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Fri, May 8 11:42am · The Fontan: Past, Present, and Future with Dr. Frank Cetta in HLHS

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We often get questions about issues facing post-Fontan individuals. To learn more about the Fontan surgery, as well as the research being done by the Todd and Karen Wanek Family Program for HLHS at Mayo Clinic, I talked to Dr. Frank Cetta. Along with being a pediatric and adult congenital cardiologist, he’s also the lead investigator on the world’s largest single-center database of Fontan patients. We discussed the nuts and bolts of the procedure, tips for preventing common complications, and hope for the future.

 


Q: For people new to the heart community or unfamiliar with congenital heart disease (CHD), can you start by giving us an overview of the Stage III surgery, or the Fontan, for HLHS patients?

A: In single ventricle CHDs, what happens before Fontan at the tubeStage II surgery, or the Glenn, is that the “blue blood” (blood that’s already circulated around the body and now has no oxygen left in it) from the arms, head, and neck gets connected directly into the pulmonary artery (PA) to go into the lungs. In a small baby, that’s more than half of the blood flow that normally goes to the right side of the heart. At the Fontan, we bring the rest of the blood from the lower part of the body, intestines, legs, everything below the diaphragm, directly into the PA.

In a patient with a single ventricle, you only have one ventricle to work with, so the ventricle is built to pump the blood to the body and not the lungs. The Fontan finishes off a complete bypass of the heart by putting in a big tube so that all the “blue blood” in the veins never really goes through the heart – it just goes from the inferior vena cava (IVC), through some fancy plumbing, and directly into the lungs. [see Image 1]

 

The procedure was first done in the late 1960’s, and there have been many variations over time. But something we’ve found over the years is that the Fontan circulation is not “normal” circulation. It has many effects on other organs in the body.

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Most Fontan patients will get some form of Fontan-Associated Liver Disease (FALD) at some point, although we’re now seeing that some may even have it before the Fontan operation. The reason for FALD is what we call “elevated central venous pressure.” [see Image 2] The pressure in the Fontan tube is a lot higher than normal. In a normal IVC, the pressure is about half of what it is in a really good Fontan patient. So the best Fontan patient still has pressure in that tube above the liver that’s twice as high as normal.

 

Image 3 is a good example. Look at the bottom where congestionthere’s a big, black, 3-prong thing. That’s dye showing the blood flow going from the liver into the IVC. See how big those vessels are? Then you move up into the Fontan tube. See how small that conduit is? Then you get above it — see how big the pulmonary arteries are?

It looks like three lanes of traffic trying to merge into one.

No wonder there’s congestion! You don’t need to be a rocket scientist to figure out why the liver is backed up. It’s a bottleneck right there. So the liver gets congested and blood flow starts backing up in the liver. That’s the road to Fontan-Associated Liver Disease (FALD). The blood flow back-up is called “hepatic congestion” which leads to fibrosis, or scarring. That can lead to cirrhosis which is irreversible damage to the liver. Some patients progress to what’s called “hepatocellular carcinoma,” or liver cancer, although that’s very rare (< 1%).

 

Q: What are some things that post-Fontan individuals can do to take care of themselves?

A: If you have a Fontan and you have liver disease, you don’t want to be drinking alcohol all the time because alcohol’s bad for the liver. That doesn’t mean you can’t have a glass of wine with dinner, it means no binge drinking, regular drinking, or excessive alcohol.

You also want to be immunized against the hepatitis virus because that affects the liver, and you can also have bloodwork done to make sure the vaccines were effective. There are some drugs, including Tylenol, that can be bad for the liver and you should avoid excessive amounts.

Sleep apnea, which causes people to snore and stop breathing at night, is another issue. That can elevate the pressures in your lungs, and that pressure gets transmitted back down through the Fontan conduit, and puts even higher pressure on the liver. So Fontan patients with sleep apnea should be treated for that.

Also, being obese and having a Fontan is a bad combination. Exercise increases Fontan longevity, so you want to be lean and get good exercise. It improves lifestyle, longevity, and reduces some of the liver and other organ problems. Studies have shown that Fontan patients who exercise more have better blood flow into the lungs. It helps the whole body and is really essential for Fontan patients.

 

Q: Are there additional factors that make people with CHDs more prone to obesity?

A: No. Part of it is that we, as physicians, told their parents when they were little to keep “little Johnny” at home. We didn’t want them to bang into people or do activities that might put too much strain on the heart. It was all well-intentioned, but we have exercise guidelines where certain activities and sports are not allowed – and a lot of that is not based on any data

There are certain things that are common sense. For example, if you have a mechanical valve and you’re on a blood thinner, you shouldn’t be into knife throwing or carpentry where you might cut your hands all the time. But you could still play basketball and be careful. For decades, we told parents to sit on these kids and not let them do anything – and that’s probably the biggest thing we’re trying to undo today.

Obviously all patients are different, but we have definitely erred on the conservative side. It’s the same with women getting pregnant post-Fontan. We used to say that no one should do it. While we do see risks to the mother and fetus with loss and pre-term delivery, there are some women who are able to become pregnant and keep themselves and the baby healthy. Prior to pregnancy, a thorough evaluation needs to determine risks for the individual patient so they can make an informed decision. (To learn more, read our earlier blog post.)

