LAL-D REAL STORIES

Meet Tristan

Diagnosed with LAL-D at 5 years old

Tristan was almost 5 years old when his parents, Holly and Steve, took him to the pediatrician because of his enlarged abdomen and persistent issues with constipation. Tristan’s doctor felt his abdomen and became concerned. X-rays revealed that he had an enlarged liver, but his doctor was unable to diagnose him.

Tristan, diagnosed with LAL-D at 5 years old

Next, Tristan’s parents took him to a gastrointestinal doctor who was also unable to diagnose him. Several months later, Tristan underwent a liver biopsy that revealed that he had stage 2 scarring on his liver. This allowed his doctors to narrow down the possible causes for Tristan’s liver damage, but they still could not provide the family with a specific diagnosis.

Tristan, diagnosed with LAL-D at 5 years old, with his father

Eventually, a genetic doctor was able to diagnose Tristan with LAL-D, a genetic and progressive ultra-rare metabolic disease associated with multi-organ damage in infant, pediatric and adult patients and premature death in infants.1 His parents described feeling a whirlwind of emotions and fear after learning their son had such a rare and serious disease.2 Learning that there was a clinical trial for LAL-D helped them cope with the situation. Tristan was enrolled in a trial for an investigational medicine. He continues to receive treatment today.

Holly says that today life with Tristan is starting to feel more routine. “He’s a typical 9-year-old kid who just wants to run and play; and he really doesn’t let this disease hold him back.”

References:

  1. 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.
  2. 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.