Human Guinea Pigs OR Helpful Pioneers?

I never have liked the term "guinea pig" when referring to human beings. To make matters worse, it is usually used in a negative way and has a negative connotation. Whether we like the term or not, the term "guinea pig" simply means "somebody or something experimented on: somebody or something used as the subject of an experiment, test, or trial" according to the Bing dictionary. It really doesn't have to be considered something negative; however, I would like to recommend that instead of using the term "guinea pig" instead we use the term "helpful pioneers"!

Pioneers paved the way for others to live a better life. They endured hardships to prevent future generations from having to do likewise. Pioneers were brave and faced uncertain futures. Don't you think that is a more fitting description of the good-hearted souls who take part in clinical studies to progress our current knowledge of whatever subject is being studied?

Today I'd like to discuss how our children and/or teens and/or adults born with congenital heart defects can become helpful pioneers and help the medical profession learn more about what it means to have a congenital heart defect. Since so many babies and children died before reaching the teenage years (much less adulthood!) there is little normative data on individuals growing up with congenital heart defects. 

I found a terrific site that lists the different research projects that are going on by state (for those of you readers who live in the United States)! Here is the link: Clinical Trials Involving Congenital Heart Defects. 

We have an aging congenital heart defect population. In order for current and future generations to know what to anticipate, we must have research conducted to determine what is "normal" and what the range of "normal" might be. Gaining this knowledge is one way we can better prepare for the future and try to prevent the anxiety that accompanies not knowing what we are facing. It is not knowing and wondering if what is happening to your child is commonly happening to other children that makes parents so anxious. The same is true once our children grow up and start experiencing certain symptoms. Knowing that others with their same diagnosis commonly share their experiences makes people feel less alone and afraid. Learning what to do when certain symptoms appear, empowers our CHD survivors to be better advocates for themselves and helps them make better choices for optimal care. 

If you don't see a clinical trial you would like to participate in, why not share the information with others you know who could be helpful pioneers?

Please visit Baby Hearts Press at http://www.babyheartspress.com for resources for the congenital heart defect community.

A New Direction for Baby Hearts Press

Dear Heart Friends:

What has Baby Hearts Press (a.k.a. Anna and Frank Jaworski) been doing for the last decade and a half? We've been publishing books and resources for the congenital heart defect (CHD) community. When the Internet was young in the early 1990s, almost everything we found online about children with congenital heart defects were created by bereaved parents. There were many tributes to heart warriors taken too soon. There wasn't much information online that provided hope to the CHD community.

What was worse was that publishers considered our community "too small" or "niche market" and thus they weren't interested in publishing books to help our community, although they wished us luck and directed us to other publishers who also turned us down. That's how Baby Hearts Press was born. We found a book on self-publishing, started Baby Hearts Press and haven't looked back since. For a long time our books were among the few books that gave parents information on a level just about anyone with a high school education could read and understand, but more importantly, the books provided real stories that hadn't been "Hollywood-ized" and instead were told by real people affected by CHDs in their own words -- the words of grandparents, parents and adult survivors.

Now everything has changed. The world is much faster paced. There are many, many pages of hope on the Internet for CHD families, supporters and survivors. Hospitals provide all kinds of information for the CHD community, some even provide videos or beautiful 3-D graphics which help to explain heart defects which seemed incomprehensible before. Big publishers have started taking notice of our growing community and there are textbooks and other books that are now available which paint a picture of hope for everyone.

Our son, Alexander, has also grown up. So we found ourselves at a crossroads and then we received a phone call that would change our lives. The call was from Josh Bernstein, an Executive Producer with VoiceAmerica. After having seen Anna's websites on the Internet, he called to offer Anna her own radio show -- a show specifically for the CHD community. This would be a chance for us to continue our community outreach and it would also be an opportunity to share our books with the CHD community.

Anna just got back from Phoenix, Arizona where she met her VoiceAmerica team! Here is a photo of her with Josh and Brandy Jackson, General Manager of Voice America.

