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Showing posts from May, 2012


Today I had my follow up with the Rheumatologist.  I was hoping for a stable report at least but the xrays showed that the degeneration has hit my thoracic vertabrae.  That is awful news!  I was not prepared to hear that.  Not only Ankylosing Spondylitis a problem for me but as of today, I have confirmed Osteoarthritis in my back.  What a complicated mess!

With ankylosing spondylitis, moving helps with the pain and inflammation.  The more you move the better your odds of putting it in remission.  With osteoarthritis, moving and exercise can actually create more pain.  Arrghhhh, so now what?

Well, the assistant I saw today was not a lot of help.  Of course they want me on injections but the cost of that is well over $1500 so it's completely out of the question.  My only option in anti-inflammatory meds and muscle relaxers.  He wants me to start physical therapy and massage therapy but those too come with a hefty price tag. 

Next week, I have to go see a pulmonologist because of t…

As usual

As usual, I spoke too soon about the side effects from this week's infusion.  It seems the slower rate only prolonged the pressure and swelling in her head.  It hit really late Friday night and lasted throughout Saturday and even today, she hasn't been feeling all that hot.  Granted, we were able to avoid the ER but it was a nagging pain that was not to be relieved with anything!

I asked Shelbie what she thought about the longer hospital stay and she has concluded that she would rather do it over 6 hours and be slammed with a headache and get immediate relief at the ER than to drag on infusion day only to have a headache that doesn't get better with medication but isn't bad enough for the ER so it becomes something to endure for a lot longer. 

I can see that but sadly, there is no perfect solution.  As usual, she has broken out into huge hives that go from her hand to her neck all because of the IV.  We are just learning all the effects dysautonomia has on her and any…


Shelbie had her infusion on Wednesday.  Our doctor was trying to come up with some different plans to help eliminate some of the side effects she has been having the last few infusions.  Well, none of his plans panned out.  It's hard to know if the hospital pharmacy just didn't want to go to the trouble of ordering a new blood product in or there really was a genuine reason. 

Anyways, the final decision was to slow the infusion down even more than we have done it.  The average person can handle the transfusion being done over 4 hours.  Shelbie has been doing it over 6 and on Wednesday, they slowed it way down so it took 8 hours.  The company claims that side effects are dependent on the rate of infusion.  8 hours, though painfully slow, seems to have helped a lot this time.  So far, she is just experiencing pressure, not so much debilitating pain.  Today, when she would normally be laying on the couch with her head wrapped in ice, she was out getting things done and able to m…


We are in a really weird place these days.  I can't exactly put my finger on it so I guess I've chalked it up to the path of chronic illness.  I have attended classes on handling chronic illness, read books, scoured articles but I'm not sure that living a life like this can be taught.  You gotta feel your way through it, often tripping up, getting lost and caught up on snags you weren't expecting.

Chronic illness is all about stages, kind of like grief; in fact, I think it could be considered a cousin to grief, a very close cousin.  It's such an emotional process and that's what makes dealing with a life of chronic illness so hard, it's not textbook.  You can prepare all you want but until you are living in the midst of chronic problems and health issues you will have no idea what to expect or how you will handle each new setback. 

It's a bit like performing CPR.  Hopefully, you haven't had to do that yet in your life but I have, twice on Shelbie.…


This has been a quiet week as far as any major problems.  We have the same old issues hanging around but at least it's crappy stuff we are well acquainted with.   I have been trying to keep my distance from Shwachman Diamond Syndrome and Mitochondrial Disease this week.  They just frustrate me so much I just can't stand to give them anymore attention than they absolutely need. 

I have been keeping up with the support groups I am in on Facebook and Yahoo Groups.  It seems whenever I am struggling with something, someone else is having the same problem somewhere else in the world.  That is comforting and I don't feel quite so isolated, or maybe 'crazy' is a better word.  I know that there are probably numerous people who think I make all this up.  They probably don't get it and quite frankly, they probably never will.  "They don't look sick." Is one of my favorite sentences of ignorance.  If we walked around everyday the way we felt, we would have …

Two for One

Does it ever feel like you are getting two weeks of living for the price of one?  That's how I feel this week.  My poor kids, my poor me!  Every single minute of everyday has been crammed with something important to do; from 6:00am to 12:30am, I have been on the go.

Tuesday was pretty insane.  I had three doctor appointments back to back.  By the time I finished with my last appointment, I ran to the grocery store to pick up a few things to tie us over until I can go shopping for real.  I think it had been easily three, maybe four weeks since I went grocery shopping.  I was so tired and in so much pain.  For my last doctor appointment, I had to have a cortisone shot in my lower back - not fun. 

