This update is a little overdue… but here we go!
Leah is doing much better since she was discharged from the hospital early November. However, the question remains: What is the cause of the high fevers she was having???
Currently, Leah is still on daily steroids to keep the fever at bay. When we tried to wean her off the steroids after the hospital, the fever came back.
She has gone through a series of appointments and testing to get to the root cause.
Leah is doing much better since she was discharged from the hospital early November. However, the question remains: What is the cause of the high fevers she was having???
Currently, Leah is still on daily steroids to keep the fever at bay. When we tried to wean her off the steroids after the hospital, the fever came back.
She has gone through a series of appointments and testing to get to the root cause.
- ABPA - testing came back negative. That is good, but doesn’t get us closer to the cause.
- Rheumatology - Her rheumatology blood work looked good and after examining Leah, the rheumatologist has very low suspicion for rheumatology disease. However, the rheumatologist would like to reevaluate Leah if the fever relapses after decreasing the steroid.
- NTM - This is a possibility, but NTM does not normally present with high fevers.
A Positive Note: New CF Drug becomes available - TRIKAFTA!
On December 8, 2019, Leah started Trikafta. Some of you may be aware of the waves this most recent CF medication has made in the news. This has been the most promising drug released so far. Trikafta is a CFTR modulator, which means it works by helping to correct the gene defect that causes CF. This is the third modulator medication Leah has been on, and by far, the one that we have noticed the most response for Leah. Leah’s PFT went up 10% since she started on the medicine and is the highest it has been since last August!
We want to thank you, our community, who has supported us over the years to help fund the research and development of these medications that are making a difference in the CF community! THANK YOU!!!!
Leah's Front Line is registered for the 2020 Great Strides walk! You can register today and join us!
We want to thank you, our community, who has supported us over the years to help fund the research and development of these medications that are making a difference in the CF community! THANK YOU!!!!
Leah's Front Line is registered for the 2020 Great Strides walk! You can register today and join us!
Denver Consultation with NTM experts
Back to figuring out the fevers… with the decline Leah had in her health as she was fighting these fevers, it became clear that it was time to address the possibility of Leah’s NTM being the main culprit of these fevers. So, doctor referred Leah to do a consultation with leading NTM experts in Denver, CO.
This past week, we headed out to Colorado. The appointments went really well. We believe having both Dr. Martiniano and Dr. Daley involved in Leah's care will be of tremendous benefit as we move forward in monitoring and treating Leah's NTM. Their level of expertise and experience in treating patients with NTM is reassuring that we will be good hands. It was really good to hear from them some of the medications and treatments that are in the pipeline and being investigated. It gives us hope for more effective treatment when the time comes to start treating again.
They both said the fevers could possibly be from the NTM.
Talking with both of them, we have identified symptoms that would indicate the need to restart NTM treatment as well as discussed in general what that treatment would look like.
This past week, we headed out to Colorado. The appointments went really well. We believe having both Dr. Martiniano and Dr. Daley involved in Leah's care will be of tremendous benefit as we move forward in monitoring and treating Leah's NTM. Their level of expertise and experience in treating patients with NTM is reassuring that we will be good hands. It was really good to hear from them some of the medications and treatments that are in the pipeline and being investigated. It gives us hope for more effective treatment when the time comes to start treating again.
They both said the fevers could possibly be from the NTM.
Talking with both of them, we have identified symptoms that would indicate the need to restart NTM treatment as well as discussed in general what that treatment would look like.
Plan Moving Forward
- Gradually decrease steroids - All doctors are in agreement that we need to get Leah off the steroids. Doctor started Leah on high dose ibuprofen to help her body adjust as we wean Leah off the steroids. We are checking her ibuprofen level, and then we will come up with a plan to very gradually decrease Leah off the steroid.
- Consider eradication of MRSA - On the way Denver, we found out that Leah cultured MRSA for the first time. This was disappointing as MRSA is another CF pathogen that is difficult to get rid of and causes further lung disease. If it is still present after we reculture her sputum, we will most likely treat this aggressively and seek to eradicate it from Leah’s body.
- If she continues to do well/be stable on Trikafta and we can get her off steroids, we probably have some time to decide about restarting NTM treatment.
Follow the Yellow Brick Road
On a personal note, we have musicals in the air at our house. Leah is a munchkin in the Wizard of OZ musical at her school. Hannah is a big kid and a salsa judge in the Matilda musical at her school. Both girls are super excited to be part of these productions. If you are looking for some fun, quality family entertainment or a date night with your special someone, the Wizard of OZ will run March 5-8 and Matilda will run March 13-15. Love to see you there!
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