Just a big ol' bag o' blood...

Today was my 2nd phlebotomy treatment. Good news: no nausea this time. Bad news: my veins apparently really hate being poked and deliberately retreat when the needle comes after 'em.

 

After getting settled, my nurse (a different one from last time) got all the tools of her trade together and started to examine my arms. She was incredulous at how my veins did not pop out. "For such a big guy, your veins do not easily show up!" Right. Isn't that the way its supposed to be? Anyway, in addition to having veins that seemingly like to play hide and seek, they also are apparently quite small compared to others. After finally finding my vein, my nurse opted to use a smaller gauge needle, fearing the standard size that comes with the phlebotomy kit was too large (thank you!). Tied off, cleaned spot, stuck in needle. Nothing. Pull needle out a little, move around a little. Nothing. Repeat. Nothing. After trying for a few minutes, the decision was made to wrap my arm in a warm blanket to coerce the frightened little bastard closer to the surface.

 

45 minutes later...

 

Tied off, cleaned spot, insert needle into a new spot, slightly higher than the first attempt. Nothing. Pull needle out, move it around a little. Nothing. Pull needle out, move it around a little. Oh, look at that, blood. Push needle in deeper (yes, deeper) and line up, attach secondary tubing for draining, tape lines to hairy arms (this will be fun later). Squeeze ball - ironically shaped like a small heart - periodically to keep the flow moving. Chill out and read another couple of chapters of Anne Rice's latest addition to The Vampire Chronicles, Prince Lestat (which is quite good and a book I highly recommend). Post sarcastically humorous FaceBook status asking if it is weird I am reading Prince Lestat  while being drained of blood. Take picture of my arm all tubed and taped just to mess with my wife (and anyone else who might be queasy at the thought of blood and having it drained). Sip on an ice-cold Coke at the insistence of my nurse. Hang out and watch my blood drain into a plastic bag for the next 30 to 40 minutes. 

 

All in all, a better experience than the last. And this time I filled that puppy to the brim (see above, left; that is my actual bag o' blood...). Next treatment is scheduled but Christmas came early in the fact that, due to the holiday, I do not have to go back until the 29th. Yay! I am curious, though, to find out how much lower my ferritin levels will get to after this 2nd treatment. Woo hoo!

Progress

Did a regular series of blood labs yesterday, just to find out where all my levels are at after my first phlebotomy treatment. My hematologist had noted that the first noticeable change in my ferritin levels could have been something of a fluke (between October and early December, my levels dropped from 1295ng/mL to 1233ng/mL, a change of 62ng/mL without phlebotomy and only really just starting to make changes to my diet after my initial diagnosis), so she wanted to get some new baselines. I am assuming I will have to do this each week prior to each treatment occurrence, in order to track progress.

 

This morning I signed onto my laptop and saw that a new test result was available. My ferritin levels had dropped again, this time from 1233ng/mL to 1124ng/mL, a drop of 109ng/mL! I do not think these particular results are a fluke. The standard drop, after phlebotomy, is usually 70ng/mL. Moderation in diet, changing what and how I eat, plus phlebotomy has already started to provide positive changes.

 

Have my second treatment later this morning and, as much as I hate being poked as often as I have and will continue to be, I am stoked to see such a significant drop occur and am hopeful to see more dropping in the levels. Almost makes having been suffering through a cold-from-Hell for about a month now - that has also contributed to ear pressure on such a level that I have now had to do two course of Prednisone (an oral steroid) to clear them - sort of worth it. Or not. Trying to find the silver lining there and it is sort of difficult...

 

Now to eat a filling breakfast and drink lots of tea & water in preparation for today's bloodletting...so I don't pass out or otherwise feel sick like a big wuss as people are getting their chemotherapy all around me...

1st Phlebotomy

Its not like I was actually looking forward to it or anything, but yesterday's phlebotomy treatment could have, well, gone better.

 

Let me start by saying I know how lucky I am. There are others going through a hell of a lot worse than I am, so I really do not have much place complaining. I am mainly relating this from a more humorous level if anything.

