But you don't look sick!

This phrase.  These five words.  I hear, All The Time.  At a friends daughters birthday party, running into an old acquaintance, a client I haven't seen for awhile, my hairdresser, the list goes on.

The thing about CIDP (and many autoimmune diseases), is you can't really see the disease just by looking at me.  Under the surface, a war is raging between my nerves and my white blood cells; slowly eating away my feelings, my ability to function.  

Those that are close to me can see the slight changes.  The way I walk.  Lifting my legs is more like lifting cement with each step.  The constant inflammation in my body causes things to swell.  The nerve roots being exposed causes muscle spasms that can be anywhere from my toes to my eyes.  The nerves feel like I'm on fire, like I have bugs crawling inside my body and on my skin.

This invisible illness has no known cure at this point.  I'm left with taking medications derived from plasma donors to keep my ability to walk.  These medications are acting as a bandaid at this point.  At any time, it could stop working.  At any time, I could lose my ability to walk.  At any time.

Can you do another transplant?

Technically speaking, yes.  But it's not that simple.  A transplant would mean remission again.  It also means months away from my family in Chicago, chemo, hair loss, immune system reboot, lots and lots of drugs, a year of recovery, post medications, time lost where I can't work.  Money. Lots and lots of money.  

My current medication costs $18,000 per month.  That comes to $234,000 per year!  That's the cost.  That's what insurance pays.  They are billed $45,000 per month and only pay $18,000.  If they paid the billing price, that would equal $585,000 per year.  All of that to say, this medication is expensive.  I cannot live without it.  

Every week, I stick 6 needles into my stomach and inject $4,500 worth of medicine in hopes of going into remission.  I have yet to experience a day without symptoms.  

So, I'm in that place.  You know the one.  Where you feel like you're between a rock and a hard place.  While I'd love to be disease free again, it will come at a great price to the people in my world, again.  

Happy Friday.

2 Years

2 Year Follow Up Appointment:

Today we flew into Chicago for the day for my checkup.  Some have asked me, "why go when you've relapsed?"  Great question!  I go because when I agreed to do the transplant, I agreed to come back every year for five years so my data could be used in the clinical trial publication.  I go because I've relapsed and my story may help other CIDP HSCT recipients in the future.  I go because these Dr.'s saved my life once and will likely have an idea how to help me again.  By going, I keep the line of communication open for me to help them with the study and for them to help me thrive.

The day started early.  We woke at 5:00 in Baltimore and arrived in Chicago a little after 10:00.  We got a Lyft to the hospital for my first appointment at 11:30 (EMG & NCV).  These are those oh so fun procedures where they put needles in your muscles and test the nerves and then use an electrified wand along your nerves to test the velocity.  Super painful but the best way to look at the health of the nerves.  I don't know the final results of those yet but I do not my left leg was slower than the right.

Next, we headed to the 14th floor to see Dr. Burt and nurse Paula.  While waiting there, we ran into a fellow CIDPer who is applying for HSCT and was meeting the Dr.'s for his evaluation.  We chatted a bit and shared "war" stories.  A strange thing to meet a stranger and feel connected in a way because most people don't understand the pain you feel.  

When we met with Dr. Burt, he asked me a laundry list of questions: how have I been since Decembers relapse?  What has been my course of treatment? How am I feeling now?

He examined me.  Had me do some walking tests: walk on my tippy toes, walk heal to toe...these went much better than when I saw him in December.

I then asked, what's next for me?  Do I do a top off of a chemo drug (Rituxan) that kills the B cells in my body?  Do I do another transplant?  His thoughts are that he would be fine if I wanted to do the Rituxan but he thinks we should give the sub q a chance to put me back in remission.  He also said that if my CIDP were to start progressing rapidly, then a second transplant would definitely be the way to go.  So for now, we are going to stick with doing sub q, every week and pray that my body stops attacking itself.

During our time with him, we learned that there have been four of us CIDP folks that got a case of shingles and then began relapsing.  Three of us are back on IVIG and one miraculous person was able to avoid a relapse all together.  With that said, my relapse has had a purpose.  While I wish I hadn't relapsed, this new data is causing Dr. Burt to change the aftercare protocol for CIDP HSCT recipients.  They will now take the anti-viral, acyclovir, for 2-3 years post transplant instead of the 1 year I (and my fellow transplant friends) were prescribed.  