 

Q: So those are things that the patient can do. What can healthcare providers do?

A: When we look at Image 3 from earlier, it’s pretty obvious – we need to make that tube bigger. That tube was put in when this patient was three years old. Now they’re 23 and that tube is too small. Image 4 shows that picture again, then the next picture is after the patient had a stent placed in the cath lab. See how narrow that tube was to start? Then they made it much bigger with the stent. You need to get rid of obstructions and create more space for blood to flow easily. If a patient has liver disease, they should have a cath done so we can see if there’s anything that can be fixed with a stent.

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There’s also a blood test – alpha-feto protein (aFP) – which is the blood test marker for liver cancer. That is useful because if it’s elevated, that usually indicates serious liver cancer. So we do check that one regularly on post-Fontan patients. And we use ultrasound, MRI, and CT imaging to look for suspicious liver nodules or tumors.

There are some drugs that are speculated to possibly help the liver: Spironolactone to decrease scarring in the liver; Sildenafil to lower PA pressure; Warfarin to thin the blood and prevent clots from forming in the liver and causing fibrosis and cirrhosis. But there are no large studies on these, so it’s just a lot of speculation at this point in time.

Thinking back to the images we looked at, part of the issue is structural. If you have a 3-year old and you put in a big tube (maybe 18-20mm conduit), it may be too big for a kid and they’ll form clots along the inside of it. So some have advocated going to smaller conduits (16mm or smaller) for small kids. But that’s short-sighted – it gives you the best flow dynamics physically for a little kid, but these tubes don’t grow. They’re made of Goretex, or sometimes a homograft of human tissue, so they don’t grow. An adult needs an 18-20mm connection, and if it’s smaller, we get the pictures above. That’s why the cath lab and stenting as people grow is so important.

 

Q: What’s on the horizon?

A: What if we could put a pump in the circuit? We could achieve two things with a pump: increase the pressure in the PA, and at the same time, decrease the pressure in the IVC so it goes down to a normal level and hopefully improves the liver function. That will likely be the biggest help for the liver because the biggest problem is congestion in the liver. There’s a lot of research currently being done – including here in the Wanek program – and there is hope, but it’s still in the early stages.

We also did a pilot study using stem cells to strengthen the heart, but that was for when the Fontan was failing and heart function was down. We don’t know if stem cells can improve liver function in any way, but things like pumps and stem cells are the future of post-Fontan care that our program is working on today.

 

Q: What’s the take-home message about FALD?

A: The biggest message today is early surveillance. We’re finding that many individuals actually go into the Fontan already having liver disease, which can be problematic because the Fontan just puts more pressure on the liver. So blood tests and ultrasound imaging are important before and after the Fontan, and maybe even prior to the Glenn, to make sure we catch any problems early.

But for general Fontan health, the message is – exercise, exercise, and more exercise!

 

 


The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.

 

Wed, Apr 29 12:29pm · Sending Hope, Healing, and Heart Hugs in HLHS

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Sisters by Heart was formed in 2010 by a group of heart moms who came together to support others on the journey of being a heart parent. The organization became a member of the HLHS Consortium in 2017, becoming the first advocacy group to join. Today, the mission of Sisters by Heart is to support, educate, and empower families affected by HLHS.

 

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One of the ways the group supports families is by providing care packages for those who are newly diagnosed to help make their bedrest or hospital stay a little easier. The Todd and Karen Wanek Family Program is proud to collaborate with Sisters by Heart to provide these packages. Our shared goal is to ship a care package to each and every family facing a new diagnosis of HLHS in the United States. We believe it is especially important to support families during times like these, which is why we remain committed to continue providing care packages.

 

 

 

As fellow moms of children with HLHS, we know the devastating toll a new diagnosis can take on families. We help parents and siblings take the first steps forward — to find hope, arm themselves with resources and specialty items, and validate the range of emotions they may be feeling. We are mindful and update the items in our care packages as new items or resources become available. For families, receiving this gift early on gives them a sense that ‘a whole community is by our side through everything.’

-Stacey Lihn, Sisters by Heart President

 

Since 2017, on behalf of Sisters by Heart, the team at the HLHS Program (led by Traci Paulson) has delivered an average of 21 care packages every month. The packages include a variety of items specially chosen to make the time spent in hospitals a little easier:

  • Side-snap shirts and onesies designed for use around tubes and wires
  • Baby leg warmers
  • Pens/notepads for tracking important details
  • Swaddling blankets
  • Hand sanitizer and moisturizer
  • Sibling gift
  • Resources and support information

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If you or someone you know has recently learned their baby has HLHS, you can request a care package at no cost.

For older kiddoes, Sisters by Heart also provides “Fontan Superhero Packages” for toddlers preparing to undergo the Stage III surgery! You can learn more and request a Superhero Package here.

 

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The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.