 

The day started with a sense of foreboding, coming as it did in conjunction with "Stormpocalypse 2014". Amy ditched out on joining me (to be fair, she was dealing with a nasty cold/cough and neither of us felt it was a great idea to have her in the presence of people with already compromised immune systems, getting their chemotherapy, etc.). So I drove myself from Tracy to Modesto. Wind and rain dissipated a bit as a neared my destination, which was good (I hate driving in inclement weather...). Checked in and took a seat with my Kindle, waiting my turn. I have to say that it was a sobering 1st view, if only because I had never experienced it previously, seeing where those getting their meds, etc., basically all hang together. My boss - herself a cancer survivor - had given me details, but nothing really prepares you. Any anxiousness or fear I may have felt at this stage was actually dispelled knowing these people were so much braver, so much stronger, and dealing with so much more than I am.

 

For my condition, the treatment is currently scheduled to be weekly blood draws, about a unit each time. Several days in advance of each draw, I have to also give blood for lab work to track/monitor my ferritin (and other) levels. Good thing I am pretty ok with needles, right?

 

Assigned a seat, get comfortable, warm blanket wrapped around my arm to get things flowing. Clean the spot, insert needle. Pretty much ok at this point. Tube inserted, bag set-up, blood flowing, however slowly, as I pump the ball she gives me to squeeze to keep the blood itself pumping. Wave of nausea hits me so hard I actually break out in a total body sweat. Not even during deep-sea fishing trips have I experienced this type of nausea. I think, "Great. I have this damn tube in my arm, blood is leaving my body, and I have to ralph. This is not gonna be pretty by any degree..." I sit back, close my eyes, focus on the cool air coming down from the overhead vent and the wave begins to dissipate. Now, keep in mind that I am not generally a squeamish person when it comes to blood; it has never really bothered me. Maybe it comes from hunting and fishing in my youth, maybe I am just a sick bastard, but the sight of it - whether it is someone else's or my own - doesn't affect me as it might others. The only thing I can imagine is that I simply did not eat or drink enough before arriving. I thought I had, but I obviously didn't. Check that off on good things to note for next time.

 

And this is where, I fully admit, to feeling some shame, remembering where I am, what others around me have been and are going through. There is an older gentleman in my little "area", the 2nd chair, lounged out, watching TV, obviously getting fluids, chemo, something...but definitely going through far more than I am. I saw a woman who did not appear to be much older than myself, getting her chemo, as well as a number of other women, older, doing the same, knowing their routine, knowing the nurses, fairly casual, fairly relaxed. I do not feel pity for them; I feel admiration. And I also feel like a wuss. I am only having a unit of blood removed per week, for now. Suck it up, buttercup.

 

I relax and drink my apple juice. At some point, my nurse comes over and comments how the blood has stopped moving. Not slowed, but stopped. I look at the mainline and, sure enough, despite my still squeezing that little ball, no blood. With that, my nurse starts to move the needle around in my arm. What the fuck did I ever do to you? Let me tell ya something, Nurse Mengele, that does not feel too good. Needle is definitely in the vein, so let's swap out the mainline. Nope, still not going. Looks like the vein collapsed. As it turns out, I was able to fill 90% of the bag; they can either a) poke me again to see if we can get more or b) I can come back next week. Yeah...next week is good.

 

Wrapped up, scheduled next treatment...and walked out into a deluge of Biblical proportions outside. Drive back to Tracy basically underwater. That was fun.

 

Overall, not really too bad. Just something that will take some getting used to as it is going to be the norm for the next few years, at least. Again, I honestly cannot and shouldn't complain. Its all manageable.

Results

Last week was pretty busy, what with a liver biopsy on Monday and then an echocardiogram on Thursday as well as some more blood labs drawn. For me, the stress of actually waiting for results is sometimes worse than the anticipation of the procedure itself.

 

I could probably come up with some pithy way to reveal my results here, but I think I like my doctor's emails better:

 

From my gastroenterologist -

"The biopsy shows very mild inflammation and some iron deposits in the liver- on a scale of 0-4, the iron deposits are 2- halfway there. It should be 0. So, minimal damage to your liver, all good, the iron deposits will improve once you have had iron removed by phlebotomy, although it may take 2-3 years. No need for a follow up liver biopsy but monitor blood tests every 6-12 months. Please let me know if you have any other questions."

 

This relieved me immensely. I have read so many stories from people with this condition who find out they have various levels of cirrhosis, even liver cancer. Hemochromatosis seems to be such an individualized condition, varying from person to person afflicted with it that there does not seem to be very much rhyme or reason as to whom gets hit with what at what point in their life. I am thankful that it appears to have been revealed early enough that treatment can be so effective.

