Sunday, April 28, 2013

One big day

What a big day today!

We spent the day at the hospital for 'Family Day'.  We spent the entire day listening to some of the top doctors in the Shwachman Diamond Syndrome Arena.

Our Hematologist, Dr. Shimamura was our host for the day.  She gave a great talk on SDS and some of the new research.  We then heard from a geneticist, a transplant specialist who I loved!  She has actually been working behind the scene for us.  Whenever the kids start a new treatment, the doctors visit with the transplant team to make sure we aren't doing anything that will cause any problems with transplant should we have to go down that road in the future.

There was also a GI specialist and a new Adult SDS Hematologist.  I was really excited to hear that Dr. Shimamura has teamed up with an Adult Hematologist to help transition her pediatric patients.  I was so relieved when I was able to visit with her and see that she has a wonderful bedside manner about her and that she is just as accessible as Dr. S has been and they will continue to work closely together on their SDS patients.  Her name is Dr. Keel.

Dr. Keel has a unique team put together.  She has all her adult SDS patients participate in a 'Coping Clinic'.  She works closely with a Neuro-Psychologist who helps young adults get through this difficult period of transitioning from pediatrics to adults.

My pen was going a hundred miles an hour as I took notes on all the talks and then wrote a million questions for the panel at the end of the day.  The kids kept making fun of me for monopolizing the Q&A time.

The other highlight was meeting 11 families who had SDS kids!  I got a little overwhelmed at one point.  It was surprising to me that most of the kids there were over age 15 so it was cool to see other kids who struggle with similar things my kids do!  I was glad to feel a small sense of belonging.  Like we were finally in a place where we were with people who understand what this road is like.  It was a room full of extremely amazing people; strength and courage!

There was one moment when a parent stood up to speak and her little girl started crying.  Dr. Shimamura left the panel and picked up the little girl and took her over to a big fish tank to entertain her.  When she needed to continue her talk, she continued to hold this little girl.  What kind of doctor would do this?  Not many if you ask me!  I was surprised that all but one of the doctors we met today were women.  They were all amazing, sensitive and smart!  We are really blessed and in good hands.

The transplant doctor reported that they are currently experiencing a 100% survival rate in SDS transplants!!!  That is totally amazing and gives me hope when I think of our unpredictable future. It was a great day, hard and tiring but great!


Saturday, April 27, 2013

Faith in Humanity

We have broken our trip to Seattle into two days.  Thursday, we got into my parent's house late and Friday, spent the morning with them.  My niece and her husband were there as well so it was a fun morning.  We headed into Spokane for lunch then Shelbie took their picture in downtown Spokane.

While they did their thing, my parents and Spencer and Sam and I just wandered around thoroughly enjoying the warm sun!  Oh how I've missed the sun!!  There was this awesome two story brick building that was home to some trendy, fun stores.  I sat on the balcony of the second floor just watching the world go by.

A lady came out of her store and made small talk with me.  The air outside her spa smelled so good so I told how fabulous the scent was.  She said, "Come in, let me show you these amazing new candles."  She explained how her friend was able to make this amazing candles that were just like an expensive New York brand.  She asked if we lived in Spokane and I told her we were just passing through to Seattle. She told me all about her 25 year career as a massage therapist. She had a great spirit about her.

"Oh" she said,"I bet you will have a great time!  What are your plans while you're there?  Are you traveling with children?"
"Yes, I have three.  We are just going to Seattle Children's for bone marrow biopsies and stuff."
Her eyes welled up with tears and she said, "What? That's awful!  I'm so sorry.  What is wrong with them?"
I explained that they were born with a genetic disease that causes bone marrow failure and we have to check on the progress of their disease a couple of times a year.  Just then, Sam walked in to find me.
"Is this your son?"
"He looks so handsome.  I'm so sorry he is sick!"  At this point, she couldn't contain her emotions and tears were pouring down her cheeks.  She wished me well and gave me a hug and said she had to go.

I left the store and went back out to the chair I was sitting on.  I felt bad that she was so emotional about our situation.

A few minutes later, she came back out and handed me a card, a beautiful, hand crafted card with a note that said she wanted to offer me a free massage on my way back through Spokane.  I was so touched!  I couldn't believe the kindness of a total stranger.

In a world that is so chaotic and full of everyone only watching out for themselves, this brought me a little hope that loving, kind people still exist!  I think she is the extraordinary person!  So, thanks to Liz, my new friend and the Pure Skin Spa in Spokane!

We are headed to the hospital in just a few minutes.  We will spend the entire day there...


