Monday, April 6, 2009


We got off to a late start but thankfully traffic was clear and we made it down to our appointment at UCLA ten minutes early. This was our first visit with the endocrinologist, and we were very pleased to find that the doctor was extremely knowledgeable and had a good bedside manner. That always helps.

Still the news was not easy to take. It is likely that Krista has adrenal insufficiency. That means that her adrenal glands aren't doing their job. This could be a problem with her hypothalamus or pituitary gland not creating the proper chemical signals to make the adrenal glands do their thing (secondary adrenal insufficiency). It's more likely, however, to be primary adrenal insufficiency which means that the adrenals are receiving the signals but are not responding. The endocrinologist said the latter would be more consistent with the high potassium and low sodium levels. (They usually see both low with secondary adrenal insufficiency.) The doctor gave her an ACTH stimulation test to make the diagnosis certain. The test is done by first testing her baseline blood levels, then injecting her with something that stimulates the adrenal-signal generation, and then testing her blood levels at 30 and 60 minutes after the injection. If the signals never get generated, that will show up on the tests. If the signals are generated but the adrenals don't respond, that will show up differently on the test. We have a follow-up appointment on Friday when the results should be available.

If the doctor's suspicions are correct, Krista's adrenal glands may have become over-stressed as a result of her previous liver-failure. The adrenal glands sit on top of the kidneys, and her kidneys were previously shut down as a result of the liver-failure. It's not too hard to imagine that the adrenals would be affected as well. If the adrenals are no longer functional, Krista will probably need to take a small dose of Medrol to replace the cortisone the adrenals produce, and Fludrocortisone to keep a balance between her sodium and potassium levels. She would need to take these two for the rest of her life. We have some concerns about long-term use of steroids, though. Krista had taken prednisone to reduce inflammation due to a lupus flare-up. It worked for a while, but after a couple of weeks the prednisone actually started causing inflammation. The doctor said that the doses are very gentle and only meant to replenish the natural levels of the missing hormones; other side-effects should be minimal.

As I mentioned earlier, the news of this was very difficult for Krista. She broke down in tears on hearing that her adrenals might be broken permanently. It helped to know that the medicines can manage the effects. But there was more to be upset about tonight... the mucositis that came back a couple of days ago seems to be spreading. There's a new sore right in the middle of her throat where she swallows. Aside from making eating difficult, it's also causing her to eat less. She's already been losing weight (also consistent with adrenal insufficiency) though she had been eating all the time because the Medrol makes her feel starving. This is all just the craziest set of contradictions, and it's very discouraging to Krista who has been trying so hard to do things right.

Out of the depths I cry to you, O LORD;
O Lord, hear my voice.
Let your ears be attentive
to my cry for mercy.
If you, O LORD, kept a record of sins,
O Lord, who could stand?
But with you there is forgiveness;
therefore you are feared.
I wait for the LORD, my soul waits,
and in his word I put my hope.
My soul waits for the Lord
more than watchmen wait for the morning,
more than watchmen wait for the morning.
O Israel, put your hope in the LORD,
for with the LORD is unfailing love
and with him is full redemption.
He himself will redeem Israel
from all their sins.
(Psalm 130)

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