 

From my hematologist -

"Good news. The echocardiogram of your heart is normal. (I sent a copy of the report by mail last week). Regarding your ferritin level, it is very minimally decreased (not sure if it's a true decrease or just lab variation), and it is still very high, so I strongly recommend to proceed with phlebotomy at this time."

 

So, at this point, I feel I can breathe easier. I have been steadily making changes in my diet (still have to work on that whole walking thing, which is really dumb as I have a treadmill in my garage...). Amy has really been doing an amazing job trying to become more aware. Sometimes I think I am annoying her by telling her about different sites and different foods, menus, info, etc.

 

I have to admit, however, that the more research I do, the more I am coming to the conclusion that no one has the final answers as to what one should or should not do; its up to the individual. Some things are obvious, some are not. Some people express opinion to the extreme while others take a more "calm" approach. Moderation has to be the key. I have already seriously cut down my red meat intake, changing hamburger to turkey burger for example (hamburger was perhaps where most of my red meat came from, with steaks being more of an every now and then sort of thing). I am having a serious dilemma over sushi, though. From what I understand, the "ban" on sushi stems from the bacterium they may foster, not the iron. But here is how I look at it: I have eaten sushi and raw shellfish (i.e., oysters and, more recently, lobster sushi) for upwards of 30 years now. Still alive. Yet some opinions I have come across say that people with my condition shouldn't even handle raw seafood, and even one commented about not being able to even walk on the beach. That seemed rather alarmist, to me. You see what I am up against here, right? I am wondering if there are any others who have my condition as well reading this blog who would like to comment with their own experiences, opinions, thoughts on this. Tell me what seems to work for you. I am trying to keep an open mind in order to make the changes that seem to make the most sense, but it does seem that a lot of the "information" I am coming across is really more "opinion" than anything else. Let me know.

A little research...

While I plan to consult with my doctor(s) on what I find online, I am finding I like this particular site dedicated to hemochromatosis and treating it, http://www.hemochromatosishelp.com/. It is done by a holistic doctor who himself has hemochromatosis and does a great job of explaining the disease as well as examining diet and options of what one can do to actually reduce iron levels in conjunction with blood letting. One page I particularly like is the one dedicated to diet, which is, of course, perhaps the biggest life change one with this diagnosis must accept.

A good quote that I have in the back of my head, almost like a mantra, is: "Nourishing our bodies and enjoying our food are just some of the many reasons why a hemochromatosis diet should not be too restrictive." Word. I have come to the conclusion that, while there are some very obvious foods to eliminate and/or cut (i.e., red meat), one needs to focus more on what one can have, as opposed to what one cannot.

I think, for myself, the biggest hit to my diet is the suggested elimination of sushi and raw shellfish. I love sushi, have been eating sushi for over 30 years, love oysters, LOVED the lobster sushi Amy and I enjoyed during a dinner out with our friends Cyndie and Andy during a trip to Vegas. So the big question is: If eating sushi and raw shellfish has not affected me detrimentally in my life so far, why should I completely eliminate it? Here's the danger: There is a particular bacterium called Vibrio vulnificus (http://en.wikipedia.org/wiki/Vibrio_vulnificus) that lives in the ocean and can be present in seafood. Cooking seafood eliminates it, but I have read that some with my condition believe we shouldn't even be handling seafood as it can be absorbed through the skin. I think this is a wee bit alarmist. This bacterium can react explosively in people with hemochromatosis and result in death. But hey, what's life without a little excitement, right? What I am getting at is that each person with my condition exhibits their symptoms individually, meaning not all are the same or on the same level of the disease. Information, testing, and I think a common-sense approach to your life changes is key.

In the meantime, I am already pretty fucking tired of being a pin cushion this week, between the liver biopsy, the shots associated with relieving the pain of that procedure, the blood labs, the IVs...yeah, pretty tired. And I still get to look forward to my first blood letting next week. Woo hoo!

Meh.

Liver Biopsy - The Aftermath

More or less. From what I have been told, it will still take about a week or so to get any results from pathology. But the thing that had been a more immediate concern, i.e., the process involved with doing the biopsy, is done and over. And, quite honestly, I am now not sure what the hell I was so stressed about.

