Parenting a Heart Kid

This is not William's X-ray, but it is an image
of someone with Scimitar Syndrome.

First of all, it turned out to be little more than a fragile, superficial vessel that popped and bled. William’s fine and we, his parents, are thankful to be nothing more than overly tired after a late night in urgent care.

But when your asthmatic, heart kid starts coughing up bright red blood after a coughing episode, you try hard not to work yourself into a panic. That’s what happened last night.

William was minding his own business, taking a shower, and abruptly started coughing. OK. Not unexpected: he has mild/moderate, chronic asthma, and it’s been triggered and exacerbated lately by a lingering respiratory virus, brutally hot weather and intermittent exposure to the smoke from Florida’s wildfires.

When my husband went to check on him, though, he was caught off guard by the bright-red, kind-of-big splotches of blood coming out when William coughed. And it kept coming. Husband went bonkers, scared witless. I tried hard to be just a “regular” parent – ah, he probably just popped a vessel somewhere, or maybe it’s really just a nosebleed traveling down his throat. But I’ll call Capital Health Plan Urgent Care; we’ll see what they want to do.

When I talked to the nurse, I told him my 7-year-old son was taking a shower and started coughing and then there was blood. The nurse didn’t seem overly concerned and asked if my son had been sick.

“He’s had a cough on and off for a few weeks. Dr. Jones saw him a week ago Friday and his lungs sounded a little junky, but when we went to see his pediatric pulmonologist that Monday at Shands, she said he sounded really good,” I told him casually.

I was not going to panic. Just because he’s a heart kid does not mean it’s a big-deal serious thing. I was going to play my “he’s just a regular kid” card, even if I died a little inside doing it.

“Oh. Um, why does your son see a pulmonologist at Shands?,” the nurse inquired.

“He has chronic, but mild asthma, and he has scimitar syndrome. He’s really fine, though. He’s been very healthy this year,” I explained.

“What was the name of the syndrome again?,” the nurse asked?

“Scimitar syndrome. It’s part of his partial anomalous pulmonary venous return, a heart defect,” I said.

“Oh. Has he ever coughed up blood before?,” the nurse asked.

“Years ago when he was a baby and had pneumonia, and then I think it happened once after he had his heart repaired several years ago,” I answered.

“Oh! How far do you live from our clinic?,” he asked.

“About 10 minutes or less,” I answered. “If I have to choose between going to an emergency room or waiting to see our pediatrician in the morning, I will choose to wait and see our pediatrician. I think he’s fine.”

The nurse put me on hold. A minute later, he came back and said, “We’ll wait. Please come now.”

And we did. We saw Dr. Henry Gunter, whom we’ve seen before, and he’s a wonderful, kind and patient physician. He had read William’s chart. He knew William had asthma and he knew he had a “great vein anomaly.”

Dr. Gunter checked William’s ears, eyes, nose and throat. His throat looked red, now raw, but like it had been painted red. Dr. Gunter decided William probably had popped some small vessel in his throat, but just to be sure, he sent William for a chest X-ray.

Aside from the wire that has popped open at the base of William’s sternum, everything looked fine. And even the wire isn’t a big deal. Dr. Gunter was gracious and glad it was nothing more than a popped vessel. So were we!

I give myself credit for appearing to be just a calm, typical parent last night. But I won’t deny that the heart mom’s little voice kept whispering in my ear, “He’s probably fine, but he’s a heart kid with respiratory issues. You can just never really know. The fact is, you might be home in an hour or you might be admitting him to the hospital. You just never know.”

That’s what it’s like. That’s how it feels when you’re the parent of a child with a congenital heart defect, or any other chronic illness. You work hard every day to be just another parent; but there’s always a little piece of you somewhere feeling a twinge of panic when something out-of-the-ordinary occurs.

Because it’s true, you really never know for absolute sure.

Always Grateful

My now 6-year-old son William spent most of the first year of his life sick. Constantly sick. No one seemed to be able to figure out what was wrong. One doctor kept telling us William was sick because he was in daycare.

Finally, just 12 days short of his 1st birthday, William was diagnosed with scimitar syndrome (sometimes called partial anomalous pulmonary venous return). A year after that -- four years ago today -- on June 29, 2006, William's beautiful little heart was repaired at Shands Children's Hospital at the University of Florida.

We are ever, ever grateful to Dr. Mark Bleiweis for his special gift of fixing babies' hearts. We thank God often, still today, for pediatrician Dean Dalrymple and pediatric radiologist Charles Williams for their sense of urgency, curiosity and diligence in getting to the root of William's illnesses. If not for these amazing people, we might still be wondering what was wrong. And we are always thankful for William's pediatric cardiologist, Dr. Jay Fricker, who makes sure William continues to do well. 

One of William's biggest supporters and cheerleaders, though, doesn't often get public praise, but he certainly deserves it for his eternal patience, his constant support, his everyday care and never-ending hugs and love. I think God specifically chose Martin as William's big brother because He knew that William would have a rough start in life and that Martin would take extra-special care of William. They have a beautiful brotherly bond, especially for siblings who are nine years apart. They are best Bubbas.

