Thursday, August 30, 2012

The Loss of Innocence


Yesterday, I took Rachel to have her blood drawn for lab work.  I guess (and hope!) Rachel's doctor is wanting to see if the prednisone is working and that her inflammation is decreasing.  Then we can start decreasing the dosage.  Hooray for that!

As an aside, Rachel asks at least once a day if we're going to the doctor.  She equates "the doctor" with the people who draw blood, the nurses who give her shots, and the nurses who gave her the infusions.  She had so much fun during her infusions as she watched movies and drank "apple juice" which was really Gatorade.  The nurses made a big deal about her, too, which fed her little ego.

So we went to "the doctor" yesterday to have blood drawn.  We hadn't been to this place before, so Rachel didn't know what was going to happen.  And she was sweet and happy even when we were taken back to the cubicle where they were going to do it.  But once I sat down with her on my lap, she realized what was going to happen and started saying, "No, I don't want to!"  Poor thing.

The technician hadn't made any kind of move toward Rachel yet, and she said incredulously, "Has she done this before?"  Unfortunately.  And actually, this was the fourth time that my little girl has had her blood drawn.  And none of my other children have had blood drawn at all ever.  It's not fair, is it?

I want to preserve the innocence and ignorance of my children.  I don't want them to know what it's like to have their blood drawn.  I don't want them to be apprehensive of going to the doctor because of painful treatments.  Thankfully, Rachel isn't apprehensive (silly girl!), but oh, how I wish she didn't know what it was like to have blood drawn.

Monday, August 27, 2012

Confusion: Something We Don't Need

When Rachel's doctor started her on prednisolone, he wrote for a 30 day supply with no refills.  Last week, being a pharmacist and all, I calculated when her medication would run out in relation to her next appointment. Oops!  It was going to run out four days before her next appointment.  Again, being a pharmacist and a planner, I wanted to take care of this hitch before it became a problem.  So I called last Wednesday and left a message, asking if they wanted to call in a prescription for four days of medication or if they wanted to see her before it ran out.  Either way was fine with me.  I just didn't want to run out.  Finally, Friday they called back.

Apparently, the doctor's office doesn't have people making phone calls like this; the nurse I talked to didn't seem to know what to make of me.  At first, she just wanted me to have the pharmacy fax a refill request to her so they could just refill it.  The problem with that "solution" is that the pharmacy that initially filled Rachel's prescription is an hour away.  I would like to have a pharmacy closer to us fill it next time.  So I gave her the phone number, and she said she would call back.

The nurse called back later to tell me that she had talked with the doctor (a different one than we have dealt with previously), and he didn't want to refill the medication.  He said that Rachel would be fine without her medicine until she could be seen.  Um, no.  I have to admit that I got a little huffy with the nurse at this point.  Being a pharmacist, I know that you can't just stop steroids.  If you've taken them, you know that, too.  Even if you've just had a Medrol dose pack, there's a reason you take six tablets the first day, five the next, and so on, tapering the dose down.  So I very firmly said that "solution" wasn't acceptable.  The nurse said she would call back.

I'm sure she was getting tired of talking to me at this point; I certainly was getting tired of talking to her.

The next time the phone rang, it was the same office but a different nurse.  She said that we could be seen this week.  I looked back at the calendar, and this week would just be three weeks since Rachel's infusions.  I had gotten the impression from the first doctor we saw that Rachel wouldn't need to be seen for a month.  I didn't want to go back too early, too early to see how her treatment was doing.  Finally, finally, finally, after much back-and-forth, we decided to be seen next week before Rachel's medication runs out.  Argh!  What a hassle!

It still upsets me when I think about it.  And the only thing I can figure is that the doctor and his nurses are not used to having someone think about these kinds of things before they happen.  I'm sure if I had called the day her medicine ran out, they would have known what to do.  But I didn't want it to come to that.  Maybe if I had waited another week or two to call...but I didn't want to leave things until the last minute, either.

We had a really good experience with Rachel's first doctor; honestly, I hope that we see him again the next time.  I plan to call and find out.

