CMT science #1 (and the benefits of belly flopping)

INSTRUMENTS (#1)

Against all natural instincts, it appears that sometimes it is better to belly flop than dive...

I've been working on the manual pump idea for the bubble organ by making some mockups of the drawing in my last post (so have a look there if you don't get how it works).

It's pretty simple. The valve is made by some weights sealing a piece of rubber over the air intake holes. When you lift the handle, you lift the weights, so the air intake holes open.

The 1/2 inch pipes on the side of the bucket are just for guidance, otherwise it just flops around. They can easily be improved I think.

Here it is in action:

I then tried to make a more compact version, which is where I realized it is better to belly flop than dive. The compact version technically works, but it is just so aerodynamic that it plunges into the water too fast to be useful.

The wide brimmed bucket has lots of surface area, so like the belly flopper, sinks slowly, producing a long and steady stream of bubbles.

I like the fact this instrument is getting bigger and bigger!

CHARCOT MARIE TOOTH

CMT science #1:

I’m obviously not going to explain everything at once, so in the spirit of all good scientific explanations, let’s begin with an assumption and then I’ll try to explain it later in another post. The assumption is that people with CMT have a defect in one of their genes, specifically one that makes proteins within the peripheral nerves.
[There are also many types of CMT, so for the moment I’ll just explain type CMT1a, as it is both the most common and the type that I have].

Peripheral nerves provide a feedback loop between the brain and the rest of your body. They transmit action signals to your muscles to make things move; they transmit sensory signals back to your brain so it know what's happening in the world. Without them you can think a lot, but nothing will happen and you wouldn't know where you were.

If we imagine that the peripheral nerves are wires carrying signals, it makes sense that they need to be insulated (covered in plastic) for them to work, otherwise the signals travel too slowly or get lost.

This is the root of the problem for people with CMT1a.


For a nerve, the insulation is called myelin. The defective gene I mentioned causes this insulation to breakdown (demyelination). Then the signal traveling along the nerve wire (axon) gets lost.

CMT affects the hands and feet most, because they have the longest peripheral nerves (i.e. the longest wires), so the most chance of the signal being lost. The reality of this for me is that most affected muscles either don’t move when I want them to, or move/cramp much later; most affected sensory nerves send no feeling to the brain, or I feel them much later (I get this quite often when someone has trodden on my foot, strange to feel when the culprit has long left the scene)...