It all began with a well intentioned trip to visit Grandma over Thanksgiving holiday, to cook.. relax, catch up and spend some QT with my favorite woman over 90 ;) I had been feeling pretty tired in general and having difficulty breathing when climbing my 5 flight walkup to my apartment for months... but I assumed I was just out of shape or over stressed and didn't think anything of that. I had also been having some weird lingering headaches on and off for about 2 weeks, but assumed they were just stress and the change in season. I had two random migraines in the weekend before I left, which was strange because I haven't had migraines since my teens but figured they just might be coming back. I'd had some weird numbness in my face and hands a couple of times in previous months... something I attributed to a little food allergy I'd never noticed or something negligible like that. A month earlier I had taken a trip with girlfriends to the Bahamas for a restorative weekend, and while there I noticed a really red - spotty rash all over my skin around my torso & thighs... but assumed it was just sun rash from my skin being in pasty white NYC for so long, it went away when I got out of the sun. In retro-spect its easy to list these things out and see need for attention but since they all happened over the course of about 6 months no one put anything in relation to the other.
So I decided the week with Grandma would be therapeutic and I took the vacation time to go. My first day there was great, we went to breakfast, got Grandma a nice perm, I went grocery shopping in preparation for some cozy afternoons making yummy healthy food. Day 2 I woke up with the throbbing head pain of what I thought I knew was a Migraine (light sensitivity, sound sensitivity, the numbness around my face and hands, inability to hold food/liquids... I sell the medication for this condition, and suffered from it in my teens, there was no doubt in my mind that this was what I was experiencing... it was just a lot worse than I had ever experienced before). This 'Migraine' went on for the entire week, I was literally holled up in my room at grandmas sleeping for four days straight... I would wake up to try to force down soda water and crackers, then throw those up and just go back to bed. I was calling mom crying for days trying to figure out what to do ... I took two Relpax (the migraine meds I sell... which two of should knock any migraine out cold) they did nothing because I threw them right up... same with over the counters I scrounged from Grandmas. So by the end of the week I was so severely dehydrated and famished (lost 12lbs!!! probably all my water) that I was getting disoriented. Everyone insisted that I go to the hospital or clinic in MI to get some meds but I was convinced that I needed to see one of the headache specialists that I have a relationship with in NYC and refused to chance getting stuck in MI any longer.
By the day I was scheduled to fly home I was a complete wreck.. I barely remember leaving Grandma's house for the airport as I was so disoriented I didn't even understand what I was doing. The car picked me up and had to stop 4 times on the way to the airport so that I could throw up on the side of the highway... I had tears of pain streaming down my face the entire time... I was too disoriented to put together full sentences at this point. When the driver let me off at the airport I spent a solid half hour clinging to the trash can outside because I couldn't stop dry heaving. If a single human being had actually been forced to interact with me there is no way they would have let me on the airplane. I stumbled into the check in, sat down in front of the electronic kiosk, swiped my credit card and realised that I didn't even understand letters anymore... looking at the sentences on the kiosk was like looking at brail... so I just pushed anything green or in the shape of an arrow until my ticket was spit printed. There was no one in the airport so I went right through customs without a word to anyone, and I followed someone with an NYC sweatshirt to find my gate because I couldn't understand the signs.
I dry-heaved into a bag through my flight back, managed to remeber from previous trips which way to walk from my gate to the cab line, sat down in a cab and said "New York Presbyterian Hospital, 68th street and York FAST PLEASE!" (this address I know by heart because I call on MDs here every week for Pharma Sales, and its the closest hospital to LaGuardia Airport so I knew if I had a shot at arriving conscious & coherent NYPH was my best bet)... the cabbie raced my dryheaving body to the hospital and that was the luckiest thing I ever did! So at this point I'm going into the ER thinking if nothing else I need something to make me stop puking and I need an IV stat. But assuming that they would give me some sort of morphine or painkiller that would kill the immediate pain, and then require someone else to pick me up (like the dentist does after wisdom teeth). So around 6pm I checked in at the ER and texted John and Heft to see if either of them could get out of work to come get me later that night. John was able to come right up to the hospital and was waiting with me before I even got admitted... and hefty came only hours later all the way from her family in NJ because thats just how amazing of a person she is :)
The first night was spent largely in the ER, they gave me many IVs and morphine and other pain things that I was completely unaware of... I basically just went in and out of sleep as the MDs came in and out running different tests. The last big clue of TTP was that my urine sample was super red... (which my morphined/disoriented brain was not surprised by... funny - I remember looking at it and saying outloud to the MD "Oh, yeah I ate beets"... I hadn't kept food down in a week).