After four years, 14 years, 40 years, we will always pause on this day -- June 29 -- and thank God for His presence in William's and our lives, and for guiding us to the people William needed to be whole and healthy.

Happy Heart Day, William. We love you and are so glad you are here and whole and healthy and happy.

Medical-Specialty Board Certification: It Matters

“Any doctor can deliver a baby, treat cancer, or declare himself a cardiologist. Certification means the doctor had special training in that field and passed an exam to prove knowledge of it.”

Six years ago, I would have said Associated Press writer Marilynn Marchione was exaggerating in her recent article, Doctors face board specialty 'expiration dates'.

Had my son never landed in the hands of a physician who “declared” himself a specialist, I might still think that way. But he did. So I don’t.

In a previous post about my son William’s heart murmur, I mentioned “the eight-month-long road to a correct diagnosis was ill-paved and rife with potholes.”

I’d like to explain now.

At William’s four-month well-baby checkup, his exam revealed The Heart Murmur. The pediatrician (whom we no longer see) referred my son to a specialist -– “a pediatric cardiologist,” the pediatrician said -– to evaluate the murmur.

If only I’d known then, what I know now: verify credentials yourself. You can’t rely on referring physicians. They might not have verified credentials. They might be assuming a specialist is certified. Or, the specialist might be so rooted in the community that everyone assumes he/she is a board-certified specialist.

You also can’t rely on advertising -- even though (in Florida anyway) claiming you’re board certified in an area you are not board certified in is against the law.

If I could do it again, my first stop would have been here, so that my second stop could have been with a real pediatric cardiologist. Despite what the pediatrician said, the local physician who sees pediatric heart patients is not this area’s “only option.”

Knowing what I know now, I'm not at all surprised this physician misdiagnosed my son. Not just once, but twice. This doctor is nothing more than a pediatrician who completed a pediatric cardiology fellowship ... 35 years ago. Since he's not board certified in pediatric cardiology, there's nothing to show he has pursued any continuing medical education (CME) in this area.

And there have been so many advances in CHD diagnosis, research and treatment over the past three-and-half decades, it hardly seems possible he could have stayed relevant without a lot of CME.

Physicians I love and trust implicitly have told me William’s congenital heart defect (Scimitar Syndrome) is not an easy one to diagnose -- implying I should cut the doctor some slack.

But if it's so hard to figure out, I ask them, then please explain how William’s eventual correct diagnosis was made by a mere pediatrician and pediatric radiologist? Neither of them specialize in, or have had concentrated study in pediatric cardiology.

Really, if not a pediatric cardiologist, then who is imminently qualified to diagnose congenital heart defects -- even those that are difficult to diagnose? If you're going to "declare" yourself a pediatric cardiologist, then you'd better well know what you're doing!

After William's diagnosis, we requested a referral to the University of Florida Congenital Heart Center -- one of this area's many options. The physicians there are extremely competent, very compassionate and ... board certified.

About 15 months after William's open-heart surgery, I filed a formal complaint with the Florida Department of Health. The complaint included misdiagnosis of condition and false advertising. However, DOH declared the physician had done nothing wrong. Nothing. Not even falsely advertising himself.

I also e-mailed the American Board of Pediatrics about the physician’s advertisements. Although the ABP can’t tell me what, if any, action it took, I noticed that about six weeks later, the physician no longer held himself out in advertisements as a board-certified pediatric cardiologist.

Marchione writes in her article, “The next time you’re at the doctor’s office, take a peek at those certificates hanging on the wall. Like gallons of milk, some of them are expiring.”

Better yet, make sure from the start they even have certificates.

The American Board of Pediatrics sponsors a campaign called “Certification Matters.”

And let me tell you: It. So. Does.

Scimitar Syndrome: A Discovery Highlight

If you’d told me in high school that one day, I would read abstracts from the Annals of Thoracic Surgery with the giddy delight of a 5-year-old eating an ice-cream sundae, I would have burst out laughing, and, well, I would not have believed you for even a second.

But here I am (more than two decades later), all excited over an abstract I found online from a 2009 edition of the Annals of Thoracic Surgery. The article is about a multicenter study in Italy of patients with scimitar syndrome.

Scimitar syndrome is a rare variation of partial anomalous pulmonary venous return, a congenital heart defect. And my almost-6-year-old son is one of the 1-3 in 100,000 babies who are born with scimitar syndrome, and one of the 1 in 125 babies born each year in the United States with a congenital heart defect.

I’ve learned a lot about scimitar syndrome since those first months after William’s diagnosis in March 2005. And finding the abstract today was a highlight of my discoveries.

The study included 26 patients. Most of the patients presented like my son, with symptoms including recurrent upper respiratory tract infections and pneumonia. Eighteen of the patients had an intraatrial baffle repair, while eight patients had their scimitar veins moved and re-implanted at the left atrium.

