Back in April 2008, our old rheumatologist thought that perhaps Emily could have Scleroderma. I didn't know what it was, but trying to be the informed parent, I promptly went home and looked it up. Then I wished that I hadn't. This was the first "too real" situation that we found ourselves in. Trying to compare diseases with limited knowledge, I thought that she had something called CREST syndrome. "CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome is a member of the heterogeneous group of sclerodermas, and its name is an acronym for the cardinal clinical features of the syndrome." Bottom line? It messes you up.
I learned that there are different types of Scleroderma. There is one type that is called linear; it mainly affects the skin, causing the skin to darken and harden. It can harden to the extent that it can restrict movement. It can cause disfigurement. Then there's Ssc, or Systemic Sclerosis. This is what really scared me. The literal translation for Scleroderma is "stone skin". In Ssc, it can literally harden your internal organs. Think about it- think of things like hardening of the arteries. It can slowly harden your insides. I spent a good two months absolutely sure that this was her problem.... and then we were told that it wasn't. Phew!, right? Ok, so another little gem that I have learned is, just because something is ruled out, doesn't mean it will stay out. Since her old rheumy failed her so badly, we tried to just be more watchful, and of course I started to really research everything. Lord knows, as my wonderful hubby said tonight, if we stayed in his practice she likely would have been dead last year. So, it felt like, if I could just research enough, maybe I could find a clue that other couldn't. Pretty farfetched, yes. But, it helped me to at least feel like I had an active role and I was doing stuff, not to mention educating myself and anyone else that would listen.
We noticed that her wrists had linear scleroderma, and she had the sclerodactyl hands, which is basically when the hands look like swollen sausages, and of course the Raynaud's was severe. When we learned for sure that she tested positively for Mixed Connective Tissue Disease, I learned that MCTD can stay the same, go into remission, or it could morph into either Lupus or Scleroderma. I think I have always seen more Scleroderma features with her than anything. I decided that I wasn't going to worry about it until we had to, which we hadn't had to do.
That said, Monday we discussed her test results from the chest CT, Echo, swallow study, and whatever the heck else we did. If you read my post after we did the swallow study, you may remember that I was able to watch the test being done myself, and I saw problems. The doctor that ran the testing and the 3 techs that were present didn't know what to do. They called in another doctor for guidance. The problem was that the barium dye wasn't going away. They sat her up, laid her down, turned her side to side, but it wouldn't fully leave her esophagus. Well, apparently her esophagus has narrowed down near her stomach. Not much is able to get through, leaving her feeling nauseous and refluxing. She is barely able to eat. While I knew that there were problems here, I really thought that it would be the muscles from a Dermatomyositis flare, which attacks the muscles of the throat so they are too weak to work properly. This is more of a Scleroderma feature. That threw me for a loop.
While I was still reeling from that, he pulls up pictures of her lungs. He shows me that they can see changes around the base of the lungs. They look different from her last few tests. While it doesn't show that she has Interstitial Lung Disease, her lungs are likely trending that way. Pulminary Fibrosis is one of the hallmarks of MCTD and Scleroderma. I don't remember a whole lot of the rest of the day. I focused a lot on trying not to cry.
So, the new plan is to stop her Methotrexate. She has not been a fan of that one lately, anyway. In place of MTX, we are switching her to a stronger medicine called CellCept. The hope is that, if we hit it hard enough, it will bounce back down. Like, if you start to treat a flare at the beginning, it will usually respond much more quickly than if you let it go. This is similar in theory. I can't even find any info on this one that doesn't pertain to transplant patients. It has a ton of warnings. It does not look like a fun med. Yeah, and because it causes such a high risk of infections, she may have to start a sulfa antibiotic daily with it. It sounds terrifying. And yet, what choice do we have?
We have a dear friend who is very similar to Emily. We heard tonight from another dear friend that friend #1 has ditched most of her meds and is working on a holistic approach. I have heard that she is doing very well this way. I don't even know where to start, but I will ask them about it a bit. It may be a while before we can afford something like that, but I will look into it.
The funny thing is that Emily didn't even have an appointment this time. We discussed all of this in Zachary's appointment. The bad side of that is I don't think we really talked much about Zachary. Maybe we did and I missed it... I don't know. The only thing that I clearly remember is that I asked if his white patches will go away from where his old psoriasis lesions were. I had hoped they weren't Vitiligo. I was assured that they are not; it simply takes a while for the skin to go back to normal since he had so many lesions and for such a long period of time. And I got a new MTX prescription for him. That's all I remember there.
So, here we are again in territory that none of us is comfortable with, that makes it all too real. I try so hard to be positive, but I have to be real, too. People need to know that these diseases are out there, and they are scary and life-threatening. I am thankful that we are homeschooling this year. At least she won't pick up any nasty bugs at school. School stuff will be on the Homeschooling blog. A new post should be up this week!
