I don't know if that means they are also unlikely to see improvement taking a more selective sodium channel blocker.

Mexiletine works well for me, though is not perfect. I've not had a Lidocaine infusion, but there is support for them being used in conjunction. http://archderm.jamanetwork.com/article.aspx?articleid=478089

Perhaps the Nav1.7 sodium channel blockers will better target the pain pathway associated with EM.

4

Thanks Carter !

5

I’m currently waiting for a pain clinic appointment for lidocaine infusions. I’ve had them before I from what I remember they do help. Well they did me.

6

Good information. Based on what I've read and heard, I disagree a little bit with one comment made by your doctor. Those who have genetic based EM have primary EM, but those who seem to have primary EM are not all suffering from EM from a genetic defect. It seems only a small subset of primary EM suffering patients have the genetic version of it. But it could be that medical terms defining primary EM have changed and now only refer to genetic based EM. This would leave those who previously had spontaneous EM with no known cause, in a undefined category perhaps. Should we call it spontaneous EM now?

BY THE OLDER DEFINITION

Primary EM is EM with no known source.

A secondary EM is one that is caused by another disease and when you treat the other disease, and resolve the other disease the cause of EM, and the EM symptoms are relieved.

If I have EM from cancer for example. The EM might be relieved when I cured of cancer, if that happens. If I have EM from cancer treatment, and that cancer treatment damaged my nerves, I might have EM from nerve damage and it might be a kind of permanent EM, with a known cause. Still perhaps referred to by some as primary EM but we know the cause. If I have EM from a drug reaction to psychotropic drugs for depression. Perhaps that was damage from those drugs. I don't have a classic source of my EM, being cancer, lupus, diabetes EM flares. So I am labelled with primary EM. As the cause becomes more known, I may know the cause, but many still may refer to this as primary EM because the damage isn't secondary from another treatable disease. It's my primary problem and I'm treating it alone.

All EM symptoms without a known source are considered primary EM.

At least that is the case in the old days.

Statements of Primary EM being defined as the genetic defect are now being made as patients with primary EM have tested positive for the genetic defect.

Think of primary EM in the traditional sense and EM with a mysterious cause. It's not caused by something that can be treated or found. Perhaps it's due to a nerve damage condition or autoimmune disease, but they just can't figure it out for your EM, so it's called primary EM, or an EM that seems to just appear.

Genetic based EM (my term) or primary EM from a genetic defect is a flaw in the sodium NAV 1.7 channel defect. This is a primary EM that develops from the genetic defect and the patient is tested positive for the defect. There are many who have the Nav 1.7 gene defect and may not get EM at all. It affected something like 20% of those who have the genetic defect.

Pain travels down nerve channels. Think of the sodium nerve channels like EQ bands of sound in your stereo system. Pain in your nerves can travel down different channels like different bands of EQ from my sound sound system. With a narrow band EQ you can perhaps turn down one band of sound in your stereo. A broad band sodium channel blocker is turning down all the EQ channels and it will reduce the volume on all channels, including the NAV 1.7 specific EQ band we would want to target. If I turned down all or many of the EQ's with a broad band EQ, then a wider range of sound would be reduced. With a broad band channel blocker other pain signals besides Nav 1.7 are turned down as well.

If one takes a broad band sodium channel blocker like oral lidocaine or uses lidocaine, it's turning down all the channels of pain. Which can help for EM symptoms if you have the Nav 1.7 channel defect. If you have the nav 1.7 sodium channel defect and have EM pain and you can turn down just the noise from that channel, then you are targeting the pain from EM more specifically. This is what they are working on a narrow target of the Nav 1.7 pain channel.

About two years ago I heard that there were only 30 known family groups in the world that had the genetic defect. So there were apparently many people who have primary EM or something like that, who didn't have the Nav 1.7 defect. Those people may not be helped by the sodium channel blocker approach, but some may still be helped. So there are those who seem to have primary EM, but can't be linked to the genetic defect. They may have some other mystery illness or perhaps just some kind of strange nerve damage which is unknown.

