I called the hematologist early this morning because I was anxious for my results. The staff said the blood work was still pending and I was being impatient. They had caller ID and knew it was me before I even said a word. They said not to call again – the doctor would call me that afternoon once she had finalized the results.
Articles by Sara Rutherford
The Hematology Consult
The meeting with the hematologist did not go well. Her silent, awkward assistant, who I am not entirely sure spoke any English or understood a word I said, drew three vials of blood from my right arm. Upon pulling out the needle, she didn’t apply any pressure to the entry point and blood started running down my arm. She also didn’t have any gloves on, which was unsanitary for both her and me. In addition, she took a half-assed patient history and had me write down my current prescriptions and medications on what appeared to be a piece of scrap paper. The whole experience was wildly unprofessional.
When the doctor met with my husband and I in her private office she rotated between moments of clarity and completely quirky and inappropriate rants and tirades. Also, my appointment was cut short because the next patient was ready. The doctor’s assistant knocked on the door during our conversation about treatment options and told her my time was up. I’ve never seen a doctor that actually sticks to assigned appointment times or schedules because it often means getting less time with the patients who need a little more.
Here are some things I took away from our brief conversation:
- After being on the steroids for 2-3 weeks, my numbers should be back to normal.
- Hemoglobin: 12-16
- Platelets: 150-450
- Steroids are an effective treatment method for approximately 90% of patients.
- Upon tapering off of the steroids, 35% of patients relapse fairly immediately. Another large percentage of patients relapse 5-10 years down the line. A small percentage never relapse at all.
- She would like me to get weekly blood draws at Quest Diagnostics to monitor my RBC & platelet counts both on the steroids and as I taper off of them
- By next week’s blood draw she should know if the steroids have worked or not
- Before I left I made it very clear that I wanted to know all of my results. She had the receptionist take down my phone number and she said she would call me or text me the next day with the results.
Here are some useless things I took away from my appointment:
- Yoga and proper deep breathing can fix everything
- Once I learn how to properly breathe and exhale, everything else will fix itself
- My body is like a tree and all of my blood vessels and capillaries are limbs coming off the heart. If the trunk of the tree (heart) isn’t receiving proper oxygen it cannot disperse it to the limbs, so they begin dying off.
- My blood vessels work like broken sprinklers. They can only spread blood and oxygen to the little area of grass around them and the ends of the vessels never get any of the oxygen. The sprinkler doesn’t water out that far.
The Overwhelming Realization
How does one find the line between cautious and paranoid? Between enjoying life and just trying not to lose it? I look at everything as a possible symptom. It’s hard to know what was a symptom before and what was just me. I can’t seem to tell them apart anymore and until that gets easier everything is going to appear to be a warning sign. I want to understand what my version of ordinary is going to be. What will my normal hemoglobin and platelet count be? It certainly won’t be between 12-18. Will doctors simply be ecstatic to see a number in the double digits at all? And how long will I be on these steroids? These addictive, angry, sweaty, hungry, appetite-inducing steroids?! What is commuting to work going to be like? Can I be around people at all when my immune system is this suppressed? Am I going to have to wear a mask? Sometimes ignorance is bliss.
The Medications
Thursday, June 2nd I started my medications – 1 mg folic acid, 40 mg Protonix (to aid in the protection of my stomach lining and prevention of ulcers due to the strength of the steroids), and 100 mg Prednisone.
My pee was dark this morning and I started panicking that I was already relapsing. It is going to be hard to tell the difference between symptoms and side effects for a little while as my body adjusts to the medications.
The Discharge
My hemoglobin number went up to 8.7! It was the first time that it increased without a transfusion of any kind. My platelet count was still low, but not dangerous, and 8.7 still isn’t very high for hemoglobin, but my body’s version of “high”/”normal” is going to be different than a healthy person’s.
The doctor cleared me for discharge around 4:00 PM. Earlier in the day even I was switched from “SBA” to “Independent” and was able to go to the bathroom on my own and walk to the gift shop with my mom. By 1:00 they gave me my last round of steroids and then removed my IV. Soon after they took off my portable heart monitor and let me change back into real clothes!
When I was discharged, my urine was still a little orange, but my stomachache, muscle fatigue, and jaundice were gone. We stayed at a local Hampton Inn for the night to relax before driving back to NJ on Thursday. I’ll know more once we see the hematologist on Tuesday! Fingers crossed that the steroids are enough to have normalized my platelets and RBCs.
The Diagnosis
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!
The Second Transfusion
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
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
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.
