Transplant

In 2014, the Seattle Children’s Research Institute will implement life-saving projects, begin new studies to keep children safe and continue searching for ways to prevent and cure diseases that threaten some of our youngest patients. We are celebrating the New Year by highlighting some of the work that has researchers excited about 2014.

Looking forward to saving lives

Evelyn Tomlin was born April 16, 2013. Doctors in Oregon did not know that she had SCID until she was 3 months old.

On Jan. 1, years of work by Children’s research and clinical staff came to fruition when a test for Severe Combined Immunodeficiency (SCID) was added to Washington’s newborn screening panel for all babies born in our state. Known as the “bubble boy” disease, SCID is a group of inherited disorders that cause severe abnormalities of the immune system. Babies with this disease often show no symptoms at birth, but after a few months they are unable to fight infections. Common illnesses, such as the flu or an ear infection, can be life-threatening for a child with SCID.

“Babies with SCID benefit from their mom’s immune system at birth, but once that goes away they have very little ability to defend themselves,” said Troy Torgerson, MD, director of Children’s Immunology Diagnostic Lab.

If caught early, more than 90 percent of SCID cases can be cured with a bone marrow transplant or gene therapy. But once a baby contracts an infection, survival rates drop to 50 to 60 percent.

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The faster medical research moves the more quickly cures can be found for countless children’s diseases. But one of the greatest delays researchers face when trying to solve medical problems is finding enough patients to study.

“Enrolling patients in a clinical trial to study a rare condition could take years,” said Mark Del Beccaro, MD, a researcher and vice president of medical affairs at Seattle Children’s.

But now a federally funded non-profit has awarded Children’s and seven other pediatric hospitals funding to create a national network of patient data with the goal of speeding up medical research and improving patient care. Read full post »

Montana teen becomes first patient at Seattle Children’s to receive the HeartMate II ventricular assist device (VAD) and a heart transplant while being supported with an implantable VAD. He is also the first patient at any pediatric hospital in the Pacific Northwest to leave the hospital with a VAD while waiting for a heart transplant. 

The HeartMate II Ventricular Assist Device

Adam Kingsbury went to see his family doctor for what he thought was a bad cold. It was there that Adam, a 16-year-old from Stevensville, Mont., was diagnosed with asthma and sent home with a prescription for an inhaler and orders to take it easy.

After a few weeks, Adam’s symptoms didn’t improve. He was having trouble breathing so his mom took him back to the doctor where it was discovered that Adam had an abnormal heart rhythm. At age 5, Adam was diagnosed with myotubular myopathy, a condition that makes the heart muscle weak. Because of this, his care team knew it was extremely important to find out what was causing the abnormal heart rhythm right away.

The clinic called Seattle Children’s Montana-based pediatric cardiologist Bruce Hardy, MD to examine Adam’s heart. An echocardiogram revealed that Adam was suffering from cardiomyopathy, a condition which causes the heart to lose its pumping strength. Adam’s heart was failing and he would likely need a heart transplant. Within three hours of seeing Dr. Hardy, Adam and his mom, Kate, were on a medical transport plane to Children’s main campus in Seattle where Adam’s condition could be best treated. Read full post »

This past weekend, baby Poppy Dahl from Belgrade, Mont., celebrated her 6-month birthday. This was a major milestone day for Poppy and her family – Poppy survived and is now home with her family after fighting for her life due to a life-threatening heart condition, hypoplastic left heart syndrome. She was diagnosed with the condition before she was born.

A program by Seattle’s KOMO 4 News which aired on Poppy’s half-year birthday, documents Poppy’s story of survival as her family and the teams at Seattle Children’s Hospital and UW Medicine do all they can to give Poppy a fighting chance.

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With two high-profile pediatric transplant cases making headlines recently, many people are paying closer attention to organ donation. Simon Horslen, MB ChB, medical director for liver and intestinal transplantation at Seattle Children’s Hospital, hopes the current debate will be a good boost for organ donation. The real issue, he says, is that there are not enough donors for everyone who needs an organ.

Horslen has cared for transplant patients for years, and he sympathizes with parents who are fighting for their kids to have a chance at life. But he notes that organ donation guidelines exist for a reason.

“It’s right that the families of these kids do everything they can to advocate for their children,” says Horslen. “But it’s a bad precedent if every time someone gets to the bottom of the list or is going to struggle to get transplanted, that they go to the courts to challenge it.” Read full post »

In a northern California suburb in 1999, Kaitlin Burns was born very sick, that much was certain. She was extremely lethargic, vomited non-stop and soon wouldn’t eat anything. When her family finally received a diagnosis two weeks after her birth, the news was devastating.

Kaitlin was diagnosed with propionic acidemia, a rare, inherited metabolic disorder that affects about one in 100,000 in the United States. Propionic acidemia prevents the body from processing protein properly, leading to an abnormal buildup of a group of acids known as organic acids. Abnormal levels of organic acids in the blood, urine and tissues can be toxic and can cause serious health problems.

