In twelve days . . .

[ Jeff and I celebrating just before my birthday 2006. ]

In twelve days I will mark a milestone birthday. It is a normal milestone birthday for most people to turn 45 but for me it is monumental because 15 years ago when I was first diagnosed with my medical condition I was informed by multiple doctors that I would not survive this long. I was told by nearly every medical professional that my condition would progress and end in my death within 5-6 years. Every bit of research I read confirmed their diagnosis, that I would not make it to 45. I have, or at least in 12 days I will.

[ My 35th birthday ]

I have survived against the odds, against the intention of medical insurance to deny my access to treatments, against the incredible cost of those treatments. I survived because I am highly educated and when necessary sought more education around my medical condition; reading every study, every article, and always asking questions.

[ Hospital stay 2006, two months after my birthday.]

I survived because I refused to work with doctors who did not have the time or energy to help me fight for the necessary treatments and medications when my insurance denied it. I survived because I fought with every health insurance rep who denied my treatments.

[ Hospital 2007 ]

I survived because I was meticulous in gathering my paperwork and medical records and able to write convincing arguments about why the medical treatment was necessary. I survived because I fought for my right to be treated at home, with home nursing that cost far less than in patient hospital stays where I would be exposed to life threatening infections.  I survived because brought copies of all those medical journals, studies and articles into my doctor's appointments to discuss the findings. I survived because when doctors did not listen to me I searched for and found new doctors. I survived because I carry my medical records with me when I go into the ER to prevent surgeons from cutting me open again to look around.

[ First big rounds of infusions, 2007 - I sit in that chair daily for 6 hours for the two weeks leading up to my birthday ]

[ Spent my birthday in 2008 - in the hospital ]

I survived because I spent thousands of hours on the phone arguing with health insurance reps refusing to let it go. I survived because I spent thousands of hours writing and editing convincing arguments outlining why this treatment was more effective and less expensive. 

I survived because I recorded all my conversations with all those insurance reps and could and would play back those previous conversations when it helped prove my case. I survived because the ACA was passed and prevented health insurance from denying me access to healthcare because of a previous condition and prevented life time caps to my medical treatment.

2009, following week of infusions for Iron - my Birthday.

I survived because I have diligently logged my symptoms, noting time, severity, and any related causes. I survived because I keep a meticulous journal of everything piece of for I put into my mouth and every drop of liquid I drink with the times they were consumed so I can cross reference what food or beverage would cause an obstruction. I survived because I researched pain medications and worked with doctors to develop a multiple ways to manage pain. I survived because I realized that my mental health greatly influenced my physical health and stress in particular exacerbates everything. I have survived because I demanded every nurse follow infection prevention protocol especially when they were too busy to spend the time doing so. I survived because I researched and demanded new trial medications. I survived because I did not let fear or shame prevent me from using medical devises in public. I survived because I did not allow fear or shame prevent me from pursuing every State and national resource to maintain access to medical care. I survived because I put in the thought and effort every single day, in every way possible.

[ 2010, disneyland near my birthday. ]

In twelve days I will celebrate still being alive. In twelve days I will rejoice in all of the wonderful moments in my life I worked to enjoy. In twelve days I will revel in my accomplishments, and remember what my doctor told me last week. She said that I am alive because I am one of the, "most proactive and educated patients she has ever had", and that, "my survival has been a direct result of my conscious decisions, unshakable attitude and enduring sense of humor." She went on to say, "you don't just keep fighting, I have lots of patients who are fighting. You understood from the beginning that never giving up was just the first step. You ask questions, stay informed, read and research and you push me to do the same. You understood that you had to take control of your own medical treatment. More importantly, you do it with grace and with humor. I wish I could teach all my patients to approach their conditions the way you do." I cannot express how validated that made me feel. So many of the interactions I have had with medical professionals have left me questioning everything about myself. Advocating for your own medical care is always difficult but add to it a rare condition and it exponentially ramps up the difficulty. Searching for and finding a doctor who you trust and who you think will fight for you is priceless. When you find a doctor who is all of that, you do what ever you can to keep them because it will mean your life. I have been her patient for over a decade and she has been there for every step in the progression of my medical condition. This is why I drive two hours to see her now.

Infusion time again, 2011 and guess what it is my Birthday

For those who do not know or do not remember, I have Short Gut. I lost most of my small intestines to an infection following an abdominal surgery in 2001. My lack of small intestine prevents my GI tract from absorbing vitamins and minerals. This causes chronic severe dehydration which is life threatening and requires me to be connected to an IV through a port in my chest. The IV infusion treatment delivers all the necessary fluids, vitamins, proteins, and nutrients to my body through my artery. My inability to absorb vitamins and minerals causes a whole host of other conditions that negatively affect every system in my body. I lose bone marrow and then stop producing red blood cells. My body strips the calcium out of my skeleton which leaves my bones weak. The scar tissue in my gut continually causes bowel obstructions. My chronic dehydration places huge amount of strain on my kidneys and liver, which is further exacerbated by my dependence on IV infusion and Total Parental Nutrition (TPN). 

[ Dec. 2013, week of my birthday: My first infusion center trip with Ellie my service dog ]

[ My R2D2 IV pump, this is my first portable IV pump and gives me the ability to move around during treatment days - Summer 2015 ]

Your body is not designed to receive the bulk of it's fluids and nutrition through your arteries. The flow of thousands of milliliters of fluid into your bloodstream negatively effects your internal organs, especially the kidneys and liver. The stress of filtering all those artificial fluids, vitamins and medications takes a real toil on the liver and kidneys. Most TPN patients experience renal failure. The other big side effect from constant IV infusions is the ever present risk of Sepsis. Every time a needle goes into my port there is a risk of getting an infection in my bloodstream. Every time the IV line is opened, every time a medication is added into the IV line and every time the IV bag is changed there is a chance of introducing bacteria into my blood stream. TPN Patients a have high risk of septic infections.

