What my ICD taught me – Part 1

icdheartI’m propped up in the bed, the window is to my right. My father sits at the foot of my bed, his hand on my leg, patting gently. My mom perched on the bed to my right, squeezing my hand.

Something is wrong.

Why the hell are they both there? Where the hell am I?

I feel woozy.

This is clearly a hospital bed. I’m in hospital. Why the hell am I in hospital?

My father is talking, gently, not his usual rapid chatter, but a gentle consoling voice. “We need to talk. Are you up for talking?”

Something is definitely wrong.

The room is full of flowers. Yellows and reds, dazzling blooms. Cards, magazines, and books stacked on the cabinet. I’ve obviously been here for a while.

I look at my father, and nod. He begins to explain I’ve had a surgery. The doctors have placed an ICD, a special device that will fire if my heart needs to have its rhythm checked, in my chest.

I clutch at my chest, but all that feels tender is under my breast, and my wrists and my throat. Dang, my throat is really sore. And I’m so tired.

“They talked to us about where to place the device. Because you’re slender, and young they’ve put it in your breast. They talked with us both first. You can have a surgery to balance out the other side if you want.”  My father continues. All of a sudden I’m focused. The fog clears from my mind and my first real memory post cardiac arrest is that moment of clarity, “What the fuck! Why would you put a metal box inside the secondary sexual organ of a young woman?”

My father gave me the easy news first, then he told me about the cardiac arrest. The metal contraption in my chest is forgotten in miasma that is knowing I’ve had a cardiac arrest at 27 years old no less.

A month later, I finally leave the inpatient rehabilitation unit. I start participating in a daily cardiac rehabilitation exercise program at the hospital, and I begin to understand why the surgeon put the device underneath the layer of breast tissue.

I am, by far, the youngest in the program.  The sixty and seventy-year-olds are whipping past me on the treadmills at two and half miles per hour while I slug away at one mile per hour. It’s a long way from the nine-minute jog I was taking when I went down.

A couple of the old guys, John, a former war reporter and Bob, an old navy guy, take me under their wing. They show me their ICDs, large odd-shaped masses, straining at the skin  just below their collar bones. I decline to return the favor for fear that a flash of my breast might cause their devices to fire.

Over the next six months, Bob and John cajole me, taunt me and cheer for me as I slowly speed up and begin to jog again. Their friendship isn’t the pity of my peers who don’t quite know what to say. This is the comradery of survival.

I don’t have the ‘other side balanced out’. My ICD becomes a built-in screening to assess any dates. Before they get to second base, I have to talk to them about my heart. They can’t kop a feel without me being willing to talk to them, and if I ain’t willing to do that, it goes no further. The ICD becomes my filter. In time, my condition – the ultimate test of love and commitment.

John died in ’99, Bob not long after. I hadn’t met my husband to be at that time, but funnily enough he shares with them that ability to cajole, to occasionally taunt, but mostly to cheer me on. I think they’d like each other. I do flash him and he does get to kop a feel of the ICD.

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How Writing is Like Compost – Illness Narratives

writing illness narrative

Compost. That’s what this blog is. Compost.

Fermenting bits of detritus that I hope some day will provide nutrients for something powerful. Something so powerful it will smother ARVD’s currently pernicious claim on my child’s heart with hope and healthy heart muscle tissue.

I’m in a room with writers and poets. The writer, the real writer with a strong voice in talking about illness or about anything it seems, is talking about compost. I listen, knowing that in this group I am hopelessly out of my depth. These folks are artists. They’re lyrical. They have wordsmithing power. Me? I can’t tell you an adverb from adjective. I’m shit at creative writing. And the writing I do is all functional. Not technical, but functional…and even that is painful.

There are narratives ready to be told, the writer says, ideas decomposed, fermented, no longer stinking of the cauliflower that you let rot in the fridge before finally throwing it into the compost. That partially decomposed arugula you bought, again, in the hope of eating salad isn’t ready to share with the world. But when the compost pile does its work, that arugula will be reconstructed into humus, loamy and fertile it no longer stinks, and it can be used by others.

But a compost pile can be helped I think. It can be turned, aerated, and here in this desert landscape – watered.

One of the writers, a playwright, looks around the room and says we’re here, we’re ready, and I crumble because the thing I need to talk about, want to talk about, is so raw I can’t get through a sentence describing it without tearing up- my child, who inherited my hair, my mouth, her father’s frame and eyes, has my heart.

