Month Two

Yesterday marked two months with my remarkable new heart. It has been a time of trial and error; tests, tests and more test; a time for new goals and achievements. This has also been a time of a lot of ups and downs. Thankfully, mostly ups.

I was able to begin a formal outpatient rehab program! That was exciting for me, as anytime I exerted with my old heart, I would go into even more arrhythmias than normal. So being able to be monitored while exerting helps reset my mindset. It has been so very nice watching the monitor and just seeing sinus tach and not tons of multifocal pvc’s! Today at the red center here in the hotel, I was able to go 42 minutes and peddled 7.4 miles. Doesn’t sound like much, does it. But considering 2 months ago, I had a hard time walking to the mailbox without stopping several times, it’s a huge improvement. I go go outpatient rehab m-w-f and still go to inpatient on Tuesday and Thursday. It forces me to get out every day. I am now walking to and from the hospital before and after my appointments. Again, it’s not a huge distance, but it is more than I would have been able to do before. THANK YOU DONOR!

I also had my fifth and sixth biopsies. So far, all my results have been 1r. So low grade infection that will continued to be monitored, but no changes in my medication routine. I have had weekly blood work. Unfortunately, we have had to do some constant adjustments with my tacrolimus, as my levels continue to jump all over the place. I’ve been told that this is very normal as my body adjusts to my new heart and my body settles in with the new drugs. Because of the high dose of prednisone and tacrolimus, I do have the shakes. It especially rears it’s head when I use fine motor skills. Trying to drink from a glass or write anything with a pen has been challenging, to say the least. I’ve been told, it will improve as I start decreasing the prednisone. And hopefully, that will happen next month when I come in for my next biopsy. I can just see myself standing in front of a patient to draw them, and my hand shaking holding the needle!

During this past month, I also met with a few new team members. I added a sleep disorder doc and a pulmonologist to my ever growing list of specialists. I pushed for both, as I’m still having problems with my lungs and my sats dropping,especially at night and when I am under exertion. I will be doing another sleep study this coming week (so not looking forward to that!). So will see what those results will show. Did I get rid of the central sleep apnea as my heart failure doc was hoping? Luckily, the testing will be done here at the hotel I am staying at, but up one more floor. So at least I won’t have to travel far, nor figure out how to get there! Sleep continues to elude me….whether from drugs or breathing issues, I guess we’ll find out next week.

I also met with the pulmonologist last week. He ordered some breathing test to measure my lung capacity. Good news is, I’m not making things up! Bad news is, I have a paralyzed left diaphragm. It has actually caused my breathing tests to be about 40% less than they were 18 months ago when I was testing to get listed. Also, when I lay flat, my numbers are greater than 25% less than when I am sitting. Not good for either. I know for any open heart procedure, they must cut the phrenic nerve (the nerve that works the diaphragm), and this is my second open heart surgery. So I asked if this could be the issue. He said more than likely. However, I was having issues with my sats dropping starting about 6 weeks before I got my call. Since I never had the appointment with a pulmonologist before transplant, we don’t know the reason. It could have just simply been that my heart was failing faster than we thought and with my blood pressure being so low, I just wasn’t being perfused appropriately. We may never know. He did have me meet with a respiratory therapist to give me a new little toy to play with. This one is called an inspiratory muscle trainer. It is supposed to help strengthen the surrounding muscles to help with breathing. Kind of assisting the diaphragm in its job, as it hopefully begins to recover. I asked if this was a common occurrence after open heart. He says for him it is, but this is what he does, so he sees a lot of it. But, in the overall scheme of things with all the surgeries done, it’s relatively rare. Lucky me! He did say to continue with rehab, as movement will definitely help.

So, as of right now, I am scheduled to go home next weekend..lord willing and the creek don’t rise! But of course, long term weather shows that there is supposed to be a huge winter storm hitting pretty much our whole route home!

Lou has been working hard on getting the house as germ free ready as possible. He will be working with a plumber next week to get a UV water filtration system installed, as we are on well water and cannot guarantee how safe the well water is. Even taking a shower could be hazardous for me at this juncture. He converted our covered back porch into a temporary storage area, later to be our eBay room. And yesterday, we got a new washer delivered. Our old one was about 15 years old, and had some mold around the gasket. Lots of changes and lots to learn!

Thank you to donors and their families everywhere! Especially thankful for my donor and their family as they go through this year of firsts.

Another visit, another surgery

Well, the next several weeks flew by, and it was soon time for another trip to Cleveland.

