Three years of JM and MCTD

It was September 2011 when we first realized that Emily was very, very sick.  We had gone to her former rheumatologist who had kind of given her a diagnosis... without any solid evidence to prove it.  After blowing us off and telling us to come back in three weeks, we begged a new rheumy to see her.  It was October 13, 2011 when we got the definitive diagnosis.  We didn't get home until late on October 19th.  Six days of hospital time- upper GI, MRI, lots of labwork, EKG, PFT, daily steroids and anti-inflammatories, and a lot of sadness.

Since then, we have been through a lot.  We had the whole Prednisone double-the-weight-in-two-months thing happen, which was horrible all around.  It caused major depression for her, and ripped our hearts out as we watched her eyes swell shut.  We have found out about her throat muscles not working due to dermatomyositis, and then later we found that the scleroderma features are causing the esophagus muscles to spread, leaving a gap between it and her stomach.  We have found out about early changes labeled as mild interstital lung disease.  I saw that at our pediatrician's while reading the newest note they had sent over.  We've had low cortisol and adrenal insufficiency, which contributed to causing kidney stones.  As yet we have not resolved any of the above, with the exception of the stones.  At this point, her pain continues to increase, likely because her pain amplification has come back.  Her medicines just keep increasing.  Below is the list of meds and vitamins that she has to deal with daily.  I print this every time we see any doctor.  I print triple copies when we have a hospital stay.  You never know what kind of a resident you are going to have!

Despite this, we still have hope.  Hope that one day she will be in remission.  Hope that there will be no more progression.  Hope that her pain levels will come down, as they continue to be an 8-9 (on a pain scale that goes from 0-10).  Hope that she won't need to use one of those electric carts whenever we have to go to a big store.

We had an issue the other night at a big box store.  I usually try to avoid this place, but I promised my oldest daughter that we would upgrade her phone there because the AT&T stores no longer carried the one she wanted.  We went in, all of us dressed in business clothes since we had just come from my honor society induction.  We chose an electric cart to help Emily get through the store, and we set about on our way until a greeter stopped us to tell us that she wasn't allowed on the cart unless she sat on my lap.  I pointed out that they were there for disabled people, and she was a disabled child. She said that children were not allowed to ride them regardless.   I am not quick to anger (except maybe at work), but I was livid pretty quickly.  I asked to speak to the store manager, or a manager on duty.  I offered to go to the car and bring back our handicap permit, but Emily decided to show off her port scar and educate this woman.  She claimed that it was company policy, but she did not call the manager for us.  Instead, she told us that she would "let us go this time".  Nice.  I wrote on their Facebook page, and their corporate Facebook, who directed me to a feedback link.  I know you can't see her illness, which makes this our job to educate.  We have never had an experience like this before, and we hope to never have another.  I ended my post with "my child has likely suffered more in the last three years than this greeter has her entire life".  And it is so true.

All in all, we are managing.  For me personally this year has been pretty amazing, but it really sucks to see my little one suffer.  It just shouldn't have to be like this.

_________________________________________________________________________

Conditions:  Mixed Connective Tissue Disease, Dermatomyositis, Juvenile Arthritis, Raynaud’s Phenomenon, Scleroderma, Vasculitis Rashes,  Esophageal Dismotility

Current meds- last updated October, 2013

*Prednisolone- (15mgs per 5ml) Take 0.5 mls in the morning

Hydocortisone 5MGs- Take one tablet twice a day

Prilosec (Omeprazole) 40mgs One capsule daily.

Procardia ER 30MG (Nifedipine)- One pill once NIGHTLY. (For Raynaud’s)

Mycophenolate 250MG (generic CellCept) 3 pills A.M. (750mgs), 250mgs P.M.

CVS Iron  65mg- 1 pill once daily

Zyrtec- 10mgs nightly

Amitriptyline HCL 20MGs- Take one tablet nightly at bedtime.

