Well Gals & Guys, here we all go once again ?

[John1945]

This latest "post" is intended to inform, and share my medical experience's with all of my fellow UK Cmt sufferers, via the 'Health-Unlocked' UK's website !

We are always being repeatedly told by our own individual 'Health-Professionals' (Doctors-Neurologists etc), that Cmt only effects, and results in muscular weakness, in the "lower-limbs" muscle groups that are connected, or controlled by our "Peripheral-Nervous-System" ? (nerves that are totally outwith the body's) Central Nervous System (C.N.S.):

C.N.S. nerves are contained, and protected, within the brain's, and spinal column's bone vertebrae !

On Tuesday 1st September 2015, I am being admitted to my local NHS Lanarkshire Trust Hospital, ENT Ward for a medical procedure to close, and seal a "Pharangeal-Pouch" which has developed in my oesophagus, the "Muscular" tube which connects, and links the throat to the stomach ?

(Please note, the word Muscular ?)

Not everyone who develops a 'pharyngeal-pouch' has symptoms — but when you do, they can be very frightening: A fear of choking is common and, as described, this can stop you wishing to eat, and lead to weight loss:

This ‘pouch’ described occurs when the oesophagus — the "MUSCULAR" ? tube connecting your throat with your stomach — develops a 'bulging' or 'blowout' at the top. Here the lining actually pushes through your "WEAK-MUSCLE" ? ? ? layers, and into the neck itself, though no swelling can be seen externally: (Note, once again ? the often repeated word's ? muscle):

The condition is twice as common in women as in men: It is not clear what causes it, although it is most likely poor co-ordination, or weakness in the patients "THROAT MUSCLES" is somehow to blame ?

As the pouch enlarges, and in my particular case, it does collect undigested food which may then be regurgitated, back into the mouth:

Other symptoms include waking in the night with a sense of blockage in the throat, or bouts of coughing - a symptom that may be easily mistaken for a mis-diagnosis of 'acid-reflux' :

Fortunately, a 'pharyngeal-pouch' is not common — affecting one or two people in 100,000. And, it is usually always in patients over the age of 60:

The best way to diagnose it, is with a 'Barium-Swallow' X-ray - you are given a drink of barium sulphate, a bit like a very dense milk shake, and x-ray pictures are then taken to record the act of swallowing:

Surgery is recommended when there are significant symptoms, especially if food is being breathed into the lungs, causing bouts of infection:

At earlier times, this condition used to be treated as a major medical procedure, where the pouch was effectively removed, via an incision into the neck's skin, and muscle tissue:

However, in recent years, ENT surgery has been via the mouth, with the patient anaesthetised, or even just heavily sedated:

This newer technique, is known as 'endoscopic-stapling', and involves widening the entrance into the pouch so it forms a wide cavity: Although it sounds odd, this actually stops food being trapped. Tiny staples are then used to seal the cut edges: This newer, and less aggressive procedure is highly successful, with fewer medical complication's:

------------------------------------------------------------------------------------

However, the bad news is that this procedure, will require the patient to also have a "tracheostomy", cut into their throat / airway + an extended stay, for wound healing: This procedure is needed to allow the anaesthetist to insert a tube directly into the lungs during this operation/procedure: It cannot be inserted via the mouth/throat, as in a normal operation, because the ENT surgeons will already occupy all space available in the mouth / throat apetures, with their "stapling / laser" instruments:

---------------------------------------------------------------------------------------

Over the last 2 years I have noticed a significant increase in Cmt sufferers, who are all reporting many different, and varying symptoms, and which they are all asking, "Is Cmt to blame for this latest symptom / condition" ? ? ?

My own list to date contains 35No. 'YES', and 'NO' named symptoms, which our "health-professionals" either blame Cmt for, or dismiss Cmt as the cause, or in their own "professional-opinion", is that the symptom(s) displayed are not connected to you the patient's own "peripheral-nervous-system" ? ? ?

I personally believe that Cmt affects every single muscle in your body ? In particular your throat muscles, are usually weak in a Cmt sufferer, causing swallowing dificulties, and snoring, and sleep apnoea, (Greek, 'without breath'):

---------------------------------------------------------------------------------------

Watch this space, for more information !

or, as Arnold Schwarzenegger, would always say "I'LL-BE-BACK" !

Best of luck, and good health to all of my fellow Cmt sufferers !

John (Glasgow) .....

Ps >>>>

Question ? How many individual muscles are in your body ? ? ?

Answer ? Most surprisingly, your body contains, (circa), 600 individual muscles ! ! !

[Southerngrace]

I agree. My CMT has caused many more problems than I was told it would.

[John1945 in reply to Southerngrace]

Hello to Southerngrace .....

I have suffered the effects of Cmt disease, for all of my previous 70 years of my life :

Whilst we, and all the existing UK Cmt Disease sufferers, totally rely on our current "health-professionals" for their own personal medical opinion(s), and advice, we must always remember, and also consider further that you alone as the Cmt "sufferer", may also have many more, and much different, and varying symptoms that only you alone, know how much they effect yourself :

Yes ! always listen and seek medical advice :

However, if the medical advice which is given to you, and in your own personal "opinion" is not what you are in reality are personally experiencing, then you must further explain to your own "health-professional", what in 'real-life' is personally happening to you !