Meet other people talking about HLHS or CHD on Mayo Clinic Connect – an online community designed to help patients and families connect with each other.  Our community is a safe space to share experiences, ask questions, find answers, and exchange support.  The discussion groups and topic pages are moderated to ensure that all posts are respectful, and Mayo Clinic Connect never sells or shares posted information to third parties.  Here are some you might like to follow:

 

Fri, Apr 10 3:58pm · A Message from the HLHS Consortium Regarding COVID-19 in HLHS

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In December 2019, an outbreak of a new infectious disease occurred in China. The virus has been named “SARS-CoV-2” and the disease it causes has been called coronavirus disease 2019 (COVID-19). Since then, it has become a pandemic that has rapidly expanded worldwide, causing a respiratory infection that can progress to severe pneumonia. Person-to-person transmission of SARS-CoV-2 is thought to be similar to the transmission of influenza and other respiratory pathogens: respiratory droplets are formed when an infected person coughs or sneezes, and these droplets are inhaled by close contacts, generally within six feet.

 

One important question is whether SARS-CoV-2 can be transmitted from a pregnant woman to her fetus, a process called vertical transmission1. A follow-up question, therefore, is whether the virus can be spread in the birth products, such as the placenta and umbilical cord blood (UCB).

 

Several studies addressing these questions have been reported recently. Over 100 pregnant women in their third trimester who had respiratory symptoms tested positive for COVID-192,3. Among the babies delivered to these women, there were no confirmed cases of SARS-CoV-2 infection. Importantly, in placentas and UCB that were tested for SARS-CoV-2, the results were also negative.

 

Findings from these studies suggest that there is currently no evidence for intrauterine infection caused by vertical transmission in women who develop COVID-19 pneumonia in late pregnancy4.

 

Furthermore, the Food and Drug Administration (FDA), the American Association of Blood Banks, and Be The Match: The National Marrow Donor Program have all stated that individuals are not a risk of contracting COVID-19 through blood or blood products. This includes receiving a blood transfusion or regenerative medicine therapies involving stem cells. The FDA reports that there have been no reported or suspected cases of transmission-transmitted COVID-19 to date. These agencies recently met to discuss whether additional screening should be added; however, they determined that no testing of mothers or cord blood is needed. They recommend continuing standard practices for collecting and processing cord blood.

 

Based on currently available information, the Todd and Karen Wanek Family Program continues to collect UCB from infants diagnosed with Hypoplastic Left Heart Syndrome (HLHS). We have also made changes in our manufacturing laboratory to comply with social distancing guidelines and ensure the safety of our staff. In addition, we will continue to perform cell deliveries at HLHS Consortium hospitals with careful consideration on a case-by-case, day-by-day basis.

 

Please contact us at HLHS@mayo.edu if you have any questions.  Thank you for your continued support.

 

 

  1. Mojgan Karimi-Zarchi, Hossein Neamatzadeh, Seyed Alireza Dastgheib, Hajar Abbasi, Seyed Reza Mirjalili, Athena Behforouz, Farzad Ferdosian & Reza Bahrami (2020): Vertical Transmission of Coronavirus Disease 19 (COVID-19) from Infected Pregnant Mothers to Neonates: A Review, Fetal and Pediatric Pathology. doi: 10.1080/15513815.2020.1747120
  2. Schwartz DA. Arch Pathol Lab Med. 2020 Mar 17. An Analysis of 38 Pregnant Women with COVID-19, Their Newborn Infants, and Maternal-Fetal Transmission of SARS-CoV-2: Maternal Coronavirus Infections and Pregnancy Outcomes. doi: 10.5858/arpa.2020-0901-SA.
  3. Rasmussen SA, Smulian JC, Lednicky JA, Wen TS, Jamieson DJ, Coronavirus Disease 2019 (COVID-19) and Pregnancy: What obstetricians need to know, American Journal of Obstetrics and Gynecology (2020), doi: https://doi.org/10.1016/j.ajog.2020.02.017.
  4. Huijun Chen, Juanjuan Guo, Chen Wang, Fan Luo, Xuechen Yu, Wei Zhang, et al. Clinical characteristics and intrauterine vertical transmission potential of COVID-19 infection in nine pregnant women: a retrospective review of medical records. Lancet. Vol 395, iss 10226, p809-815, March 7, 2020. doi:https://doi.org/10.1016/S0140-6736(20)30360-3.

 


The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.

Fri, Mar 27 1:40pm · Dedicated to Delivering Hope in HLHS

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FRIDAY, MARCH 27 — What happens to clinical trials when an unprecedented global outbreak shuts down life as we’ve known it?  The answer, as with most things right now, is — It depends.

 

The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) currently has a Phase IIb clinical trial open at seven hospitals in the US through the HLHS Consortium. Stem cells taken from a baby’s umbilical cord blood (UCB) are injected into the babies’ own heart during a surgery to repair the congenital heart defect with the hope of strengthening the heart. Our team has been performing cell deliveries since 2013, and typically brings the frozen cells from our lab in Rochester, Minnesota directly to the hospital where the surgery is being performed.

 

Hospitals are currently reviewing which studies offer a potential benefit to patients and which should be modified or put on hold to avoid exposing patients to unnecessary risk. As of today, all hospitals in the HLHS Consortium have determined that our UCB Collection Study and Phase IIb Cell Delivery Study both offer a significant potential benefit that outweighs the risks, and will continue to enroll and treat patients on our trials.

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We are also doing our part to further minimize possible risks during this time. For example, all non-laboratory staff began working from home in mid-March.  (We have so many people on video calls, we hum ‘The Brady Bunch’ theme song at the start of each meeting.)

