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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