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!

Write Something Today

I started using  a Full Focus Planner, and I put on my daily list to “write something” the last three days in a row. Monday and Tuesday I deferred it to the next day, but today I was determined to get thru my list. A lot has happened since the last time I wrote something.

I had my 22nd heart cath and my biopsy came back with no rejection! All of my labs came back fine and my heart is doing well. The hematologist is still mystified by the bruising I’ve been experiencing for several months. It started before I moved back home and has only gotten worse. They are now looking for genetic forms of anemia. So far I have genetic markers for two, but why would their symptoms show up now and not earlier in my life. For now I’ll just deal with the bruises. They aren’t life threatening in any way, just an annoyance really. Especially when one shows up in an inconvenient place, like my face.

I’m still dancing, only one day a week but it’s a start. I’ve been loving it! Loving it so much that I took the leap and registered for school to get my degree in dance. To get prepared for a heavy schedule of dance classes starting in the fall I hired a personal trainer to get me into shape. So far I’ve been getting my rear end handed to me. I’m taking a Buff Camp five days a week and following a strict meal plan. It’s taken some getting use to, but it’s totally workable. If anything it’s a lot more food than I’m use to eating, but with my increase in activity I need the calories. The next thing I need to start working on is my flexibility and balance. Bother were affected by the lack of mobility while I was sick.

I’ve been trying to get to yoga twice a week. I can’t express how relaxing it’s been and healing. It seems like such a simple thing but it’s been working for me. The challenge is going to be to figure out how to fit weekly yoga with my school schedule, but I’ll cross that bridge when I come to it.

 

The Challenge

The beginning of the year I challenged myself to get my camera out every day and take a photo of something or someone. The purpose was to get reacquainted with my camera that had been sitting for so long like a paper weight, gathering dust. I went 84 straight days of taking photos, some not so bad, most pretty crappy if you ask me, but they were pictures none the less. Unfortunately, being stuck in Salt Lake City recovering from a heart transplant in the middle of Winter doesn’t always spark inspiration. In the Winter, Salt Lake City is shades of grey, and after awhile I became bored and somewhat depressed over the lack of color.

So, I took a break and was able to come home to Wyoming, where there wasn’t much color either, just more snow.

I fell short by seven days of a three month streak by missing the last week of March. April was a wash because I really didn’t feel like picking up my camera. I was and am so overwhelmed at home that the thought of getting  up and finding something to shoot every day felt a little burdensome. I don’t want a hobby I truly love to feel like a burden. The camera came out of the bag this last weekend though, when I went to the dance showcase of one of my former Ballet students. I was able to capture some amazing photos of her thru my tears as I watched her. She most certainly sparked some inspiration in me to pick up my camera more often.

So, here is #85 – “Dear Clarice” featuring an amazing young dancer that I’m proud to say graced some of my Ballet classes when she was younger. She’s so grown up now and blossomed into the amazing dancer I always imagined she’d be.

View the entire Gallery.

Dear Clarice