On Monday, August 28 I dropped Ayva and Abby off at school and Emma and I hit the road up Highway 99 on the 121 mile drive to Valley Children's Hospital in Madera. This road knows Emma and I well, as we have traveled it countless times over her life. Emma and I have found joys and heartache on this highway, and this day, proved to be the same.
As we sat waiting to see Emma's pulmonologist, we tried to stay entertained, Emma engulfed on the ipad with headphones in watching "Once Upon a Time" and me scrolling through Facebook getting caught up on everyone's life. Her doctor entered with a rather grim look on his face which, caught me a little off guard since he is normally full of smiles. His first few words were "Emma has gone from complicated to extremely complex." He pulled up a chair, sat down and began to explain: The fungus they found in her lungs while she was admitted was different from what they originally thought and we needed to make some changes. Emma is growing a black yeast in her lungs. It is very rarely found in humans, but has been known to colonize in lungs of those with Cystic Fibrosis. This is difficult to treat, the IV medication that she has been on since being discharged from the hospital is not working because sensitivity levels showed she is resistant to it.
So we are back to the drawing board...the doctor is hoping and working to get her approved for an antibiotic that she would take via injection twice monthly, but, like all IV Antibiotics, there are some risks involved with this drug. If we cannot get her approved for this med, there is another alternative, but it has very serious complications to her liver, so we are praying that is not the option we have to take.
Which brings us to issue number two and three...
These next two issues go hand in hand... first off, Emma is being treated for ABPA (Allergic bronchopulmonary aspergillosis) with high doses of prednisone to help bring her allergic reaction to it down. This is a normal treatment for ABPA.
Secondly, Emma's bronchoscopy showed that she is growing mycobacterium abscessus again. Emma originally tested positive in North Carolina and has been on a six antibiotics treatment therapy since March 2016 (yes for 17 months!!). She went 10 months (no break from the treatment plan during this time) with negative cultures but M. Abscessus has once again reared its evil head!! So, we now will continue for other 1-3 years on this 6 antibiotic treatment pending future culture results.
Now, here lays the issue: the prednisone that she is on for the ABPA causes the m. Abscessus to grow like wildfire, so we have to slowly wean her off. This will take approximately 4-6 weeks. In the meantime, we risk the M. Abscessus growing very rapidly. Once she is weaned off, she will have to have another surgery to scope her lungs and see how things are looking.
Secondly, Emma's bronchoscopy showed that she is growing mycobacterium abscessus again. Emma originally tested positive in North Carolina and has been on a six antibiotics treatment therapy since March 2016 (yes for 17 months!!). She went 10 months (no break from the treatment plan during this time) with negative cultures but M. Abscessus has once again reared its evil head!! So, we now will continue for other 1-3 years on this 6 antibiotic treatment pending future culture results.
Now, here lays the issue: the prednisone that she is on for the ABPA causes the m. Abscessus to grow like wildfire, so we have to slowly wean her off. This will take approximately 4-6 weeks. In the meantime, we risk the M. Abscessus growing very rapidly. Once she is weaned off, she will have to have another surgery to scope her lungs and see how things are looking.
The M. Abscessus is our biggest concern at this point. Mycobacterium abscessus is a multiresistant, non-tuberculous mycobacteria. It is associated with a rapid deterioration in lung function and increased rates of morbidity and mortality. It requires long term multi-antibiotic treatment and limits or removes their ability to receive a lung transplant.
The bottom line is Cystic Fibrosis is an invisable illness that takes a beautiful, blonde, 11 year old and destroys her lungs. It's a vicious monster that tries to rob her of her breath and freedom. Many advances have happened over the year in the world of CF, but these warriors are still fighting a never-ending battle with no cure. We are constantly saying goodbye way to early to these precious lives, and I wish we could bring a stop to that!! Please pray for a cure for all those enduring this illness!!
Now with this being said, Matt and I are diligently looking into some option to help Emma and our family as a whole. We are discussing these with her team, and have her doctor's complete support on some potential upcoming decisions. I will explain this is further detail in the near future after we have some final answers. I just ask, in the meantime that you would please partner with us as we pray for clear direction and open doors.
We are so grateful to each of you for your continued love and support. This has been a very long road, but the journey isn't over yet! We are trusting that the Lord has all things in control and has a purpose and plan for it all. We are humbled that He would allow us to be Emma's (and the other girls) parents and that He trust us to endure this life with her.
Friends, remember no matter what you are enduring today to "consider it pure joy when you face trials of many kinds, because you know that the testing of your faith develops perseverance. Let perseverance finish its work so you can be complete and mature, lacking nothing" James 1:2-4. Hang in there... God has a plan for everything!!
We love y'all!!
The bottom line is Cystic Fibrosis is an invisable illness that takes a beautiful, blonde, 11 year old and destroys her lungs. It's a vicious monster that tries to rob her of her breath and freedom. Many advances have happened over the year in the world of CF, but these warriors are still fighting a never-ending battle with no cure. We are constantly saying goodbye way to early to these precious lives, and I wish we could bring a stop to that!! Please pray for a cure for all those enduring this illness!!
Now with this being said, Matt and I are diligently looking into some option to help Emma and our family as a whole. We are discussing these with her team, and have her doctor's complete support on some potential upcoming decisions. I will explain this is further detail in the near future after we have some final answers. I just ask, in the meantime that you would please partner with us as we pray for clear direction and open doors.
We are so grateful to each of you for your continued love and support. This has been a very long road, but the journey isn't over yet! We are trusting that the Lord has all things in control and has a purpose and plan for it all. We are humbled that He would allow us to be Emma's (and the other girls) parents and that He trust us to endure this life with her.
Friends, remember no matter what you are enduring today to "consider it pure joy when you face trials of many kinds, because you know that the testing of your faith develops perseverance. Let perseverance finish its work so you can be complete and mature, lacking nothing" James 1:2-4. Hang in there... God has a plan for everything!!
We love y'all!!
