Disease progression despite so many meds...

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!






          

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

Quiet time

It's been a good week for me and Zach, but a rough one for Emily. Monday her shoulder and ankle hurt. By Tuesday, despite several rub downs, BioFreeze and a hot bath, the shoulder was really bad. Her occupational therapist rubbed it out. Em cried, she begged her to stop. We all knew that the benefit would outweigh the pain. Sure enough, she woke Wednesday able to move it with little pain. Her knees have been "tired" and her thighs have hurt. The thighs worry me the most. The thought of her JM coming back sends fear ripping through us all. Not as badly this time. The storms that we had in June threw our monthly med schedule off. While 2 of her meds are monthly, Rituxan is every 3 months. So far, it has been the drug that helps her the most. We would normally have gone for meds this week, so I'm sure that is why she is hurting.

We had placement testing yesterday for school. She got lucky; she wasn't entered into the computer yet, & the person that normally does it was in a meeting 2 hours away. She will be able to take it at home soon. We need to set up an IEP meeting for her before she's 100% clear. Zach is all set, complete with passwords for the online part.

Em has been determined to go to the beach for a while. Where our summers usually have a brief daily storm & usually drought levels, this year we have had more rain than we can believe. We finally got to the beach this evening. With her JM, a sunburn can cause her disease to flare, so we go when the threat is less. I'm sitting here at the beach now, watching them play, watching the sun go down. There is a constant breeze as I listen to the crash of the waves and the kids playing. It is a perfect end to my week. She's feeling good today, and the kids are so happy here.

Emily & Zach have such a special relationship. I love sharing their lives with them. I am in awe of them. I kept catching them hugging each other. So much love!

Oh, & on a last, different note, I am so proud of them. They have been sitting in my church services with me. While some adults may talk all through the service, my kids are clasping their hands & bowing their heads in prayer. I am just so proud of the people they are becoming. I am so ready to start homeschooling! I am so blessed to have this opportunity to be with them more. <3

The Misconceptions of Juvenile Arthritis- as written for my Comp class

The Misconceptions of Juvenile Arthritis

Approximately 300,000 children are affected with  forms of autoimmune arthritis that are clumped under one umbrella term, juvenile arthritis.  These different diseases cause pain, stiffness, rashes, fever, and inflammation of the joints and internal organs.  Some forms also destroy muscle tissue.  They can be deadly without proper treatment.  These diseases do not get very much awareness, despite having more children affected than children stricken with muscular dystrophy, sickle cell anemia, and cystic fibrosis combined. (Dr. Manny Alvarez)   One form, Dermatomyositis, is covered under the arthritis umbrella and is a form of muscular dystrophy.  Many believe it is because of the word “Arthritis” in their label that these diseases do not tend to be taken seriously by the public.  There are many misconceptions associated with these debilitating diseases, as well as a negative stigma surrounding those affected and their families.  Even children feel the stigma.  Careful examination of the facts will show that many of these misconceptions are inaccurate.  My goal is to raise awareness for these horrific diseases while dispelling some of the myths.  

A recent research study  written by A. Tong called “Children’s experiences of living with juvenile idiopathic arthritis” that was based on records of 542 children proves that many of the children feel misunderstood,  stigmatized, out of control of their bodies, and they are in overwhelming pain much of the time.  As the parent of two children with arthritis, I agree with this research.  What this research does not tell you is how misunderstood the diseases themselves are.

Why is there a negative stigma?  One problem is that people do not believe that children get arthritis. When people hear the word arthritis, many tend to think about their grandparents who may have severe osteoarthritis.  A larger problem is that many commercials for over-the-counter products ranging from oral pain relievers like Tylenol Arthritis to topical creams and lotions tout that these products work for arthritis.  The truth is that they really only work for specific types of arthritis.  Most of these commercials, as well as commercials for prescription medicines like Enbrel, show older people walking along, smiling, and doing every day, normal things.  These advertisements do not accurately portray most of these diseases that are autoimmune, nor do they show children in them.  If people don’t see proof in advertising, how would they know that kids get arthritis, too?  To quote Tiffany Westrich, founder of  the International Autoimmune Arthritis Movement from an article on HealthCentral.com , “So what is contributing to this gross misunderstanding?  Look around you.  It's everywhere.  It's on our televisions, it's in our print materials and it's on our computer screens.  The best way to stop it is to change what is publicized in the media.”   People will never understand that there are different forms of arthritis that are autoimmune, or that they can affect children until advertising changes are made.

