When You Stand Alone

I read this post today from another CHD Mom, and I was struck again by the familiarity of experience.

A great deal of our family was there - my mom, dad, my in-laws, sister, brother, sister-in-law, grandparents, etc. But there were many who weren't. Many friends and family members never bothered to email or call. They made no attempt to see how Lukas was doing before, during, or after his operation.

For me, the situation was flipped. My friends, my chosen family, were there for me. My family was not.

This past operation was probably one of the loneliest, and a turning point of sorts. It was the most dangerous (or, perhaps, it was the first one where I was fully aware of and fully felt emotionally the brunt of the danger). It was the third time, and yet the first time where we were effectively alone. It was the first one in which we traveled, and also the first one where we were within minutes of family.

That family, though, didn’t show up. My only brother, who is politically opposite from me, had “unfriended” my son after I asked him not to rip into our beliefs on Facebook. We haven’t spoken since, and he has not spoken to his nephew again. My mother and her husband spent several weeks in Houston with my brother and his wife as they were the proud parents of a new healthy baby, and they wanted to spend time with their new infant grandson. They left Houston to return to their home two days before the open heart surgery and stopped by to see us for about an hour on their way home. I had no contact or help from my brother at all, just 40 minutes from the hospital. My parents did not extend their trip to help us out in that first horrific week in the ICU.

It was a turning point for me where I realized that my concept of family, and theirs, were unbridgeable divides now, and sitting in the quiet of that hospital room I was for the first time truly aware that it would always be like that and nothing I did or hoped for or wished for or needed could ever change it. I was different now. They were the same. And not what I needed. And hurtful during a time of trauma. Their actions and lack of support made it worse.

I remained aware and cognizant, like an undercurrent, as I watched other extended families rally around parents that that my family was different. I knew that in dealing with the surgery, the struggles, and the fears that this isolation from them would always be there.

My family really does not deal with adversity well. They tend to banish that which bothers them, ignore things that are alarming. Dealing with things has never been and never will be their forte, and depth is something that they just don’t manage well. It is a thing I never mastered, this ability to simply ignore the elephant in the room so that everyone can go on blissfully ignorant of reality.

If it is slightly uncomfortable, it is not something anyone willingly walks into. Trauma? Forget about it.

The collateral damage is insane with CHD. Insane.

With each birthday, a sigh of relief

I took a bit of a break from writing in this – from writing everything, really. I think part of it was the overwhelming frustration of the medical bills. I kind of broke there for a while and nothing I could have written would have been anything other than sound and fury, signifying nothing. And likely told by, at that time, an idiot. But anyway.

We gave up a lot to try and get some semblance of a life. DirectTV is gone, and most of the cell phone subscriptions will be next when the term runs out – I have to have one for work, though technically I don’t, so I’ll be switching to a pay as you go phone and hope that few people will call me (and will nuke the data plans). We canceled the analog telephone, which was frightening, but the Cardiologist said we only needed to call the pacemaker in once every 3 months now, and we just couldn’t figure out how we could keep paying $70/m for one phone call four times a year. We took our second car off the insurance and will stop driving it.

What to other people seem necessary to us seem like conveniences.

I’ve been reading lots of other CHD folks blogs, though, and I can’t complain in comparison. One blog I read detailed what it was like to file for bankruptcy. Another detailed the latest bill from the latest trip to the hospital that ate yet another vacation. Another’s child is in a coma. Another celebrates a heart transplant while planning the fundraiser to help pay for it.

I understand, since it’s open election time and the insurance plan I had is no more. The comparable one had raised the deductible along with the rates. Normal. Same old same old. Never changes. Another year without a vacation.

Sorry we never went on any trips, honey, but I got you heart surgeries!

My kid has now had more surgical procedures than family vacations. There’s just something wrong with that. He’s not even picky – Orlando or Vegas. Those are the only two places in his mind, and as he ages Vegas is more magical than Disneyworld. Go figure.

He’s going to be fourteen next weekend. Fourteen years old. It’s now been twelve years since I’ve lived with CHD running my life. It’s strange.

It’s strange, and I’m grateful… but as he gets closer to adulthood and the bills and financial burdens don’t ease, my heart grieves for the fact that there probably is nothing I can do to shield him for this or prepare him for these kinds of challenges. No explanation I can give for why this isn’t all outrageously unfair.

