Half the Energy, Twice the Spunk

So much for my diligence with updates...  good news is I was too busy not being sick to get on the computer!

I saw Oncology yesterday and things are going reasonably well.

The good news:

• This week marks 12 weeks of remission for me - this is the longest I have sustained a remission in over 2 years.  
• The chemo injections are not making me violently ill as the last few drugs did.  
• My bone marrow is tolerating it beautifully (the only other drug that elicited a sustained remission put me in bone marrow failure and earned me an extra month (Christmas sadly) in the hospital fighting severe infections) and my liver is standing up beautifully.
• I'm eating about 75% of my calories at this point - the most ever since this started! 

Well, as usual it's been a while.  Even with the updates being spread out, I like using this site as an easy way to keep my (very VERY large and wonderful) family up to speed.  Please - any family/friends who read this and can think of any family that might want to see it, send the link along for me.  I only have a few email addresses.


Anyways.


It's been a bumpy few months... lots of good, lots of frustrating.  The good was pretty awesome.  We got lucky twice and managed to plan 2 family trips that did not get bunged up with hospital admissions or sick kids.  In April (or May??) we drove to Minneapolis for 5 days and did the Mall of America/Science World/Museum/Ikea/Outlet stores/Mynards trip - we all loved it and desperately needed it.  We'll be paying the whole thing off until Tyler graduates but it was well well worth it!

Then, in July, in between blood infections and hospital admissions we managed to plan and actually pull off a 4 night stay at a cabin on Blue Lake (Duck Mt Park - it's on the MB and SK border).  4 days of beach, boat, fish and sun - it was wonderful!  I had the added perk of having just finished several IV courses of heavy duty steroids (whole other story) so the entire trip saw me HYPER and feeling awesome in the wake of the steroids.... it was good.

Unfortunately the rest of the time in between has been challenging.  I relapsed in June and spent a few weeks getting plasma treatments to get back into remission.  Then we upped the chemotherapy dose in the hopes that it will KEEP me in remission.  That finished up and I promptly developed a sepsis infection that saw me in and out of hospital for another 2 weeks.  Then I basically lost the will/strength to do anything more strenuous than lift a tea cup and turn a book page for 8 weeks.

Luckily, I have 2 fantastic little boys who are quite fine with a mom who has to trade bike rides and beach trips for story time and fiddle lessons.  In spite of my complete exhaustion the boys and I have had a pretty nice time just being together all summer.

Yesterday I lucked into a 'good day' (the first in weeks) where I could actually think about leaving the house, so Dave came home from work and we took the boys to Wal Mart to do their school shopping - shoes, supplies, and they each got to pick out an outfit, and their 6$ of allowance was at hand.  It was a lovely day.

So that's about it.

Right now I am sick.  not as sick as I have been - but sicker than usual.  The disease seems to be in check at the moment (knock on wood), so we're not sure if it's the side effects of the chemo, or something else altogether, but I'm worn flat out.  I'm maxed out on my pain meds and nausea meds etc and while I don't hurt (a blessing I don't take for granted), the net effect is that I am very stoned from the pain meds, and when im in that shape I cannot drive, concentrate, or accomplish alot of anything.  It's better than hurting, but it's a pretty miserable state to try and muddle through for more than a few days.  I'm on day 15 roughly.  Searching for that light at the end of the tunnel.

The boys head back to school in a few weeks, and while I'm thrilled for them (they are SOOO excited!!), I'm also really sad - I had so many things I WANTED to do with them - it's mid august and I have yet to put my feet in the sand at the beach 2 blocks from my house, and we planned on zoo trips, picnics, mini golf, carnivals, swimming lessons, bike rides, walks to the ice cream shop on Sunday evenings - and instead they spent a whole lot of time sitting around the chemo room, the ER waiting room, and our living room, watching movies because it was the only activity we could actually do TOGETHER.