John and Heft stayed with me all night, spoke with all of the different MDs kept track of what they were giving me and what they thought might be going on after each new test. They said it was just like an episode of House, where the groups of white coats just run in and out of the room and each time they ask weird questions then come back with a completely different idea of what could be wrong. Apparently it was a younger Fellow who actually specialized in Kidney treatments who really picked up on TTP as a possibility... he had checked my kidneys after the urine sample and remembered something from another case that came by him in the past. And as it turned out, an MD named Gordon at this hospital was one of the pioneers in developing a treatment for TTP. Luckily for me NYPH was were I landed, and Dr. Gordon is now my Dr.!!
Initial Diagnosis & Treatment ----------
So they decided its TTP, what does that mean? Here I will try to give you my explanation of what TTP is, which I must preface by saying will surely fall short in many ways because even Dr. Gordon (who has worked on it for 20+ yrs, and helped develop a life saving treatment for it) will openly say that even he doesn't know exactly what it is and why it happens (reassuring I know :) ). I have put links to medical explanations etc. on this site so if you are so inclined, or would just prefer to hear this part in medical terms versus my explanation please feel free to check them out (they are in the column to the left of this text).
TTP is an extremely rare auto-immune disease (affects 1-3 people per million per year and anyone can develop TTP but it is most common among 20-40 year women, its not transmittable between humans unless its genetic - mine is not genetic, this they tested and found negative so I won't pass it to children which is good - and the cause is unknown) in which one's blood forms clots within their own circulatory system for no apparent reason, and the clots do not un-clot as they normally should. It is extremely dangerous because these clots are so small that they do not show up in X-rays or other normal procedures until they are large enough to cause deadly strokes or aneurysms, or until the blood supply is cut off from a vital organ long enough to kill it and cause you to seek medical attention for that. Additionally, even when these clots are still letting some blood flow by they are rough and end up breaking most of the passing blood cells as they are squeezing through... so all the red bloods cells that bring the body oxygen/nutrients are dying off faster than they can regenerate and causing anemia & organ damage as the clock ticks. Top this all off with the final situation that even before the stroke/aneurysm/organ failure red flags that would alert you to something being wrong, when you're walking around as a severe anemic (makes even small physical exertion very difficult so you easily pass out and fall down, this was me climbing my 5 flights of stairs 3 times a day clinging to the railing whenever I started to black out) with no platelets left in your blood (because they formed clots where you don't need them, there are none left to clot and stop bleeding if you do fall and get a little cut or internal bruise).... and you're cruising around thinking nothings wrong, if you fall and start to bleed your body can't stop that bleeding, so any injury and you're screwed before you know it.
They don't know why or how exactly the clotting function gets messed up, but they've narrowed it down to a malfunction in the Von Willebrand factor of one's Adams13 Enzyme. Normally this enzyme would cut up blood clots after they did their job, in TTP for some reason it stops doing that. They also don't know why the random clotting starts, but somehow the combination of the two is TTP. The Adams13 enzyme is found in the plasma (watery part) of your blood, so the treatment is to replace this part of the blood through a process called Plasma Pheresis.
This treatment is what Dr. Gordon helped develop. Through Pheresis, which involves me having two tubs connected somewhere into my circulatory system with a fast blood flow... the tubes suck my blood out of my body and into the machine where it gets spun around until my plasma can be skimmed off the top. Then the blood goes into another part where it is mixed with bags of donor plasma and re-heated then returned to my body... Heft says its like an oil change :) . The process takes 3-6 hours each time, and they have to give me several different preparatory drugs to prevent any reactions to the donor plasma (if your body rejects the plasma its a whole other problem... this you will hear about more later) sometimes they have to put me in a little hot sack that keeps my body temperature up for more blood vessel dilation so I have to lay there in a sweaty sack for the whole thing. It is very uncomfortable while the machine is running ... it feels like your body is puffed & swollen with too much liquid and theres a constant rushing feeling everywhere in your body. When I finish treatments my whole body feels heavy & weak like its vibrating for a few hours, sometimes I have to throw up and am all dizzy... its really icky.
