What is Marfan Syndrome? - Marfan Foundation

WHAT IS MARFAN SYNDROME?

Marfan syndrome is a genetic disorder that affects the body’s connective tissue. Connective tissue holds all the body’s cells, organs and tissue together. It also plays an important role in helping the body grow and develop properly.

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Connective tissue is made up of proteins. The protein that plays a role in Marfan syndrome is called fibrillin-1. Marfan syndrome is caused by a defect (or mutation) in the gene that tells the body how to make fibrillin-1. This mutation results in an increase in a protein called transforming growth factor beta, or TGF-β. The increase in TGF-β causes problems in connective tissues throughout the body, which in turn creates the features and medical problems associated with Marfan syndrome and some related disorders.

Because connective tissue is found throughout the body, Marfan syndrome can affect many different parts of the body, as well. Features of the disorder are most often found in the heart, blood vessels, bones, joints, and eyes. Some Marfan features – for example, aortic enlargement (expansion of the main blood vessel that carries blood away from the heart to the rest of the body) – can be life-threatening. The lungs, skin and nervous system may also be affected. Marfan syndrome does not affect intelligence.

Who has Marfan syndrome?

About 1 in 5,000 people have Marfan syndrome, including men and women of all races and ethnic groups. About 3 out of 4 people with Marfan syndrome inherit it, meaning they get the genetic mutation from a parent who has it. But some people with Marfan syndrome are the first in their family to have it; when this happens it is called a spontaneous mutation. There is a 50 percent chance that a person with Marfan syndrome will pass along the genetic mutation each time they have a child.

Knowing the signs of Marfan syndrome can save lives

People with Marfan syndrome are born with it, but features of the disorder are not always present right away. Some people have a lot of Marfan features at birth or as young children – including serious conditions like aortic enlargement. Others have fewer features when they are young and don’t develop aortic enlargement or other signs of Marfan syndrome until they are adults. Some features of Marfan syndrome, like those affecting the heart and blood vessels, bones or joints, can get worse over time.

This makes it very important for people with Marfan syndrome and related disorders to receive accurate, early diagnosis and treatment. Without it, they can be at risk for potentially life-threatening complications. The earlier some treatments are started, the better the outcomes are likely to be.

Knowing the signs of Marfan syndrome can save lives. Our community of experts estimates that nearly half the people who have Marfan syndrome don’t know it. This is something we are working hard to change.

Learn more about the signs of Marfan Syndrome at The League of Extraordinary Tall People

The League of Extraordinary Tall People

As of recently I have come across a lot of clubs, chapters, groups, and chat rooms that are designed for people like me, tall and the extremely tall. 

  The League of Extraordinary Tall People is the exception to the rule when it comes to a tall group, led by a tall person, who is not affiliated with the Tall Clubs International brand.  In this group, all people of tall stature or who think they are tall are welcome with open arms.  The only requirement is that they share idea, stories, and an overall level of camaraderie among the tall population of the world.  

The difficulties of being a tall person in a not so tall world, is that there are few resources designed for the benefit of being tall.  Automobiles are few and far between, and the auto makers will go out of business before they have a commercial that highlights their vehicles specially made for the height enriched.

The Tall Club: Clothing Shopping Extravaganza

If you're in the National Basketball Association as a player, or the National Football League, then shopping for clothing is just a matter of deciding who is going to custom design your clothes.  If you're just a normal every day kind of guy like me, then this is not an option, and clothing shopping takes on a whole new definition of exploration.  Check out my profiles for stores that I have found to provide for the Tall man.

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The Tall Club: Over-sized, Under Weight

Over Sized and Under Weight

The world is changing, and to stay on pace with the constantly changing status quo, there has been an entire industry raised from near non-existence into a multi-billion dollar profit system: Weight Control.  Thermogenics  will raise your body temperature to help you sweat, combining caffeine with cayenne pepper to keep you awake and burning calories, but, the problem that I have suffered through for most of my life isn't burning the calories, but keeping them inside of my body, creating fat cells, and keeping that bad reputation fat from leaving.  I am tall, 6’9” to be exact, and my body has a build that can sustain 300 lbs. but the metabolism of a race horse keeps me at a much lower threshold.  As a tall man in a 5’10 average size man world, the problem isn't losing weight, but rather keeping the weight ON! 

Do you play basketball? Absolutely.  Could I be a valuable source of intimidation through sheer size and breadth on a court, volleyball pit, softball field, or goalie post, yes I could, but, only if my body had the width to go with the height could all of those presumptions come forward. 

Over the 40 years of life, I have seen, and tried many things to put this weight on, while in a world where everyone else seems to want to strip it from their bodies.  Protein has evolved as well as other products not governed by the FDA as nutritional supplements.  Below is a list of products that have worked for me in putting weight on, and the timeline that I used them:

Protein – Whey protein is what my father would put in a morning milkshake, the kind that busy parents make for their kids before sending them off to school.  The combination of two scoops of whey protein, a banana, strawberries, and milk, does the same thing as cereal, but with the addition of the whey protein, sent me into the metabolic world of super available energy.
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Nitric Oxides – I have found, for my body, that taking these kind of supplements before working out helps with the vaso-dilation of my veins, meaning my veins expand allowing for a rapid absorption of supplements, while also decreasing muscle strain during times of heavy weight lifting.  As evolution of body composition continues throughout the aging process, I used this supplement a lot more during my younger years, 18-30, because mass was my goal, putting it on, and keeping it on.  From age 31-Present, I use Nitric Oxide (N.O.) less because my metabolism has slowed down, and mass is second to lean muscle building. 