Baltimore – A Time for Hope

johnshopkinsI went to Baltimore following the recent outcry regarding the death of Freddy Gray at the hands of the Baltimore police. The city was officially in a state of emergency, the National Guard, guns slung across their backs standing bored, leaning on the big armed humvees along the Inner Harbor.

I was shocked by the militarization around the ‘tourist’ areas of Baltimore. This display of power was sending a very clear message that even this white girl from the Southwest could understand.

‘These physical structures, THEY are valuable. You, people of Baltimore, you are not.’

The taxi takes me past the Inner Harbor and toward Johns Hopkins Medical Center, nestled in neighborhood where both poverty and college life exist cheek by jowl. An elementary school is letting out for the day. Six and seven year olds tumble out on to the street met by parents with hugs and kisses. Not a policeman or National Guard in sight.

‘We will use military force at huge cost to protect buildings, but not invest in children, not protect children.’

The following day I attend a meet and greet at a downtown hotel. My friend drops me off. There are at least 50 Maryland State Troopers standing outside the hotel. A few miles away several of my friend’s friends and their children are helping to clean up, talking to their neighbors. Is this who the Troopers have been ordered to protect the hotel or it’s visitors from? The people of Baltimore’s outrage is justified, but it didn’t start with death of Freddie Gray and now several weeks later the media has disappeared and my guess is it won’t return until the arraignment in July. Anyway, here are some people who know a crap load more than I do.

The Clock didn’t Start with the Riots by Ta-Nehisi Coates 

Beyond the Headlines There’s Much More to West Baltimore by Mary C Curtis

Baltimore is like my hometown, beautiful, leafy, green, in parts, but also a complex depressed northern town with charm and character and in need of investment. I was there at Johns Hopkins for a consultation and a conference for patients and families with ARVD, meeting for the first time in my life, people with the same heart condition and doctors trying to figure it out.  In a place in need of hope, with people driven by hope.

More to follow on Hopkins

Perspective – The deterioration of a patient doctor relationship

Taking an up close look at the deterioration of a patient doctor relationship

Taking an up close look at the deterioration of a patient doctor relationship

One minute you’re asking for folks to get back on task so you can end a meeting on time, for once, the next you feel the oxygen drain from your head, and reeling you collapse.

When you recover, you feel a cold towel on your neck, you hear the buzz around you as they decide whether or not they should call an ambulance, whether they can access the nurse’s office and find a blood pressure cuff (yes, but only part of the machine), they can’t find a pulse, but you’re alive, you move your left hand fingers to your right wrist and find the pulse, it’s hard to find, but it’s there, and it’s fast, really fast for someone for someone who is sitting down.

What do you do?

If you don’t have a heart condition already, you might put it down to the lack of sleep for the past week, the cold your children just gave you, but if you’ve just been talking about getting an AED for your child’s school using PTA monies (multiple children at the school including mine at increased risk of sudden cardiac arrest due to genetic heart conditions) you’d be forgiven for thinking driving home is not the best idea.

Like many women I am prone to dismiss the signs of illness, to tough it out and not be wimpy. This time though, in front of a room full of people I couldn’t, plus this time I had collapsed, I was sweaty, shaking and my heart was racing. A friend took me to the nearby hospital where my specialists are located and I spent several days, mostly waiting to see a cardiologist or an electrophysiologist who I never did get to see.

Much to the embarrassment of the ER doctor, the electrophysiologists eluded them, out-of-town and deeming my case (no firing of my ICD) not worth a visit. The short ventricular tachycardia episode that happened at exactly the same time as I collapsed was described as not being related to one another by one EP, who never actually talked to me, the pacemaker nurse, or the ER doc. So I languished in the ‘not quite admitted, but still in the Emergency department’ for 44 hours.

When I finally left, the internal medicine guy said that the electrophysiologist (who wouldn’t see me) wanted to change my medication, and I should follow-up with the EP in two weeks. The doc had been sure earlier that the EP would come, that they might sit with me and see how multiple PVCs plus pulsing of the pacemaker was still making me observably dizzy, that they would realized that the unlikeness of a time stamped vtach episode at exactly the same time as an observed collapse being unrelated were small, but he was wrong. I was sent out with no answers as to why I had collapsed; why I was having Vtach episodes suddenly; why I was continuing to have small dizzy spells, or what I should do to stop it.