Tuesday, August 28, 2012
Friday, August 3, 2012
Tough day
While most days I can manage to get through without seriously contemplating the kids' medical issues, for whatever reason, the drive to and from the hospital is where I really feel the stress of the situation. Even now with Em having a port, it is easier but once we're there and ready for them to access she really panics. Just thinking about the fact that they need to access my child is freaky, isn't it? I guess it's because going makes it all more real. Funny, really, since I'm with her when she's miserable or can't walk too, but that isn't as real as hanging at the hospital. Even now I have days where I mourn for my sweet tiny little bean pole that isn't anymore, thanks to steroids. It seems awfully unfair sometimes, but we move on.
This trip started with the Pulminary Function Test. Now that it doesn't scare her, she actually enjoys it. I thinks she's a little crazy but hey, at least she does it without trauma. She did very well doing the test, & while the doctor hadn't seen the final report, he said the preliminary report looked great. The tech scared me a bit; he asked me with a concerned tone in his voice if we were seeing her doctor that day. It was the way he said it that made me think, "uh-oh", but it really does seem to be ok.
Later, things were okay in the infusion room after she was hooked up. She screamed bloody murder first, but she calmed down soon after the needle went in. I guess she will always have major anxiety. I really had hoped that it would get better, but it just doesn't. She screams like they're trying to kill her- flailing her arms and all- until the needle is in; then she relaxes. She got her Orencia first, soon followed by her Rituxan. That is a huge relief since Rituxan is what helps her the most. I finally got to speak with someone about scheduling the swallow study. Naturally when they called me back, Em was screaming and I missed the call, but I managed to get them while still in the infusion room. They wanted to schedule the study for the 17th, when we already have appointments up that way on the 21st & the 27th. If they weren't 2.5 hours away that would be fine. She hung up & called me back to tell me that they could do the swallow study while she is inpatient, but it needed a different referral than the outpatient one. That led to another round of calls because the check-in desk at the hospital doesn't document the way that the other staff does, so I actually had to call the doctor's office even though she sees them there. The funny thing is that not long after the person I left a message for found us in the infusion room. She said she'd find out & let me know what was up. That's the last time I spoke with her. That's cool- as long as it gets to the doctors I don't care. Long story short, they scheduled it for Thursday. That would have worked much better if pharmacy had sent her pre-meds up a few hours earlier. Instead of starting her IViG around 6, it was started at ten.
The plan was to finish the IViG as inpatient, then move to the infusion room for Remicade, while hopefully squeezing in the upper GI somewhere. Eventually they got her scheduled for her testing at two. They also ordered an x-ray for her arm because for once, Ms. Careful fell out of bed onto her arm! I really thought that she broke it. She never gets this upset! And yes, I was pretty ticked off about that. She fell while leaning out of her bed to pick something up off of the floor. I had just told her that if she needed anything to let me know, and the nurse had just walked out of the room. She'll make sure to ask next time, I'm sure.
So they tell me that the plan was upper GI & x-rays at two, then Remicade. I pointed out that this would allow her 2 hours for a five hour infusion. (4 for Remicade, one for Solumedrol.). That is how we dropped Remicade & decided to go back to Enbrel. Yeah. Personally, I think it's a better move. I don't think Remicade has been as good for her as Enbrel was so I'm good with that. And we had talked about dropping it after if the Orencia worked for her, but we still have another month or two before it would be considered fully in effect. That's why the Enbrel is back- probably. He wants us to wait to see if she will really need it. Again, I'm good with that. Enbrel seemed to do more for her before anyway, but also this will allow us to go back to simple 5 hour infusions on the months that she doesn't need Rituxan. That is awesome because staying overnight isn't really too much fun for anyone. Benlysta was also thrown at me as a replacement for Rituxan. I have read up quite a bit, and while I still have a lot more research to do, I think that we will stay with Rituxan. Rituxan is an awesome medicine for her. She feels it wearing off about a week before it is due, which is why they discussed switching. From what I've read, people that do extremely well on Rituxan don't seem to do nearly as well on Benlysta. It's like a downgrade. Why go backwards?
And then there was the upper GI. She totally freaked out for it. She wanted x-rays of the arm so she was perfect for that but she was scared to death for the rest. The doctor wanted to reschedule but I begged them to do the test. She just got herself so wound up. I knew that if we put it off this would only happen again, but probably worse. I know how she is, and she would be thinking about it every day for the next month until it happened, and then she would have had more time to make it worse in her head. It needed to happen today for that, because she's been having a hard time eating lately, and because I feel that she's starting a Dermatomyositis flare. I've seen the red dots reappear, I know she's had some muscle aches lately. Knowing that she had the "air-filled esophagus" on the chest CT at least helped me to prepare for this.
