Seth Hollander, MD, Pediatric Cardiologist, Medical Director, Pediatric Heart Transplantation at Lucile Packard Children’s Hospital Stanford talks about Justin’s life with his disease.
Interview conducted by Ivanhoe Broadcast News in September 2019.
We’re going to start at the beginning of Justin’s journey. What was wrong with him?
HOLLANDER: Justin was born with a blood disease called Loeffler’s syndrome in which his body makes too much of a certain type of white blood cell called eosinophils. And one of the many problems with having this disease is that these cells can build up in the heart and cause the heart not to function well. Loffler syndrome is another name for Hypereosinophilic Syndrome.
How serious can this disease syndrome be?
HOLLANDER: Loffler syndrome is a very serious disease. It can be life threatening and can cause problems in lots of different parts of the body. For Justin, it caused many problems including for his heart not to function well. He had some of his feeds given to him through a feeding tube which he had from the time he was young. He had to take medications to slow down the production of these cells, but over time the cells built up to the point where he needed multiple surgeries to keep his heart functioning. And when that no longer was suitable for him he was placed on the heart transplant list.
What were the symptoms to start with?
HOLLANDER: There are a number of symptoms that Loffler’s can produce that would be found either by a parent or a doctor. In Justin’s case, there was a noticeable weakening of his heart that we were able to see on a heart ultrasound called an echocardiogram. So it was very clear early on that his heart was suffering as a result of this disease. And when your heart’s not functioning well, we call that heart failure. When you have heart failure, a number of things can happen. You can tire easily. You can breathe fast. You can have trouble eating and have trouble gaining weight. These would be typical symptoms of heart failure and things that Justin had to fight basically throughout his childhood.
Before the heart transplant and surgeries that you guys treated the blood disorder with some type of drug. Tell me about that.
HOLLANDER: Justin was initially treated with medications essentially designed to slow the production of these cells. These medications are used for people who have different type of cancers because the cancer drugs are really good at slowing down the body’s inappropriate production of cells. He was on these drugs for a long time, but they did not slow down the production of the eosinophils – he did not slow down the production of the eosinophils sufficiently such that they built up in his heart and over time he developed heart failure. So we decided a different approach, which was to treat his malfunctioning heart with a series of surgeries to make his heart not have to work as hard. And he had a couple of surgeries which sustained him over time, but we knew from a pretty early age that at some point he was going to have to have a heart transplant.
His mom said he had about 40 percent function.
HOLLANDER: His heart was not squeezing as hard as it should have been.
The PACT team – tell me about the innovative approach you guys take.
HOLLANDER: Sure. Here at Lucile Packard Children’s Hospital we have a group of providers, including doctors, nurses, pharmacists, psychologists, and social workers, who make up the Pediatric Advanced Cardiac Therapies or PACT team. We specialize in diseases that cause the heart to be weakened, whether these be genetic diseases, blood disorders or as a result of congenital heart disease. And we provide medical care for these patients. When medical care is not enough, we are able to implant artificial heart pumps called ventricular assist devices, which help the heart function. We often use them as a bridge to heart transplantation, which is when a person’s diseased heart is replaced with the heart of a donor. And here at Stanford, the PACT team provides care to these patients at all three phases – the medical phase, the mechanical support or mechanical pump phase, as well as the heart transplant phase. And we are one of the largest ventricular assist device programs and one of the largest heart transplant programs as well.
You treated Justin his whole life basically right?
HOLLANDER: We started taking care of Justin essentially when his medical condition got to the point where his original providers weren’t able to care for him. He required subspecialty level care that we can provide here that other hospitals can’t provide.
Tell me about what it was like for him health wise – the symptoms he was having.
HOLLANDER: Growing up, Justin had a hard time doing a lot of the things normal kids would do. For example he needed a feeding tube because he quite frankly just didn’t have the energy to eat enough calories to grow and develop the way any other child would. And as a result of his heart failure, he just had trouble summoning up the amount of energy that a typical kid would typically have to go through school and play and grow and have fun. Despite his condition, Justin actually has been remarkably energetic and positive and has led a relatively normal life, but always sort of struggling. The way I like to think about it is where the rest of us are walking around on flat ground, life for Justin was always uphill. And he’s able to walk uphill because he’s so strong but it’s that much harder for him because prior to his transplant his heart wasn’t pumping as hard as it should.
When Justin became a teenager is when we really started to see that his heart really was failing. He developed rhythm problems. He really wasn’t able to keep up with his energy level. And so when he was 15 years old we placed him on the heart transplant list.
