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Strength In the Moment

Updates for family and friends following my journey with aplastic anemia

Day 4 of ATG

I was beyond exhausted last night through today so didn’t post about last night’s ATG. It was my final round and it has left me very weak. I also have continued to have itchiness issues and my skin looks even worse than it did. ATG runs from about midnight or 1 to 8:30 or 9:00am. As treatments have continued, the more it seems I have at least some allergic response to it (though not enough to discontinue treatment).

I feel like it is finally hitting all of us just how sick I am. I am extremely shaky and even once I am discharged, I will not be able to be Fiona’s primary caretaker right away. That cost makes me sad. I have missed seeing her so much but at least I get to see her for a few hours in the hospital and once I am home, I can at least spend more time with her.

One of the most frustrating things is that I’m having a very minimal response to platelet transfusions. My platelets were 3 today and immediately following a transfusion, I only jumped to 17. This has happened multiple times now. My other test is run at midnight and by then, my platelets are back down to 2 or 3. This means platelets are only lasting about a half day in my system. Compared to the times I was able to hold platelets for a week or more, this feels pretty discouraging. That said, the ATG treatment can cause thrombocytopenia which is just a fancy word for lowered platelets. This means for the foreseeable future, I will require daily platelet transfusions. It’s looking like I may even require daily red blood transfusions as well. I’ll continue to be weak and shaky and I don’t even know if my petechiae will have a chance to heal before I accumulate more.

In other news, I can’t even report on my white count and neutrophil levels because in that respect, the ATG has done it’s job by totally killing off my white cells. It is registering so low in their tests that there’s nothing the machines can report. This means I’ll also be giving myself daily injections to try to boost my white counts.

I don’t know exactly when I will be discharged. We think it will depend how weak I am, how long I hold blood products, and my confidence in being able to move around. Being on a second story apartment may prove difficult and beyond the physical weakness and exhaustion, the medications I am on cause a host of side effects from confusion and deep bone pain to high blood sugar and blood pressure. Whenever I stand up to try to do something, I need help walking and oddly, sound becomes very muffled for me. I get dizzy and I am very slow.

I know many people have offered to come visit and help but with literally no white count, I think we can’t risk it for now. If I haven’t gotten back to you yet, I’m sorry. I definitely will but I fall asleep in the middle of writing this blog and talking to people in person (and even during my regular check-ups like getting my BP and temperature). So, honestly, much communication seems a bit beyond my grasp at the moment.

Thanks for keeping us in your thoughts and prayers and staying up to date on what is happening.

Day 3

I will start with the good news. Today I did not have rigors because they took better precautionary measures to try to prevent it. I was shaky and lightheaded today but overall did much better on that front. Unfortunately, whenever I stand up to do anything, I get faint and shaky. The worst of this happened while my family was here. I was organizing my things when I had to sit down and I had what felt like rigors but they think it must have been something else because it happened so far away from my ATG treatment.

In the bad news category, my pain is still through the roof. I’m pretty solidly anywhere between an 8 and a 10. I think this is due in part to the rigors yesterday and also because I’ve been so weak today that I haven’t done much walking and the bed, while nice enough, really doesn’t do what I need it to with my EDS.

In addition to this, I seem to only be holding platelets and red blood for a day, sometimes even less. The ATG could be the cause of the platelet issues but we don’t know for sure. I had gotten up to 96 platelets my first night, 15 platelets my second, and by 4pm today I was down to 2. I knew something was wrong because I’ve also been having severe itchiness and my body looks like some sort of morbid road map or something from a horror movie. Some of the petechiae have even bled. We do think the itching is being caused by the ATG and it covers my face, arms, chest, stomach, and legs. I have some pictures if people are curious.

My hemoglobin was 7.8ish yesterday. I received blood sometime that day and yet my hemoglobin fell again all the way to 6.8ish. This is the lowest I’ve ever been. Despite this, and despite the fact that the protocols my original doctor has on file say I should receive 2 units whenever my hemoglobin hits 8.1, they follow a different set of rules here so I only ever get one unit at a time and at a lower level. So far that doesn’t seem nearly as effective. My blood levels will be checked again at midnight tonight and I received plateletes and red blood cells today. So we will see where I am.

UPDATE: After one unit of blood and one unit of platelets, my hemoglobin is up to 7.6 and my platelets are 16. They only have me scheduled to get another blood draw at midnight but I’ll probably push for a midday draw since the emerging pattern is that I require blood and platelets on a daily basis.

My blood pressure has been vacillating between being too high and being too low so they’ve been pumping liquids into me pretty much nonstop. I’ve gained a bunch of weight because of it which is discouraging but they are saying it should drop down pretty quickly once I’m out of the hospital and no longer receiving fluids. Problematically, too, the sheer quantity of fluids has made it difficult and painful for me to breath very deeply and I can’t eat much at a time.

