Evan’s Syndrome – Me, Myself, and Evan’s

The Post-Treatment Blood Draw

Posted on July 7, 2016

My IVIG treatments finished on Monday, June 27th and my one-week blood draw was this past Tuesday, July 5th. I called my hematologist’s office yesterday morning and they had the results of the CBC from LabCorp already. To my surprise, my hemoglobin went up to 11.6! Even though my Prednisone was decreased to 75mg, my hemoglobin went up…? Can that really be true?! Ecstatic doesn’t even begin to cover it. The hemolytic anemia was, originally, the worse of the two problems. It was the AIHA that caused my jaundice and elevated bilirubin and landed me in the hospital in May. I am VERY excited that the AIHA is essentially in remission at this point. It will still need to be monitored as I continue to taper off the steroids, but I am no longer worried about it.

My platelets, on the other hand, plummeted again. My last platelet count was 159, which was great considering 150-450 is considered the normal range. Fast forward to this week and my platelet count was 62. In case you don’t feel like doing the math, my platelets dropped 97,000 in eight days. That’s not ideal. I had read that the problem with IVIG is that it is often a quick fix, but a lot of times doesn’t work long term. The infusions are meant to give you a large boost in platelet count, but then it is up to your body to continue producing platelets and to know not to kill off the good ones. I think if there was an underlying cause to my low platelets, such as alcohol consumption, drugs, etc., IVIG and abstaining from the problem substance would have been enough to fix it. Take away the problem – take away the destruction. Then it simply would have been a matter of infusing the platelets and PRESTO! Since my problem is ITP, which, by definition, is an autoimmune disease focused on platelet destruction with no underlying cause, my treatment options are more trial and error. The infusions worked to boost my platelets, but my antibodies are still latching on to these platelets and marking them for destruction. Until/unless my immune system can begin to recognize its own healthy platelets again, I’m not super confident my numbers will ever be under control. It seems ITP is going to require constant maintenance.

The thing that I find really frustrating is that, of the two autoimmune diseases that comprise Evan’s Syndrome, ITP is the one that is harder to recognize or diagnose. With AIHA, I felt sick and miserable and tired and weak. With ITP, I just bruise easily. I don’t feel sick, I don’t feel broken or weak, and yet, I’m undergoing long treatments and being kept home from work. I know I’ve said it before, but it is so hard to grasp the concept that I’m sick but don’t feel sick. The idea that my body is destroying itself without any indication is frightening. How am I ever going to know if my platelets are low without a blood draw? I won’t. At least with AIHA, there is a chance I will be able to notice when something is wrong (to some extent). I’m afraid with ITP that I’ll never know there’s a problem until it’s too late.

When I see the doctor Monday I find out what comes next. He’s probably going to recommend a bone marrow biopsy (no, thank you) or another round of IVIG infusions (not loving that idea either). Hopefully he doesn’t immediately jump to Rituxan (the chemo-like treatment). If I had to guess, I’d say it’s likely that there is more IVIG in my near future, but maybe he’ll try a different route considering the reaction/side effects I had by the final treatment. Maybe he’ll say 62 is a safe enough number and he will hold off on any additional treatments and let me wait it out to see if the number holds! Either way, at least half of the ES is under control and responding to treatment the way it should be. One down, one to go!

The Paranoid vs The Cautious

Posted on July 5, 2016

This morning my husband and I had to drive into Saratoga so that I could have a blood draw. We had to drive 50 minutes there and 50 minutes back all for a three minute blood draw, but otherwise it went well. I’m not sure how long it will take to get the results. I am much more likely to hear my numbers from the doctor than from the lab. LabCorp said they are going to mail me a copy of the results, but it could take 7-10 business days to receive. I’ll already have my next blood draw (and results) by then, rendering today’s results useless. I’m hoping my hematology center gets the results by Thursday and I can call them and ask for my hemoglobin and platelets. I’m not worried about the platelets, but I’m a little nervous about my hemoglobin. Since the RBCs were responding to the steroids, I’m afraid the taper will have negatively impacted them. I would love to at least be able to maintain a double digit hemoglobin number and triple digit platelets.

I had a lot of trouble with the hot flashes today. It was 80 outside and humid. Being indoors wasn’t enough to beat the heat. I had the fan directly in front of my face and was still dripping sweat. I had to go into the lake to cool down, which did work, but then when I got back up to the cottage my oxygen saturation was at 93 and my heart rate was at 164.

In addition to the heart fluttering and profuse sweating and shortness of breath (only when my heart is racing) I’ve been feeling lately, my stomach hurt earlier. This is the part I hate. The first time my hemoglobin was low I didn’t recognize any of the symptoms as a cause for concern. At the hospital, the nurses and doctors kept saying “at least next time you’ll recognize the symptoms”, but that’s not necessarily true. The reason the symptoms didn’t concern me the first time isn’t because I didn’t notice them happening – it’s because they are such common symptoms. They can be caused by anything. My stomach ache could be because I ate too much or too fast. Back pain could be related to an injury or stressor. These aren’t things that are going to set off any bells in my head. Right now it has the opposite effect though and ALL of these things are setting off bells. My brain keeps thinking any one of these common symptoms could be the sign I’m supposed to recognize or be looking for in terms of a relapse or drop in my numbers. Am I paranoid or simply being cautious?

