First post of 2016! Update

I know, it’s been forever since I’ve written.  So long, in fact, that I don’t even consider myself a writer at this point.  I’ve had so much going on outside of the kids’ health that I just fell out of practice.  So, a quick catch up.  I’m kind of excited!

Zach is doing pretty well right now.  They determined that he had a pannus, which is a large pocket of inflammation in his C2, the second vertebrae down in the neck.  The meds he is on have reversed the pannus.  He still has some residual damage that makes it stiff often, but he is doing much better than he was.  Three month’s worth of physical therapy helped him to regain quite a bit of range of motion.  Otherwise, he’s a pretty happy kid!

Emily was thought to be in remission, but now we know she wasn’t.  With her disease, she does not have any characteristics that would point to any joint destruction.  Most people with MCTD don’t have destruction if I remember right from my researching.  She does.  Her jaw is a mess.  We meet with a TMJ doctor this coming Tuesday.  We are anxiously awaiting it.

We have some work to do on her.  One thing that has been driving me crazy about her is that when you suggest she try something new, or that she has tried before, she will tell you, “no thanks.  That doesn’t work.”  That is the response for everything.  I have been trying to figure out how to help her, but it has been out of reach.  I changed my major in school partially so I could better help her, but naturally, it will take years to get where I can help her.  The other problem has been her anxieties.  I have finally realized that they aren’t going to just go away.  In fact, if anything they are getting worse.  We chatted with a med student a couple months ago who told us how he battled terrible anxiety for years, but he eventually overcame it.

I decided that we needed to take the plunge and try psychology again.  Our last psychologist was very sweet and I liked her a lot, but she and Emily never really hit it off.  They didn’t click, and we made almost no progress.  After a year and a half she was still stuck on Emily’s sleep habits.  Well, perhaps the problem is partially with her anxiety, and by addressing that we will make progress.  Our GI doctor suggested we try the psychology department at her hospital.  Even though it is so far away we decided to jump in.  We had her first session yesterday.  Emily was immediately comfortable with her- enough to let me leave the room!  That’s huge! 

The other big things I have been contemplating for a while.  It begins with a visit to her pain management doctor last week.  We have been anxiously awaiting this appointment for a while because Emily decided that she wanted to change her sleep medicine to an anti-anxiety medicine.  She almost didn’t speak up, but fortunately, I did when I saw the opportunity slipping away.  I told Em that this was for her, and if she wanted change then she needed to ask for it.  She cried a little because she was nervous, and I think because she had her hopes up, but it worked.  She will be weaning off of her sleep med and starting Lexapro by next Thursday.  I am really hoping this will help her as much as it has helped others. 

Also, one of our rheumatologists had mentioned that we should ask our pain doctor about her intensive therapy and pain program.  It’s hardcore- an hour of physical therapy, an hour of occupational therapy, and an hour of cognitive behavioral therapy DAILY for 4 or so weeks, along with a family meeting with all of the doctors and therapists weekly.  I wasn’t sure how we could make it work before, but I have decided after watching the video on their site and talking to people that we NEED this.  The cognitive behavioral therapy will help readjust her mindset.  That alone is huge.  I feel this could be life-changing.  We have an evaluation next week for it.  I am hoping we can start the program right away. 

We are watching her digestive system.  They feel she has an unspecified esophageal dysmotility, but we are hoping it is maybe from her previous muscle relaxer.  She was switched to a new one that shouldn’t interfere.  We are hoping that helps.  And that pretty much sums up most of it.  I will try to not write so infrequently. It’s so hard since my Dad passed to even get on Facebook.  There is so much negativity and pain that I just cannot handle right now.  I’m in a good place, but only because I forced myself to be.  Aside from being sick this week, I’m doing pretty well, all things considered.  I could use a long vacation and no bad news for a while, but I know I can’t win them all!

     

National Drug Facts Week- Medicine Safety

As a professional patient (or caregiver), some of the most important things that you have to worry about are medicine related.  How many of you patients out there know the side effects of your medicines?  How many of you understand what your medicines do?  Do you know what to do if there is a problem?

