The Wins and Up-hill Journeys of 2023

Boy, it’s been an interesting year, to say the least. But then again: aren't they all?!

At the end of 2022, I lost my dad to several massive strokes (I spoke about this in my first blog this year - https://livingwithfh.blogspot.com/2023/01/new-year-new-thoughts.html) . Always the overachiever, he could not get just one stroke when his time came. Instead, he stroked in every area of his brain - small brain, large brain, and the brainstem, for safe measure ... All because of atherosclerosis due to untreated FH. 

This past year’s been largely spent in the shadow of this very, very dark and sad event that I started the year with. Dad was my beacon, the guiding force in my life ever since I can remember. Without him, I have felt like someone just turned the lights off all around me and I have been feeling around, trying to figure out how to live in complete darkness. 

It’s been a year of emotional un-health for all of us left behind, but mostly for my mom, for me and my sister. Our center, our patriarch is gone and the balance is off for all of us. The common belief in my family is that I am the strongest one. So, I have tried hard to be here for my sister and my mom. But even the stronger ones need strength - and I have surely felt this this year and have struggled to find it ... Trying to stay mentally afloat has been hard. Working and focusing on my physical health have been good distractors. 

My physical health has not been the best, but I’ll have to say it has not been the worst in my life, either. I am grateful for this. At the end of the day, truly, I am just simply grateful for another day. A friend of mine living with lupus has said to me something like this year “doesn’t every day feel like a victory?” - and she is so right. 

I am happy that I made it into the last days of 2023 - here I am adding another year to my life bouquet. Remember, I was told I was not going to make it past 18, or 25 at the most. Here I am stepping into my 49th year next year - God willing! This is definitely the best accomplishment of this year and of my life, really. The fact that I have stayed away from the ER and the ICU this year are the cherries on top! 

As I am looking back and drawing the line to close out this past year (something my dad always did), I think of all the good things, but also of the let-downs and disappointments about my health this year. Worth mentioning:

• We still have not figured out if my GI issues are caused by abdominal stenosis or not. We are still investigating this with both specialists (vascular surgeons as well as a GI doctor). More tests to come. I can only hope that it is not my stenosis because a whole year is a long lease if my gut is not getting proper blood-flow. 
• We have not figured out my dizzy spells. After years of my cardiologist refusing to do a scan of my brain blood vessels, I have finally gotten a vascular PA (that is right, not a surgeon, but a vascular PA working for a vascular surgeon) to admit that a head CT is past due for my advanced atherosclerosis, family history of strokes, and for my symptoms of dizziness that no other tests could elucidate. So, the test is coming up but it has not been done yet. I hope we find out something ...
• We have made almost no progress in improving my shortness of breath, despite achieving some good heart numbers this year ... (more about this below). I still get very winded when I do very light chores around the house. Climbing a hill or walking is much worse. 
• My cholesterol values started climbing, despite continuing the same combination of drugs that has been beneficial in the recent past. No idea why the numbers are up, but I wonder if stress has something to do with it ... The LDL cholesterol is 144 mg/dl (up from 101 mg/dl last December) and my total cholesterol is 210 mg/dl (up from 164 mg/dl last December). 
• Despite this trend, I am still waiting for my clinic to approve my Evkeeza treatment. Apparently, the insurance approved it but the bureaucracy of the clinic itself of trying to figure out how to manage a new drug as an infusion is crazy complicated. So, we are still waiting. We're also waiting for my liver to get better.
• To that point: my liver enzymes are also climbing. They have gotten as far as more than three times the upper limit (the AST and ALT values). They tell me they need to be much, much higher than these numbers for liver failure, but they are still concerned and trying to figure out what’s happening.
  
  We have been trying for months to stabilize them and they remain high as long as I am on a statin drug. I am planning to come back with a more detailed post about this, but I am currently “playing with drugs”, as I call it - trying to turn meds on and off and see what will keep the liver levels down and get the most benefit for lowering the cholesterol too. So, until I have the full picture and until we pin down what’s causing this and what drugs I will be on going forward, I will keep the confusion to myself for now.
  
  Right now, they have stopped the one statin that works best for me (Atorvastatin) and they are asking me to take a lower strength of a less potent statin (Pravastatin) to see what the liver is doing next. When I take no statin at all, the enzymes are normal.
  
  We have not checked the cholesterol levels during this trial-and-error period, but logic and experience tells me the levels are high, even higher than the ones listed above. The levels I wrote above were trending higher when I was on the full-strength of all four medications I normally took for cholesterol (Lipitor, Zetia, Praluent, and Nexletol).  Of course, the longer I go with higher cholesterol levels the worse it is for my vascular and heart health. But I’d rather not add liver damage to my laundry list of problems, so I am willing to try this new experiment to revamp the drug cocktail I am on.
  
  I wondered if stress has something to do with the liver functions too, till I saw the liver enzymes come to the middle of the normal range only by stopping the Atorvastatin for 10 days! 