Osteoarthritis is what the older people generally have.  According to Tiffany Westrich, “The truth is Rheumatoid Arthritis  is a systemic disease that affects joints, connective and soft tissues, muscles and sometime organs, while Osteoarthritis only affects the weight bearing joints.”  While they have not isolated an exact cause, researchers do know that Rheumatoid Arthritis, Lupus, Scleroderma,  Mixed Connective Tissue Disease (an overlap syndrome, similar to Lupus), Psoriatic Arthritis, and Dermatomyositis are all autoimmune in nature.  All of these can affect children, but the medical community clumps children under the diagnosis of “Juvenile Arthritis”.   It is possible for some children to have several different types overlapping, such is the case with my daughter.  Yet, when people hear that you have a child with arthritis, the reaction is generally disbelief.

Despite the belief that people with arthritis have poor diets, while some people with forms of arthritis have food sensitivities that can cause reactions severe enough to mimic arthritis, most people do not benefit from changes in diet.  There have been numerous studies based around diet and rheumatoid arthritis, but there is no proven link.  According to the Arthritis Foundation and Dr. Yukiko Kimura in a Fox News report called “Arthritis in Children”, a healthy diet is necessary to keep the child healthy, but there is no diet that will significantly help or “cure” arthritis.  Staying away from inflammatory foods will help a bit, but it is far from a cure.  The most important diet related item for these children would be additional vitamin D as most people in this category are vitamin D deficient.  Children on steroids for long periods of time benefit greatly from a calcium supplement because steroids can cause osteopenia, brittle bones. Of course, weight should be controlled to help the weight bearing joints to not have to work so hard.    

People tend to not believe that affected children are sick because many of them still act the same.  Eventually children get used to daily pain to a degree, and with the medicines that are available now, many people can function at an almost-normal range.  That does not mean that they are cured, or even that they are feeling well; it simply means that they have learned to adapt.  They try to keep up with their peers, and be more like the other children. 

Some  also believe that the children are exaggerating their pain to get out of doing activities.  This is a common misconception before diagnosis, also.  On the contrary, many of these children struggle to be like their peers, and will push past their limits to do so.   No one can “see” arthritis at work.  Aside from joint or muscle pain and potentially affected organs, other possible symptoms include: chronic fatigue, chronic pain, stiffness, disfigurement, disability, joint deterioration, blindness, hearing problems, and problems with speaking when the joints in the throat are affected. 

             People have a difficult time understanding how the affected children may feel well one day, terrible the next, and fine again on the third day.  Chronic conditions come and go. A child may have a sore knee for days, then the pain is suddenly gone, only to be replaced with a sore hip or elbow.  With the medicines being used today, some children do go into remission.  Many will not achieve remission for several years if ever, but their symptoms can be controlled fairly well.  This does not mean that they live pain-free. 

Regardless of whatever myths or misconceptions that people may believe, a special diet will not cure arthritis, it is very real, very painful, and can be life threatening.  These diseases are nothing to take lightly!

Works Cited

 Alvarez, Dr. Manny. “Arthritis in Children”, Fox News, N.p. Web, June 23, 2012

Dr. Alvarez is a doctor for Fox News that reports on medical conditions.  In the article, he interviewed a leading Pediatric Rheumatologist, Dr. Yukiko Kimura, of Hackensack University Medical Center, to learn more about Juvenile Arthritis and the conditions that the umbrella term covers.  They discuss prevalence, diet, and symptoms of the disease.

Eustice, Carol. “Parts 1 and 2 of 2- Inaccurate Portrayals of Arthritis Lead to Misunderstandings” .  About.com, September 15, 2008, n.d. Web.

This article is very relevant to my work.  It details the misconceptions about rheumatoid arthritis disease, and gives specific examples of what people seem to think when dealing with a person with arthritis.  She points out that some people try too hard to help an affected person, which does not help at all but can, in fact, cause depression and a feeling of uselessness.  She also discusses the myth that so many people believe arthritis can be cured with diet.  She informs the reader of  what rheumatoid arthritis is and isn’t, and she explains that there is no cure.  This paper is written by a nurse that suffers from rheumatoid arthritis.       