I am trying really hard to stay positive and remember the 'fun' parts - but it's really hard to focus on the positive when the negative is so friggen stubborn and prevalent.

That said - I am extremely thankful to have had the chance to read my babies their bed time stories at least 90% of the bedtimes this summer.  For that I'm grateful.

Hoping the fall will bring me the stability that we've been desperate for now for 2 years.

Thankful for my 2 happy/healthy sleeping boys in their beds.

Well it's time to dust off the keyboard and rejoin the land of the living I think!

Just a quick update for now - I will try to keep this somewhat updated for family/friends.


Things here are going fairly well.  I had a few incidental hospital admissions this year but other than the meningitis in January, I haven't been really SICK for a while now.

I started Methotrexate (chemotherapy drug) about 8 weeks ago now, and it is safe to say at this point my 'better' state is because of the Methotrexate - this is awesome as the side effects, so far, are not prohibitive. This is the first drug we have found even a bit of a balance - they have all worked on my disease, but the side effects were too extreme.  So far so good....

We just got back from an impromptu, budget-vacation to Minneapolis last week - it was exactly what we needed - a family escape, just the 4 of us, with us all well...  It was awesome - we just had loads of fun the entire time!

The boys are doing very well - aside from some anxiety around mom disappearing (I can't even reassure him - 3 times this year already the poor kid has either gone to bed, or gone to school, only to wake up/come home and find mom gone to the hospital for days or weeks).  But they are working through it pretty well and we're hoping things will settle down once summer gets here and they get to spend all day long with me - they will be begging for an admission by July I'm sure!

So that's that...

It's all a bit surreal - I don't remember how to not be 'sick'...  After 2 years of the 'lets figure out how to survive the week' mindset, I have no idea what to do with myself now that it's not such a focus...  I find myself thinking about things (the future - my 'career' down the road - whether we want more kids - what we want to do next winter...) that I haven't had the luxury of thinking about in almost 24 months... it's really unfamiliar and a bit hard to get used to...  But it's definitely wonderful.


So - Here's hoping this didn't jinx it - it's always such a very fine, delicate balance when things are good - it's so so easy to upset it.

Fingers Crossed...

Well I wrote a 5 page diatribe on the past few weeks and realized it was idiotically long. Here's the Cliffs Notes version:

In November I relapsed, in December I started IVIG and TPN again. Last week my second IVIG infusion caused what we are hoping is Aseptic Meningitis (a very rare side effect - sounds right up my ally) after the "we just want to cover all our bases" Spinal Tap (Holy Hell... "some discomfort" is apparently doctor speak for 'it'll feel like we're cutting you in half with a chain saw - then the real pain will start'). I will never shed the guilt I now have over allowing them to do this to my 13 month old baby without any sedation or pain meds... My heart will always ache when I think of it. Don't allow your child's doctor (or yours) to talk you into an unsedated/unmedicated spinal tap if your child (or you) can tolerate the sedation meds - it takes all of a minute longer and some closer observation for a few hours but it's humane. I HAD sedation and it still cleared my pain scale (this from the girl who watched as her surgeon performed a deep-thigh muscle biopsy!). Barbaric.

But, as it turns out, necessary. The spinal fluid was a nightmare and was full of puss essentially, along with a bunch of other unwanted jumk. (lovely eh? pus-filled spinal fluid - excellent ice breaker).

Within hours I was diagnosed with Meningitis, admitted and the rest snowballed from there. (thankfully the spinal fluid did not have any visible bacteria, and the cultures grew nothing, it is assumed Aseptic Meningitis brought on by IVIG treatment. I'm an awesome date I tell ya.

All immune treatements were stopped until the meningitis started resolving. Thursday I had Plasmaphoresis (google it) and had a severe and terrifying reaction to the treatment... very nearly had a code called on me, spent the next 3 days violently and horrifyingly sick.

