A Study: HNPP ,COPD, Weight loss, and Vocal Cord Involvement.

Linking to the same study. This is of interest to me, as there is a mention of COPD (Chronic Obstructive Pulmonary Disease) as a precipitating factor. ie persistent coughing causing nerve stretching, as well as weight loss due to COPD possibly being another contributing factor.

Laryngeal and phrenic nerve involvement in a patient with hereditary neuropathy with liability to pressure palsies (HNPP).

OR

https://www.ncbi.nlm.nih.gov/pubmed/27241821/

Having severe COPD I am fairly certain I have experienced both the voice and swallowing problems after extended episodes of coughing, and the increased difficulty of breathing due to limited chest expansion during episodes of middle back/rib pain brought on by nerve root pinching in the thoracic spine as diagnosed by a GP many moons ago before my HNPP diagnosis. It’s not much fun, I can say that for certain.

Whether or not I get episodes of phrenic nerve palsy is unknown. Being episodic it rarely coincides with medical examinations or testing. Howver, chest muscle and muscles accessory to respiration I am fairly certain are involved in my case. Even so it hasn’t been easy engaging with Doctors/specialists on this issue. Downright frustrating in fact.

Very distinct problems with no causal link, but with symptoms that complicate each other.

 

 

 

Just a Quickie – e

I’ve seen it written many times, mostly in medical published articles, pubmed etc, that HNPP is mild compared to CMT. I think that’s a nonsense, it’s just too simplistic and does a disservice to both those with HNPP as it does CMT.

Equally one could say that CMT is just a mild form of HNPP, and equally nonsense.

They are both hereditary neuropathies, and CMT1a  and HNPP are caused by mutations of the same gene, Duplication in CMT1a and Deletion in HNPP. They each have unique presentations, and when that is factored with individual variation, the range of signs and symptoms is quite extensive.

Individualised patient care, labels are important but they don’t define the person or the presentation of that persons hereditary neuropathy.

I could be talking nonsense…

HNPP Dot Org

For some unknown reason HNPP.org is no longer online.

It had a rather old-fashioned web interface, but other than that it had a wealth of information about HNPP which was gathered over a long period of time some of which pre-dated the internet age. It had an HONcode kitemark.

I feel lost without it.

However, thanks to the Wayback Internet Archive Machine the information can be found at this address

https://web.archive.org/web/20161126113558/https://hnpp.org

I will temporarily update the links on this blog to point to this address, and hope that at sometime HNPP.org makes it’s way back online.

 

Double (multiple) Crush Nerve injuries – an HNPP perspective

Looking in my drafts folder, I found this post, which I believe I may have posted elsewhere. Six years ago… doesn’t time fly. Amended a broken link, but I thought it was time for this to be posted here. I suppose ‘crush’ in HNPP terms can actually be rather minor pressure, but I think it is a fairly common presentation in HNPP. So without further… Here it is… zombie post no more…

Generally speaking the consensus among medical professionals is that Double crush injuries, ie two (or more) nerve entrapments along the same peripheral nerve bundle is relatively rare. It has been suggested that some refractory (those not responding to treatment) carpal tunnel injuries may be due to another peripheral nerve entrapament/lesion at a proximal (closer to the spine) location, such as the shoulder or neck.

There is some controversy surrounding this syndrome and some believe that it doesn’t exist. In terms of treatment and the success or failure of it for carpal tunnel in the absence of any other nerve pathology, it may indeed be debatable. But what about the cases where other nerve pathology exist, for example another peripheral neuropathy.

Having been diagnosed with HNPP (Hereditary Neuropathy with liability to Pressure Palsy) it has become fairly obvious to me that such a problem of multiple nerve compressions do exist. With HNPP these compression could be momentary and of fairly short duration but will cause prolonged entrapment-like symptoms. I would have thought that double crush syndrome would be far more common in HNPP than in the general population, simply due to the increased liability.

Part of of the postulation for Double Crush, is that the distal compression, eg Ulnar nerve entrapped at the elbow, appears to be far worse for the contributary factors due to a proximal compression of the same nerve branch, for example a compression at the axilla or the neck at vertebrae C8 and or T1. (Diagram of Brachial plexus and nerve division)

The upper nerve compression (proximal) is likely to have much wider effects than the lower compression (distal), but is it possible that the lower compression can appear much worse due to rather trivial compression at the higher (more proximal) location.  It has been my experience that this does indeed happen in HNPP, and can cause sudden worsening of a lower palsy, i.e. an area of sensation loss with varying degrees of muscle weakness.

Another example from my own experience is that of foot drop and lower leg neuropathic pain, which can worsen considerably when I have problems of the lower back, with sciatica in attendance. Any neuropathic pain seems to be amplified at the distal location during these times as does any sensation loss and muscular weakness.

Perhaps this is more likely to happen as the person with HNPP ages, as the multiple locations of previous palsies begins to take it’s toll. It can make this highly variable condition even more unpredictable and difficult to manage.

HNPPWellbeing Blog

I must give another heads up for this blog. It’s great that another HNPP blog is active and posting regularly, and what makes it just so good is that it is so well written and offers plenty of tips and suggestions of how to cope with the type of problems that HNPP can throw at us. Lots of positivity over there.

So, if you have HNPP or know of someone with HNPP, a Doctor or other health professional (do they have time to read blogs), or anyone else, then get yourself over to

HNPPWellbeing

and grab yourself a slice of positivity

You know it makes sense….

PS. And just in case you missed the links here it is again

https://hnppwellbeing.com