impending doctor marathon.

I can’t seem to find the energy to write, I just keep staring at a blank screen mostly wishing I could sleep at night.  I feel like Christmas is creeping up much too fast and I’m not getting to enjoy the season.  I’m even still decorating.  Every since we have moved it feels like the days fly by and I’m not sure why.  Time moves faster 50 minutes south I guess.

We finally got word on Ki’s blood tests from the Monday after his last hospital stay.  The cortisone seems to be working very well and is upping and maintaining his sodium levels.  This is both good and bad.  Good for obvious reasons but the bittersweetness comes from the fact that when the sodium meld scores kick in in January he may not get as many additional points as he would have previously.  It is my understanding that he should still get some as he’s still under the normal range but I’m not entirely sure how it all works.  We are going to have to ask the transplant hepatologist. I’m just ready for him to get offered a liver.  The sodium being added to the meld gave me some hope of an end in sight… Will have to wait and see what the dr has to say about it all.

Speaking of that, next Monday.  I hate driving down there but it is a much easier drive now that we do live 50 minutes further south.  It will be a long day though… Both transplant hepatologist and TIPS check.  So blood work, dr office, ultrasound, dr office.  It is very wearing emotionally more than anything.  I hope we will both have enough energy to do something afterwards to make it feel like we weren’t there just for medical stuff… There’s a florist next to trader joes that decorated for Christmas I hope we can stop by.  And maybe if we’re both still in a good mood I’ll drive alllll the way to the other end of 86th street and go to the Asian and Indian grocery.  

best intentions.

Today is the last day of November and I had every intention in the world to make a very informative infographic for Alpha 1 Antitrypsin Deficiency due to the fact that November has been designated an awareness month for it (November seems to be a catch all month for a lot of random disease awareness.)  I think when Ki was diagnosed I was too tired and overwhelmed to even bother explaining to people what it was… I was just kind of like “well if you care, look it up.”  I mean I explained it to our immediate family and friends but as far as things like Facebook and stuff… yeah, just too exhausted.

When Alpha 1 progresses to liver disease it is kind of hard to even talk about the Alpha 1 part of it because liver disease is just shit.  One of the nastiest ruthless diseases out there.  Cuz your liver does every fucking thing.  And most of us spend our entire lives taking it completely for granted.  Alpha 1 liver disease is not any different than liver disease caused by anything else, even Alpha docs will tell you, once it’s liver disease there isn’t really anything we can do… just look out for your lungs in the meantime.

Liver disease happens when the liver gets scarred up and unable to function.  This is called cirrhosis and I hate that fucking word more than any other word in the world.  It even sounds every bit as ugly as it is.  And it’s mostly only talked about in terms of alcoholism.  But there are many many reasons for cirrhosis, it is just until you experience one of them intimately you just never really learn that much about liver disease and its causes.  In the case of Alpha 1, cirrhosis is caused because the liver makes the antitrypsin protein that your body needs to protect your lungs, however because of a genetic defect the protein is misfolded and cannot escape the liver, there it builds up causing the tissue to scar.  That is what cirrhosis is, your liver slowly turning into just a mass of scar tissue.  It really makes the liver seem like a dumb organ doesn’t it… it has an amazing ability to regenerate and yet it scars itself up as a mode of protection.  There’s also a version of Alpha 1 where the genetic defect causes the liver to make no version of the antitrypsin protein… in this case these people will never be at risk of Alpha 1 liver disease but since they don’t even have the slightest bit of protein to protect their lungs, they are pretty much guaranteed lung disease.

As far as the lung aspect goes I thank God every day we do not have to deal with that.  His lungs have done good so far and don’t show signs of damage.  Once he gets a new liver he will essentially be cured of Alpha 1 since the new one will make antitrypsin correctly.  His uncle suffers from lung disease caused by Alpha 1 and despite the prolastin infusion which is the only current pharmaceutical approved for Alpha 1 (In the US… I know some of our European alphas are not so lucky and have zero approved treatments), his disease continues to progress… it can help but unfortunately it doesn’t slow the progression of the disease for every body and can lead for the need for a double lung transplant.

So one little genetic defect causes all this.  And it’s estimated 25% of Americans are carriers. The rate can be much higher in some European countries where it is believed the disease originated from our ancestors.  It is also believed to be one of the most under-diagnosed genetic defects in medicine.  It is the number one reason for pediatric liver transplants.  Usually the liver issues show up in babies and young children, although most go on to recover function and don’t have anymore issues… but then it goes to show up again in adults, and it is believed that many alphas are liver affected at the time of death (assuming they live otherwise normal lifespans) but because our livers are so amazing, most people don’t get the point of having cirrhosis.  It’s also believed to be a major cause of liver cancer.  Gee, what doesn’t this disease do eh?

It’s a shit disease.  And I’m not even speaking on the lung affected population who struggle with COPD because of this shit, which is another awful hellacious disease.

Someday, when my husband is on the road to recovery because I know with all my heart he will beat this shit, I want to go out and talk to people about this disease, I want to spread knowledge about it, I want to help others suffering with it because no one should have to go through this alone.  It’s awful and scary and frustrating and still so misunderstood.

weird feelings

I stalk the Organ Procurement and Transplantation Network several times week.  It contains all the data on transplants including center data about the waiting list, how many are added, fall off the list, and transplants.  It is getting to be what I call “transplant season”… the time of year that the amount of transplants taking place goes up dramatically.  We have learned from our support forums that during the holiday season there are more deaths leading to more organs leading to more transplants. It’s kind of morbid feeling to feel positive about a thing such as that, to think of a family losing their loved ones especially this time of year is heartbreaking, but at the same time… if these deaths were going to occur any way, at least they can give the gift of life to someone else.

Our center, which contains a smaller list than many other transplant centers in the region has been listing many people as the end of the year draws near.  Every time I see more people added to the list… especially those with higher MELDs and thus sicker… I just feel a twinge of what I guess you could call jealousy.  I know that they are higher on the list, they will be transplanted first.  Logically I know they obviously deserve a new liver over less sicker patients but it doesn’t erase the feeling because feelings are rarely logical.

There is a guy on one of our support forums, he is at our center and has gone through a bunch of crazy shit in order to get listed.  He is listed in two different states in two different regions, the second being recently added to the list here.  Because of his condition and his higher MELD I know he will be transplanted very soon, probably before the end of the year.  I am fucking jealous. There’s no other way to put it.  It just is that.  It makes me feel a bit shitty of a person yes.  It makes me feel weird.  It just part of the territory of all this I suppose.  A bunch of weird conflicting shitty feelings.

That’s probably the reason I have difficulties participating in our support groups… both our real life ones and our online ones. All these weird feelings, especially being on the wife-caregiver end.  I feel like nobody can possibly even begin to grasp my emotions and I just end up getting pissy.  People will give me the most well-intentioned advice and it can just… completely rub me the wrong way and I’ll be shitty about it for days afterwards.  And then there is the other problem of just finding shit too depressing.  That’s mostly the online ones.  A caregiver support for liver transplant group got to be too much.  People dwelling on every single negative thing….. give me positivity… give me a reason to get through the hard times…. damn.