The Symptoms
Leading up to my ER visit, I had a variety of symptoms that all presented themselves at different times and were seemingly unrelated. All of them could be explained away by outside forces – my crazy diet, moving apartments (lifting boxes and furniture), etc. It wasn’t until I was on my way to the emergency room that I realized everything had been a symptom. My body was shutting down and was trying to warn me, but I didn’t even think twice about it. The dates below are when I first noticed a symptom or something out of the ordinary, but it may have been going on much longer and I was too oblivious to notice.
Tuesday, May 17th
My husband and I signed a lease for a new apartment on May 9th and spent the next couple of weeks packing up our old apartment and driving things to our new place. On Tuesday the 17th, I didn’t think twice about the lower back pain I was feeling. It was fairly intense and I had no history of back pain, but it had been a few years since I’d gone through the stress and effort of moving, so I figured I was just a little sore.
Thursday, May 19th
I had a successful weigh in on my crazy protein-heavy diet and headed back to NJ with my husband. My crossbody purse felt heavy on my chest and I was very short of breath. It was late at night, I’d had a long day, and I have asthma, so I didn’t think much of it. I stopped frequently to catch my breath on the walk home. My chest felt tight, deep breathing wasn’t working, and my heart was racing (tachycardia).
Simultaneously, I was experiencing extreme muscle fatigue. I had only walked a couple of blocks, but it felt like I’d been walking for miles. My arms and legs felt weary and heavy like they were dragging me down. Again, I just assumed I was extremely tired from work and moving.
Saturday, May 21st
My friend came over to help us go through some boxes and make our place feel a little homier. After we returned from a walk around our complex, I started having an awful stomachache and a complete loss of appetite. I had to leave the room when my husband cooked dinner because the look and smell of chicken was making me physically ill. My food aversions only intensified over the next week. It was hard to get myself to eat anything at all and my caloric intake barely hovered around 500 calories. I assumed I was simply sick of the lean proteins I had been eating day-in and day-out for the last six weeks. The only thing I could manage to eat was scrambled eggs.
Monday, May 23rd
I still felt pretty crappy so I talked to some friends at work about my symptoms and they all had the same reaction – sounds like morning sickness. My friend and I bought a test from Duane Reade and set a timer… Negative! Thank goodness! Some of my symptoms just seemed to mirror pregnancy, I suppose.
I came home from work and almost immediately went to bed. I laid down for a “quick nap” around 7:00 PM and was awoken by my husband at midnight telling me I needed to eat something for dinner. Eggs again!
Wednesday, May 25th
My head was throbbing. I have had headaches before and I’ve been known to have the occasional migraine, but this was something different. It felt as though both sides of my head were being squeezed together and I could feel my heartbeat in my ears. I drank some water, chalked it up to stress, and let it go away on its own. That night I thought I had a fever, but we couldn’t find the thermometer, so I cranked up the A/C and tried to sleep through it.
Thursday, May 26th
The headache was back in full force, so I took a couple of Aleve and hoped for the best. It wasn’t until later that day that I got a little nervous. My urine had become a dark orange color. The vitamins I was on were known to make urine very bright/neon like a highlighter, but this was something different. I had no idea that dark urine was a sign of anything, so I didn’t worry about it. I figured I had eaten something weird – maybe something with food coloring in it.
Friday, May 27th
The first thing I noticed when I woke up was that my eyes seemed fairly yellow (yellow sclera). Perhaps I just didn’t sleep well. I forced myself out the door and counted down to the long Memorial Day weekend ahead simply relaxing by the lake.
At work my headache raged again. I took two Tylenol and just hoped to make it through the day. Around lunchtime I noticed that I was sweating profusely. This isn’t unusual for me, though. I tell people, “I sweat when I smile”. It has always been an issue for me. It wasn’t until I came back from the bathroom and my coworkers started telling me I looked pale that I got nervous. I headed to Dunkin’ Donuts and grabbed myself a bagel. I didn’t want to cheat on my diet because I had been doing so well, but I knew I needed to eat something – anything. I scarfed down the bagel and took the train back to NJ.
On the ride to the cottage I napped and tried to ignore my stomachache. It didn’t hurt when I was sleeping, so sleeping was my go-to. We made it safely to the lake and my mother-in-law saw how pale I was and immediately suggested a trip to the ER. I was stubborn and exhausted and just wanted to go to bed, so I forced down a protein shake and agreed I would go in the morning if my symptoms hadn’t subsided.
Saturday, May 28th
I called my aunt, a Nurse Practitioner, when I woke up and explained my symptoms to her. She is always very upfront and practical, especially with medical advice, so I knew when she insisted I go to the emergency room, I needed to go. At the time, I still didn’t realize how serious my symptoms were.