When doctors told Bill Soloway that he had familial hypertrophic cardiomyopathy (FHCM), he felt both overwhelmed and like a mystery had been solved.
Bill’s brother had died at age 27 from a variation of the same condition, hypertrophic obstructive cardiomyopathy (called obstructed IHSS), one of the most common inherited heart disorders. His brother had suffered a stroke years before his death, but back in 1987, the genetic component of the disease was unclear, and no one thought about testing family members.
Nine years later when a young, fit and seemingly healthy Bill was in an airport when he suffered a syncopal episode which he later found out was caused by ventricular tachycardia (VT). At the time, Bill had no idea why it had happened. In fact, after he came to, he boarded the plane, and it wasn’t until the next day when he saw his family doctor in Pennsylvania that he realized how serious and even deadly that could have been. During the visit with his family care doctor, EKG abnormalities had Bill’s physician rushing him to the local hospital. After doctors heard his family history, he was immediately transferred to Philadelphia.
The condition that killed his brother was finally known to be genetic. Bill was diagnosed with FHCM - a condition that causes the cells in the heart to enlarge, changing the structure and therefore the function of the heart. The form of FHCM that Bill had was non-obstructive, meaning blood flow was not blocked, and likely the reason he was still alive.
“When I was told I had the same heart condition that my brother Jack had died from it was like ice water to the face. It was sobering, paralyzing. Being in a center that specialized in these conditions became incredibly important.”
In Bill’s case, the immediate course of treatment was the placement of a defibrillator, a device that would shock his heart back into rhythm if he encounter another episode of VT. After that, there was no way of seeing the future. The only certainty was that Bill’s heart would be closely monitored.
How Genetics is Changing Medicine
Twenty years ago when Bill was diagnosed, genetic screening was just breaking the surface. Patients were being diagnosed after a deadly or near-death episode, not before.
“I wish genetic testing was an option for me. That syncopal episode in the airport could have been prevented. Now we can take the guesswork out of past family heart history. Better to know and prepare than for an event to occur and it be too late.”
Bill and his daughter
Fast forward to today and programs like Penn’s Familial Cardiomyopathy Program offer personalized treatment for patients diagnosed with forms of inherited cardiomyopathies. Genetic counselors can provide counseling for the whole family and genetic testing for those with family histories like Bill’s. And as a father, this is essential.
Bill’s only daughter, now an adult, has received genetic testing for the condition that killed her uncle and almost her father. She is being followed by a team of clinicians, giving her the ability to be proactive instead of reactive.
With his new heart, Bill is living life to the fullest and sharing his story with us. Read part one of his story.