This morning I called the hematology center to see if they had any update from insurance. The insurance company had told them it would be an additional 24-48 hours. They had all the paperwork they needed, but claimed it was still in the “review process”. I decided to reach out to my company’s HR Director, who was able to get our broker involved. I heard back from her around 4:30 that the approval went through! I don’t know what she and the broker did to speed things along, but it worked like a charm! I am so relieved to know treatment was approved. By the time the approval was faxed to the office, they were already closed for the day. My guess is that they will see the fax in the morning and immediately call me to come in. It looks like treatment will almost definitely be Tuesday-Friday!
Treatment
The New Hematologist
I decided on Thursday evening, after my rude encounter with the staff at the hematology office, that I needed to find a new hematologist. I know I am going to need to see this specialist for a very long time and I refuse to settle. I want a doctor I can trust. Thankfully, I found that today.
I called the old doctor’s office this morning around 10:30 hoping to get my numbers from last week so I could share them with the new doctor. The receptionist said “oh. Nobody called you?!” and then proceeded to tell me the results were finalized yesterday and I needed to come in right away because my platelets weren’t responding to the steroids. At this point I was fed up so I said I’d call back to schedule an appointment. My hemoglobin was up to 10.4 from 8.7, but my platelets had only gone from 41 to 44. These results were a week old at this point and I was just finding out about them. This only served to solidify my decision to switch hematologists.
I worked from home today because I had an appointment with my new hematologist at 11:30. The office was easy to find and spacious. I filled out some paperwork and was taken in the back around 11:50 to have my blood drawn and my vitals recorded. The nurse took three vials of blood and immediately ran one through a machine that spit out a reading in less than five minutes! Do you know how amazing it was after the fiasco with my original doctor to know my numbers before leaving the office?
By the time the doctor met with me, he already had my counts. My hemoglobin dropped ever so slightly to 10.2. This drop wasn’t that worrisome or significant, but the steroids should have at least kept my numbers stable. He then explained my condition to me and discussed that yes, figuring out a treatment plan is important, but so is taking ten steps back to figure out why this condition started in the first place. Until we know what caused it, it’s very hard to stop it. He then got very serious and said “you might want to conference in your husband for this part”. He proceeded to tell me that my platelet count was at 13. 13?! How did it drop by 31,000 in one week? Seven days?! The thing that REALLY concerned him and, in turn, frightened me is that the number was able to drop so much and so low while on high dose steroids that were meant to do the exact opposite.
Because of my dangerously low count – at 10 and under some people start spontaneously bleeding internally without any cause or underlying injury – he said the first step was to send me to Jersey City Medical Center for an immediate outpatient platelet transfusion. By the time the hospital finished registering me it was past 3:00. The infusion center drew my blood to send to the lab for cross matching. I couldn’t get platelets until I had been matched. Matching takes the lab about an hour and then the transfusion takes about an hour as well. Unfortunately, the infusion center closes at 4:00. The nurses called the doctor to ask if I could do the transfusion tomorrow morning at 8:00 instead and he said that it can’t wait. He told them that if they can’t do it today I would need to be admitted. Getting admitted would suck for two reasons – it would mean staying overnight even though I just needed a quick one hour transfusion & it would mean another $500 admission fee.
Luckily, the lab was able to rush match the blood and had the platelets to the nurses by about 3:45. One of the nurses graciously offered to stay past her shift to administer the transfusion, even though she doesn’t get paid overtime. The platelet bag finished around 4:30 and I was able to head home.
Here is the plan put in place by the new hematologist:
- Look at my blood smear under a microscope to see if there were any Giant Platelets the machine weeded out and didn’t count.
- The platelet transfusion – Check!
- Begin IVIG (pending insurance approval, which takes 24-48 hours). He wants to have me do IVIG four days in a row (excluding weekends). He said it takes about five hours each time and it involves being hooked up to an IV for those five hours.
- See how the IVIG works. If it works, I can begin tapering off the steroids, but he will have to closely monitor my hemoglobin to see if it starts dropping. If the IVIG doesn’t work, he wants to do a bone marrow biopsy to see if my bone marrow has stopped producing platelets for some reason.
He has me out of work until after IVIG is complete. I can work from home, but he doesn’t want me commuting into the city on the train. All I would need is for one person to accidentally elbow me and I could very likely start bleeding internally. Unfortunately, there aren’t usually any visible signs of internal bleeding until it’s too late.
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 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 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.