Unfortunately, Anna didn't get photos with all of the members of her team, but here is her Trainer and Sound Engineer for Episode 2 and 3. The Trainer was Randy Jackman and his brother, Justin was Anna's Sound Engineer.

Justin, Anna and Randy

Perhaps the most exciting part of the trip, aside from meeting all of the wonderful people who make VoiceAmerica what it is, was the fact that Anna actually taped the first 3 Episodes of Heart to Heart with Anna. Here is a photo of Anna in the studio after her first Episode was complete.

Thanks to the generous support of the CHD community, Anna had 12 Guests on the first 3 shows which will begin airing on Tuesday, November 12, 2013 at 2:00 p.m. Central Time. The first three Episodes and Guests are:

Episode #1: You Are Not Alone with Guests: Nancy and Jesse McCain, Callie Rickard and Carl Wolford

Episode #2: Post Traumatic Stress Disorder, Anxiety and Survivor's Guilt in the CHD Community with Guests:  Lauren Bednarz, Yasmin Southwood and Bryce Bagwill

Episode #3: Organ Donation and Transplantation with Guests: Eileen Pearlman, Jessica Cowin, Emily Wiebke, Kathy Keller and Anthony Pugliese

All of the Guests did an excellent job of providing information and sharing their experiences with the listening audience. Here's how powerful their stories were -- after Episode #1 was completed one of the VoiceAmerica employees found Anna eating some soup in the kitchen and proceeded to tell her that he'd heard about the show we'd just completed. He went on to share that his niece was born with a serious heart defect and needed surgery in her first days of life. He couldn't wait to tell his brother about the show because he wanted him to know that he was not alone.

We haven't even aired but we have already touched someone's life. Now you see the new direction Baby Hearts Press is taking. We hope you'll join us! Please LIKE us on Facebook!  Heart to Heart with Anna  If you'd like to receive an eCard about the show, please send Anna an email at Anna@BabyHeartsPress.com and give her your preferred email address.

We are looking forward to hearing from you all soon!

(The front door of VoiceAmerica, a.k.a. World Talk Radio)

Please visit Baby Hearts Press at http://www.babyheartspress.com for resources for the congenital heart defect community.

Who Needs Prophylaxis Antibiotics Anyway?

When a mother and father are given the news that their baby has a severe, congenital heart defect (CHD), they are given a lot of information that needs to be discussed again and again as the child ages. One of the areas that my son's cardiologists have been good at reviewing with us has been the need for taking medication in anticipation of dental procedures -- which is known as prophylaxis antibiotics by the doctors.

What are doctors worried about? A horrible, possibly life-threatening condition known as "infective endocarditis" is an infection of the endocardium (heart muscle) that usually involves the valves and adjacent structures and is caused by a wide variety of bacteria and fungi. Although infectious disease clinicians may be familiar with this disease, general internists may not have much exposure to this condition, therefore it behooves parents of children with congenital heart defects to understand and prevent this condition if at all possible. (See http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/infectious-disease/infective-endocarditis/ for more information, but be prepared; some of the photos are pretty graphic.)

Since infants aren't receiving dental cleanings, taking antibiotics before cleanings doesn't become an issue for many of us until our children have recovered from at least one open-heart surgery and in my son's case, it wasn't necessary until after his fenestrated Fontan (since his surgery was done at 9 months of age). The good news regarding that situation is that our CHD babies have time to heal from their cardiac surgery before worrying that something as mundane as a dental cleaning could cause an infection that could cause a hospitalization, or worse -- more surgery or even death.

Why should parents worry about whether or not their children need antibiotics before dental cleanings? The simple answer is because parents of babies with severe, congenital heart defects are dealing with a medical condition which transcends their hearts and has the chance to affect them in other non-cardiac areas as well. A prudent parent is always on watch against potential problems that could be prevented with something as benign as a dose of penicillin before a dental cleaning or some other procedure.