So now that they think I have TTP, around 4am they moved me from ER to the ICU because of the severe consequences of further clotting (again see the links for mortality rate/permanent damage/dissability etc). All of the symptoms I showed in the last day at grandma's were signs of my body shutting down because of all the clots that had been there and built up over the past 6 months or so. Thats why I had ultimately started losing brain function and processing skills, because the clots had cut off the circulation to that part of my brain (basically lots of mini strokes MDs said). Everyone in the hospital that worked with me in these first few hours came back over the course of the next week and marveled that I made it through the trip from MI to NYC. I've never heard "You're so lucky to be here" from Dr.s that many times...pretty freaking scary. Thank you Universe :)
The next afternoon they brought up the machine for my very first treatment of Plasma Pheresis. By this time John & Heft had been kicked out of the ICU (someone who felt more strongly about visiting hours took over my room... booooo) so the details are a little foggy over the next few days since I didn't have anyone there with me, and I was still being administered all sorts of things through IV that I didn't know, my memory is vague. Sometime in the afternoon they began the first Plasma Pheresis treatment.
They tried to do it through my two arms... but I was still severely anemic from all the blood loss and they couldn't get enough of a flow to run the machine. So they called in a surgeon to insert a femoral catheter (tubes that came out of my groin to give access to those huge arteries that run into your torso). That hurt like hell... and I was on morphine still so you know it must have been bad. But once the catheter was in they hooked me up to the machine and I went through a very successful (meaning nothing went wrong & my body accepted the donor plasma) Pheresis :) My cousin Sarah was there with me through it and an incredible comfort during such an uncomfortable situation!
By this time my mother had been notified by Heft of my situation, and probably nearly lost her mind (after talking to me crying for the past week.. I’m so sorry mom!). She had looked up flights to NYC and was running around like a mad woman who just entertained a houseful for Thanksgiving then got a call that her daughter was in intensive care across the country… because she had. When she spoke with the MD and he said ‘Well, she’s in intensive care because what we think she has is essentially fatal if left untreated and so we’re starting a treatment to try and fix it right now…’ she got in the car and headed for the airport. About 8 hours later Sarah picked her up from the airport and there she was.. standing in front of my bed with the look on her face that made me realize for the first time that something very bad was going on with me.
Up until that point I very honestly didn’t understand what was going on at all. I was on the drugs so I wasn’t upset, but if you asked me why I was where I was at the time I couldn’t have told you. John, Heft, Sarah, Daniel… they were all coming in periodically to check on me and I was happy to see them and really didn’t know why they were there too. But when Mom showed up and I saw her face as she looked at me, I lost it. I cried and cried… and I was just scared for the first time realizing that I should be. That was a very tough moment.
For three days I lived in a bed in the ICU with about 10million tubes/straps/monitors sticking out of me & connecting to the most elaborate collection of beeping boxes you’ve ever seen. I had a Plasma Pheresis each day, and each day I was a little more cognizant. My visual hallucinations started to go away (it was like being on acid with the rainbows & squiggly distortions everywhere, very weird)… I was starting to understand more of what people said to me… I could read letters and numbers and know what they stood for… I still felt like absolute shit but I was increasingly closer to me. This meant that the Plasma Pheresis was not only accepted by my body but it was also starting to work to remove the clots. The Platelet levels in my blood started to rise (this is how they measure whether my body is responding to the new plasma… if the # of platelets floating in my blood goes up that means the number stuck in clots has gone down and my body is making more platelets as it should… healthy levels are within 200k-500k, when I checked in to the ER my Platelets were 17k!... pretty bad!). My platelet levels slowly rose and I floated back to the surface with them.
I was moved to a regular room in the hospital after three days in ICU, and aside from the first night (during which I unknowingly explored the wonderful new world on massive doses of Prednisone, plus a few other mystery drugs mixed in for fun by some angry MD… that full story you only get in person) I continued to get daily Plasma Pheresis and continued to improve.
So once there is an immediate response to the Plasma Pheresis (which I had, my levels went up and very quickly according to MDs) the next step in the puzzle of TTP is ‘How do we get the platelets to stay within range, without needing a Pheresis everyday?’ Again the answer to that is still largely unknown, and while I will describe what they think it is here, I provided links to medical outlines that you are welcome to view at will. They are also in the column just to the left of this text.
