But you still do not look sick. . .

I have posted about this link (Funny, you do not look sick- Spoon Theory) previously.

I hear this quite often, and it is true. I really do not look as bad as this can feel at times.  I find I have to keep reminding some people anyway, that just because it appears I am OK does not mean that I am totally fine.  On the contrary, I am not fine, and it really appears at this point that any recovery I may have now, or at some future speculative date, will only be a partial recovery at best. And probably will not last very long.  I hope I am wrong.

This is a common problem for many with a disease that makes one just feel worse than they may look to the untrained and some trained eyes as well.  There is an acquaintance of mine, who has spoken to others about my condition saying to the effect, “I do not get this.  He looks OK to me. What is up?”  Even my current Physical Therapist who has had a prior patient with PTS/NA and knows at least some of the limitations, had appeared to forget recently that I may not be up for any exercise right now even of a fairly low-key type.

I had to re-explain to him that my muscles do not respond at this point, to any physical “training” that other PT patients may be expected to do at this stage of treatment.  I told him that there may have only been one time in my life when my muscles really felt normal, and that at that time it all felt so good to me that I over-exercised and set my recovery back quite a bit.  I mentioned that currently my body feels extremely shaky with almost all movements.  True, there are some days it is a bit better, but overall it is way out of any normal muscle response to exertion.  All my muscles fatigue easily.  What I can do is limited by this fatigue.  They warned me my muscles may feel stiff after exercise.  I said I really doubted that since to be stiff, they would have had to do some work- beyond what the basics of my mobility currently.

I think that maybe now they get it.  I hope so anyway.  I may be the last PTS patient they see in there practice, but I am there now.   There are things I just will never be- and one main thing is, that I will never be muscular to any normal degree.   I no longer seem to be wired that way, if I ever was.

Life with Hereditary Neuralgic Amyotrophy

Day to day, living with PTS/HNA is not really so bad.  But, it can be limiting.  I can do stuff, but only for a while before the muscles just fatigue and say “NO MORE TODAY.”  My Phrenic nerve is still OK, so I think that makes a bit of a difference.  I understand how limiting it could be if I was tied to an oxygen tank for any time of the day.  Or just to feel constantly as if I had to use all my energy simply to breathe.

I have often wondered if I really had HNA, or if I was just unlucky as far as having a case of PTS that repeated for its own reasons.  Over the years, I have begun to accept that even if I have no other PTS related cases reported in my family, I can still have HNA.  My case is really not as severe as some I have heard about.  Maybe other cases that may be in my family were just not severe enough that they were caught and diagnosed by a legitimate doctor.  I have some suspected reports of weirdness in my family that suggests a possible related type of syndrome as PTS.  Maybe that is enough. Then there is my own history; that if I look back far enough and with the knowledge I have of HNA now, maybe my history also suggests a diagnoses of HNA is the correct one for me after all.

First, I have always been for lack of a good descriptive word, small.  My legs were OK, and I made some good use of this from an exercise standpoint by riding a bike as much as I could to get my lungs into at least fair shape.  I think that has continued to put me in better shape now even though I no longer feel safe to ride.  My upper body just never responded to any sort of exercise program.  I could do exercises, but only a limited number before my muscles just gave out.  I wondered about this at the time, but figured it was just normal for me.  Twenty pushups seemed to be my limit for whatever reason.  Certain other arm related exercises escaped my ability entirely.  Pull-ups?  Nope, not until much later and even then I was limited to only a couple.  Rope climbing?  Surely you jest.  I could not even think how to do that with my limited arm strength.

Others in my PE classes may have started out as I did. But they grew stronger with the work.  I stayed the same, or grew so slowly in strength that I was rapidly left behind.  I began to just know that there were things everyone else seemed more able to do that I could never hope to accomplish physically.

It was not just this memory of having lacked muscle strength in my early years that I am remembering now.  I know that I had periods of neck and shoulder pain even back as far as the 6th grade.  I have memories, and others from back then have told me they were aware of the fact that I had periods I was in a lot of pain.  Some of the shoulder pain was centered on my scapulas.  This sort of shoulder pain repeated many times from those early years to my now documented attacks of NA.  So, it appears I have had a long history of pain and weak or slow muscle growth that I now think of as being caused by HNA.

