Tuesday, May 29, 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 the inflammation being in my ribs.  My body thinks it is healing the bad joint by replacing it with bone.  If it gets too bad, my lungs will not be able to expand because the ribs won't be flexible enough to allow for that function.   My doctor is concerned I have already lost some function in my lungs so I have to go for a baseline test. 

It's annoying and frustrating and I really don't have the time or patience to put up with this; not now, not ever!  I came home from my appointment and found out my credit card number had been stolen and they racked up a bunch of charges and a letter of denial from my insurance company on a couple of recent claims that are well over $1000.00.  I will be fighting with them of course, it's just nonsense.  I pay $800 a month for private insurance with an $11,000 deductible and co insurance, you would think they would cover something!! 

I need to meet with the physical therapist and see what he thinks and then I will have to assess my cleaning jobs and see if there's anyway I can retire from cleaning to try and save my back.  I'm pretty sure I can't since that is pretty much the only lame income I can count on.  Days like this just make me want to throw my hands up and run away.

Sunday, May 27, 2012

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 anytime her skin is irritated, as in the case of getting an IV started, hives are the end result. 

I am glad to have this week behind us.  Now, we plan for 3 bone marrow biopsies.  Incidently, our Oncologist's nurse called me this week to let me know our Oncologist in Seattle wanted to make appointments to see us.  I just don't have it in me to make that trip; not financially, not emotionally so we will stick with our plan to do the biopsies right here at home...well, at the hospital. 

Friday, May 25, 2012


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 manage with advil for pain management.  It's hard to say if it was the rate of infusion or the immunity in the plasma.  Some people have said if you don't get the side effects then it really isn't working.  Who knows. 

She probably overdid it a bit today.  Tonight the headache is getting worse but still hoping that it doesn't get unmanageable.  If you ask me, I think that her dysautonomia creates a lot of the problems.  Her autonomic nervous system just has a hard time regulating itself to new things, like the IVIG.  We have also found that just getting an IV makes her break out into hives and that has been a problem.  They are spreading up her arms and really itchy.  No vasculitis at this point so all in all, it's been a pretty good transfusion this time.

Shelbie really needed a break and I am so glad it finally came.  I just hope things continue to get better rather than worse. 

Tuesday, May 22, 2012


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.  I have taken many CPR classes.  Like the back of my hand, I knew how many chest compressions to each breath and the rescusitation dolls were easy and familiar for me.  The first time I had to actually use my knowledge, I froze.  I was terrified and all of sudden, I was in a foreign land.  Everything felt different on a human being than a plastic dummy and no one prepared me for the rush of adrenaline that would make me dizzy and disoriented.

That's how I feel these days, dizzy and disoriented.  It's been such a tumultuous year, the wind has been knocked from my sail.  Sometimes in one week, I can go from crisis to isolation to anger to reconstructing my life to depression and then renewal but only for minutes sometimes, until the next crisis hits.   Time becomes distorted and survival is the main concern. There is no expectable future and that makes it hard to plan anything, even a few hours from now.  It's hard to work with all these emotional distractions.

In some ways, we've established new routines over the past couple of weeks as both the boys are doing better now that their medications are both working better for their inflammatory bowel disease.  Like Humpty Dumpty, the King's men and all his horses too have been working round the clock to put us all back together.   Despite this period we are in, I can feel the next crisis arriving and I hate that feeling. 

This week, we give the IVIG one more try.  The whole process is going to be different on Wednesday.  They had to change up the pre meds since they are complicating side effects and putting her in a precarious position.  I'm not sure what we will use or what to expect.  We were unable to get a different brand of plasma or a different concentration so we will try to infuse over 8-10 hours rather than 4-6 hours.  The doctor's hope, and mine, is that the slower infusion rate will eliminate the horrible side effects like Vasculitis.  This may be our last attempt, especially if things deteriorate like they have the last two months.  We have to get a handle on this adenovirus.   Immune compromised people have died from this virus and if we can't eliminate it all together then we have to at least keep it knocked down but so far, the IVIG isn't working as well as it use to. 