As I got ready to leave, I asked a unit staff member if it was normal to be kept like this, to never see the cardio team. No, they said. It’s not. They, like the ER doctor, were apologetic. She took my blood pressure which was 186/91 much higher than my normal 110/70. Then she  circled the patient care manager’s name and number on the board and giving me a knowing look said something along the lines of ‘feedback from patients is really important. The Patient Care Manager and that department is there for this reason.’ I know they had already contacted my patient care manager.

What have I done?

I have changed electrophysiologists.

I’m done with having to beg and plead for an Echo and a measurement of my ejection fraction every few years and being made to feel as though I’m wasting his time.

I’m done with being put on a prescription that keeps my heart rate regular only to have the prescribing doctor ignore the pharmacy’s request for refills for weeks.

I’m done, watching my primary care physician shake his head as he asks if the EP is watching the impact on my liver of one of the EP prescribed meds, and taking care of the blood work and sending him the results.

I’m still angry that for years I asked, from before I got pregnant and subsequently, if there were more gene mutations that I could be screened for and was told no when at some point there was.

I’m irritated at his dismissal of the research coming out of Johns Hopkins regarding exercise limitations and suggesting that”stop when you’re coughing up a lung or puking” is okay for people with ARVD.

He and his colleague have finally eroded the last of my trust I had with this visit, the excuses I made for them, and while this might not have been an ICD firing event, it is a change in my heart’s behavior and that worries me rightly.

The new EP I saw the following day explained that it was unusual for someone to have syncope with a short Vtach, but did not question whether or not it had actually happened. They said it to my face, and said let’s try another medication. When I asked about the Hopkins’ exercise limitations, they said they hadn’t heard of that, but went to the computer and looked up reports and discussed them with me, still questioning if that was what it meant I should do, but willing to talk, to admit he wasn’t sure, to look something up, and to address my concerns.

I recognize how lucky I’ve been to survive a Sudden Cardiac Arrest, to have access to good health care, to not have significant reduction in function, but that doesn’t mean I should go meekly and allow my health to deteriorate due to neglect. These doctors are geniuses without a doubt, but they are human, they have egos, and lives, interests and priorities other than my particular health care. It is my job as a patient to understand that it is okay for a doctor to have faults, but not to accept a doctor who continually ignores or dismisses my situation however minor it may seem to them. It is, after all, my life in their hands. And yes, I’ll be following up with the hospital.

 

To test or not to test – It was never really a question

heartwaterfall

Genetic testing: Jumping off the cliff into a heart-shaped pond. Enlightening, but tumultuous waters.

“I have my suspicions as to the genetic basis for you and your brother’s cardiac events. I do think it is genetic. We might be able to find out with genetic testing. We might not, it wouldn’t mean it isn’t genetic. There are a lot more gene mutations that we can test for than there was when you first had your event. But you have to think about what you would do with that information, what it means for you, your children, your family members.

I would recommend testing, but talk with those you love, know what you’ll do with that information once you have it. In the meantime we’ll petition your insurance to pay for this. It could cost several thousand dollars”

That isn’t exactly what the new cardiologist said to me last year, but it’s close.

For me genetic testing was a no brainer, the answer to nearly 20 years of questions, but still I took it to my husband, to my father and mother and asked. unequivocally and unanimously they agreed.  How could we not find out? This was a little surprising as one of my parents had said previously that they were glad they hadn’t know before my brother died, otherwise they would have wrapped him up in cotton wool and never let him truly live and that would have been a crime too. Of course, that was before I went down too.

How could we not do the genetic testing? The information might answer the question  What killed my brother? What almost killed me? but more importantly what risks did my children, my cousins and their children face?

If my children, my cousins had the gene there was something that could be done. No, not a cure, that doesn’t exist for conditions like ARVD. Not yet. Having the information lets us mitigate the risks as best we can.

Like it or not we knew that this was a genetic condition, although I hoped it might be that 1 in a trillion chance it wasn’t, that it was some sort of environmental tetratogenic issue that affected both my brother and I. Knowledge was power. Moreover, not knowing didn’t make it not so.

So we tested. We got the results and we’re making a plan.

Just a week ago I lay, resplendent in my jeans and a hospital gown, and talked with an echo tech as he looked at the function of my heart.

“I have ARVD. My seven-year-old has it too. We just found out. We just told her. Do you see many people with ARVD?”