Also, the fact that exertion of any kind, no matter how small, completely knocks me on my ass isn’t going to be good. Walking up about 20 stairs from the dock to the cottage caused my heart to beat like crazy. Imagine how my commute to/from work is going to be everyday. That is 52 stairs in the morning and a fairly lengthy walk uphill in the evening. It wasn’t easy or pleasant before and now it’s going to be even worse. Essentially, any time I have an “episode” where my heart starts racing, I have to sit down, relax, drink some water, sit in front of a fan or apply ice packs to my face and neck, and take deep breaths until my heart rate returns to a safe level. That’s going to get old FAST in the mornings. But I’ve already been out of work for nearly six weeks. I’ve been working from home as much as possible, but the last couple of weeks were useless between treatment and the nasty side effects of treatment. I feel guilty because my hematologist keeps pushing back the date in which I will be able to return to work. At the same time, though, I trust his professional opinion. The earliest I will be back in the office is Tuesday, July 12th, pending the results of my July 11th appointment and blood work.

I’m not even sure what the game plan for Monday’s appointment is. I’m assuming the discussion will be largely based on my blood counts. I figure if my counts are good, he will taper the Prednisone again and set up an appointment for the following week to check my progress. If my counts dropped, I’m not sure what happens next. I doubt they will have dropped low enough to warrant any kind of immediate action, but he may bump the steroids back up to 100 mg. I highly doubt he will increase the dose because he wants me off the Prednisone as much as I want to be off of it, but there’s always a chance he will choose that option. Regardless of my counts, I’m sure I’ll have weekly blood draws and appointments until I am off of the steroids completely.

Once I’m off the Prednisone, the doctor and I will be able to focus on what caused the sudden AIHA & ITP (ES) and how we are going to treat it long term. In the meantime, things are all still up in the air:

How did this first 25 mg taper affect my numbers? Is it going to affect my platelets too or just my hemoglobin? If my numbers didn’t drop does that mean my immune system fixed itself? Is it possible that this has gone away and will never be a problem again? Will the next taper be more drastic or would we expect to see the same type of results? At what point during the tapering process is relapse most common?

There are so many things I wish I could know before it’s too late. I don’t want to find things out the hard way this time.

The Inconsiderate Assumption

Posted on July 3, 2016

On our way to the cottage yesterday, my husband and I stopped at a thruway rest stop to use the restroom and grab a snack. As I was washing my hands, the lady at the sink next to me was blatantly staring at the bruises on my arms in horror. She was NOT subtle at all. Judging by the look on her face, I am 99% certain she thinks someone beat me and gave me these bruises. Part of me wanted to yell, “I have low platelets, bitch!” or perhaps a simpler, “I have a medical condition”, but then I realized it’s none of her damn business! I shouldn’t have to explain myself to strangers.

Though I find it rude, strangers have every right to stare, but they have no right to know my personal business. I shouldn’t have to feel self-conscious washing my hands in the restroom. If you’re going to make assumptions or judge me, fine, but please at least be subtle about it. It would be different if her face showed concern, but it was just disgust. I’m sure she went back to her car and told her husband or friend or driving companion some crazy story about the bruised woman in the bathroom. Speculate all you want, lady, I’ll just be over here trying to find a long sleeve shirt to wear all week in the eighty degree weather. So, thanks for that. I know I shouldn’t let other people’s looks or judgments bother me, but it’s hard to ignore the looks.

Fade, bruises, fade!

The Semi-Productive Sunday

Posted on June 26, 2016

Today was the most productive day I’ve had in a while. I woke up around 7, made myself breakfast and sat down to watch Netflix. After I ate I took my pills, ordered my husband a present off Amazon, added to yesterday’s blog post, and emptied the dishwasher. I laid down for a couple hours and then around 11 folded all of the clean laundry in our apartment – about four baskets full – before making lunch for my husband. We caught up on a few of our shows that we had fallen behind on and then he fell back asleep. I took this opportunity to do some work I hadn’t been able to get to during the week. I don’t like being behind and I finally had a three hour block where my headache was under control and I felt focused. That has been one of my major problems this last month that I didn’t even realize was happening until today – I have been completely unable to focus or concentrate on something for very long and I have been confused a lot more easily than usual.

Anyways, my day was productive and I felt accomplished. The only downfall, besides the massive headaches, is that I couldn’t manage to make it through my shower tonight. It felt like someone was sitting on my lungs. I tried pointing the water off to the side so it hit the wall instead of pummeling me in the chest, I tried making the water colder, and I tried just standing for a minute with my arms at my side, but no matter what I did, I couldn’t get my heart to stop fluttering and I couldn’t catch my breath. I would normally be afraid I was relapsing already, but the doctor said the Prednisone can cause the jittery/fluttery feeling.

