Advocates in Action

We recently sent out an Action Alert, asking you, our incredible advocates, to contact your legislators to request increased research funding.

Many of you sprung into action.  Here are a few cute snip-its:

1) Say Cheese!

Atkinson girls with DeLauro's Office

Mirabel, her twin sister and her mom, stopped by Capital Hill to visit Congresswoman DeLauro’s amazing staff person, Eric Anthony.  Thanks Meredith!

2) Did I just hear you correctly?

Another rockstar advocate from Arkansas was simply trying to get an email address.

Melissa: (talking to receptionist in Sen Mark Pryor’s DC Office) Does the Health Care LA prefer mailed correspondence or email?
Staffer: Email — it’s much quicker and he can respond to you.
Melissa: May I have his email address, please?
Staffer: No — we don’t give those out.

Thankfully, Melissa knew the solution.  She simply asked for the correct spelling of the first and last name for the Health Care LA, to insert into the standardized email format (first_last@senator’slastname.senate.gov)  Thanks Melissa!

It’s not too late to join in on the fun!  For instructions and a sample template to email your legislators using their online form, click here.  If you are feeling more adventuresome, email advocacy@conqueringchd.org and we can talk about how to send an email with an attachment, like Melissa, or how to visit your local or D.C. legislative office, like Meredith.

CHD Symposium, June 21

The Children’s Heart Foundation, Mended Little Hearts and the Pediatric Congenital Heart Association have joined together to host a National Congenital Heart Defect Symposium. Join us to hear firsthand updates from renowned speakers in congenital heart defect (CHD) treatment and about issues that affect children living with CHDs, including clinical updates, neurodevelopmental issues, exercise, and transitioning to adult CHD care. Get inspired by others and find out what you can do to be a champion for change. 

 
Where: Westin Indianapolis 

When: June 21, 2014

Time: 10:00 a.m. to 5:00 p.m.

Cost:  $35 per person includes materials and lunch.

View the Symposium Flyer here
Check out the exciting list of topics and speakers!

We have negotiated room rates for the Westin Hotel at $119 Single/Double occupancy; $129 Triple occupancy; $139 Quadruple occupancy; plus the current rate of 17% for state and local taxes.  Guest room rates are also available three days pre and post the official event dates, based on availability.
Reservations must be made by May 18, 2014 to secure the MHI room rate.  To make a reservation, please call the hotel at (317) 262-8100.
To get these rates mention “Mended Hearts” when making reservations.

Register here

Questions:  Contact Jodi Lemacks at jodi.lemacks@mendedlittlehearts.org or 804-419-7028

 

Kieran Touney

kieranstory1

When Kieran was born, I felt like I was giving birth in a theater filled with an expectant audience—a repertoire of doctors stood around, anticipation on their faces, waiting to spring into action. As Kieran had been diagnosed with HLHS during our 20-week ultrasound, we were all ready for him—OB, residents, nurses, neonatologist, cardiology fellows, and critical care specialists. I had a very long delivery, so they were milling around for quite awhile, waiting much longer than they had planned to see him safely through his first echocardiogram and to his room in the NICU. The news came that I might need a c-section, but I was going to give it one more try. About 45 minutes later, after a long night and day, around 8 pm on August 7, 2008, Kieran was born in front of all of these “strangers,” to sighs of relief and cheers of “you did it!”

 

He was placed in my arms and my husband Shawn and I got to hold him for about 5 minutes before he was whisked off, accompanied by my husband. But for those 5 minutes, everything felt normal. Kieran looked healthy and robust, and was a healthy weight and size. Despite the crowd of specialists and staff bustling about the room, for 5 minutes everything else stopped as I gazed at my son. Although he looked like any other baby at the time, when I look back now at pictures taken from his first weeks and months, I see how cyanotic (blue) he was. His tiny heart, made tinier by being absent a left ventricle, was desperately trying to get enough oxygen to both his body and lungs.

 

Being left alone while Kieran and my husband left for the NICU was one of the hardest times in my life. I was unprepared for how it would feel to have my baby taken away so quickly, although I was grateful we had had at least a few minutes together before the whirlwind of medical intervention began. At the same time, I had a lot of joy to comfort me through those first few hours, because Kieran looked perfect despite having such a serious heart defect. I knew, somehow, he was going to fight through what was coming.

 

I had always felt that way, from the moment my OB had called me to tell me “I can’t see all of the heart,” through the perinatologist appointment where we were given the official diagnosis, through the long Easter weekend that had followed the news. Of course, it was devastating to learn something was so wrong with our baby. But along the way, we had consistent hope that Kieran should be given as many “fighting chances” as he possibly could. We were lucky to be close to one of the best pediatric cardiology centers in the country, where we started going for prenatal echocardiograms and meeting with the cardiologist who would become such a major part of our child’s life.