Any physical activity now is even more limiting.  I worked in the yard today for an entire hour and a half.  It may have been less time than that.  I had a clean-up job to do. It did not involve heavy lifting, but did require a bit of kneeling down and bending over followed by standing again.  The muscles worked for a while.  Getting down was hard from the start.  My legs shake all the way down.  Standing is a bit easier.  What hit me eventually was my lower back.  This low back muscles start to fatigue and I know I better finish up because my time is now limited by the ability to stand at all.  Luckily, the size of the cleanup was such that I could do it, and finish before my muscles just gave out.  People tend to misunderstand at this point, because they can work long enough to actually get a sore back.  Nope. For me it is not about pain as I live with HNA.  The acute periods of an attack surely have the most severe pain I can think of. But ongoing, it is about muscle fatigue and weakness, and also about feeling good that I can even work for an hour or so doing limited work, but at least working.  And no, it would not help to exercise what I do not have to exercise.  That, as I have learned from experience, can cause more problems than it seems to help at the time.

Physical Therapy is working- Shoulder is "thawing out"

Well, with the help of physical therapy three times a week and stretching on my own in between appointments, I am slowly getting range of motion back to my right arm. I am sure the orthopedic doctor who gave me the shot of cortisone will be disappointed at that- assuming I do go back to see him.  What I have found is that the muscles of my shoulder had allowed it to get into a position that stopped it from moving to normal range of motion.  With concentrated effort to move my shoulder prior to stretching, I was able to get it past the previously frozen limits to some extent.  And my Physical Therapist has been getting it to go even further in the manual stretching portion of the treatments.   It is slow progress, but it is working out of it.

Now a frozen shoulder and cortisone inspired hiccups

Towards the end of last summer, I had written about a possible flare. This caused enough shoulder pain that I tended to avoid use of the arm for a while.  I did not really think it had been long enough to cause any problems, but as the first pain subsided a bit, I realized I had lost some range of motion.  I tried to stretch, but by this time it was too late. Now the right shoulder was a bit frozen.

Here are a few things to avoid in the case of future flare-ups. I have to try to remember them myself.

If the pain from a flare is intense enough to cause reduced use, you have to do passive range of motion exercises to keep the shoulder limber from the start.  I am not sure why I forgot about this. It is how I had treated my fingers long ago.

As soon as possible, and as soon as you can stand it, try to do active range of motion exercises.  It may hurt to do so, but it hurts more if you let the shoulder freeze on you like I did.  Now, you may not have the same issues I do. From both MRI's I have had over the years, it has been mentioned that I have a downward sloping Acromial space. This means for me, that the space between the end of the collar bone and scapula is smaller than the norm. This makes me susceptible to impingement disorders. And that may be what I have been dealing with for the last few months.  And, I could have made things better if only I had kept moving.  

Speaking of moving, I have been to a physical therapist recently who actually has treated patients having PTS/NA in the past. I think he really gets it, and I am happy to have been directed to him.

Also, I have been given a shot of cortisone by an orthopedic doctor who really did not get it at all.  I had filled all sort of paper work out explain about my history with PTS/NA.  His first words were, “Well, how did this happen? Were you doing some painting or tree removal?”  Me- No, I really have not been able to do stuff like that. I have Neuralgic Amyotrophy.   Him- “What is that?“    Me- You may know it by its other name, Parsonage Turner Syndrome.   Him-  “Well, that is self limiting and people get better and get immunity to further attacks.”  At this point, I should have run away very fast. Immunity from PTS is nothing I have ever heard of.  I told him of my first two documented cases, and that I know that what started this most recent attack was the same pain in the same part of my shoulder that it has happened the first two times.  The weird thing too is that he is in the same medical group of my original hand doctor who told me I would never recover hand function without surgery.  I told this doctor about that and showed him that my hand was in fact better without surgery.  He then did a short exam and about pulled my arm out of the socket trying to see if I was faking how locked up it was.  Or maybe he thought he could do a quick snap move like a regular chiropractor may have tried.  He also was quick to mention that if I could not get it to free up, that surgery would be needed to fix it.

Surgery.  Hmm. I have not heard much good coming from shoulder surgery.  I am not sure how it would be for me anyway since I am prone to entrapment issues.  Fix it this time, and next time it is going to be worse no doubt.  So, on to the shot.

