The Weekend

What a miserable way to begin the new year!

I couldn’t believe I would be stuck in the hospital. I was so scared and angry. I still had no idea what was causing all of my problems, especially my blood pressure. I kept asking myself WHY ME?!?! What did I do to deserve this?!?! I was getting no answers from the universe, or the doctors.

I had no idea what the morning would bring, but sleep did not come. And, as most know, sleep in the hospital is kind of a joke. At around 4am a knock on the door brought the resident vampire. Time for more blood work. In and out, thank goodness. I just wanted to be left alone.

Soon, the rest of the floor started to awaken. A new day begins.

I was to find out, blood tests were the first of many tests to be done that day. Xrays, scans, imaging, you name it, it was done. Finally met with the weekend oncall doctor. Right away, we clashed. He didn’t feel as if I had the right to ask questions about MY health! Lou liked him. He felt he was right to the point. Of course, everyone was still blaming me for my health issues. It didn’t help my mood at all. But still no real answers to what was going on. And my blood pressure continued to remain high.

Trial and error with blood pressure medication finally started working. Unfortunately I was on 5 different medications just for blood pressure. There was one more test the doctor wanted to do, but required a special medication that had to be flown in. That night we got a snowstorm, and the plane was delayed. So, another night in the hospital.

I also remember during this time, I found out that the hospital I was in was out of network with my insurance. I knew it was an emergency admission, but I was panicking, so placed a call to my insurance company. Wrong move! The gal that answered the phone was less than helpful. And quite honestly, made things worse. According to her, I should have gone to an in network hospital. I explained what happened and the reason I couldn’t go to another hospital…and I told her what my heart rate had been. Quite seriously, she then asked me…”Is that high?” I was incredulous! Seriously!! A person working at a medical insurance company, and she didn’t even know BASIC information?!?!?! I began yelling into the phone. Lou heard me and came back into the room. I explained to him what was going on, and that we would be responsible for the out of network charges….which by this point, was nearing over the 6 digit mark. I started packing up my stuff. Lou asked to take my phone and speak with the person on the other end. He then walked out to the nurses station to “chat” with her. Not really privy to the whole conversation, but the outcome was that the insurance company would be paying the bills as if it were in network. Pshewwww!

Sunday morning. The doctor comes in and explains the delay for the drug needed to do one last test. As we were talking, I asked if he had done certain tests to see if it could help answer my high blood pressure issue. Nope, he hadn’t. At least he listened to me on this part. The tests were ordered, along with a KUB (kidney, ureter, bladder xray). More blood work, and back down to xray. Just like all the other tests, they came back negative. One last test…just waiting for the drug. And, I needed to be NPO (just sips of water to take my numerous drugs!)

Finally, around 5:30 that night, they came to get me to do the test. It took forever, and got back to the room around 8pm. Still not allowed to eat or drink anything pending test results. I was just ready to GO! About an hour later, the doctor walked in. Test was negative and I was free to go. Of course, being a hospital, checking out is quite the ordeal. I think it was finally about 10-1030 that night that we got to leave.

I was so very glad to leave, but without answers. I was to follow up in 2 weeks with the doctor I really had no respect for. We shall see how that works!

Cardioversion

Sorry to leave everyone hanging…but life and work kinda got in the way!

Cardioversion!! The next step in trying to get my heart back into a normal sinus rhythm.

Yeah, I knew what it meant, as I had done it several times on other people during my time as a paramedic and ER tech. Shoe is on the other foot, as they say.

What exactly does this mean? Well, remember, my heart was in an arrhythmia called rapid afib. In order to get my heart back into normal sinus, drugs are typically the first line defense. But, multiple types of drugs didn’t work for me. This next step requires the placement of two electrode pads to be placed on me…one on my chest and the other on my back so that they are kinda mirroring each other. (Sometimes you will see them placed one below and to the left of the left breast and the other towards the right shoulder. Either way works.) Then an electrical impulse is sent to the pads and the heart is (hopefully) shocked back into a normal rhythm.

The doctor came back into the room and asked if I minded if a medical student could join us. Of course not! So, the medical student started to place the pads on me, and I helped direct the placement, as he had never even seen it done, let alone done one. Got the pads placed and hooked up and we were set. The doctor came back into the room, and had the nurse give me the “twilight” drug…typically versed. But I honestly don’t remember what it was. This was supposed to help sedate me, but still be awake and then help me to forget what happens next. SUPPOSED to help…..KABAMMMMM!! (I need to find one of them Batman cartoons with all the funny captions!) However, it wasn’t funny. Yes, I totally felt the jolt and I totally remember the feeling. But even more importantly, IT WORKED!!

So, heart was back into normal sinus rhythm, but my blood pressure was still sky high…even after all the drugs I had been given. I found out that the ER doc had been in contact with the cardiology department during my stay…so he came back in and told me I was being admitted. NOOOOOO!!!! Yes, I did actually say it. Even though I knew it was inevitable. UGHHHHH. I didn’t have the time or money to be sick! I called my supervisor back and told her I was going to be admitted and that she needed to find someone else to work the next day because I didn’t think I would get released that night either. I was so angry and so very depressed at this point. I’m sure I acted like a petulant child….but I was so done with all of this! And it hadn’t even started. Little did I know, this would be the start of a 2 year long battle to get at the truth of what was happening to me.