 

Knowing about his HLHS before he was born made us, at least, familiar with what was happening, even though it was certainly difficult. Kieran stayed in the NICU for 7 days, and I was able to breastfeed a few times, and pump milk for him the rest of the time. He was like a typical newborn baby, in that he wanted to be held, fed, and he slept a lot. As the days wore on, however, his oxygen saturation levels were getting lower and he was getting tired eating even a little bit. It was time for his Norwood operation, although his surgery was pushed back an extra couple days to make room for emergency cases.

 

Before Kieran’s Norwood, we met with a series of cardiology fellows, a cardiology nurse, and a cardiac anesthesiologist. They explained the procedure in great detail with diagrams. They told us that Kieran was a classic case of HLHS, and that they felt he was a good candidate for the Norwood. They explained that there were two common ways of doing the Norwood procedure: by doing a BT or a Sano shunt. The Sano shunt was the newer of these two methods, and had been developed by Dr Sunji Sano. The BT shunt was named after Alfred Bialock, Helen Taussig, and Vivien Thomas, who were pioneers in pediatric heart surgery (a movie called Something the Lord Made does a wonderful job telling the story of their work).

 

Our hospital, along with other facilities, had just concluded participation in a large study comparing the BT and Sano shunts, and while there were not large differences in outcome, it appeared that the Sano shunt had better short-term outcomes, especially for lower birth-weight babies. Our hospital had concluded their portion of the study a mere day before Kieran’s surgery. His surgeon would decide in the operating room which shunt to use. He ended up having the Sano shunt.

 

Following his Norwood, Kieran did very well. At first, the number of monitors, IV drips, wires, tubes, and patches was overwhelming.  My mother-in-law was visiting from out of state, and she thought to take pictures of the equipment in Kieran’s ICU room. Looking at these photos now, I am amazed by the amount of care and hard work that is invested in the treatment of HLHS. For the first night following Kieran’s surgery, the critical care doctors in the ICU did not take their eyes off Kieran and his monitors. They watched him so closely that I felt we must be in good hands.

 

Little by little, those monitors, wires, tubes, and patches began to disappear. For us, each one was a victory. It meant progress towards healing, sufficiency, and going home. And of course, we could start to see more and more of our baby under all of that equipment! After three days, Kieran’s chest incision was closed up. Thanks to online networking and support groups, we had been prepared to see Kieran’s chest open after the Norwood. I had seen surgical pictures online, which for the average person may be alarming, but for me, it was enormously helpful in knowing what my son would look like post-operatively. For the most part, the ICU nurses kept his chest incision covered with a blanket until it could be closed, but there were a few times they had to remove the cloth. There was a clear patch over his open chest, and I could see his heart beating away right in front of my eyes. Later that day, a visiting friend told me, “You’re one of the few parents in the world who can claim she’s literally seen her child’s heart.” That was comforting to me. As a new mom, there was a lot that was “abnormal” about our first days together. In some ways, the normal bonding process and mother-child relationship had been disrupted. But there was a special kind of intimacy in being with our son through this difficult process.

 

Kieran slowly started to eat by mouth again, just a little more each day. I was able to pump breast milk for him through the entire hospital stay, and he could drink some orally, as much as his recovering strength would allow. The rest was fed to him through an NG (nasogastric) tube. After 10 days in the ICU, we were moved to the “step-down” unit, where we spent two more weeks. Progress was not completely linear or constant, and there were days we felt discouraged by setbacks. One of nurses told us that this was completely normal, and that the journey “is a marathon, not a sprint.” As the inter-stage period (the time between the Norwood and the Glenn operations) is a period of a high mortality for children with HLHS, Kieran’s doctors wanted to be sure he was in the best possible shape before sending him home. There were some days where Kieran wouldn’t eat enough by mouth to satisfy the cardiology team, and he had unexplained tachypnea (rapid breathing). The hospital stay began to feel long because we could hardly wait to get home with our brand new baby. Since our stay there, the hospital has been remodeled, but at the time, we had to share a room with another family and only one of us could spend the night in the room with Kieran and his roommate. It was hard to have to say goodbye to Shawn every night, as one of us left for home and the other stayed by Kieran’s bedside. We did not qualify for staying in the Ronald McDonald House because we lived within an hour’s drive of the hospital. But our drive each way was 45 minutes, and this seemed far enough away to feel distant and lonely at night. We longed for the day we would all be under the same roof for the first time.