They’ve given me something to help me sleep so I’ll leave it at that for tonight. If you are curious about pictures of the petechiae, let me know! I don’t want to put it in the blog post in case they add the picture to my crosspost to Facebook (it doesn’t seem like the  type of thing people would like to jump out at them).

We are about to start my third dose of ATG – wish me luck!

Day 2

I am beyond exhausted so my update will be short. I finished my first ATG treatment from midnight Saturday to 7:30am Sunday. They are giving me a lot of steroids so I was pretty wired and only slept for about 2 interrupted hours. At the very end of the treatment and directly after finishing, I developed terrible rigors. In my case this was accompanied by chills at the beginning.

I spent hours shivering and shaking as we tried one medicine after another to get it under control. I was given more steroids, benadryl, and a whole host of medications that eventually helped but left me groggy and out of it for most of the day. The rigors also tightened all of my muscles terribly and my pain has skyrocketed to well beyond a 10. It has just made managing my pain more difficult and I’m left confused and groggy after some of the treatments they’ve used. I also ended up with a fever of 103 which can be another side effect of the ATG. That is under control now but we don’t know what is going to happen when I start the ATG tonight.

Today was one of the worst days I’ve experienced (aside from labor!). I really hope that my reactions lessens with time. I meant to finish this post before starting my next round but I’m onto my second round and it is taking a lot longer in the hopes that I will do better but they just increased the rate and I’m starting to get itchy everywhere. It is difficult to tell if I have hives because any time I scratch, a new petechiae rash appears. My platelets rose all the way to 96K after two units of platelets midnight Saturday but by last night they were back down to 15K so not only did the petechiae I had before not get a chance to really heal but now I’m getting more and more of it along with random bruising along my veins.

Day 1

Today is a hard day. I arrived at OHSU around noon. So far I’ve had blood work done and one unit of platelets. They’ve also done a skin test to see if I would have an allergic reaction to the ATG. I’ve been waiting forever for a second unit of platelets and then will start the ATG after that which takes at least 4-6 hours and they will be checking my vitals throughout the night. I had hoped to get some rest since I’m not watching Fiona through the night but at least tonight, that won’t be the case.

My platelets were 1K today. This is the lowest I’ve ever been. My hemoglobin is 7.8 which is nearly the lowest and my neutrophil level is .06 (also the lowest I’ve ever been). I’ve been really discouraged by this in part because they are not really wanting me to have visitors outside of my family – especially people with kids in daycare or school. It also feels like my body is just giving up or something which is ridiculous, of course.

With my platelets so low, I have terrible petechiae everywhere and even with one unit of platelets in my system it seems to be getting worse. I also have bruising all over my body. I have huge blood blisters in my mouth and on my lips that are extremely painful and in some cases bleeding. My gums are also bleeding without doing anything to cause it. I’m not allowed to floss or brush my teeth or even use mouthwash. I’ve had some cavities but no dentist wants to touch me with a ten foot pole until my aplastic anemia is under control so I don’t know what’s going to happen with my teeth and now all of a sudden my jaw hurts a lot. When showering, I cannot shave because I could cut myself and the bleeding won’t stop. I can’t use a washcloth because it will give me more petechiae. I can’t use any type of scrubs or abrasive soaps. I have to shower every day with a plastic sleeve over my picc line (today I used a grocery bag and press ‘n seal at home!) and while in the hospital, I will then have to follow a special  cleansing protocol after I shower and change all of the bedding in my room, wear different clothing, etc.

I’m also struggling because tonight is the first night I’m away from Fiona. She was here for only a few hours today and probably I will only see her for a few hours each day because we are trying to keep her on a good nap and sleep schedule. I miss her already. Even though I have to wake up several times a night to feed her, I am sad to be away from home. Unfortunately, Zack will have to work while I’m here and so I won’t get to see him much either. I just want to be with my family.

I am sure things will look brighter tomorrow. I have a nice room and a pretty view. My friends and family are so supportive and kind. I know it will be okay but today is hard.

ATG

Well, good news (I suppose relatively speaking). The bone marrow biopsy has come back and there are no new surprises. No cancer, no other complicated diseases. This means I can begin ATG.

I got the biopsy on Wednesday. They used a drill this time which was a lot less painful and went much more quickly. If I ever need another one, I’m requesting that they do it this way. I also had to get platelets because mine had already dropped to 3K (remember normal is 150K-450K). Unfortunately, after the transfusion, my platelets only went up to 24. I don’t know why this is the case when I usually get a much bigger bump in numbers. I got that transfusion on Wednesday and as of today I already need another one because my platelets are 7K. More than likely, I will get platelets tomorrow when I begin my ATG. I will also need a red blood transfusion soon as well.

