Good to Have Goals…

And every time I sit down to write I get distracted.  Forgive my attention issues.  There seems to be too much to attend to at once – and I have issues.

I didn’t want to go today.  I didn’t.  But I had to.  Cause it’s time.  It’s actually way past time to address the chronic, throbbing, aching in my legs.  I saw a doctor last summer when I was near the end of my rope.  That was a mess, which ended with me quite sure that a doctor with a staff that obnoxious would never be operating on me.

end of my rope

And, maybe it was for the better.  Because I never felt quite right about him anyway.  So I pulled myself together and I went to another doctor.  A second opinion.  This one was worried about an abdominal aneurysm, which thankfully was not the case, but at least he paid some attention.  “You need a CT scan,” he said.  “I need to make sure nothing vascular is wrong in your abdomen triggering all these veins to go bad. ‘ (I’ve had 7 addressed so far.)

I explained that I should only have CT scans when absolutely necessary.  I told him about the radiation risk, which is especially dangerous for those of us with a PTEN mutation when cells can misbehave and develop into tumors of all sorts with ridiculous frequency – especially when provoked.  I even explained I was allergic to CT contrast dye.

I was sent for the scan – premedicated for the allergy.  Left only to pray that the radiation minded its own business.  And the report came, and there were unsettling omissions – like checking the box that my ovaries and uterus (gone now since May 2012) were doing fine, and ignoring the large tumors being carefully watched on my spleen, and the cyst on my kidney.  I was bothered.  The doctor called to go over the report with me but didn’t have my chart and couldn’t answer my questions.  I had the report amended.  I tried to find someone else to read the disk.  I thought I had struck gold in November when a doctor took an interest in our case, but that – like all other things – was not to be.  So I waited a few more months.  When January came and I was pretty sure Meghan would not be using the February week for surgery, I called to schedule mine.  It was time to get the vein removed – starting with the right one.  Triage.

I asked if I should see the doctor again since it had been a few months.  I was told no, and given a date for the surgery.

hand in a door

Today was Pre Admission Testing at the Hospital.  I think I’d enjoy getting my hand stuck in a door more than that.  It is a mess of people who know very little asking the same questions over and over when they don’t know the answer.  Instead of Pre testing, perhaps they should call it CYA – a way to prove to everyone you were healthy before they did whatever you needed.

Stop all your over the counter medications today.

Why?

Because some of them thin blood.

Which ones?

I don’t know – but they tell us to have you stop all of them.

My probiotic thins blood?

I just have to tell you to stop them all.

sigh

We were moving along until I read the consent form.  “Left leg.”

Um… right leg…

At first she questioned me.  Then she picked up the phone to question the doctor.  The answer came with a new consent form and a request for me to see the doctor.  Tomorrow between 9 and 2.  Um, no.  How about Tuesday?  That I can do.  Cause I LOVE spending my entire vacation – every time – in a doctor’s office of some type.

And as I was leaving she said, “You forgot your chest X-ray.”

Insert speech about radiation and Cowden’s here.  As well as, why would I need a chest X-ray if I am not sick and my lungs are clear?

Right.  You can get your x-ray down the hall.

radiation

No one asked for a supervisor.  No one checked in on anything.  This woman undoubtedly knew nothing of my condition, only told me my procedure would be cancelled if I didn’t go.  For a hot second I did wonder if that wasn’t a better idea.  But, there was a kid to get home to, and a dance she wanted to get to, and so many reasons to run away, and so many reasons it has to get done.  But, in the end the pain in the legs drove me down the hall.  Where I took another, albeit small, hit of radiation to the spot where my breasts that were removed harboring early stage breast cancer once lived.  But, for good measure they put a cape on my abdomen.  To protect the uterus and ovaries THAT AREN’T THERE!

I left angry.  Sad.  Mad.  Frustrated.  And grateful.

Grateful at least that the bungling was being practiced on me and not Meghan.

Next Thursday the 19th.  It’s a minor procedure.  But, wow.  I think I need some prayers.

