First heart-liver transplant at UTSW saves singer’s life
A genetic disorder dealt singer and songwriter Andrea Joyner a serious blow in 2004. She received a diagnosis of juvenile hemochromatosis, which can lead to multiorgan dysfunction before the age of 30. The clock was ticking, and by late 2016 she faced imminent death without a rare heart-liver transplant.
But thanks to the exceptional care of multiple UT Southwestern transplant teams, the 40-year-old musician is now back to writing songs and living life to its fullest. The surgery that saved her also marked a historic milestone as the first heart-liver transplant performed at UT Southwestern.
“She’s done very well considering how close to death she was – within hours, most likely,” said Dr. Malcolm MacConmara, who led the liver transplant team through the September 2016 surgery.
With the double transplant, UT Southwestern joins the ranks of only four other Texas medical centers – including affiliated hospital Children’s Medical Center Dallas – that have performed the complicated heart-liver surgery, in which a newly implanted heart must withstand the stress of a liver transplant and the toxins released from the donor organ.
In 2006, Children’s completed a heart-liver transplant on a girl whose family came from Miami for treatment. The treatment team for that case included Dr. Sarah Blumenschein, Associate Professor of Pediatrics at UT Southwestern, and transplant surgeons Dr. Kristine Guleserian and Dr. Jay Roden, former faculty members.
More than 200 such transplants have been done in the U.S. since Feb. 14, 1984, when a 6-year-old patient from UT Southwestern traveled to Children’s Hospital of Pittsburgh to receive the world’s first heart-liver transplant. That patient, Stormie Jones, lived six more years before her body rejected the transplanted heart.
A long road to diagnosis
Miss Joyner, from Wills Point, 50 miles east of Dallas, is the lead vocalist in the country duo blacktopGypsy. For years, she struggled with health issues. After five years and trips to multiple doctors, Miss Joyner’s condition was identified in 2004 as the rare genetic disorder hemochromatosis, which affects about 1 million people in the U.S. In this disorder, the liver fails to prevent excess iron from entering the bloodstream, causing the body to accumulate toxic levels from food.
Over the next 12 years, the accumulating iron quietly ravaged Miss Joyner’s heart and caused cirrhosis of her liver.
In March 2016, on her way home from a multiple-city tour, Miss Joyner collapsed, completely exhausted and with pain in her chest and stomach.
She then began a series of emergency room visits, starting at a small hospital in Sunnyvale, Texas, where a doctor who knew physicians at UT Southwestern and their affiliation with Parkland Hospital recommended she go there.
The musician spent 10 days at Parkland in June. Then, on July 28, with her heart failing, she returned. Days later, in critical condition and in need of a heart transplant – and possibly a liver transplant as well – Miss Joyner was transferred to UT Southwestern’s William P. Clements Jr. University Hospital.
Heart-liver transplants are “quite complicated. They’re not offered by a lot of institutions,” explained Dr. Matthias Peltz, Associate Professor of Cardiovascular and Thoracic Surgery, who led the heart transplant team at Clements University Hospital.
“It requires having the expertise of both sets of surgeons, clinicians, nurses, perfusionists – everyone involved for each organ – working together,” said Dr. MacConmara, Assistant Professor of Surgery.
On Aug. 25, Miss Joyner went on waitlists for both organs. Within days, however, her condition deteriorated to the point where keeping her alive long enough to get any organ became the issue. By Sept. 4, her heart had weakened to the point that she required placement on an external heart-lung machine to stay alive.
Fortunately, on Sept. 5, a heart and a liver became available. The surgery was scheduled the next day, with the heart transplant completed first.
With her second chance, Miss Joyner said she plans to continue her music – and to speak out to educate people about her rare disease, which went undiagnosed for so long.
Miss Joyner also thinks a lot about the organ donor who died and the family that donor left behind. She said she’d like to meet them one day. “I can’t thank them enough. They saved me.”
Orchestrating a rare heart-liver transplant
Prior to the transplant
Patient is connected to a heart-lung bypass machine, which keeps her alive until donated organs are available. This machine does the work of her heart and lungs.
Separate UT Southwestern teams fly out of Dallas Love Field to retrieve the donor’s heart and liver.
Three-member heart surgery team begins the heart transplant operation.
Donated heart arrives; surgeons begin removing patient’s old heart.
The new heart is in place.
The new liver arrives.
Four-member liver team begins removing the patient’s damaged liver.
The new liver is in place.