It's finally starting to improve, however my gut remains in full revolt. Haven't kept much at all down (including meds - compounding things) and rely on IV everything - fluids, fats, nutritions, antibiotics, antiemetics, narcotics, vitamins/minerals, etc etc - We wont know just how hard a hit my GI system took this go - we always seem a bit further behind after illnesses. Monday's goal will focus on alternatives to IV meds - or getting them covered.


We're working with Palliative Care to come up with an acceptable arrangement that keeps me at home. Hoping for things to improve once we have them on board (This is not Hostice care which focuses on helping people/families who are dying navigate the system in a manner that keeps their priorities on the foerfront; Palliative care is 'comfort care' - they focus on making patients comfortable and coming up with solutions that work for everyone... terminal or life-threatening/life-limiting diseases are all open to Palliative input. I'm hanging my hat on them.

Tomorrow will be a better day. It HAS to be.


Thanks for popping by - Family/friends, feel free to pass this along to others I may have missed!


~K

I just typed a far far too long and disjointed entry.

To save on time, I will be brief today. Should you want more details on any of it, pleas ask... Questions are always good. Especially when the come from the '12 and under crowd! Either email, or just leave a comment with a ? and I'll explain whatever you want.

GggyjQuestions are totally encouraged, and I'll answer any questions as best I can. Please encourage the kids in the family to express their questions... All in all the questions from kids tend to be the best ones.
;66
Alright... Here's a timeline that will bring us to today:

July - 5 cycles of plasmaphereskis followed by the IV rituximab (chemo drugj) every week for a month.

August - two admissions for uti/kidney infection/urosepsis.

September - inpatient again for urosepsis. At this point I was seeing benefits of the rituximab, and I was eating almost totally by mouth. The tunneled line was pulled before we got word on the cultures.

October was lovely. No TPN, no central line... It was a glorious month!

November was the start of the spiral. By late November I was losing weight , dizzy, my heart was a mess, I wasn't eating and more often than not I felt pretty crappy.

December was spent getting a new central line (I have a port a cath), starting the new drug, and getting a few cycles of IVIG. First cycle landed me inpatient for a weekend due to aseptic meningitis.

This past week has been a bad one. Too many bad days and far too few tolerable ones, never mind 'nice' days.

I chose today to postpone the next IVIG dose, as I've had a very sick week and needed a break.

A huge thank you to everyone, for the moral support, physical supports (my laundry gets taken care of)' and financial supports (6 months of no work after he got let go for missing too msuch work on account of what's going on, combined with the tremendous drop in income when he finally found work that allowed the flexibility we require... Add in the thousands of dollars we pay for insurance, meds, travel, parking, gas money, missed work to care for me on bad days throughout the year... It's been a challenge as the life we had was based on twice the income.
I'm reminding myself that I'm here, at home, with my family and that should be more than enough... But when we are constantly reminded that any attept to get ahead financially is usually tossed out the window (we spent two years pouring money into our adoption account. We managed to save up over four grand. In one swoop it was all gone, between insurance co pays (it's great that my wheelchair is 80% covered but that still leaves nearly a grand from us) and life in general.


Just a grumble... I always knew it was expensive stuff being sick, but I had no idea. At this point we have about $20,000 worth of equipment for Brandon and I waiting to be ordered (power assist wheels for my chair, a new chair for brandon, basic supplies for tube feeding, and a host of smaller things we have on that list) because we don't have the money or time to organize the orders.

Just sharing the realities most families like ours are faced with...


Well enough of me babbling. I'll try harder to update a bit more often.


Please pass this link to anyone else you think might want to stay updated.

K

Wow - I need to do some serious revival work on this blog!! Life is so hectic it's hard to remember to update!