Those were the two repair options William’s brilliant pediatric heart surgeon — Mark Bleiweis at the University of Florida Congenital Heart Center — discussed with us. He said he wouldn’t make a decision about which repair to use until he saw William’s heart. Then he would figure out which would be the better solution.

Once inside William’s chest, Dr. Bleiweis thought William’s left atrium was a bit small to accommodate the right pulmonary veins, so he created an intraatrial baffle — a tunnel directing the blood flow from William’s anomalous right pulmonary veins (located near the junction of the inferior vena cava and the right atrium) into his left atrium, where it belonged.

We knew then that Dr. Bleiweis knew what he was doing, and we trusted him. Now I’ve found a study of 26 patients over a 10-year period that shows “the intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the re-implantation of the scimitar vein onto the left atrium.”

We’ll take it.

The Heart Murmur

I did it when my 14-year-old son was a baby. And I was doing it now. Holding my breath. Praying. Watching the pediatrician’s face. He was listening to my 4-month-old son’s heart and lungs during a routine well-baby check-up.

One minute. Two minutes. My son’s chest. His back. Three minutes. Four minutes. His chest. His back again. This is taking too long. He is listening to my baby’s heart too long. I closed my eyes and prayed.

The doctor rolled back his chair to look at me. I looked at him, thinking, praying ... Don’t say it. Please don’t say it.

“I hear a murmur.” He said it.

I pulled my son, William, tighter to my chest.

“Most of the time, these things turn out to be nothing, Ms. Chavez,” the pediatrician gently told me. “But this sounds significant; I’m afraid this is going to turn out to be something.”

“I’m going to refer you to a pediatric cardiologist,” the doctor explained.

My own heart dropped. There was a problem with my baby’s heart. His heart.

I wanted to rewind that day. July 30, 2004. It had been just a normal day. Now it was anything but.

“If this is something,” I asked the nurse practitioner on my way out, “what do I look out for? How will I know if something has happened or has gone wrong?”

“He’ll have trouble breathing, he’ll look blue,” she answered in a hurry as she went to see another patient. But she looked sad.

That was it? That wasn’t nearly enough information. I was terrified. I cried all the way home from the doctor’s office. I reached toward the backseat, trying to hold William’s hand. I realized I had to tell my husband our infant son has a heart murmur and needs to see a pediatric cardiologist.

The innocence and naïve joy of having a new baby were gone. Everything changed that day. Our lives were never going to be the same.

Unfortunately, the "pediatric cardiologist" we were referred to in Tallahassee was not board certified. The eight-month-long road to a correct diagnosis was rife with potholes and detours. We spent five months in the hands of physicians who were poor listeners and even worse diagnosticians. The local "pediatric cardiologist" twice failed to correctly diagnose William's heart defect. 

It was a fearful, heartbreaking, stressful time. William was never well. I developed Bell’s palsy that September and my 16-year-old nephew was killed in a car crash five days before Christmas.

After that, getting William well took on new meaning and our efforts to do it amplified. We “fired”’ our pediatricians and found a new one. One who listened, examined and treated carefully, methodically and cautiously.

William’s correct diagnosis – scimitar syndrome, a rare variation of partial anomalous pulmonary venous return – came just days before his first birthday. (William's version of scimitar syndrome includes an underdeveloped right lung that receives no blood flow; he functions entirely on his left lung. He also right pulmonary vein stenosis, mild right ventricular hypertrophy and superior vena cava stenosis.) The diagnosis was the result of the divine meeting of the medical minds of two thorough and thoughtful physicians. 

The first thorough and thoughtful physician was our new pediatrician who had immediately questioned and investigated the diagnosis that William’s heart murmur was functional. He thought it sounded pulmonary and was concerned because he could hear the murmur in William’s back. Functional murmurs, he said, typically can only be heard through the chest.

The second brilliant mind was a pediatric radiologist who had read two sets of William’s chest X-rays – four months apart – and noticed the same problem in each: William’s lungs were unequal in size and function. Of all the patients that pediatric radiologist sees in the hospital and in his radiology practice – and he remembers my son and these minute details of his lungs. That still amazes me.

Once William was diagnosed, we were referred to the UF Health Congenital Heart Center in Gainesville. That's when everything started to get better. We learned so much about William's particular condition and we no longer feared that he would drop dead while he was running in the yard with his brother. And wonderfully, at UF Health, we continued to find doctors who listened, examined and treated carefully, methodically and cautiously.

I hug, kiss and tell my children every day that I love them. I stare at them and marvel at their beings, their health and their lives. I will never forget William’s four-month, well-baby check up and hearing those words “heart murmur.”

Those words and all the days that have followed have changed our lives forever. I would not have believed it then, but I know now, they have changed for the better. Our family is a stronger family. We appreciate our lives in ways we never did before. We have an incredible team of physicians for William's continued care. We have met hundreds of families online and in real life who have similar experiences as ours. We have William's heart to thank. We didn't think it then, but we know now, we wouldn’t have William’s heart any other way.



Updated: Feb. 3, 2018.