Next was a blood draw.  Only 10 vials this time.  😉

Then to end our day, we met with Dr. Allen.  We answered questionnaires about what are things I can and can't do...walking for long distances, run, turn a key, open a jar...so forth and so on.  Next I had to test my hand strength.  Thankfully, my hands have not been affected much and my strength remains strong at about 73lbs per hand.  Then we talked.  Dr. Allen told me how glad he was to see me doing better since I last saw him in December (December was a rough time people).  We discussed the long term issues with being on sub q ig and my risks.  

All in all, we ALL agree that doing sub q ig is the best course of action at this point.  

So, now we fly back home and get this new rhythm down of doing sub q every week.

I haven't posted much over the past few months.  Life has been busy and I honestly haven't had much good news to report, so I've remained silent online and confided in my closest people when the burden has been too overwhelming to bear.

IVIG Still Sucks!  It still makes me sick.  It still robs a week of my life every single time I receive it.  During my last IVIG, my 3 year old looked at the IV in my arm, kissed it and asked me if I was going to die.  Hard.  So hard.

My oldest, not sure of what to do to help, brought me an ice pack for my fever and a teddy bear to hold.

I know hard life circumstances don't break kids but I sure wish they didn't have to experience this.  Hell, I wish I didn't have to experience this, but here we are.

Sub Q IG:

The sub q injections have begun.  So far so good!  It's not a walk in the park in the least but the side effects are much more tolerable than the IVIG.  I basically stick myself with 6 small needles in my abdomen and then use a self regulating pump to inject 200 ml of immune globulin into my body.  The amount of fluid causes my belly to swell.  It reminds me of being about 3 months pregnant.  But, I'll take looking like I'm pregnant over migraines, vomiting and body aches like the flu any day.

One more silver lining in the "adventure" I've been on, I have gained a lot of insight into what cancer patients go through.  I've had chemo, I've had neupogen, my severe reactions to IVIG are just as bad as reactions from chemo (minus the hair loss and wasabi nose).  My bestest friend, has been fighting breast cancer for the past 6 months.  She's gone through it all: hair loss, fatigue, bone pain, nausea, bowel issues...you name it, it's happened.  But, when she texts me on a chemo week and tells me how horrible she feels, I get it, I know, I've lived it.  While our circumstances for all of these drugs are different, our experience is the same.  And I am thankful for being able to know what her heart is feeling as well as her body.

All of that to say, life is hard, we can choose to dwell in the muck, or we can try to find the silver lining in the midst of absolute chaos.  My life continues to dwell in the realm of chaos, but I don't want to live there.  I, like you, don't know when my time on earth will be done, but I do know, life is still worth living and people are still worth loving.  

What a Month

This month has been a whirlwind to say the least.  I'll hit the highlights then go into detail: we moved into our home, my best friend donated her hair in my honor and was diagnosed with breast cancer, I had an appointment with my new Dr. at Johns Hopkins, my husband went part time at work, and here we are, IVIG again.

Moving:

We had a great weekend of moving.  We rented a Uhaul truck for 2 days.  One day we moved all our things out of my parents house.  The next day, we moved all our things out of our large storage unit we had filled when we moved out of our town house.  We had so much help from family and friends.  From kid watching, moving refrigerator food, moving boxes and furniture, it was a fun moving day.  We are getting settled and enjoying our yard and room to play.

Hair Donation:

When I lost all my hair, Jenn wanted to shave her head with me.  At the time, her family was in the process of adopting again and would be meeting their new son in the months to follow.  As honored as I felt that she would go bald with me, we decided that her donating her hair was a better use of her awesome hair.  So, we made it a special date.  We went to her stylist, and then went out to dinner to celebrate.  At dinner, she told me that she had found a lump but wasn't worried about it.  That week she got into see the Dr. for an exam.

It's cancer.

Never words you want to hear from anyone you love.  My best friend since 3rd grade, has triple negative breast cancer.  A very aggressive form of cancer.  The team of Dr.'s acted quickly.  From the time of diagnosis to the time treatment started, was less than a month.  She is now the one undergoing chemo and all the side effects that go with it.  Silver linings: I kept my favorite chemo caps and wig, I was able to prepare her a little for what to expect, I know how she feels...what it feels like to have drugs in your body that make you feel like death.  She is strong.  She is Wonder Woman. I love her dearly and am sad for the road that she must travel.