Not surprisingly for an academic hospital with a bunch of cardiac research projects, he sees a fair number of folks with ARVD. We talked about how difficult it was to come up with the right words to tell your child they have a life-threatening chronic and progressive disease. How it was important to be honest, but gentle with kids. And then he mentioned that there are patients with ARVD who choose not to tell their families, to not test their children, to hope that it will all just go away. After all, what is there that they can do? And I get it. I get why you might not want to test. I get why it feels futile, and the feeling of helplessness could be overwhelming and why would you choose to live like that? The temptation to wrap my beautiful girl up in cotton wool is great, but that would not be a life. Not testing, but knowing there was a 50:50 chance your child has the disease seems even more worrying. What if you didn’t test and then something happened, something you could have prevented. Would you be less likely to wrap them in cotton wool and limit their life? Testing meant a 50:50 chance we would hear that my child didn’t have the mutation and we wouldn’t have to worry about that any more at all.

Last week, I told my parent that originally had said they were glad we hadn’t known about my brother’s condition prior to his death of my daughter’s genetic results. They sighed, “I’m so sorry honey. This isn’t result we wanted, but now we know and we can do something. If we’d known this about your brother he might be here today.”

Knowledge is power. It doesn’t make ARVD less terrifying, but knowledge gives me a little sense of control. Knowing that we have this mutation in our family means I know I will do everything in my power to protect my child be it using medication and making sure there is an AED at her school to an implantable AICD; signing up for an ARVD registry to raising awareness about ARVD and cardiac death in the young; and finally campaigning for research fund for a cure.

 

How do you tell your child they have a genetic heart condition?

Telling your child they have a genetic diseaseWhere the hell is that in the parent guide books?! Seriously, where is it?

“Hey honey, looks like you not only got your uncle’s name you also got the condition that killed him and almost killed your momma. Hell, still might kill your momma…and you. Sorry about that.”

Yep, serious suckage. But this isn’t about me, this is about having to tell one of the most precious people in the world to me that I gave them something awful. That there is nothing to be done, but to wait and to see.

So we didn’t say that to my near eight year old. In fact we forgot to even tell her the name of the disease, Arrhythmogenic Right Ventricular Dysplasia/Cardiacmyopathy also known as ARVD/C.

On this rainy, quiet afternoon while her younger sibling napped, we curled up on the sofa with her and told her we’d be going to see a pediatric cardiologist soon. We told her that a recent blood draw had shown that her body, like mine, had this little bit that wasn’t functioning right, that it didn’t always make the stuff her heart might need it to. That for some people this wasn’t a big deal and for others it was a big deal. No need to deny that, there is no way around it, she knows of our family history. We were honest, we didn’t know what it meant for her, but we did know that we were glad to have the information so that the doctors could watch her more carefully; that if she needs medicine to help her heart we would know now. We talked about her friends who face epilepsy and diabetes every day, how this was just the thing she had to face.

She told us how she didn’t want to die of a cardiac arrest. She made the connection between the gene and possible outcome.

Could she tell her two best friends? ‘Yes, you can my love.’

Then she asked if she could watch Wild Kratts. And it was all over.

While we watched the Tasmanian Devil episode of Wild Kratts for about the 50th time, I wondered if we had done a disservice to her not explaining the true implications of ARVD. But at eight, how much can you handle? She needs to know not to ignore symptoms and to tell us about them. She needs to know that we’re going to be seeing the pediatric cardiologist shortly. She needs to know that not everyone who has this gene mutation develops the symptoms as a result (although most do).  She needs to know we’ll do everything in our power to keep her safe. She doesn’t need to know it’s progressive at this time. She doesn’t need to know that an Implantable Cardiac Defibrillator is a possibility in her future. She doesn’t need that information yet.

Before we talked with her I touched base with a genetic counselor who specializes in pediatric concerns and confirmed what I suspected.

1. If it could affect her at her young age then a discussion should happen.
ARVD can impact children, although it seems to be mainly teens where the disease first shows up. This is not like the BRCA genes where they don’t test children with first degree relatives who carry the BRCA mutations until they’re 18. They don’t test those children, because what could be done in advance of the adult years?

2. Keep it simple
We didn’t enter into a major conversation with loads of details.  We did the KISS approach. We answered the questions as she asked them, but we didn’t go into all the details at once. That would be overload. No need to talk about the implications for the future.

3. Control the environment
This was no rushed conversation before a doctor’s appointment. We knew that we had some quiet time to talk and discuss. She was somewhere she felt safe.

4. Keep it calm
It’s taken me two weeks since I received the information that my daughter has a mutation that causes ARVD to be able to talk about it without crying. I didn’t want to talk about it to her and burst into tears although I still do cry most days about it. We practiced a few times with other people before talking to her. Those pseudo trials with other folks helped a lot.