Tong, A., et al.  “Children’s experiences of living with juvenile idiopathic arthritis: Thematic synthesis of qualitative studies”, Arthritis Care Res.  Hoboken, April 13, 2012. Web.  

This was a peer-reviewed medical article on PubMed, added by the American College of Rheumatology discussing how juvenile arthritis affects children.  The researchers used clinical data from electronic databases and other relevant clinical articles.  This research found that affected children feel stigmatized, unable to keep up with their peers, they feel a lack of control over their bodies, and many are depressed.  This source will help me to portray how the affected children feel.   “They have a sense of being misunderstood and stigmatised, and feel perpetually caught between having hope and control over their bodies, and overwhelming pain and despair.”  This was said on page 1 of 1.

 Walker, Gayle-Cillo and Marylou Killian.  “Myths and misconceptions about rheumatoid arthritis”, N.p., July 2010, Vol. 5 no. 7.  Web.  

            This article on American Nurse Today also helps to dispel some of the myths associated with autoimmune arthritis.  This article delves a bit deeper into the treatments and medications,  how these diseases are diagnosed,  and some of the other side effects that people don’t always know about. In paragraph two, the nurses wrote, “The variable onset of RA, subjectivity of symptoms, elusive manifestations, and occurrence in relatively healthy young females can frustrate both patients and healthcare pro­viders trying to make a definitive diagnosis.”  This also shows that many in the medical community have a difficult time diagnosing these diseases.  This will be important when building my case.

Westrich, Tiffany. “Misunderstanding RA- how Much is the Media to Blame?”.  Health Guide on

This article is important to my research because it compares osteoarthritis with rheumatoid arthritis.  Most people do not realize that there is a difference, but in reality these are very different diseases; osteoarthritis is not an autoimmune disorder, for one.  This article also discusses how the media plays a huge part in misrepresenting rheumatoid arthritis as being similar to osteoarthritis.  On page one, Ms. Westrich says, “So what is contributing to this gross misunderstanding?  Look around you.  It's everywhere.  It's on our televisions, it's in our print materials and it's on our computer screens.  The best way to stop it is to change what is publicized in the media.” She also discusses specific advertising methods that over-the-counter drug companies use to mislead the general public into believing that mild products such as Tylenol can stop the pain of their rheumatoid arthritis, and not only for osteoarthritis.  She points out that while many children are affected, the drug companies don’t show children on their commercials, or even younger adults.  She also talks a little bit about the true first line of treatment for each disease.   Ms. Westrich is the founder of the non-profit organization International Autoimmune Arthritis Movement.  She has coordinated with many different rheumatology doctors and other professionals. 

Test results and such

There hasn't been much time to post lately!  I have been studying away.  My last day of summer session courses is Wednesday.  I still have a lot of work to do, but I finally feel a bit less pressure.  And I've had some time to mull things over.

The test results from the EKG, and the echo were both pretty good, but the chest CT showed "an air-filled esophagus".  It took me a while to get a good answer on that but it boils down to they likelihood of dysphasia in the muscles of her throat.  The nurse practitioner replied, "

An air filled esophagus can be seen with dysmotility of the esophagus, or if the muscles are affected in the esophagus. We can see this with MCTD.  We would only worry if this affected her ability to swallow or if anything she was swallowing was aspirated. This would show up on the barium swallow.  Let us know if she has trouble swallowing or chokes easily of foods or liquids. I will try to set up her barium swallow and PFTs for the 1^st." 

I found a nifty research paper on pediatric MCTD that also mentions just this problem.    The entire article is a whole paragraph, but it does say that out of the 12 patients that they followed for fifteen years, they that:  "Inflammatory manifestations (arthritis, fever, and skin rash) improved following treatment, whereas sclerodermatous features (sclerodactyly, esophageal disease, and vasculopathy) persisted and were often unresponsive to therapy. The organ involvement-free rates at 2 years, 5 years, and 10 years were 91.7%, 78.6%, and 52.4%, respectively." 

