The Heart, part 2 - Heart Throbs: Crash Course A&P #26

The Heart, part 1 - Under Pressure: Crash Course A&P #25

Faces of CHD: Matthew's Story

Faces of CHD: A Heart of Gold Stopped Beating

Matthew’s Story

“O dry the glistening tear that dues that marshal cheek, thine loving children here, in them thy comfort seek, with sympathetic care, their arms around thee creep, for O they cannot bear, to see their parents weep.” – William S. Gilbert

            Last time, we talked about vaccines and their role in protecting CHD patients. Now I would like to begin a series where every so often, we will get to know a CHD patient, either a survivor, or an angel, someone other than me who has gone through this, because as we covered in CHD basics back in July, I am far from the only one.

            On May 20^th, 1996, one of my best friends, Matthew Ethridge, was born. Matthew was a beautiful little boy from the very start, with his bright, blue-green eyes, and his thick blond hair, but while he looked healthy on the surface, he was far from ok. You see Matthew, had been born with two very serious congenital defects. One of them, was Transposition of the Great Arteries which is one of the worst kinds of single defect Congenital Heart Disease that a person can have without needing a full blown heart transplant. Instead, doctors have twelve months to fix it over the course of three open heart surgeries, left untreated, it would have killed him. The other major defect that Matthew was born with is called Aspleenia, which is exactly what it sounds like, he was born without a spleen. This was particularly bad, for someone already vulnerable to infection.

In a normal individual, the spleen acts as a filter, recycling old blood cells out of the circulatory system, and when it detects dangerous microorganisms in the blood, it creates a type of white cell called lymphocytes and it's them that produce antibodies to fight the infection. Without a spleen, Matt didn’t have the same capacity to defend himself, which made endocarditis and sepsis, which we discussed in the post before last, all the more likely, and him even more likely to succumb to either one of them.

Matthew suffered many medical close calls over the years, almost all of them due to his weakened heart and immune deficiency, and in the course of that he never once complained, he never stopped smiling, he never yelled nor cried out in pain or fear. It was so much that we actually wondered if he understood that he was in pain. I’ve never met anyone, not before, not since, who could take that with childlike faith, honor, and acceptance. That takes a strength and a bravery that cannot be taught, it was just in his nature and it carried him through everything he had to face and lifted up those around him. To know him, was to love him, and to spend any time with him at all, was to feel better about what was going on in your own life. His resilience in the face of things that would make most adults cry, the fact that in spite of it all he actually seemed to be enjoying life, was truly inspiring.

It wasn’t until he was six that his health began to stabilize, he was stronger and was actually spending more time out of the hospital than in. He could finally play T-ball and run around with his friends. Unfortunately, before anyone really knew what was happening, that all crashed and burned and the good times came to an end.

On August 17^th 2003, Matthew was admitted to a local children’s hospital with an infection. It wasn’t the first time this had happened, but this time was different, whatever the actual illness had started as, it quickly turned to sepsis and raged through his body like a fire beyond control. While doctors tried antibiotics and antivirals, the infection was spreading too fast, and without lymphocytes to stop it. His body started shutting down and within hours he was on life-support, unable to breathe on his own. He slipped in to a coma and for the last time, his eyes closed. At some point on the night of August 18^th, he lost brain function completely and on the 19^th, his parents, doctors, loved ones, and friends let him go. The doctors shut off the machines and allowed his body to die, but he wasn’t even really there anymore, he’d left the previous night.

It shouldn’t have had to be that way. He had been doing well, there had finally been reason to think that he might actually have a life ahead of him, that he might grow up, raise a family, grow old… all of that, was taken away from him in the space of two days, just a few months after his seventh birthday. If anything, Matthew’s story highlights the dangers of infection, just how quickly it can take a CHD person down, as well as the need for research into how Aspleenia might be better managed, those with the condition better treated and protected… Perhaps in time a method can be developed for giving these children a normally functioning immune system, so that this; never has to happen again.

I knew Matthew personally, I knew him, I love him, and I miss him every day.

“A simple child, who lightly draws his breath, and feels his life in every limb, what should he know of death?” – William Wordsworth

Preventing Infection 2

Today, I want to talk about Vaccines and their role in keeping CHD children alive and healthy. 

CHD children are a conundrum when it comes to vaccines, while we are at a higher risk of certain infections and a higher risk of complications of still more illnesses, we are also at a higher risk of complications from the vaccines themselves. So rather than just following along with the recommendations meant for healthy individuals, parents and pediatricians need to be careful about which shots to give, when, and how spread out or close together in time they are. 

Remember that CHD children are exceedingly prone to developmental delays, not just in terms of cognitive and motor skills, but also in terms of physical growth and development. That includes the development of the immune system and metabolism. The things we use to deal with diseases, vaccines, and chemicals that enter our systems. This is why someone with congenital heart disease may need to wait on some vaccines or spread out vaccines that would ordinarily be given at once or close together.

There are also certain things which may make some vaccines totally inappropriate. I for one, suffered a few seizures following my 1st heart surgery. This makes the Pertussis vaccine, which protects against whooping cough, which is known to cause seizures in some cases, totally unavailable to me, the rest were delayed to keep pace with my development, not the "normal" curve that I wasn't on. 

At the same time, CHD also makes some vaccines that children normally don't need, a must. The recommendation for people with chronic heart/pulmonary disease is that they receive a dose of the PNR Vaccine, which protects against the most common strains of bacterial pneumonia, every 5 years as children, followed by one booster after age 18 to be followed by the newer Prevnar13 vaccine a year later. This final two shot sequence is repeated one last time after age 65. I actually just got my adult PNR. 