In the meantime, I had been able to find and secure us a place to live. Lou was still making his treks across the country getting our stuff out of Utah and preparing to move into our new house. The owner allowed me to take early possession so that I could begin to get it ready to receive the horses. I had found fencing and other supplies, and Louis was kind enough to come out and help me, not only pick up supplies, but to put the fence up. I was excited. The horses were going to have some grass….almost 5 acres….to munch down. And, we were not going to have to worry about snow and freezing weather, as we were already into May.

Again, Lou and I arranged our schedule for me to fly in and him to be in Cincinnati. He would pick me up at the Cleveland airport and we would make our way to the hotel. My procedure was scheduled for the next day, early. I had been off my coumadin for the last 4 days in anticipation of this procedure. It had been a very long day for both of us, and it was promising to be a long day tomorrow. I knew I would have an overnight stay, so Lou would be able to stay in the room with me.

Well, the day dawned, as it is wont to do. We had arranged for an early morning ride into the clinic via the shuttle, and our truck would just stay in the hotel parking lot for the time being. Once I got released, we would come back, stay the night and head out the following morning. The shuttle driver dropped us off at the entrance, and we proceeded to get checked in. Once checked in, we made our way up to the cath lab area and sat in the waiting room.

A few minutes later, my name was called…but I was only taken into a little closet type room to check my INR. It had come done nicely, so I was set to go. I went back into the waiting room to wait until I would go back into the prep area. About a half an hour later….I was paged. I went back by myself to get ready. I got an IV placed, hooked up to the monitor, vitals taken and then another wait. Pretty soon, an EP fellow came to consent me to the procedure. In the meantime, Lou had been paged and he was allowed to come back until I was taken into the cath lab. Dr Bhargava and his surgical team made it into my cubicle where they verified who I was and the procedure I was there for. Then off to the cath lab.

One thing I can tell you, walking into the cath lab is like waking off a plane onto Antartica. It is FREEZING in there! So, of course I asked for nice, warm blankies! Then I was mostly out. I remember bits and pieces of what went on, but most of it is a blur. The next thing I remember for sure, is I was in recovery and screaming because my back was spasming so badly. I found out later, I had been lying flat on that hard table to about 8 hours…so even longer than the time I was cut open for my myectomy.

Dr Bhargava had told Lou it would probably take about 4-5 hours, and then when it was done, Lou say him walking out into the waiting room and pulled him into another little room. Lou thought the worse. Dr Bhargava explained that, even though my arteries were clear, the artery he wanted to place the left wire had a sharp angle that made it very difficult to place it correctly. As it was, he was only able to get it about 50% of what he wanted to do. But it was in.

I was eventually taken to my room and rest. They have the best nurses at Cleveland! Everyone is so helpful. I asked for their most amazing water filled heating pad machine. It helps my back so much, especially being on those awful hospital mattresses. I was finally able to get settled down. The nurses had brought in pillows, sheets and blankets for Lou to sleep as well. It had been a very long day.

The next day, Dr Bhargava came in and went over what had happened. He wanted me to follow up with another interrogation in a month. But, I could do that locally. Other than that, I was free to go, and to follow up with him when I came back in about 3 months to see Dr Thamalarasan and Dr Tang.

We were released. The calm before the storm.

The Next Two Years

I could not believe the difference in concern and care with my new group! After 2 years of not being heard, I was in tears. Someone was not only listening to me, but actually asking me to take part in my care! Blame was shifting away from me and onto this disease. I was learning more and more about what living with HCM meant for me, my life, and my family. I tried talking to my family and explaining that this was a genetic disease. I even got gene tested…although that came up as inconclusive. Remember, only about 50% of the genes have been identified. So, even though I had a wonderful group of medical providers, I was still hitting a roadblock with my family. Frustrating is an accurate word!

Onward and upward! My whole care plan was adjusted. More testing was done. I found out I was obstructed….which was what was causing the majority of my symptoms. Surgery was discussed, but dismissed (by me!). I was terrified of the thought of having to go through open heart surgery! I started doing more research and I stumbled upon the HCMA Facebook group. What a godsend they were! I admit, I was a lurker for several months before finally posting my first post introducing myself. So many were encouraging and trying to put my fears at ease about surgery.

But, me being me…..I wanted to put it off as long as possible. My doctor, Kia Afshar, told me about a trial they were participating in with a drug called perhexiline. He thought I might be a good candidate. The drug company was trying to determine if this drug could have an impact on exercise capability and tolerance in the obstructed group of HCM. And, as my exercise tolerance had decreased dramatically, I was all for trying. So, I went through the consenting and screening process.

One of the tests that I had to do was called a VO2 Max. I had never heard of it, but apparently its pretty popular with athletes. It measures how well your body utilizes the oxygen it takes in, and how well you recover after exercising. What a fun test! (NOT!) I would tease each time, I felt like a pilot in a fighter jet plane. You wore a contraption similar to their helmets. For this test, I could choose to do treadmill or stationary bike. As a group, we decided the bike would probably be my safest route. Not only did I have to wear this funky mask, but I also had to be hooked up to the monitor. No chance for modesty during any of these tests!