I start ATG tomorrow and it will take at least 5 days in the hospital. I’m really struggling with the idea of being away from Fiona for such a long period of time. She will get to come visit me but she can’t really stay overnight and we need to try to keep her on a schedule so she is getting a decent amount of sleep. I don’t think other visitors besides my immediate family will be allowed because the ATG will lower my immune response. I believe that will continue for at least a few weeks after I get home. Once the ATG is over, it will probably take 3-6 months to determine whether it has worked.

That’s all for now since I’m going back and forth between packing, playing with Fiona, and resting.

Updates

Hi Everyone, today I drove into Portland for a Picc Line. This is a central line that threads from my arm to a vein at my heart (medical people correct me if I’ve explained it poorly). It really freaked me out but I barely feel it now.

This is in anticipation of needing ATG starting this weekend and in anticipation of requiring additional transfusions until the ATG works (which could be months).

I am having trouble with the concept of having a central line. I’ve been getting transfusions for 9 or more months now without getting one. Aside from all the annoying things about it like no swimming or baths, and awkward showers, it just feels like giving in to a new reality. It is like getting this line says that I’m going to be sick for a long time. Over the long run I know it will be the better option and maybe I won’t look like a drug addict or abuse victim with all the bruising on my arms. But instead, now I have two IV tubes sticking out of my arm. It feels like this guarantees we’re in for an even longer haul. I know just having a Picc Line doesn’t make that true and I understand it is the practical choice – especially since I’m not a candidate for a port due to my low platelets.

But I’m just very tired. I’m tired of getting a diagnosis and then having it change and I’m tired of waiting for things to work. I’ve tried desperately to not think too hard about my health or how long this has been going on but some days I just get overwhelmed and today has been one of those days.

I find I am stressing out about very small things. I’m upset that my order got lost online and now they are sold out of an item I wanted. I’m stressed that I am missing a sale on clothes for Fiona. I’m stressed about driving into Portland again tomorrow during rush hour. I’m obsessing over these tiny things because I just can’t deal with anything much bigger. When I do start thinking about things, it can be hard to stop.

I’m incredibly nervous about my bone marrow biopsy tomorrow. I’m fine with the procedure though it is painful (IV morphine, here we come!). What has me more nervous is the idea that they could find something they missed or that indicates my disease has progressed in some way. In reality, the doctor doesn’t expect anything different and plans to start my ATG this weekend. But I just keep thinking that this could show something worse.

I just want things to go back to normal. I want to be healthy and doing fun things with my daughter and husband. I want to somehow let go of this weight and fear. I don’t want another biopsy. I don’t want more bad news. I’m tired of feeling like I’m being punished for some cosmic wrong I perpetrated on the universe. Of course I know I didn’t cause this. But it’s so easy to think that way. It’s easier to blame myself than accept that sometimes bad things just happen. It just feels like things are always getting worse though and I would just like to catch an effing break.

Tomorrow I’ll feel better and it will be back to our regularly scheduled programming – staying upbeat and as in the moment as I can for Fiona and my family. Life has to keep going and I want us to have as much normalcy as possible. Sometimes a little denial can go a long way toward surviving the day.

News

Hi everyone, today’s post will be full of new information. Sorry if any of it is long winded or confusing – my red cell count is down again and so I’m pretty exhausted.

In good news, preliminary searches show a few potential matches in the bone marrow registry. This is just a preliminary search and more detailed testing would be done if we definitely move to transplant. For people requesting to be tested, I’ll do a post about that soon.

At my Monday blood draw, my platelets were only 14. My last transfusion had been on Friday so all of a sudden, my platelet transfusions were only lasting a few days instead of a full week. We got set up to do platelets on Tuesday after meeting with my specialist.

On Tuesday, my platelets fell to 4. I got my transfusion and we rechecked today – unfortunately, my platelets are now 11. So we are now in a situation where I need platelets nearly every day. You may recall this has happened before. I restart dexamethasone today in the hopes that it will help me accept platelets again. Last time I got about 2 months out of it. Unfortunately, this is also the medication that landed me in the ER with a terrible migraine. I can’t take any of the usual migraine meds for it either. I take 10 pills of dexamethasone for 4 days and hopefully this will help my platelets hold.

I met with my specialist yesterday. We can see very clearly that the increased dose of promacta is not working. The plan is now to put me on the highest dose possible while simultaneously seeking approval from my insurance company to move forward with ATG. As I mentioned in a previous post, this would involve an extended hospital stay while they give me a serum. They can use either a horse or a rabbit to create the serum and we will start with horse as it tends to be more effective. I would also take cyclosporine and promacta indefinitely. Prior to the ATG I would be on steroids and antibiotics. Many of these medicines cause weight gain. The cyclosporine caused my weight gain over the past few months so I’m really frustrated that I have to go back on it and also go on steroids and a few other things that will cause more weight gain. This weight gain, among other things, has made me feel pretty crummy about myself but that is another post.