It is also of particular importance to keep up on one's flu vaccinations, as a severe case can turn into viral pneumonia. 

Endocarditis and the Wider Threat of Infection, How to Prevent it, and how to Cope with Preventative Treatment Measures

Endocarditis: A CHD Survivor’s Worst Nightmare and what a Cardiologist or Primary Care Doctor Should do to Prevent it.

This is a big one. Endocarditis is basically a viral or bacterial infection that is attacking the heart directly. We, people with CHD, are both more likely to suffer from endocarditis and less likely to survive it. The microbial menace in question will eat away at the heart muscle which can cause holes and defects where they didn’t previously exist, causing the heart to work less efficiently and possibly fail.

Since the site of the attack is also the center through which the entire blood supply is pumped, and endocarditis infection can also easily turn into sepsis which is potentially fatal for anybody.

There are a couple of steps that can be taken to help prevent endocarditis in the first place.

1.      Keep a CHD survivor, particularly if they are currently in need of treatment, immune compromised, very young, or very old (there are a few…very few), away from anyone with a serious or highly contagious illness of any kind. 

2.      Put them on prophylactic antibiotics, whether this is situational or a regular thing will need to be determined by the doctor prescribing the medication.

I have been on Amoxicillin, situationally, for as long as I can remember. Every time I go to the dentist for example, I down four Amoxicillin pills an hour before the appointment. The same was true when I got my ears pierced. Anytime there is a plan to expose my blood to potentially less than sterile instruments I have to take it. 

Personally this is one of my least favorite things about this. Not that there’s much to like about growing up with birth-defects but most of it has reached a point where no part of my mind even suggests protesting. It simply is. This, for some reason, as necessary as it is, has not become one of those things. The side effects are annoying. It turns my stomach into a churning vat of acid and drains my energy so that by the time whatever I took it for is over I just want to sleep for 2 to 3 hours.

The worst thing you can possibly do is to take it on an empty stomach. It makes the stomach churning that much worse if you do that. What I have found really helps, is to eat a milkshake before taking it. Since milk is a base substance, it combats the acid somewhat, really any food will help, but I have found milkshakes to be the best thing. 

 If you notice that you (if you are yourself a survivor) or a CHD person you know, is sick and worsening quickly or fading fast, get them to a doctor whether that means a visit to an Immediate Care clinic or an Emergency Room. Go!

CHD: The Facts

CHD, the Basics

Congenital Heart Defects are the most common birth-defect in the world. Affecting 1 in 100 live births across the globe.

There are over 40 known varieties, ranging from the ones that don’t really damage the function of the heart all the way to those that make a heart transplant a matter of survival. Most are congenital flukes, with environmental risk factors including Mother’s Age, Use of Fertility Hormones, Conception through IVF procedures, Smoking While Pregnant, Paternal exposure to toxins such as Agent Orange, and use of some prescription medications while pregnant, playing the biggest role.

Only a few forms of CHD have a known genetic factor, one of these is Dilated Cardio Myopathy, which usually results in heart failure prior to the age of 10.

CHD and other defects tend to occur in clusters, this is particularly true of the less serious varieties. There can be multiple CHDs in a single person (as was true in my case), there can also be issues with the function of other organs or other congenital disorders in addition to one or more CHDs.

People with congenital heart defects are much more likely than the rest of the population to have ADD, ADHD, Dyslexia, OCD, other personality and mood disorders, and Autism.

40% of people on the Autism spectrum have at least one CHD, and 60% of CHD survivors find themselves on the Autism spectrum, though the nature of this correlation is not yet understood by science.

Survivors who don’t need to undergo full transplants will always walk a thin line when it comes to athletic activity. On the one hand, maintaining a healthy weight and doing cardio exercise to keep the heart strong and healthy is essential, on the other hand though, physical and emotional stress in excess is a danger. Most people face the threat of heart attacks and cardiac arrest episodes in later life, but for us they can happen at any age. It’s a delicate balance that requires listening carefully to what one’s body is telling them it can and can’t handle and careful monitoring of a more scientific nature by a congenital cardiologist.

Author's Introduction

Hello, my name in Ana, I’m a 21 year old university student. I manage A’s, I play Tennis, I write, I read fantasy, historical, and mystery novels, I play tennis, and I was born with 4 Congenital Heart Defects. These are structural or electrical problems with the heart and major arteries.

Congenital Heart Defects effect around 1 in 100 live births across the world, in the United States, this rounds out to 40,000 babies each year on average. The truth is that CHD effects and kills more than twice as many children than all forms of childhood cancer combined and receives less than 10% of the research funding from the US government that childhood cancer does.

Until the late 80’s hardly anyone with a serious form of Congenital Heart Disease survived long enough to tell their story. In the last 20 years however, the mortality rate for CHD has fallen from 50% to about 35%, this significant improvement in the survival rate means that for the first time in history, there are more CHD survivors who are now adults, than we have CHD babies being born.

The improvement is still young however, which is probably why I can find blog and blog, and website after website talking about CHD from a parent’s and activist’s point of view, but find it almost impossible to find this story told from the perspective of the child, the survivor themselves. I hope this blog will change all of that.

I was born at the beginning of the shift, in 1994, when I was 1 of the 40,000, the chances of me seeing my 18^th birthday were 50/50, and I almost died more than once along the way. I have seen the inside of at least 5 different Pediatric wards in three different states, I was medivacked from one hospital to another at least once, and endured 3 heart surgeries over my first 11 years of life.

I am one of the fortunate, I am not in a wheelchair, I did not suffer severe brain damage, I am now healthy enough to participate in the sport of my choice…I can live a fairly normal life. Because of my good fortune, I am able to share with you now, the long road it took to get to this point.