-Side effects-  Any pharmacist will tell you that you should read the leaflet that comes with your medicines every time you refill!  A reaction will not always happen right away.   Take antibiotics, for instance.  Most of my (many) sinus infections, rounds of strep throat, tonsillitis, and bronchitis were handled by the antibiotics of the penicillin family.   Suddenly, when I turned 21 I had a raging reaction.   Antibiotics aren't the only class that may react the same way.  Over several years of use, Plaquenil (hydroxycholoquine) can destroy your retinas.  It does not happen overnight.  I am sure there are others.

-What to do if you have a reaction? - If you are able to isolate it to one medicine, do not take another dose until you call your doctor or your pharmacist first.  While the doctor's should know and understand side effects from their years of experience of writing prescriptions, pharmacist's understand the science behind them better.  They can also tell you if any interactions exist between your medicines.  For instance, many acid reducers cannot be taken with other medicines.  Many medicines should not be taken with vitamins, like thyroid meds, which tell you to wait four hours before taking vitamins.  Sometimes, one medicine may decrease the effectiveness of another, or may not mix well.  It is always a great idea when you have a new medicine thrown in the loop to ask your pharmacist about interactions.  Also, please be sure to tell your doctor about ALL of the medicines you may be taking, including vitamins.    If you are a drinker or imbibe in recreational drugs, you really need to tell your doctor (and STOP THAT!).  One of your medicines may really react and kill you.  That would be a very dangerous gamble.  It is very important for your doctor to know what you are taking.  Sometimes it is very difficult to keep track of your meds.  We began making up lists.  We take them everywhere we go.  We bring multiple copies when we have our hospital days.  Here is a sample:

































-Understand your medicines- My daughter was very young when she first became ill.  As she grew and we discovered more about her diseases, we tried to explain them to her, in an age-appropriate way.  She did NOT like taking medicines, and she would often flat-out refuse.  The way that I was able to get her to take her medicines was by explaining to her what they did.  I would ask as many questions as I could about her meds, I read the leaflets, but I also took it a step further and did my own research on them.  I would take what I learned, and then tell her how they were supposed to help her.  If she didn't want an antibiotic,  I would point out that would help her to feel better, and make her not so sick anymore.  At the same time, you need to know how to take your medicines properly.  Should you space them out exactly?  Do you have to avoid certain foods, like broccoli on blood thinners?  What happens if you accidentally take an extra dose?  Do you have the number for Poison Control handy?  (If you don't, it's: 1 (800) 222-1222)

-Use a pill tray, but double check it.-  We make sure to use pill trays in this house.  There are various trays out there, some for one day, some for a week, some with AM/PM, others have 3 rows.  The Arthritis Foundation  has easy-to-open trays for those who have problems with their hands. Pill trays keep you organized, and help you to know that you have taken your medicine.  I often have those moments of, "did I take my morning meds today?"  A pill tray prevents me from taking them twice, or at least helps me to not mess it up.

I don't think that I mentioned this on here before, but right around Christmas we had a big scare.  Emily has been on Nifedipine for years for her Raynaud's.  It had gotten so bad that her quality of life was greatly diminished because of it, and the Nifedipine was a perfect addition with the high doses of Prednisone she was on back then.  (Pred raises your blood pressure.)  Eventually it stopped working so well, and they doubled her dose.  Our pharmacy changed the appearance of her Nifedipine several times in a six month period.  One month, they made them white.  Since about half of her meds are white, she did not notice it when she filled her tray at her next refill.  The new bottle went back to mustard, so she didn't even think about it until after she took it.  Thankfully she did realize it.  I spent quite a while on the phone with pharmacy, our on-call doctor, poison control, and then finally at the ER, who also called Poison Control.  We hung in the ER until 6 A.M.  Fun times!  At least there she was well monitored.  One of my biggest worries was that she would fall asleep and not wake up.  
As wonderful as modern medicine can be, there is always a risk if it is not taken appropriately.  Please make sure that you are taking your medicines properly, and educate yourself on them.  

