July 21, 2014
EDMONTON – Doctors at Stollery Children’s Hospital in Edmonton, together with University of Alberta researchers, have successfully completed three pediatric heart transplants by using an anti-rejection therapy similar to one sometimes used in adults awaiting kidney transplants.
The Stollery is the first and only academic health centre in Canada to successfully use the treatment in children in preparation for transplant, which combines a common agent used to treat cancer with a blood plasma therapy that slows a patient’s immune response. Together, the two therapies prevent the donor organ from being rejected.
“From our perspective this has become our standard approach for these complicated patients,” says Dr. Simon Urschel, Pediatric Cardiologist, Clinical Director Pediatric Cardiac Transplantation, Stollery Children’s Hospital, University of Alberta and researcher at UAlberta’s medical school.
In December 2011, six-month-old Abigail Fraser became the first patient in Canada to receive the innovative treatment, without which she would never have been a suitable recipient for a heart transplant. Today she’s a happy, healthy three-year-old with a new heart and no serious health issues.
Abigail’s mother, Nancy Fraser, learned during an ultrasound that her daughter would be born with Hypoplastic Left Heart Syndrome (HLHS), a rare congenital heart defect in which the aorta and left ventricle of the heart are severely underdeveloped and cannot pump blood properly.
Approximately one per cent of newborns are born with a congenital heart defect; HLHS makes up only one to three per cent of those cases.
The current standard of care for patients with HLHS involves three life-enhancing surgeries staged from within days of birth until age four. About 75 per cent of children successfully reach the third stage and can live for decades.
Nancy and her husband Jamie were familiar with the treatment option, having been through the surgeries with their older daughter Hailey, who was also born with HLHS. Abigail, however, experienced complications following her first surgery.
Following a post-operative cardiac arrest and days spent on a heart-lung machine, Abigail was eventually able to go home. Further complications arose and her heart was too weak to undergo the second and third stages of the HLHS repair. At three months of age she was transferred to the Stollery Children’s Hospital where she was assessed for a heart transplant.
Unfortunately, Abigail’s immune system had developed antibodies to donor tissue used to repair her aorta during her first heart surgery. If a donor heart became available, her body would likely reject it immediately.
To help suppress her natural immune response, a team of doctors at the Stollery and University of Alberta developed the new immunosuppression protocol. They combined cancer treatment drug Rituximab with intravenous immunoglobulin (IVIG) to slow down her production of antibodies that would reject a donor organ while providing enough antibodies to fight off infection.
“We needed to get rid of Abigail’s antibodies. Used in combination, the Rituximab would prevent her from developing new antibody-producing cells while IVIG would help slow down her own immune response,” says Dr. Urschel. “We needed to desensitize her so that she could eventually receive a donor organ without the risk of immediate rejection.”
Abigail responded to the immunosuppressive therapy, and when a donor organ became available two months later, she was a suitable candidate for transplant.
“Pediatric organs don’t come available very often, so to hear your daughter would reject most organs was devastating,” says Nancy. “They did their best to save her heart, but eventually this treatment became our only option. We are so grateful to the family who made the difficult decision to donate following the loss of their child, and so happy this treatment was available so Abigail could accept their gift.”
Abigail remained on IVIG for six months following transplant until she started producing sufficient antibodies for her protection.
Abigail celebrated her third birthday on June 7, 2014. Since her transplant in December 2011, two other pediatric cardiac patients have successfully been transplanted following the Stollery anti-rejection therapy protocol. Other prospective candidates under this protocol are also awaiting transplant.
Alberta Health Services is the provincial health authority responsible for planning and delivering health supports and services for more than four million adults and children living in Alberta. Its mission is to provide a patient-focused, quality health system that is accessible and sustainable for all Albertans.
UAlberta’s medical school is committed to advancing health through teaching, research and patient care.
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