Category: The Drugs

It’s Official…

…I’ve had ENOUGH!

Holding it together these days hasn’t been easy, and I’ve cracked a few times, maybe a few too many.

Medically speaking I’m doing OK. I’m almost 10 months post heart transplant and the doctors still haven’t been able to fine tune my medication so that I’m not always adding or removing something I take daily. Some of it makes me sick, some of it makes me feel weak, and the big one makes me feel exhausted for no reason at all. I know my body is strong; how could it not be? I have Primary Amyloidosis (AL), which by the way, has a 6 to 12 month mortality rate, I’ve survived over four years. I had a heart transplant because the AL put me in acute congestive heart failure and caused a lovely case of restrictive cardiomyopathy. Now I’m told I have Osteoporosis in my lumbar 1 – 4 and am close to having it in my hips as well. All that is a result of the chemo therapy I received for the AL and the anti-rejection drugs I take for my heart.  I’ve been hitting the gym for several weeks now. Not knowing at the time I started about the Osteoporosis, it was a good thing I did. The most effective treatment for it is weight training, which is what I’ve been focusing on to build up the strength I lost while I was sick. But my back and hips hurt and although weight training is ideal, not necessarily at the pace I’m going at. I can’t win with this body of mine.

Then there’s the food. The trainer at the gym wants me to eat a certain way, which I’m totally willing to do within the parameters of what I can and can’t eat being a transplant patient. In reality it’s not that different than how I was eating before the transplant. However, my stress level is so high I can’t eat, and not eating is just as bad as eating too much. How do I get the calories I need in a day without throwing it all up because I’m so stressed out my stomach is in knots? I’m trying, but there isn’t a week that goes by that more stress isn’t placed on my shoulders by someone.

There’s also a family component to all this. Issues far too complex for me to even begin to figure out, and I’m not 100% sure I want to. I have two married sons, one of which doesn’t have much to do with us and his wife even less. The other and his wife live with us, which creates it’s own set of issues that I can’t address because it’s causes me even more stress than I can handle. I have a mother who I’m afraid of and I couldn’t tell you why, other than I hate the sound of disappointment in her voice when I can’t do what she wants me to. I still have no idea who my biological father is, which causes me more hurt than my mother can imagine. But I keep the peace because I think that’s what I’m supposed to do? I don’t know?

I left the church I’ve been a member of for over 10 years. That’s been a gut punch to say the least. I think what hurts more than anything is they either haven’t noticed or don’t care; either one isn’t great. I’ve tried to get past it and attend church, but I can’t get out of my mind how little they did when I needed them the most. I’ve gotten a handful of phone calls in the last five years, but no significant support for my “church family.” Now I’m torn about finding a new church because I don’t want to be hurt like that again. I surrendered myself to God in that church and the keepers of the church were not very good stewards of my vulnerability. How could I go back to that, and how do I try a new church not knowing if they will do the same thing?

School is starting in about two weeks and I go back and forth over whether or not I can handle it. Some days I feel confident that I can do it and others I think it could be a huge waste of time and money. I’m 47 years old getting a degree in dance! What was I thinking? I’m not sure I’ll be able to keep up, but I’ll never know unless I try. The fear in me wants to stop before I get started, but then I’ll always wonder if I could have made it.

Every way you slice it I have stress coming from all directions. Some of it I may have control over but some of it I never asked for, but have to deal with it none the less. I lose sleep at night thinking about all of the things I should have done or didn’t, or should do, but don’t feel confident enough to stand my ground. On the surface I may appear strong, and my body may be, but my emotional state is not. I wish I had the emotional confidence to tell the people in my life what I need to tell them and not have their reactions affect me so deeply. And, of course, the ultimate fear is that they will all stop coming around all together and I’ll be physically alone and emotionally alone at the same time.

Amyloidosis Speakers Bureau

I have the distinct pleasure of being a member of the Amyloidosis Speakers Bureau, a part of Mackenzie’s Mission to bring awareness to not only the general public but the medical community about Amyloidosis. I have my first speaking engagement in September at the University of Illinois College of Medicine Rockford. I’ll be speaking to a group of about 100 to 120 second year medical students about my journey from diagnosis through treatment. Below is my short bio that goes out to medical schools where I might speak. This is the ultra short, “down and dirty,” Readers Digest version, and of course you have to know the title of my bio…

A Fist Full of Pills

My name is Rayna and I’m a 47 year-old female with primary amyloidosis (AL) with cardiac involvement that is currently in remission.