2014, in hospital with both service dogs (mine and my husband's)
they are ganging up on me to make sure I am well cared for.

Patients who have already had sepsis are at a greater risk of contracting it again. I have had sepsis three times. Most patients who are on TPN do not survive longer than ten years, with the most common cause of death coming from sepsis or liver/kidney failure. I have always understood that my time was borrowed and that I would most likely die from side effects to my medical treatment, as in liver failure or sepsis. Last week I was hospitalized for severe pain in my chest and abdomen which turned out to be coming from inflammation of my liver. Chronic dehydration has causes sludge to develop in the bile duct and is obstructing that same duct, which is causing the bile to back up into my liver and making it very unhappy. When your liver is unhappy it is very unpleasant for the rest of your body. And my liver is very, very unhappy, which means I have intense pain.

Nov 2016 - Antibiotic Auto Infusion Ball - this cool little ball is designed to give the timed dose of antibiotics into my IV line without having to use an IV pump - which allows me to take it with me and still get the medication I need.

I am used to living with severe pain, but liver pain is a huge new level of intensity. I have not developed the coping skills to handle this new pain so my doctors placed me on high levels of pain meds. I am back on IV infusions with fluids, antibiotics, and steroids. This signals a turning point in the progression on my condition. When you start experiencing liver and kidney trouble you have moved to a whole other level. Or as I like to refer to it, I have leveled up! I have officially moved into that portion of the progression where the constant stress placed on my internal organs is starting to cause inflammation and eventually failure. This is a significant step towards the door in terms of my medical condition. And this new step forward in particularly devastating for many reasons:

[ Ellie and I resting together, I am getting infusion at Home with  the help of home nursing care, 2012 two weeks before my birthday ]

1. I was doing so well: The IV Infusion treatments were going so well: I started on this IV infusion maintenance treatment in June of 2015 and since then I have had remarkable success. My numbers have not been this good or this stable in a decade. I had more energy and began to feel somewhat normal again.

2. I found new meds: I have been reccomended to start a new treatment that is revolutionary and holds great promise. The injections make the cells in your intestines grow and therefore assisting in absorption in the intestines, with the idea that the new cells could allow me to spend less time on infusions. The treatment is exciting and has the potential to be life changing - granting me years more.

3. Just when I was ready to celebrate: I am approaching the milestone birthday that every doctor swore up and down I would never see. Why do i always hit the turning points right when I think i am finally winning?!?

[ Disneyland 2014, for my birthday. ]

[ Navigating Disneyland for my birthday - going to the happiest place on earth with a PICC line in is tricky, but it is also a much needed distraction. Disney has become the much needed respite for me because they are so good with disabled accessibility. I am able to go there and feel safe, and I can feel human again because of how they assist guests. Trust me when you are dying - Disneyland is a great distraction and safe way to escape. ]

So looking around and taking stock of where I am:

I am coming to terms with having leveled up toward the end of the game while simultaneously passing the birthday I was never supposed to live beyond. I feel conflicted to say the least. Why now when everything was finally working? It feels like two steps forward and three steps back. I can tell you that these kind of hilarious paradoxes become the norm the further down the rabbit hole of terminal illness you go. Which just so happens to be a great source of humor and continual reminder to take nothing for granted. That new medical treatment I am so jazzed about - it uses one life threatening disease to treat another. In my case the medication causes a 'controlled cancer rate' in my intestines; the idea is that the new cells will absorb enough to allow me to have fewer IV infusions. Fewer infusions means less risk of organ failure and sepsis. So the idea is the benefit of the new level of absorption outweighs risk of the controlled cancer becoming uncontrolled. The major draw back (besides possible bowel cancer) is that the new growing cells also cause a huge amount of pain. So much so that most patients are prescribed morphine in similar dosage to actual bowel cancer patients. The growth of the cells will stop when the medication is stopped for most patients, but in 30% there is real risk of the cells continuing to grow and becoming cancerous. Only in terminal world do you think it is exciting and promising to grow new cells through bowel cancer. When you are in the Hail Mary stage of the game - you begin to consider all the option even the ones you never thought you would.

As I look back over all the years I have been dealing with this, I have a lot of pictures of me in the hospital or in treatment somewhere. I noticed that I have spent so many birthdays in the hospital. I thought, "holy shit I have been in the hospital on nearly every birthday." But then I realized it is not being in the hospital that is weird, that is more my norm. It is having birthdays that is the odd thing. For some reason that made me giggle. Oh and that reminds me, I survived because I giggle a lot. I also survived because I made the choice to be with a partner who giggled a lot too. We survive together, giggling along the way.

In twelve days I will celebrate my 45th birthday. I am not sure how or where I will celebrate it, but I am sure at some point there will be dancing. I will roll my little R2D2 IV infusion pump case along with my service dog, Ellie out to the middle of the dance floor to dance. 
I will dance in joy. 
I will dance in defiance.
I will dance in self accepting love.  

I will enjoy the day, mark it with celebration and cherish all those who are there with me. Cause in twelve days I turn 45 years old. Forty Fuckin Five and that is a good good thing. So I am gonna celebrate my birthday.

Then I will get back to fighting to make it to the next one.

[ It has been a long journey through all of this - and I have been through so much. It struck me as I was going through the photos how many birthday I have been in the hospital or in an infusion center or in treatment at home. But then I remember that I am in treatment all the time. The medical treatment is normal daily life and birthdays are the weird things that happen every now and then. ]