Once the platelets are within healthy range from Pheresis, the next problem is that they just don’t understand TTP well enough to know how to make sure they stay stable & within range. The body might just turn around and start clotting again, or it might pick up on the new pattern set by the Pheresis and be fine. The current methods to try and make it stay level are to use a drug called Prednisone, a steroid used for many autoimmune diseases which suppresses the body's immune system so that it hopefully doesn’t attack itself again. There are other steroids used, other immuno-suppressive drugs used, some have their spleens removed… there is a whole list of methods to try and stop the body from attacking itself in an autoimmune disease. Dr. Gordon currently thinks that combinations of steroids and other immuno-suppressive drugs like Rituxan (this will reappear later on) are the best methods.
Initial Hospital Release & Follow Up -----------
So I was placed on a regimen of really high Prednisone doses, IVs of it in the hospital, then oral doses of 70-60mg/day once released (steroids are really bad for you... tons of nasty side effects and this is a very high dose). After a week of Plasma & steroids they finally deemed me well enough to go home ☺ I was so fortunate to have my mother there with me for another week post-hospital. She helped me get around NYC, made sure I ate well and allowed me to feel human again… thanks Mom!
After a week of going back to a very restricted version of normal life, with the extra help of Mom I seemed to be recovering very well. And I was dealing with the drugs as best I could. I was back up to the hospital every other day to have my platelets checked, and they were falling but only very slowly (from high 300s at my release) which MDs said would happen for a while before my body found its ‘normal’. As long as I am 150-200 or up I am out of the hospital till the next test, and that’s my new life with TTP. Its like trying to make it past the 'Go to Jail' spot in Monopoly each time around, every morning I wake up is another roll of the dice. If I land on that spot/get that card (ie get a low platelet test) its 'Go Directly to Hospital, do not pass Go, do not collect $100, and you lose a turn'. :(
After a week at home with good tests Mom had to get back to her life (and the house full of furries) and Dad who so kindly held up everything for her in CO when she rushed out to NYC… Thanks Dad!! So I sadly watched her go and slowly adjusted to being on my own again, working & finding my new life with TTP.
Since monitoring was all that could be done, and things were looking ok thus far, they gave me permission to go home to CO for the holiday. I was under strict order to be careful with exertion etc. because of the altitude, and had two blood tests set up at the local hospital so that everything could be monitored in case the changes spurred a reaction in my levels. My tests were good while I was home (platelets actually rose for the first time since the hospital while I was home! I was so proud that I had started growing my own platelets again), I came back to NYC and they were still good (even off medication which I was super stoked about) and my MD basically looked at me and said 'You are solid, I'm confident you're in remission and don't need to get checked for a couple of weeks'. This was of course fantastic news just to think I was healthy and under control of my body again, but it was also amazing news because John had booked us a week+ trip to his business partner's place in Turks & Caicos and I couldn't wait for that time in the sun :)
Relapse & Return to Hospital for Treatment---------
Then the weekend before we were supposed to leave I had a slight headache (ignored it), woke up with a stronger headache... nearly passed out giving my Yoga certification final (total of 30 minutes standing and instructing... should not have been physically taxing AT ALL)... threw up my dinner that same night and the red flag was raised. When I went into my Hematologist the next morning he came out of the scary blood room with a piece of paper, six flagged MD business cards, his open cell phone and a big nervous look on his normally smiley face. Not good. He said "Well, Lauren... I feel kinda bad like I jinxed ya or something... your platelets fell to 106 and the other counts look bad... so I've called Dr. Gordon (big wig MD at the hospital) and he has a bed ready for you. You need to go straight there and get hooked up for plasma this afternoon." Shit. I landed on the 'Go Directly to Jail'. So I broke into tears/dizzy texting to let ppl know that, surprise! - no I'm not going on with life today to go to work and eat my lunch and go on with tomorrow etc. .... I'm being sent back to lay in a bed while people poke me and fill me with medicine that makes me feel crazy. great.
