LAL-D REAL STORIES

Meet Albie

Diagnosed with LAL-D as an infant

When Albie was born, his mom, Charlotte, said that he was, “Absolutely fine. Everything was fine for about 2 weeks.”

Albie, diagnosed with LAL-D as an infant, with his mother

And then Albie started to have intestinal problems. His doctor thought he might be lactose intolerant or have reflux. However, he kept getting sicker, developing a large abdomen and groin, and wasn’t gaining significant weight. At 2 months of age he had only gained 1 pound since birth.

After numerous tests, physicians, and hospitals, Charlotte still did not have any answers. Finally, a specialist narrowed the issue down to a metabolic storage disorder, but was not yet able to narrow down the diagnosis. According to Charlotte, when she asked what was going to happen to Albie, she was told, “Well, he’ll either live or he’ll die.”

These were words Charlotte would never forget.

Albie, diagnosed with LAL-D as an infant, wearing a motorcycle helmet with his mother Albie, diagnosed with LAL-D as an infant, riding a bike Albie, diagnosed with LAL-D as an infant, holding a puppy

After more tests, it was confirmed that Albie had a genetic and progressive rare metabolic disease called lysosomal acid lipase deficiency (LAL-D).1 LAL-D is associated with multi-organ damage in infant, pediatric and adult patients and premature death in infants.2 At the time there were no treatments for LAL-D, but there was a clinical trial for an investigational medicine. As Charlotte says, “It didn’t matter, the fact that it was a clinical trial, it was something, so I was quite happy.”

After several weeks of treatment, Albie gradually began to put on weight and improve. He continues to receive the treatment once-weekly.

When Albie started preschool, Charlotte remembers bursting into tears. When a teacher asked if she was sad because he was leaving her, she said, “I’m not crying because I’m sad that he’s leaving me, I’m crying because I can’t actually believe he’s here. I never thought I’d see the day.”

Charlotte says her hope for Albie in the future is “that he carries on being the way he is now, so he gets better. I just feel proud. He’s fought for himself, he’s been strong.”

References:

  1. Bernstein DL, Hülkova H, Bialer MG, Desnick RJ. Cholesteryl ester storage disease: review of the findings in 135 reported patients with an underdiagnosed disease. J Hepatol. 2013;58(6):1230-1243.
  2. Jones SA, Valayannopoulos V, Schneider E, et al. Rapid progression and mortality of lysosomal acid lipase deficiency presenting in infants. Genet Med. 2016 May;18(5):452-8.