Bo Tsuen Bernabe Velarde-Chan

His Microvillous Inclusion Disease caused severe dumping within the 1st three days of life; it took 2 hospitals and 5 weeks for diagnosis of this rare and life-limiting disease. Fewer than 300 documented cases worldwide exist in the literature, ever. Historically, kids with the early onset (what Bo has) do not live past the age of nine months; largely due to the liver toxicity of standard TPN's plant-derived lipid component. A UMich study confirms this with their metrics showing: if the bilirubin is over 2.0 for 4 or more consecutive months, the mortality rate is 80%.

Although the UMich had only seen 2 other MID patients in the last 10 years, their prognosis was grim; they told us to consult with Palliative Care "go home and die" Team if we did not want to consider Transplant.

Bo's bili spiked up past 9, and was elevated for 2 months before we got it in gear and got to Boston to start Omegaven, mid-Sept 2007. We were there a mere 4 weeks when it dropped to 2.2. We were on a plane home by early November. His bili dropped to 0.0 by January and bounces around there and 0.1/0.2. We are planning an pig roast, birthday and baptism party where we were once dreading a funeral.

Through the Oley foundation, we are in touch with 2 families with 2 kids each living in their teens and 20's. The older kids are arguably the oldest living survivors of this disease.

He only has a central line for his TPN. Because he is considered in intestinal failure, he does not have any g, j, gj, or ng tube for enteral pump feeding, and could probably get listed for transplant whenever he wants. We hope we never have to cross that bridge. We are still hoping for a miracle (yes, I started going to church, and praying), and continue to feed him. I nurse him very small volumes (1 oz each?), 4x/day. He eats a little (less than a teaspoon)yogurt or baby food 1-2x/day.

Although the literature suggests doom and gloom, since it is a rare disease, and the literature around MID is sparse at best, and scientifically questionable at worst, we think it's important to consider the source of this prognosis. Most of the literature is around babies who did not have Omegaven, and did not live very long. We believe that the Omegaven variable will turn this probability on its head. There is no literature that discusses MID in later years, as there was never an adult patient population to follow. This may all be changing now.