Tenacity and Timing

One of the things that is so unnerving about DBA is that is unpredictable.

5-weeks between transfusions.  Then only 3.  Children that don’t respond to steriods suddenly responding.  And vice versa.  Iron appearing “suddenly” in organs that shouldn’t be overwhelmed yet, and then “suddenly” disappearing. Children with DBA across climate zones doing better around Christmastime; doing worse in the spring/summer, and then suddenly, it all switching up.

Or is it unpredictable?  Is it, perhaps, that we have yet to find the pattern?

Chinese medicine is all about patterns.  Connecting the dots between seemingly “random” symptoms is kind of the hallmark of what I do.  It is what I do best, and it is what I really enjoy doing.

But DBA is a puzzle fit to confound the most tenacious puzzle-master.

To let you in on my world a bit, let me give you my latest puzzle.  Like all DBA puzzles, it is a wait-and-see game.  It WILL resolve in time.  Positive or negative, we eventually find out if the child needs transfused, if the medication worked, and if things are currently “really bad,”  “really good,”  or somewhere in the middle.

It is the waiting that is the hardest.  Like much of life, it is the not knowing that is most difficult.  And this is why, when people come to me with their health puzzles, they feel so satisfied working with me.  I show them why it all makes sense.  I don’t take away any mystery or miracles, but I do help them complete their connect-the-dots.  It actually makes the miracle and mystery all the grander.

I only wish I could do it for my own daughter.

And perhaps you have some insight.  So here it is:

Adahlia is due for her t2* MRI.  Actually, she is overdue.  She was due to be scheduled for this examination back in September of 2016.  As of mid-October, she was overdue.  This is the radiological study that (imperfectly) measures the amount of iron that has built up in her heart and liver from so many blood transfusions.  (We do not have a natural way to rid ourselves of iron.  Because it is so valuable, our body stores whatever iron it cannot use immediately.  In someone who is chronically transfused, who receives a massive influx of iron monthly, this creates a problem.  Iron stored in the heart can lead to heart failure (ie, death) and so goes the story for the liver and other organs.  And so Adahlia takes a chelation medicine, which pulls it out of her organs for her.)

Typically, iron is stored in (more-or-less of) the following sequence:  Liver. Pancreas.  Heart.  Pituitary.  But this is not always true.  Some DBA folks store it in other orders.  Adahlia, for example, seems to preferentially load iron into her heart.

Because of hospital costs and limitations of equipment and software (not all hospitals can “read” all organs), most DBA patients do not receive full studies of all organs.  The hospital takes a reading of the liver.  Or if lucky, the liver and heart.  Extrapolations are made about guess-timated levels of iron overload in the rest of the body.

This is obviously an imperfect system, since the children do not load into all organs the same across the board.  A pancreas could be bursting with iron and no one ever know until it shuts down and the child is suddenly diabetic, or worse.

I’ve been trying to schedule A’s t2* MRIs since August, and to no avail.

There are scheduling difficulties.

Adahlia needs her t2* exam conducted prior to blood transfusion, reportedly so that it is the clearest reading possible.  This means we cannot transfuse her a week prior (and certainly not the day or morning prior) to the exam.

We need her t2* and transfusion to be conducted on the same day. First, so as to minimize emotional trauma, and second to save her veins from unnecessary needle sticks (they are beginning to form scar tissue and may become unusable).  When conducted on the same day, Adahlia receives only one IV stick for both procedures– first the IV is used for the sedation necessary for the MRI, and second it is used for the donated blood.

These scheduling requirements mean that we need the earliest possible MRI appointment in the day.  First, because the MRI requires a couple hours and the transfusion requires a few hours and the hospital won’t start a blood transfusion after 2 pm.  And second, because the MRI requires sedation, and to be safe, the child cannot eat or drink ANYTHING (including water) for something like 6-8 hours prior to sedation.  Denying your child food and water is tough.  So most parents, like myself, try to schedule the “MRI Under Sedation” first thing in the morning, so as to minimize the child’s hunger and thirst.  This means we have a lot of competition for prized appointments.