 

The days in the hospital, however, were busy. I had milk to pump, and in the step-down unit we were able to assume most of Kieran’s baby care—feeding, bathing, changing, and rocking. We had to pass infant CPR class, learn to draw up medications, and start recording his daily weight, oxygen saturation levels, and cubic centimeters of food in a binder. We had to learn to mix high calorie formula with breast milk so that he would get enough calories every day. Kieran had to pass his car seat test, to make sure his oxygen levels remained stable when he sat upright for long periods of time.

 

Finally, after 31 days in the hospital, we were going home! It was just after Labor Day, and the weather was thankfully still beautiful. During the interstage period before Kieran’s Glenn, which would be performed just a couple months later in November, we were supposed to avoid illnesses as much as possible, which meant not taking him around big groups of people if we could help it. Instead, we took him on long walks in the stroller on our local bike trail. Even at such a young age, you could tell he loved being in the fresh air, listening to the birds and the sound of the leaves through the trees. To this day, he loves the outdoors and wants to be outside as much as possible.

 

The 10 weeks or so between surgeries were a time of both gratitude and challenge for our little family. We were thankful to be out of the hospital and have support from so many, but we were also feeling the pressure of another open heart surgery and hospital stay looming before us. We had a lot to catch up on, and a lot to learn. There were a lot of “eyes and hands” on Kieran, and although we were out of the hospital, it felt like our life was not completely our own. When we say “it takes a village to raise a child,” when it comes to HLHS, that village is made up of many medical professionals! We had an in-home nurse visiting weekly, as well as an occupational therapist and physical therapist. We continued to record Kieran’s weight, feedings, and oxygen levels on a daily basis in the hospital’s binder. Every week, a pediatric nurse would call us to review the numbers, and if any of the numbers were amiss, we would be asked to bring Kieran in for a special pediatric or cardiology appointment. We got to know our local pharmacists very well, as well as our pediatrician and clinic nurses, lab technicians, cardiologist, hospital dietician and of course, our insurance company.

 

Before we knew it, it was time to schedule Kieran’s second surgery, the Glenn. Kieran had grown very quickly and put on relatively a lot of weight, so it was time by November. His Glenn was performed the day after Thanksgiving, when he was almost 4 months old. Although this was a shorter hospital stay—14 days—this surgery was more difficult for Kieran. Usually, the Glenn is considered a more straightforward surgery, but Kieran had problems with low oxygen and lowered heart function after the Glenn. No one is entirely sure why this surgery was harder for him, although one theory is that the incision made in the right ventricle for his Norwood may have somehow damaged his heart. After we were discharged, his decreased heart function continued to manifest itself over time, and finally, at a post-Glenn echocardiogram, his cardiologist became very concerned. Kieran’s heart was working too hard. The hard work was putting pressure on his tricuspid valve, which was moderately leaking, and his right ventricle was a bit enlarged. She told us that Kieran might not be a candidate for the next surgery, the Fontan, and that he might eventually need to be listed for a heart transplant. You can imagine how hard this was for us to hear—after all he had been through, it wasn’t working? Our cardiologist, however, decided to try two different medications, although she was somewhat doubtful that it would make a difference.

 

With sadness and alarm, we went home to try the new medication regimen. This involved adding a diuretic to his daily aspirin and blood pressure medications—one that is usually used only after the Fontan. She also added digoxin, which comes from the foxglove plant. Over the next several months, we waited anxiously to see if things could improve, and miraculously, they did! No one can quite explain how Kieran’s heart was able to improve, but at his pre-Fontan catheter many months later, he was found to be a candidate for the Fontan.

 

It is hard to live knowing your child will need another major open heart surgery. But I have come to the point where I am so grateful that he even had the opportunity to receive this very surgery. As “heart parents,” we do not want to see our children suffer, but we know our children need these life-saving surgeries to live their lives and fulfill their very special places in this world. And Kieran is definitely living his life! His life is different in some ways—he is on daily medications, he gets tired more easily than most children, he gets therapy to “catch up” with kids his age, and he has more doctor’s appointments and procedures than many children. In other ways, his life is “normal–” he goes to school, takes swim class, loves music, and plays outside. And yet, his life and his experiences are exceptional in so many ways—he inspires many around him with his strength, resilience, and unique personality. He’s brought us closer together with friends, family, neighbors, and strangers through his story and circumstances. He has completely changed our lives for the better, and for good.

kieranstory2

Thank You Volunteers!

Recognizing Volunteer Appreciation Week, April 6-12, 2014

It’s time to do some appreciating!!!  Thank you to the more than 100 volunteers who have stepped up to partner with PCHA.  Together, we are doing great things.

Grandfather and granddaughter holding hands

Last night, my son asked if we could “pay it forward” and buy the meal for the person behind us in the drive-thru.  The conversation eventually led to volunteering our time as a means to pay it forward.