Thursday, August 23, 2012

Managing Other Side Effects of Prednisone


In order to deal with a few of the side effects of prednisone, Rachel has to take a couple of additional medications.  She takes generic Zantac twice daily to keep from developing a stomach ulcer.  Do you know how medications like ibuprofen can upset your stomach?  Steroids do the same thing in the same way.  Because Rachel cannot get any sunlight and to help strengthen her bones, she takes a calcium and vitamin D supplement.  There isn't a liquid form of that, but we found these (thanks, Mom!), and they're even better than a liquid.  Rachel thinks they're candy, and they make me wish I needed a calcium supplement!

Prednisone also can make you moody.  Rachel has always been exuberant, and now she is more so.  Her highs are higher, and her lows are lower.  We enjoy the highs, but oh!  her fits are bad.  Thankfully, there are more highs than lows.

As for the weight gain and water retention, there's not much we can do.  She ought to lose the extra weight when she comes off the prednisone.  And her growth should catch up, as well.

I'm so glad that Rachel should only be on this medication for a few months.  And we're praying that that is what happens.

Wednesday, August 22, 2012

Immunosuppression

With JDM being an autoimmune disease, it means that her immune system is attacking her own body.  We turn that off by giving her high dose steroids and keep it off with the low dose methotrexate.  But since her immune system is off (or close to it), that means that Rachel is susceptible to getting infections.  We're going to have to be careful to keep her away from people who are sick.

There are many reasons that I'm glad we made the decision to homeschool, but one more is that Sarah Beth and Daniel aren't going to school, picking up germs, and bringing them home.  Yes, I know that any of our children can still pick things up at church or wherever, but things seem to be worse at school.  I think it's because children are cooped up in a room together all day long.

Anyway, as fall and then winter approach, cold season is also coming.  We've got to be vigilant because even a simple cold could be bad in someone whose immune system is depressed.

So here's another prayer request:  that Rachel won't pick anything up.

Tuesday, August 21, 2012

Photosensitivity


Since JDM can be triggered by too much sunlight, these children must be protected from the sun.  As a matter of fact, we think it was our trip to the beach at the end of May that triggered Rachel's.  Poor Rachel loves to play outside, but we have to limit our time out there now.  The hours between 10 am and 4 pm are off limits completely.  And when she goes out, she has to wear long sleeved shirts, long pants, and a big-brimmed hat.  Every morning, as a part of getting dressed, we put sunscreen on her.

Rachel has taken all of this in stride.  I think it helps that she's only two and doesn't realize that what she's doing is different from others.  When I remind her about her hat, she says, "So the sun won't get me."

Apparently, the methotrexate will also make her sensitive to the sun.  But with all of the precautions we're already taking, it won't add anything to that.

Monday, August 20, 2012

Rachel's Treatment



As you may recall from this post, Juvenile Dermatomyositis is an inflammatory disease.  So how do you stop unwanted inflammation?  With high dose steroids.  The infusions that Rachel received two weeks ago were high dose corticosteroids, and we started prednisolone (the form of prednisone that can be in solution) on that Friday.  She will take prenisolone at this dose for a month.  Assuming that she continues to do as well as she has been, her doctor will start to decrease this dose at her next appointment.  You cannot just stop steroids; since your body produces its own steroids, it stops producing them when you take them.  So if you just stop taking steroids, it will send your body into shock.  You have to wean off of them.  Rachel should be on steroids for only five months, assuming....

Meanwhile, we have also started methotrexate.  That is also an anti-imflammatory medication, and it is used in high doses for cancer.  We're using it at a low dose, and I have to give it weekly subcutaneously or under the skin.  Rachel will receive this medication for two years, again assuming....  Methotrexate allows us to take her off the steroids because they have such a bad side effect profile.  Giving her this injection gives me the heebie-jeebies.  As a matter of fact, I couldn't sleep last Sunday night after I gave her the first injection that afternoon.

The side effects of steroids include, but are not limited to, indigestion, weight gain, increased appetite, water retention, brittle bones, mood swings, immunosuppression, and stunting of growth.  You can see why we want to get her off of her prednisolone as soon as possible.  According to our doctor, the only real problem with methotrexate is stomach upset.  (It has all kinds of side effects at the higher, chemotherapy doses.)

Does this make sense?  If you have any questions, let me know.