I would deal with them all gladly and without complaint if I knew that when he grew up it would magically get better for him.

I wish I could give him that for his birthday.

There’s less to talk about, and more to talk about.

I hadn’t ever really intended on blogging so much about Congenital Heart Defects – it just sort of happened. I also never intended on Jacob having to have another open heart surgery, either, but that just sort of happened, too.

When I started occasionally mentioning it, I wrote the posts from the perspective of someone who knew that people didn’t really understand, and trying to get people that hadn’t been through it and who didn’t ever think about it some glimpse. It was from within battling CHD outward to everyone else that never had to deal with it. There was always a place I would stop and not go beyond because… well, why scare people. They weren’t going to understand, anyway.

The closer we got into last year’s medical crisis, the more it overtook what I thought about – it was the Cardiac Catheterization, or it was the next doctor visit, or some decision we had to make. I felt myself getting less observational about it all, and more emotional. I began connecting with other CHD parents, and started reading their blogs, and I realized that they were all to one extent or another journals of personal hell. I tended to stop right before that line, and not share the more personal things.

I never wrote about what it was like to sit over an ICU bed in the middle of the night, hear the alarms go off, and stare as nurses and doctors rushed in and out, worried. I never wrote about the horrific and gut wrenching struggle I went through fighting against my own cynical atheistic beliefs and how, in the face of potential death, the magnitude of that void that death represented almost made me lose my mind. I was gripped by a fear that my child could drift off somewhere I couldn’t protect him, or just be snuffed out like a candle flame. I felt so many levels of anger, sadness, resentfulness, fury. It’s an indescribable thing to go through.

And when talking to “normal” parents, I don’t go there. When talking to “normal” people, I stop short.

It is better now, but I do realize there is a need for those of us willing to share to go further – there were things I felt that I never even conceived could be normal until I read about it in five other parents blogs. I also realized I could never do that so long as these stories were wrapped up in all the other stories of my life. This one is different. Perhaps the defining one.

And so, I moved the blog. It now stands alone, separate from my “other life”. Eventually, I will likely disengage it from “me” altogether. A shift in focus, a shift in consciousness, too, in a way. It will probably be more personal than it was. And Jacob has talked about participating, so that will be interesting if it comes to pass. Probably more journal than education, more support group than awareness, more connection than soapbox.

This is a journey that, aside from the medical issues, comes with so many challenges. As much as I talk, I don’t talk about them much.

I think I may start.

Rare Disease Day 2012

Today is Rare Disease Day – what is Rare Disease Day?

Rare Disease Day is an international advocacy day to bring widespread recognition of rare diseases as a global health challenge. The day is celebrated on the last day of February every year and this year will be observed February 29, 2012. Learn more about the History of Rare Disease Day, Past Successes or the Goals and Plans for this year.

To my great surprise, Jacob’s particular CHD is actually considered a rare disease according to the U.S. Office of Rare Diseases Research. I knew it was rare, but didn’t know it was rare rare.

For Rare Disease Day, think about writing your reps.

We’ll be spending Rare Disease Day, ironically enough, at the doctor’s office.

How Humana Makes a Profit #chd #healthcare

Humana gives you this fantastic, happy little download so you can see what you are spending – the picture to the right is our happy little download for 2011 while we were with Humana. In three months, we racked up almost $2,000 in costs.

They didn’t. We did.

I was actually curious about whether we were profitable for them because I started to have the sneaking suspicion that we were – and honestly, I don’t think of our family as “profitable” when it comes to insurance companies.

In the 4 months we were on Humana (we were still on in January 2012, which is not reflected in the quarterly happy summary), we paid $1518 in premiums. My employer paid more than that but let’s just work with what we paid – which is $1518 and change.

In that 4 months, Jacob’s CHD treatments billed us $3603.85. Humana paid $228.31 and left us owing $1997.95.

My husband didn’t make any claims at all. I had one $89 visit that I paid a $35 co-pay for, and the plan paid $26.78 for. So, for those keeping track:

We paid in premiums:	$1518
We paid out of pocket:	$2032.95
We paid a total of:	$3550.95

for four months of healthcare.

In the same time frame where we paid $3550.95 in premiums and bills, Humana spent only $228.31 on us. That’s it. That’s what we got from having the insurance company.

Humana made a profit of $1289.69 just off of what we paid them. That’s not counting the money that my employer kicked in and if you factored that in, we would be outrageously profitable.

