HPP REAL STORIES

Meet Adelyn

Diagnosed with HPP at 2 days old

When Adelyn’s mother, Jennie, first found out she was pregnant with her third child, she remembers thinking, “I don’t care if it’s a boy or a girl, as long as it’s healthy.” And sure enough, Jennie had a healthy pregnancy with no signs that anything was wrong.

Adelyn, diagnosed with HPP at 2 days old

And then, Adelyn was born, and she was very unhealthy.

“When she took her first breath, her whole chest cavity collapsed. She was blue in color. Her limbs were going the wrong way. It was really terrifying,” said Adelyn’s father, Joe.

Adelyn’s doctor took X-rays, which came back showing limited bone growth, or, as Joe says, it was “similar to a jigsaw puzzle with all the pieces missing.”

Adelyn’s doctors thought she could have osteogenesis imperfecta, a type of dwarfism, or hypophosphatasia (HPP). After several days involving many tests, including one that indicated her alkaline phosphatase level was zero, they determined that she had HPP, a rare, genetic, life-threatening metabolic condition that prevents minerals such as calcium and phosphate from being properly deposited in bones.1-3

Joe and Jennie were told that there were no approved treatment options but that there was a clinical trial involving an investigational medicine. Joe and Jennie didn’t have to think twice about enrolling Adelyn.

Adelyn, diagnosed with HPP at 2 days old, with her father Adelyn, diagnosed with HPP at 2 days old, with her mother

At 12 days old, Adelyn received her first treatment, and after a few months of continued investigational treatment, Adelyn was able to return home.

Adelyn transitioned out of the clinical trial and continues to receive treatment.

As Joe says, “When I hold Adelyn now, it’s like holding the sun—a ball of warmth. She just looks at you and warms your heart. I think, while I’m looking at her, that, ‘You’re amazing. You’re a miracle. You probably shouldn’t be with us, but here you sit, smiling hugely at me and making my life better each day.’”

References:

  1. Rockman-Greenberg C. Hypophosphatasia. Pediatr Endocrinol Rev. 2013;10(suppl 2):380-388.
  2. Fraser D. Hypophosphatasia. Am J Med. 1957;22(5):730-746.
  3. Whyte MP, Greenberg CR, Salman N, et al. Enzyme-replacement therapy in life-threatening hypophosphatasia. N Engl J Med. 2012;366(10):904-913.