When Emily was first diagnosed with Dermatomyositis, they did an upper GI and found that the muscles on one side were not functioning properly.  That made sense at the time; she was having trouble swallowing, she was losing her voice, and her bird-like appetite became worse.  This time... I wasn't expecting this.  It was really rather out of the blue.  And I can't help but wonder, if there is dysphasia, how long has it been present?  Is it a result of the Dermatomyositis, or is it a scleroderma feature?  I had noted that her AST, one of the liver enzymes, was just a touch elevated.  It hadn't been elevated in at least a year.  My new mantra has become, "there is no point in worrying when you don't know for sure what you are worrying about."  In every other way she is currently doing fabulously!  

I'm not sure if it's the Orencia kicking in or perhaps it is the magnetic jewelry as she credits, but her joints have done a 180 in the past week or two.  Previously her ankles, knees and hips were really bothering her.  Right now it seems to be more fatigue than anything, and that isn't even that bad.  I made her new anklets and necklace the week after coming home.  For those that don't know, we were told that magnets help somehow.  To buy a magnet bracelet is only about $10, so we thought that we would give it a shot.  She swears that it works, that it really helps with the pain.  I can't say that it really does, or if it's maybe psychosomatic, but either way it seems to help her so why not?  I can buy a bunch of beads, 5 meters of string, and a string of magnets pretty cheap, and it's so easy to make something that she will like and wear.  Since she didn't want to start the Orencia in the first place, I think that even if she thought it was working, she would still say it's the magnets!   We were told that if the Orencia starts to work then we can drop the Remicade.  Remicade is a 4 hour infusion, plus an hour for Solumedrol (IV steroids) first, IViG can be 4-24 for her, and Orencia is a half hour.  So, if we move to Orencia only, unless it is a Rituxan month, we may be able to make an 11 AM appointment and NOT have to stay the night at the hospital!  On a Rituxan month we would have to stay late; that's another 4 hour med, but perhaps we wouldn't have to stay the night.  

In other news, Zachary is doing pretty well.  Despite Prilosec, he's had a few really bad stomach nights, but I may be able to pass it off as a bug.  I'm still keeping a weary eye on the situation, given his penchant for spending very long periods of time in the restroom, but it doesn't seem to be an emergency.  His psoriasis is almost non-existent.  He has had some random pains, but nothing lasting.  I am going to ask next time he is seen what is up with his blotches.  Where there were once psoriasis scales, there are now white patches.  It may possibly be Vitiligo, which wouldn't surprise me.  I really was sure of that for a while, but they do seem to be darkening a little.  I'm hoping that by the time he is seen next they will be gone.  We shall see!  

My next post in the next few days will be from one of my paper's from school.  For our persuasive paper, I chose to write about what I know best- the myths and misconceptions of Juvenile Arthritis.  If we all help to raise awareness,  perhaps one day we won't need to.

 

Unexpected tests and such

I swear, sometimes I jinx us.  Just the other day I posted on new research that points a bigger finger towards developing Scleroderma for patients with Mixed Connective Tissue Disease.  The funny thing is that, while she has had the sclerodactyl hands and some wrist involvement with the skin on her wristsfor years now, it hasn't really been brought up at all by our current rheumy group.  Until yesterday.  And it wasn't that Dr. S was saying by any means that this is turning; it was more like, "we need to run these specific tests every year so that we catch any disease progression just in case it turns into Scleroderma."  Now, I know that having MCTD puts her at a much higher risk of pulminary fibrosis than almost anyone else, but this is the first time that Scleroderma has been brought up.  So, last night she had a chest CT with contrast, and an EKG.  She had an echocardiogram a little while ago, and she's supposed to be scheduled for another Pulminary Function Test.  It was just surprising.  She did NOT like the echo.  I truly didn't think it would be a big deal, but she said that the ultrasound probes hurt her. She didn't like the contrast for the CT, either.  She spent quite a long time in tears about that, despite my explaining that it was just a dye.  At least they are both over now, done for a year or so.

Leaving for the hospital, I knew that there was something important that I was forgetting.  I knew we had another concern to bring up but I could not remember what it was until after the doctor left.  Her hearing!  Lately we've noticed that her hearing is not that great.  There are many things that she doesn't hear correctly.  I know that, while it's very rare, there are actually joints in the ear that can cause hearing loss.  I believe that it could be the sinus pressure is starting to build once again.  I can't believe that we forgot about that!  I did have a chance to ask the immunologist about it (because he is the one covering for rheumatology today).  He didn't say too much about it because he couldn't see any problems.  But, he does feel that we should schedule a hearing test.  I assumed that would be the case.