In addition to all of this, the specialist has put me on a medication to prevent shingles and is having me get injections of a medicine called zarxio. It is a very short-acting medicine designed to boost my white cell count for 24 hours. I will get these 2-3 times a week (I’m not sure why they wouldn’t do it every day) and the hope is to stave off infection. The bad news is that it causes severe, deep bone pain every time I get an injection.

So that’s basically where things stand. I get platelets, red blood, and the zarxio injection tomorrow so I’ll be at the transfusion center for about 7 hours. Continued thoughts and prayers are appreciated. We are all pretty exhausted.

No Match

We have learned now that my brother is not a match for transplant for me. Lots of people have been asking about whether they can be tested to see if they will match. Initially I was told it was statistically unlikely that anyone I know would be a match but there are 5 million donors in the donor bank. I am meeting with my doctor next week to discuss treatment options. If we end up eventually having to consider a transplant I will ask whether people I know can be tested. If they prefer to just go through the database, then my thought would be, if you feel moved, get put in the donor bank anyways. If not a match to me, you could still help someone like me who is scared and running out of options.

Right now, my doctor wants to begin with a combination of the two drugs that have not worked and ATG which I talked about a little in my previous post. Basically, they inject a horse or rabbit with human white blood cells and then extracting the antibodies produced. It can take up to 3 months to stop the need for transfusions and up to a year to fully work. I would be on medications for a few years at least as we try to reduce the dosage without seeing a relapse. It looks like horse works better than rabbit. I am not sure why one is chosen over the other except perhaps insurance dictates it. I will advocate as much as possible for horse and see what happens.

We are all disappointed that my brother isn’t a match. We had hoped to kind of have that in our back pocket if all else fails. But ATG has a high chance of working and even if I end up needing a transplant, it sounds like it is pretty easy to find a match.

A New Diagnosis

I’ve been trying to work on an update for a while now. The reality of how sick I am is sinking in and that’s made it more difficult to crystallize my thoughts.

I now officially have a diagnosis. What began as just a platelet problem has become a more systemic issue and my red and white counts keep falling too. This means I’ve advanced to something called acquired aplastic anemia. In addition, I have some PNH cells though right now the percentage isn’t clinically significant (I don’t have symptoms of the disease associated with these cells).

I have a new doctor to add to the mix. She specializes in aplastic anemia as well as transplants.

To sum things up:

The first medication, cyclosporine, didn’t work. The second medication, promacta, isn’t yet working. They have doubled my dose and I will be on that for 2 weeks. If it hasn’t worked by that time, they will put me on the maximum dose for 2 weeks. If that doesn’t work, I have 2 options:

1.) We try a combination of the two medicines along with something called ATG. This treatment is actually kind of interesting but a little weird/creepy to me. You should look it up. It’s crazy what they can do. Odds of success are at least 70% (studies show that just cyclosporine and ATG have a 70% success rate and promacta is too new to know how much it will boost those odds).

2.) If Ryan is a match, we could move straight to a bone marrow transplant instead of ATG – we would have some decisions to make about whether to do that. If he isn’t, we definitely would try option 1 first.  If that fails, we move to the donor bank. It turns out blood type has nothing to do with being a match but the doctors said it is statistically unlikely that anyone in my friend group would be a match. That said, you could always be put in the donor bank and if you aren’t a match for me, for very little effort, you could really help someone like me.

Transplant tends to be considered slightly superior to ATG but it terrifies me. I would have one round of a chemo medication and would lose my hair (if it comes to it, I expect you all to shave your heads). I would basically have a baby immune system and surprisingly, Fiona would actually be the safest person for me to be around. I’d be in hospital for a while and then at home would need to somehow stay infection free. The highest risk is in the first 100 days.

In the plus column for a transplant, I wouldn’t have to take the pill that costs $3000+ per month forever (or at least for a few years). It would also eliminate my PNH cells which would remove any risk of developing the disease (the medication for this costs nearly $500K a year for life). In the minus column is rejecting the new bone marrow or getting an infection during recovery.

In the plus column for ATG is that I don’t go through major surgery and recovery. In the minus column, we aren’t sure if my mast cell issues will impact the likelihood of an allergic reaction.

Basically, there is still a lot we don’t know and we have a ton of questions that need answering. We now have three doctors following my case and helping. We don’t know much about how things started but they are guessing I had some kind of immune disorders and pregnancy triggered all of this.

I’m pretty exhausted and just trying desperately to stay in the moment – Fiona’s needs are so moment by moment and so that makes it a little easier to focus on something other than my illness. My brother is still here helping out and that’s the main reason we are eating well and staying sane.

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