March is Amyloidosis awareness month, right now you are probably thinking "what is Amyloidosis?" and "why have I never heard of this?".
Well if you were thinking that you would not be alone, many people have never heard of Amyloidosis, it is not something that you hear about in the media. When I was diagnosed with it I was the only person in my county ever diagnosed. I live in El Dorado County in the Northern part of California with 188,722 people that live in the town. My general and heart doctor had only heard about Amyloidosis in school.
Mayo Clinic overview states:
Amyloidosis (am-uh-loi-DO-sis) is a rare disease that occurs when a substance called amyloid builds up in your organs. Amyloid is an abnormal protein that is produced in your bone marrow and can be deposited in any tissue or organ.
Amyloidosis can affect different organs in different people, and there are different types of amyloid. Amyloidosis frequently affects the heart, kidneys, liver, spleen, nervous system and digestive tract. Severe Amyloidosis can lead to life-threatening organ failure.
There's no cure for Amyloidosis. But treatments can help you manage your symptoms and limit the production of amyloid protein.
Amyloidosis is rare with fewer than 200,000 US cases per year. Many experts suspect that Amyloidosis disease is not that rare — it is just rarely diagnosed.
There are several forms of Amyloidosis the 3 most common are:
AL – amyloid Light Chain
AA – amyloid Serum A Protein (also known as SAA)
ATTR – amyloid Transthyretin (also known as TTR) protein
The form I have is called IAA - isolated atrial amyloidosis.
When amyloid deposits in the heart it causes the heart to become unusually thickened and stiff, basically hardening the heart making it unable to function efficiently. This will cause shortness of breath with little exertion . Amyloid can also affect the electrical system of the heart, causing your normal heartbeat to speed up or slow down, which was my first symptom.
My story with Amyloidosis starts around the end of 2006. It was early evening and I was sitting on the bed talking to my best friend on my cell, I stood up and the next thing I know I am on the ground and I can hear my girlfriend yelling my name on the phone. I grabbed my phone and told her I blacked out and hit my head, told her I would car her back in a min and hung up. Now this is the 1st time this has ever happened to me and at the time I was a CNA so I looked at my symptoms, I was sweaty, heart racing, shaking. With all of these and a family history of diabetes (not me) the 1st thing I thought was I was low in sugar. I hobble over to my fridge and I get OJ and sugar and take both then go sit on the couch. I called my girlfriend back up and watched some shows then went to bed feeling fine the next day, no other symptoms.
The next day I wake up and I have to take my daughter to a doctor appointment with a new doctor that we have never met. Now they know this appt is for my daughter as they had asked her age when they made the appt. We sign in at the desk and the receptionist ask who the appt is for, I point to my young daughter. The receptionist ask if I was a patient here? I reply no I am not yet one. She then states that they will not be seeing my daughter and they are going to see me instead and asked if I was feeling ok this morning. I tell them that I am feeling fine and drove here to the appt but I did blackout last night. They take me and my daughter straight back to a room, then they take my vitals and leaves the room not saying anything. My daughter and I are so puzzled by this time. A few mins later she comes back in and hooks me up to an EKG still really not saying anything, now like I said I am a CNA so I know what this is for. She leaves the room again and what seemed like forever but only maybe 5 mins the doc comes back in and tells me they are sending me to the hospital. I am scared and freaking out by now. This was the start of all my problems. I was in A-fib with blood pressure around 190/130. I had no heart problems before that and my blood pressure was typically low around 110/65. Stayed 3 days in the hospital with a cocktail of meds to straighten things out.
For the next year we just kept the a-fib and BP under control and I would have occasional blackouts still, was hospitalized 10 times that year. Towards the end of 2007 my pulse started dropping in the eves, it was noticed in my many stays in hospital when I slept at night. It would get into the 30's and 40's while I slept. During the day I started feeling faint often, it did not matter what I was doing. My pulse started getting even lower during the day but my BP was still high. If I felt a hot flash coming on I knew that was telling me I was going to blackout again so I would stop what I was doing and sit/lie down or hold onto something. Well I was at Living Light Institute in Fort Bragg a raw food chef school and I was cutting something in class and I blacked out and sliced my finger, luckily someone was walking behind me and caught me. They called 911 and when I came to I was told my pulse was 20.. EEKS that is really LOW! The hospital told me that I needed a pacemaker but they are a small hospital and it would take days for this to happen, so I decided to just go home. At home I thought "I do not need a pacemaker, that is so permanent and I can BEAT this". Boy was I wrong for the next couple of months I kept having dizzy and fainting spells with my pulse staying in the 20's. In Feb of 2008 I ended up in the ER again after I blacked out and they would not let me leave, they sent me to the main Mercy and I got my pacemaker. They set it to keep my heart rate at 70 BPM, it does it's job well and many times has worked 100% of the time. I will be getting a new one the end of this year so the 10 year battery will have lasted almost 8 years (just to show you how much it does work). Whenever they test it and turn it off for a sec my pulse drops down quickly.
