Lipodystrophy

How many people have often wondered what I do to keep my muscles so prominent?

Just a preview of what I look like with Lipodystrophy. The ripples you see along my abdomen in this dress are my muscles, not fat.

Do you think I’m a body builder?  Do you think I lift weights all the time?

I wondered the same thing throughout my entire life.

Why am I so muscular when I don’t lift weights?

I remember when I was 10 years old, the summer of 1982, I joined the summer swim team.  I was actually a pretty good swimmer and won quite a few awards for my swimming.  From that time, I finally realized that my body was rather different from everyone else’s.  Kids at the pool started calling me “She Man”, because I had such well defined muscles.  Along with these over developed muscles were a few other strange physical characteristics such as having a rather long torso which made many shirts difficult to wear without showing my belly.  It made those one piece bathing suits for swim team rather difficult to fit my body.  My face was always a “full face” with a double chin, so I was rather self conscious of these things and there are not very many pictures of me because of my low self esteem for how I looked in pictures.  Back in the early 80s, this was just kids being kids and teasing was just a part of life.  Today, we would call this body shaming.

It wasn’t until Terminator 2 came out in 1991 that things shifted in the body fashion sense.  That opening scene with Linda Hamilton, working out in a prison cell made all the difference.  She was buff and strong and had well defined arms.  That’s when people really started going for the strong body look.  Prior to this, I was being approached by people left and right asking me “Are you a body builder?”, “How much can you bench?”  Well, no, I wasn’t a body builder.  “It’s all natural,” was the best response I could give, followed up with “I can’t step foot into a weight room or else I’ll bulk up that much more.”

Age 19.  1990.  Well defined muscles due to no subcutaneous fat on my arms, legs and torso.

Let’s fast forward a few decades.  I’m still very muscular.  I have some really weird health issues that would make you think I exist on a diet full of pizza, cake and alcohol.  I’ve always had an impressive appetite and I can almost eat anyone under the table if I wanted to.

I have insulin resistance, which is a fancy description for Type 2 Diabetes.  My body can produce insulin, but my body can’t utilize the insulin I produce.  I am on an insulin pump with a special insulin that is 5 times stronger than regular insulin.  Even though I eat rather “clean”, my blood sugar levels are quite unstable and high.  I’ve often been accused by my doctors of “cheating” on my diet.

I also have extremely high cholesterol and triglycerides.  These are the lipids/fats in your blood, usually filtered out by the liver, except for me.  Normal cholesterol levels are under 200 on a blood test.  Normal triglycerides are under 150.  My triglycerides range in the THOUSANDS.  Having such high triglycerides is what causes pancreatitis, and I have had pancreatitis way too many times to count now, since 2011.  The standard treatment for pancreatitis is to be admitted to the hospital with IV fluids, IV pain medication and NPO – meaning “nothing by mouth”.  Recently, we’ve added a new treatment for the triglyceride levels: plasmapheresis.  This is similar to dialysis, but it focuses on the plasma, cleaning the lipids from the plasma before it’s returned to my body.

Why is all this happening?

Definition borrowed from LipodystrophyUnited.org

I have an extremely rare metabolic disorder called Lipodystrophy.

Lipo = fat          dys = bad         trophy = tisssue changes.

Lipodystrophy is any condition that results in the loss or redistribution of fat tissue, as well as a defective metabolism of fat.  This is one of those rare genetic diseases that has a prevalence of 1 out of 1,000,000.  There are many different types of Lipodystrophy and mine is called Familial Partial Lipodystrophy type 2.  Here is the definition from the rare disease information from the NIH – National Institute of Health:

“Familial partial lipodystrophy type 2 (FPLD2) is a rare, genetic disorder that affects the amount and distribution of fat in the body. It causes a loss of fatty tissue from the torso, buttocks, and limbs, while causing a buildup of fatty tissue in the face, neck, shoulders, and abdomen.[1][2][3] Symptoms typically develop around puberty. FPLD2 can be associated with a number of metabolic complications, including insulin resistancedyslipidemia, diabetes, and liver steatosis.”

Now that I know what my genetic disease is called, I am able to reach out to others just like me.  I believe that one of the components that has not been addressed just yet in the medical realm is the musculo-skeletal component.  All those prominent muscles are actually quite tight, tense and painful, but children and most adults don’t know how to communicate what they are feeling as something ‘out of the ordinary’ because they have been living this way since birth.

If you think any of this sounds like you, or you would like more information, please feel free to contact me.

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