Dean is getting a liver!

Dean is a very fun loving 5-year-old boy that loves to play with toy trains, build exquisite tracks, and loves even more to hang out with his big sister Grace! Dean, born with a rare metabolic disorder called a Urea Cycle Disorder is missing an enzyme in his liver, not allowing it to function properly. This UCD causes Dean to be on a very strict low protein diet of just 16 grams of protein a day! In order to protect Dean’s brain and to provide him with a better quality of life, Dean has been listed for liver transplant.

Dean was born with a rare Urea Cycle Disorder (UCD), Argininosuccinic Aciduria or ASA. He was diagnosed at five days old through the newborn screening test. A UCD causes a build-up of toxic ammonia in his bloodstream, which travels through his body and causes damage to his organs, brain, lead to comas, and can be fatal. Due to his specific UCD, Dean also lives with argininosuccinic acid in all the cells in his body, which is not only dangerous to his entire body, but causes slow cognitive deterioration.

The first two years of Dean’s life were very rough, as he was often in and out of the hospital with NG tubes. Anytime that Dean is sick, it is imperative that he still receive a minimum calorie and protein intake. Thankfully, Dean has been more stable the past couple of years, but any time he is feeling under the weather, it is a battle of getting in enough foods to keep his body and brain safe. This creates a lot of stress for Dean and his family and more potential hospital admissions.

Dean’s transplant will take place at Texas Children’s in Houston, TX. This transplant requires Dean and his mother, Shelby, to be away from home in Oklahoma City for almost two months. Dean will also have to be medical flighted to Houston once they get the call for his new liver. While Dean and Shelby are in Houston, they will incur many costs in addition to the costs of the surgery. Food, lodging, and parking to name a few. In addition, this transplant will require Shelby and Dean to fly back and forth many times post-transplant in the following months and years for follow-up medical checkups and consultation. The family will incur medical, airline, lodging, and many other expenses during these times away from home. Throughout all of this, we cannot forget about the needs of Dean’s older sister Grace. She will be at home in Oklahoma with Shelby’s fiancé, but needs to travel to see her mother and Dean during this highly stressful time.

Please consider donating to Dean and his family to help him in changing to course of his future. Help Dean to be able to experience all the joys of being a kid that includes ALL the ice cream flavors he dreams to eat, not just those with no protein! Please help in giving this family a sense of normalcy back.

More details regarding Urea Cycle Disorders:

A Urea Cycle Disorder (UCD) is an inherited disease that affects how the body removes the waste made from breaking down protein.

Everyone needs protein. When a person eats food that contains protein, the body breaks it down into amino acids (the building blocks of protein that are used by the body for growth and tissue repair) and uses only what it needs. It changes the rest into nitrogen, which must then be removed by the body.

In a healthy person, the liver supplies several enzymes to change nitrogen into urea, which is then removed from the body in urine. This process is called the urea cycle. The liver in a person with a urea cycle disorder is missing an enzyme necessary to convert nitrogen into urea. As a result, ammonia, a highly toxic substance, builds up in the bloodstream and is not removed from the body. Often, the symptoms of high ammonia levels are invisible and undetected, until the levels are dangerously high. Untreated, these high amounts of ammonia can cause brain damage, coma, and eventually death.