And them Friday the 20th it’s off to the orthopedist for Meghan

You see the MRI she had January 22nd – that I battled to have read until February 2nd- showed a decent size residual AVM in the knee.  It’s not gone.  In reality, its not much smaller than it was.  Perhaps a bit less angry, without as intense of a blood flow, but yet still 2 x 1 cm embedded in the meniscus, and aggravating all sorts of other things.

ER 11/24
ER 11/24

And just for fun this MRI showed evidence of problems in the knee itself.  Too much messing around with loose blood flow for too long.  There is some deterioration and its time for an orthopedic surgeon, the chief at a Long Island Children’s hospital to take a look.

I didn’t write the week I was ready to string up the vascular surgeon for not getting back to me.  I thought it better to say nothing, because I could find NOTHING nice to say that week.  Now,  I am calmer.  And we need him.  So I breathe deeply.  And I told his PA in my calmest angry voice, that no matter how busy they get, they need not forget there are humans, with families and real pains, and anxieties on the other end of those CD images.

In reality it doesn’t matter, because I suspect there is no good solution.  They offered us another embolization.  She recovers poorly from them.  And truth be told, they aren’t working.  And since insanity is defined as doing the same thing over and over expecting a different result – we decided to hold off a bit.

Just-Tired

Plus, there is the whole endocrine thing to deal with.  Like the TSH level that again doubled in 6 weeks on a higher dose of synthroid.  And a kid who is functioning with numbers that would level me.  So the doctor said, “I can’t explain it.”  And he won’t prescribe an alternative medication.  But he acknowledged the idea of insanity, and raising her synthroid again with no plan is definitely insanity.  So we talked about T4 (Synthroid) and how she is at the upper dosing for her weight.  And then we talked about how its the body’s job to change T4 into T3 so it can be used.  So, I asked if it was possible that her blood levels of T4 were there, but her body was flubbing the conversion, which it has done before.  If that was the case, that would explain her symptoms.  So, we added some T3 to her existing dose of T4.  And then she gets to wait 6 weeks again for a chance she may feel a but human.

Thankfully we’ve chosen to surround ourselves with positive distractions.

Thankfully 163 people are coming on Sunday to the Jeans for Rare Genes fundraiser, benefiting the PTEN foundation and the Global Genes Project.

Fortunately, my girl had influenced lots of people, and motivated them to raise awareness and support her favorite charities.

Fortunately, we have places to look to besides ourselves.

As I sit here with the seating chart to my right, and raffle prices to be typed on my left, I am grateful that there is a project.  I am humbled by my girl and her determination to be “normal,” and a powerful advocate all at the same time.

photo 4 (1)

It will all work out.  It’s got to.

We’ve got things to do…

We are BEATINGCOWDENS!

Rare Disease Day Fundraiser

The Productive Struggle

Tomorrow I was scheduled to attend a professional development workshop entitled “The Productive Struggle.”  I was kind of excited to go, as it sounded like quality PD right up my alley.  “The Productive Struggle” is supposed to focus on helping students find their way through the rigorous problems and questions of the Common Core Learning Standards (CCLS.)  And regardless of your opinion on standardized testing, when you break down the basic principles of these standards to their center, there is much value, and much to learn.

As a math teacher I like to focus on having students “Persevere to solve problems.”

productive struggle 3

This is in no way as easy as it sounds.  Children are programmed to wait.  They want the “right” answer.  They fear trying and making mistakes, and learning, and trying again.

As a teacher it is my job to give them the skills they need to solve problems.  I need to make sure their knowledge of math is broad.  I need to tap off the skills their classroom teachers have taught.  I need to set firm boundaries and standards for not giving up.  Then I need to step back and let it happen.

Some days it is like a well rehearsed symphony.  And other days it sounds a bit like an early childhood music class.  But I try, and try again, and we make progress.  Because giving up is not an option.