 

Essential lab staff are taking steps to ensure that our ongoing cord blood collection and processing is done safely. In addition to our normal sterile field clean room processes, staff are working alternating shifts, regularly disinfecting surfaces, and conducting verifications virtually to reduce the number of staff on-site. Additionally, while our cell delivery team (pictured above) usually flies to sites on commercial airlines, our program is currently chartering the Mayo Clinic Air Ambulance airplane to reduce exposure and keep our staff and patients as safe as possible.

 

We remain committed to conducting the vital work of bringing hope to CHD families through the development of regenerative medicine products. However, we respect that the situation is rapidly changing, and will only continue our work when and where it is safe to do so. We will continue to evaluate each cell delivery treatment on a case-by-case, site-by-site, and day-by-day basis.

 

We appreciate your understanding and support. Please feel free to contact us at HLHS@mayo.edu if you have any questions or concerns. Thank you for your efforts to keep our communities safe during the COVID-19 pandemic.

 


The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.

Mon, Feb 24 11:18am · Organizations with Heart: An Inspiring Conversation for CHD Month in HLHS

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During CHD Awareness Month, we hear many families share stories of their journey in the heart world. The community is very tightly knit, and many families are inspired to give back and find ways to support others. This support can be as simple as posting reassuring comments on social media, or donating a gift card for gas to a family traveling for care. Many also get involved with their local support groups or hospitals.

Some families are motivated to create a new organization to address a need in their community. To celebrate CHD Month, I talked with the founders of three incredible CHD family organizations: Beats for Bella, The Heart Hero Project, and the Ethan M. Lindberg Foundation. These inspiring women share their stories of how they got started, challenges they face, and what’s been the most rewarding.

Note: This article is a bit longer than usual, but well worth the read! (~15-20 min. read time)


 

Brianna Tranby: Let’s start with an introduction to each of your families and organizations.

Erin Borkowski: We organized Beats for Bella in 2014, about a year after our daughter Bella was born. Our focus initially was just awareness of HLHS and sharing our story. We had so much support from the Children’s Hospital of Philadelphia (CHOP) and our families while I was pregnant and through her first year of life, so we wanted to give back by sharing her story and letting everybody else know who was interested where we were with our journey.

Today, our focus is on post-Fontan care because that’s what affects us at this point. Bella will be seven this spring and is almost four years post-Fontan. In researching the health struggles that will probably present themselves as she gets older, we felt like that was the right place for us to be and we’re passionate about helping fund research to make sure that Bella, as well as adults living with single ventricles, have the best chance at long, healthy lives. As an organization, we’ve been able to support research and the community with $220k in donations over the last six years.

Alicia McPeters: My son Liam was born in November 2017 and I started The Heart Hero Project shortly after that, so our organization is a little newer. I started it because when I was diagnosed at 20 weeks and trying to find information about HLHS, there were a lot of personal stories out there and it was really helpful to me to see the broad spectrum of everything. We’re also a CHOP family and had a lot of support, so our goal was to support HLHS families and the community. My son passed away in October 2019, so our mission now is raising funds for research.

Jessica Lindberg: We started The Ethan Lindberg Foundation in early 2015. My oldest son Ethan was born in 2005 and passed away in 2012. Our goal when we started was to address some of the gaps we saw. The financial impact of this disease is huge and no one’s doing anything about it. We’re from Illinois but went to Boston for care. There was no affordable housing and the travel and medical bills were financially ruinous for so many people we met. It’s hard to make good medical decisions when you’re in that position, Lindberg 1so we wanted to advocate and raise the bar of how we care for families.

We started by getting two apartments in Boston where we house families getting care, and we give grants and have helped families from 44 states. We also fund music therapy because Ethan loved music and we have to have joy in our experience. And we’re now starting to do research looking at the financial impact of CHD on families and presented our initial findings at the CHOP Cardiology 2020 conference.

 

BT: What inspired each of you to create your organization?

Erin: When we got Bella’s diagnosis and found out that we’d need to stay close to the hospital, our families created a t-shirt fundraiser for us called “Beats for Bella.” They raised enough money for us to stay in a local hotel and close to Bella. Soon after Bella was born, I got involved in the local Mended Little Hearts chapter helping with their fundraising, and originally thought was that’s where I was going to be as a part of the community. Then after about a year, I started looking at the need for local organizations. My husband and I both have large families and a huge support system who have gotten involved, so it kind of starting growing on its own and got to a point where that became our priority after it took on a life of its own.

Jessica: My husband and I had gotten married, we were both working and I was in graduate school, and we were just going along in our lives until we got this diagnosis and it changed everything. I quit my job and school, we lived apart since my husband was working to keep insurance, we plowed through our savings. Ethan went into the hospital in May 2011, and we were in the hospital for 13 months until he died in June 2012. In that time, we lived between Boston and Children’s Hospital of Wisconsin in Milwaukee since that was our home hospital. And in the midst of that, I also gave birth to my third child so we have four boys all together now.

We experienced horribly difficult decision-making around medical care, we struggled to find affordable housing, and we went through all of this as a family and got out of it and thought “We don’t want any more families to feel the way we do right now.” There’s a lot of focus on research, which of course is important, but there’s not enough focus on families and what this journey does to them. There is a part of the human experience we are not talking enough about.