I exhibited 9 of the 12 symptoms of primary amyloidosis (AL) initially: arrhythmia, diarrhea, tingling/numbness in hands/feet, weight loss, edema, feeling full quickly, shortness of breath, and enlarged tongue.

In addition, I was also experiencing: chronic atrial fibrillation, tachycardia, and long Q-T syndrome.

I started by seeing my primary care physician in 2012 at the age of 40 for an annual check-up. I was referred to a local cardiologist because my blood pressure was slightly elevated and my heart rate appeared to be irregular and too fast. Over 19 months I saw a local cardiologist as well as an electrophysiologist from Salt Lake City (Intermountain Medical Center). During that time, I started being treated for congestive heart failure and had the first of two cardiac ablations for chronic AFib. The treatment by both doctors was ultimately ineffective and I was referred to Mayo Clinic in Rochester, Minnesota.

I visited Mayo Clinic twice before I received a definitive diagnosis. I saw several doctors, most notably I was seen by cardiology and hematology, who both immediately suspected Amyloidosis. During my visits to Mayo Clinic I did 24-hour urine tests, a fat pad biopsy, a bone marrow biopsy, a right heart catheterization, several blood tests as well as numerous ECG’s, echocardiograms, and ultrasounds. The echocardiograms showed a thickening of my left atrium, which then led to having a heart biopsy that confirmed amyloid buildup. The Congo red stain confirmed primary amyloidosis with cardiac involvement (I was 42 years old). All other tests showed no other organ involvement; however, nervous system involvement was suspected due to a tremor I developed before diagnosis that I still have today. As an aside, at the time of my diagnosis the staging system for AL had not been established; however, if we were to go back and look at the progression of my symptoms, I was in late Stage 4 of the disease before I started receiving treatment.

Under the guidance of my hematologist at Mayo Clinic I received four rounds (16 weeks) of the CyBorD treatment with a local oncologist, however my light chains continued to rise. The doctors felt that I had two choices…stem cell transplant with a 20% chance of survival or do nothing and I would die within six months. Needless to say, a 20% chance of survival was still better than 0%. I then received Melphalan and an autologous stem cell transplant at the Colorado Blood Cancer Institute (CBCI) in April 2015 (I was 43 years old). At that time, I not only survived, but I achieved complete hematological remission and have not received any maintenance treatment since.

The healing time after the stem cell transplant took approximately 18 months, during which my congestive heart failure and AFib continued to get worse. I had a second cardiac ablation that almost completely resolved my AFib. In 2016 at the age of 44 I was referred to the Heart & Lung Transplant Department at Mayo Clinic for heart transplant consideration. In August of 2017 I was listed for transplant. After 427 days and transferring to a new a transplant center, I received a new heart in October 2018 at the University of Utah.

I’m currently 47 years old, have been in remission since April 2015, and am healing from a heart transplant.

Understandably, due to the nature of the disease and the lack of knowledge among general cardiologists, I was frustrated with the time it took to get a diagnosis. That being said, my cardiologist never gave up looking for answers and when he exhausted his knowledge base, he referred me to a facility that could offer me more than he could. I was also extremely frustrated with the medication used during the period before my diagnosis. Many of the common drugs used for congestive heart failure, AFib, arrhythmia, tachycardia, and edema did not work for me. We can’t know if the reason the medication didn’t relieve some of my symptoms was because of the AL or because I’m female, but it was frustrating none the less to constantly have to deal with side effects and the ineffectiveness of the medications during that time.

Lastly, what I can’t stress enough, is doctors need to learn how to say, “I don’t know” and do the appropriate follow-up to either find the answer or find someone who has the answer. Giving vague, non-answers is beyond frustrating when you’re facing a terminal disease.

Anyone who would like to see photos of my native heart can click here. Of course if you’re squeamish, I’d skip it!

My Scale is Evil!