I plan to write a little bit this week about some of the concerns we have and what we are doing about them, things like photosensitivity and immunosuppression.  But we all know what happens to the best laid plans of mice and men....

Thursday, August 16, 2012

Catching Up on Rachel


I'm sorry that it's taken me so long to update you on how Rachel is doing these days.  I wanted to get those other two posts over on this blog, as well, since it's dedicated to Rachel and her Juvenile Dermatomyositis (JDM).

First of all, I want to relate how the rest of last week went.  Rachel received three infusions of high dose steroids, and she did great.  Getting the IV was awful, but they were able to leave it in for all three days.  After they got the IV in and got everything taped up really well, Rachel's fingers were covered almost completely.  She got a frightened look on her face and cried, "They took my fingers!  They took my fingers!"  Poor thing!  Honestly, it was hard not to laugh, but we were able to reassure her that her fingers were still there, and she could even see the tips of them.  Then everything was fine.

I took Rachel back for her last two infusions by myself since John needed to work.  And we were fine. Rachel actually was excited to get there.  When we got to the exit off the interstate, she would yell, "There it is!  There it is!"  I think she enjoyed being an only child and receiving all of my attention.  The nurses made a big deal of her, too.  We just sat there and watched DVD's while the medicine dripped in.  Since it didn't hurt or burn going in, she didn't care at all.  When the nurses came in to take her vital signs, she demanded to be allowed to "push the button."  Her first nurse had asked if she wanted to push the button to take her blood pressure, and Rachel thought it was her right from then on out.

Rachel keeps asking to go back to the doctor.  She enjoyed being at the hospital way too much!  Thankfully, we don't have to go back for a month.

My mother raced down Monday afternoon so she could stay with our other three children while I took Rachel for her infusions.  I don't know what we would have done without her.  Mary was the hardest because she was just starting solid foods and was still getting most of her nourishment from nursing, but they made it through.  I thought I was going to have to wean her in order to take care of Rachel, but there's no point now since we expect to be home with occasional doctor appointments.

Friday was a nice quiet day which we all needed.  My mother headed back home, so we just relaxed all weekend.  And this week has been pretty quiet, too, which has been nice.

As for Rachel, she looks great.  We didn't think she was feeling poorly before, but she obviously feels better now.  There's an extra sparkle in her eye and spring in her step that was missing lately.  We just didn't realize it because it was such a small change from her normal.  Other than the rash on her face which is clearing up nicely, you wouldn't think she was sick...and she's not really.

Our prayer requests have changed a little bit now that we know more of what's going on:

  • Mostly, we want God to be glorified through this whole experience.
  • And we pray that Rachel won't ever get sick.  Oh, we know that she's got a disease, but she doesn't look or feel sick, and we would love to avoid that, if we could.
Tomorrow, I hope to let you know a little more about her treatment.  

Wednesday, August 15, 2012

Rachel's First Week of Treatment


 Rachel waking up from the MRI.

This post was originally published on Nikki's Notes:  An Update on Rachel.
John took Rachel for her MRI on Monday, and she did great.  He was able to be with her while they put her to sleep and when she woke up.  It did show muscle involvement, but we weren't surprised about that.

The doctor did not want to wait until today to start treatment, so we headed back yesterday for Rachel's first infusion.  She did great again although getting the IV was pretty awful.  Thankfully, they left it in so we don't have to go through that again today and tomorrow.

Rachel asleep on the way home yesterday.  It's been a long couple of days.

She still looks great, like nothing is wrong except these odd rashes.  When John asked about further infusions or whatever, the doctor basically said that we're not going to worry about that yet.  We're going to assume that this treatment "fixes" her and that there won't be any relapses.

We want to thank each of you who are praying for our little girl.  She is being a trooper about all of this, and it is amazing to see how resilient she is.  We praise the Lord that we caught this disease when we did.

Tuesday, August 14, 2012

Rachel's Diagnosis


This post was originally posted at Nikki's Notes:  Rachel.