Alicia: I started the organization because something in me was telling me that I needed to do something. For some reason, I always felt like I wasn’t going to get a long time with Liam, so I think I always knew that whatever I did was going to be for other kids and not my own. So I wanted to make that journey mean something, and help other families get through the stages that we all go through.

HeartHero - Liam (1) - CopyI also felt like it was a little lonely, as a heart mom. I also have a very large family and good support system, but trying to talk to someone who didn’t understand “the lingo” at times was difficult. So sharing our story online was an outlet for me to get my thoughts and feelings out there and have others reply “I hear you” or “I also felt that way.” And then as Liam’s health declined, I wanted to share the whole process even more because I felt like it was important for me when I was diagnosed to see the whole spectrum of things that could happen, so I wanted other families to see that as well. And then of course with his passing, I think research and awareness are so important because there’s not enough funding out there, and there are so many doctors who can do so many things if they have the ability to do it.

 

BT: What’s been the most rewarding experience so far with your organization?

Jessica: I’ve always been so inspired by the families that we meet and by the individual stories. These are just normal people – these are your neighbors and who you’re checking out next to in Target (laughs). Life happens to them in a way that they weren’t expecting, but they’re doing it because the human spirit is so powerful. I think there’s this fallacy that people who have a sick kid are these outlier people. But we’re not! We like cute clothes, we read People magazine, we like to go get a beer – we’re normal people! So that’s one of the myths that I want to dispel. Anybody can have things happen to them, and we’re just normal people doing our best. We love helping to improve the family experience for families just like ours.

Alicia: This is only our 2nd year, but I just feel like I have this purpose to help people. For example, Christmas is my favorite holiday in the world. But I know that many families never get to spend any of their holidays at home, so our first year we posted on social media that we wanted to help make people’s holidays a little brighter and did a donation of toys. We got about 60 responses and sent out our first “Holiday Hero” boxes. A lot of families sent back photos of their heart kiddoes smiling, and it made me happy to see that I’d made someone smile. We did it again last year and plan to keep going in the future.

Erin: I feel like any time we have an event or even just a conversation withBeatsBella (2) somebody, I’m always encouraged when somebody “gets it.” Like Alicia said, you have this large community of people who support you, but there’s very few people who really understand. So when somebody who wasn’t thrown into this world connects with you and believes in what you believe in, that’s what gets me every time. We have neighbors, friends and so many others who don’t need to be involved in our organization, if they believe in what we’re doing and want to know, that’s where we’re sustainable.

 

BT: Can you tell us about any challenges you’ve faced? Have you overcome them, and how?

Erin: I think the biggest challenge is getting people to understand that Bella’s not fixed. Her life will never be “normal.” There are things that other families worry about, but with us they’re amplified because of her heart defect. People think because she’s almost four years post-Fontan, that she’s great. And that’s the blessing and curse of CHD – she looks normal. So it’s hard for me to not get upset with people when they assume that she’s fine. And she is doing great, she’s thriving, she’s in school, and we’re truly blessed. But it’s a life, and I’m going to watch her struggle over things as the years go by. So that’s my biggest challenge, and it’s why we believe that awareness and making people “get it” is so important.

Alicia: I agree 100%, the hardest part is the personal aspect. Trying to explain the condition to someone and they say “Well, they’re gonna be ok, right?” Liam was just like Bella where if you looked at him, you never would’ve known except by the scar on his chest. But I feel like it’s a lonely world as a heart family because other people don’t understand the worry that we have 24/7. They’re concerned about their kids getting a cold – but we’re concerned that if our kid gets a cold, we’re going to end up in the hospital. So it’s important to raise awareness that there are a lot of kids who thrive and look good on the outside, but there are still a lot of parents who don’t get to bring their kids home.

It was also hard after Liam’s passing for me to get back into it, because seeing a lot of the kids online who are thriving was difficult at first. But it makes me happy now and makes me want to raise money for research even more so there are more kids like Bella and other post-Fontan kids. They have so much ahead of them, so it continues to draw a fire in me that I want to help these kids have a great life.

Jessica: From the organization side, creating an organization is like creating start-up company. I don’t say I have a job – I say I have a “lifestyle” (laughs). The Four Hearts Shop is in my finished basement, we’ve taken over our whole house to do this, and we’ve thrown ourselves as human beings into this cause. And that’s a challenge because it’s hard to have boundaries sometimes, like the times when every family needs an apartment on Sunday at 3pm. I’m learning to set those boundaries, and hopefully as we grow we’ll be able to hire more people and create a better division of labor which you don’t usually get in the beginning.

 

BT: What advice would you give to someone starting a new organization?

Erin: My biggest piece of advice is to find your “Why?” Because you need to know the big, deep down why, not just “I want a cure for HLHS.” It’s a hard question for people to answer and you have to give it a lot of thought. We didn’t know our “Why?” in the beginning, we just felt we needed to do something and spread awareness, fund research, and help families because people had helped us. But over the years, we’ve grown by doing some self-discovery and understanding why we do what we do. I feel very strongly that if we have this community that can come together and we can do our small part to inspire people and give them hope, then it makes all this worth it. But it’s also a lot of work. I work full and have other kids, so you’re going to be tired and you’re not going to want to do it sometimes. And you have to give yourself those breaks.  But to really make an impact, I feel like you have to do some self-discovery, talk to people, collect your thoughts, and figure out why you want to dedicate yourself to this.

