Hemolytic Anemia | Me, Myself, and Evan's

The Two-Week Platelet Check

Posted on July 11, 2016

This morning I had a 10AM appointment with the hematologist. As per usual, the nurse began by drawing blood – two vials – and putting me in an exam room to wait for my turn with the doctor. The doctor started by saying that he intends to disregard the LabCorp results from last week. He said blood should be tested as quickly as possible otherwise it has time to pool and clot, which depletes the platelet count. Since the LabCorp in Saratoga had to send the vial to NJ for testing, the sample was likely compromised.

Today’s hemoglobin held steady at 11.5 and the hematologist said he considers my RBC problem resolved and in remission! Obviously the number will still be monitored as I taper off the steroids but he seems very confident that it will stay at 11.0 or higher and that will be my version of “normal”. The hematologist said that, unfortunately, until I’m off the Prednisone, he won’t know what my “normal” platelet count will be. My guess is somewhere between 60 & 80, but I honestly have no idea. People with ITP are known to have a lower platelet count than the average human, but where exactly that count falls is different in each individual. Some people hover around 20 as their version of normal, but I’m hoping my normal will be a higher, safer number. Time will tell!

Today’s count was 96, so if we ignore last week’s skewed results and look at the bigger picture, my count Monday the 27th after 3 days of IVIG was 159 and my count two weeks later on the 11th was 96. My count only dropped 63,000 over two weeks – I don’t think that’s too bad! Going forward, as long as it drops at a slower rate or stabilizes completely, I should be fine! I have a good feeling about this! Maybe I’ll be completely off the Prednisone by the end of the summer and I won’t require any further treatment! That would be ideal!

The doctor’s plan is to taper the Prednisone about 20% at a time. That means the taper that starts tomorrow should technically be 60 mg but they don’t make pills in that dosage so he’s tapering me to 50 mg. Here’s to hoping for little to no side effects. Since the first taper and the IVIG occured simultaneously, it was hard to tell which side effects were due to the treatment and which were due to the lower dose of steroids.

When I was asking the doctor my questions today I happened to mention my frequent heart palpitations, shortness of breath, and inability to exert myself (walk up the stairs, shower, put on pants, etc.). Originally, a few weeks back, when I expressed this same concern, he said it was due to the Prednisone, which can make patients feel anxious and jittery. When he found out today that my frequency of episodes had increased to daily or twice a day, despite the decreased dose of steroids, he became a little concerned. He said that shouldn’t be happening – the episodes shouldn’t be that frequent and they should have decreased when the dose did. As a precaution, he is sending me for an echocardiogram (an ultrasound of my heart). I know it’s just to double check that everything is okay with my heart, but I’m still a little nervous – not for the procedure, but for the results.

The only thing that has been keeping me sane these last six weeks is knowing that my condition had nothing to do with my weight. It’s unrelated to my bad eating habits, my lack of exercise, or the extra pounds I’m carrying around. Knowing that Evan’s Syndrome has literally nothing to do with outside forces and is completely random and unable to be prevented would probably worry and terrify most people. But me? It makes me feel relieved – relieved knowing that I couldn’t have done anything to stop this from happening. I couldn’t have prevented Evan’s Syndrome by eating more vegetables or running or weight lifting. I would much rather “fate” be to blame. That gives me peace of mind in a twisted way.

That is also why this ultrasound is so terrifying. It reopens the possibility that I am the enemy. I am petrified they will find something wrong – a murmur, heart disease, heart failure, etc. – and will determine the cause to be weight-related. I was fine with these episodes happening when I could chalk it up to the Prednisone, but if suddenly I have to face the reality that my habits and way of life caused these symptoms, I might lose my mind.

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 Second Transfusion

Posted on June 3, 2016

Monday morning they drew multiple vials of blood. They, of course, wanted to check my numbers, but also wanted to start ruling out certain illnesses and determining the underlying cause of my anemia. They checked for various things, including low iron, vitamin B12 deficiency, rheumatoid arthritis, hepatitis, lupus, and HIV. They tried to determine if my antidepressants or birth control could be causing a problem.

My hemoglobin was down to 5.9 overnight. The doctor wasn’t super concerned because they had only expected it to hit 6.2 the day before, but they upped the steroids to three times a day and scheduled another two units of blood to be transfused. My mom and dad hopped in the car and rushed back to Saratoga. Luckily, the transfusion went well again and my numbers an hour later were at 8.9 (another three point jump)!

The hematologist had the nurses put foot compressors on my feet to keep my blood flowing and prevent clotting. This was due in part to the fact that my platelets dropped to 27,000. Also, I was switched from “wheelchair to bathroom” to “standby assist”, meaning someone only had to walk me to and from the bathroom and not wheel me there! I was able to sit up without being out of breath and now we just had to wait to see what Tuesday’s numbers and test results brought!

The First Transfusion

Posted on June 3, 2016

With a preliminary diagnosis of Hemolytic Anemia, there were still a large number of unanswered questions. The most important thing, regardless of the cause of the anemia, was to figure out my blood type and get a match from the local blood bank so that I could receive a red blood cell (RBC) transfusion. My cells needed a boost. The initial lab work showed that I was A Positive, but that my blood had multiple antibodies in it, making my blood type rare. This meant the lab had to draw another vial to be sent to the Red Cross in Rochester for further testing. A courier literally drove to the hospital in a van to collect my sample and drive it the four hours to Rochester.

While waiting for a transfusion, the lab set out to find the cause of my hemolytic anemia. There are three causes for low hemoglobin: stunted creation of red blood cells, premature destruction of red blood cells, and internal bleeding (excessive loss of red blood cells). Creation of RBCs happens in the bone marrow. If creation is low, treatment usually involves a bone marrow biopsy and eventual transplant. Thankfully, in my case, my lab work showed that my young red blood cell (retic) count was high. My bone marrow was working overtime to try to get enough red blood cells into my bloodstream. Unfortunately, my body was destroying these red blood cells faster than my bone marrow could produce them.