Humana’s quarterly net income jumped to $199 million, or $1.20 a share, from $107 million, or 63 cents a share, a year earlier.

Earnings were in line with Wall Street’s average estimate, according to Goldman Sachs analysts. Excluding a charge of 13 cents a share for a donation to its charitable fund, earnings would have been 11 percent above expectations, according to David Windley, an analyst at Jefferies.

Revenue rose 9 percent to $9.06 billion, below the $9.24 billion that analysts expected, according to Thomson Reuters.

from "Humana Hits Analysts’ Targets for the Quarter", NYT 2/6/2012

Yeah, I bet they did.

Do you like Jazz? Meet the Baby Blue Sound Collective #chd

Hurt–Baby Blue Sound Collective

 

A group of heart surgeons, cardiologists, cardiac nurses and perfusionists walked into a bar with instruments… ok, that’s not the beginning of a weird CHD joke.

The Baby Blue Sound Collective is comprised mostly of health professionals who care for children and adults with Congenital Heart Defects. The group of heart surgeons, cardiologists, cardiac nurses and perfusionists have donated their time and talents to record and perform an eclectic mix of Jazz, Classics, and Easy Listening music. All proceeds from their work go directly to charities who support patients and their families through financial aid, education, and research.

 

Baby Blue Sound Collective

 

You can currently purchase their release “Songs for Spencer” to support Spencer’s Fund. What is Spencer’s Fund? From the Spencer’s Fund page.

 

For the last year and a half of Spencer's life, over 8 months were spent in the hospital. Through the generosity of friends, relatives and strangers, Spencer's parents received snacks, books, cards, and meals to help during a very stressful situation. During Fall 2001 at Glennon, the Kults became friends with other families that were coping with extended hospital stays: one teenager waited over 3 months for a heart transplant and another family was dealing with an extended pleural effusion. If Chris and Tina were exposed to several families at one small Midwest hospital, just imagine the hundreds of other families around the country experiencing extended or far from home hospital stays, and the financial difficulties they have to face. 

With Spencer's passing, The Kults and C.H.I.N. wanted to honor his memory in some way that would help other families who might be faced with financial difficulties. In an effort to reach out to these families, C.H.I.N. is now pleased to offer Spencer's Fund, a financial assistance program that will help families with expenses incurred during extended or far from home clinic appointments and hospital stays. Spencer's Fund will provide groceries, meals, travel subsidies, lodging assistance, and utility payments to families in need.

 

You can purchase a CD from The Congenital Heart Information Network, or a download on on iTunes, Amazon MP3, Spotify, and EMusic.

CHD: Our Particular Congenital Heart Defect Explained

Most of us find it easier to just say “CHD”, even though CHD can mean a lot of things. CHD is, in short, anything structurally wrong with the heart and/or great vessels that someone’s born with. Because of the number of folks that donated and some of the questions I got, I wanted to get a little more in depth to give an explanation of what all this stuff is.

Welcome to CHD 101. This is long - there are a lot of goofy ways the heart can grow wonky, and in alphabetical order, here they are:

Aortic stenosis
Atrial septal defect (ASD)
Atrioventricular septal defect (AVSD)
Bicuspid aortic valve
Dextrocardia
Double inlet left ventricle (DILV)
Double outlet right ventricle (DORV)
Ebstein's anomaly
Hypoplastic left heart syndrome (HLHS)
Hypoplastic right heart syndrome (HRHS)
Mitral stenosis
Pulmonary atresia
Pulmonary stenosis
Transposition of the great vessels

dextro-Transposition of the great arteries (d-TGA)
levo-Transposition of the great arteries(l-TGA)

Tricuspid atresia
Persistent truncus arteriosus
Ventricular septal defect(VSD)

Some conditions affect the great vessels or other vessels in close proximity to the heart, but not the heart itself. Those are still classified as congenital heart defects.

Coarctation of the aorta (CoA)
Interrupted aortic arch (IAA)
Patent ductus arteriosus (PDA)
Scimitar syndrome(SS)

Partial anomalous pulmonary venous connection (PAPVC)
Total anomalous pulmonary venous connection (TAPVC)

Jacob’s Flavor of CHD – Subaortic Stenosis

There are generally four types of defects: Hypoplasia, Obstruction, Septal, and Cyanotic. Jacob has an “Obstruction” defect. There’s also classifications of “Simple CHD”, which means it tends to be just one issue and an easy fix and the surgical results can seem curative, and “Complex CHD”, which essentially means that the CHD is hella wonky or quite a pain to fix or tends to need ongoing care and fixes and more intense monitoring. Simple tends to be the majority, and Complex the minority of cases.