The problem is that this combination of requirements allows us a window of approximately one week per month to schedule the necessary procedures. In January, we were all set-to-go, but Adahlia came down with a wicked chest and upper respiratory cold.  It is unsafe to put a child under sedation when she has a cough/mucus, and so, confounded by yet another requirement, the t2* MRI was cancelled and reschedule for — you guessed it, March.

But last month she burned through her blood at record pace.  So instead of going an easy four weeks between transfusion (transfused at the upper-end of Hb range), she barely made it 3 weeks (and had a shockingly-low Hb count).

To make it to her March MRI + transfusion, she needed her blood to last 4.5 weeks.

Not impossible in the past.  But…

She hasn’t been looking so good.

And there’s another requirement to the MRI Under Sedation:

It is reportedly unsafe to do if the Hb is under 6.0 due to the risk of heart failure / not waking up.  (Some hospitals actually say they won’t do it under 8.0.)

So last week, I called her hematology team and told them she wasn’t looking so good.  I said I doubted she’d make it to March 14th, and asked if they could check to see if anyone had cancelled their morning MRI this week, so we could bump it all up by week.

They were not pleased.  As if I was somehow foiling their plans, instead of making sure the plan could go through. They told me they’d call me back.

And quite honestly, I don’t think anyone even checked.  I heard nothing back from them.

(I can’t call Radiology myself.  Because Adahlia is a Cancer/Blood Disorders patient, all requests for appointments must be made by her Hematology Scheduler.  Trust me, I’ve tried to go around this system in the past — it would just be so much easier if I could arrange it myself, as I care more about getting her appointments set up correctly than any scheduler ever could.  But it didn’t work out well and it didn’t make me any friends.)

I’m not entirely sure why Adahlia’s hematology team is dropping the ball on this so hard.

It may be because her last t2* MRI was entirely cleared of cardiac iron and liver iron was down to a moderate range (back in October 2015).  Six months prior to that, her hematologist was conferencing across the country about her, as her cardiac iron was extremely high and they were worried about heart failure, as historically, the heart is the toughest organ to remove iron from.  They even managed to schedule a repeat ‘critical’ t2* MRI exactly six months later for her. But, at that second MRI, after intense chelation and some of my complementary Japanese moxabustion and Shonishin therapies, the iron was completely gone from her heart.  Her heart was functioning at a very high level.  Like the problem never existed.

Their reluctance to push for Adahlia may also be because a few months ago, I had a relatively illuminating argument with Adahlia’s lead hematolgist about steriod treatment.

A paragraph or so into his steroid pitch, A’s doc made the mistake of saying that ultimately, he just wanted A to have the “opportunity” to try steroids.  

As if insinuating that I was not doing the proper job of a mama.  That I was refusing her “opportunities” in life.  

Now, I had been expecting this conversation.  It is A’s doc’s job, after all, to make sure his patients follow current protocols, or at least that he is strongly recommending them.  I was sure his bosses, whoever they are, who track these rare DBA cases, were all over him about why we hadn’t put this particular child on steroids.  And I was sure that somewhere in her file, like the Seinfield skit, there was also mention of how troublesome I am as her mother.

Knowing he has a job to do and medical license to protect, I was willing to tolerate what I knew would be his every-3-months steroid pitch.  And I’m perfectly fine with having a cost-and-benefit, logical, and analytical discussion about steroids and whether or not we are ready to try them (I’ve explained my reasons to him.  And we arent’t “against” steroids.  But we haven’t been ready to try them.)  

To use this particular angle — to put such a coated and manipulative and salesmany spin on the disagreement — well, it was not acceptable to me.   I lost it.

“Opportunity?!?”  I retorted.  “The only reason you are looking at the little girl that you are looking at is because I REFUSED the so-called “opportunity” of steriods that was so strongly pushed at us when she was just 9 months old that her doctors stopped speaking to me.  Had I taken the “opportunity” you all medically recommended, you would NOT be seeing the developmentally normal little girl you now see before you.”