Despite all these setbacks I have had some brighter spots this year too: 

• I finally made some progress on Evkeeza with my cardiologist. The jury is, of course, still out on this newer than new drug, but I am hopeful it will do something (good) to my numbers!
• Earlier this year I barely got the doctor to pursue setting me up with this (https://livingwithfh.blogspot.com/2023/08/access-to-newer-treatment-might-not-be.html), and now I am waiting for my liver numbers to stabilize before we introduce the new drug, but the clinic is finally ready to start. 
• I have managed for another year to keep my carotid stenosis at a lower than 50% amount of plaque build-up. After having had it as high as 65% in the past, having the readings come in as 50% or lower than 50% in some areas is an accomplishment. (The theory here is that Praluent has helped with stabilizing and stopping the progress of the plaque - just a theory from doctors). 
• I had a xanthoma successfully removed from under my eye (https://livingwithfh.blogspot.com/2023/12/xanthoma-removal-surgery.html) . This is the fourth fat deposit (most likely caused by high cholesterol) that I have had to remove in 48 years. 
• I have finally (after two years of trying different treatments) pinned down the right drug and the right dosage for my blood pressure and I have improved my BNP number (which shows the severity of your heart failure).
  
  What is even better than seeing lower numbers of the BNP (still not normal but lower), is that I feel better, especially when I walk. In normal weather, my angina and the cramps in my extremities are much less than they were before I started Amlodipine. My BNP has come down from 692 pg/mL (end of last year) to 323 pg/mL this past September (the normal values should be under 190 pg/mL). In weather that is too hot (above 80 F or too cold (low 40’s or lower), the angina and cramps are still pretty bad ... And the shortness of breath has not improved. The blood pressure numbers are more often “normal” with only weekly spikes (as opposed to daily). I take the little bit of good news where I can. 

All is not perfect. But all is not bad ... One foot in front of the other and I hope I can live another year to tell that year’s tale going forward. 

From all the good that’s in my life, I am most grateful that despite all the (un)health bumps,  I enjoy a full life, with good people, good food, and lots of travel and freedom to move about. Yes, being tied to a pill box, a blood pressure taker, a bi-weekly injection pen, an INR meter is not a completely care-free life, but I am never taking the freedom to move my body and drive and jump on a plane for granted. They are precious gifts! 

Going to New Orleans for Thanksgiving was one of the highlights of this year - here is me, in the French Quarter on Thanksgiving night: 

Other adventures included visiting three very different national parks for the first time (Saguaro National Park in Arizona, Indiana Dunes in Indiana and Jean Lafitte in Louisiana), two Frank Lloyd Wright homes (Taliesin West in Arizona and the Westcott House in Ohio), going to Europe and celebrating mom’s 70th birthday, spending a week with my nephews in the summer and taking them to their first baseball game and their first musical (Wicked), among other fun things. So looking forward to more adventures!  

I wish each and every one of you reading here much health and strength. They are so important to keep us going. Whatever makes you happy or makes you whole - hold on to that and you’ll stay afloat! 

Happy New Year! 

Xanthoma Removal Surgery

A little behind on this post: at the end of October, I had a xanthoma (or it could have been a lipoma, or a fat deposit - the jury is still out on what exactly they wanted to call it) removed from under my left eye. This was the fourth fat deposit that I have had removed or treated in my 48 years. They are pesky things that show up on various parts of your body if you live with high cholesterol. They show up small, in the beginning, and if left alone they grow sometimes to a painful or an esthetically displeasing size. 

I had one removed from the tendon in my right elbow when I was in my early 20’s, a xanthoma removed from my eye lid in my mid-20’s, a large (bigger than a golf-ball) fat deposit removed from my neck a couple of years ago, and now this last deposit from under my left eye. The surgeon called it “a mobile orbital mass, firmer but consistent with orbital fat” in his post-op notes. Regardless of what you call it, the surgery to remove it might be similar to what happened here.

I am usually nervous about any procedure that puts me under general anesthesia because I just never know how my heart will behave during the drug-induced “nap”. This time, it didn’t help that the anesthesiologist did not agree with the surgeon on what type of anesthesia I should be under: the anesthesiologist wanted a light anesthesia, where I am coherent and talking back but not remember much and the surgeon wanted me “completely out”. The surgeon won, eventually, and I agreed with him (general anesthesia is usually my least favorite kind), considering how he went in to get this fat deposit: he had to make an incision behind my cheek’s skin, in my internal lower lid. He recommended general anesthesia so I would not move during the procedure and cause any other complications. And I agreed, although I am sure I didn’t have much choice if I wanted the surgery. 

I had a black eye for a couple of weeks and I still feel some firmness in that area, possibly from the scar tissue, but the visible bump is gone from my face, so I consider this a success. The deposit he pulled out was small compared to the ones I have had removed in the past - the one I had removed from my eye lid of the same eye when I was younger was probably the smallest. This year’s “fat mass” was actually made up of two pieces measuring 0.8 x 0.7 x 0.4 cm and 1 x 0.5 x 0.4 cm, so anywhere between half and one full centimeter each. 

I originally went to a dermatologist for this growth but he would not touch it because it was too close to my eye, so he sent me to an ophthalmologist. I tried to make an appointment directly with an oculoplastic surgeon, but the clinic would not have it. They said I need to see a regular ophthalmologist first and let them decide if I need a surgeon. I told them it’s useless and pleaded to please let me skip a useless step and make the appointment (in the same clinic) with the right specialist, but they would not budge! 