Rachel is our sweet, little girl. Well, sweet may not be the best way to describe Rachel. She's a precocious, mischievous two and a half year old, and my mother refers to her as our "pot-stirrer." Rachel loves to pick at her siblings: she inserts herself between Daniel and the television so he can't see it; she steals Sarah Beth's doll as soon as she puts it down; and we catch her sitting on Mary frequently. All of this said, there's not a mean bone in Rachel's body. She can't seem to help it. (Yes, we do discipline her when necessary.) And Rachel walks through our house and her life with a smile on her face, a song on her lips, and a dance in her step.

When we returned from our beach trip, we noticed that Rachel had pink cheeks. We figured she had just gotten too much sun and didn't worry about it. Two weeks later, however, when her cheeks were still pink, we reconsidered. And we found a few more "spots:" some of her fingernail beds were red, inflamed, and extremely painful; she had a rash on her knees and elbows; and a bug bite on her toe wasn't healing like it should. None of these things were worrisome in and of themselves, but all together, we were concerned. So I took her to the doctor who ordered some lab work.



Most of Rachel's lab work was normal except that her ANA was elevated. (ANA is basically a nonspecific marker for an autoimmune disease.) Our doctor got her an appointment at a nearby teaching hospital, but the soonest they could see her was the end of August. (At this point, we were in the middle of June, so that was a good bit of time away.)

We waited patiently until Rachel developed another rash last week, this time on her arm. Thankfully, our doctor had recently seen this same thing in another of her patients, so she was able to diagnose it quickly: lichen striatus. This skin rash looks much worse than it really is; topical steroids can help, but there's really no other treatment. It doesn't usually itch or hurt, and Rachel wasn't bothered by it at all. However, whatever Rachel had was getting worse and affecting her more. Our doctor called around and got Rachel another appointment at a different hospital, a children's hospital, that's a little further away.



We took Rachel for this appointment on Wednesday last week, and the doctor there immediately knew what she had: juvenile dermatomyositis. Basically, this rare, autoimmune disease attacks blood vessels and causes them to become inflamed. This inflammation makes the skin and muscles become inflamed. It can start with a skin rash, like Rachel did; but some children are not diagnosed until their muscles are so affected that they cannot even stand up. Treatment involves high dose steroids for quite a while. Children are usually photosensitive, and poor Rachel loves to play outside. Usually, there is a "trigger," and we think the trip to the beach was that trigger.

Thankfully, we seem to have caught Rachel's early enough that her muscles aren't too involved yet. John is taking her for an MRI this morning to see how much her muscles are affected. Then we have an appointment on Wednesday to discuss treatment.



This whole thing is surreal to me. We have our happy, smiley little girl, and nothing seems to be wrong with her except for a rash in a few places. However, there is an insidious disease process going on inside her body that we must stop before it becomes debilitating.

Monday, August 13, 2012

The Beginning


When Rachel was diagnosed with Juvenile Dermatomyositis, I thought about starting a blog to chronicle our journey with that disease.  I mentioned this idea to John, and he encouraged me in this endeavor.  Why am I starting a new blog instead of following her on Nikki's Notes?  John and I refuse to let this disease define who Rachel is.  This blog is for Rachel and her JD; Nikki's Notes is for everything else.  Honestly, I hope not to post too often here because she is doing so well.

Another reason that I started this blog is that John and I looked for blogs dealing with Juvenile Dermatomyositis, and we couldn't find any current ones.  We hope and pray that this blog will be of benefit to someone else who is dealing with this disease.

I also would like to educate my readers about this disease.  I hope that you never have to deal with it, but if you know someone who is being treated for it, maybe this information will be helpful to you.  Even if you don't know someone with this specific disease, maybe you do know someone with an ill child.  I hope this blog will help you identify with them better.

Why is this blog called "Stirring the Pot"?  We chose this name for two reasons:  Rachel is our little pot stirrer; she loves to pick at and aggravate her siblings.  I think sometimes she even picks/aggravates Mary in order to push my buttons.  *Sigh*  The other reason is that God certainly stirred our pot when He chose Rachel for this disease.  Our apple cart got overturned with our move down here to Florida, but we've found our new normal.  We found a church, made friends, bought a house, and started school.  God stirred our pot, though, with this disease.

We don't know what the Lord has planned for Rachel and our family during this journey, but we know that He does have a plan.  We trust that that plan will be the very best thing for our family, even if it doesn't feel that way at the time.