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!