Hopkins:

Northwestern helped me make an appointment with them back in December and the earliest I could get was late March.  I got a call around 3:00 pm from my new neurologists office asking if I could come in the next day at 9:00 am for a cancelled appointment.  I made childcare arrangements and took the appointment.  I wasn't prepared for being there from 8:30 until 3:00.  I met my new Dr. went over my history and he drew a timeline of my CIDP.  I was with him for an hour.  He asked if I could stay for testing (EMG, NCV and blood work).  I stayed and got all the testing done.  Before I left the testing room, the technician told me that the Dr. wanted to see me again before I left.  I go back to his office, and he calls me in with another CIDP Dr. from Europe.  He looked over my test results and wanted me to know that they looked nearly normal now.  That we caught the relapse quickly before damage had been done.  Good news!  He also said he wants me to continue my monthly IVIG (every 4 weeks instead of 3) for the next 3 months.  In May, I go back to see him and at that appointment, he hopes to switch me to sub q injections of immuno-globulin.  The side effects with sub q are much less than with IVIG.  I will just have to get used to giving myself 5-6 shots every 3-4 days.  So, here we go and continue on the CIDP management.  In June, I'll go back to Chicago for my yearly follow up.  While I know I'm not in remission, it's important for me to go and continue the study so Dr. Burt can use my data and figure out why the transplant didn't work for me.

Hubby Goes Part Time:

Last Fall, we were seeing the need for Jesse to be home more to help our boys.  What we've learned with my being sick, trauma happens, and it takes time to heal from it.  In order to qualify for our loan, we needed Jesse to stay full time, even though we can afford for him to be part time.  So, we had to wait until we closed before changing anything with his schedule.  He started going part time this week and it's been glorious.  It's freed me up to work more and for us to have more time together as a family.  Both of us working full time, on opposite schedules wasn't conducive to any sort of healthy home environment.  As good as this is for our family, there are some who might see this as Jesse being weak, not providing for his family...blah blah blah.  I will say this once, if you have that opinion you may go take a very long walk away from our family.  We don't want or need the negativity that some people have of stereotypical gender roles.  We are and will continue to keep our families safety and well being at the for front of our lives.  We have never been ones to take the gender roles we are "supposed" to have.  So, we march on.

Birthdays:

It's birthday season in our family.  Daniel just celebrated his 3rd birthday!  He's 3!!! Woah.  Next up is me then four days later is David then 4 weeks later is Jesse.  It's a crazy house on the late Winter early Spring.  Add everything else and it's just insane.

IVIG:

Yesterday, I had my IVIG treatment.  Same old same old.  Makes me feel awful.  So, here I sit, with my steroids, zofran & Tylenol, hoping that I can be a mom again when the boys come home tonight after dinner.

Just Keep Swimming

Where to start...

We went to Chicago on December 2nd and were told I had relapsed.  I was declining so quickly.  Everyday that went by I was losing more feeling, losing my stability and ability to walk, having more and more muscle cramps, eye twitches...and so on.  I was getting weaker by the hour, not able to lift either boys up.  In a matter of a week, from when we were in Chicago, we started my IVIG infusions again.  My old nurse Bello cleared his schedule for me and spent two days at my parents house administering my medicine.

An odd juxta position: to know the horribleness of what the infusions will do to me and wanting it so desperately to stop the damage that it was causing.  

Thankfully, the medicine started working very quickly.  By the third day post infusion, I noticed improvement in my legs.  By week one post, the numbness/pain/tingling had begun to recede down my feet.  By week two, it was down halfway on my right foot.  By week three, my left foot started showing signs of improvement.  Today, I can wiggle my toes again and I can feel the heels of my feet.  

I had another infusion on December 31st (Happy New Year), not so much fun when you are sick.  I'm in bed recovering still and will likely be very ill tomorrow.  But, it's helping.  I can feel the improvements and that makes the pain and discomfort worth it.

How are we?

I am past the initial heartache of it all.  It was a major loss that we all felt.  Seeing my dad so sad about me feeling sick, having IV lines hanging out of my body, it's something I've gotten so used to.  But as a parent, I can imagine how sad that must feel.  To see your child, sick...knowing there is absolutely nothing that you can do to help them...a helpless feeling.  

I told Jesse that if me being sick will keep everyone else healthy, then I'm fine with that.  I don't want to begin to think about anyone else in my world falling ill.  I know that I have absolutely no control over that, but it's a nice thought.

The boys have been handling it all pretty well.  They are enjoying mommy movie dates in bed and cuddles under the covers.  They are learning empathy and how to help when someone doesn't feel well.  Wonderful traits that their friends and future loves will reap the benefits of one day.