I paused, glaring, and he obliged me and looked at her.  And I watched his face change, as he and I both knew what we would have been seeing, what her chart would be reading, had she gone on steroids.  And what we’d be looking at it in just two more years — a 6 year old little girl going through puberty.  A 6-year-old the size of a 3-year-old.  

“And now, NOW you say that you’d never recommend steroids to a child that young, that the ‘guidance’ has changed, but it would have been too late for her, wouldn’t it have been?  It is MY JOB to sift through your medical recommendations — recommendations that I understand you HAVE to follow or you will lose your license — for things that actually make sense.  And it is only because of MY OWN medical training that I have any ability whatsoever to do so.  My intention is provide her the BEST POSSIBLE opportunities in life, and the way to do that, in my opinion, is to keep her as healthy as possible.  To NOT forever alter her future. My job is to minimize any LASTING and IRREVERSIBLE effects.  And so far, we have done that.”

He did not argue with me.  He knew I was right.  While he may not volunteer the information and connections between steroid use and diabetes and osteoporsis and precocious puberty and severe growth stunting and 8 year olds with joint degeneration when he “sells” steroids (his word, not mine), he knows the effects of them.  

And so he replied, “You have to understand,” he said, “my job is to sell the steroids.”

His posture changed and his energy grew somber and reflective.

“At night,” he admitted, “I think about the girls and boys who I have physically altered with steroids.  How I’ve destroyed their bodies and changed their futures.”  And then he looked up.  “But for some of these children, there was no choice.  They would have died without the steroids.”

I was astounded.  It is not everyday nor every medical professional who can admit that he or she “destroys” the bodies of children (again, his wording, not mine).

“But that is NOT her situation,” I growled.  “She HAS another option.  And it is not perfect.  And it is dangerous.  But if we are vigilant and lucky, it will not PERMANENTLY change her body. It will not destroy her own natural hormones and endocrine system, and irreversibly alter her chance at a ‘normal’ future.  If her situation changes, and we must use steroids to save her life, or if I feel there is a chance that the steroids might ‘jump-start’ her system, so that she needs them only temporarily, then we may try that.  But I will not let you leave her on them.  Even if they work.  And it will kill me to administer them to her.”


Back to the present — storytime over…

Whatever the reason, I’m not super thrilled either way about how Adahlia’s medical care has been over the last 6 moths.

Last month’s conversation with her team was a little stilted.  Adahlia’s heme team expressed “surprise” that she still hadn’t had her necessary yearly iron overload t2* exam.  And they were “upset” it was so overdue.  (And of course, it was their own / the hospital’s fault for not prioritizing it.  So I found their expressions of shock unimpressive.  If its so damn important, SCHEDULE IT, people!!  I should think that she’d get some sort of priority.)

So what’s my point?

My point… my point….

My point is that I’d rather not have Adahlia’s heart stop while under sedation.

And I’m a little upset that the hospital doesn’t even seem to care to find out if the procedure will be safe for her.  (They don’t test the Hb prior to sedation.)

And so I must —- again —- be the Tiger-Bear Mama.

My plan?

Well, her t2* is scheduled for Tuesday.  The day before, on Monday, I’m thinking of taking her to get her Hb checked via finger-poke at a closer hospital branch (we go to the Main Campus near Denver for transfusion).  I’ll call the main hospital that afternoon to get the results, and if its too low, I will cancel the MRI.

Until then, Adahlia will continue blade-running her hemaglobin.  She’s low, but I think she can make it to Tuesday.  It doesn’t make much sense to check her before Monday if they aren’t able to alter the plans to see her before then, anyway.

I just refuse to let them sedate her, and have our story end with:

“We are so sorry.  We tried everything….”

I just refuse to let that happen.  I’m not going to let the stupidity of medical protocol / medical error destroy my child.  She’s MY charge.  She’s my Light Being to Raise Up.  And I take that duty and honor seriously.

So, does everyone agree — Good plan?

Alright then.  On the count of three, let’s do this.

One-two-three:  TEAM!