Monday, April 22, 2013

Racking up the bill

Bills are so much fun!!  I had to go pick up the Fidaxomicin for Samuel this afternoon and it came to $3500!! For a 10 DAY supply!  A 10 DAY supply!! Sheesh!  Add that to the rest of the medications they take and this month alone, we have racked up a whopping $9500.00 pharmacy bill!

I'm afraid to look at our yearly prescription maximums because I fear we are getting really close to exceeding the amount they will allow us to spend.  After that, it's going to be coming out of my pocket and that is a scary thought!

 Let's not even mention the fact that on Friday alone, our hospital bill was nearly $15,000.   Then, let's not mention the fact that all the testing for Sam was over $1000.00

Just think of all the things you can buy for $9500.00!

-I could finish my basement
-buy all new furniture
-buy a new car
-go on a much needed vacation to somewhere warm
-I could sponsor two kids with Make A Wish
-I could buy a whole new wardrobe for everyone!
-I could make a dent in my debt load

The list is endless really.  Here's another interesting piece of trivia; it would take me almost 6 months to make $9500 working at my current 9 jobs and that's only if I don't spend one dime on anything else.

It's kind of sad and overwhelming too when you look at from this perspective.  I just need to focus on the moment and try not to think too far in advance because there is just so much I could be worrying about!  It doesn't make sense to me how it will all work out!  Somehow it does!


Friday, April 19, 2013

As our world turns

No antibiotics today for Sam.  We decided that using IV antibiotics would be relatively ineffective since it doesn't cross the gut mucosa, so....we are going to start a new medication on Monday.  It's only been on the market for about 2 years if that, it's called Fidaxomicin.  In the past we've tried Flagyl and Vancomycin and wasn't entirely impressed with the results so I'm hoping this one works better.  It better because it's going to cost me a small fortune.  The story of my life.

After a couple of weeks on that, he will be switched to another one in hopes of preventing a recurring infection.

Sam is so sad!  Holy Cow, he is one stubborn boy when it comes to being sick.  He is so mad that he can't be with his friends.  Our doctor said if he is just around healthy individuals, it's probably fine and no one should catch it.  But, he's the only one who feels that way.  At the hospital, they make people gown up and mask up and wear gloves when they go near someone with C-diff and I have been instructed to keep the surfaces of the house wiped down with bleach whenever he touches anything.  I have had to designate a bathroom just for him, keep his laundry segregated from everyone else's and a whole bunch of other things they require me to do.  It's a bit of a hassle, I'm not going to lie.

I'm a little confused as to how to handle this whole thing.  When he had it really bad like this 5 years ago, he missed 2 months of school because they told me he was too contagious to go.  Tomorrow he has been invited to a birthday party and he is begging me to let him go but I seriously don't know what to do.  I don't think anyone would catch it because he is the best hand washer ever but if someone did...I would feel horrible.

Shelbie did fairly well at her transfusion today but her blood pressure dropped to 72/45!!  That is so dangerously low.  The nurse came in trying to wake Shelbie up and get her sat up to see if they could bring it up.  It never fully recovered to normal but at least to a range we could all live with.

There was one disturbing thing about the day.  Her Monocytes keep climbing.  For the past four months they have steadily increased.  Now they are flagged as high, 4% above normal.  Monocytes are the white cells that go after bacterial infections.  Raised counts mean there is a bacteria or cancer, somewhere in the system.  It's hard to say what is going on.  She often has a fever for no explained reason and she almost always feels poorly.

Not only that, her neutrophils are low, lymphocytes are really low and her total white count is low. It's not a good picture.  I am glad we are going to Seattle in a week.  As much as I hate watching the kids go through bone marrow biopsies, I am always relieved to see into their marrow and see how things are going.

Tonight, I am so exhausted.  More fatigued than I have ever been in a long time.  I wish I could sleep for a week but I have so much work to catch up on, sleep is not going to be a priority.


Thursday, April 18, 2013

You don't know what you're missing!

If you aren't lucky enough to have at least one kid with Shwachman Diamond Syndrome, you have no idea what you are missing!! Seriously...okay, not serious.

Sam woke up this morning to a mess of blood in his stool.  So much for my mother's instinct and choosing Cellulitis over C- Diff. to treat.  We actually didn't have a positive stool study when I was guessing he had C-diff but we do now.  Believe me, we have C-Diff.

We also have a little bit of a plan starting to percolate.  We may start IV Vancomycin tomorrow in the hospital.  There are only two antibiotics that treat C-Diff, Flagyl and Vanco.  Vanco is super hard to obtain in pill form and extremely expensive.  We stopped the antibiotic for his Cellulitis and if that causes things to get worse, then Sam will have a procedure to remove his toe nail so they can get down to the abscess and drain it. Yuck.  Sounds awful! It is awful.