Some handy resources are: American Association of Poison Control Centers,  Medical Prescribing Reference, found in doctor's offices all over, and Drugs.com. Our doctor's also use this.  Also, the American Recall Center would like you to think of them.  They are a great resource!  They also wanted to make everyone who is on Xarelto, a popular blood thinner, aware of problems that have recently been reported.  They now have the  Xarelto Side Effect page.

All is Well!

Oh, for once I can say that it's wonderful to not be writing much!  I write much more when things are going wrong.  Lately, everyone is doing pretty well!

I truly feel that all of the work that we put into getting Emily on growth hormones has helped tremendously.  She has been so much better.  In fact, we are supposed to try stretching her Remicade and IViG infusions out from every four weeks to every five weeks!  I am so excited about that!!!  We have waited a couple of months to make sure she was doing as well as I thought she was, and I feel pretty confident now.  I got her a gym membership.  Now all I have to do is drag her there with me.  LOL!

Zach is doing well.  His asthma started flaring, but at least we have everything we need for that.  He had one month where his joints were red and swollen, but they have evened back out.  His rheumy (who is leaving us!) mentioned that we would likely be asked by the other members of the group to decrease his meds when the warmer weather comes.  In sunny Florida, we also have very high humidity levels.  They can be overwhelming for some things, but it's good for psoriasis, asthma, and Sjogren's!  Hopefully around March we will drop his Methotrexate injections, and then consider dropping his Enbrel to once a week instead of twice a week.

My oldest and my hubby are doing well.  :)  I've been plodding along.  I had quite a flare for about 2 months, but I think I am past it now.  I've been hanging at the gym because it makes me feel better.  I just finished one of my easier semesters at school, but next semester is going to be hell.  I will likely not write at all through the next semester.  Things are good, though.  Eyes are good, lungs are good (except for the asthma), and the kids are happy.  We just finished our homeschool year, so the kids are on a short break until the new year.  Life is good!

Lastly, Emily turned 13!!!  She had a nice birthday.  We celebrated over 2 days; one day was quietly spent at home, and another day we went to an amusement park for the day.  We all had a great time!  So, now to change the blog name.  Shall I change it to "My 13 Year Old is an Old Lady Who Turns Purple" or "My Teen is an Old Lady..."?  Decisions, decisions.  It may be hard in a google search if I change it to teen, but then I won't have to change it again for another seven years.  Thoughts?  Let me know!  :)  I hope y'all are doing well!

Stable!

I know- it's been forever since I have written. Life has finally evened out, but for a little while it was so odd and difficult that I didn't really know what to say. We kept having more problems with no reasons, and I just simply didn't feel positive enough to post.   Emily was really doing poorly, and I didn't really have any good answers.  I knew that there had to be something that was being overlooked.  The problem with having so many specialists is that they are just that- specialists.  ENT doesn't know much about gastroenterology; endocrinology doesn't either.  To get everyone on the same page can be a real challenge that requires quite a bit of persistence.

I'm sure some of you were thinking, "what the heck is she doing?" Or, "does she want the kids to have more doctors?"  Totally not. It almost drove me crazy. What we did do is take a chance. And it seems to be working. 

We got ENT involved. We had to subject her to another sinus culture, which she actually wanted. She wanted that crap sucked out. What we found is that she did not have a year-long sinus infection; it was all due to reflux. The ENT suggested we use a hyper-tonic saline, & follow-up with GI. So we got with her gastroenterologist. They scoped her. They think she may have EoE, eosonphilic esophagitis, but while she is still on steroids we won't have an accurate scope. They suggested we change her Prilosec to twice a day, which I had wanted anyway.  She is now doing so much better!!!  She hasn't really been "sick" in a while, and she can eat. Hooray!