I had to get out of the shower and sit on the bathroom floor until my heart rate returned to normal. I then proceeded to cry for a solid ten minutes. Why are even the simplest of things so much harder lately? I don’t want a pity party and I don’t want to whine about how life isn’t fair, because it could always be worse and I’ve been blessed with so much in life, but I just wish I could know what’s normal. I wish I could know how much longer things are going to feel this way. I just want answers to questions that have none. For instance, how does one’s immune system just all of a sudden stop recognizing its own RBCs and platelets? Is there a cause? Is there something I could have done to prevent this? Is there a chance my immune system will ever reverse its decision to hate itself? I want to know more but ES is so rare that there is little to no research on cause, prognosis, or effective treatments. Every patient responds differently to the medications and it is essentially a guessing game – trial and error.

At this point, all I care is that I can get something for this headache because the Tylenol isn’t cutting it.

The Game Plan

Posted on June 21, 20162 Comments

I spoke too soon… treatment will begin tomorrow (Wednesday) at 10:00 AM. I will be at the hematology center for 5-6 hours a day Wednesday, Thursday, Friday, & Monday. On Friday the doctor will check my platelet count to make sure the treatment is working. From what I understand, the IVIG is meant to give a quick boost to my platelets and my numbers should skyrocket.

When I called the hematology office this morning to see if I would be starting treatment today, I was sure to describe my spontaneous bruising. The doctor had his staff call me around 1:00 to come in for blood work. My hemoglobin was up to 11.1! The steroids definitely seem to be working for the RBCs! My platelets, however, were down to 22,000. They dropped 9,000 in five days. Once I noticed the spontaneous bruising, I had a feeling they had dropped, so I’m not all that surprised. The doctor said that since I’m starting IVIG in less than 24 hours he’s not concerned about the drop, but that if any of the following occur I should go straight to the ER:

A large unexplained bruise
Bleeding from the gums
Nose bleed

After IVIG ends on Monday, the doctor is likely to schedule an appointment with me for Tuesday or Wednesday to check my counts and start tapering me off the steroids. I am a little nervous about my numbers dropping once I start tapering off the Prednisone, but the side effects are becoming a nuisance and I can’t stay on the medication long-term. The best case scenario here would be a very successful IVIG treatment, 2-3 weeks of tapering off the steroids and getting regular blood draws, and then remission! If my numbers hold steady as I taper off, I should definitely be set for a while! Maybe then blood draws will only be once a week or once a month! Say a prayer that this course of treatment is going to do the trick!

The Haunting of the Platelets

Posted on June 19, 2016

My low platelet count is starting to haunt me. It is affecting more and more things in my daily life. First, the problem was that I couldn’t commute into the office anymore and had to work from home. Then, I had to start using a soft toothbrush and electric razor. This past week I found out I cannot drink any alcohol until further notice, which is basically all people my age do in social settings. I’m nervous to ask when the alcohol ban will be lifted. I know my health is most important and I truly believe that, but I hate being told I can’t do something, especially with July Fourth and summer right around the corner. Today presented a couple of new challenges. To celebrate Father’s Day, my in-laws and husband and I went to Stony Creek Brewery in CT for a tour and tasting. I had to sit back and watch as everyone around me drank. Luckily, I don’t like beer anyways, otherwise it would have been hard to resist.

When my husband and I got back to NJ tonight I noticed a couple of bruises on my body that weren’t there earlier. I have a purple bruise on my chest and two brown/yellow bruises on my arm. It literally feels like they appeared out of nowhere. I didn’t run into anything or bump myself at all. I tried researching what could have caused the mystery bruises, but the internet told me what I already knew – my low platelets (ITP) caused the bruising. I’m not used to being considered “fragile”. This just proves, though, that even if I am extremely careful and I don’t bump into anything, I am not immune to bruising.

The Diagnosis

Posted on June 3, 2016

My Tuesday morning hemoglobin count was 7.9 and my platelets were back up to 33,000. The threshold they usually use to determine whether or not to transfuse a patient is 8.0. Since I had only been on the three-times-a-day dose of steroids for 24 hours, they decided to wait it out and see what Wednesday’s number looked like. They wanted to give the steroids time to kick in and do their job. Also, there is always a risk with a transfusion, especially when the blood isn’t an exact match. If Wednesday’s number is up then I will likely be able to go home. If Wednesday’s number is down, despite the added steroids, I will have to start IVIG treatment, which is an infusion once a day for three days (this would make my earliest departure Saturday).

Everything came back negative. By process of elimination, it was determined that I have Evans Syndrome. Evans Syndrome is a drop in two of the three blood groups (in my case, red blood cells and platelets) with no underlying cause. The primary course of treatment is steroids. These are used to suppress the immune system and temporarily stop it from destroying healthy cells and platelets. I will likely be on a large dose of steroids for the foreseeable future.

I got to walk around the hospital halls with an aide on Tuesday while wearing a pulse oximeter so they could measure my heart rate and oxygen levels. Luckily, I did so well that they took me off the oxygen mask! As I slept Tuesday night, my heart rate dropped to 45 beats per minute and ICU sent the nurse in to wake me up and make sure I wasn’t crashing. If anything, this just proved that my tachycardia was fading!