Once again hefty my angel came to the rescue and showed up instantly at the hematologists office, took me home so that this time I could at least pack a quick bag for the hospital, then drove me to the hospital and stayed with me till I was all changed into the dreary blue gown and hooked up to tubes again. Bless her. At this point everyone was still very positive and saying that 'I caught it early, should be a quick few days... just 2-4 Plasma treatments and I should be all set and ready to go again...' And I went through the first 3 treatments largely as they expected. When I went to sleep the night before plasma #4 the plan was to wake up early, get the plasma.. have an hour off the machine then possibly finish the day with my first treatment of a drug called Rituxan.
Now, I had been warned by Dr. Gordon (big wig) about 'possibly being put on Rituxan' during my first hospital visit... he said "don't be scared if they want to give it to you" and there wasn't really any further mention of it, they never brought it up again and it wasn't given to me. Still, what Dr. Gordon said made me curious so when I was at home over the holiday I did some research and found some online support groups, and subsequently scared the living shit out of myself hearing everyone else's horror stories about their life (or lack thereof) since being diagnosed with TTP. Riutxan had a few shining moments amongst these stories, and I looked at the most recent studies/reports on it... basically its a full fire-power blaster of a new drug that was only recently (about 10yrs) approved by the FDA for treatment of cancer that doesn't respond to chemo combos. It has a list a mile long of active side effects (not unlike many drugs if you read the fine print) and then basically says 'We have absolutely no fucking clue what this drug will do to humans long term'. That scares me more than anything I think I can imagine. Genentech (reputable, but newer company that pioneered a lot of the stem cell drugs that CA fought about the past few years) made it out of a human cell mixed with a mouse tumor (um... excuse me? no I don't want mouse tumors placed in my blood thank you) and somehow this resulting drug completely kills your Bcells (one of many cells produced by your bone marrow, which in turn makes multiple antibodies that float around trying to protect your body from infection). This method of killing the Bcells apparently has shown great success in cancer patients, and although it is not even remotely approved let alone formally studied in TTP patients, it is being used to treat TTP more and more with decent ranges of success. Dr. Gordon is apparently one who supports this as a great option.
So, on the morning of plasma #4 I am frantically speaking with every MD who will talk to me and arguing that my situation is not dire enough to put my body through the immediate stress and potential long term damage of this fire-power cancer drug... please please please tell me I have another option because I'm losing my mind thinking about the millions of things that this could do to me. And the basic response I got from Dr. Gordon's right hand lady was: "We have taken to using this drug on first case TTP patients, so this is what he wanted for you from the get go. And while its scary to think of what the drug might do, the reality of TTP is that each relapse happens faster and harder and increases the likelihood of another relapse. No one relapse is less deadly than the first episode, if anything its worse because it will happen to quickly to treat. (interlude the story of a girl like me who left the MD fine and then half died and had lifelong brain damage 12 hrs later from a relapse - pretty harsh thing to hear to your face)". So as I lay there in the hospital letting them hook up the tubes for plasma I tried to just come to terms with this new state that is apparently my life and reality, and accept it with the most positive outlook possible... something I think I'll struggle with for quite some time actually.
Then after about 2.5 hours of plasma pheresis and me working on accepting my life, I started to feel a little more dizzy than usual... a little more lightheaded... a little more confused... and my legs started shaking, then my arms and my torso and about every muscle in my body and suddenly I was surrounded by people in white coats trying to hold my body down, hold the bed still and somehow unplug my convulsing body from the machines (tricky with all the tape and needles when those limbs were shaking)... My body didn't like something that Plasma donor of bag #7 had for breakfast that day and decided to rather violently reject it. SO they frantically pulled the plugs (a small problem since this treatment involves so much of my blood being outside of my body and in the machine thats sitting next to me filtering shit in&out before returning the blood to me) to cut the line of plasma coming in, and this left my body with very little fluid left to recover on. Once they stopped the convulsing they plugged me into as many other drips of things to fill me up again as possible, and spent the day poking and measuring vitals until I came out of the accompanying delusional fever. So much fun I tell you, wish you all could have been there :) Obviously the Rituxan was removed from the menu that afternoon.