Symbacort 160/4.5-    Two puffs twice daily  (as needed- through winter and sickness)

Sucralfate 1GM- Take one half  tablet twice a day  

Xanax .25 mg- Take 1 half of a tablet as needed for anxiety

Lidoderm 5% patch- apply as needed 12 hours on, 12 off  ******** NEW*********

Multi-Vitamin & 2500 Citracal 400mgs calcium with Vitamin D 500 IUs– once daily

Vitamin D- 1000 IUS, 2 fiber gummies per day

***IV Infusions:***   

Remicade 325mgs monthly

Rituxan 400mg- Every 3 months

IViG Gamunex 10% (15 grams)

Stone Update

She had aquatic therapy twice this week so far.  Monday's was not so great.  She promised me that she would give it a chance, but she wouldn't cooperate at all.  On Wednesday it went well!  I cannot say for sure that she enjoyed it, but she at least cooperated.  She looked like she was having fun.  Part of the reason that she did so well, I think, was Tuesday's Biofeedback session.  What a wonderful tool!  Again she was hooked up to the little EKG probes, and again we could see her tension visibly on screen.  The cool part about that was that when she was upset about something we could see it shoot up to like 18, but once she started deep breathing and listening to her relaxation exercise it dropped down to about a 4.  As Ginny pointed out, "see, Emily?  You have the power to relax yourself.  You don't need anyone else to do it for you".  How cool is that?  We discussed aquatic therapy quite a bit that day.  I was very pleased that she did so well the next day.

Yesterday was the kidney stone procedure.  Of course, it wouldn't be our bumpy road if anything worked as intended!  The doctor called me on Tuesday as we were walking into a hospital for Emily's biofeedback to tell me that we may not proceed with the lithotripsy (shockwave of the stones).  I really had no clue what was planned, but we are pretty used to this type of thing.  Things always work out for the best whether we stress or not, so I chose not to stress.  He called me the next day to say that they had a new plan, but it would likely mean two different procedures.  He had initially said that the lithotripsy would require two procedures also, so it wasn't much different to me in that respect.  He told us that he conferred with specialists all over the world to determine the best course of action for our special girl.

They put a stent inside of her urine tube to try to make it bigger.  He told me that it was likely they would not be able to get the ureter opened up enough to grab the stones today.  Sure enough, he was right.  He said that she would have to have the stent in place for 1-2 weeks.  It should dilate the tube enough to pull them out later.  Right now she hurts but not too badly.  They gave her pain medicine, an antibiotic, a medicine to help with bladder spasm, and another med that I cannot recall right now.  The first few hours were awful!  She felt so terrible, and she was in so much pain.  Right now she seems to be her normal self.  It's probably somewhat the pain meds, but it is reassuring.

The only beautiful thing about this is that aside from my working this weekend, we have no appointments planned until 2 weeks from now.  It really just depends on when we go back for the second procedure now.  Thank you so much for your prayers and love.  We felt it, it helped me to be calm and collected.  Emily was still a bit of a mess, but still much better than most kids would likely be.  Thank you for your support.  It is very appreciated!!!

The next post will be a how-not-to-die-at-the-hospital type of post, I think.  I have spent a lot of time comparing how I was ten years ago to now.  I have learned so much, become so organized which is really weird for me.  I feel like I need to share it.  That is coming!  Stay tuned!

Anyone have a cheap helicopter for sale?

Looking back on the past few years, I have NO idea how I worked full-time.  This year has been so hectic, and we have added so many new specialists.  The past few months have been extremely difficult and overwhelming.

Zachary is doing better than Emily still, but we have noticed... things.  For instance, now that I have "kicked the kids out of the house" (to play), he complains more about pain from behind his knees.  His rheumies hadn't mentioned anything before about enthesitis-related, but with Psoriatic Arthritis it does make sense.  His ankle pain comes and goes, but lately it is more knees than anything.  Last month when he saw our rheumies, we were talking about a friend of his who has had a lot more disease activity.  I pointed out that Zach's case is relatively mild, but the response that I got back was, "PsA is a lot harder to treat 10-15 years out.  PsA kids tend to be the hardest to treat and control down the road".  Great.  My heart sank.  Fortunately, we live day-by-day!  I am not going to get caught in that trap, and I won't let the kids get caught, either.  I'm just happy that he is outside playing.  Emily has been playing outside too, though she doesn't last as long.  I am sure that she has been depressed, but hopefully the plan we have will address that and correct it quickly.