I don't know what to expect, or what to plan on this week.  The only thing I do know how to do anymore is rely on God and have hope that this time will be better.  I have faith that we will find solutions but I know that doesn't mean it won't be without some trial and error, tears and frustration because that's just the way it is in the world of chronic illness.

Sunday, May 13, 2012


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 a sad, sad life.  No one would want to come near us, it would be so depressing so we go alone, trying to protect everyone else from the reality of our life. 

I have thought about abandoning my blogs just for this reason.  This was suppose to be a place for me to journal about life with three chronically ill kids.   You don't see situations like this everyday.   I don't have a spouse or anyone significant to share these burdens with and sometimes dragging my fears and worry around all day, everyday gets to be too much.  It's just me and my thoughts which I mostly keep to myself except when I dare write them here.  Sometimes, writing from my perspective, from my heart, puts me on the hotseat.  People are able to form all sorts of opinions and judgements which though well meant I'm sure, are not easy to hear.  Misunderstandings emerge all the time.  Sometimes, that just adds more problems to the mix I don't have the energy to address the emotions of everyone else around me let alone my kids.

I guess it's just the nature of chronic problems.  We are really good at dealing with acute problems, things with an end in sight.  We aren't good at the monotony of the day to day struggles and fitting into this world is a big one.  It's like a marathon that you were thrown into on a whim.  There is no time to train, to build endurance and stamina.  The starting gun goes off and if you don't start running, you will surely get trampled.   I guess that's where all this rambling is going.  I'm just tired of the process and wish I had a little more experience in marathons under my belt.  I don't like being criticized about my form, my stride and my thoughts on running marathons.  It just gets old because really, if I had any say in the matter, I would have dropped out a long time ago, in fact I did try to drop out but this is where I am suppose to be, stumbling or not.

This week, a few people have suggested that things aren't as bad as I make them out to be.  One person eluded to the fact that they think my kids just have a sensitivity to some foods and if I would get on top of that, they would get better.  Apparently, it's that simple because that was the answer for them and their chronic fatigue.  I wasted so much time this week just in second guessing myself as a mom to these kids.  It's been sort of frustrating. 

Anyways, despite these communication setbacks with people who I really shouldn't be giving any of my time or emotion to anyways, I am glad for the little breathing room and stability.  I should find out this week when the kids will have their bone marrow biopsies and a different way to tackle this adenovirus of Shelbie's. 


Thursday, May 10, 2012

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 years that contributes to the, 'all is well' kind of story that is completely fabricated of course.   Sneaking up from behind me, came the most horrendous, huge and uncontrollable crocodile tears!  I wasn't expecting it and from the look on the lady's face, she wasn't expecting it either!  How embarrassing!  Her husband said, "Here, just give me your list."  What a wise man, leave the scene of a crying lady in the bread aisle and move on to the vegetables. 

I was apologetic and tried to pull myself together again.  "Weird things happen when I am both tired and in pain." then trailed off with that awkward chuckle as I tried to hide my red, blotchy face from the other unsuspecting shoppers on a Tuesday afternoon.  I don't like sounding so negative so then I back tracked through my sugar coated words and said, "Really, everything is great!  I got a couple of great little jobs this week, I got paid for a job I finished a long time ago and I am playing the organ in the Temple.  So many wonderful opportunities, it's just great!"

Thankfully, we were both able to escape a very awkward situation.  It's really weird how we try so hard to look good, we try so hard to feel good or make people around us think we are feeling so good but when a moment comes along and the reality of our life spills out unexpectedly, I realize how much energy we expend trying to be normal, trying to fit in and not scare anyone or appear too negative, needy or sick.  It's a hard thing to balance.  I have many more thoughts racing through my mind but perhaps it's better saved for another time.

Monday, May 7, 2012

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 these days and leaking veins from the vasculitis, I wasn't sure what we would be facing. 