I have had some time to think, being cooped up here in the hospital this last week.  And tonight it is easy to understand while I am glad to be by my daughter’s side, I am sad to miss any lesson in the “productive struggle.”

productive struggle 2

In many ways it is what is missing in our lives as patients with Rare Diseases.

Follow my logic, using my Meghan as an example.

So Thursday she woke up vomiting bile, and in tons of pain.

Virus?  Nope.

Flu?  Nope.

Terrible reflux, awful stomach cramps.

Appendix? Nope.

Fever, back pain.

Kidneys? Nope.

productive struggle

And so it continued, and still continues.  We have met many nice, well intention-ed doctors and residents.  And we have met a few that should really use their intelligence to become researchers and stay far away from people.

But 5 days later my daughter still sits with stomach pain, difficulty eating, and some pretty significant GI issues.

We have had ultrasound, CT scan, and countless lab tests.  Tomorrow she will have endoscopy at 2 PM.

Some of the medication changes have been beneficial.  And we may see a slight decrease in the severity of her pain tonight.

But, what is the root of the problem?

She didn’t develop GI issues so severe they require a lengthy hospital stay without there being any connection to the rest of her body.

I have a 4 inch thick binder with medical history.  It reads as a chronology of 11 surgeries, several hospitalizations, outpatient procedures and more.  There is lab work, sonograms, MRI reports.  I actively cataloged the whole thing by date.  And I brought it.  And I offered it to the doctors.

And then I took it home.  No one even laughed at the cartoon on the cover.  I’m not sure it was ever opened.

binder cover

See the problem we have, Meghan and I, and so many others, transcends Cowden’s Syndrome.

The problem is that most practicing doctors have never encountered, experienced, or worked through the “Productive Struggle.”  So when a tough case hits them – they only do what they would normally do.  And when all those tests are fruitless, instead of thinking outside the box – they assume OUR box is broken.

Meghan and I went through the chronology of the last year together last night.  We spoke about the AVM on her hand.  We continued through the MRIs that exposed her to extreme amounts of gadolinium.  We talked about the two rounds of heavy metal chelation, and how even though she excreted significant heavy metal, the rebound effect triggered crippling migraine headaches.  We recalled the neurologist, and the instant diagnosis of migraine without much history, touting them as “very common.”  We remembered another MRI to make sure the brain was clear.  We talked about the medicine for the migraines and whether it really worked, and how it kept getting raised every time she had another headache.  Raised, not changed.  We talked about the tickle in her throat that started in November, and the neurologist’s flippant remark that it might “just be a tic.”  We talked about the neurologist telling us to increase the Celebrex to stop her last migraine – which it did, but it was never his drug to change to begin with and he never consulted the prescribing doctor.  We talked about the thyroid nodule, and the subsequent biopsy and thyroidectomy.  We talked about the surgery and the hospitalization that followed with GI issues in February.  We spoke about the throat clearing “tic?” that we had hoped would resolve after the thyroidectomy.  Then we spoke about thyroid hormones, and what having a body in a hypothyroid state for months can do.  We spoke about the ENT and his determination that there was inflammation in the nose.  We discussed his nasal spray and admonition to double the reflux medicine.

And then, after ten minutes of talking out the medical highlights of the last 10 months we realized NO ONE had asked about any of that.

It was like she is here being treated for a totally separate problem as a kid who “happens to have” Cowden’s Syndrome.

So they order their tests.  One at a time.  And they watch and wait.  And we go for ultrasound and CT scan.  And we change-up some medications.

But no one asks what the effects will be of her stopping Celebrex.  No one asks why her body required such a dose, even as I start to see the initial effects of the NSAIDs  being totally our of her system.

They come in and tap her belly.  It still hurts in the same spots.  But that’s not what they want.  They did what they know.  Now they want it to be better.

I have to be here with Meghan tomorrow, but there are a few of them that could stand to attend the PD on the “Productive Struggle.”

Goodness, work for it people.  It’s not always easy.

But she’s young.  And she’s bright.  And she’s frightened.  And she’s worth it.

You Never Know How Strong You Are Until Being Strong Is The Only Choice You Have