I have always been the mom that knows what is happening.  It has been very rare that I was wrong- but the past few months I have felt so out of the loop, like I'm part of a guessing game.  I think we are on the verge of getting that situated, but still it has been hard on me.  Being this hard on me, I can't imagine how my little people cope.  We did switch back to Remicade, which definitely seems to be helping.  She has gone from having mostly 7-9 on the pain scale days to 4-5.

I think I am finally figuring out Emily again.  Ok, this is a lot.  So, she does have kidney stones, which we feel are definitely making life more difficult.  However, what makes everything tougher is that she has "Pain Amplification Syndrome".  I have mentioned before that it was brought to our attention that when one isn't growing properly, their cells aren't reproducing the way they should, which makes the body hold pain in.   After a friend mentioned it, I started suggesting it to different doctors.  They all agree.  That said, we see endocrinology on July 17th.  I am truly pinning all of my hopes here.  As KM said, in their case once growth hormone was added, the first month was horrible but after that everything settled down.  Pain came down, growth started; the best part is that the hormone is a natural hormone, and for them it helped them to decrease some and eliminate other medicines.  I would LOVE that!  I am quite sure Emily would, too.  I am also hoping that this will help her headaches.  Neuro-opthamology didn't see any reasons for her headaches, which I now think may be a combination of hormones (pre-pubescent) and the pain amplification.  We are scheduled to see nephrology the same day we see endo.  The very next week Em has her first psychology appointment, and the day after that she has a procedure to blast her kidney stones out by sonic waves.  They will sedate her because a tube needs to be inserted up into her kidney.  They tell me they will repeat this procedure on another day because sometimes they stones are stubborn and either won't break up or will break up but won't leave the kidney.  We really hope that having these little nasties gone will help her pain level go down a bit.

Another new specialist that we have added is pain management.  That doctor wants her to try aquatic therapy three times a week, as well as biofeedback.  She says that these will both help the amplified pain syndrome.  I'm just not sure how to fit these in.   We have found the aquatic therapy close by, but I still haven't spoken to a provider for biofeedback.  Right now it looks as though it may be an hour south for BF, close to urology.  Comparatively speaking that is not bad.  Rheumatology, who handles pulminology and GI are all two & a half hours north.  Pain management, endocrinology and nephrology are all 2.5 hours east.  With all of these appointments, school work for the kids, and my own schooling come fall, how the heck am I going to swing aquatic therapy three times a week?  I'm not sure, but I know that we will.  If it will really help her, we will do it.

The pain management doctor prescribed pain patches for her.  Just lidocaine, but it seemed to help her knees a bit.  I love that she chose something that didn't require another pill.  I am not familiar with biofeedback, but from what I understand, it involves teaching the child how to cope and deal with their situation using relaxation techniques and monitoring bodily signals like blood pressure and pulse oxygen.  It sounds really cool!  Our advocate has suggested waiting to see what psychology said.  Our psychologist was thrilled that we found a doctor who believes in hypnosis and biofeedback.  I think psychology is a little overwhelmed by our situation.  We had a good laugh over that tonight.  She was pretty amazed at what we are dealing with.  Like I told her, one day at a time and we stay close.  None of us could do it alone.  We need to take this all on as a family.  It would just be easier if we had a helicopter.  Or two.  Or a fleet.