It's been a good few months, and a pretty rough few as well. The boys are at school and loving it, taking the bus like big kids ( **sniff** ). Very healthy and growing like weed. :) Parents often report a bit of a honeymoon from the major mito stuff when kids are about 6 or 7 - the high energy demands of infancy are no longer in place, while there is growth and development happening, it's at a slower pace so it doesn't require as much energy from the body. We have definitely seen that - Between Brandon's feeding tube, and this plateau we are hanging out on at the moment, he's really just thriving. We know the next major hit (often a harder hit than the one children deal with in infancy) is expected to come with puberty, but we're hoping it's not too hard on him.

Dave has settled into his new job and is really enjoying the people there as well as the ability to come home at the end of the day. We're all happy to have him home!

Onto me

It's been a ride.

I'll do the 'nutshell' version just to cover all the bases:

January
Discharged in January '11
PICC infection, line pulled, tunneled line placed, days in the hospital.
Several doses of IV solumedrol, and high-dose prednisone orally.

February
High Dose Prednisone through the month
Noticeable improvement
LOTS of energy, jogging, feeling pretty great.

March
Steroids induced Type II Diabetes
Weight doubled to 165lbs in less than 3 weeks.
Off the steroids and start investigating chemo.

May
Line breaks - HSC repairs it
Severe sepsis blood & line (due to line repair)
Heart/Blood pressure goes down
Motility stops

June
Recovering at Moms
Preparing for Chemo (Rituximab)
Surgery (Vas Cath)
Plasmaphoresis

July
Weekly chemotherapy (rituxan)

August
2 week-long admissions for UTI/kidney/sepsis infection (Urosepsis)
Officially in remission mid August

September
another week-long admission for resistant Urosepsis.
Line pulled, TPN/Fluids stopped.

October
Fungal skin and mouth infections (lots of antibiotics = lots of yeast)
heart/blood pressure deteriorating
GI deteriorating

November
Officially relapsed.
Prepping for a port-a-cath placement, starting fluids and TPN this week or next, and gearing up for some 'real' (nasty) chemotherapy.

Merry Christmas.


We are now looking at a very upside down holiday season once more.
The hope is that we can keep me home for the holidays this year - and with the speed my GI team is arranging stuff I think I might actually manage to stay home.

I'm discouraged, exhausted, scared, angry... the whole gammut. The first 3 relapses and 'starting over' that followed were awful. Now I'm there again, starting from a place where I am sick, weak, nauseas, in pain and a touch emotional and even thinking about the weeks and months that lie ahead leaves me completely befuddled.

I hate this disease - it 'gets better' with treatments just long enough for me to go 'wow! I'm actually better!' then it pulls the rug out from under me. Over and over again.

So yea - I'm totally overwhelmed at the moment. We are working on getting set up with a private nurse so I can stay home through infections when possible. Working on a Port date ( https://www.haemophilia.ie/uploaded/image/Port%20A%20Cath.jpg - this is a diagram of what a port is - if you want more info just google it - it's a central line that lives under my skin and is accessed with small needles daily - the perk is at the end of the day I can remove the needle, and not have anything at all on my skin!

I'm looking forward to being hydrated again - and not waking up miserably sick..

So that's us - I see Oncology on Monday to arrange Chemo and the TPN clinic is working on getting my line and tpn underway.

I'll keep the blog update better as we go.


K

Well my last fatigue driven blog entry was pretty flaky and disjointed, so I figured I'd just give the technical specs of things in this one.

Thursday morning I go to CancerCare for round 2 of the chemo we are attempting to control my immune system with. (and by control I mostly mean 'kick it in the butt so it stops making me sick - but hoping not to kick it so hard that it lets other garbage make me sick - yay tightrope walking).

Basically things went down like this:

March 30th - spent my birthday at CancerCare discussing our options. Options ranged from 'really crappy and scary' to 'even crappier and scarier'. We decided on the blander choice of really crappy and scary - decided to try a 3 month trial of Mycophenelate (MMF - an oral chemotherapy drug - also called CellCept). The upsides - it was oral and could be done at home. The downsides - it was a 'broad immunosuppressant' and would likely wipe out all my immune system. It also was slow - it would be 3 months + before we knew if it was going to work or not.