The other thing that y'all may have thought I was crazy about- growth hormones. While I hate having to give her a shot nightly, this has been such a blessing. She never agrees with me when I say this but she seems to be so much better. She seems to feel less pain, which is a big reason we wanted to start her on the GH. It also doesn't hurt that she is finally growing, after years of little to no growth. It has truly been a Godsend. I had to be pushed into it, and I am so grateful to those that pushed and helped me to research it so I knew what to look for to get the ball rolling. Next step is a gym membership so they can get a good daily workout, hopefully with other kids. 

Emily had an amazing time at camp!  We are so grateful to her camp and the Arthritis Foundation. She really loved her cabin mates this time.  It is such a huge blessing to see her happy and shining. 

Zach is doing better than most, but he was warm and slightly swollen all over at his last appointment 2 weeks ago. All of his pertinent labs looked good, so now we are just watching him. Overall we can't complain. He's been on Enbrel since February 2011 (I think), and holding steady. The weekly methotrexate really seems to help. He's one of the lucky kids that has no side effects. 

Tomorrow we see a new opthamologist. Our rheumies want both kids checked for Sjogren's symptoms. Emily's labs are positive for it, and both kids have had dry eyes. Hopefully that will be good.  Overall, things are good!  I love quiet and boring, but it certainly doesn't lead to a productive blog. We have simply been enjoying it. :). I'll post as things happen, but for now we are enjoying the quiet. <3

The GI appointment

Before I begin my post with our news, may I ask you to pray for our friend Parker and his family?  Parker is having a very, very tough time right now.  He needs as many prayers, positive thoughts, or anything else you can throw his way, as he can get.  His family needs some peace and strength, too.  You can follow his story here.

Okay, so when Emily had her swallow study a couple of weeks ago, I knew it wasn't right.  For a minute there I thought they were playing with the camera.  I realized that more comes up than goes down when she drinks.  I actually cried watching the camera, thankful that she couldn't see me.  She was standing up, swallowing hard, and still nothing would clear until she had some water.  Not what I wanted to see.  

Because our rheumy group is amazing, they set up an "emergency" appointment with a new GI doc.  The nurse practitioner emailed me about that Wednesday, and by Thursday we had an appointment set for the following Monday.  I worried a bit about how the doctor would be, but when I pulled up his profile I saw that he was the head of the department for feeding disorders, and he writes the curriculum for the school that his office is affiliated with.  

The first thing that the doctor asked me after he introduced himself was, "how can I help you?"  For just a minute there, I was worried.  I explained that I just want her to be able to eat.  He said that he had already looked through her file.  He asked me if anyone had mentioned a problem artery to me.  Ummmm, no.  Apparently, she has what they call an "aberrant subclavian artery".  This means that there is an extra artery that snakes around behind the esophagus.  (Below is the exact picture the doctor showed us.)  This by itself can cause all sorts of really awesome problems like chronic respiratory infections and problems swallowing.  The NIH Rare Diseases page  says this about it: 

 Aberrant subclavian artery is a rare vascular anomaly that is present from birth. It usually causes no symptoms and is often discovered as an incidental finding (such as through a barium swallow or echocardiogram). Occasionally the anomaly causes swallowing difficulty (dysphagia lusoria).[1][2] Swallowing symptoms in children may present as feeding difficulty and/or recurrent respiratory tract infection.[2] When aberrant subclavian artery causes no symptoms, treatment is not needed. If the anomaly is causing significant symptoms, treatment may involve surgery.[1][2] Children with symptomatic aberrant subclavian artery should be carefully evaluated for additional vascular and heart anomalies.[2]

So, because that's never quite enough with us, he also points out that just because she has that doesn't mean it is the culprit.  It is likely from the scleroderma side of the Mixed Connective Tissue Disease, but it could also be gastroparesis, which is where the stomach doesn't empty properly.  For more info on this, I went to my trusty friends at NIH again.  

