Monday, July 2, 2012


Well, I had this post ready to go for the end of June but somehow it didn't make it up!  So this is more of HHT Awareness Month!  Hope you are learning something!

 I found this fabulous article and wanted to share parts of it.  Now I can't find the link!  EEK!  Just being honest here...this it totally not my writing.....unless I interject in which I will change the font color!! ;)

 About one in 5,000 people—or 1.2 million people worldwide—have hereditary hemorrhagic telangiectasia (HHT).

Patients with HHT tend to form blood vessels that lack capillaries between an artery and a vein. As a result, arterial blood under high pressure flows directly into a vein; normally the blood has to squeeze first through very small capillaries. When an artery is connected directly to a vein, it tends to be fragile and can rupture, resulting in bleeding. A small blood vessel that is abnormal in this manner is called a telangiectasis. A lesion involving larger blood vessels is called an arteriovenous malformation (AVM). Whether a telangiectasia or an AVM, the basic abnormality in the blood vessel is the same.

Telangiectases often occur at the surface of the body, including the skin and the mucous membrane that lines the nose. Almost all HHT patients develop at least a few telangiectases (small red or purple spots) on the skin of their face or hands by the time they reach middle age.
I have some of these in my mouth and on my lips.  I also have one above my left eye.  Kennedy has a few on the inside of her mouth and a larger one on the curve of her lip.

AVMs tend to occur in the internal organs—most commonly the lungs, gastrointestinal tract, brain, and spine, in that order. Abnormal blood vessels also are common in the liver.
Between 30% and 50% of patients with HHT have AVMs in their lungs. These pulmonary AVMs (PAVMs) are poorly understood. Little is known about their history or growth rate. Between 5% and 20% of patients have an AVM in their brains. They also are present at birth.
My father has had PAVMs and had them treated.  I have had PAVMs and had them treated (4-5 times now).  We don't know yet if Kennedy has any.  Sam had some at the base of his brain.  

The greatest risk for patients with PAVMs is stroke or a brain abscess, which also can be life threatening. “In the normal lung,” he explains, “the capillaries between an artery and vein serve as a filter for impurities in the blood, including clots, bacteria, and air bubbles. The impurities are removed before the blood circulates to other parts of the body, including the brain. But when one or more AVMs are present, these impurities can pass through the AVM, go to the left side of the heart, and then on to the brain and other organs.
“Patients who have very large PAVMs also can have problems with their oxygenation, so they can have very severe exercise intolerance,” he continues. “They can occasionally literally turn blue.” 

My father, myself and Sam have had strokes.  I was lucky that I had almost no side effects.  My father was not so lucky.  He has had 3 strokes.  The first was major - it is truly a miracle her survived and recovered.  Sam had a least one stroke in utero, possibly more.  Complications from the stroke(s) are what caused him to die.  

HHT is definitely a manageable!  I will be back soon to discuss ways to treat AVMs!!!

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