The other thing that we were curious about was her current rash.  She's had one like this before.  It starts out with one or two areas that look like mosquito bites, but more keep coming.  They are just red spots.  When she itches a lot, the middle gets a little speck of blood.  They don't respond to Benadryl.  I hadn't even thought to try cortizone on them.  Dr. S agreed that it is likely an autoimmune rash.  He said that often with rashes that are associated with an autoimmune disease that is being treated, they don't look the same as they would in non-treated disease, so it is more difficult to know what to attribute it to.  MCTD'ers are more likely to break out in different types of rashes than people with JA or RA alone.  She's had more vasculitis-type rashes on her face and hands, too.  She gets spots that look like small pink blotches in those areas.  On the bright side, he was showing a medical student what he looks for when looking for dermatomyositis.  He explained that he can't show her much because he sees no active disease!  That made my day!  :)  At onset, she had the speckled capillairies, the pink elbows and knees, more spots on her hands (they didn't look like Groton's Pauples to me, but I guess they were), and she had the classic heliotrope rash around her eyes.  Right now, she looks darn good! 

I am not used to her needing Zofran before Methotrexate yet, and we both forgot about it! So at 1:30 A.M. she was still awake with a sore tummy.  The nurse called the doctor, who added Zofran in.  She was able to sleep after that, at least.  Of course, respiratory came in at 6 to give her Symbicort, along with vitals every 4 hours.  She's tired, and she just wants to go home but she's doing well.  At least now we're in the home stretch.  She's got her Remicade going now; when it is done, we are free to leave.  I had originally thought we would be home by now.  Ah, well.  At least she's got chips and TV.  She's comfy, so it's all good. 

Due to the holiday, I'm not sure when we will have her test results, but I will post more once we have some answers.

Favorite New Site

     Today I was lucky enough to stumble across Rheumatology News, a website devoted to research articles of the different rheuamtic diseases.  I was pleased to find that they have a section on Lupus and Connective Tissue Diseases.  While hunting there, I found this article, whose author believes that MCTD is a subset of scleroderma.  While I would love to say that the author is wrong, I believe that she is correct.  I have told several people in the past that I see more Scleroderma symptoms in my daughter than Lupus symptoms.  She has significant hand involvement; her hands are very affected by Raynaud's, arthritis, Scleroderma features (including the sclerodactyl look.)  Her wrists have been affected by Scleroderma in the past, also.  She has had some issues that I don't know how to classify, or how to attribute.  While I don't put a whole lot of stock into research anymore because much of it turns out to be wrong,  I also know that this is far from a fully- proven piece, and there is a lot of research needed to explore this theory deeper, it is something interesting to think about.  What I'm really hoping is that this research will get more funding for some of the diseases in this spectrum.

      This is what I remember reading when we heard the words "Mixed Connective Tissue Disease" for the first time.  "Some studies have found that patients who originally were diagnosed with MCTD often over time develop predominantly the features of one disease (such as Scleroderma or Lupus)", writes Coburn Hobar, and Arnold Postlethwaite.  (See footnote). 

The Lupus Foundation seems a little confused on the matter, but this is still a good comparison between Lupus, RA, Scleroderma, Ssc,Vasculitis,  the Myositis diseases and Sjogrens. 

While looking for statistics, I found this awesome slideshow, presented by Janet Pope for the 2006 Scleroderma National Conference.  While a bit incomplete on a few slides, overall it is excellent for anyone that does not have a basic knowledge of Scleroderma, Lupus, RA, MCTD or Poly & Dermatomyositis. 

 My searching wasn't only limited to MCTD today.  I was first led to an article on Rheumatology News  called "New Anti-Inflammatory Drugs Will End Anti-TNF Dominance., (by one of my Dermatomyositis board mom's) and then I found this article on a new study drug that is showing strong promise for psoriasis.  It does not mention psoriatic arthritis, but I imagine if the drug is similar to Enbrel that it will likely work for PsA.


*Coburn Hobar, M.D., Rheumatology Fellow, and Arnold Postlethwaite, M.D., Professor of Medicine and Director, Division of Connective Tissue Diseases, University of Tennessee Health Science Center, Memphis, Tennessee, and Department of Veterans Affairs Medical Center, Memphis, Tennessee (originally published in "Scleroderma Voice," 2003 #1)