Patients suffering from hereditary hemorrhagic telangiectasia (HHT), an inherited vascular condition characterized by profuse nosebleeds, may find hope in a cancer-fighting drug called bevacizumab, also known as Avastin.

“Bevacizumab is a drug used to treat cancers and eye disorders by restricting the growth of abnormal blood vessels,” said Terence M. Davidson, MD, professor of surgery and director of the UC San Diego Nasal Dysfunction Clinic. “The experimental intranasal application of bevacizumab by injection or spray has produced excellent results for patients at UC San Diego Medical Center.”

HHT, also known as Osler Weber Rendu, is a genetic disease characterized by a malformation of the small blood vessels.

This condition presents itself as small red or purple blister-like spots inside the nose and mouth and on body extremities such as the lips, ears, and fingertips. The most notable symptom is recurrent heavy nosebleeds affecting 50-80 percent of patients. These nose bleeds can occur several times a day, lasting minutes to hours and often requiring frequent trips to the emergency room. For many, repeat blood transfusion is a way of life.

In a 2009 study, Davidson determined the effectiveness of treating HHT-related bloody noses with a KTP laser cauterization and nasal injection of 100 mgs. of bevacizumab. Eighty percent of patients who received the drug and laser treatment reported an 80 percent improvement in nasal health compared to 56 percent in the laser group alone. The benefit of laser alone generally lasted four to six months, and of the laser with the Avastin, up to two years.

A recent case study was published in the February 2010 edition of Laryngoscope. This was the first scientific report of controlling HHT epistaxis by spraying the Avastin into the nose.

Retired pilot Jack Sardisco, 63, is one of the patients who has benefitted from Davidson’s novel drug-laser therapy.

“Years ago, when I was an airforce pilot, I noticed blood in my oxygen mask after flights. I attributed the nosebleeds to changes in cabin pressure,” said Sardisco. “At the same time, I was aware that my father and uncle had frequent nosebleeds but did not make the connection that I was suffering from an inherited disease.”

By the time Sardisco was in his 40s the nosebleeds began to occur weekly and increased in severity. The bleeds were profuse, bringing Sardisco almost to the point of unconsciousness and in need of blood transfusion.

Sardisco underwent an intensive evaluation at UC San Diego Medical Center for genetic testing and radiographic imaging in order to document bleeding sites throughout the body, including the lungs and brain. Receiving the recommended laser and bevacizumab injection therapy, Sardisco’s nosebleeds fully disappeared for two years.

“Unless you have these nosebleeds, you don’t understand how devastating they can be. It’s not an everyday kind of nosebleed,” said Rose Sardisco. “You don’t just go through a box of tissues to stop the bleeding. You go through towel after towel after towel. When Jack’s nosebleeds stopped after treatment, it was like a miracle.”

According to HHT Foundation International, HHT affects 1 in 5,000 people, representing 1.2 million men and women world-wide. The organs most affected are the nose, lungs, GI tract, brain, and spine. More than 90 percent of individuals with HHT have nosebleeds by adulthood. Larger blood vessel malformations may also develop in the body’s internal organs, posing a risk for serious bleeding. Approximately 30 to 50 percent of HHT patients have one or more malformations in the lungs while 5 to 20 percent have malformations in the brain.

Source:  University of California, San Diego Health Sciences