On goes the nose clamp, then a hollow “bite” tube for breathing. Then a pulse ox attached to my brow. All of this held in place by straps that go around my head in various spots. On to the bike I climb.

To do this test, that exertion intensifies at measured intervals. So, every 2 minutes the resistance would increase. I had to keep my “speed” between a certain parameter, I could not close my eyes, and I had to hold on to the bars. My blood pressure would be taken every minute. They wanted me to “go” as long as I could and gave me a number (7 minutes) to shoot for. Nope, didn’t make it! I think I lasted 4 or 5 minutes. I had to wait an hour after this test to do what was called the walk test. How far I could walk in a premeasured distance in 6 minutes. After all of this, I finally got to meet with my doctor.

I was excited and nervous at the same time. Hopefully I would pass the screening process and be put on the study…..and since it was a phase 2, I would be getting study drug! Hopeful was my name!

Dr Afshar came into the conference room and went over the results thus far. EKG, blood work, echo all fit well within the protocol guidelines. Then he started talking about the VO2. He threw out some numbers and then mentioned a word that literally stopped my world. I heard nothing after that.

TRANSPLANT!

Are we talking about me?!?! Transplant? What?

For this particular study, I had to repeat all these tests within 2 weeks and fall within a certain guideline to be enrolled. I decided to use these next couple of weeks to find out more information on what the VO2 results meant. What I found out devastated me. My first VO2 result was 11. Someone for my age and sex, to receive a poor number, was roughly between 27-30 (depends on the chart you look at). Below 12 they start talking transplant.

On to round 2 of testing! Again, after echo, ekg and VO2, I met with Dr Afshar. My VO2 number came back at 9 this time! I could just cry!!! We were thinking that I might be too sick to be enrolled, but all the numbers and tests were sent into the drug company to analyze. I remember, I was really down on myself during this time. Nobody I could really talk to that could truly understand, except the HCM group. I’m sure they were getting sick of my boohooing. But, I honestly had nobody else I could turn to.

I finally heard back from the study coordinators. I was accepted into the study!! I scheduled my first visit, and looked forward to trying a new drug. I so did not want surgery! In the meantime, I met with the EP (the original doctor I had scheduled with) and the genetic counselor. It was decided because of my family history, multiple episodes of NSVT (non sustained V-Tach) and multifocal PVCs, that an ICD would be very appropriate. So, we got that scheduled and I started taking study drug.

Let me digress for just a moment and do some explaining of terminology.

EP – Electrophyisologist (the heart electrician, if you will)

NSVT – Non Sustained Ventricular Tachycardia (a fast heart rate – greater than 100 – that originates in the ventricles) This can be life threatening

PVC – Premature Ventricular Contraction In and of itself, this is a pretty benign arrythymia…we all get them. Kinda feels like a hiccup of the heart. In my case, I have PVCs that originate in different parts of my heart, and a lot of them. Again, all in all, not horrible, but could be a sign of an unstable heart.

For the next 4 months, I took my study drug, in addition to my other drugs, and had my ICD implanted. I was feeling pretty good! I found out that my coordinators had enrolled another fellow hcm’er into the study and he was doing well, too. End of the study came, and time to do the exit testing. I was kind of excited to see how well my numbers increased, since I did feel so much better!

Come to find out, it was a short lived euphoria. My number did go up from 9…..up to 10. I was devastated. The week after my exit testing, the drug company actually pulled the study. They were not getting the numbers they had hoped for. I finished the study, but the other guy in my group did not. But we both agreed we should go to Europe and get some of the drug! (Perhexiline is available in Europe and in Australia).

My next followup appointment with my doctor, we discussed in detail the need to myectomy. But, there was one more option to try. Its a drug called Norpace that has had some positive results for some of those with obstruction. He was willing to give it a shot, but I would have to do an inpatient loading of the drug to make sure I didn’t develop long Q-T. We scheduled a time for me to come in….after work on Friday, and to be discharged on Sunday evening….and a weekend Dr Afshar was on the floor.

Into the hospital I went. Unfortunately, the nursing staff did not know how to “manage” me. I was not their typical heart failure patient, and the doctor allowed me to go anywhere I wanted within the hospital. I just needed to be there to get my drugs, and to do my ekg’s. I even had to educate a couple of the nurses about this disease, as they had never even heard of it! I stayed my weekend, no long Q-T and got discharged. I gave it the old college try, but 7 weeks later, and I noticed no difference in how I felt. The end had come. No more options, except SURGERY!