What makes this so hard is that Sam's white count is low, low, low and he is neutropenic so he has nothing really to fight a simple infection like a cold let alone complex bacterial infections like staph and C-diff.  I'm a little nervous.

Add to that the fact that all these infections have made his Colitis flare up so we have that to contend with as well.  

Add to that the fact that Shelbie has no immunity right now and C-diff is highly contagious or so I've been told and Spencer has chemotaxis which means his white cells don't recognize infection to go after it.  So, I have to somehow keep the two kids from getting infected.

Do you know what that means?  I have to bleach every surface of my house that Sam touches.  I spent half the night doing that. I'm not sure he is contagious to the average person but I'm not really certain about that.

Shelbie has her IVIG tomorrow and I am pretty sure we will just put them in the same room and fill up Sam with IV antibiotics and call it a party!


Tuesday, April 16, 2013

A worm in horse radish...

I heard this Yiddish saying the other day and immediately fell in love with it!  "To a worm in horseradish, the world is horseradish."

It's really hard to live in the world of chronic illness but at the same time, it's easy too.  I kind of know what to expect from day to day.  I know that while each day will be different, that's what stays the same; the unpredictability of it all!  Did I just talk you in circles?

I never know what to expect but then I know to expect that I will never know what to expect.  Some days, are very good and I think to myself, "Wow, there is life beyond the exam room doors, the poking, the prodding, the unexplained fevers, the bacterial infections that never end, the colitis, the celulitis, the dysautonomia, the IVIG, bone marrow failures, the this, the that."

Some days, are very bad and we spend the day drowning in horseradish and it is bitter and stings our throats and burns our cheeks and it seems that we will always be in this world of chronic illness.

Technically we WILL always be in the this world.  Here's to awesome genes!

 I love the days, knee deep in horseradish, when we can keep our head in a different world.  A world beyond the toil and trouble.  I love when we spend the day laughing in the face of illness, feeling peace despite uncertainty.  This is the the Holy place, that sweet spot when I realize horseradish isn't that bad.


Sunday, April 14, 2013

Something about Sunday

But then...Sunday arrives!

There is something awesome about Sundays!  God was really smart to declare Sunday a day of rest.  While there are some Sundays I have to work, it remains the day that I am able to realign my focus.

The end of the week feels like the end of a marathon race and the sprint in my step has given way to bulging blisters from the uncomfortable rubs of life but then Sunday arrives!  Somehow, engaging in spiritual things is as much a soothing balm for my physical body as it is for my soul.

Sometimes, more often than I care to really admit, I wonder how I am going to continue with this frenetic pace of life; like a clown sentenced to a life of spinning plates on flimsy sticks but then, Sunday arrives!

Friday, I was left with the unpopular job of figuring out the best treatment for Sam.  I don't love making these life changing decisions but then, Sunday arrives...I think I have made the best decision for him and the clarity I feel on this day, is just that, clear.  My faith is renewed and I can confidently turn him over to God and know that the best outcome will prevail.

On Sundays, I have this strange idea that I can go another day at this frenetic pace.   On Sundays, the chaos of this crazy world calms down.  On Sundays, my home is quiet, peaceful...On Sundays....


Saturday, April 13, 2013


We have dealt with Cellulitis about 100 times, not really but if' you've dealt with it once, it's one time too many and feels like 100 times.  We have also dealt with C-Diff more times than we care to admit.

Both infections are hard to treat and both can become serious if you aren't vigilant. So, it's been a couple of months at least since Sam started in with Cellulitis and now C-diff with a side order of Colitis. Awesome!

All of a sudden, I feel completely lost.  I know how to treat each one separately but I have no idea how we treat them both together.  The doctor was a little miffed too.  He told me to stop the Keflex antibiotics for Cellulitis and start the Flagyl for the C-diff and Pentasa for the Colitis.  We gathered our prescriptions and left.  As I drove home, I had a really sick feeling about stopping the Keflex.  As it is, the infection is so bad, we may have to see a Podiatrist next week and Sam might lose his toenail.  That will be an awful predicament for him and there's still no telling that that would allow the toe to heal any faster.

On the other hand, not stopping the Keflex means the C-diff could get worse than it is.  We seem to have caught it early which is a good thing.  Remaining on the Keflex is putting stress on his already crippled GI tract and is going to cause more stomach upset the longer he takes it.