So, I don't have any official results yet, but what I can tell you is that there is something going on. For those that haven't done this before, all that she had to do is swallow strawberry flavored barium while under a big camera. The camera is like a video camera; they can watch the barium flow through her esophagus and into her stomach. The problem is that it really didn't. Yes, some flowed down, but quite a bit didn't. And some of what did go down passed out of her stomach, but much of that stayed, too. They called in an additional doctor to ask what to do. In the end, we waited about ten minutes and they took one last picture of the barium that was still in her esophagus and stomach. Is it from JDM, MCTD, possibly Scleroderma or from something different? Not a clue. I hope to find that out soon, along with what to do about it. I'm just so glad that we did the test today. I just knew that something was wrong. I really hope that we don't have to go back up on her steroids. That would really kill us.
We were told that we could go home right after, but we waited around to make sure that the x-rays didn't show a break. I asked them to give her some pain meds, so they gave me a prescription. Since they wrote for something that my CVS doesn't usually have, I filled it there. They told me 30 minutes. No worries. After 45 minutes of waiting, I asked them if there was a problem. They didn't have the correct insurance info right there. So, instead of calling the cell phone number that I wrote on the scrip, they figured they would just wait until I wandered over there, then they told me it would be another ten minutes. I was so mad! I didn't say anything but I was fuming. We ended up getting our meds as we were leaving, with me pushing her chair and carrying a suitcase, with 2 pillows and a bunch of our bags hanging from the chair. (Laptop, sleeping bag, blankets, etc...) I was not happy. But we're home. I am so glad that we're home. Hopefully we will find out more soon. I will keep everyone posted. Prayers would be wonderful, please. Especially for her throat issues and for no flare to hit. Thank you to anyone that prays for us. I am off to dreamland now. I will leave you with the words to a Natalie Grant song that Emily loves off of Natalie's "Relentless" album. The part that I have in bold I just can't even sing. It tears me up every time.
"Our Hope Endures"
You would think only so much can go wrong
Calamity only strikes once
And you assume this one has suffered her share
Life will be kinder from here
Oh, but sometimes the sun stays hidden for years
Sometimes the sky rains night after night
When will it clear?
But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged
How do we comprehend peace within pain?
Or joy at a good man's wake?
Walk a mile with the woman whose body is torn
With illness but she marches on
Oh, 'cause sometimes the sun stays hidden for years
Sometimes the sky rains night after night
When will it clear?
But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged
Emmanuel, God is with us
El Shaddai, all sufficient
We never walk alone
And this is our hope
But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged
This trip started with the Pulminary Function Test. Now that it doesn't scare her, she actually enjoys it. I thinks she's a little crazy but hey, at least she does it without trauma. She did very well doing the test, & while the doctor hadn't seen the final report, he said the preliminary report looked great. The tech scared me a bit; he asked me with a concerned tone in his voice if we were seeing her doctor that day. It was the way he said it that made me think, "uh-oh", but it really does seem to be ok.
Later, things were okay in the infusion room after she was hooked up. She screamed bloody murder first, but she calmed down soon after the needle went in. I guess she will always have major anxiety. I really had hoped that it would get better, but it just doesn't. She screams like they're trying to kill her- flailing her arms and all- until the needle is in; then she relaxes. She got her Orencia first, soon followed by her Rituxan. That is a huge relief since Rituxan is what helps her the most. I finally got to speak with someone about scheduling the swallow study. Naturally when they called me back, Em was screaming and I missed the call, but I managed to get them while still in the infusion room. They wanted to schedule the study for the 17th, when we already have appointments up that way on the 21st & the 27th. If they weren't 2.5 hours away that would be fine. She hung up & called me back to tell me that they could do the swallow study while she is inpatient, but it needed a different referral than the outpatient one. That led to another round of calls because the check-in desk at the hospital doesn't document the way that the other staff does, so I actually had to call the doctor's office even though she sees them there. The funny thing is that not long after the person I left a message for found us in the infusion room. She said she'd find out & let me know what was up. That's the last time I spoke with her. That's cool- as long as it gets to the doctors I don't care. Long story short, they scheduled it for Thursday. That would have worked much better if pharmacy had sent her pre-meds up a few hours earlier. Instead of starting her IViG around 6, it was started at ten.
The plan was to finish the IViG as inpatient, then move to the infusion room for Remicade, while hopefully squeezing in the upper GI somewhere. Eventually they got her scheduled for her testing at two. They also ordered an x-ray for her arm because for once, Ms. Careful fell out of bed onto her arm! I really thought that she broke it. She never gets this upset! And yes, I was pretty ticked off about that. She fell while leaning out of her bed to pick something up off of the floor. I had just told her that if she needed anything to let me know, and the nurse had just walked out of the room. She'll make sure to ask next time, I'm sure.