The shot itself was no big deal.  And it feels like it may be helping me to regain some movement.  I wonder about side effects of cortisone that are not mentioned in the main websites- at least not that I can find.  They mention that it can send your blood sugars through the roof so be sure to tell your doctors that you are diabetic if you are.  It can also make you jittery, as they mentioned at my appointment.  What no one talks about is a possibility that the shot can give you hiccups that can last a couple of days.  For me, I woke up the morning after the shot hicccupping, and it kept up most of the day except for just a few brief breaks.  It seemed totally out of the blue.  A friend asked me if they could be a side effect of the cortisone.  I said, not that I knew about.  But Googling “hiccups after cortisone shots” verified that it happens to many people. And to some who have to take the shot long term, it happens every time they take the shot.  I have no idea why, but I will ask.  Very odd.  I was lucky my own case only lasted the one day.  It was about driving me crazy after a full day of it.

Update: I see on Web-MD that Oral Cortisone is listed with hiccups as a possible side effect.  Not that I had the cortisone orally. . .

Is it really Anterior Interosseous Nerve syndrome, or just a more rare type of Neuralgic Amyotrophy?

PTS/NA can cause symptoms that are exactly like Anterior Interosseous Nerve syndrome (AINS). I know this because I had this diagnosis originally. When my fingers suddenly could not flex to make the traditional “OK” finger gesture, it followed a month or so of arm pain and weakness. At the time, I was told I had a textbook case of AINS. But during all of my exams, no one could tell me why I had AINS.  They kept asking me about the pain in my forearm, which I did not have.  I kept telling them I had pain in my upper arm and a bit in my shoulder. At the time I had checked the known causes of AINS, and found that none of them seemed to match my case.  But, what else could it be?

 Eventually, the other shoe dropped and my diagnosis was altered to PTS/NA and I realized that nerve damage that PTS causes can be misdiagnosed as AINS. That had originally been the reason for this post. I had recently noticed some who had been diagnosed with PTS/NA reporting that they had now also been diagnosed with AINS and now have to worry about two rare diseases. And in some cases surgery had been suggested as a cure.  I had found this article which designates the names of at least some of the nerves impacted by NA.  Notice in the anatomy section the mention of the anterior interosseous nerve

http://wiki.cns.org/wiki/index.php/Neuralgic_Amyotrophy

“Description
Neuralgic amyotrophy is also called Parsonage-Turner syndrome, brachial neuritis, and brachial plexitis. In response to bodily stress (e.g., surgery, flu, unusually excessive exercise, post-vaccination), the brachial plexus, branches of the brachial plexus, or other upper (rarely lower) extremity nerves may develop idiopathic inflammation. The exact cause is unknown. This inflammation is quite painful, which limits use of the arm. As the pain slowly resolves over a few days or weeks, the patient now notices paralysis and a variable degree of numbness in the shoulder and arm. The amount of paralysis varies, with unusually severe cases having complete arm paralysis. Weakness and numbness slowly resolves over time. This may take many months, or even 1-3 years. Although most people improve, the arm may not return to normal and remain partially paralyzed. Brachial plexitis may involve nerves controlling almost any muscle; however, nerves to the shoulder, scapula, and forearm are more commonly affected.

Anatomy
The following nerves may be affected (unilateral or bilateral): brachial plexus, long thoracic nerve, anterior interosseous nerve, posterior interosseous nerve, suprascapular nerve, lumbosacral plexus, and others).”

I hate to see anyone go through surgery that would possibly not be needed or even be an answer for the problem being experienced if there were a viable alternative that may help, namely just to wait it out.  If you have a case as AINS, and do not have the typical causes in your history, it may be caused by PTS/NA.