Did we buy the house?

Yes, yes we did.  It was not really ever a real question. It was more of a fear of the unknown.  We signed papers on the 30th and spoke with the sellers at length.  This isn't just any house.  They built it in 1976.  They lived there for 40 years!  They had their children there, their grandkids too.  There are memories of them everywhere.  Their first Christmas tree planted in the yard, the foot bridges that they built with their grandkids, the marks on the shelves in the garage showing how tall the kids had grown.  They showed us such generosity in leaving tools and yard equipment that they no longer needed.  Now we get to continue making memories there.  Start new traditions, add on to the foot bridges, plant our own first Christmas tree next year.  While I am hesitant to dream again, I know I must.  

I am a planner.  I like to know what's going to happen and what I can do to prepare for it.  With this disease, there is no opportunity for planning.  At any time, my body could go on strike and say, "Nope! That IVIG will not work anymore.  What else you gonna try?"  

As Dory encourages, I keep swimming, even when I feel like I'm drowning.  Feelings are useful tools but they are also very unreliable at times.  

IVIG Infusion

Yesterday and today I saw my old friend and nurse Bello.  He came to my parents home and spent the day giving me my infusion.  He didn't leave here last night until after 9:00.  I am thankful that I was able to get him again as my nurse.  I'm a rare case in how badly I react to the medicine and we have to have a very strict order of events.  The days before I drank nearly 100 ounces in water.  This is to ensure hydration and to prevent negative side effects.  The day of, he gets my line in, I take oral Tylenol, he gives me fluids via IV, then steroids via IV, then Benadryl via IV.  If you've ever had the pleasure of IV Benadryl, I'm sorry, it burns your veins like liquid fire when it goes in.  This is also when my body goes into a twilight sleep where I can't really move but I can still hear what's going on around me.  After all that, we start the IVIG.  Both days, we ran it at the slowest speed we could since it's been over 18 months since my last infusion.  After the IVIG is complete, we run another bag of saline to make sure I'm properly hydrated.

My dad came in at one point and looked at the line in my arm.  He gently hugged me and said, "I'm sad that you are having to do this again."  Me too dad, me too. 

My post medications this time consist of a steroid pack and anti-nausea meds.  The worst side effects will start showing up in a day or two so if we can stay on top of them, the better off I'll be.

I have never been too good about taking time for me, or downtime.  As a wife, mom and business owner, I am constantly going.  I enjoy the hustle and bustle of life so being forced to lay down for days on end is really difficult for me.  You see, I desperately want to live a normal/healthy life.  I had a taste of that for a little over a year and it was sublime.  There was no scheduling conflicts around my infusions, no lost work, no little boys being sad that mommy couldn't play.  Since I have tasted freedom and health, I long for remission like a dessert yearns for rain.  I desperately want to live a normal life free of IV lines and Benadryl comas.  I desire to run in our new yard kicking around a soccer ball, climbing trees and hiking the nearby trails.

Dr. Burt is hopeful that remission is possible again.  The course of action for now is we will infuse for one day, every 3 weeks.  I was able to only do 2 days and not 3 because I've lost 15 lbs since before the transplant!  Yay me and yay one less infusion day!  So, we march on, hoping this works and stops the progression.  Until then, I'll keep snuggling with these guys.

Relapse

Back in August, when we were at the beach, I developed shingles.  It was one of the worst cases my Dr. had ever seen.  I took the medications prescribed and the shingles cleared up completely about 6 weeks later.  Also during this time, I noticed some tingling in my toes.  I wasn't too concerned at the time as this is a common occurance for us HSCT veterans when we get ill, the old damage/symptoms reveal themselves.

At my one year checkup in June, the only symptoms I had were slight numbness in the tips of my toes.  Fast forward to today, my fingers, feet, and parts of lower leg are numb.  The shooting electrifying feeling is back and I'm having major difficulty walking.

From my shingles outbreak in August to today, I have been in touch with my team in Chicago.  Up until a few weeks ago, we were all hoping that my symptoms were just my body dealing with the shingles outbreak.  My symptoms got so bad so quickly, that Jesse and I flew out to Chicago for appointments and tests yesterday & today.  

We sat in the exam rooms, staring at the floor, anticipating the truth that we already knew.

I am sad to say that I am no longer in remission and my CIDP is back.  I have officially relapsed.

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How did this happen?

