“When your child has rare diseases, you don’t just need doctors — you need fighters, believers, and people who never stop looking for answers.”
If you’ve followed our journey for a while, you’ve probably seen glimpses of my daughter Jentri’s complicated medical life. But one thing I get asked often is:
Why does she have two different medical teams?
Let me take you into our world for a moment — and show you why that’s one of the most important decisions we’ve made for her care.
💜 Her PCD Team — Fighting for Answers from the Start
Jentri has been seeing Dr. Maria Rivera-Sanchez, a pulmonologist at Children’s Medical Center Dallas and UT Southwestern, for over six years. She is deeply involved in the Primary Ciliary Dyskinesia (PCD) space and helped get her clinic officially accredited by the PCD Foundation.
Even before we had genetic confirmation, Dr. Rivera-Sanchez believed in Jentri. She advocated for more answers, pushed for additional testing, and even submitted her case to a medical conference because she was so complex. She’s the kind of doctor every rare-disease kid deserves — one who doesn’t give up.
💙 Her CF Team — Understanding the Overlap
In 2023, after years of inconclusive testing, Jentri’s genetic panel was rerun — and this time, it revealed a rare CFTR mutation called RQ75. This mutation leads to thick, sticky mucus — which compounds the issues already caused by her PCD, since her cilia can’t move mucus out of her lungs properly.
That’s when we were referred to Dr. Preeti Sharma, Head of the Cystic Fibrosis Clinic at Children’s Dallas and UT Southwestern. She’s everything you’d hope for in a specialist: kind, knowledgeable, validating, and endlessly supportive. She helped us understand Jentri’s “atypical CF” diagnosis and started her on Trikafta, a life-changing CFTR modulator therapy that she only qualifies for because of that rare mutation.
🔄 Why She Sees Both
Jentri alternates between her PCD and CF teams every three months. Both teams monitor her lungs — but through different lenses. And for a child living with two rare lung diseases that intertwine, both perspectives are absolutely essential.
CF causes her mucus to be thicker than normal.
PCD prevents her body from clearing it out.
Together, this combination puts her at high risk for serious infections and lung damage.
These teams work together, not separately — and that makes all the difference.
💊 Her Daily Medications & Treatment Regimen
Managing Jentri’s care is a full-time job, even when she’s well. This is her baseline routine — what she takes daily to stay stable:
Medications:
- Trikafta (CFTR modulator)
- Xopenex (bronchodilator)
- Hypertonic Saline 7% (to thin mucus)
- Pulmozyme (breaks down sticky mucus)
- Flonase (nasal steroid)
- Symbicort (maintenance inhaler)
- Azithromycin – 3x/week (prophylactic antibiotic, 8 years running)
- Linzess (GI motility)
- Zoloft and Hydroxyzine (mental health support)
- Cetirizine (antihistamine)
Airway Clearance:
- Monarch Vest System – a wearable high-frequency chest wall oscillation device that shakes her lungs to loosen mucus. It’s portable and life-changing.
When she’s sick, everything changes.
Her regimen increases to:
- Treatments every 4 hours
- Close monitoring of oxygen levels, lung sounds, and fevers
- Rapid escalation if things worsen — including hospitalization when necessary
This is why consistency, quick access to her team, and proactive care matter so deeply.
📚 A Glimpse Into Her Medical History
Jentri has been through more in 13 years than most do in a lifetime. Here’s a quick summary:
- 25+ hospitalizations across 4 different States
- 5+ bronchoscopies
- 4+ PICU admissions
- 1 ventilator event at age 5 — the worst 96 hours of our lives
- Too many IV’s and PICC Lines to count
- 5+ CT scans, 3 sweat chloride tests, countless X-rays and thousands of labs
- Followed by: Genetics, Cardiology, Immunology, Infectious Disease, Allergy, Ophthalmology, ENT, Audiology, GI, and Psychiatry
✈️ Why We Fly for Her Appointments
A lot of people ask why we fly to Dallas instead of seeing new doctors in each new city we travel to for my husband’s job.
Here’s why: continuity of care matters.
We live full-time in a fifth-wheel RV, but Jentri’s care team stays the same. Changing doctors every few months would mean re-explaining her rare diagnoses, redoing tests, and losing the trust and deep understanding we’ve built with her current teams.
We’re incredibly grateful to Children’s Flight of Hope, who covers round-trip airfare for Jentri and one adult for every long-distance appointment. They’ve flown us back and forth since she joined the CF clinic, and we honestly don’t know what we’d do without their help.
📣 How You Can Help Families Like Ours
If you’ve made it this far — thank you. You’re part of Jentri’s village now. Here’s how you can support families like ours:
💜 Support Research & Awareness
✈️ Help Kids Get to Care
📢 Share Our Story
Raising awareness helps with early diagnosis, funding for research, and more support for rare disease families. If Jentri’s story moved you — share it.
Having two teams doesn’t mean she’s sicker. It means she’s stronger.
She has people in her corner who understand the puzzle that is her lungs — and they’re not giving up.
Neither are we.
Thanks for being here. 💛
-Kursti, Founder of Breath in Bloom Collective
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