The next couple of years my a-fib and High BP were still there but now I also got Flutters and my heart was enlarging on the left side. The doc decided to do a Heart Ablation to see if he could correct my heart Rhythm. Well that worked but for only a year, DARN. Docs could still not tell me why I was having heart issues and would just say it was a problem with my electrical that I did not have a problem with my heart.
So now we are in 2010 and it is getting hard for me to do anything, everything takes so much effort to do and takes my breath away. By everything I mean everything like just getting up to go to the bathroom. I am showing signs of CHF, I am spending most my time on the couch. The end of the year the doc has a Right Heart Cath (RHC) done and it says that my Mitral valve is very leaky and heart is enlarged.
So Feb of 2011 I have my 1st open heart surgery to repair my leaky valve and they also did a procedure called MAZE (scaring the up the outer chambers of the heart to slow down the A-Fibb), last but not least he did a biopsy (not normally done but when I freaked that I had to have a surgery as I have a daughter that is ill and needs me to be there 100% for her and told the doc what was going on with her. He stated that there is a RARE condition that he does not think I have but a biopsy would tell him for sure that could explain my daughters condition). The new valve works fantastic, and the MAZE corrected my A-fibb and I do not seem to have the flutters anymore also, Life is good. Then the biopsy came back POSITIVE I have something called Amyloidosis. Talk about scary, when I read about it on the internet it just made me want to CRY! I got sent to Stanford as they have an Amyloidosis clinic. There the docs did many test and determined that the Amyloidosis called "AMY" for short is only in my heart THANK GOD! It is called Isolated Atrial Amyloidosis (IAA) Now for those that do not know what AMY is, the simple way I explain it is "an unwanted protein in the blood that deposits a cement like structure. Think of Alzheimer it is a form of AMY. My Amy is only with heart involvement, what does that mean? It means that I have "restrictive cardiomyopathy" which is rare in the United States. In this disease, the walls of the ventricles stiffen and lose their flexibility due to infiltration by abnormal tissue such as amyloid deposits. As a result, the heart cannot fill adequately with blood and eventually loses its ability to pump properly.
So the 1st year after my surgery I was doing great, had tons of energy, no a-fibb or flutter, no blackouts or feeling dizzy/faint and my BP is back to normal and off those meds. Life was back to good. Then comes 2012 and I am now in CHF signs again and the a-fibb is back. I am having a hard time walking up stairs or doing simple task. The doc decides to just put me on more diuretics to take the pressure off my heart but he does now say I actually have heart problems and it is not just my electrical. In November I go with a group of friends to Jamaica and started having severe problems feeling faint and dizzy. As I was going through customs it was all I could do not to blackout there (did not want to end up in their hospitals). I did make it home to the US with the help from the people I was with.
The beginning of 2013, 2 years after my 1st open heart surgery I can not take it anymore especially with what happened in Jamaica. I am tired of living the way I do and feel I am too young (51) to be like this so I tell the doc I want my heart fixed. He tells me you know that means another heart surgery (trying to talk me out of it). I tell him I do not care just want my life back. He sends me to a specialist and they do more test and another RHC. The doc is amazed with what he sees and tells me we need to do surgery immediately. My mitral valve was just trickling blood in, this is bad because our blood carries oxygen and we need that for our organs to survive. So March 2013 exactly 2 years and 1 month from my last surgery I had a 2nd one. This time I was getting a Mechanical Valve. When the surgery was done the doc said that my muscle had somehow swallowed up the repair from before and that was why it was only trickling blood, he said this is very rare to happen. He does not know how I was actually alive the last year. Well after surgery in recovery I kept bleeding, I received 9 liters of blood, the docs prepared my 4 children and loved ones with the fact that I may not make it as they took me in the operating room again that eve to reopen me to see why I was bleeding so much. They did not find any 1 reason so they just cleaned up in there and closed me back up. Thank God that worked and I am still here to this date.
It has been 7 years since my last surgery, my valve seems to be doing well but my heart is hardening/stiffening up from the AMY, they can not do an MRI on me so we do not know how bad it is only time will tell. If I get bad again the only thing left to do is a Heart Transplant. I really hope it never comes to that but if it does then the AMY will go away with the old heart. My a-fibb seems to be gone along with the flutters and the blood pressure is awesome 100/70 with my pulse set to 60 now. The only problem I have is when I exert myself I will feel like I can blackout again. The reason for that is when my pulse rises my heart is stiff and can not accommodate the extra blood flow so it backs up in my arteries, I then get exercise induced Pulmonary Hypertension but as soon as I stop doing whatever and my pulse goes back down and I am fine. Like I said at the beginning it is time to replace the battery this year on my pacemaker but that is the only surgery in sight for me.
For more information on Amyloidosis these are some great sites to go to:
https://www.mayoclinic.org/diseases-conditions/amyloidosis/symptoms-causes/syc-20353178
https://rarediseases.org/rare-diseases/amyloidosis/
https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/amyloidosis.html