Carhartt for Tall men

Creatine – The most widely known and advertised creatine is by far mono-hydrate, which when used properly and instructions followed, will fill your muscles with muscle building nutrients.  The problem I found with Creatine Mono-hydrate is that it also filled my muscles with a large amount of water weight, which looking from the outside in, makes your muscles look bigger and fuller, but, only if you can maintain a constant flow of water drinking, and a workout routine that provides enough muscle shock to keep your muscles from getting into the sustainment phase, which is a bad thing when you are trying to gain mass muscle density.  The Creatine that I use today, Creatine HCI, has a better effect for my body and workout routine.  The HCI allows me to build Lean Muscle without the water weight, and it comes in capsule form, also allowing me to control the amount that is taken, thereby adjusting the dosage to fit my body, and keep waste down.

The Tall People FAQ

#1 Question of my life

Q:  Do you play Basketball?
A:  Do you play Miniature Golf?
Alternate Answer: (Spit above their heads) and then tell them its raining.

Frequent Aliases

" You look like Bull from Night Court"

"Lurch"

"Too Tall Jones" from Police Academy

"Wingspan"

" David Robinson"?

Popular Quotes from the past 40 years of life

"Unfortunately we don't have a protective vest that will cover your entire upper body, but, we still want you to deploy to Iraq"

" We can cut two sleeping bags in half and then sew them together so that you can do the cold weather training, but, until then, we advise you wear a warm jacket to cover the parts of you that don't fit"

" You must be under this height to go on this ride"

"  Would you like to sit at the rear of the plane where there is more leg room?"

"If I were your size, I would be in the NBA"

"Do you dunk"

"You are going to need to get on your knees when talking to me, so I don't have the feeling that you are superior to me" - Army Officers

" You can't come in to this club because it would take all of the bouncers to escort you out"

The Tall Club: Vehicles for Tall People

As some people have found out, myself included, automobiles, trucks, SUV's, and crossovers are not created equal.  In the past years I have sat in just about every kind of car auto, and have been greatly surprised by some, and happily driven away in others that I didn't believe I would fit in.  Below is a summary of the vehicles that I have been able to fit in, and others that I have not.

Ecig Avenue

Toyota Trucks - Plenty of leg room, but not at the top.  My legs fit with extra space under the steering wheel, so that I didn't have the split knee thing going, but, the vehicles lack the head clearance at the roof.  This is just for the Tundra version, as I have not tried to sit in the Tacoma, but, when you are a large person, the going thought is that you should only try to drive a big Truck.

Geo Metro - This vehicle surprised me, as it had plenty of leg room, to the point where I had to move the seat forward, and head clearance was a breeze.  The only problem tall people will have with this vehicle is that you may look like Shaquille O'neal getting out of a mini cooper commercial.

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Ford Trucks - I once test drove a F-250 Texas Ranch  version, with the thought that "Everything is bigger in Texas" only to find out, that is not the case with this truck.  No leg room under the steering wheel because the seat sits higher, which is also the problem for head clearance.  On the other hand, I drove a Fleet Ford F-150, and had plenty of both, driving off the lot and enjoying the experience until I ended up living in a area where Air Conditioning and power windows was a necessity.  The Fleet Ford F-150 would be a great starter vehicle for somebody tall, young, and needs a reliable vehicle for in-city travel.

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Lexus ES-350 - I still drive and enjoy this vehicle, especially since I fit in it with plenty of head clearance and room for my legs.  This is probably the best vehicle I have driven for my size, as coupes go, and having 30 MPG helps a lot in the $4 a gallon economy that we currently have.

Jeep Wrangler Sport 2-Door - Lots of leg room, plenty of head space clearance, and the option to take all the doors, lower the window, take off the back cover options that comes with owning a Jeep.

BMW 4 series - This vehicle is one of the vehicles I am very interested in seeing if I fit in.  It has a longer wheel base than the 3 series, has been clocked at 8.3 seconds in a 0-100 mph test, and is shaped like a bullet.  If this vehicle can fit me, it will go to second place in vehicles preferred for tall people, right behind the Lexus ES350.

A Tall Man in a Small World

A Website for Tall People

 I was born at a normal size and weight, 9 lbs. 21 inches, but, by the time I was 8 years of age, I was 5 feet tall and as skinny as a rail.  At 12 years of age, I was at 6 feet tall, and finished my growth at the age of 19 with the height of 6'9".

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  My wife asked me to write about what it was like when I was "little", the problem with the question lays in the question itself, as I was never "little".  I grew up, and continue on today as the tallest person in my Army unit, as it has been for the past 19 years of service.  I can however, talk about the pains of growing, and the advantages and disadvantages of being non-height restricted.

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  As I stated in my opening paragraph, I grew quickly, adjusted to the awkwardness of having more body than I knew what to do with, and became aware of the expectations that the general public had of extra tall people, especially boys/men.  The part of being tall that most people never hear about, is the pain that is associated with rapid growth.  Rapid growth in my body was not something that by body wanted to accept, sort of like chemotherapy for a cancer victim, its something that is foreign to the body, and forces it do something that is unnatural.  In my case, the growth phenomena meant that my bones grew at a pace that my muscles, tendons, and fibers could not keep pace with, and often lead to massive full body cramping.  The cramping would take over all body functions, causing a racking of pain that felt akin to having all of the bones in my body cracking at the same time, locking in rigidity, and then swelling with searing hot pain.

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In Combat, when a weapon malfunctions, the steps to correct the problem is called remedial action, such as when a bullet double feeds, we do what's called SPORTS, Slap, Pull, Observe, Release, Tap, and Squeeze the trigger. 

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Unfortunately, when my body would go into full cramp mode, there was no known remedial action to call upon, so, my parents went into the depths of their encyclopedic brains, and pulled out stretching exercises for me to do, to try to loosen the muscles, which worked for the most part, and I still use to this day to help with the random cramping that my body inflicts me with at random times of the day.  Shop Amazon - Back to School