Alicia: I agree – find your “Why?” because it is a lot of work. If you feel that fire and feel like you have to do something, you have to put all of you into it. Because of how my journey has gone, at least for me, if I can make a difference in even one person’s life, that makes this worth it.

Jessica: I totally agree. What people sometimes forget is that this is a business. The “non-profit” label is just an IRS designation – this is a business. I have to do an audit every year, pay bills, and make financial decisions. It’s not only this “do-gooder” thing, although there’s certainly that piece to it, but you have to be business savvy.

I also think it’s important for everyone in the heart community to be willing to work together. My organization isn’t going to be innovating in the cath lab, or in D.C. lobbying for research money; that’s not our role. But we understand the people experience. As a community, we should cheer each other on as we work in our lanes and expertise. I think that’s the best way to be effective.

 

We would like to thank Erin, Alicia, and Jessica for the amazing work they do, and for taking the time to share their stories and experiences! You can learn more and connect with their organizations on social media:

 

Lindberg 2 HeartHero - Liam (2) BeatsBella (6)

 


The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.

 

Tue, Jan 21 11:06am · Tips on Preparing for College When You Have CHD in HLHS

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Going away to college can be both an exciting and scary time for young adults, including those with congenital heart disease (CHD). This article is designed to help incoming college students with CHD navigate this new chapter in life and learn how to take charge of their own healthcare.

 

After being accepted and choosing your college:

  1. Notify the Housing Office of special requests you might qualify for: Air-conditioned dorm, lower-level floor room or elevator building, or dorm near classroom buildings.
  2. Notify Housing/Admissions Office of need for available AEDs, if appropriate.
  3. Notify Disability Office of any special requests, such as needing additional time or a quiet location to complete testing.
  4. Contact the Student Health Office to discuss your health issues. You can schedule a meeting with them, if needed.

 

Six months before you go:

  1. See your cardiologist. Have all of your cardiac prescriptions refilled by your cardiologist and ask for written prescriptions to take with you if you need to refill medication away from home.
  2. Ask your cardiologist to recommend an Adult Congenital Cardiologist near your college. The Adult Congenital Heart Disease Clinic Directory is also a great resource.
  3. Create a file with information to keep with you when you leave home. You should be able to find most of this online in your electronic medical record, but ask your cardiologist if you have questions. Make sure the file includes:
    • Your heart defect
    • All of your surgeries and dates
    • All current medications and allergies
    • Copies of recent electrocardiogram and echocardiogram reports
    • Your primary care provider’s name and contact information
    • Your cardiologist’s name and contact information
    • The address, logins, and passwords for all online medical record accounts (keep in a safe place)
  4. See your primary care provider. Make sure all appropriate immunizations are up to date including: Meningococcal, Human Papillomavirus (HPV), and any booster immunizations. Make sure to get flu shot in early Fall (every year!) whether at home or at school.
  5. When visiting your college, scope out the nearest Emergency Room and pharmacy that are covered by your insurance.
  6. Order a MedicAlert ID. Wearing a medical ID band could be lifesaving if you have an emergency and can’t communicate your medical history. Plus – they have tons of cool, sporty designs for under $30.

 

One month before you go:

  1. If you have a pacemaker or defibrillator, notify the Pacemaker Lab of your upcoming relocation.
  2. By now, you may have met your new roommate(s) either online or in-person. Take this time to let them know about your heart history. It’s important to have those closest to you know this about you so they can get you help, if needed.shutterstock_317941118
    • Remember – they might have their own medical conditions to share with you.
  3. If you have been contacted by your Resident Advisor (RA), let her/him know your heart history, too. Be sure you inform them shortly after arriving on campus.
  4. Refill your prescriptions. Go to college with a full supply.
  5. Contact your chosen cardiologist near your college. Set up an appointment around your arrival, if needed.

 

Arriving on Campus:

  1. Be sure your roommate(s) are aware of your heart history. Answer their questions, or explain what’s private versus what they need to know.
  2. Touch base with your RA and Student Health. Ask them where AEDs are located on campus, and locate the one closest to your room.
  3. Find the closest insurance-covered pharmacy. Inform your cardiologist and PCP of that pharmacy’s telephone and fax numbers. Do a practice run to the nearest Emergency Room.

 

Life on Campus:

  1. As you get to know people and establish close friends, let them know about your heart condition. It’s important that those you spend time with know how to help you.
  2. Get flu shots every Fall, and let your PCP’s office know you received it.
  3. Caffeine, marijuana, alcohol, and other stimulants can cause arrhythmias. Be cautious.
  4. Stay hydrated, exercise as directed, and get plenty of rest!
  5. Never ignore any symptoms!  Report to Student Health or call your cardiologist. Don’t wait! Early intervention can help prevent serious problems.
  6. Weekend road trip or Spring Break? Be sure to take your file of information with you when you travel.
  7. Most important of all — Have Fun! 

 


The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.