Last thing- Camp starts Saturday for Emily!  She is SOOOOO excited!!!  In truth, for the first time I am glad for her to go to get her out of here, to get a break.  She is so sweet and wonderful, but it seems like every day we are at some appointment.  That will be one week where we are guaranteed not to have to go to an appointment for her.  The down side is that we had stopped Mobic (an NSAID) a couple of weeks ago because it was starting to hurt her stomach.  Since we were stopping Mobic we decided we would drop Carafate (similar to Xantac, it coats the stomach).  Today we ended up back at the pediatrician's office, thinking that her antibiotic wasn't working for her sinusitis.  It turns out that her sinusitis is gone, but her reflux is acting up again.  The Carafate is such a large pill.  I cut it in half for her but it is still so big.  She really hates taking it, and we were so excited to drop basically three pills a day.  Now it's back on the Carafate.  I'm not sure how she handles it, but she is pretty amazing.  I am trying to get us on a wheat-free, gluten-free (or very reduced) diet.  It is very difficult when you have children who are as picky and stubborn as Emily.  She literally won't eat if we don't give her one of her staple foods, but we are learning how to find healthier options.  For instance, I could buy all kinds of fresh fruit but it will sit in my fridge and rot.  I discovered an organic fruit pouch (no GMO!) that my kids have sucked down.  It's actual fruit- not juice.  If that is the only way I can get them to eat fruit, I will do it.  Baby steps.

I will keep y'all updated as we determine new things.  It has just been tough.  Thank you for reading and supporting us.  As always, prayers, blessings, whatever you have to offer, we will always gladly accept and  appreciate fully.

November appointments

Well, I'm not really sure where to start tonight.

We had already pretty much decided that Orencia wasn't really working for Emily, however I wasn't expecting our doctor to suggest just dropping it and hoping for the best.  Granted, we would be keeping the Rituxan, which she feels is actually helping a lot, but still...  I mulled that over for a little while, and decided that with her recent daily thumb and toe pain, that is probably not a good idea. She has told me that they sometimes hurt so badly that she wants to rip them off.  I totally love our rheumy's- ALL of them- and I think this was the first time the question seemed completely not aggressive.

The night went by uneventfully, with no visit from the doctors until just as I was about to go down the hall to take Zach to his appointment.  Just as we were starting off, one of the other rheumies came over to see us.  She asked me how I felt about dropping the Orencia, and I told her my fears about the thumbs and toes.  She told me that she was not fond of the idea, enough to have mentioned it to the head of the department.  This is part of the reason that I love our team- they actually communicate.  I like our other option much better- instead of dropping Orencia, we will spread it out to every six weeks.  She also asked when our last Prednisone decrease was.  Ummm... long enough ago that I can't remember when.  Well, permission to drop 1/2 of a milligram... granted!  :)  That had me over the moon!  I was so excited!  No, it isn't a huge drop, but it is huge to us!

So, I left our room extremely happy, expecting an easy appointment for Zach and then on to home.  Not so much.  While he looks great, our doc noticed that his toes were swollen.  And they hurt.  And his back has an arthritis spot.  Which usually means Spondylitis in a person with Psoriatic Arthritis.  So, yeah.  Not thrilled.  I was very glad that his appointment was today and not yesterday.  I like thinking our appointments over on the way home.  It's harder to think straight when they keep talking and giggling.  It just hurts your heart to hear these things.  This is not what you want for your kids.  I had really hoped that Zach's would be easier to deal with because we caught it early.  He responded so well to Enbrel at first.  I really thought that this would easy.  He's HLA-B17 positive- that is a psoriasis marker, and it often means a more mild form of arthritis, if I remember right.  I thought we were clear.  So far, it doesn't bother him too much, so it obviously isn't really bad right now.  He has complained of his back once or twice.  He complains of the feet often, and on Halloween it was the ankles.  No changes in meds yet- we are to just keep an eye out and see what happens.  We will go back in 3 months, but call if anything changes.  

On a sweeter note, we were asked by one of the rheumies if we were going to be at the Jingle Bell Walk.  :)  We don't go to that event, only because it is so cold, but I thought it was cute that they were thinking about it, especially since our walk is so far away from the hospital.  I really do love our doctors.  But I would like to put them all out of business by finding a cure, please.  Then we could all just hang out and talk medicine without actually having any medical crises ourselves.  On the bright side, we don't have to go back for six weeks.  Woot to that!    