Michelle Burns, Kaitlin’s mother, recalls how the local hospital at the time was their second home. “During the first year of her life, I can’t even count on my fingers and toes how many trips we made,” she explains.

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Evelyn Sherman had a kidney transplant this past Halloween, just 10 days before she turned three years old. Her dad, Keith, was her donor. In honor of Donate Life Month, Evelyn’s mom, Julianne, reflects on their journey as they near the six-month anniversary of the transplant.

Getting the diagnosis

Evelyn was nine months old when she started falling off the chart. I thought my milk supply had dropped off so we were just sort of waiting it out, but she was still falling off the curve. Then we tried to beef up her diet and that wasn’t working.

 By the time we got the diagnosis when she was 15 months old, we had made a tour of Children’s. She had some other physical manifestations of something being wrong. She had a sixth toe. She had wine stains on her skin. So we’d been to orthopedics to have her toe removed. And we’d been to dermatology. We felt like we were ending our tour when we got to nephrology. It was our last stop.

Evelyn was diagnosed with renal dysplasia, meaning that the kidney developed incorrectly in the beginning. She also has kidney reflux and that means that the urine is going back up into the kidneys from the bladder. It often causes a kidney infection, but for whatever reason, she didn’t really manifest that.

When the doctor delivered the news that there was something wrong with her kidney, I remember thinking, “I feel like you’re saying something really important to me and it’s just not registering because you have got to be talking about somebody else.” I couldn’t comprehend what he was saying, that I had a critically ill child.

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Heart transplant recipients (clockwise, from top left): Brooke Naab, Hannah Campbell, Batul Al-Salami and AJ Baird.

In one five-day span this September, four patients – one teenager and three critically ill infants – received life-saving heart transplants at Seattle Children’s.

For four families, the week meant the end of an agonizing wait and the start of a new life.

To perform four transplants in five days is very unusual, says Dr. Lester Permut, heart surgeon and interim chief of Cardiothoracic Surgery at Seattle Children’s. “But these are situations we train for.”

The first notification came at the end of a busy Friday: A donor heart was available for 6-month-old AJ Baird, who had spent half his short life waiting for a heart in Children’s Cardiac Intensive Care Unit (CICU).

A team from Children’s – two heart surgeons and a transplant nurse – set out immediately to get the donor heart while a team led by Dr. Permut and another heart surgeon, Dr. Michael McMullan, readied AJ at Children’s. The transplant surgery started early on Saturday, Sept. 22.

The team was finishing AJ’s surgery when the beeper went off again: a heart was available for 14-year-old Batul Al-Salami, who has been followed by the Heart Center since birth.

Then it happened again, and again, until Wednesday, Sept. 26. By then, the team had completed a total of four heart transplants – more than Children’s had ever done in a single week.

The cluster of transplants made for an exhilarating week.

“When a heart comes up for one of our patients, we’re excited about it and the energy from that sustains us,” says Dr. Permut, who traded the role of lead and assistant surgeon with Dr. McMullan for all four surgeries.

“It was a tour de force for a big group of people,” adds Dr. McMullan. “It took an incredible team and a very good system to pull this off.”

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Despite being born premature at 30-weeks gestation, Rachel Robbins’ new baby boy Ethan was an extremely alert and cheerful newborn. But at three days old, doctors first noticed that something was not right with Ethan. He had a heart murmur. The cause, ventricular septal defect (VSD), a hole in his septum located in the middle of his heart. Due to the hole, when his heart would contract, Ethan’s aorta would become so blocked that blood could not get out of his left ventricle causing pressure on his lungs.

It was only one week later that Ethan developed congestive heart failure.  By the time he was six weeks old his condition had worsened so that doctors diagnosed him with hypertropic cardiomyopathy, a genetic condition that may have been inherited from Rachel that caused the left ventricle of Ethan’s heart to enlarge and thicken in utero.

“He began to have difficulty breathing, he was sweating, and had a greyish-blueish color in his skin,” said Rachel. “He was also sleeping a lot more than he should have been, and it appeared he was using most of his energy to breathe. I knew something was not right.”
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Issue

Good news! Today, Facebook CEO, Mark Zuckerberg, announced a plan to encourage Facebook users to indicate their organ donor status on their Facebook pages. Under the plan, Facebook members can register to become organ donors via links on Facebook to online state registries. Given the company’s social networking muscle and global reach, some organ donation experts are speculating Facebook’s plan could radically increase the number of registered organ donors in the coming months.

“Transplantation is the best solution to end stage organ failure. This is a historic moment for organ donation and awareness. Organ shortage is a global public health problem. Mark Zuckerberg’s initiative, with Facebook’s global impact, will tremendously increase awareness which will result in more lives being saved, ” said André A.S. Dick, MD, MPH, FACS, Assistant Professor of Surgery in the Division of Transplantation at Seattle Children’s Hospital. “As of today, there are 114,000 wait list candidates in the U. S. and the gap between donors and recipients is increasing every year. The American Society of Transplant Surgeons and the transplant community support the campaign and hope it goes viral.”

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