Update: Any ideas or feedback

[Guest guest]

Hi all :-)

Read daily but have not had much of a chance lately to reply, but do

have everyone in my thoughts and prayers.

Things are going a little hay wire here and of course, its all unusual

stuff the docs have not seen before and don't really know how to address.

Chance(9) started having problems with chest pain and shortness of breath

during exercising last fall and it progressively got worse. In December

our cardiologist did exercise testing (normal), an Echo (normal) and pulmonary

function testing (PFT's)(mildly abnormal before exercise, severely abnormal

after exercise). They tried giving him a breathing treatment of Albuterol

and then re-did the PFT's, but he actually got worse after the breathing

treatment. Cardio dx'd exercise induced Asthma and he was put on

a Foradil aerolizer twice a day. There was no improvement and a continued

decline.

January we saw the pulmonologist and PFT's were re-done. They

had gotten worse and he too tried a breathing treatment, and again, his

PFT's got worse after the treatment. Also has a very flattened Flow

Loop which means he's not breathing in as deeply as he should, nor breathing

out like he should. Nixed the asthma dx at that time and said he thought

it was Tracheomalacia (floppy airway collapsing in on itself) and maybe

inflammation. Scheduled a bronchoscopy and bronchial lavage

if needed. Started him on Advair 250 2x a day. Two weeks later since

there had been no improvement based on symptoms, we added Foradil as needed

before exercising. We saw some mild improvement with this combo,

though it quickly started to diminish.

April went back to repeat PFT's and see if there was some improvement.

He had lost another 5% function (down to 65% now), Flow Loop remains flattened,

and had begun having symptoms apart from exercise by this time. Symptoms

now include pain at the base of the sternum, shortness of breath, and times

where he feels like he cannot get a deep breath, mostly with exercise,

but sometimes without. Pulmo notes a slight wheeze in left lung,

something we've not heard before. Starts him on Prednisone, 80mgs

a day for 5 days, we continue with the other meds, and he schedules a pH

probe to see if reflux is playing a part. We were to return in one

week and repeat PFT's.

Went in on Monday (took Pred Sunday-Thursday of last week) and repeated

PFT's. He had lost another 2% function in less than 2 weeks while

on prednisone. Flow Loop still very flattened. Calls and gets

us into Pulmonary Lab that day to do Heliox testing (they have him breath

a helium oxygen mixture for 20 minutes and do PFT's before and after).

Had there been some change in the PFT's after the Heliox, would mean he

had Vocal Chord Dysfunction, however, there was no change, so thats not

the answer. Then went over and had the pH probe placed.

pH probe was removed yesterday morning and we should have results in

the next day or two, though now neither the pulmo or me think that reflux

is the root cause, or the pred would have taken care of it for atleast

a little while. Honestly, he is as stumped as he can be and I have

no idea where we go from here.

All of the reports are saying that there is some kind of airway obstruction

causing the problem, though nothing the pulmo saw on the bronchoscopy was

significant enough to explain the loss seen on the PFT's. He did

confirm the Tracheomalacia, as well as Bronchomalacia....both never seen

in kids this age that have not been intubated or had a trach (Chance has

had neither).... there was a constriction seen in the left bronchial tube...and

the lavage fluid showed inflammation.

Chance is now in pain almost all the time and is absolutly exhausted

(even fell asleep on the exam table at the cardiologists yesterday and

this is not a kid that sleeps during the day and is always anxious at doctor

visits).

Then he saw Madison(6) who had full PFT's done last year in April and

all was normal. PFT's this time, one year later, showed a severe

decrease in PFT's (a drop of 17%), a breathing treatment was tried, and

like her brother, she got worse afterwards (dropped another 5%).

He thinks she too has the Tracheomalacia and maybe more. At the moment

we're trying Pulmicort with her and will go back the end of this month

to repeat PFT's and see if there has been any improvement (though he doesn't

expect there will be based on what we have seen with Chance). At

that time he'll decide if he's going to do a bronchoscopy on her also while

she's under getting her ear tubes (3rd set and permanent this time) in

early June.

So, in a nutshell, both are losing about 5% function every 2 months,

they are not responsive to anti-inflammatories, get worse with bronchodilators,

don't have vocal chord dysfunction, and we're pretty much stumped and not

sure where we go from here. If anyone has any ideas, I am getting

desperate. We cannot keep going downhill at this rate.

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

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