I was rolled up to the step down unit and got settled into my new bed. No sooner than that happened, when the echo tech showed up at my door along with the admitting doctor. The tech did a quick echo…lasting about 15 minutes…while the doctor watched. After the echo tech left, the doctor invited my family into the room. He told them that my heart wall was thickened, and the reason it was thickened was because of my extremely high and uncontrolled blood pressure. He estimated that it had been going on for at least 7 years. I tried to explain again that that couldn’t be the reason, as I hadn’t suffered with high blood pressure before. I did biometrics every year for work, and my blood pressure was generally in the 130s/80s. Again, nobody paid any attention to the body on the bed. Yes, I was angry…but even more to the point is, I was very hurt. Why wouldn’t anyone believe me?!?! Of course, everyone started blaming themselves and apologizing. I just sank deeper into myself and tried to drown out the noise. The rest of that day, and even into the weekend, a lot more tests were ordered and performed. I found out that my BNP, troponin and CK levels were elevated. BNP is a marker for heart failure, while troponin and CK are markers for muscle damage….in this case specifically, cardiac. Because of the blood work, at first they were thinking heart attack. So more tests were scheduled for the weekend to rule out or confirm various potential problems.

So, my first night in the hospital, I went to bed not knowing what was happening and scared of what the future might hold.

The Journey Begins

January 3, 2014

Such an innocuous date, isn’t it?

This is the date that would be the starting point of this path I am now traversing. And one that I remember every detail.

It started innocently enough. Got up, got my shower, got dressed…just a normal, every day occurrence. I had done this for so many years, it was just automatic. Except, that day I needed to take my box of homemade soaps and lotions, as I would be meeting with a potential customer after work. I was loading the box in the back of my car, and it started. “Oh crap!”, I thought. “This is NOT good!” Being a former paramedic, I had a very good idea what was happening, but without any equipment, I couldn’t know for sure. That day, I was scheduled to work in an ob/gyn office at St Marks hospital in Salt Lake City, Utah. All the way in to work, I could feel my heart racing. I couldn’t count the beats, nor could I break the cycle. I would try another couple of tricks once I got to work. Once I got parked, I just sat in my car for a bit….I didn’t want to get out, but I didn’t want to stay there. I kept looking at the insurance card I took out of my wallet….knowing I would most likely be using it. I was terrified. I was angry!

I finally found the courage and the strength to leave my car. I rode up in the elevator to the 5th floor office. Once I got in there, I immediately went into the bathroom to try my remaining tricks..all to no avail. What I tried was called the vasovagal maneuver. It is done by bearing down like you are having a bowel movement. I also tried splashing cold water on my face….all to try to stop my heart and hopefully have it start back again in normal sinus rhythm.

I went to my desk and noticed that I already had a patient waiting for me to draw. I was feeling pretty crappy by now. I went out to the lobby to grab her, put her order in the computer, and drew her blood. I was shocked I was able to do it. I was very shaky and dizzy. The receptionist brought in another order for me….this time it was for a 3 hour glucose. I was able to get the patient back, put the order in the computer, draw her fasting blood, give her the glucose to drink…and I was done. The receptionist brought another order in for me….said, “You look horrible!” Told her, I wasn’t feeling good and that I thought I needed to go to the ER. She asked if I thought I could make it down by myself. I told her no.

I called my supervisor. I said “Jenn, I don’t feel so good, I think its my heart.” She told me to get down to the ER. I was still waiting for the wheelchair to get me down there. I could barely talk by this time. I called Lou, my husband, told him I was going to the ER, something was going on with my heart. Finally, the wheelchair got there! Got into the wheelchair, grabbed my stuff and waited in the hallway for another 20 minutes. All the time my heart was racing.

Finally, someone from the office started pushing me downstairs to the ER. Got down to triage, nurse asked me what was going on. I told her “I think I’m in rapid afib. I can’t count my pulse, nor can I break the cycle.” I don’t think she really believed me. She took her time putting on the pulse ox. As soon as she saw the number, she rushed me back into one of the trauma rooms, where I had 3 other nurses come in to help me get undressed and hooked up to the monitor, IV’s started, blood pulled and sent to the lab.

Being me….I asked what they saw, because of course, the monitor was behind me and I couldn’t see it very well. Nurse said vtach. I said, “are you sure? I think its afib, because I couldn’t break it.” About this time, the ER doc walks in and asks the same thing. Nurse said vtach. I asked the doctor to look again, because I thought it was afib. Sure enough, afib. Rapid afib. My heart was was about 188. And I had been in this rhythm for going on 3 hours now. The other issue I had, was my blood pressure was sky high. That is probably the only thing that kept me conscious this whole time. Problem is, I had never had issues with my blood pressure before. So really didn’t know what was going on.

Doctor came over and started talking to me and about that time, I saw my parents outside the room. What? I did a double take. I kept answering the docs questions….health history, medications, family history, etc. Then listened to my heart and lungs. He told me he was going to try some drugs to break the rhythm.

