Archive for September, 2013

I’ve been holding off on actually doing a detailed post until I got a couple appointments out of the way.

So here goes…

L had a Pulmonary appointment and Pulmonary Hypertension Specialist appointment on the same day.

PULMONARY

Pulmonary listened to her, and agreed something was wrong, but they thought her PH was getting worse. They shuffled us off to the PH specialist, but told us that we were to start giving her an inhaler before activity, as opposed to just giving it to her when she had a cold and we needed to do chest percussions.

We’re also supposed to keep monitoring her and the plan is to do a pulse-ox overnight test. 

He also had us sign a consent, saying “If the (PH) specialist wants to do a right-heart cath (which is the gold standard for getting a real read of pressures – echoes are just an estimate), that they would go ahead and do a bronchoscopy with bronchial lavage at the same time.” The bronchial lavage would be a salt water rinse of the lungs if they saw evidence of thick mucous.

PH SPECIALIST

The PH specialist did an echo, and said that the pressures in her heart looked great, and therefore he did not believe it was her PH getting worse. That, instead, it was her lungs. So he’s pushing it back onto Pulmonary, although he does agree with the inhaler before activity, Pulse-ox monitoring, etc.

He also said “If the (Pulmonary) specialist wants to do a bronchoscopy, we’ll go ahead and do a right-heart cath at the same time.” So do you see an issue there? Both of them want to do something, but don’t want to do it unless the other is going to do it. sigh

She had her first six-minute walk, and did fantastic on it. They don’t like to do them in kids younger than six, because of their ability to stay focused and such, but L did it with no problem whatsoever. I stood at one end of the hall, K at the other, and she got a kiss and a swift hug every time she reached one of us. She did 1100 in distance, and her sats stayed in a good range.

That’s another reason why the PH doctor thinks its pulmonary. He says if it was her PH, it would be affecting her all the time – not just sporadically. 

I wanted to yell at him at this point, because I felt at first like he wasn’t acknowledging something was going on, when its obvious that something is.  But he is acknowledging that something’s up, just that ‘objective evidence says its not her PH’.

So…

So now its wait, see, monitor, record, and poke both specialists frequently until we get some answers.

I understand why they don’t want to put her under. I do. Because each time she gets put under, her risks of complications from the anesthesia increase. The chances of her needing supplemental oxygen for a bit afterwards (because of the lavage) is higher.

But I still feel that we need to do it. We need to find out what is going on, specifically. Because something is

Also, the PH specialist does think we should have L tested for the FLNA mutation, as the genetics doctor had recommended, because it could ‘change the course of treatment’. He, however, did not say  HOW it would change things.

Genetics…

So I called Genetics, and asked them to submit the order for the FLNA and POL-G testing for L to the outpatient lab, so that next time L went for her monthly finger poke, we could just get it done at the same time. No extra trips for blood draws. No more pokes than necessary.

“Oh, well, we have to consult with the doctor to make sure its okay.” ARE YOU FREAKING KIDDING ME? THEY are the ones, in their letter to me about G’s condition, had said that they wanted to get L tested. They are the ones that said they feel strongly that L does have x-linked periventricular nodular heterotopia. They’re the ones that said contact us to get things set up. They met with us a couple times over G. They have all the family history they could possibly need. We know all they need to do is this blood test.

So how freaking hard is it to just order. the. damn. test?!

….

Anyways, so I gave L 2 puffs on her inhaler this morning. Then I took her to the babysitter’s house. I took the inhaler and the sat monitor over there. L has a meltdown. Complete and utter end of the world, how dare you leave me at the babysitter’s house, meltdown. Complete with purple lips. So I took her pulse ox.. 91! After a few minutes it got to 93, and that was it.

If we still had oxygen around, 93 would mean her tank was being turned on.

I am so, so freaking frustrated and irritated right now.

I’m not ready to start this fight again.

But… I will.

 

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Look at your child, and tell your child that you can make any one physical wish happen for them (within reason). 

They want to go to Disneyworld? Done.

A ride in a helicopter? Done.

Meet Dora the Explorer? Done.

Watch the excitement fill their eyes.

Now, tell them that they can have that one special wish, that people have made it possible, because they have a terminal illness.

Inform them, in the nicest way possible, that the world has shown its generosity to them because they are expected to Not Grow Up. 

Watch their reactions. Watch them struggle to understand what you are saying. Watch them begin to comprehend that they are different. That something is Wrong.

Do this (In your head), and you will perhaps begin to have a taste of what parents of children with terminal illnesses go through.