The American Heart Association has been very good at providing wallet cards for patients (or parents) which outlines the people who need to be aware of the guidelines to prevent infective endocarditis (http://www.heart.org/idc/groups/heart-public/@wcm/@hcm/documents/downloadable/ucm_307684.pdf)  My son's cardiologist frequently asked Alex if he needed a new card for his wallet whenever he had his regular visit (every 6 months). What should perhaps cause us concern is that these guidelines are based on logic and not on quantifiable research. That is why I was so interested to read a study actually looking at cumulative incidence and predictors in CHILDREN with congenital heart disease (http://circ.ahajournals.org/content/128/13/1412.full.pdf+html). The reason there haven't been more studies like this is because of the enormity of the task in gathering the information needed to make statistics meaningful. Thankfully, the incidence of infective endocarditis is relatively small (between 2 and 6 cases per 100,000 person-years), but the incidence in patients with underlying valvular heart diseases is greater.

The study referenced above fascinated me because it looked at all of the patients in the Quebec CHD Database who were children 0-18 years of age between January 1, 1988 and March 31, 2010. The authors did a good job of explaining how the data was gathered and analyzed and even detailing possible flaws with the study. What was most interesting were the results:

They found that children most at risk for infective endocarditis were the following:

• children with cyanotic CHD lesions
• children with left-sided lesions
• children with endocardial cushion defects
• children recovering from open-heart surgery, especial during the 6-month postoperative period and in children 3 years of age and younger

This study confirmed the AHA guidelines for giving antibiotics before dental procedures or other surgical procedures but it found that the relaxed AHA guidelines (from 2008) may have left out children who suffered from some of the conditions above who had not had surgery, and especially the patients with left-sided lesions or endocardial cushion defects. My son suffers from left-sided heart lesions (hypoplastic left heart syndrome), and even though the AHA relaxed its guidelines in 2008, my son's cardiologists always insisted that Alex continue to take antibiotics prophylactically and I'm thankfully they did! This study shows that people like Alex are more at risk for developing infective endocarditis than other CHD children who don't have the conditions listed above.

We need more studies like the one done in Quebec. If we can get funding for the Congenital Hearts Future Act, then we will start to develop a database of information like the one that the Canadians can refer to and we will be able to gather information like that in this study here in the United States. The fact that this study questions some of the AHA Guidelines for prevention of infective endocarditis is indicative of the need for more research. Research like this is much easier to do if we have the data in a national database. Let's keep pushing for the United States Congress to provide funding for the Congenital Heart Futures Act so we can continue to modify the AHA guidelines when needed so our children, and aging population of adults with CHDs, won't be at risk for infective endocarditis.

Please visit Baby Hearts Press at http://www.babyheartspress.com for resources for the congenital heart defect community.

World Heart Day

Dear Heart Friends:

Since 2000, the World Heart Federation has instituted September 29th as World Heart Day which is a day around the globe to inform people that heart disease and stroke are the leading cause of death. This year the theme for World Heart Day is Take the Road to a Healthy Heart.  http://www.world-heart-federation.org/what-we-do/awareness/world-heart-day/about-world-heart-day/

Although this effort does not necessarily highlight congenital heart defects, it still brings awareness to a very important issue -- we only have one heart and we have to keep it healthy! This is even more important for those of us who have children who were born with less-than-perfect hearts. In addition to having congenital heart disease, our children can also develop acquired heart disease. It's our job as advocates of the heart, to encourage people to take a road to a healthier heart -- walking, exercising, eating right and just generally reducing stress in our lives. All of these actions taken together will ensure that everyone has a healthier, happier heart.

I love how they created a flyer to encourage children to exercise. I especially love how the hero has a cape and a costume depicting the heart. It's very cute. The flyer/poster also has the character telling fast food to "beat it"! It encourages playing soccer instead of being a couch potato. These are all images children need to see and hold dear.

http://www.world-heart-federation.org/what-we-do/awareness/world-heart-day/infographic/

Go to the link above for an excellent infographic on congenital and acquired heart disease. They really did a good job of showing, in a pictorial way, how your heart can be affected from birth and throughout one's lifetime. It gives statistics on many heart issues, including that 1,000,000 babies are born annually with congenital heart defects worldwide. This is too large a number to ignore!