Do not let them dismiss your own symptoms as certainly "not-applicable" to you !

Best of luck, and good health to you.....

John (Glasgow) .....

Ps, I am also aware of NHS Neurolgists, who after confirming your Cmt diagnosis, will sometime say "Yes ! you do have Cmt disease, and as you know at present there is no cure available, so just off you go, and learn to live with it, and manage it as best you can" ! ! !

Sorry, we will not accept that "high-handed-attitude" !

[strawberryshortcake in reply to John1945]

That is what they said to me. Discharged me from hospital. Haven't see him since.

[missspain in reply to John1945]

well john,thats exactly what i was told more than 40 years ago.that said i have had no help from any of my g.p.s nothing .i think they pretend they don't here me.i get fed up in telling or trying to explain what c.m.t. is and how it effects me.really they don't want to know.

all the best to you

[Primary-Bursar]

Hi John

I'm wondering if what I'm experiencing maybe what you are describing. whenever I've eaten I get a lump in the back of my throat which will not disappear and feel like I'm choking when eating. Sometimes the feeling is so severe I have to leave the table. Also I do snore really heavily and do wake in the night coughing and struggling to breathe sometimes. All these symptoms have been attributed to acid reflux by the ENT department even though after all the tests nothing was evident either in my stomach or during the 24 hour acid reflux test. I think I may take your info to my GP and see what he thinks. Thanos gor sharing.

[John1945 in reply to Primary-Bursar]

Hello to 'Primary-Bursar'.....

Yes, I totally agree that you, should visit your G.P. and request them to arrange that you have a "barium-swallow" 'x-ray' to examine your throat area !

If you have any possible problems within your throat, or oesophagus, this investigational 'x-ray' will reveal them, and may result in a diagnosis, of a negative or, positive result, which will then in due course, may or not require a further medical investigation, and a decision to be made ? ? ?

Best of luck, and best of health.....

John (Glasgow).....

[Whiterose]

Thanks John for the update. I hope it all went well for you.

[beludesigns]

This is very interesting, John. Firstly, I hope the ENT procedure went well and was successful in removing your swallowing difficulties and discomfort.

I have all of the symptoms you mention re throat and swallowing! Just hope the guy I'm seeing on Thursday knows his stuff! Watch this space....

PS I assume you trachaeostomy was temporary?

[John1945 in reply to beludesigns]

Hello again to beludesigns.....

The ENT procedure is called Endoscopic-Stapling' went

reasonably well with a discharge 4 days later :

The "tracheostomy" opening in the neck is NOT stitched, and is left to heal, and close over naturally, although the dressings are changed every 3/4 days :

Why ?

The wound is allowed to heal naturally, which means the skin heals from the inside outwards, and mean that you have no "scar-tissue" or wound scar in your neck !

Clever, 'Guys and Gals these ENT professional's are" :

Thanks for your kind wishes.....

John (Glasgow).....

---------------------------------------------------------------------------------------

PS,

Remember on Thursday your appointment is with a NHS Consultant Neurologist, who deals with neuropathy (any problems of your nervous system), also with muscle wasting problems (dystrophy) : He/she will not refer you to ENT Consultant, until they have completed their Cmt investigation, and final diagnosis !

[beludesigns in reply to John1945]

Finally decided to stop apologising and start demanding some relevant 'help'. Emailed my GP last week... requesting a Barium Swallow x ray and explaining why I felt it was appropriate for me. I also pointed out that as Speech and Language Therapist (non practising for some years now) a have a good understanding of the swallowing process and what might cause discomfort at any point along the way!

I followed up the email with a phone call on Monday of this week. Referral routes have changed significantly since I last worked in the NHS. Whilst on the phone, my GP looked on the 'list' of tests they can refer patients for... she could not find the Barium Swallow x ray! I explained that I had asked for a direct referral because, a) I knew that it was the most appropriate assessment for my difficulties b) It would happen more quickly than having to wait for an ENT appointment and THEN for the BS x ray .... and finally c) I thought it might be cheaper for the Practice to refer me directly for the investigation rather than ENT!!!

She said that she would look into it and make a referral to one or the other. Unfortunately, she only works part time (1/2 time) and I have not heard back from her yet. Watch this space!!!

Take care.

[jazmina]

Hi John and all others who are bewildered, confused and frustrated with cmt. Professor Mary Reilly youtube 2016 explains the apparent confusion between Hereditary Neuropathy Diseases who include all disorders experienced by people with cmt as part of cmt by saying that cmt is peripheral, although some nerves are long, thereby giving rise to peripheral disorders; and that some other malfunctioning genes come with cmt and thereby give rise to the same disorders as found by the HND.

The sad experience in the UK seems too often to be, so what? even the cmt consultants couldn't give a damn. But hopefully there are some people somewhere who are receiving positive help.

The cmt organisation in America, free to join, I have found to be very helpful. They even have medically trained people who have cmt handing out advice. They also adhere to the HND list of areas invaded (my words) by cmt.