This is an excerpt from the HHT Foundation website. My mother is seriously afflicted with great amounts of blood loss. This disease has nearly taken her life. Her heart is currently being monitored and I suspect the problem she is experiencing with an irregular heartbeat is the cause of HHT. I am also afflicted, but my symptoms are not as severe:
About HHT
Posted By Ann On September 30, 2007 (2:20 am) In About HHT
Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of the blood vessels, which affects approximately 1 in 5,000 people. It affects males and females from all racial and ethnic groups. The disorder is also sometimes referred to as Osler-Weber-Rendu (OWR) after several doctors who studied HHT about 100 years ago. In 1896 Dr. Rendu first described HHT as a hereditary disorder involving nosebleeds and characteristic red spots that was distinctly different from hemophilia.
Before Dr. Rendu’s work, doctors did not understand that individuals with what we now call HHT have abnormalities of their blood vessels, not a clotting problem in the blood itself. Drs. Weber and Osler reported on additional features of HHT in the early 1900s. More than one hundred years later, HHT is still often misdiagnosed in affected individuals and many doctors do not understand all of its manifestations.
HHT is a genetic disorder that causes abnormalities of blood vessels. Most blood vessels in the body of someone with HHT are normal. However, a small percentage of the blood vessels in a person with HHT have a specific type of abnormality.
Blood vessels are the tubes that carry blood around our bodies. There are two types of blood vessels: arteries and veins. Arteries carry blood under high pressure out to all areas of the body after being pumped by the heart. Veins carry blood that should be under low pressure, back to the heart. An artery does not usually connect directly to a vein. Usually there are very small blood vessels called capillaries that connect an artery to a vein.
A person with HHT has a tendency to form blood vessels that lack the capillaries between an artery and vein. This means that arterial blood under high pressure flows directly into a vein without first having to squeeze through the very small capillaries. This place where an artery is connected directly to a vein tends to be a fragile site that can rupture and result in bleeding. We usually call a blood vessel that is abnormal in this way a telangiectasia (tel-AN-jee-eck-TAZE-ee-ya), if it involves small blood vessels. We tend to call it an arteriovenous malformation (AVM) if involves larger blood vessels. So, an AVM might be thought of as a big telangiectasia. The basic abnormality in the blood vessel is the same.
Telangiectases tend to occur at the surface of the body such as the skin and the mucous membrane that lines the nose. AVMs tend to occur in the internal organs of the body. The telangiectases (plural for telangiectasia) and AVMs of HHT occur primarily in the nose, skin of the face, hands, and mouth and the lining of the stomach and intestines (GI tract), lungs, liver and brain. It is not currently known why these abnormal blood vessels tend to occur in certain parts of the body and not others.
1 comment:
I understand what it is like to have a parent who suffers from a very severe case of HHT. My father passed away last October at age 58. The complications got to be too much for him. The Mayo Clinic did wonders for keeping my Dad alive as long as he was.
I hope the best for your mother.
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