We can likely state that if you have the Nav 1.7 genetic defect and EM, then it's definately primary EM that you have. But I don't think we can say every person that has Primary EM, has the genetic defect.

In either case, with the genetic defect or without, a sodium channel blocker may help the EM sufferer, so it's worth a try.

7

Watchman,

I am someone who has had exceptional success with a sodium channel blocker. It doesn't seem to function purely as an analgesic. It actually stops the flares from occurring. Perhaps the pain and the flare are inexplicably linked and if you stop the pain, you stop the flare? The skin is still often discolored if i'm in a dependent position (such as standing), but there is no heat. Without the heat, there is no flare.

8

Good information Carter. Thanks for the info. The article in the link was really interesting. It looks like lidocaine and Mexelitine can help quite a bit for some patients. I'm hoping that something like that will help my mom. It sounds like the person in the article had the genetic version of it.

From a perspective of being able to reverse the treatment in case of bad side effects, the lidocaine and pill sounds a lot better. I found it interesting in the article that the Oral lidocaine medication helped when it was starting to get at a higher dosage of 200mg. Some neuropathic pain treatments with that are supposed to be effective with a smaller dose according to my doctor. This shows that some of the research that doctors find may not tell the entire story. Imagine getting a small dose of Mexiletine and having it not work, when a higher dosage works. The higher doses can affect heart rythms.

I also find it interesting but this is off topic that one report showed a person developing EM and only having a history of treatment for depression and anxiety. But the article didn't mention what that treatment in the past consisted of. I wonder if some drugs that affect the brain can nuke the perephrial nerves as well. Some patients have luck with drugs like Cymbalta which affect the brain, but this doesn't really help my mom much.

Regarding water and skin issues. One can put a little bit of hydrogen peroxide in the water to held deal with the effects of possible skin conditions. And some can put epson salts in the water as well. These seem to help some, but we have only used one or the other not both in the cold water bath. It's probably safer but a slower chilling method to use cold air and fans.

9

Watchman,

Yea, Mexiletine is what i'm on. In my case, it began working almost instantaneously. The last full flare I had was about 4 hours after I took the first dose on August 1st. I was only taking a single 150mg capsule a day in the first week, so I obviously didn't need a large dose for it to make an impact. I take 150mg 3 times a day now, but even at just once a day it turned it off. I'm not sure I saw a whole lot of benefit from increasing to 3 times a day, but the higher dose might help during exercise. That's hard to say because I wasn't exercising when I was taking just a single dose. The only side effect I get is heartburn if I take it on an empty stomach. Otherwise I can pop the pills without any problem. I am relatively young, so there was no concern about my heart. Actually, it wasn't even discussed. I had a recent EKG and echocardiogram, results they had, so I think my doctor knew my heart was fine. In fact, when I had the echocardiogram performed back in 2013, during the procedure the tech asked if I was a professional athlete because my heart was so ridiculously efficient, haha. So, it's probably more than just fine.

10

The doctor I'm seeing now is actually really good. He had 5 EM patients before me. Considering most doctor's have never heard of the condition, that's a lot. I had to fight really hard to find him. After going to a series of doctors who didn't know what the condition was, with one particularly bad appointment in July, I was beginning to feel hopeless. So I told my insurance company if they didn't find me a doctor who was experienced treating EM, I would have my friend who is a news anchor put me on television. We'd turn it into a story about what a disaster it is to have their insurance when you have a rare condition. My best contact sent that message up the food chain and after that everything changed. I ended up with the HMO director's own office number and can call him anytime I have concerns about my care. It was very helpful to be able to just go straight to the top whenever I wanted. They were the one's who found my doctor.

My story shows how important it is to advocate for yourself. I leveraged what influence I had to get the help I needed. I hadn't even asked my friend about doing the story for the news. I don't know for certain they'd have aired it. Name dropping and making the threat was enough though.

