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HHT: Silent symptoms can result in stroke

Media contact: Lisa Parro, senior public relations specialist, Adventist Midwest Health, Lisa.parro@ahss.org 

La Grange – Chicagoan John Callas always had frequent nosebleeds, but because other family members had them, too, the 36-year-old never gave them a second thought. An excruciating headache in 2005 sent Callas to the emergency room, where he learned he had a genetic disorder that caused a bacterial infection his brain and malformations in his lungs. The disorder also was to blame for the nosebleeds.

A nosebleed might seem innocuous enough, but recurrent ones – more than four a year – should be evaluated. As it was in Callas’ case, frequent nosebleeds could be a sign of hereditary hemorrhagic telangiectasia (HHT), which occurs in one of every 5,000 people and can result in low oxygen levels, bleeding or stroke.

Unfortunately, there are less than a dozen U.S. centers that treat HHT. Adventist La Grange Memorial Hospital is in the process of becoming the first such treatment center in the Midwest, said Dr. Luke Sewall, an interventional radiologist who treats patients at Adventist La Grange Memorial Hospital.

Sewall and his partner, Dr. Steven Smith, have performed many coil embolizations to correct pulmonary shunting. This condition occurs when a malformation creates a direct connection between the arteries and the veins in the lungs. This “shunts” or steals away oxygen-rich blood from the lungs.

Since 2005, Callas has undergone two coil embolizations and has another scheduled for this summer at Adventist La Grange Memorial Hospital. He hopes that the hospital soon becomes an HHT treatment center, at least for his mother’s sake. She also suffers from HHT and was previously treated at Mayo Clinic in Rochester. However, Callas’ mother now lives in a nursing home.

“An HHT treatment center in the Chicago area is sorely needed,” Sewall said. “We are taking the lead, starting by raising awareness about this disorder.”

That awareness includes education. The March 2010 Vol. 137 issue of CHEST Journal, the official publication of the American College of Chest Physicians, published a paper co-authored by Smith and physicians from Yale University School of Medicine: Hemothorax Due to Rupture of Pulmonary Arteriovenous Malformation: An Interventional Emergency.”

However, HHT does not cause all forms of pulmonary shunting. Nancy Kranz, a 61-year-old Brookfield resident, never knew hers existed until undergoing a pulse oximetry test last summer at Backyard-ology, a family health exposition sponsored by Adventist La Grange Memorial Hospital. Subsequent tests revealed Kranz did indeed have pulmonary shunting.

People with pulmonary shunting can experience shortness of breath – although Kranz did not – but older patients might attribute such breathlessness to aging. Feeling winded is not pulmonary shunting’s only hazard. The condition actually short-circuits the lungs’ ability to strain harmful substances such as air bubbles and blood clots.

“The lungs are your body’s filter,” Sewall said. “When you have a direct connection, you lose that filter. Now all the bad things go into the arterial system, where they can cause a stroke or brain abscess.”

HHT presents many silent symptoms. Unfortunately, the first symptom of the disease may be a stroke. This was the case in Callas’ mother, who suffered her stroke immediately before Callas’ brain abscess.

“Sometimes, the symptoms occur so gradually, the patient becomes accustomed to them,” Sewall said. “We are trying to reach the people who have hereditary hemorrhagic telangiectasia and don’t yet know it.”

Patients typically have a history of nosebleeds and might also have abnormal blood vessels that resemble little spider blood vessels on the fingers, lips or gums. Blood vessel malformations also can occur in other major organs, such as the liver, kidney and bowel, each one presenting their own difficulties such as liver or kidney failure. HHT in the lungs can cause bleeding instead of shunting.

Today the disease can be treated in an outpatient setting in about an hour. If the repaired malformations were unusually large, the patient might need to stay overnight in the hospital for observation.

“In the old days, doctors removed the piece of the lung that housed the bad connection,” Sewall said. “Now we can treat HHT in a minimally invasive manner, by inserting a catheter into the abnormal connection and plugging it with a set of coils.”

Kranz had two abnormal connections in the same lung; separate coils were needed to block those connections.

People diagnosed with pulmonary shunting with no genetic cause require a CT scan one month after the procedure. If that is normal, the condition is considered cured. Individuals with HHT should be screened every few years for new malformations.

Kranz, who never experienced difficulty breathing, nevertheless said her breathing now feels different. She is grateful for attending Backyard-ology.

“I never had trouble exercising before undergoing the procedure, but I didn’t know what normal was. Working out has never felt so good,” Kranz said. “Even though I get a check-up every year and exercise regularly, it took a health fair to discover this hidden condition. It showed me the importance of screenings.”

 

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Adventist Midwest Health includes Adventist Bolingbrook Hospital, Adventist GlenOaks Hospital, Adventist Hinsdale Hospital and Adventist La Grange Memorial Hospital.  To find a physician, visit www.keepingyouwell.com.