He was only on the heart transplant list for 17 days before we found a donor. He had his heart transplant on April 27.
So most people have to wait.
HOLLANDER: A typical patient waiting for a heart transplant can wait anywhere from a few months to five or six years depending on their size and how easy they are to find a donor. Someone like Justin would typically have waited anywhere from six to nine months, but he got very lucky and we were able to find a donor within just a few weeks.
At the point when he needed the transplant, what was his condition? How grave was it?
HOLLANDER: When Justin came in, he was still out of the hospital fortunately. So he was living with heart failure. Many of our patients are in the hospital or they’re on machine pumps – these ventricular assist devices. Justin didn’t require any of that, but his heart failure was severe enough that his ability to do things that he would normally do was limited.
How will that affect him moving forward?
HOLLANDER: Loffler’s is a lifelong condition, and so his body will continue to produce these abnormal cells and he will continue to require medications to try to suppress his body’s natural tendency to produce these cells. And so in addition to his heart transplant we are continuing him on his medical therapies so as to best to prevent this happening in his new heart. Whether or not his new heart will suffer from the effects of the Loffler’s syndrome – we’ll just have to see over time. Loffler’s syndrome is a rare reason to get a heart transplant in children. Out of the over 400 transplants we’ve done here, he is the only child with Loffler syndrome that we’ve had to transplant. And so quite frankly we’re just going to have to see how he does.
Can it attack other organs?
HOLLANDER: Yes. Loffler syndrome can affect other organs in the body.
Has it with Justin?
HOLLANDER: Justin fortunately is doing quite well. He’s developing nicely. He swims and he runs and he does a lot of active things particularly since his transplant and the rest of the organs in his body for the most part are doing just fine.
I understand it’s a rare disorder. So how rare?
HOLLANDER: I actually don’t know exactly how rare it is since he’s the only patient we have in our practice who has it but it’s quite rare.
How is he today?
HOLLANDER: Justin is doing great. He’s an ideal post transplant patient. He takes all of his medications. He has not had any heart rejection. And his new heart is functioning very well. He is active. He is exercising. He is going to school and as a lot of people know he’s actually written a heart healthy cookbook to share with other children and families who have heart disease. So Justin is outstanding. If you were to see him on the street you wouldn’t know that he had any health condition at all.
Considering how he’s had to deal with such a battle since he was a little baby, how impressed are you that he’s basically taken charge of his health now?
HOLLANDER: Justin is an extraordinarily mature and motivated young man. He’s always been this way. And so rather than letting his heart disease get him down, he has always done his best to live a normal life. And his parents have always encouraged him to live a normal life. And when you think about it, it’s extraordinary to think that a child who was on a feeding tube for a good portion of his childhood is now essentially a chef who not only loves to eat but loves to cook and share his cooking concoctions with other people. He also does not show any resentment at all for the suffering he’s gone through or the need for the heart transplant. Quite the opposite actually. He is a wonderful representative of the miracle of transplantation and a wonderful advocate for organ donation, which is an extraordinarily lifesaving choice that any person can make. And so Justin is quite honestly just an inspiration to us all to be very honest about it because he just shows not only how beneficial transplantation is for people, but he encourages other people to make that extraordinarily generous choice for other children. And that’s something without which we can’t do our jobs here.
Lastly just to understand the timeline, how did he just have two surgeries?
HOLLANDER: Justin had two surgeries prior to his transplant and then his transplant was his third surgery.
His first surgery occurred when he was a baby and it was something called a Glenn surgery which takes some of the workload off of the heart by diverting blood away from the heart. And then he had a second surgery which was essentially designed to do the same thing. So although it didn’t make his heart condition better, it was designed to really to allow his heart not to work as hard by having blood essentially detour around the heart. We know that these procedures which we perform in children who have heart defects can be lifesaving, but ultimately their benefit is temporary. And children who have these surgeries often will need heart transplants either as older children or as young adults.
Most children who require heart transplants require heart transplants because they have either a genetic condition that makes their heart weak or because they have a congenital heart defect, which did not respond well to surgery. That is the reason that the overwhelming majority of children require heart transplants. What makes Justin’s case unique is that his heart essentially was normal when he was born, but he also had this blood disorder in which cells in his bloodstream rendered his heart unable to pump effectively. And when medications failed to control the blood disorder, we opted for a more creative approach, which was to do a heart transplant and give him a new heart, which was unaffected by these cells.
END OF INTERVIEW
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