Most of April ticked by waiting for an appt to see my cardiologist and start the Mycophenalate trial. I was acceptably functional - almost completely NPO (nothing by mouth - no food or drinks) and doing OK on the TPN. The steroids packed a literal 65lbs of fluid on me over the winter, and much of March and April were spent watching that extra weight fall off me - it continues to do so.

May came along and kicked my butt. I can't remember specific dates but I wound up with a broken central line in mid-April sometime - the seal at the tip of the line (the part where the cap 'screws on' to the line) had given way. the nurses cut the line, and repaired it with a new end and basically a bunch of crazy glue. 2 weeks later, I had bloodwork done at the hospital through that line (first time I used that lumen since the repair). 2pm the nurse drew the blood and flushed the line. 7pm I was at home, septic, with a temp of over 105. It was unreal. I spent several days inpatient, on massive antibiotics, the line had to be pulled, placed a PICC line (central line that goes into the upper arm rather than the chest) and just overall got good and freaked at how horribly sick the infection (which was considered 'mild' for the organism they grew - and it was treated very fast... I cannot imagine a 'non-mild' version of this infection... the doc explained that the specific bug I had carried with it a 48-52% mortality rate. Scared me strait.

Fuelled by the terrifying sepsis ordeal and how close I realized I was walking to the edge while depending on TPN daily was a good kick in the butt. I called my oncologist and said I no longer want to trial anything - I want the most agressive, most likely to work treatment they could offer.

Within a week I had a vas cath (IV in the jugular that is the size of an HB pencil - i kid you not) and was starting a 14 day course of plasmapheresis (the treatment where the blood runs through a centrifuge and all my plasma is removed and replaced with donor products... 5 times over 2 weeks). Last Thursday I started my first round of IV chemotherapy drugs. These are the big guns and we are all very hopeful we will find some improvement with them.

So far the chemo is making me profoundly tired (told to expect that) but not horribly 'sick' like I expected, so that is a bonus. I spent the weekend once again shuttling back and forth to the ER for IV antibiotics for what was quickly evolving into a kidney infection - but again we nipped it early and only needed a few days of Rocephin.

My opinion of my local ER has grown immensely - the docs I have seen there lately have been fantastic, and the nurses are beyond awesome.

I have 3 courses of chemo left, and then we wait.

We are all hopeful it will work.

If it doesn't, we have other options... bigger, scarier and more sickening types of chemo, or (last resort) plasmapheresis forever (this poses serious and unique access issues - a small central line in the chest or arm is fine long term - a vas cath is massive, and has always led to signs of infection for me (high sed rate/ESR, left shift, elevated white counts, etc etc) - while I've never gone septic from one they have also never been in long enough to do much long range damage. Having a vas cath of any kind in one's body long term is a serious serious risk.

But - it's an option... and one that beats the alternative at least.

So we have lots of options yet - lots of hope - and several weeks to wait and hope that this current trial works.

I will very quietly say that my appetite is dramatically improved - I am eating small meals all day long and while they do hurt/make me sick, it's manageable - which it hasn't been since September - so this is a huge 'upside' - but we're told to expect it to get worse again as the plasma wears off - then slowly improve once more as the chemo works....

so roller coaster time.

Sadly we are in the process of trying to find a loving home for our 2 wonderful cats. because of the type of chemo im on, and cats knack of carrying/contracting/spreading otherwise harmless germs that could kill or maim an immunosuppressed person very rapidly, we have been forced to make the choice to find them new homes. We're all very sad. the boys are crushed. I'm very upset about it. But - it needs to be done. Life will go on.

Just one last stupid thing we have to 'lose' in order to try and 'live'.

Upside - everyone is fairly 'well' at the moment.

Chemo is going well.

My family is fantastic.

I'm beat and recognize how lucky I am to have a bed, in a warm house, with my loved ones, waiting for me to sleep in. Focusing on that.