How a Viagra-like drug changed a little girl's life

If it weren't for the tiny tubes delivering oxygen into her nose, you'd never guess 3-year-old Elizabeth Jones was sick.

"Something happens and it just makes you realize how fragile she is," Diane Jones told us in a 2010 interview. 

Fragile, because for her first three years, Elizabeth spent half her life in hospitals.  A simple cold could land her in intensive care, or worse.

"It's really hard, because I cannot, having almost lost Elizabeth four times, I cannot even imagine that next step where you don't ever see them again," Diane said.

Then doctors began a treatment using a drug you'd never expect.  Elizabeth was given a pill called Revatio.  The active ingredient is the same as Viagra, the little blue pill men take for erectile dysfunction.

Diane told us in 2010, "It was surprising that they were going to use it in a little girl."

Back then, both of Elizabeth's parents were equally surprised at how well it worked.

"The energy level, the gross motor skills improved.  Her strength improved. Even her eating habits, she began eating more off the table, improved.  There was a light at the end of the tunnel," said Steve Jones, Elizabeth's dad.

Elizabeth has pulmonary hypertension, or PH for short.  A defect in her heart caused critically high blood pressure levels in her lungs.  Without treatment, patients can die within three years.

"We always hear about cancers and the heart diseases, and pulmonary hypertension is just as deadly.  It's one of those diseases that can strike at anytime, and when it does, its vicious," Steve said.

Elizabeth would need more than just a pill to survive.  She needed major surgery to close a hole in her heart.   Harvard professor Doctor Gerald Ross Marx is her pediatric cardiologist, and he said treating her has been a delicate process.

"We had tested her a number of times over a two-year period by cardiac catheterization to be as relatively sure as we could be that this was the right time to intervene," Marx said.

The "right time" came almost two years ago, at Boston Children's hospital.  Elizabeth's doctors say it was possible thanks to a previous procedure and Revatio—that Viagra-like drug—that kept her blood pressure down.

"We think it was the combination that allowed us to move forward with the surgery.  There's no question about it, I certainly think, or I would be led to believe that we have made a major difference in her case because of the medications," Dr. Marx said.

Today, Elizabeth is swimming.

"If you told me two years ago that this is where we we're gonna be, I would not have believed it," Diane said.

Elizabeth is also dancing with her video games.

"I think we're out of the tunnel now, and we're heading on out down the tracks," Steve said.

Elizabeth only needs oxygen when she sleeps, and there have been no more trips to the ICU.

Her parents credit the drug, which Elizabeth still takes to control her blood pressure. The unfortunate reality is that Elizabeth may never be free of this life-threatening disease.

"I never lost hope, I never will lose hope," said Steve, who is attending conferences and stays up to date on the disease.

Diane tells us that Elizabeth seems to live every moment to its fullest.

"I look at her and she's so full of life. And she loves every minute of every thing she does.  She's the closest thing I've ever seen to a miracle."