I realized, then, how easy it is to apply the “pay it forward” principle to the CHD community.  As patients and family members, we have been through a lot.  But, most of us can recall a bright spot here or there.  Usually because some thoughtful person helped us out or did something nice in some way.  For me, it was a bag of Hershey’s Kisses quietly left on the doorstep by a friend.  It is these moments that make me want to pay it forward, to give back.

There are many ways to do that.  Some people donate money.  Others donate time.  Both are equally valuable to the success of the Pediatric Congenital Heart Association

When I asked my son what he thought it meant to volunteer, he described people who give up hours, days, even months of their lives to help others.  I would call those power house volunteers.

We have a few of those powerhouse volunteers who have given countless hours of their time to ensure the success of PCHA.

A resounding THANK YOU to:
David Kasnic – Chairman of the Board
Michelle Gurvitz – Medical Advisor
Gretchen Whitehurst – Education Committee Chair
Sara Schuh – Public Policy Committee Chair
Meredith Atkinson – Advocate Extraordinaire

Want to be a volunteer but just don’t feel you have the time?

There is GOOD NEWS!

Volunteering comes in all shapes and sizes.  There are volunteering opportunities that take just seconds! Sometimes it is the little things that matter most. When we each do our small part, it adds up to something big!

Here are a few ways you can pay it forward and volunteer for PCHA:

2-3 Seconds: Social Media – we are always looking for social media volunteers.  The easiest thing is to volunteer a few seconds each day to Like, comment, share and retweet our posts.  Doing so is a great way to spread awareness.

5 minutes: Advocate – we currently have a letter writing campaign and we need advocates to send an email to their legislators.  To send a quick email, get instructions here.

A few hours: Blog – We are currently looking for people who would be interested in sharing a little bit about their experience as a guest blogger. If you are interested let us know!

A few more hours: Leadership – PCHA has a couple of volunteer leadership openings.  These positions require an application and a little bit more in-depth orientation process, but if you are interested let us know!

And, of course, we need to thank those who give of their money, too! Thank you to our incredible donors who ensure our success through their generous financial support.

“Volunteers do not necessarily have the time; they just have the heart.” – Elizabeth Andrew

Thank you to all of you who give!

Gregory Guzzo

Gregory Guzzo photo

Gregory has a congenital heart defect, yet we never knew until a few months ago. Gregory is an active child and 13 years old. He wrestled for 5 plus years, played soccer, never sits still unless he is playing XBox and even then he is standing and jumping around.

Out of the blue, as he was going for his wrestling physical at school in Oct of 2013, he fails. The school physician calls and says he heard what seemed to be a murmur and he felt it was significant enough to require an ECHO and I should have him seen by his Pediatrician and Children’s Hospital only to be diagnosed with Atrioventricular Septal Canal Defect, a congenital heart defect, a condition he has had from birth.

Atrioventricular septal defect (AVSD) is a multiple heart defect that involves the valves between the heart’s upper and lower chambers and the walls between the chambers. In children with AVSD, the tricuspid and mitral valves do not develop normally which then affects the growth of the atrial and ventricular septum. There is one “common” valve separating the hearts upper and lower chambers (1)(or cleft) instead of two separate valves. In addition, there is a hole in the atrial septum (2) called an atrial septal defect (his is half the size of the wall itself) and a hole in the ventricular septum with a left to right shunt (meaning the blood is lost threw the hole)(3) called a ventricular septal defect. He also has a right bundle block…which means his heart is thickened for being over worked and the electric currents have to work harder to get past the dense heart muscle.

We were informed to correct these defects Gregory was to undergo Open Heart Surgery. This was not optional. The surgery went well, and he is recovering nicely, however in Gregory’s own words, he is “FIXED not CURED”.

Since the surgery, we have established a foundation (Gregory’s Heart Warrior Foundation) to educate, promote awareness and provide charitable contributions to hospitals and families in need with CHD. We have also work with local and state legislatures to receive proclamations for CHD Awareness with the hopes of funding for research. We work in conjunction with Children’s Hospital of Pittsburgh’s Heart Institute Auxiliary Board for funding for research and we utilize social media to express the seriousness of the CHD’s with facts, statistics and CHD news. The best part is Gregory, himself speaks on behalf of his condition, he is his own advocate and the voice for so many children who are too young to express what they are going through or have lost their battle with the number 1 killer in deaths due to birth defects.

We will continue our fight for awareness, early detection, hope for a cure… as going un-diagnosed for 13 years is unacceptable and we want to prevent this from happening going forward. If we save one life, if we spread the awareness for CHDs and one child is helped… then we have done something good.