While he was getting that together, my mom and dad walked in. When asked, they told me Lou had called them and they rushed up there.

Drug number one given. Rhythm broke for all of 2 minutes…down to a low of 155. Drug number 2 on board….nada. Drug number 3. Rhythm broke for a bit longer this time, down to the 150’s for all of 5 minutes, then back to the 180’s I went. Doc came over to me and said “You know what’s next”. Yup, I did, as I had done it myself many times.

Cardioversion.

What is Hypertrophic Cardiomyopathy?

HCM.

Three little letters that don’t mean much to most people.  But to those of us that have this disease, it can and does stop your world when you hear them.  Am I going to die?  How much time do I have left?  Will I have a good quality of life?  These are just some of the questions we wonder about and ask.

To others, tears of relief are shed…as we now have a reason for all the symptoms we can have! 

The truth is, for the vast majority of HCMers, quality of life is minimally impacted, if at all!  Actually most people and families don’t even know they have the condition, until it is discovered at an autopsy.  Signs and symptoms are dismissed as other issues, even by the medical profession.

Hypertrophic Cardiomyopathy is the most common genetic heart condition in the world. And, unfortunately,  it is the one that is least understood.  It is estimated that this condition affects 1 in every 500 people, and can be as high as 1 in every 200.  The vast majority of people are unaware that they even have this condition, even when they are symptomatic.  The symptoms are often contributed to other causes, such as asthma or anxiety.  Because so very little is understood about this disease, it is stressed that the person be evaluated by a cardiologist well versed in the variabilities of HCM, even within the same family.

Cardiomyopathy is a condition in which the muscle of the heart is abnormal in the absence of an apparent cause.   Hypertrophic cardiomyopathy is an excessive thickening of the heart muscle (hypertrophy literally means to thicken).  In addition, microscopic examination of the heart muscle is abnormal.  Normal alignment of the cardiac muscle cells is absent, and instead you see myocardial “disarray”.

a) normal cardiac muscle
b) cardiac disarray
c) microscopic view of cardiac disarray

While HCM is typically recognized by its structure, the electrical component of the heart can be adversely affected as well.  Arrhythmias, irregularities of the heartbeat, are a common complication. Symptoms such as palpitation may occur, but not often. Exercise testing or Holter monitoring may detect them. The arrhythmias called ventricular tachycardia (arising from the ventricles) or atrial fibrillation are particularly important and may require treatment. In atrial fibrillation, the normal regular rhythm of the heartbeat is lost and replaced by an irregular rhythm, which may be episodic or persistent. The loss of normal atrial (the top of the heart) contraction produces a risk of clot formation in the atria. Anticoagulants and drugs to slow the heart rate are required.

Some other complications that can arise are:

  • Endocarditis – infection of the heart (rarely occurs with HCM)
  • Heart Block – the normal electrical signal to the ventricles may be slowed or blocked completely
  • Sudden Death – sometimes this is the first sign of any problems, it can strike at any age, estimated to affect 1%-2% of the HCM population
  • Heart failure –
    • Fluid congestion – heart becomes less effective as a pump resulting in the body trying to compensate for it, clinical symptoms can include shortness of breath and edema
    • Reduced blood flow to the body – isn’t always apparent in the early stages of heart. failure, often unmasked during increases in physical activity.  In advanced heart failure, organs and tissues may not even receive the oxygen required to properly function.  Symptoms can include difficulty exercising, dizziness, increasing fatigue.
  • “Burn Out” or “End Stage” HCM – occurs in about 3% of the HCM population, poorly understood why this happens, similar to dilated cardiomyopathy, heart reaches a point of generalized weakening and thinning (often referred to as remodeling), ejection fraction goes below 45%, often leads to transplant
  • Diastolic Heart Failure – occurs when signs and symptoms of heart failure are present, but the ejection fraction is over 45% but less than 60% (termed preserved heart failure), raises pressure gradient of blood in pulmonary vessels which causes fluid to leak into the pulmonary alveoli causing pulmonary edema, impairs oxygenation of blood in the lungs, causes shortness of breath, can cause death if not treated promptly, managed closely by medications and treatments, can lead to transplant if medications and treatments fail

What can you do?  If you have a family history of sudden unexplained deaths, been told you have an “innocent” murmur, or know that your family has a history of hypertrophic cardiomyopathy, please see a cardiologist well versed in HCM and all its variabilities for a full workup.  The HCMA (4hcm.org) has a list of places that are designated Center of Excellence for HCM on their website.  If at all possible, make an appointment with one of these centers for the most optimal diagnosis and care.  If it is determined you do have HCM, then your first degree relatives need to be screened as well.  Try and do a detailed family tree prior to your appointment, this may help establish history.  But most of all, be an informed patient!!  YOU are your own best advocate!

I wanted to start out this blog by explaining a little bit about my condition.  As I have been symptomatic for over 5 years, a lot of this will be written from my memory.  I will try to be as open and honest as possible.  Please don’t hesitate to ask questions and comment.  I am hoping this will help me get through the next steps in my path towards transplant.

Thank you for joining me on this journey.

Debbie