Meet other people talking about HLHS or CHD on Mayo Clinic Connect – an online community designed to help patients and families connect with each other.  Our community is a safe space to share experiences, ask questions, find answers, and exchange support.  The discussion groups and topic pages are moderated to ensure that all posts are respectful, and Mayo Clinic Connect never sells or shares posted information to third parties.  Here are some you might like to follow:

Mon, Jan 6 9:40am · Interview with Tim Nelson, MD, PhD - Director of the Wanek Family Program for HLHS in HLHS

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2020 marks the 10 year anniversary of the Todd and Karen Wanek Family Program for HLHS at Mayo Clinic! To kick off the celebration, I sat down with the program’s director, Dr. Tim Nelson, for a look back at the first 10 years – and a sneak peek at what’s coming next.

 

Tim, take us back to the beginning. How did you first meet the Wanek family?

I had the privilege of meeting Todd and Karen Wanek back in 2009. The family had long been patients of Mayo and were here looking at what was being done in the research community, specifically in the Mayo Clinic Transplant Center. They wanted to know what’s being done to change the way we take care of congenital heart disease (CHD) patients in the future.

We got to talking and I showed them the lab, and before we knew it, we were proposing a grand plan for them to fund a transformative program to do something that had never been done before. It was kind of a fast and furious introduction and planning of the program in 2009.

 

What inspired you to help create the Wanek Family Program at Mayo Clinic?

I was a medical student at the Medical College of Wisconsin in Milwaukee in the late 1990s and they were well-known for their care of complex CHD. I was actually aspiring to be a cardiothoracic surgeon, but I was doing my PhD in research at the same time and realized that many of the treatments we did were quite simple – even old-fashioned – and the outcomes of our patients were not what anybody wanted.

We were doing research with cell-based technology because Dr. James Thompson in Madison had developed the first embryonic stem cells in 1998. So there was a lot of excitement in Wisconsin about the future of stem cells. Then I came to Mayo with the Clinician-Investigator Training Program and got more involved in research. So when I met the Wanek Family, it was perfect timing because technologies had developed to the point where there was a real chance at bringing cell-based therapies into clinical practice. It was that vision that led us to double-down on research innovations as the pathway forward.

 

What have been your “Top 3” highlights with the HLHS Program over the last 10 years?

That’s hard to answer because there have been so many! And sometimes one that you don’t think is a big deal actually becomes the thing that unlocks the future.

One, for sure, was just meeting the Wanek Family, and the moment when they agreed that we could do something massive together. I remember that being an awesome opportunity, but I also immediately felt an awesome responsibility to do something that no one had ever done before.

The next was the day we got the call from the Food & Drug Administration (FDA) that our first clinical trial was approved to open. Clinical trials take years of planning, so that moment and that sense of accomplishment with our team was an awesome responsibility once again.

But the responsibility of what we’re doing culminates in the greatest highlight of all – actually treating each and every patient. Our work is about giving families hope that they didn’t have before. And that’s another level of responsibility that everyone on our team appreciates.

 

With over 60 people in the Program, we have the largest HLHS-focused research team in the world. But we’ve also now created the nationwide HLHS Consortium. For anyone who doesn’t know or still has questions, what is the HLHS Consortium?

That’s a great question to clarify because the HLHS Consortium is a unique group. We realized that our program had built an incredible infrastructure to support research.  However, the rarity of HLHS means that each hospital only sees few of these patients each year.  So although Mayo has a great heritage of breakthroughs including the heart-lung machine and outstanding clinical practice, the size of our practice here was just too small to support our research by itself. So we started expanding to more institutions to include more patients in our studies.

The HLHS Consortium today spans the country and includes hospitals and advocacy groups that are committed to providing the best care for CHD patients. But they’re also committed to innovating and transforming the future of HLHS and CHD with new products and services. So it’s a really unique club that works together to expand technology to our patients.

The identity, direction, and focus of our Program at Mayo Clinic will always be HLHS. But the Consortium allows us to expand to other sites, and also allows us to start taking on other challenges in the congenital heart world so we can enhance the impact of what we’re doing beyond just HLHS. I think this is the really exciting part of where we’re going – our HLHS-focused work at Mayo will help guide us as we take on other CHD problems with groups across the country.

 

What are you working on right now that people can look forward to in 2020?

The pride of what we do is clinical trials and offering hope to families. As of today, we’ve treated 70 patients with CHD in four different clinical trials. Our goal is to continue to increase the number of patients treated, and doing that takes new protocols, new treatments, and new products. What we talk about on a daily basis around here is “What is the next clinical trial? Where do we go? How do we do better with our resources and technologies?”

We’re developing new trials that we expect to announce in 2020. Looking beyond this year, I anticipate that we’ll move toward bioengineered cells where we can actually create beating, contracting heart tissue from induced pluripotent stem cells, and that we’ll start printing and making tissues or constructs that will be surgically applicable. This is our commitment and the next frontier, and our team’s experiences over the past 10 years will help us to more aggressively take on the new technology that’s coming.

 

On that note, when we talk again in 2030, what do you hope will have been the “Top 3” moments of the HLHS Consortium and our work?

I envision that there will be a day when, as much as we celebrated the first FDA-approval to open a clinical trial, we will be celebrating much bigger when we have FDA-approval for a product. When the product goes beyond the testing phase and becomes a standard operating procedure for all patients, that’s the goal that we’re all working towards.