The next day they spent poking and measuring again, until about 8pm when they decided that I was stable enough to try the Rituxan. Hefty was again at my bedside watching everything like a hawk, doing everything she could possibly do to help me be calm and just feel human. They brought in and started the drip around 8:30pm, and it finished around 3am. Amazingly my body had zero reactions during the drip, and zero bad reactions the day after. It left me feeling like I was beat up on a sports field for about3 days straight, but apparently that is how these drugs hit your body and to be expected. The best way I can think to describe it is, like the way I use to feel on day 1 &2 post spring break... you're sober, and supposedly functioning normally again, but your body feels like its full of some sort of sludge that has to get worked out and you're in a fog and can't hardly figure out what normal should be?.. remember that? thats the best I can compare it to.
Anywho, that was all good news at the end of a pretty bad week. So they finally released me this past Saturday afternoon, and I've been under strict care of John and Hefty (bless them both) ever since. Since the trip to Turks & Caicos was for this week, to relax and be happy that things were healing, John was kind enough to keep his vacation days from work and spend the week taking care of me, making sure I don't drive myself crazy :)
Release from Hospital Relapse & Follow Up Treatment -----------
So I've been going up to the MD every other day since last Sat to have tests run, and for reasons unknown to any of us my Platelets have been great (300s which is very within range). This is fabulous news because as long as my platelets are within normal I don't need to be in the Hospital for pheresis. However it is the most frustrating part for me (and the MDs) because none of us know why they have been great this week... especially considering the fact that all last week while I was in Pheresis they didn't budge from the 150's... even after 4 treatments. I was super nervous going home on Sat because I had basically no cushion in my platelet count and was positive that the next time I was tested they'd just send me straight back in. Why my body decided to cooperate this week no one knows. But I am grateful that it did, because now I am sitting in the Hematology office, having what seems to be a very sucessful drip treatment no2 on Rituxan. Been here since about 930am, will prob be done by 2pm. And the forseeable status beyond this is as follows: The rituxan works like chemo, they fill your body with it and give your body time to recover, then fill it again etc. until they think that the drug has sufficiently impacted those Bcells. I'm currently scheduled for treament consisting of 4 drip sessions, so I'll be coming in once a week, and having this half day drip treatment. IF my platelets don't drop in the days between drip treatments then they'll just keep me on the oral steroids (and the pile of other drugs to prevent the horrid side affects of the steroids... please never take them if you don't absolutely have to... they are a whole other story) and then blast me with Rituxan treatments weekly for 1-2 months. The hope is that if my body can handle the blasting, it will put me into a long term remission. The uncertainty is that if my platelet levels drop during the Rituxan blasting, I'll have to go in for plasma pheresis treatments to get the platelets back in a safe zone, and the pheresis treatments will wash out all of the Rituxan and steroid residuals that have been building up in my system to prevent my body from attacking itself again. So the Rituxan/steroid combo theorhetically can and should put my body into a stable place... but if my levels waver too much in the time before I'm done with Rituxan treatments and steroid tapering, I'll have to start the process over again.
The good news about the Rituxan is that my MDs think it should really help to put me into a stable remission. There is very good hope and reason for that to happen... Hefty has even put me in touch with someone whos living proof of success with this treatment, a super nice woman and she's been in remission for 5 years since her Rituxan! SO thats my goal. I have absolutely no control over whether or not it will happen, but thats about all I've got to aim for now.
I took a month of Medical Leave from my job, and my manager is incredibly supportive of me so I can always take more time if needed. I am blessed to have had insurance when this all hit so I am not destitute from the bills yet. (a side note for everyone... I know people always preach about insurance but last week I saw why... I got one single paper from Aetna with a total for $72,000 !!!!!!!!!!!!!!!!!!!!... and that wasn't even for the whole first week in the hospital. My portion to pay was around $700.... that is just one bill. Please always have insurance!!!).
The most difficult part of all of this is the unpredictability. I could at any moment get a test with platelets that dropped 200pts and be sent directly back to the hospital for and undetermined amount of time. Thats why you guys are always getting these random texts that say I'm fine, then sick, then fine etc. And no one has any way of knowing when things will stable out because that is the nature of TTP and how little is known about the disease.
Its difficult to write all of this down, largely because I'm still in shock that this is my life right now. But my hopes in doing so are not to dwell in the details of whats happening, so that when the people I love already know whats going on with my health, I won't have to talk about just that with them in the moments we are able to chat. Then we can talk about what I really want to be thinking about again... LIFE :) Real Normal Life... it gives me strength to hear all about the stuff I know I will be doing again when I beat this bitch :)
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