So they tell me that the plan was upper GI & x-rays at two, then Remicade. I pointed out that this would allow her 2 hours for a five hour infusion. (4 for Remicade, one for Solumedrol.). That is how we dropped Remicade & decided to go back to Enbrel. Yeah. Personally, I think it's a better move. I don't think Remicade has been as good for her as Enbrel was so I'm good with that. And we had talked about dropping it after if the Orencia worked for her, but we still have another month or two before it would be considered fully in effect. That's why the Enbrel is back- probably. He wants us to wait to see if she will really need it. Again, I'm good with that. Enbrel seemed to do more for her before anyway, but also this will allow us to go back to simple 5 hour infusions on the months that she doesn't need Rituxan. That is awesome because staying overnight isn't really too much fun for anyone. Benlysta was also thrown at me as a replacement for Rituxan. I have read up quite a bit, and while I still have a lot more research to do, I think that we will stay with Rituxan. Rituxan is an awesome medicine for her. She feels it wearing off about a week before it is due, which is why they discussed switching. From what I've read, people that do extremely well on Rituxan don't seem to do nearly as well on Benlysta. It's like a downgrade. Why go backwards?
And then there was the upper GI. She totally freaked out for it. She wanted x-rays of the arm so she was perfect for that but she was scared to death for the rest. The doctor wanted to reschedule but I begged them to do the test. She just got herself so wound up. I knew that if we put it off this would only happen again, but probably worse. I know how she is, and she would be thinking about it every day for the next month until it happened, and then she would have had more time to make it worse in her head. It needed to happen today for that, because she's been having a hard time eating lately, and because I feel that she's starting a Dermatomyositis flare. I've seen the red dots reappear, I know she's had some muscle aches lately. Knowing that she had the "air-filled esophagus" on the chest CT at least helped me to prepare for this.
So, I don't have any official results yet, but what I can tell you is that there is something going on. For those that haven't done this before, all that she had to do is swallow strawberry flavored barium while under a big camera. The camera is like a video camera; they can watch the barium flow through her esophagus and into her stomach. The problem is that it really didn't. Yes, some flowed down, but quite a bit didn't. And some of what did go down passed out of her stomach, but much of that stayed, too. They called in an additional doctor to ask what to do. In the end, we waited about ten minutes and they took one last picture of the barium that was still in her esophagus and stomach. Is it from JDM, MCTD, possibly Scleroderma or from something different? Not a clue. I hope to find that out soon, along with what to do about it. I'm just so glad that we did the test today. I just knew that something was wrong. I really hope that we don't have to go back up on her steroids. That would really kill us.
We were told that we could go home right after, but we waited around to make sure that the x-rays didn't show a break. I asked them to give her some pain meds, so they gave me a prescription. Since they wrote for something that my CVS doesn't usually have, I filled it there. They told me 30 minutes. No worries. After 45 minutes of waiting, I asked them if there was a problem. They didn't have the correct insurance info right there. So, instead of calling the cell phone number that I wrote on the scrip, they figured they would just wait until I wandered over there, then they told me it would be another ten minutes. I was so mad! I didn't say anything but I was fuming. We ended up getting our meds as we were leaving, with me pushing her chair and carrying a suitcase, with 2 pillows and a bunch of our bags hanging from the chair. (Laptop, sleeping bag, blankets, etc...) I was not happy. But we're home. I am so glad that we're home. Hopefully we will find out more soon. I will keep everyone posted. Prayers would be wonderful, please. Especially for her throat issues and for no flare to hit. Thank you to anyone that prays for us. I am off to dreamland now. I will leave you with the words to a Natalie Grant song that Emily loves off of Natalie's "Relentless" album. The part that I have in bold I just can't even sing. It tears me up every time.
"Our Hope Endures"
You would think only so much can go wrong
Calamity only strikes once
And you assume this one has suffered her share
Life will be kinder from here
Oh, but sometimes the sun stays hidden for years
Sometimes the sky rains night after night
When will it clear?
But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged
How do we comprehend peace within pain?
Or joy at a good man's wake?
Walk a mile with the woman whose body is torn
With illness but she marches on
Oh, 'cause sometimes the sun stays hidden for years
Sometimes the sky rains night after night
When will it clear?
But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged
Emmanuel, God is with us
El Shaddai, all sufficient
We never walk alone
And this is our hope
But our Hope endures the worst of conditions
It's more than our optimism
Let the earth quake
Our Hope is unchanged
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