I’m having a minor disagreement with my scale, it says I weigh more than I want to. It’s a problem a lot of women have, I just have it for a different reason. With congestive heart failure comes water retention and I fought that battle for several years. Now with a new heart the battle continues. Not to the same degree, but it lingers just a bit, enough to make me hate stepping on the scale every morning.

For all intents and purposes I’m “dry” according to the doctors, however the scale would argue otherwise. I don’t eat enough to maintain this weight; my medication knocks out my appetite. And the food I do eat is healthy, so, I’m not eating a gallon of Rocky Road every night. Unfortunately, a touch of anemia has left me a little tired the last several weeks and I haven’t been getting as much exercise as I’d like. Regardless, I’m tired of the extra pounds and would love for them to disappear.

There really isn’t anything that can be done about it right now. I’m still on that vile drug, Prednisone, and it causes your mind and body to behave in the most strange ways. Among a variety of awful side effects, “changes in the way fat is distributed in the body” is one of them. Now if the fat could be distributed to my rear end maybe I wouldn’t complain so much! I know my weight isn’t permanent, it’s just uncomfortable.

There’s a possibility I may own only one size of jeans in the future, but for now I’ll keep a variety of sizes just in case.

Advocate

Another short stay in the hospital has reinforced the concept that as patients we need to advocate for ourselves better.

It’s not necessary to get frustrated, anxious, angry, etc. Being kind, polite, and calm has been the way I’ve been able to get my point across to my medical team the last several times I’ve seen them either in an outpatient or inpatient setting. That’s not to say I’m not frustrated, anxious or angry; I’ve simply found that succumbing to those emotions doesn’t help me get what I need. What I need is to be heard! If they hear me then ultimately, I receive the care I need, not the care they “think” I need, which by the way, wastes a lot of time.

What’s the point of getting frustrated with the nurse who’s simply waiting on the doctor to put in the orders for your medication? Why get angry at the pharmacy tech because they’re out of Tamiflu and you have to go to another pharmacy to get it, it’s not their fault they’re out. And will getting anxious make a procedure go smoother, or test results come faster?

Most of the people we encounter on a regular basis as we go to and from our medical appointments didn’t go into this line of work to get rich. They started off with a desire to server others and care for them. By treating them with dignity and respect, they hear us, and in turn we get the care we need. I’m not suggesting this as an absolute, we’re always going to encounter the medical professional who doesn’t want to hear what we have to say, but I believe that’s the exception and not the rule. It’s all in our approach and how we advocate for ourselves.

Week 19…The Flu!

I spent the last 24 hours in the hospital because I have the flu. Kind of a bummer for a person who had a heart transplant 19 weeks ago, but my stay could have been longer had I not advocated for myself and made it clear to my medical team what I was expecting regarding my care.

Please follow my medication schedule to the best of your ability. I have it down to a fine science and deviating can sometimes cause problems. After being given a detailed list of my meds and when I took them, they made every effort to follow it; not to perfection, but good enough. I certainly appreciated the effort. I don’t feel “that” bad, and my blood work would suggest I’m stable, so let me go home and be sick in my own bed. It’s people like me in a hospital that makes hospitals huge germ factories. They had me in isolation, but every person in and out of my room was a potential carrier of my flu ridden germs. My doctor found my argument for release to be totally valid and put in orders for me to be released right away.

Now, I’m home in my little apartment, drinking my own bottled water (cold), eating my own food, laying in my own bed, not spreading my flu around, and significantly more comfortable than I was in the hospital.

The Pills

Not even all of them…

At some point in the future I’ll be able to dispense my pills by the week, but right now while we’re still tweaking dosages I don’t. I had a blood test today that I should get the results from tomorrow so I don’t want to sort my pills past Wednesday because they will likely change when the team calls with the results. Adding pills isn’t an issue but taking them out can be time consuming and a huge pain in the rear end. My bag is a little stuffed so I can’t fit everything because right now I take 23 different meds, I had to number them.

Yes, I use a binder to keep all my transplant information organized. They gave me a binder, but I like mine better, plus I added tabs! It has my schedule, med list, daily vital signs, an updated medical history, a POLST and advanced directive, current clinic notes, nutrition information, and notes. The transplant team seems to appreciate my organizational skills.

Some of these I will only take temporarily, some I may not need at all over time, but for now there are a lot to keep track of.