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.