I feel handicapped and my usual skill at multi-tasking no longer exists in this case.  How do you treat two really bad infections.  I can't even wrap my head around it.  After a day to think this through, I have decided to remain on the Keflex and be more careful to soak his toe several times a day and dress it in Tea Tree Oil and/or Coconut Oil which I hear has antibacterial properties.   I bought several herbs and probiotics to help with the inflammation and stomach upset and hopefully prevent anymore complications from the Keflex.  As long as the C-diff isn't getting worse, I am going to wait until he finishes three more days of Keflex, then switch to Flagyl for a week or so.  By then, we will be in Seattle and able to have our Oncologist and Immunologist look at things and maybe find a better way to take care of these latest happening.

It's kind of a weird feeling to manage two infections at the same time.  I wish there was one magic treatment that took care of it all!


Wednesday, April 10, 2013

Calming down...oh wait, just kidding

It's been a tricky sort of week and a weird one at that!

I am doing better today.  The swelling is going down, I could actually wear pants today.  There are just a few patches of hives left and tonight, the overall swelling in my joints seems to be subsiding.  Best of all, I haven't cried all day!  Yay...I hope that means the awful and nasty Prednisone is working it's way out and my kidney's have remembered that they have a job to do.  That would be extra nice.

So, as far as I go, things are calming down.  I may sound selfish here but I really can't be sick.  It's true what they say, the mother sets the tone for the entire family.  When I am down physically or emotionally, it drains the entire family of life preserving hope and energy and it's a hard thing to get back.  Our groove gets interrupted and my efforts to have life run like clockwork gets all reset into something that looks a little more like chaos.   The kids start worrying that I am dying and if I am dead then who is left to take care of them?  That starts the hamster wheel of thinking that does nothing for our positive attitude and gets us nowhere.

I am grateful for a couple of friends who came to my rescue today too.  Miss J was so sweet to come over for a visit and bring delicious muffins and Pepsi...ahhhh!!!  I am going to miss her horribly when she moves in just a couple of weeks.  Then Miss K brought over a very tasty chicken pot pie!  I know these are two busy moms yet they took the time to help stabilize our topsy, turvy week.  It helped so much!

Life can't all be smooth sailing.  I'm no doctor but, it would appear tonight that Samuel has C-Diff!  Ugh... Truthfully, I had a feeling that if the cellulitis wasn't cured in the first round of antibiotics, we would be dealing with C-diff at one point or another.  Sure enough, three rounds of antibiotics has arrived, I think.

So, tomorrow, it's back to the doctor but at least, I feel a little more like I can manage things from here and for that I am so grateful!


Monday, April 8, 2013

Turning the Tables

You haven't lived the life of a single mom until you have to have your kids drive YOU to the emergency room!  You haven't lived until your emergency contact person for yourself is your kid.  I am not a happy camper today, in fact, I'm down right discouraged.  Bleh!

I decided I needed to do another Enbrel injection on Friday and try to make this work.  Three weeks ago wasn't fun and my leg is now permanently discolored purple from the swelling and tissue destruction that happened.   I did the injection in my other leg and within an hour, I had a 3" welt.  I took Benedryl, Ibuprofen and a muscle relaxer to try to keep the swelling to a minimum.  It wouldn't stop.

I called the Enbrel Helpline when it was 6" in diameter and while the nurse was super friendly and had a great phone side manner, she was not at all helpful.  She just kept telling me how interesting it was that my leg was swelling up.  Still not sure what the point to a 24 hour on call nurse with Enbrel is, I guess if you need pointless adult conversation...?

By Saturday, things had not improved but as long as I could stay off my leg, it seemed to be a bit better.  Sunday night, it went crazy!  I had gained 1 1/2" in the circumference of my leg, the welt now covered the entire length of my thigh and 3/4 around.  It was hot to the touch, I was running a fever and I couldn't hardly bend my leg, the swelling had spread up to the top joint. There was so much swelling, it was cutting off my circulation and my entire leg was numb and prickly with pain.  I tried everything I could at home to get things under control but it had a different agenda.

Finally, at 6:30 I asked Shelbie to take me to the hospital.  A couple of hours later, some IV meds including steriods and heavy duty doses of Benedryl, I was able to come home.  The Benedryl made me wacky!!  Holy Cow!  I was so groggy and tired but jumpy all night long.

Today, every single joint in my body is swollen and sore and I have taken on about 4 liters of fluid I'm sure.  The leg is not any better to look at but the blood seems to be circulating better.  Now, I have a week of steroids and Benedryl ahead of me.  I had to cancel some cleaning work today because I can hardly move.