Ok, that's not entirely true. Having a needle that has to be long enough to reach whatever organ is being examined stuck into you is never going to be a very appetizing thought. I have tattoos, have had multiple piercings...but that doesn't mean I want those needles going any deeper, know what I mean?

I was surprised yesterday to learn that the procedure I had viewed on YouTube (and posted videos of in my previous post) has actually been modified at some hospitals. As explained to me by my attending nurse, instead of making an incision on the right side and inserted a fairly large needle (which is hollow to allow smaller needles to be used in collecting biopsy samples), they use a CT scan to find the exact placement of my liver had me follow some basic breathing exercises in order to pinpoint how the liver moves and the best place to insert the needle. Before all of that, I was given an IV with a mild sedative to relax me (which was awesome, by the way...), but not enough to put me out. After determining exactly the best spot to go in, the surface of the skin (and slightly deeper) was numbed with shots of Lidocaine. The biopsy needle was positioned and inserted. As expected from the overview the nurse gave me, I did indeed feel a bit of a push when it went into my liver. Four samples were taken and then we were done. Had to hang around for about three hours of observation and blood pressure monitoring, but then Amy and I were able to head out and go to lunch. Soreness started kicking in a bit in the afternoon and I crashed for a bit, the adrenalin rush that accompanied my anxiety having been depleted. Still a little sore today, but not horribly so. At least now I know what is involved in the event I have to do it again which, lets face it, is highly likely given my condition.

Now it is a waiting game for the results. This biopsy was mainly to determine if any cirrhosis is present in the liver, a condition brought on by the hemochromatosis. I am hopeful as my gastroenterologist told me that no growths, spots, or sign of liver cancer were indicated in my ultrasound or MRI, nor any early indicators of cancer shown in the plethora of blood work I have already done. Next Thursday I have my first phlebotomy treatment. I think I will try to record it and hyperlapse the whole thing to post...

Liver Biopsy Procedure

So...I couldn't help myself. I had to look up videos of the procedure. Yeah....still doesn't seem fun and I would much rather be knocked the fuck out completely. Here are two videos, one actual video and the other an animation for information. For the first video be advised that there is a small amount of actual blood/bleeding shown.



 

This is what I get to do tomorrow around 9:30am. I have to admit it is stressing me out a bit...but it has to be done. Sort of like flying; I hate flying, but it sometimes needs to be done. Actually, it isn't flying I am afraid of. Its that whole falling out of the sky and becoming a charcoal briquette on the ground thing that makes me a bit anxious. Same analogy can be applied here.

 

Sigh. With any luck, I will get some great after-procedure pain meds...

You can't get something for nothing...

The Information Age is truly a wondrous era. Last night I was doing some more research, visiting various sites with info about hemochromatosis, trying to determine what might be the best changes to make in my diet...all from my iPhone while watching Pierce Brosnan in "The November Man" (not a bad film, actually...). If there is one thing I am certain of with this disease, it is that confusion and contradiction surrounds it. Of easily half a dozen sites I checked out, one half contradicted the other with respect to their dietary recommendations, and multiple contradicted what my gastroenterologist told me directly.

 

Red meat is a no-brainer here. It sucks, hardcore, for me to have to give it up, being a life-long affirmed carnivore. I used to go into restaurants and, if ordering a steak (typically a nice, juicy rib eye), my standard response to my server was, "As rare as you can legally get it!" when asked how I liked my steak done. I am slowly weaning myself, with this most recent Thanksgiving being one of my "last hurrahs", of sorts, preparing a prime rib instead of the traditional turkey (this had been planned well before my diagnosis and I could not bring myself to change the menu...). Beer was something of a shocker, and equally a bummer as I not only enjoy drinking beer but also brewing it when I am able. Breads, pastas...ok. I had actually been working at curtailing my consumption of those (although having difficulty with pasta...) already. The few veggies I actually enjoy? Yeah...on the list of iron-rich foods. Funny how my gastroenterologist said pork and poultry were good...but several sites disagree.

 

There are various blogs, as well, started for the same reasons I have started mine...each seeming to disagree with what is best to eat and what isn't. Shocker.

