Monday, January 23, 2012

What’s been going on…

This post is for all you who have been wondering what on earth has been going on with All the Marletts…thanks for caring.

If our main doc got paid for each and every e-mail he receives and sends about Lucy he just might have enough to retire, but then we would be without him, and that would be no good!  Last night I sent an e-mail to our good doc who just so happens to be in South America helping another family in need, yeah he is amazing like that!  He e-mailed me back saying that he is “concerned”, and then discussed a plan that he was going to relay back to the other doctors in the group.  Concern, concerning, concerned, those three tenses just about sum up Lucy’s past, present, and future health! 

I have mentioned on more than a few occasions how Lucy has been battling reoccurring yeast, she is coated with it from head to toe!  In particular she has been suffering with severe oral thrush that we just can’t get rid of!  After several e-mails, discussions, appointments, and cultures, we learned that Lucy is growing a very rare form of candida yeast that is resistant to the antifungal that we have been using to treat it.  Infectious disease was consulted, and it was decided that we need switch Lucy’s antifungal.  This type of med change typically requires a stay in the hospital; however, Dr. R was able to get permission from infectious disease to allow us to have her first infusion given in day med.  This  meant no overnight hospital admission, but if an admission was the only way she was going to be able to get the med, we were willing to do it.   That’s how badly she needed to get started on this med!  Poor girly, the corners of her lips and and mouth were covered in blisters.   

Since starting Lucy on the new anti-fungal on Monday, she has developed quite a few concerning symptoms: diarrhea, fever, tachycardia, erratic heart rates, hypotension, and vasodilation.  Why am I surprised(insert sarcasm, fatigue, and worry)?  Antifungals are difficult meds to be on, this particular med that Lucy is on came with an eight page packet of side effects.  What’s confusing is that our girl already experiences a lot to the side effects of the medication.  After talking with the on call doc this weekend, we have decided to do an on-off-on trial with Lucy to see if indeed her symptoms are related to the new med, if her symptoms improve while off of the med then we are going to have to decide whether to continue or change her to a different anti-fungal.  Of course our main doc is still out of the country which makes these decisions all the more challenging.

In other issues, because you know there’s always more, we recently had an appointment with pulmonology.  Lucy is not new to pulmonology, early on her life we had a need to definitively rule out cystic fibrosis as a possible diagnosis.  However we  have been referred to a new pulmonologist, one who specializes in neuromuscular diseases.  In the past few months we have struggled with seeing a decline in Lucy’s overall muscle strength and vascular health.  She is a shallow breather, she has been most of her life and her body has compensated for this up until now.  Her need for supplemental oxygen is not going away post transfusion, she visually looks blue at times, and her profusion in her hands and feet are little to none .  The thought is that everything that we are seeing in Lucy is as a result of disease progression, and that Lucy is in need of more support respiratory wise.   A sleep study was ordered to get a better picture of what is happening to her when she is sleeping, discussions about which meds we can use to help open up and clear out her lungs were had, and orders for chest PT, a.k.a vest therapy, were given.  We don’t disagree with any of these recommendations, we feel that they will help to keep Lucy at her best.  What is difficult is knowing that we are having discussions about disease progression and not being able to do anything to prevent it!  We can treat the symptoms but not the disease.

5 comments:

Kate said...

Yuck. Praying for good, accurate information regarding the antifungal and that Lucy will be able to have the yeast treated effectively. Praying also for all of the respiratory decisions to be made!
Blessings,
Kate

Cory said...

Our continued prayers are with you. (And yeah, we were part of the gang wondering what was up with All the Marletts! ha!)
Peace,
Cory (Mom to Lizzie, Charlotte, and Cara from Musik)

Susan said...

Thanks for the update. I've been thinking about you all, and wondering how things are going. I'm sorry to hear that things are so challenging. May God give you wisdom as you navigate all these decisions. Are you guys going to try to come to the MOPS and POPS event on Thursday night? Hope to see you there.
- Susan

Jessica said...

I've definitely been wondering how things were going, so thank you for the update. :)

Eithene struggled with severe oral thrush for almost all of the last year and a half of her life. We probably did 40 weeks of IV fluconazole and a good 20 more of IV mycafungin to just keep the yeast at bay in her GI tract, diaper area, and bladder (also an attempt to keep it out of her blood, of course).

I remember that when I'd describe the problem to new drs, they would look at me like I was nuts, with an "all this fuss over a thrush infection?" attitude clearly visible. But, it was a huge detriment to Eithene's quality of life. At times her oral thrush was so bad her tong and lips would just bleed, and once the yeast from her mouth spread all over her poor little face in pustules. It was painful to her, made speech difficult, and radically increased her thirst.

Of course, it was a risk to her life too. Anytime the yeast made it into her urine, she started to get septic within a day or two. And bugs from her gut were *constantly* leaking out into her bladder, so the fact that her entire GI tract was coated in yeast was a very serious problem!

We mostly battled Candida Lusitanea and Candida Paropsilosis. Both were multi-drug resistant, and the paropsilosis was completely resistant to the mycafungin and only partially sensitive to the fluconazole... Unfortunately, she couldn't take any of the enteral meds, and amphoterracin/ambizone weren't really options for Eithene, since her 1 kidney was already stressed out, her electrolyte status was already a disaster, and she already had daily life-threatening arrythmias from low K.

It was a real pickle. I remember we saw several different Gi drs, ID drs, and even the hospital dental team to try and find anti-thrush suggestions. So, I feel your pain, and hope that you can find the best antifungal regimen for Lucy!

Eithene also developed respiratory failure as her disease progressed- we found that adding in nighttime BiPAP was very good for her, and though it didn't stop all of her CO2 retention (from her inability to expand her lungs fully), it really helped! COntinuous O2 use was also helpful, as it lowered her heart and respiration rates, which saved her body energy (though it increased CO2 retention, so we had to be sure to have her on the BiPAP at nights).

megan said...

Haven't seen you guys in awhile but certainly have tried to keep up with Ms. Lucy! Thinking and praying for you!