• Kenneth Raymond

Any Excuse to Eat Ice Cream is Good Enough for Me

Updated: Jan 18



What if I told you that I am having a tonsillectomy at forty-seven years old? That is nothing that is necessarily out of the ordinary, right? Tonsillectomies usually happen in childhood, but during adulthood is not out of the realm of possibilities. Right? Now, what if I told you that my tonsils are coming out because of my CMT? Would you believe me, or would you think I’m just making things up as I go along? My tonsils are coming out, at least in part, because of my CMT. The connection between me having a tonsillectomy and my CMT is interesting.

First and foremost, there is no known evidence connecting tonsil issues of any kind to any subtype of CMT. I’m not suggesting either that CMT affects tonsils, or that there is a remote and latent connection. If, by chance, somebody out there has established that CMT has a tonsillar component, they are keeping that information to themselves. However, my CMT is the controlling component behind my tonsillectomy.

I have respiratory impairment that has been determined to be a component of my CMT. Both of my diaphragm hemispheres have paresis (paresis is the medical term for muscle weakness caused by nerve damage or nerve disease). My intercostal muscles (the muscles of the rib cage and chest), as well as my back muscles, also have paresis. In totality, my respiratory pump, as the respiratory musculature is also known as, is significantly impaired. I have no indication of any lung tissue disease. My respiratory issues are completely the result of my CMT. What does any of this have to do with my tonsils though? Patience, young Padawan, patience.

My tonsils have no abnormal pathology. I have never had issues with tonsillitis, tonsillar stones, or any other adverse tonsil problems, not even as a kid. Instead, as it turns out, my tonsils are ginormous. This ginormity (I made this word up, feel free to use it) is causing significant airway restriction. The significant airway restriction caused by the size of my tonsils causes my respiratory musculature to have to work even harder than what it already is. My respiratory musculature already exhibits fatiguability. The airway restriction caused by the ginormity of my tonsils causes increased respiratory musculature fatigue. My throat muscles, vocal cords, and vocal muscles undergo compensatory activation to make up for my respiratory musculature paresis. These muscles exhibit fatiguability because they are trying to do the work of the breathing muscles while, at the same time, doing their own job. It’s like they are being forced to do their coworkers’ jobs because their coworkers won’t, and we all have been there a time or two.

After having lengthy discussions with my wife and my physicians about the pros and cons, ins and outs, and the what-if contingencies, we arrived at the informed decision that the tonsils have to go. The short-term objective is to open my airway by removing the restriction posed by my tonsils, thereby lessening the workload of my respiratory musculature and that of my voice and throat muscles. The long-term objective is to hopefully slow down any advancing progression of respiratory pump paresis that might occur if things remain as is. Naturally, with CMT, there is no way to know what progression of paresis will or will not be. However, we decided that if there is something that we can do now to reduce respiratory muscle workload, so doing would have long-term benefits for my overall CMT condition.

Surgery is on Monday, September 21, 2020. The next few weeks of an ice cream diet are going to rock!

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My name is Kenneth Raymond. I am a CMTer who was diagnosed in 2002 at 29 years old, and then genetically confirmed to have 1A a year later. Since diagnosis, I have made it my mission to learn and absorb everything I can about CMT as a whole.

 

I am passionate about sharing everything I have learned, and to share it through engaging narratives and easy to follow explanations. Charcot Marie Tooth disease is inherently confusing. I do everything I can to remove that confusion, and to do so from a CMTer perspective, for a CMTer. The Cryptid Sloth Blog is the vehicle that allows me to share everything I’ve learned and continue to learn.

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