Well, then I just found this suggesting that maybe doctors have realized that inflammation of the nerves can cause AINS in a similar way as PTS.  See the link here:

http://en.wikipedia.org/wiki/Anterior_interosseous_syndrome

“Most cases of AIN syndrome are due to a transient neuritis, although compression of the AIN can happen. Trauma to the median nerve have also been reported as a cause of AIN syndrome.
Although there is still controversy among upper extremity surgeons, AIN syndrome is now regarded as a neuritis (inflammation of the nerve) in most cases; this is similar to parsonage-turner syndrome. Although the exact etiology is unknown, there is evidence that it is caused by an immune mediated response.
Studies are limited, and no randomized controlled trials have been performed regarding the treatment of AIN syndrome. While the natural history of AIN syndrome is not fully understood, studies following patients who have been treated without surgery show that symptoms can resolve starting as late as one year after onset. Other retrospective studies have shown that there is no difference in outcome in surgically versus nonsurgically treated patients. Surgical decompression is rarely indicated in AIN syndrome. Indications for considering surgery include a known space-occupying lesion that is compressing the nerve (a mass) and persistent symptoms beyond 1 year of conservative treatment.”

Notice it says in most cases it says that surgery would not be indicated for most AINS cases now a days, and outcome for surgery rarely improves the outcome of just waiting.  Unless they can be relatively sure there is something to decompress, you may be better off just waiting.

Sometimes a nodule is just a nodule- back to HNA/PTS discussions

After a biopsy (close to $3,000) which was inconclusive, and a CT scan at around $950.00, it has come to light that the nodule in my thyroid is benign.  Both of these tests, plus around $800 for blood work, are things my old insurance would have not helped much as far as payment- and as such, I would have declined them.  At least now, I can know for sure it is OK to not do anything right away and to monitor the growth over time, for unwanted or unexplained growth.

So for now, I can get back to concentrating on the HNA question. I think it is really a done deal as far as the hereditary part of the NA.  I have clearly had another attack at the end of last summer concentrated to right shoulder as far as worst symptoms go.  This was alluded to in recent posts, and right now is better overall as far as pain goes, but still I have lingering pain in right shoulder, and have to at times use left arm to support the right in daily use.  It was/is clearly nerve related pain, responding to prednisone long term, but not even touched by any amount of ibuprofen. Sleep is an issue again.  Any time on my right side results in pain that wakes me up.

Weakness continues, but it is not quite as bad as it can be.  I really can not even think of trying push-ups until my right arm recovers a bit. Also with this new attack, I am having a bit of vertigo and nystigmus at times. In the past I also went through a short period of this, and it passed eventually.  This time, it is a bit worse and the lengths of time of the "spells" seems to be longer. It makes me wonder again about other possible issues, but I also know that HNA can produce periods of vertigo. And I know that others with HNA have reported dizziness at times.  I will just have to be more careful until it gets better.

New doctor in the house. . .

The first thing to mention about the comparison between my old doctor and the new ones deals with timeliness.  Can a doctor really be on time?  Can you arrive a few minutes early and actually get in before the scheduled appointment?  Well, after having fought my way in to the last few appointments with my regular old doctor, I can say that “Yes you can.” For my appointment, I got there a bit early and got into the exam room ten minutes or so early. The doctor entered right at my appointment time and apologized that he was late. What?

I had lab work of course.  All in all it was almost the same as it was the last time I had blood work.  The difference was that last time my doctor said- It is all normal.  This time, with virtually the same test results, the new doctor went in detail into the results and what they all meant, and then told me the only slight problem was that I was just a bit anemic and that I could benefit from a dietary supplement of B-12 and iron.  I mean, I already knew that from last time but I had not done anything because I was told it was of no concern to be just a bit off as far as being anemic.  This time, I jumped at getting the supplement because I was told it could help.

Oh, and this one actually examined me. To be fair, it has been a while since I had scheduled a real extensive physical with my old doctor.  But, I would have expected that for whatever reason I have had to see him that he would at least have done a cursory exam, since that was how my visits were billed to my insurance.  The new guy did not do an extensive physical either. But he did a quick exam and found a tiny lump on my thyroid.  It is too soon to say what this is as the biopsy is still in the future.

Long time readers, if there are any, may already know that I have been afraid for a while that doctors would have missed something about my overall health because they were thinking that all my symptoms could be explained by PTS/HNA.  And maybe while I was seeing doctors specifically about that earlier on, they were not really looking at my overall other health concerns as much as I may have thought they would or should do.  You know what though? I am not sure how long this lump has been there, but it is possible it may have been overlooked earlier from lack of interest. I can’t remember the last time any doctor anywhere examined my neck.  They may have had reason to check.  It turns out my thyroid function is still OK, but my thyroid could have been playing a part in my overall weakness.  No one ever bothered to check that before. . .