Oh, and we got a dog!  We swore that we were NOT getting a puppy, and we were NOT getting a big dog.  You know what they say about the best laid plans, right?  We have a shepherd/ husky/ lab mix.  He is three months old.  We were both thinking that he was six months old, but no, he's three months.  His name is Brody, but I swear I may change it to Bitey.  He is definitely like a baby, and so cute!  Assuming I can get his immunizations tomorrow, we should be good to start him in training classes tomorrow!  I am so hoping.  We need to get him to stop biting, and so far the tips that we have found on YouTube are not working.  He is good with the younger kids; it's just me, Kevin and poor Ash.  He especially loves to bite Ash!  She'll be just sitting on the couch texting, and he comes up and starts biting her.  Poor kid!  PetSmart said that they can get him close to therapy dog in training.  I can't wait to start!  I'll keep ya'll posted!

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.

 

June was crazy!

I have been trying to find the time to write an update for a while now.  I wanted to honor a little boy named Mason here.  I "met" Mason's wonderful mother via Facebook just after Emily was diagnosed with Dermatomyositis.  In my book, Emily was severe.  She couldn't really walk for about 2 months, and for a while there she was too weak to roll over in bed.  Her throat was affected, so she had problems swallowing and she was losing her voice. (The upper GI test where they make you drink barium and watch how you swallow proved that her throat was weak on one side.)  She was significantly weak, and it took quite some time before seeing improvement.  When I first met Mason's mother, I saw her pics of Mason and thought, "oh, dear God..."  As severely affected as Emily was, Mason was ten times more so.  The poor little man was in a very complicated looking wheelchair, and he had a trachea.  I don't think he ever lost the skin rash that accompanies JM.  To many of us, he was the face of JM.  He was diagnosed when he was very young (17 months old), but he fought bravely until he could fight no longer.  Last month he developed an infection that the doctor's couldn't get under control.  It was very fast.  Mason can once again move freely, and fly with the angels.  Rest in peace, Mason, and thank you for helping to fight JM instead of hiding behind it.   If you would like to learn more about Mason, or help his family with their fundraiser, click here.  They have a huge event planned!  If you are close to the Hillsboro, OR area, pop by to join them!  God bless them.  I simply can't imagine, and hope that I don't have to. 

February 25, 2002 to June 19, 2012.

What a crazy month June was!!!  This week is a hospital week for us, barring any other natural disasters.  We drive up tomorrow for an overnight.  Emily will have her Remicade, Orencia, and IViG tomorrow.  We were supposed to go last week, but we had severe storms tearing through our area.  When I looked at the weather maps, everything showed big storms heading towards our hospital.  So, I changed the appointment to Thursday.  Next thing I knew, they predicted the storm to hit our hospital that Thursday, and many of the major roads in my area were closed due to flooding.  These were areas that many didn't even think were flood zones!  My parents were in a rather large area that was evacuated.  They were not going to leave despite water covering a road directly behind them!  I panicked, but I finally convinced my dad to come stay with us.  I really, truly thought that they would go home to a soaking house, but thankfully that was NOT the case.  In fact, by Thursday I was kicking myself for not keeping the Thursday appointment.  That's okay.  We will go this tomorrow.

Em's doing much better than I thought she would be, actually.  She's had a couple of random things pop up.  She had a bad knee day, she had a bad knee and hip day, she had a bad ankle day.  Overall she's done very, very well.  Especially considering that last week was already a week later than she should have gone, meaning that now she's two weeks past her normal infusion day.  What's more, she's been dancing around, and acting silly... with energy!  I am sure that some of it is the bed.  She is so excited about having her hospital bed, and she has finally slept so well that she feels better.  I'm certain that it's also partially just being home that helps.  I am so happy that I can accommodate her that way.  My only concern with her is that she has some sort of skin thing going on.  At first glance it looks like bug bites.  She's had this happen before.  When she was in the other bed and the other room she would get these things, but her brother wouldn't.  Now she's in with her sister in the new bed and again, it's only her getting them.  No one else in the house has anything like this, and when she's gone outside she's had jeans, socks and shoes on, but they're on her feet and legs.  I took pictures to show to the doctor.  I suspect that it's something autoimmune.  It's just strange.  I'll have to try to google it tomorrow.   If ya'll have any ideas, feel free to shout them out!  Many of them are on her thighs, and not something I feel comfortable posting pics of, but this pic is one fully formed and one starting out.