I hope all of you had a wonderful World Heart Day and that you all treat each and every day as a chance to celebrate the heart you have and follow the road to a healthy heart.

Please visit Baby Hearts Press at http://www.babyheartspress.com for resources for the congenital heart defect community.

Can One Person Make a Difference?

When we are young, many of us dream of making a difference in the world. Whether this difference is due to the career path we choose or the hobbies we are passionate about, we feel that our efforts can change the world, or at least our little corner of the world. As we age, it's not uncommon to get so busy with the business of dealing with life, especially once we marry and have children, that it's not uncommon for that dream of making a difference to be relegated to the back burner and seldom, but wistfully, thought of.

Can one person make a difference? Of course he or she can! The Internet is full of speeches about people who have done so (check out TED Talks for some great ones!), libraries have books full of stories about people making a difference and it is the fodder for many great sermons given on any given day in any given house of worship. So why is it that most people might believe that someone else can make a difference but certainly, not them? What is it that makes some people make the extra effort to actually do the work that makes a difference?

I believe the ingredient that is necessary for a person to really make a difference is passion.  If people are passionate about their beliefs and steadfast in their efforts to really do something to make a difference, they will make a difference.

Let's look at three women in the congenital heart defect community who have made a difference, what they did and how you can be part of their movement to help others!

Deb Hilton 

Deb Hilton is the mother of a child with a severe, congenital heart defect. She has made it her mission to help doctors understand the importance of their communication with parents when they are diagnosing severe, congenital heart defects and making suggestions for treatment. In order to help doctors understand, not only her story but the story of many others, Deb conducted a survey of parents in the congenital heart defect community. She then took her information, analyzed it and has written two different articles that have been published in Pediatric Cardiology -- a journal for doctors!
The titles of the articles are:  How Not to Tell Parents About Their Child's New Diagnosis of Congenital Heart Disease: An Internet Survey of 841 Parents and  Prenatal diagnosis of hypoplastic left heart syndrome: impact of counseling patterns on parental perceptions and decisions regarding termination of pregnancy(for links to the full articles, go here: http://chdresources.homestead.com/PublishedArticles.html)

Kristine McCormick

After the loss of her precious baby, Cora, Kristine McCormick became a mother on a mission. Cora died in Kristine's arms due to an undetected congenital heart defect. Kristine started raising awareness about congenital heart defects and the importance of screening newborns before leaving the hospital. There is an inexpensive test that can be given to babies, it's non-invasive and very quick, called pulse oximetry. Kristine has been advocating for this test to be required before newborns can leave the hospital. Had Cora been given this test, she might still be alive today.  Cora's story so influenced her U.S. Congressman, Senator Brent Waltz, that he proposed a law be passed in Indiana requiring newborns to have pulse oximetry performed on them before they are discharged and he called it Cora's Law. You can read more about it here:  http://corasstory.com/coras-law/

Michelle Rintamaki 

Usually Michelle likes staying in the background helping others and not drawing attention to herself, but I'm going to use her as yet another example of a mom on a mission and making a difference. For almost two decades Michelle and I have been working hard to bring resources to the CHD community. Michelle has amassed the most comprehensive clearinghouse of resources of interest to the CHD community on the Internet. Her support group, Kids with Heart, has done parent matching and holds annual events for those in the Wisconsin area. Michelle is another example of a person who has made a difference, albeit quietly and without the fanfare that Kristine and Deb have garnered. Considering the thousands of people Kids with Heart (with Michelle at the helm) has helped, her contribution is significant.  To purchase resources from Kids with Heart, go here:  http://kidswithheart.org/


Whether you want to create a survey to develop a database of information worthy of writing articles for medical journals or whether you're willing to share your story and possibly influence lawmakers to create laws that could save lives or whether you're willing to gather resources to help others, these are just three ways an ordinary person (with an extraordinary passion) can help members of the CHD community. Any one of us can make a difference in our world. It just takes passion, a mission and determination to see a project from start to finish. You, too, can make a difference, or you can contact one of these ladies to find out what you can do to help them with their mission.