Dr. Burt explained it in major medical jargon so I will do my best to interpret.  The shingles virus was dormant in my spinal fluid which lives right next to a large cluster of nerves.  When my body started fighting off the shingles virus, it also started fighting those nerves because they were all right there, next to each other, breathing the same yucky shingles virus air.

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Now what?

Dr. Burt told me he has had one (1) other CIDP patient who relapsed.  (Remember, the remission rate for CIDP is in the 90% range).  That patient went back on IVIG for 18 months and was able to stop taking it and go back into remission.

So, we again get to figure out this horrible balance of medicine that makes me feel like dying versus a disease that is killing me from my extremities inward.

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Did the transplant really work?

Yes!!!!!!!  Yes it did.  Hands down.  100%.  My CIDP was gone and I was disease free for over a year!  

The transplant let me get to play soccer with my boys, paint, move and sell our old house to get our boys a yard they so desperately need.  I am thankful for the year I had disease free to live a normal life.  I am of course sad and in grief over this relapse and what that means for our family, for our future.  Dreaming has now stopped again.  

We are scheduled to settle on our new home on December 30th and I am scared out of my mind.  Wondering if we should break the contract and wait and see where we are in a few months.  When we put the offer in on this house, my symptoms were minimal and we all thought my body was just taking its time healing.  The joys of being an adult mean you get to ask the super fun questions like, "are we making the right choice? Is this the path we should go down? How will my disease affect the boys again? Will I be able to keep working? Will Jesse ever get to pursue his career? Will he ever get to finish his degree? Will he ever get to stop putting his life on hold for my breaking and failing body?...". The list goes on and on and on.

I am sad, very very sad.  As I told Jesse the other day, I will keep fighting but there will be times when I don't want to, when the physical pain is to great to bear.  Those are the times when I need help picking myself back up, dusting myself off and starting all over again.  

For whatever reason, this is my journey.  None of us get to choose what hard things we endure in this life, we just get dealt them.   We do get the privilege to choose how we respond to the hard things.  

I will try to remember that how I react to this is what I can control and that my example will shape my boys into some amazingly compassionate men.  But, that is only if I choose the higher road, the road of love and vulnerability.  

I desperately wish that this was not something I had to deal with in this life.  I wish the only hard things I had to endure were things like career choices and whether or not to relocate our family.  

Having this disease has given me one thing that I am grateful for, perspective.  It really takes a lot to ruffle us, to disturb our souls so much that it causes a panic attack.  When you're constantly in the hard, daily issues like traffic, disgruntled customers, terribly tantrumy two year olds just don't affect you the same way.

So, we march on and keep fighting to live and love.

Busy, Busy, Busy (Day +416)

Things are eventful here.  Life doesn't slow down and I've thankfully been able to keep up!

We've fixed our deck, helped my brother roof his house, taken the boys swimming...none of this would have been possible before my transplant.

Life is constantly moving.  

I still can't feel my toes.

I still have pins and needles on my feet.

I still have numbness that sometimes hurts.

Did HSCT work?  YES!

The goal of HSCT was to stop the progression of my CIDP.  Any improvement that patients experience after the transplant is considered a bonus.  

Thankfully, I have experienced a ton of improvements.  

-the constant electrifying feeling is gone.

-muscle weakness is gone.

-debilitating fatigue is gone.

-I no longer take any medications related to my CIDP or HSCT.

-intimacy has improved.  This was an area of life that I hadn't realized had changed from being sick from CIDP. 

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Thoughts:

We are in the throws of school prep here. Our oldest son, is going to kindergarten in a few weeks.  While most moms experience some feelings about their kids going to school, I've had some different emotions.  

-all that I missed out on in his life.  You see, I got CIDP soon after he was born.  When most new moms are dealing with curious crawlers and sleep deprivation, I was dealing with worries of why my legs weren't working, why I suddenly wasn't able to hold my son standing up, why my toes and feet were going numb.  The week before his 1st birthday, I had my first IVIG infusion in the hospital that lasted 5 days and made me soooo very sick.  His entire life has been mommy leaving, mommy throwing up, mommy not able to play.  I've missed so much and while I know sending him off to school is what he needs, I'm more than sad about all I missed.  And all at the same time, thankful for all the love my family and friends have shown him over the years.  When I couldn't be mom, he had grandparents, aunts, uncles and friends who cared and loved him.

With all of that, I'm trying to live presently.  Trying to be a better mom.  Trying to make new and fun memories with him that will show him that I'm well.

Here's to 2016-2017, a year of new experiences.