Successful Domino Liver Transplants in Children with Rare Disorder Allow Them to be Liver Donors as well as Recipients

In a new milestone for the MedStar Georgetown Transplant Institute, transplant teams have successfully completed domino liver transplants from young people suffering from a rare genetic disorder to adults on the liver transplant list who could safely benefit from them.

March 31, 2015

A Liver that is Toxic to One Person Can Save the Life of Another

(Washington, D.C.) In a new milestone for the MedStar Georgetown Transplant Institute, transplant teams have successfully completed domino liver transplants from young people suffering from a rare genetic disorder to adults on the liver transplant list who could safely benefit from them.  The liver that contributes to life-threatening health problems in one person can be used to save the life of another.

Andrea Scott of Maryland knew from the time her son Korey, now six, was just a few days old that he suffered from a rare, life-threatening metabolic disorder that would rule his diet and his ability to survive. Maple Syrup Urine Disease (MSUD) happens in 1 in 185,000 people in the United States and means kids run the risk of coma, seizures and brain damage if they eat normal amounts of protein. Infants cannot have breast milk. Patients are strictly limited in their protein intake. For Korey it was just 10 grams per day.

“We found out about MSUD from his newborn screenings. Since then we’ve had to watch Korey’s diet so carefully. The daily challenge is balancing low protein food with enough calories for him to have energy and fight the hunger.  It’s a very tough way to live.”

Named for the sweet smell of the urine in people with MSUD, having the genetic disorder means the body cannot break down certain proteins or amino acids made by the muscles, brain and liver. Those proteins build up in the blood and can cause seizures, brain damage and death. The only cure is a new liver; a liver transplant.

“For a child with MSUD, the transplant is really to protect their brain,” said Nada Yazigi, MD, pediatric transplant specialist at MedStar Georgetown. “For them, eating something like a hamburger can cause a metabolic crisis which can lead to severe brain injury. After a liver transplant they take anti-rejection medication but they feel very normal and can eat normally. ”

“The amazing thing is that while the liver is toxic in the person with MSUD, it can be a life saver to another person who doesn’t have the disorder,” said Thomas Fishbein, MD, executive director of the MedStar Georgetown Transplant Institute. "In that recipient, the rest of their body can compensate so that the metabolic deficiency of the liver has no effect on them. In many cases, we are able to give these livers to recipients who desperately need the transplant, but haven’t reached the top of the liver transplant waiting list yet.”

Isobel Short, the mother of six boys from Virginia Beach is one of those people.

“I had cirrhosis of the liver and was pretty much confined to my house,” said Isobel. “I went from my usual 118 pounds to just 87 pounds. I was huffing and puffing from doing the smallest things and I was tired all the time. I knew I would die without a new liver.”

Then, in late January Isobel’s husband Jerry got the call at work that a liver was available from a donor with MSUD. Isobel and Jerry had discussed this possibility at length with her transplant team.

“He called me at home and said, ‘Belle this it.’ I was just wearing my jeans and boots. I had nothing packed but we were out the door in 35 minutes.”

Korey Scott and Isabel Short share a moment after meeting for the first time in February. Isobel received Korey’ liver moments after he had a liver transplant thanks to a deceased donor.
Korey Scott and Isabel Short share a moment after meeting for the first time in February. Isobel received Korey’ liver moments after he had a liver transplant thanks to a deceased donor.

As part of a twelve hour, tightly choreographed series of operations, Isobel would receive Korey Scott’s liver while Korey received a liver from a deceased donor.

Each step of the surgery had to be precisely planned because on that very same day 14 year old Quadejah Harris of Maryland, also suffering with MSUD found out that she would receive a new liver, thanks to a deceased donor.  Quadejah’s liver could then be used to save the life of another.

“She kept saying how she wanted to help somebody else,” said Quadejah’s mother Alicia Brooks. “It was her choice to go through with this liver transplant and to donate her liver to someone.”

That ‘someone’ was Jeremy Dick, 49, an IT consultant from Northern Virginia and a member of the District of Columbia National Guard.

Jeremy was diagnosed with an auto-immune disease called Primary Sclerosing Cholangitis (PSC) about a year ago. He had lost 30 pounds; his skin had turned yellow due to the liver’s inability to function normally.

“I had never heard of Maple Syrup Urine Disease before the doctors explained it to me. I think it’s amazing that the only thing to cure people with MSUD, getting rid of their liver, could be a treatment for me,” said Jeremy.

The MGTI performed its first domino liver transplant in a patient with MSUD in November 2014.  But to successfully complete two domino liver transplants in the same day took a team of more than 100 people.  It had never been done before in the world.

In addition to the two domino liver transplants performed that day, the teams completed four additional liver transplants and two kidney transplants all within a 48- hour period.

Less than two weeks after his transplant, feeling better and his color returning to normal, Jeremy and Quadejah got the chance to meet each other at the MGTI.

“I was nervous to meet him at first,” said Quadejah. “But we talked for about 20 minutes and it was amazing. I’m just so happy that I could help someone who was so sick.”

Jeremy Dick and Quadejah Harris meet for the first time at the MedStar Georgetown Transplant Institute two weeks after he received the teen’s liver and she received a new liver from a deceased donor.
Jeremy Dick and Quadejah Harris meet for the first time at the MedStar Georgetown Transplant Institute two weeks after he received the teen’s liver and she received a new liver from a deceased donor.

“Meeting Quadejah, my living donor, gave me the chance to thank her for her sacrifice,” said Jeremy.  “It was also fun to meet both her and her mother and talk a little about our similar challenges – walking hunched over, the telltale transplant scars, and our similar road to recovery. I appreciate hearing from someone with a more youthful perspective about what she’s looking forward to in her life and knowing that she too was grateful for the donation she received.”

Days later, Andrea and Isobel, both the mothers of sons, would meet through tears of emotion and gratitude.

“I cried when I learned that Korey’s liver was working in someone else,” said Andrea. “It’s just remarkable. We’re now looking forward because his best quality of life is yet to come.”

“This beautiful little boy has saved not only my life, he has brightened the lives of everyone in my family,” said Isobel. I can’t express how much I’ve been through and how much this means to me. My liver disease has been a problem for my entire family.  I’m over the moon that I have a second chance at life and I am humbled by this little boy and his family. I also think it’s pretty neat that I have the liver of someone who is still alive."

About MedStar Georgetown University Hospital

MedStar Georgetown University Hospital is a not-for-profit, acute-care teaching and research hospital with 609 beds located in Northwest Washington, D.C. Founded in the Jesuit principle of cura personalis—caring for the whole person—MedStar Georgetown is committed to offering a variety of innovative diagnostic and treatment options within a trusting and compassionate environment.

MedStar Georgetown’s centers of excellence include neurosciences, transplant, cancer and gastroenterology. Along with Magnet® nurses, internationally recognized physicians, advanced research and cutting-edge technologies, MedStar Georgetown’s healthcare professionals have a reputation for medical excellence and leadership. MedStar Georgetown University Hospital—Knowledge and Compassion Focused on You.

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