Thankfully, there were no broken bones, just severe bruising and tons of pain.  It is ugly!  We have to be careful about infection now since her immune system is barely hanging on and two full days, later, they are still bleeding!  I don't know what to do.  I guess I will call the doc tomorrow.  That darn vasculitis! 

So, her little piggys are not feeling so well and they will not be going to market anytime soon.


Sunday, May 6, 2012

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 kidney.   As long as it hasn't grown too much or better yet has remained stable, I get another year before rechecking things.

Most people don't know that I suffer from a degenerative arthritis called Ankylosing Spondylitis.  Simply put, the joints in my back, hips and neck are turning to bone.  Eventually, if it doesn't go into remission, I will be in rough shape with little to no flexibility and possibly a wheechair.  I stopped going to my Rheumatologist a couple of years ago because I couldn't afford the treatment he wanted me to do.  $1800.00 a month is a little high for my modest income.  My last visit, I was told that my spine was already fused just over 3" and inflammation was increasing in my neck and ribs.  My risk for cancer, because of the excess inflammation is more than triple the risk for the average joe.

I have tried really hard to control the pain with diet and exercise.  I don't even take Tylenol or Advil.  The disease progresses when I am not moving.  The last few months, it has been almost impossible to get away from the pain.  Exercise actually makes me feel worse and by the time I finish cleaning houses in a day, I can barely walk and breathing becomes more challenging because my rib cage doesn't seem to be expanding like it use to.  I can't sit for more than an hour and even at that, it takes several minutes for me to stand up straight and walk normal.  Some mornings when I wake up, it takes all I have to roll out of bed.  Sitting up, is totally out of the question so yes, I literally have to roll out.  I am a little worried about how much things have progressed, even in just a month. 

I decided I will check in with my Rheumy and see if there are any new treatments that may be more affordable.  The past two weeks I have hit a special diet hard and to some, it may seem extreme.  It's basically huge portions of vegetables and fruit, mostly raw.  I eat nothing processed, nothing refined, nothing with fat unless it's canola oil or olive oil. For me, it's not too far off from what I normally eat but I have been missing my Pepsi, Gummy Lifesavers and chocolate chip cookies, the three things I indulge in. 

 I have actually had an insane craving for tomatoes.  Here's a moment of truth, when I go to a Mexican restaurant that brings salsa and chips out to the table, I drink the salsa- hopefully while no one is looking!  It's like people who eat chalk or dirt, I drink salsa.  I drink it for breakfast, snacks, and every other meal.  It's sort of out of control but I know it has something to do with what is off in my body.  Hopefully, the doctor will have some good answers.  It would be devastating if something happened to me since I am sort of a one man band around here and I'm not ready for a wheelchair.

Finally, if I don't feel beaten down by the end of the week, I will go see the dentist!  I HATE the dentist!!!!  It costs a fortune!  I hate the lectures they feel obliged to give you and all the questioning; like you have just assaulted the tooth fairy...do you floss? How many times do you brush?  You're brushing to hard, you aren't brushing hard enough, don't you want to keep these teeth forever?  Sometimes, I just want to tell them first off "I don't care what you have to say to me, just fix the 9 cavities and be quiet!"  9 cavities is probably a conservative guess- It's been nearly 10 years since I went to the dentist, well, with exception of an emergency wisdom tooth extraction 3 years ago at 11pm one very painful night. 

So, as long as the kids maintain the loose holding pattern of 'good' health they are in, this is how the week will go.  I will also be praying like crazy that there is nothing horribly wrong. 

Thursday, May 3, 2012


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 is the Emergency Room for IV Morphine.  He said we could try narcotics but the last thing we need to deal with is an addiction to pain meds; I don't even want those kinds of drugs in my house.  I declined the prescriptions so I guess we will just plan on an additional maybe two hospital trips starting 36 hours after transfusion.