How can we distinguish which problem may be causing this?  More tests!  This was one of the two things that we somewhat expected.  Em & I had talked about how she would probably need another upper endoscopy so they could really figure it all out.  (We assumed it was from the esophogeal separation, and would need to be stretched with a balloon.) Not only does he want that done, (minus the balloon) but he also wants a different type of swallow study done.  Instead of drinking barium, they have you eat something like eggs with radiation.  (UGH!!!)  If we didn't really need her to eat I would be against this, but we have to figure out what needs to be done.  Hopefully she will have these tests with her next hospital overnight.  We go back on June 2nd & 3rd.  On June 4th we have her endocrinology follow-up; it will be the first one since she started the hormones.  

She is still having some wicked headaches, but we think that they may be from the sinus issues.  Tomorrow she will be heading to our ENT's office for a sinus culture and suction under anesthesia.  We are hoping he may have some answers for this.  I feel like she may need a prophylactic antibiotic.  She is sick more than she is well.  Perhaps that won't be possible with the artery issue.  Maybe she is just going to have to deal with being sick constantly forever because of it.  I don't know.  I discovered that if she has gastroparesis, sometimes low doses of erythromycin help.  I hope she does not have gastroparesis, but if she does maybe we can kill two birds with one stone there.  Hopefully by having her appointment now, her IgG levels will be high enough to not knock her back, and there will be enough time between this procedure and her endo appointment to really be able to tell if the headaches are sinus or hormone.  We are also hoping that her levels are staying high enough consistently for them to stop her IViG (platelets via infusion at the hospital).  That would eliminate the overnight hospital visits, and really make our lives much easier.  We shall see, I guess.  We are staying hopeful that we are on the right path, just with our usual bumps in the road.

Zach is doing well.  :)  For that we are very fortunate.  I will update more when we know more, or to point you towards Parker's page for his next update.  Please pray for all of their family and ours?  Thank you.  

Picture credit:  Sonoworld.com

World Autoimmune Arthritis Day!

Greetings!

I would like to invite you all to come join me for a live blog chat/ interview from 7 P.M. to 8 P.M. EST for World Autoimmune Arthritis Day.  Because it covers several countries and time zones, the event starts on May 19 and runs until May 21st.  There will be many, many features.  Here is a little intro video for it.  Here is the official YouTube event video.  

This link brings you to the home page for World Autoimmune Arthritis Day!  The page puts it this way:

 "Have you registered yet for World Autoimmune Arthritis Day 2014 (WAAD14)?  WAAD14 is 100% ONLINE, which means you can join the convention from anywhere there is an internet connection.  It is designed for ALL people to attend, regardless of location and physical lim itations- all you need is a computer.

The convention site is built just like you are at a physical convention site, with booths to visit, Exhibition Halls, live Presenters (via "Live Chats"), and specialty features, such as this years' major event: A Day in the Life of an Autoimmune Arthritis Patient where patients can invite supporters to 'walk in my shoes for a day' by participating in challenges that mimic those faced by patients on a daily basis. " 

I was a part of this event two years ago.  This non-profit was brand new back then.  They have come a long way!  The founder, Tiffany, realized quickly after her initial diagnosis that people did not understand autoimmune arthritis at all, and that everyone seems to equate it to osteoarthritis.  While the two couldn't be more different, it takes educating people to make them understand it.  This reason is also why I blog.  It started out as a way to vent, until I realized that I was helping people.  I can assure you, that is NOT what I expected, but it's pretty cool!  :)  Anyway, please join me!  If you can't join me, there are many other people who are also doing live chats and interviews.  You have the opportunity to have questions answered.  It's volunteer run, and really has become quite the event.  Please join us if you can!  The registration info is below.  I hope that you can join us!

$5.00 USD Pre-RegistrationEntrance Fee to Live Event

May 19^th 6am ET/USA-May 21^st 5am ET/USA

& Access to Booths & Resources through May 31^st

$7.00 USD Entrance Fee During Live Event

May 19th 6am ET/USA-May 21st 5am ET/USA

& Access to Booths & Resources through May 31^st

FREE to Skip the Live Event

Access to Booths & Resources from May 21^st at 5am ET/USA through May 31^st

Our last hospital visit. Ugh.

