Category: The Doctors

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!

Apprehensive

After having so many health issues over the last 6+ years I’ve become a bit apprehensive when it comes to seeking out medical treatment when maybe I should. Right now I’m sitting here, no doubt, in denial, that the pain I’ve had in my upper left abdomen will go away if I ignore it long enough. The fact is, it’s getting worse and starting to radiate toward the right side of my abdomen and around to my back. I’d like to tell myself it’s just a pulled muscle or something, but I know it’s not. Who knows what it is, all I know is the thought of having to go to my local hospital scares me to death.

Out here in Wyoming our medical options are limited, not to mention my issues are way above the pay grade of the doctors around here, and I really don’t want to make a trip to Utah to see my transplant team right now.

What I’d like is to have a single day where I’m pain free. Fighting Amyloidosis and then getting a heart transplant doesn’t come without a little discomfort, but my pain threshold is starting to wane. With that my mood isn’t the greatest and is probably bordering on depression. I feel like I need a break…

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.

VOICES of Amyloidosis

This is an awesome project! I didn’t participate because at the time I was dealing with the whole heart transplant thing and was pretty sick. But if you want to see what the face of Amyloidosis looks like watch these videos. They aren’t long but their full of amazing information and stories.

VOICES of Amyloidosis Part 1 of 5

VOICES of Amyloidosis Part 2 of 5

VOICES of Amyloidosis Part 3 of 5

VOICES of Amyloidosis Part 4 of 5

VOICES of Amyloidosis Part 5 of 5

A First Look

Here’s a first look at the Post Its that I put on the wall in my ICU room while I was waiting for a heart…there are over 200. Included are also the number of days I waited for a heart in the ICU and the names of the doctors, nurses and aids that took care of me.