Please visit Baby Hearts Press at http://www.babyheartspress.com for resources for the congenital heart defect community.

Connections

Have you ever met anyone you've communicated with only over the Internet? Were you afraid? Excited? Nervous?

When the Internet became a phenomenon in the 1990s, people worried that the Internet wouldn't be good for social interactions and that people would just sit in front of their computers choosing not to even talk to people in the same room. People worried (in some cases rightfully so) about meeting people who were not who they appeared to be online. There were a lot of warnings against meeting Internet friends or at least precautions that should be taken.

My experience has perhaps been a bit extraordinary. To be fair, most of the friends I meet in person are from the congenital heart defect (CHD) community. We have shared very intimate experiences with each other regarding the life and possible death of our children. We often share photos of our children in the hospital. We cry with each other over our frustrations and celebrate with one another over our little triumphs. We are like family. So is it any wonder that meeting these friends face-to-face has been nothing but joyful?

Here is a photo of my heart-healthy son Joey with me in Washington, D.C. I was so excited to finally meet a mother who called me about my first book, Hypoplastic Left Heart Syndrome: A Handbook for Parents, about 16 or 17 years ago. Barbara McFadden and I had talked on the phone for quite a while about our children with HLHS. It was so exciting to meet Tara -- all grown up and doing great! -- and to give Barbara a hug. All of us were in D.C. to be advocates for the CHD community along with the Adult Congenital Heart Association and Mended Little Hearts.

I will be doing a bit of traveling the latter part of this year so I once again let my heart friends know where I would be and asked if any of them wanted to get together. I am overjoyed that it appears I will finally have a chance to meet the mother of a daughter with HLHS who I have communicate with for a good while. The daughter is an adult and is taking part in the Hypoplastic Hearts book that I hope to eventually get back to (after I finish updating/retooling my website and preparing for my Internet talk radio show!). I am eager to work on the book project because I want to know what kind of situations adults with single ventricle hearts face as they age. Knowledge is power and the more we understand about what happens as a single ventricle heart ages, the more prepared we'll be to deal with whatever happens.

One of the most active Facebook groups I belong to is Heart Mamas. I love that group! We have so much fun sharing, advising, praying for one another, encouraging one another -- it's like being part of a community of mothers, sisters and aunts who all love and care for each other. It's probably the most nurturing group I belong to and I'm so excited that I'll have a chance to meet some of my Heart Mama friends when I go to Chicago in November.

One of the worst parts about finding out your child has a life-threatening, congenital heart defect (CHD) is feeling alone. One of the amazing outcomes, after you reach out to others, is realizing that far from being alone, instead you belong to an surprisingly special community. The CHD community is full of wonderful people who reach out to strangers and help each other through our darkest times. No one understands us like we understand each other. By helping each other, we end up helping ourselves and, most importantly, we realize that we are not alone.

Please visit Baby Hearts Press at http://www.babyheartspress.com for resources for the congenital heart defect community.

Changes

Accepting change is one of the most difficult tasks we face as adults. As children we can't wait for change! To be a year older or a head taller in order to do things like ride on a roller coaster or play certain games are goals of the young child. The funny thing is that once we age or become taller there is no stopping the changes that occur. As adults mature they may not grow taller, but they may become rounder or at least physically different than before. As adults we realize it's not the changes that occur on the outside that are most important. Instead, the changes that help us to grow on the inside to become better people are what's most important.

Over the years I've watched many changes occur in the congenital heart defect (CHD) community. When I first got dial-up Internet in the 1990s there were few pages by CHD parents. Most of them were tributes to CHD angels -- children taken from this earth way too soon. Back then there was no MySpace, no Facebook no YahooGroups. The way we CHD parents connected was by chance a lot of times until we found a way to get better organized. Back then, AOL ruled the Internet as a form of communication. I remember actually being excited when I heard the familiar voice come from my computer saying, "You've Got Mail!"

