Families’ Stories with CHD
by Loleta Snow (Brady’s Mother)
My Personal Journey with CHD
Written by Dana Hageman (Mother)
In 1997, my daughter, Allison, was born with a Congenital Heart Defect called Pulmonary Atresia with Intact Ventricular Septum. Within 2 hours, she was being transported to Arkansas Children’s Hospital. She was placed on special medications to keep her heart functioning as it had been in the womb, and on oxygen to further increase her oxygen levels. She had her first Open Heart Surgery at 2 days old (July 1997), and her second open heart surgery at just under 5 months old (Dec 1997). These surgeries allowed her to function with a “free-flowing Pulmonary Opening” where her Pulmonary Valve should have been. The upside to this was that her ventricle and her body would have time to grow and develop; the downside is that there was no valve to prevent backflow of blood through the pulmonary opening, gradually creating possible further damage to the ventricle and to her tricuspid valve from the increased flow and pressures. This was monitored at her regular cardiology check ups, complete with EKGs and Echocardiograms. She was otherwise able to lead a relatively normal healthy life through her preschool and elementary years. Her cardiologist told me several times that Allison was “the exception to all the rules” and that she was amazing.
In ’97, when Allison was born, my husband and I were told that this CHD was not hereditary. We were told that there was no “known cause” and that we should not have any worries about future children. The general statistic is that there is a 1% chance of a CHD in normal pregnancies. Over the 10 years after Allison was born, the statistic was adjusted to a 2%-5% chance IF YOU ALREADY HAD A CHD CHILD. (New notes: Certain forms of CHD are NOW known to have a much greater chance of being genetic than others)
However, when I became pregnant with my son, Arley, in 2007, my OB/GYN had NO concerns about me being at an increased chance for a heart defect. I disclosed all of Allison’s information to him. He performed regular ultrasounds. He discussed amniocentesis, as I was “on the upside of 35″. NEVER did he mention, advise or imply that I MIGHT need to consider having a fetal ultrasound done. Never did he advise further testing or express concern regarding increased chances for CHD due to my already having a CHD child. I do not believe he was incompetent, I believe the general knowledge is just not out there. I know of many OB/GYNs who would have had the same opinion and would also not have referred for further testing. This is also a situation which needs changing, requiring Better Education regarding CHDs and Better Guidelines for when to refer to a cardio specialist.
Just one week before Arley was born, Allison was seen by her regular cardiologist for a check up, and the cardiologist was surprised to learn that I was extremely pregnant, and asked about what testing had been done. I felt that she was somewhat concerned that I had not been referred for testing, but did not wish to distress me. One week later, I gave birth to Arley, my third child, my second CHD child. Specifically, my second child with PA-IVS.
Alli and Arley now share that same wonderful cardiologist! Arley’s initial treatments went almost identical to Allison’s. Special medications and oxygen kept him alive for the first several days of his life. Catheter advancements since Allison’s birth had made it “POSSIBLE” that his condition might be treated with catheter interventions. This process was attempted, but was not successful. Therefore, Arley had his first OHS at 6 days old (Nov 2007) , and his second OHS at 10 months (Aug 2008). He has had MULTIPLE catheter procedures in the last 4 ½ years. He has some complications that Allison did not have; most importantly, he has severe Bi-Lateral Branch Pulmonary Artery Stenosis. His LPA (left Pulmonary Artery) has been patched, and was recently stented (Feb 2012). His RPA (right PA) has been stented at the lower lobe branch (Nov 2010) and this stent was expanded in the Feb 2012 procedure. His RPA may also eventually need to be stented at the upper lobe branch as well, although we are hoping that the recent interventions will delay the need for further procedures for another 2 – 3 years. He will almost certainly eventually need a new Pulmonary Valve, just as Allison eventually needed one.
Five months after Arley was born and had his first surgery, and approximately five months before his second surgery, my daughter, Allison, had her third OHS (March 2008). She now has a prosthetic pulmonary valve. 10 years before, this valve implant would have meant that she would be destined to have further OHS, at regular intervals, to replace each prosthetic valve as it degrades or is outgrown. Due to research advances, her valve can now POSSIBLY be replaced (multiple times throughout her life) by catheter procedures, which is far less intrusive and traumatic than the previous open heart options. My daughter’s valve was the first of its kind placed by her surgeon at Arkansas Children’s Hospital (ACH). Over the last 4 years, medical research has led to even further valve advancements, offering many new options for some patients.
My son’s RPA stent was placed in November 2010 via catheter. Prior to this time, the cardiologists had expressed Arley’s NEED for a stent, but they had also expressed a reluctance to place one until he was much larger, as his expected growth would also mean outgrowing the stent, bringing the need for an OHS to remove and replace it. The new technology of the stent which he eventually received allows it to be expanded by catheter, so unless he reaches giant proportions, that stent should not be the reason for another OHS. This specific stent technology had just been introduced to ACH when Arley’s stent was placed. Since then, in February 2012, he has had another stent placed via catheter procedure AND his first stent was expanded via catheter procedure. These are all options now available due to medical research, medical research funding and recent advancements.
In Summer of 2011, Allison began having problems with extreme fatique, then with appetite and weight loss, and eventually began having spiking high fevers and extreme headaches, then came a chronic little cough. All of these symptoms “grew” over a period of weeks, between late June 2011 and August 2011. Unfortunately, different General Pediatricians failed to put all of the symptoms together and create a big picture. Finally, one very concerned and attentive Gen Ped made the effort to call in an Infectious Disease specialist. By this time, in September 2011, Allison had gone from a healthy active 101 pound teenager to a lethargic, extremely pale, 82 pound invalid. Getting out of bed to eat and drink were major efforts for her. Her “bloodcounts” were practically non-existent. She was hospitalized and finally diagnosed with an infection that had decimated her system. Special multi-antibioitic treatments were prescribed, and Allison slowly recovered. However, even though her blood counts are back to normal, she’s gained back her weight and her heart does not appear to have any permanent damage seen in our normal tests, she is still not back to the normal activity levels she had prior to this infection. It is heart-wrenching what a basic infection can do to a child with any kind of compromised system. I cannot say how much I appreciate the support of other CHD parents telling me to “not give up”! That if I felt that there was something terribly wrong with my child, to keep pushing, and keep asking, and continue looking for answers! That is a part of what support is all about.
I have much to say about medical research, medical advances, and how much of a difference that research is making in our CHD world. Each of these advances mean a higher quality of life, and less surgical risks for my children, and THOUSANDS of other children! Please help support CHD awareness and research.
I have learned a lot about medical research and reference through necessity. I have, through my own research, found two, and possibly three, references to familial PA-IVS cases involving siblings over the past few decades, and I have initiated contact with members of two of the teams on those research cases. There is interest in furthering this research. There is local interest in furthering this research as well. I believe my children ARE the exception to the rule; they can provide the answer to some of these questions; and there is a reason for our situation. It will just take the right person to find that answer. It will just take me finding that right person, or them finding me. Finding our answer will lead to more answers for others, and someday, those answers answers will hopefully lead to prevention studies.
Too many parents have gone through the nightmare of living in a CVICU waiting room while their child is struggling to live. We are many, and we are everywhere. There is no reason for any parent to go through this experience alone. There is a need for sibling support, and ways to integrate siblings into the CHD world with (hopefully) less trauma. My two CHD children have obvious scars. Their older sister, Tasha, has scars that are unseen, but are also important to be tended to and acknowledged. We are intent upon spreading the word about our existence, spreading word about the prevalence of CHDs, and spreading the word that more support for awareness and more funding for research is needed.