I've had a few days to ponder and mull now.  I still feel a little numb, and just exhausted.  My Facebook friends know most of this, but I didn't want to leave anyone out.

I had emailed the nurse practitioner at our rheumies office last Monday, in preparation of our visit that Thursday and Friday.  She spoke to the doctor, and we all agree that she needed a new swallow study done.  Emily had told me that she feels like there is a bubble in her throat when she tries to swallow.  Hmmm.  Since she had started to get stuffed up again (on antibiotic number 2 for the month), I mentioned that, as well as the headaches she has been having.  I assume they are either from the sinus infection from hell or maybe from the growth hormones.

I wasn't going to tell her about the upper GI until right before.  I asked for Xanax for her.  She never uses that stuff except for medical testing.  She just freaks out.  It's bad.  I guess that everyone thought I was overreacting.  Nope!  It was supposed to be outpatient at 11, but knowing how difficult it would be to get us out on time, they made it inpatient at 11:30.  She woke up on Friday morning to the nurse telling her it was time to go downstairs for the test.  She got herself so worked up that she almost hyperventilated.  She cried for a good hour.  She didn't calm down until someone finally got her a 1/2 a Xanax, but it was too late by then.  She was rescheduled for 3:30, though no one told the scheduler downstairs.  We were discharged at 1:30, so we had time to kill.  We couldn't really leave, and since she couldn't eat, we tried to avoid all of the food places.

They told me to give her another 1/2 Xanax around 3.  Despite having one full Xanax in her, when they took her back she still had another hour meltdown.  I can't blame her.  She has no control, no say in any of this.  It sucks.  If I didn't suspect it was bad, I never would have pushed.

After she finally calmed down, she worked with them to help herself.  She asked to be slightly elevated.  I watched the barium start to go down, then back up, several times.  At one point, I thought the camera was showing something else, or glitching.  Nope.  What it basically showed is that she has, as the nurse practitioner emailed it, "Her study showed severe reflux and little motility of her esophagus."  Hmmm.   I suspected as much, but it still hurts to see it.  At the moment, that is really all that I know.  This is in conjunction with her patulous esophagus, which if you missed it, means that the muscles at the base of her esophagus have dilated and spread away from her stomach.  .  Well, this explains why she doesn't eat.

At the same time, she has spent 30 days on Augmentin and 10 days now on Clindamyacin, and she is still sick.  She had an x-ray last week that showed she is still gunked up, so the ENT is working on getting an authorization from rheumie to get this expedited so they can culture her and suck out the junk up there.  I really, really think she needs to be on a prophylactic antibiotic.  Then maybe we could stop IViG, and maybe not have to sleep over the hospital every month.

But, wait!  There's more.  I have noticed for the past couple of months that her ALT (muscle enzyme) has been a tad elevated.  I'm no longer the one to panic at the first sign of trouble, so I've just been keeping an eye on it.  However, Monday her thighs hurt badly- like a 6-7 on the pain scale.  That could mean that her dermatomyositis is also coming back, which would mean adding Prednisone back in.  We really, REALLY don't want to do that.  It makes me so sad.

If all of that wasn't enough, they ran her cortisol levels on Thursday morning before she had any of her hydrocortisone.  A normally functioning adrenal system will have a count of 10 or higher.  She was only at 1.3, so the hydrocortisone stays for now.  And the final kicker was the email from camp that she is on the wait list for both sessions.  At least we had a few good months.  Hopefully this will all pass quickly.  I guess we need to find a set Gastroenterologist now.  We've seen a couple before, but mostly as favors to our rheumies.  We don't really have anyone set.  Hopefully endo will be able to help her more with the cortisol, and ENT will see her fast to help her sinuses.  Please pray that all of this comes together just right.  Thank you.