 

I think the key here, as I begin all of this, is moderation. I am not always that great with moderation, as many who know me well can attest. My liver biopsy is looming closer and closer, something I am still not thrilled about, but the results of which will probably have a greater influence on my dietary changes than anything else. My first phlebotomy treatment is already scheduled and I am actually curious as to how that will help bring my ferritin levels down. Some comments I have seen from others with my condition on other sites actually detail how they do not really change that much with respect to their diets (other than cutting down on the red meat...dammit...) as the treatments have proven to be quite effective at lowering their levels regardless. In the meantime, I have found something of a consensus noting that black tea or milk at meal times helps as well, so I am exploring that.

 

The need to seriously cut certain foods out of my diet sucks. I would rather focus on what I can actually eat, rather than what I can't. Its going to take some focus and commitment to change, but then you can't get something for nothing, now can you?

 

 

Welcome to my new journey...

Hey there.

On Tuesday, November 20th 2014, at approximately 10:45am, I was diagnosed with hereditary hemochromatosis. This diagnosis came after months of blood tests, an ultrasound, more blood tests, an MRI, more blood tests...you get the point. So, what exactly is hereditary hemochromatosis?

Let's start where I started, looking terms up on-line, shall we? Per Wikipedia:

"Haemochromatosis (or hemochromatosis) type 1[1] (also HFE hereditary haemochromatosis[2] or HFE-related hereditary haemochromatosis[3]) is a hereditary disease characterized by excessive intestinal absorption of dietary iron resulting in a pathological increase in total body iron stores.[4] Humans, like most animals, have no means to excrete excess iron.[5] Excess iron accumulates in tissues and organs disrupting their normal function. The most susceptible organs include the liver, adrenal glands, heart, skin, gonads, joints, and the pancreas; patients can present with cirrhosis, polyarthropathy, adrenal insufficiency, heart failure or diabetes.[6] The hereditary form of the disease is most common among those of Northern European ancestry, in particular those of Celtic descent.[7] The disease is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations.[8] Most often, the parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but do not show signs and symptoms of the condition.[8]"

Whoa, right?

The biggest clue to having this condition is one's ferritin levels. To give you some form of reference, a male my age should have an average range of ferritin in the 22ng/ml to 365ng/ml; mine were noted to be 1295ng/ml. Pretty damn high. After a genetic blood test, it was confirmed that I was positive "...for two mild mutations (homozygous for H63D) within the HFE gene. This individual is homozygous for the mild HFE mutation. The risk for clinical signs/symptoms of hereditary hemochromatosis in the absence of other risk factors (e.g., hepatitis, alcoholism, thalassemia, etc.) is extremely low."

Well, that's good news. I guess. This indicates that both my mother and biological father each carried a mutated gene...and passed the clinical version of the condition on to me. And that's just rather annoying but at least I have something solid to blame on my parents.

Its a genetic condition, so it ain't going anywhere. Ever. However, it can be treated. How you may ask? Through a fun thing called phlebotomy. Basically, I will have to go in and have a unit of my blood removed weekly for about three to four months. Once lab tests show my ferritin levels are down to 50ng/ml, those treatments will be dropped to once every couple of weeks, then once a month, then, perhaps, bi-monthly. This will go on for the rest of my life. My first treatment is scheduled for Thursday, December 11th. Woo fucking hoo.

Right now I have two big concerns. One is that I will have to have a liver biopsy. This does not make me happy, but it is really the best way to gauge what damage, if any, has already been done to my liver. Mine has been scheduled to occur on Monday, December 1st 2014. I am really not looking forward to it at all.

My second concern is not really anything that I can much about and that is the knowledge that I have passed the carrier version of this gene mutation on to my kids. We will have to monitor them and their diets carefully from now on. it isn't like we were feeding them crap before, but we now have to be hyper aware of what they eat.

So that is where I am at in these first few weeks after being diagnosed. I know this condition is manageable and that there are many others who may have better or worse aspects of it than myself, whom are dealing with it proactively via treatments, who have been there and done that when it comes to what I am about to. That doesn't stop it from being scary or frustrating.

I am doing this blog mainly for myself, to log the different things I go through, the treatments, the feelings, as well as information I come across as I study everything I can about it. To that end, I encourage anyone else who has this condition to please feel free to leave comments, suggestions, their own stories, etc. Perhaps by doing so we can not only educate each other and share experiences, but also help everyone else to better understand this disease, one that seems to fly under the radar completely but really should be given more consideration.