Right around the bread aisle, I ran into some people I know and they asked how things were.  I gave them the Disney version of life these days and that was going well.  I had my usual happy face on, my falsetto voice of 'just fine' and the little laugh I have perfected over the ye…

This little piggy...

Shelbie was in a super freaky accident on Friday night.  I had to drop off my laptop at a repair place out in the country.  When we got there, they were just finishing sawing up a huge tree that had blown down over their driveway.  While I was talking to my computer guy, Shelbie noticed that his daughter was struggling to lift a large log into the already heaping, full of logs utility trailer. 

As they through it in, the trailer slammed to the ground, landing on Shelbie's toes!  At first, I didn't compute what had happened as I watched from a distance.  She just stood there so I questioned what I had seen.  It took her over a minute to realize herself what had happened.  finally, I saw the horror and pain on her face and she was trying so hard not to cry.  I walked over to her and when I saw her feet, I too was horrified! 

After getting some ice, I rushed into town to the Emergency Room.  There was blood pouring out from her feet.  Considering the condition of her blood thes…

Taking care of business

I don't know if other mother's share my bad habit, but it seems that by the time you take care of everyone else in the family, there is very little time, energy and resources to take care of yourself. 

Well, this week, all that is about to change.  I have been receiving threatening letters from the Radiology clinic for the past three months because I am way past due for my mammogram and ultrasounds.  Seriously, about every three weeks, I get a letter saying, "Dear Ms.  We are sending this letter as a reminder that you need to schedule your ultrasound and mammogram.  Our records indicate you are way past due for this screening.  Given your history..."  They go on to tell me the risks I am facing by putting this off and mention horrifying statistics about women who die from breast cancer every year.   So, in an effort to stop the 'hate' mail, I'm going to take care of that this week.   At this visit, I will also check on the slow growing tumor I have in my…


Yesterday, Shelbie had her Oncology appointment.  He confirmed that she does indeed have Vasculitis.  I predicted that a couple of posts ago.  She has a lot of petechiae and purpura.  She had huge patches of purpura on her arms, it was sort of frightening to see, even for us oldies in the cycle of low platelets.

The doctor thinks that the IVIG is just slamming her body with inflammation, even causing her veins to become inflammed.  When this happens, the walls of the veins become leaky and blood escapes and pools under the skin.  It should resolve over time but more than likely, this will be our new normal with each transfusion from now on.  That's an awesome 'suck' factor but wait, it gets better. 

We have to change up her pre-med list because now the Toradol we use during the transfusion is aggrevating the vasculitis so that has to be discontinued as well as Naproxen for the headache pain.  That leaves us very few options and the only one that I will really consent to …

Sometimes I surprise myself

I am part of a list group of support for Shwachman Diamond Syndrome and Mitochondrial Disease.  I am able to connect will all sorts of families in various places on this rough and tumble road of life.  A couple of days ago, a Father introduced himself, his wife and 7 1/2 month old daughter.  Their daughter was just diagnosed with Shwachman Diamond.   They are in the horrible state of confusion, sadness, worry and most of all overwhelm.  I responded to his long email of questions and my response was equally long.  I found that as I kept typing, the fog from my mind seemed to burn off and I could remember clearly that fact that Shwachman's has not beaten me down for good. 

The last few months have been unbearable with suffocating worry over everything going wrong with all three of the kids.  To say I am tired and beaten would most assuredly be an understatement.   When I finished typing out my response to this family, I closed the computer and didn't read what I had written. 

Of Course...

We got genetics back on the POLG1 test....drum roll....shock and awe.... NEGATIVE!!!  Negative for one of the most common Mitochondrial diseases.  Of course it's negative!  Another dead end.  I talked to the nurse again today and asked her what we do now.  Well, we could continue with diagnostic testing which means a muscle biopsy or we just continue to treat it as clinical mitochondrial disease.  I'm leaning towards putting off the muscle biopsy for now, maybe a year or so.  

The advantage to having a name for this beast is that we will know the progession of the disease and there may possibly be other treatments that could be more supportive than what we are doing.  Keep in mind, there is no cure, no great treatment for Mito. 

It is so frustrating!  Shelbie has yet to bounce back from her IVIG infusion.  She feels crummy and sleeps alot.  She has now developed more petechiae and purpura which is strange since her platelets are holding nicely at 174,000.  Her red blood coun…