A transfusion was the only option. I was admitted to the hospital around 4:30 PM on Saturday, May 28th and it took until the morning for a transfusion. During that time, my symptoms grew increasingly worse. My platelet count dropped to 33,000 and my hemoglobin dropped to 4.2. I officially had about one-third of the amount of red blood cells that I should have. I didn’t know it at the time, but my aunt and mother, who drove up Saturday evening and spent the night at the hospital, were very nervous about me even making it through the night. A code went off in another patient’s room and my aunt jolted up thinking the worst. The doctors talked about moving me to the ICU instead of the cardiac ward, but I wouldn’t have been able to have nearly as many visitors. I was glad I didn’t know the severity of my condition at the time. It would have made my 18-hour wait for a transfusion even more excruciating.

Saturday night was a struggle. Anytime I had to use the bathroom, I needed a nurse to wheel me into the bathroom in a wheelchair, wait outside the door, and wheel me back to bed. This was because of my oxygen levels and tachycardia. Also, I only got shakier and more jaundiced through the night. The reason my levels dropped to 4.2 by morning was because I had been given three IV bags of fluids and all of the fluid was diluting what good blood I did have left.

My blood type was rare and they didn’t have time to check out-of-state for a perfect match so they pumped two units of the “least incompatible” blood into my system. The transfusion took about two hours per unit. Thankfully, I didn’t have any of the side effects that can come with a transfusion, probably due to the other medications they had me taking to prevent such a reaction: folic acid, Tylenol (to reduce the risk of fever), steroids (to suppress the immune system and keep it from killing these new cells), & Benadryl (to reduce the risk of an allergic reaction).

The doctor expects that with each unit of blood that is transfused, a patient’s hemoglobin will go up one point. An hour after the transfusion, it should have been at 6.2, but was instead at 7.2! Hooray! My aunt and my mother helped me freshen up, got me some food, and then drove back home Sunday night. As long as the steroids were keeping my numbers steady in the morning, I was good to go!

Sunday night I was still being carted to the bathroom in the wheelchair, which turned out to be a good thing, since it meant the nurse knew where I was at all times. I got up to go to the bathroom and while I was in there, my heart rate spiked to 150 and ICU (the team that was in charge of my portable heart monitor and tracking my heart rate) called the nurse in a panic. Luckily, she knew where I was and was able to get to me and make sure I was okay.

The only reaction I was having from the steroids on Sunday was extreme hot flashes and sweating. The nurse helped arrange ice packs around me like an igloo so I could sleep comfortably and my husband stayed with me until I fell asleep.

The Emergency Room

Posted on June 3, 2016

My husband, my mother-in-law, and I arrived at the Saratoga Hospital Emergency Room around noon on Saturday, May 28th. The minute I mentioned chest tightening as a symptom, they whisked me back to Triage 1 and immediately hooked me up to an EKG. I think it is the fastest I have ever been seen in the ER.

Thankfully, the EKG showed that the strain wasn’t on my heart. They walked me back to a private room where an ER nurse took my vitals and started me on IV fluids for dehydration. She also drew blood for the lab and asked for a urine sample. The bathroom was literally two doors down from mine and I had to stop twice to catch my breath. When I returned from the bathroom she checked my vitals again and informed me my oxygen saturation was low and my heart rate increased 40% when I stood up (tachycardia). I was put on in-room oxygen while I waited for someone from radiology to wheel me in for a chest x-ray.

The x-rays showed no spots/shadows on my lungs, no clots, and no concerns. Whew! By the time I got back from radiology, the phlebotomist was waiting to take more blood. The first round had hemolyzed because it was taken through the IV instead of directly from the vein.

After the phlebotomist finished, the PA told me I would be getting three CT scans (chest, abdomen, pelvis) to check for signs of internal bleeding or clotting. Two nice ladies wheeled me to the machine and informed me that, if I consented, my abdomen CT would be with contrast to better highlight blood vessels and tissues and check for tears or bleeds. I’m happy the technician vividly and accurately described all of the strange sensations the iodine injection would make me feel, otherwise I am 100% positive I would have thought I soiled myself. The scans showed an enlarged spleen and liver, but, thankfully, no internal bleeding!

When I returned back to my room, the results of the urine sample and blood draws were back. The PA explained to me that my liver enzymes were slightly raised, my bilirubin count was high, and my hemoglobin and platelets were both extremely low.

Important definitions

Hemoglobin is the protein molecule in the red blood cells that carries oxygen to tissues and returns carbon dioxide from the tissues to the lungs to then be released. An average hemoglobin count is between 12 and 18 (usually 12-16 for females). My count was at 5.3. My baseline blood work from six weeks prior, when I had first started the diet, showed a count of 10.7.

Bilirubin is an orange-yellow substance made during the breakdown of red blood cells. It passes through the liver and is eventually excreted. A high bilirubin count can indicate an increased rate of destruction of red blood cells, which is also called hemolysis. My raised bilirubin levels were the cause of my dark urine and jaundice.

Platelets are cells that travel around the bloodstream and bind together to repair broken blood vessels. When your platelets are low you are at a higher risk for internal bleeding and blood loss because your body cannot form clots. A normal platelet count is 150,000-450,000 per microliter of circulating blood. My platelet count was 39,000. My baseline blood work from six weeks prior showed a count of 43,000.

So what does all this mean? My numbers were low – too low – and I was admitted with jaundice and a preliminary diagnosis of hemolytic anemia.