Emily had a great time at camp, too!  She had a really great week.  She had some truly wonderful roommates!  Zach had some great roommates, too, but he's still pretty young and a momma's boy.  He said that he's never leaving home again.  His stomach gets badly upset when he's away from me; apparently his stomach hurt all week.  Poor little man  :(  He didn't have that much fun, but it wasn't for the camp's lack of trying.  They did everything that they could.  Below is Emily, Zach, and Kevin while getting checked- in at camp!

Well, I will try to update either Tuesday or Wednesday.  It should be interesting sleeping in the hospital with both kids with me again...  (Sigh)  We'll get it figured out.   Ta-ta for now!  :)

True Love

It is hard to put into words how much you love your children.  I think I love all of mine equally, yet differently.  Certainly I have different relationships with all of them.  Ashley was always fairly independent.  She was always off with friends or biking around the neighborhood.  She was a typical kid.  Now she's become quite an extraordinary young lady.  She's going to be a pretty amazing adult.  I can definitely be the mom to her but I can also be friends with her.  We have achieved a good balance.  Zachary is a lovely little guy, working hard on becoming a little man.  He's a regular boy, but he's sweet, sensitive and lovely to a point.

We have different opportunities with Emily.  On the one hand, she's so much more grown up than she should be.  On the other hand, she's still so much like she was when she was five.  She still cuddles and needs us like she did back then in many ways. 

With our other kids, we look at them and see how they are growing up.  We see different opportunities in their lives;  we see them able to kind of do what they want to do.  For Emily, something always seems to go wrong whenever she has something planned.  And it kills us to see her so sad, but she always seems to bounce back.  I guess the hardest part is that she shouldn't have to miss so much.  Looking through her school yearbook tonight I thought of so many events that she wanted to go to that she missed.  And it isn't fair.

I am usually a rock.  I have learned how to survive and deal with the stress as it gets thrown at us.  I try to always do it with a positive attitude.  This is our reality.  It's times like tonight, driving in the van on the way back home from the hospital, watching her in the rear view mirror and thinking about medicine changes and disease progression that I really start to feel like I've been punched.  I wonder what her adult life will be like, and if she will always struggle, if she will always be so sick.  Sometimes I think that I have gotten so used to all of the medical stuff that it doesn't really hit me, like I try not to think about everything too much.  When I do it makes me so sad.   We have to love her harder, be more gentle.  And no matter how much crap gets thrown at her she is always so sweet, so cuddly and gentle.  And I have to say that right now I have actually grown to appreciate a bit how puffy the steroids have made her.  She's extra-cuddly.  I'd rather have her off of steroids but I can try to appreciate the little things, right?

 While waiting in our hospital room, I spent some time cuddling with Emily on her bed.  One thing that I noticed was her sweet smell.  When she was eight, before she started taking steroids and so many other strong daily medicines, she still had that sweet baby smell.  That was yet another thing that Prednisone stole from us when it gripped her.  Today I could smell that again.

While she still gives great hugs, before she got really sick she used to give the best hugs ever.  She would wrap herself around you and cling tightly with all of her might.  It's days like this, cuddling in a hospital bed, that I wonder where the time has gone.

We did have good news tonight.  The Cystic Fibrosis test came back negative.  I assumed that it would, but it's still stressful waiting.  The Orencia that I wrote about yesterday was not approved through insurance today, so we will wait and see.  There is a constant up and down in our lives of extreme good and extreme bad- our counterbalance.  We certainly have rough days, but I wouldn't trade any of them. 

Crazy day coming to an end. (Long)

Today went by in a whirlwind!  We both had a difficult time waking up this morning, but we actually got to our first appointment on time. We waited for about ten minutes to be called for the CT scan of Emily's sinuses; the actual scan was even faster. She did very well. She was a little scared once the machine picked up speed. It sounds like the whirring of a washing machine. That can be scary when you are not expecting it, especially when it is over your head.