The other problem is that the IVIG hasn't been working to knock down that Adenovirus she has had for a solid year now!  I didn't realize how dangerous that virus is and there are only two treatments that offer any hope for killing it.  One is IVIG and the other is an IV medication given a couple times a week for a few weeks.  He wanted to start it in a couple of weeks but the side effects are worse than aseptic meningitis.  He had a transplant patient last fall who contracted Adenovirus and the sent her to University of Utah for the IV treatment.  I asked him how she did and if the treatment helped or worked.  He hesitated then said, "Well, unfortunately, she passed away."  UGH!!! Scary!!!  There is also a little boy in the UK who has been clinging to life because the adenovirus is in his lungs.  He's been on a ventilator etc.  This does not sit well with me! 

How do you choose between those two options?  Shelbie decided to stick with the IVIG.  The doctor is going to look into trying a different brand or running it at a different concentration but that means the transfusion will take up to 12 hours instead of 6 or 7. 

We have started to schedule bone marrow biopsies the week that school gets out.  I just want to get that done and out of the way.   We are about 6 months late in getting it done!  We are going to stay here instead of going back to Seattle.  I just can't stand the thought of another 14 hour drive.  Here, they all get a bed in the same room.  It's so much easier on me to be able to have them together in one room rather than running back and forth down the hall to check on each one.   Easier for them too. 

Big SIGH!!!  Why doesn't this surprise me?  Who knew we could have high platelets but petechiae anyways?  Her red count is low so hopefully as the inflammation goes down, her counts will recover.  Onward....

Wednesday, May 2, 2012

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. 

Today, I read my post again and surprised myself.  I really sound like I have it all together; an old veteran at the Shwachman thing.  Here's an excerpt from that post:

" Don't let SDS redefine your life, your marriage or your daughter. It's so easy to 'become' the disease but don't let it rob you of everything. I hate SDS but there is one thing that I can thank SDS for- we cherish every moment, every milestone. Life is richer than I think it would be otherwise. We don't take anything for granted. My kids love each other deeper, I love them deeper, they are my greatest joy! When we laugh, we laugh hard and we laugh together. When we cry, we cry hard and cry together. Somewhere in all the tears, the strength of a parent is born. Your shoulders become broader to carry this burden and your heart bigger, not just for your family but all those who suffer, so love the tears and sorrow as much as the joy. "

It's true.  Everything I said is true but I didn't really remember until I re-read my thoughts.  I do hate Shwachman Diamond.  I hate everything that it has taken from me and will eventually steal away but there are many blessings that have come from the ashes and sorrow.  We are closer than I imagine a family can ever become.  The smallest, insignificant thing is a thing to celebrate.  Everything in life is in High Def; even the sadness.  The sad moments seem to hurt more and run deep but so do the joys.  Every little, happy moment is one to embrace.   I don't like having to take the good and the bad, I just want it all good but that is a dream for another life, not mine.

There are treasures to be found in being a mother to children with chronic, life altering diseases but at times, you must do a little digging in order to discover the little gems.

Tuesday, May 1, 2012

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 count took a dive just 24 hours after infusion and naturally, her hemoglobin did as well.  I have been wracking my brain trying to figure you why she has petechiae. 

I think I have figured it out.  Vasculitis.  Vasculitis is inflammation of the veins and arteries.  It can weaken the cell walls so blood spills out, hence the petechiae.  It can be serious, even life threatening and in rare cases can result from IVIG, so we are going to the doctors on Wednesday to confirm it or for him to make a different diagnosis.  Whatever it is, it's not right.  I also read it could be that one of the plasma donors had TTP- another platelet disorder. 

I have a feeling I will be super angry if Shelbie has contracted another problem from plasma transfusions.  I guess I will wait for the doctor to weigh in.  Either way, it stinks.  She is just not getting much of a benefit anymore from IVIG, in fact, I feel like it is doing more harm than good so I am hoping we can take a break without risking her life with an infection. 

So many hard decisions to make.  It will have to be made with faith and courage.  Hopefully, after we meet with the Oncologist, we will have a better plan.

It's the Most Wonderful Time of the Year!!

29 gifts!  My traditional way to start the new year...give away 29 acts of service in 29 days is back in my life and week one is done! Eve...