So frustrating.  I have to see my Rheumatologist today and I have no idea what is left for me to try and the thoughts of living the rest of my life in pain is not getting me excited. I guess for now, all I can think about is trying to do what I can for work and get these immediate problems under control.

Shelbie was so good to stay with me.  I felt bad since she had had a friend over for dinner and they were having a great time together.  Her friend leaves on a mission this week so I know it's going to be tough for Shelbie to say goodbye.  I kept telling her to go back home and would call when I was ready to leave but she wanted to stay with me.  I really appreciated her sacrifice but it felt really weird having to rely on her instead of me being the strong  and healthy one.


Wednesday, April 3, 2013

The Difference a Day Makes the evening, I was spent and just getting started on 6 hours of drafting I needed to have done by last night.

Things with the Neurologist are working out.  Not the one we love in Seattle but after venting with a few friends in my support group, one of them reminded me of THIS foundation that could offer me some help.  I called them and spoke to a wonderful and helpful woman.  I told her I needed to find a  Mitochondrial Specialist within a 15 hour radius of my house.  It didn't even take 5 minutes and I had a list of 6 doctors to choose from.  I have settled on the only two that are in Utah, the rest were in Colorado.  One is in Salt Lake and is relatively young and trained under one of the top Mito docs in the United States, Dr. Falk.  There are 3 names you hear all the time, the ones who research and publish papers and Dr. Falk and Dr. Saneto are two of them, so that is promising and the fact that she is young and hopefully excited to learn and on the cutting edge of treatment. The other is in St. George but I think the Salt Lake doc will be a better fit.  She is specializing in Metabolism and Genetics so that is exactly what we need.

I was reminded yesterday of how important it is for me to get the kids to this doctor.  Sam called me at 2:00pm from school and needed to be picked up.  He had lost his vision again in one eye but this time it came with extreme nauseous feelings and was then followed by serious pain in his head and eye.  These are new symptoms for him.

I felt so bad, I was on my way to the hospital for a meeting.  I hurried to the school then up to the hospital.  I didn't have time to take him home first.  I settled him in on a nice bank of soft chairs and he patiently waited until I could excuse myself to get him home.

Today, I am not as wired and need to focus on working, get ahead even so I can spend a bit of time with the kids while they are out on Spring Break. Sam announced last night that he has narrowed down his wish to three places...I can see he is getting really excited and that's cool to see.


Tuesday, April 2, 2013


I have been working so hard to get appointments scheduled for Seattle, it's like another part time job trying to get everything to work out.  It seems like everything these days is like pulling teeth that are rooted in concrete!  I tell ya....

I started this over 2 months ago and finally heard yesterday that our Neurologist will not have time to see us. All along, the nurse who has been doing the scheduling for me completely dropped the ball. I call and call and only get her voice mail.  I leave message after message and still don't get a return call.  I finally just called to the Neurologist's scheduling nurse to find out if we were set up and she said, "Well, the other nurse called a couple of months ago but she didn't confirm the dates so now the doctor is booked.  Can you come back in June?"

Trying my best to be patient with her, I said, "I can't just come back in June.  Gas is nigh on $4 a gallon and by the time I pay for a hotel for a few days, it's too much!"  I begged her to put us on the cancellation list even if we could only get one appointment.  I told her I would choose my sickest kid to take the appointment which seems totally crazy.  Who would I take, Shelbie whose body is struggling to regulate blood pressure and temperature, whose muscles are wasting.  Spencer who has now lost feeling in his foot, still having issues with breathing at night, and a host of GI problems too long to list or Sam who has a deformed ACL that still is not fixed, loses his vision without warning and is now showing increased signs of problems with his leg muscles.  It makes me so mad.

Today, in between all the other things I have going on, I am trying to find another Neurologist that will take us on.  Someone who specializes in Mitochondrial Disease and lives within 10 hours of my house.  I will never leave something like this to someone else ever again.

In other news...heading to the pharmacy for round 3 of antibiotics for Sam's toe.  It's actually looking worse again despite all the antibiotics, soaks and other home remedies.

Spencer's health history for his mission papers is lost.  There goes 10 pages of long hard, emotional work gone.  Whether they are lost at the doctor's office, in the mail, at the post office or mixed in with the Bishop's junk mail, I have no clue.  The office swears they mailed them last Wednesday.  Again, I should have just picked them up and hand delivered them which is what I have to do today.  This doesn't happen to other kids leaving on missions!  He had all his final interviews lined up for Sunday and had to put them off until those papers show up and all the powers that be are leaving this week for the rest of the month!

I need a vacation!


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