I’ve never actually asked if we were formally classified as simple or complex since we started with just one issue, but I get the feeling if we ever were simple we are probably not simple anymore.

Common obstruction defects include pulmonic stenosis, aortic stenosis, and coarctation of the aorta, with other types such as bicuspid aortic valve stenosis and subaortic stenosis being comparatively rare.

His is, of course, the comparatively rare Subaortic Stenosis - because my kid really doesn’t want to do anything like everyone else.

I usually have this long description to try and explain what goes on, but a few days ago someone actually uploaded a video that explains exactly how this all started, and what the main defect is. It’s all cartoony, no blood, I promise:

 

Subaortic Stenosis

 

English Translation:

• hypertrophied – part of the heart becomes enlarged. You know how when you lift heavier weights your muscles become bigger? Yeah, in the heart, that’s bad if its due to wonkiness. Muscular hypertrophy is what you get when you do strength training. Ventricular hypertrophy is also what you get from doing cardio (athlete’s heart) – you don’t want to get it from one part of the heart trying to overcome wonky.
• Fibrosis – in short (for us), the wonky. Tissue that forms and grows and shouldn’t be there.

So, why all the talk of the valve last year? Well, the narrowness and the way the fibrosis grew caused the blood to shoot at the leaflets of the valves instead of down the tube – think of a hose, and then squeeze it to make the the water velocity faster. Now, take the hose and hold up a Kleenex tissue right in front of it and fire away – but don’t hurt the Kleenex.

Now do it for 13 years straight.

Yep, valve leaflets were not happy, and the fibrosis grew on them to boot, making them really wonky and not work right. So from Subaortic stenosis, we developed…

Aortic Insufficiency

Aortic insufficiency is a heart valve disease in which the aortic valve weakens or balloons, preventing the valve from closing tightly. This leads to the backward flow of blood from the aorta (the largest blood vessel) into the left ventricle (the left lower chamber of the heart).

And when that blood kicks back, the mitral valve gets all confused and wonky because it’s all connected, and we get…

Mitral Insufficiency

Mitral valve regurgitation — or mitral insufficiency — happens when the heart's mitral valve doesn't close tightly, which allows blood to flow backward in the heart heart. When the mitral valve doesn't work properly, blood can't move through the heart or to the rest of the body as efficiently.

All of these things were worked on during the open heart surgery in August of 2011. The surgeon painstakingly “resected” (i.e. cut out) the fibrosis, peeled it off the leaflets (that are only 3 cells thick) without damaging them, and attempted to repair the mitral valve.

That, my friends, is a lot of cutting on the heart in a lot of places, and so from the repair, we got…

Third-degree Atrioventricular Block

Third-degree AV block, also known as complete heart block, is a medical condition in which the impulse generated in the SA node in the atrium does not propagate to the ventricles. In English, not only is the electrical system that controls the heart beating wonkily, it’s more or less stuck on idle.

In this case the cause is iatrogenesis or “iatrogenesis artifact" which translates to “originating from a physician”. Yep, the surgeon caused it.

But it isn’t entirely unexpected - post-cardiac surgical patients (and particularly valvular surgery patients) are at a high-risk of complete AV block. The incident rate is 2-3% for your first open heart, and climbs to 10% when you go back for subsequent surgeries. We had no problems the first time, or the second time, but we caught the bullet on the third time.

So where are we now?

• Third-degree AV block – Pacemaker dependent, and expensive calls to the mothership. Oh, and that awesomely fashionable medic alert bracelet.
• Mitral valve regurgitation – much better than it was than before the surgery
• Aortic Insufficiency – leaflets are still not closing 100% and we still have leakage, but much, much less than before.
• Subaortic Stenosis – much, much clearer than before. Hopefully will not grow back. Again.
• Ventricular hypertrophy – shrinking, now that the heart is under less stress.

And that should answer both the questions that I tend to get fairly frequently, which are:

1. What does he have?
2. How’s he doing?

Answer is all this, and pretty good.