AOL had chatrooms and we CHD members, mostly parents and grandparents, would frequently set up chats with each other. We even got organized enough to have a Friday Night Chat where we talked about the challenges we faced, the questions we had but had forgotten or didn't have the courage to ask our children's doctors and where we celebrated small triumphs that other family members and even friends couldn't possibly understand. We talked about the "forbidden topics" like spanking, whether we should let our babies sleep in bed with us and breastfeeding the baby with a CHD (how do you measure how much milk they got?). As our children aged, the needs of our core group changed and we had other duties on Friday nights so our chat just faded away.

Many of us changed from being the parents of toddlers or young children to the parents of elementary-aged children or the parents of angels. The angel parents went off and formed their own group. Those of us with older children sometimes found ourselves so busy that we actually didn't need the intense support we had needed when our children were between surgeries or just recovering from an open-heart surgery. Some parents believed that their children had finally been "fixed" and they no longer needed our group. Others had other children to attend to and so our group dynamic changed over time just as our needs changed over time.

In the 1990s and early 2000s there was a great need for books. There was so little written on CHDs for the non-medical person that I received many letters from people thanking me for what I had written and published. Finally they read stories like their own. They had heart brothers and sisters -- other people who understood the challenges they faced. They were not alone!

One surprising change was to see the printed book give way to electronic books. The Kindle and the Nook changed the publishing world and it will never be the same. Some changes are good changes! I remember in 1998 receiving a phone call from a gentleman in California. His best friend was in the hospital and his baby daughter had hypoplastic left heart syndrome. The man desperately wanted my book Hypoplastic Left Heart Syndrome: A Handbook for Parents so I told him I would put one in the mail first thing in the morning. That wasn't good enough for that gentleman. This was before PDFs were invented. This was in the day before Internet service like what we have today. This was when, if you wanted printed material in a hurry, you used a fax machine. This gentleman insisted I fax him the pages from my book that showed the first open-heart procedure and Alexander's Story (the first chapter in the book). I remember feeding page after page into the fax machine and feeling lucky that we had that tool as a resource! How antiquated that process seems today!

Our CHD groups have changed, too. We've become more sophisticated, more knowledgeable. There was a time when I was actually scolded by more than one doctor for wanting to understand my son's heart defect better. The doctors told me that they were the experts and that I should trust them and not question what they wanted to do to my baby. My heart friends today would be furious with those doctors! Doctors today expect parents to come to meetings with questions and pages printed from the Internet with information they want to understand regarding their children's conditions. That is definitely a change for the better!

Instead of meeting in chatrooms like we did in the olden days, now parents set up CarePages, Caring Bridge pages or Facebook Pages devoted to their children. We often use our abbreviations for our children's heart defects to help us find each other -- so it's not uncommon to see HLHS or TOF somewhere in a person's name or on their page. In fact, there are YahooGroups and Facebook Groups devoted to certain heart defects where parents can feel free to talk about issues of common concern and where they can use jargon or abbreviations that everyone understands.

Before MySpace and Facebook we used to have listservs. Yes, there was no "e" at the end of the word -- which was something that drove me crazy as an editor! But our listservs were our lifeline. They were the places we could talk about our fears, our experiences and our frustrations with a group of other people who really got it and who didn't respond with the platitudes or silence that our family members and non-heart friends did. They supported us, cried with us and offered helpful advice. They consoled us. They helped us to know that we weren't alone. 

In the future we'll have yet one more change to look forward to. Now in addition to Facebook, CarePages, Caring Bridge and other places where members of the CHD community can come together, we'll have a weekly radio show to bring us together! The radio show will also address issues of significance to the heart community. It will be a safe place where we can celebrate triumphs, overcome obstacles and rejoice in knowing that we aren't alone. I can't wait to see how the radio show will grow and change, just as I've observed these so many other changes in communication over the years.

I started with a quote and now I will end with another quote:  

I'm looking forward to reaching new heights with you, my dear heart friends! Tune into Heart to Heart With Anna Marie Jaworski: From My Heart to Yours on VoiceAmerica starting November 12th!

Please visit Baby Hearts Press at http://www.babyheartspress.com for resources for the congenital heart defect community.