VCU news

Lisa Brown
VCU School of Mass Communications

No one is immune. It can attack at any time. It is not specific to any age, ethnicity or gender – but African Americans may be at greater risk. And the worst part: pulmonary hypertension, known as PH, is difficult to diagnose.

That’s why two professors at the Virginia Commonwealth University Medical Center are part of a nationwide campaign to create public awareness about PH, which is often associated with sickle cell disease.

Wally Smith, M.D., and Paul Fairman, M.D., are hoping to educate people in the Richmond area about the warning signs for both conditions. Now is an opportune time as November is PH Awareness Month.

“There are no symptoms that are unique to PH, and that’s a potential problem,” said Fairman, director of the lung transplant program at the VCU Medical Center. “Shortness of breath is the primary problem. But there are other causes for shortness of breath, so PH is often misdiagnosed.”

Because of the complexity of PH, the diagnosis requires several tests to determine how well a patient’s heart and lungs are functioning. This is quite a strenuous process, as Rhoda Perozzi has learned.

Perozzi taught in the biology department at VCU from 1993 to 2003. For years, she has suffered from a variety of heart problems and several cases of pneumonia. Last winter, Perozzi developed a particularly dangerous form of pneumonia, called pseudomonas. She then developed blood clots in her lungs.

Her doctor ordered an echocardiogram – an ultrasound of the heart, which showed mild pulmonary hypertension. Last month, Perozzi had a formal sleep study to rule out sleep apnea as part of the problem.

Perozzi still must undergo a few more tests before the diagnosis of PH is complete.

The underlying cause of Perozzi’s problem is still unknown. For many patients, however, there is a disease that is common in causing pulmonary hypertension: sickle cell.

Smith, co-director of the adult sickle cell program at the VCU Medical Center, said sickle cell is a “blood disorder causing anemia, pain, early death and too many complications to name.”

The connection between sickle cell disease and pulmonary hypertension is a rather recent discovery.

“Sickle cell disease was not known to cause PH until recently,” said Smith. “The way it seems to be doing it is the reduction in hemoglobin ends up essentially poisoning the blood vessels, causing the blood vessels to constrict, leading to high blood pressure in lots of places, especially the lungs.”

PH develops in one-third of adults with sickle cell disease, causing a much higher death rate in sickle cell patients. Sickle cell is the “most common inherited disorder – 8 percent of African Americans have the gene,” Smith said.

There is no cure for PH, according to the Pulmonary Hypertension Association. But several treatments are available, the group says on its Web site. “Most patients have more options that ever before.”

For more information on pulmonary hypertension:
Pulmonary Hypertension Association – http://www.phassociation.org/Learn/What-is-PH/
PH Symptoms and Diagnosis – http://www.pph-net.org/pph-symptoms-pph-diagnosis.htm?GAW-PH
Dr. Paul Fairman’s PH Website – http://www.pulm.vcu.edu/
Primary Pulmonary Hypertension – http://www.healthdangers.com/injuries/pph/pph-symptoms.htm