From the CT lab, we wandered upstairs. We had a longer stay in the pulmonary waiting area, but they called her at exactly nine. The tech that performed the test was such a sweet lady! In fact, her daughter has chronic conditions, and we share a doctor. She was telling me how her daughter decided that more kids needed things to do in the hospital, so she decided to gather supplies and bring them to kids in a wagon. She was seven years old at the time. That really touched my heart!

Anyway, the tech was wonderful. She made Emily feel more at ease, and she explained everything very well. She took gauze and soaked it in a solution made to produce sweat, then she wrapped the arms, put electrodes on them for five minutes per side and wrapped them in ace bandages. Em grabbed the tablet and watched "My Little Pony" for the majority of the time. When we finished there it was already after 10:30. We walked down to the fourth floor to let them know that we may be late. I'm so glad that we did! Emily hadn't eaten yet, and we still had to get to the ENT for eleven. I stole a few minutes and grabbed her some chicken from Wendy's. The wait was interminable!
We actually made it to the ENT on time. I don't know how considering that we had to wait for valet to bring the van. (Using valet is so much easier than walking for blocks with a hurting, tired child and a bunch of stuff that you need.) I had thought that we were in the home stretch. We would have been, but the ENT was running about forty-five minutes late.

I really thought that I knew how this appointment would go. I was not expecting to see so much inflammation still on her CT. Her right side is nice and clear. The left side remains all swollen and shady. The doctor can't say for sure that it's mucus. What they do know is that it is inflamed. They decided not to worry about treating it right now because she has not displayed any symptoms of a sinus infection. Of course, they didn't have the results yet from the Cystic Fibrosis test. I am assuming that will be negative.


We did not get back to the hospital until one P.M.   I could not believe that we were so late! I was very thankful that I let them know that we may be held up. Shortly after arriving we met up with an advocate who works for a company that works closely with our insurance company. She asked me a ton of questions, and she told me that she will try to get us whatever help she can. Her job is to help get medicine and equipment approved, help to find resources, programs, clinical trials, and things of that nature. She flew in just to meet with me for about an hour. I was very impressed. We are going to work on getting a hospital bed approved!  What a blessing this service is.  And she was wonderful!

After she left things started to settle down a bit. The rushing around was mostly done; we just had to wait for the IViG to get started so that we could move to a room. I didn't expect to see a doctor since we did not fill out any of the paperwork, but as we were packing up to head to our room for the evening our main doctor came over. We totally love her!!! She is just so sweet, but she also has more years of experience than the other two rheumatologists combined. She did the strength tests and was very pleased with Emily's muscle activity. We talked a little about medicines- mostly which ones Emily is on and which dosages. She had just started looking at Emily's hands when she asked if we thought that the Remicade was doing enough. I replied that I didn't think that it had been quite enough for a while. Emily was actually doing so well today that I was surprised that she even asked. She told me that, as much improvement as she has seen in Emily, she doesn't think that it is quite enough. Because Emily now receives her IViG to boost her immune system back up, she feels more comfortable about aggressively attacking the arthritis. She wants to see her running more, being more active, and acting like a kid. If insurance approves it, tomorrow she will be starting Orencia again. She had three doses with our old rheumatologist, but when we changed doctor's, they chose the right route, which at the time was high steroids, Methotrexate as second line and Remicade as third line for Dermatomyositis. It wasn't thought to have any effect on DM before, so we changed. Emily is a little nervous, but I'm actually a little excited. We are pushing the envelope, but lately she's had more days where something hurts than not, and that isn't how it is supposed to work.

As one last little shocker, once we moved to our room and got settled the nurse announced that she was going to briefly stop the IViG and give her a round of Rocephin, a good all-around antibiotic. At first, I was very surprised, wondering what I missed. It was a very long day. I realized that Dr. E likely saw the CT scan results from this morning, saw the inflammation, and decided to tackle it. This is one of the reasons why we love her! She isn't afraid to treat the WHOLE child. While some doctor's only take on little pieces, she takes over everything that she can. And this is why we would rather drive two and a half hours from home instead of drive an hour away.

We shall see what tomorrow holds. It will be today by the time this posts... Hopefully we will also know the results of the sweat test no later than Wednesday. Praying that tomorrow is an easier day!