11

Great info. Glad to hear about your success. I'm hoping that something like Mexiletine or some variation of a tibial nerve block will help my mom and give her more mobility and ability to function more normally. In our case, I'm just one of the caregiver/helpers for my mom; in a functional, but not necessarily legal sense. She still has autonomy of decisions, it's not like she is being controlled by a guardian or something. We deal with the long term side effects of being unable to get out and socialize or even be of much help to others, because the EM workload destroyed our immediate families chance at a normal life.

My dad and I help my mom who has had it for 14 years or so. And she has had help from heavy duty pain pills to take away some of the pain, but her flare is more or less constant - for 14 years. So she's a rare case. And walks less than 25 feet per day with a lot of pain as a result.

It's important to find the right doctor or team and EM patients have to have patients in trying different treatments and doctors, but also realize they have to be proactive if they want to hope to have improvement, if that is possible at all.

Some doctors are rather conservative, others may experiment a bit off label with drugs that might help. With a rare disease they may try a lot of drugs that don't work for you, so we have to keep moving on in trying drugs.

As I've mentioned before mom's pain was level 10 all the time and her pain meds, basically cut this back to level 7 most of the time with occasional flares. Each day with an old father and mom, is almost the same now. . . like Groundhogs day in a bad way. We basically tend to the basics and don't get much done. My fathers health due to his age is not as good and he's not able to do as much. Our case is not typical and is rare in a bad way compared to those with a more minor form of EM. I'm just stating this not to brag, but because I don't want to scare those who have a more minor form of EM. A lot of EM patients may not get as bad as my mom, so we are not necessarily the typical case. I don't want to scare anyone or new EM suffering patients, because some have a milder form of the disease.

Mom tried a lot of things that didn't help. This included pills like Lyrica, Tramadol, Neurontin, Cymbalta, Oxycontin, Metropolol for blood pressure, and vicodin alone. She also had some relief with oral morphine, but that was to difficult to get. Now she uses Norco, Fetynl pain patch, 30mg cymbalta per day and Ativan 1mg 4x a day. As well as some other medications, even Motrin at times. She is using 50mg every three days of pain patch medication and was up to 75mg per day, but that has tapered down. She takes muscle relaxers on occasion as well, and Lisinopril for blood pressure. That's some of the pills she takes each day. She also takes pills due to her lifestyle restrictions which aren't listed here.

We have found some doctors are more conservative in trying new pills and others are clueless as what to do and some will say, I can't treat you. With a chronic disease or syndrome like this there is plenty of time to find a different doctor. We have found many doctors will of course treat the other little problems that can crop up, but the pills and treatment for EM tends to become something of a rut of a treatment. This because many of the pills that might help don't and the patient will get stuck with fewer options that work a little bit and help a little bit.

Thanks again for the information and sharing your story.

12

She's had Erythromelalgia for 14 years, been prescribed all those medications, and none of her physicians ever thought to try a sodium channel blocker? That's terrible. A case study for using sodium channel blockers for Erythromelalgia was published 16 years ago in the December 1999 issue of JAMA Dermatology, a peer-reviewed medical journal. That means it's been published the entire time your mom has had EM. All her physicians would have had to do was search for it. I'll put the link below.

I've seen pictures of your mom's EM that you've posted. They're probably the worst I've seen on this website. Still, the man in the case study was worse. His feet and legs were, quite simply, horrific. There are pictures included with the paper. Mexiletine and Lidocaine infusions resolved his Erythroemalgia and allowed him to live a normal life. If it could work for him, severity is not a limiting factor in the effectiveness of sodium channel blockers on EM. Because his was the most severe case of Erythromelalgia i've seen documented. Yet, it worked for him.

I'd show that paper to your mom's doctors. Immediately.

http://archderm.jamanetwork.com/article.aspx?articleid=478089

13

You know, this just makes me mad. Your mom has had such a poor quality of life for so many years. If any of her physicians ever cared a lick about her, they would have found that paper and tried said approach years ago. That's just gross incompetence. There is no excuse.

14

Carter

Thanks for the comments. Yes it can get frustrating.

Edited by Moderator January 14, 2016.

15

Members, out of respect to the original poster pleased keep responses 'on-topic' or open your own discussion thread to explore other EM relevant issues. Thank you.