My name is Annmarie and my 16 year old daughter was diagnosed in August of 2013 at the age of 14.
After six months of sinus and ear infections that seemed to temporarily respond to antibiotics, my daughter's runny nose and clogged ear wouldn't respond to anything. For several weeks I brought her back and forth from her pediatrician to our ENT. She was on three separate antibiotics and had her blood work done twice. She just kept getting sicker including joint aches and then rashes on her elbows. On a Friday I brought her to our pediatrician who gave us a prescription for a CT scan of her sinuses (he believed she had polyps), and over that weekend she continued to deteriorate until her breathing was labored and the whites of her eyes were red. She was admitted to our hospital on that Monday for what they thought was pneumonia.
On her second day there she was transferred to ICU. On her third day a rheumatologist was finally brought in and told us she was pretty sure it was GPA, but was waiting on confirmation from bloodwork. The following morning my daughter needed to be intubated. The pulmonologist did a bronchoscopy and saw that there was blood, not infection in her lungs. At this point they felt confident enough that it was GPA that they began treating her with steroids. That Saturday she had her first of four infusions of Rituxan.
My daughter's stay in the hospital was a total of 36 days. 26 in ICU and she was intubated for 15 of those. During her time in ICU (while intubated) she developed blood clots and pancreatitis. Both were believed to be from ICU, not GPA.
Anyway, she missed her first day of high school and had to receive home instruction for the first half of her freshman year and then only went to school part-time for the second half of the year. She was getting stronger all the time, but despite physical therapy her shortness of breath was getting worse, instead of improving. Her pulmonologist (and other drs) felt that she was still severely deconditioned after running PFT's and exercise tests etc. I was paralyzed with fear beginning to believe she had ILD.
By the very end of July 2014 she developed stridor and was choking on her own mucous. Her pulmonologist could now hear it in her throat. We went straight to her ENT (different ENT-GPA specialist) and he scheduled surgery.
On August 1st she had her tracheal and bronchial tubes dilated and scar tissue removed. Last month she had the same procedure done and will likely need it again at least one more time. Her doctor (Robert Lebovics) believes that the scar tissue was likely due to a combination of GPA and prolonged intubation. Mostly the latter. He saw no signs of active disease during either surgery. She also had four more infusions of Rituxan this past January after her B-cells started creeping back, and she's receiving IVIG infusions once a month. She is still on 2.5 mg of predisone. She also takes Bactrim 3X a week.
Crazily enough, she's doing pretty well overall. She needs to get back into physical therapy so that she can build her cardio back (she's avoiding it), but she's really strong otherwise.
I, unfortunately, suffer from OCD and can tend to spend hours online finding the worst possible outcomes of GPA. I also, fortunately, have an email pen pal who is a doctor that has GPA and leads an incredibly full life (kids, rock climber, mountain biker, runner, x-country skier and an ER doctor) and he reassures me constantly that this is a very treatable disease that just needs to be watched so that it can be treated in a timely manner.
On top of the GPA, my daughter also deals with her own anxiety disorder and so I work very hard at not passing my anxiety over to her... which frankly I fail at a good part of the time. While I want her to hang out with friends and go away to college when it's time, I also want to cover her in bubble wrap and purell.
Any reassuring words and advice that will allow me to let her enjoy life while staying on top of her appt's, symptoms, etc., would be really appreciated.
I know that we'll always have to deal with this, but I don't want it to define her. I can't let it. Thanks!
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