In addition, I can imagine the stories of hope from patients across the country, and maybe even the globe, as we pioneer new products. One product isn’t going to be the solution for all patients, so the goal is building a sustaining ecosystem in which developing one product leads to us being able to research and develop five more products. Those are definitely going to be the accomplishments that we look back on with pride.

It’s amazing how fast 10 years goes! Day to day, we can lose sight of what our teams have accomplished and the massive amount of work that everyone does. To have our infrastructure and ability to do important work for CHD patients is a real privilege, and we’re grateful to the Todd and Karen Wanek Family for their commitment and allowing us to do what we’re doing together.

 

Stay tuned for some exciting announcements from the HLHS Program and Consortium this year!

 


The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.


Meet other people talking about HLHS or CHD on Mayo Clinic Connect – an online community designed to help patients and families connect with each other.  Our community is a safe space to share experiences, ask questions, find answers, and exchange support.  The discussion groups and topic pages are moderated to ensure that all posts are respectful, and Mayo Clinic Connect never sells or shares posted information to third parties.  Here are some you might like to follow:

 

 

 

Dec 9, 2019 · Your Guide to Children in Research in HLHS

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Demystifying Clinical Trials Series – Part 4
The research world can seem confusing or overwhelming at times. Our ongoing series is designed to give you the information you need and help answer common questions. Part 4 in this series focuses on research involving children, and the special regulations that are in place to protect them. You can find our previous posts here: (Part 1, Part 2, Part 3).

 

The Importance of Children in Research

Clinical research is the formal process through which doctors and scientists study diseases and develop better treatments. However, most diseases that get studied – and most drugs and doses that get developed – are only tested using adults.

But children aren’t just little adults. Their bodies grow and change in ways that are very different from adults. For example, new research is showing that infants’ heart cells have a much greater potential for regeneration than adults’ hearts. So it’s vital that researchers design studies specifically for children.

“Children need clinical trials that focus on them, as medical treatments and approaches often differ for children. For example, children may need lower doses of certain medicines or smaller medical devices. A child’s stage of development also can affect how safe a treatment is or how well it works.” – National Heart, Lung, and Blood Institute (2019)

 

Protections for Children

Children aren’t just little adult, and the Department of Health & Human Services has special legal regulations to ensure that children (and pregnant women) are kept safe. For example, these rules require an Institutional Review Board (IRB), the impartial group that reviews and approves research studies, to look more closely at research involving kids and evaluate the potential risks and benefits more closely. There are also special requirements for both parents and children to agree to participate. This is called giving “consent” and “assent”.

Consent: The process that gives a research participant all the information s/he needs to make an informed decision about whether to participate in a study.

Assent: An additional process for children ages 7-17 to make sure s/he understands the study and is comfortable participating. Most institutions have a separate document that explains the study in kid-friendly language, and the research team and parents will work together to ensure the child understands.

In most cases, a child under the age of 18 cannot sign a legal consent form agreeing to participate in a research study. However, they can participate in research with their parents’ permission.

 

The Rights of Patients and Parents

It’s important to note that while “assent” is not a legal requirement, most IRBs require it for research involving children. If assent is required for participation in a study, the parents must give legal consent and the child must give assent. If a child declines to give assent, they are not allowed to participate in the study even if the parents give legal consent.

In all research, at least one parent must give consent for their child to participate. In some studies, both parents must give consent. Parents have the same rights as the child to understand the study completely, ask questions, get results of the study – and say “no”.

Some good questions to ask before considering participating in research include:

Why am I (or my child) eligible for this study? Does my doctor recommend this study for me?
What are the possible risks? When will we be told about the results?
What are the potential benefits? Will the tests hurt?
How does this study compare to the normal standard of care? Am I eligible for other studies?
Are there extra tests or visits needed for this study? Can we talk to a doctor who is not a part of this study?

Connecting with Other Parents

Some studies now have websites or social media pages to provide more information and allow participants to connect with each other and share their experiences. For example, the Mayo Clinic HLHS Program has a private Facebook group for families who have joined our stem cell trial. The National Institutes of Health has a Facebook page “to increase understanding of research in kids and help families make informed decisions about participating in a pediatric study.” In addition, most physician researchers keep a list of previous study participants who are willing to share their experiences with new patients, and you can ask to talk to them.

“We felt very passionate about getting involved with the study right away because there was someone before our son who paved the way to get him to where he was. So we felt very connected to that, and it was important for us to make sure that he was a part of this study.” – Staci Strand, 2019 Feel the Beat Panel Guest

We are grateful to the families who are involved in our research work! This year’s Feel the Beat event featured a panel discussion with three families whose children participated in our Phase I stem cell delivery clinical trial. You can watch their conversation – and hear Dr. Tim Nelson discuss our current and future research – on our Facebook page.

 


Meet other people talking about HLHS or CHD on Mayo Clinic Connect – an online community designed to help patients and families connect with each other.  Our community is a safe space to share experiences, ask questions, find answers, and exchange support.  The discussion groups and topic pages are moderated to ensure that all posts are respectful, and Mayo Clinic Connect never sells or shares posted information to third parties.  Here are some you might like to follow:


The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us at HLHS@mayo.edu to learn more.