So, I went to a regular ophthalmologist who decided my vision was just fine and who ended up referring me to their coworker who is an oculoplastics surgeon who finally performed the procedure. I tell you, the mystery and the maze of the medical world never ceases to surprise and bewilder me ... 

Here are the before and after pictures and if you can’t really tell where the bump was (under my left eye in the picture to the left) you’re not alone. It was not very visible but it was palpable and I knew it would only grow bigger, having had the experience of at least two other ones that got enormous and prevented function in either my elbow or my neck. To prevent scarring too badly when it ended up being much larger, I decided to take care of it now. The surgeon agreed.

In the first picture, you can see it under my eye, closer to my nose. Click the picture for a larger view.

Just another procedure that us, FH folks, might have to tackle. 

Much health, all!

The Loss of the Blood Sample

This will be a short post just for a bit of comic relief, I hope. 

Well, it finally happened to me too! 

I have been at this for a really long time. 40 years to be exact, this year! I should have had a party or something but I didn't. 

I have felt like a pin cushion with many doctors repeating the blood tests because they could not believe my numbers were real. I have felt like a Guinea pig trying to figure out what drugs and what diets might work for me, often with little or no results, often disappointed, especially in the beginning. Often feeling empty, because if all the science in the world fails you, what hope is there?!  

But I always manage to fight back with what I can. I always think that knowledge is power and even if I don't have all the answers, and even if there are no drugs to cure me and magically prolong my life, I at least know what I have to do: I need to know my body and my symptoms better than anyone, I need to always advocate and insist on getting tested every year to learn and know the progress of my atherosclerosis, before this disease throws me into some sort of "event" from which I may not come back ... There are ways to stay on top of this, even with bad numbers and no treatment at play ... 

I have fought with insurance companies, and bureaucrats who think they know better than medical professionals what is best for me, even when they had no medical code for my disease. 

Of course, every failure of a drug working or every encounter with a doctor who does not know their stuff and refuses to learn me as the patient with a unique history that I am, every battle lost and appealed with the insurance company is frustrating and sometimes even maddening. 

I have been embarrassed in the hospital when recovering from open-heart surgery when I could not so much as walk to the bathroom by myself and I did everything in bed ... Oh, I have many stories ... I know this disease is not for the faint of heart - and no pun is intended. 

But till this week, I have not had this happen to me ever before. I have heard of people going through this, but never me. 

They lost my blood sample! 

As every FH patient knows, the only way we know what our numbers are and what our livers are doing if we choose to be on some of the treatments that cause liver damage is with a "simple blood test" - the literature will tell you. Well, except it turns out that these blood tests are not ... so simple, after all. 

I am going through a bit of another Guinea-pig moment (for the umpteenth time in my life!)  where both my cardiologist and my PCP are playing with my drugs and every two weeks I have to go in for blood tests to see how they influence my liver numbers (which are high lately, but we're not sure why yet). So, every two weeks, I go and have a blood sample drawn to see where my liver numbers are after they have switched (added or removed) another drug in my cocktail. 

This has been my routine for the past two months (I have one more blood test later this month and then we make a decision in January about what drugs I will be on). So, this week, I went to my bi-weekly blood draw and the "regular" blood test results failed to post in MyChart the following day (which they have always done for the past 6 years since I have been seeing these doctors). 

I knew something was wrong from the minute I left the office when they drew the sample, because the phlebotomist printed the wrong labels first - they had my name on them but the wrong tests. I thought it was an easy mistake because I have several standing orders from two doctors in the computer and they are very similar. But then, she reprinted the labels and never asked me to check the information on it, like they normally do (name and date of birth). At the end, she had the label at the bottom of a tray with a bunch of throw-away junk. They usually stick the label on the bottle before I even leave to ensure I see this being done and to feel confident that the sample truly is submitted. But not this time. This time, she shooed me out the door with "have a nice day, ma'am", my sample sitting in a tray full of garbage and no label on it either ...  

I called three days later to inquire where my blood test results were. After I had to put up with abuse of the sassy and not-so-helpful receptionist who ensured me sometimes it takes longer than 72 hours for blood tests to be processed (and after I ensured her that not for this blood test!), she begrudgingly took my complaint and number down and ensured me "someone from leadership" will call me back with an answer. 

The nurse (I guess the nurses are now "leadership" too? Good for them. They deserve it!) called me to apologize and tell me they have a record of me coming in and giving the sample but the lab has no record of my blood sample ever being received. 

"We are so sorry, ma'am. Can you please come back now and give us another sample? Do you mind?"

Of course I mind but I have no other choice. So, I dropped everything at work and drove myself back to the clinic and gave another sample in my bruised right arm (the only one with the good vein). An hour and a half later, they had a fresh sample which I was hoping had better odds of reaching the lab than the first one. 

So, here I am, inaugurating another bump in the road of a perpetual patient: the loss of the blood sample. Hurray! 

I won't ask "what's next?" because I know I am asking for trouble!