New and can only handle encouraging words.

[annmarie]

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!

[Mas78]

Hi there annmarie
your friend is correct that Wegener's / GPA is treatable. I never got to take Rituxan, it wasn't around so I got Cytoxan. It took me a few years before I was off all my meds for Wegener's. but I have been in remission for 12ish years now.
it sounds like your daughter now has a good medical team in place now and that's a great deal of the battle.

here is a link to my story I put on here last fall, http://www.wegeners-granulomatosis.c...r-s-story.html
I was 22 when I got Dx'd perhaps something in there will give you even more confidence that your daughter is going to continue getting better.

I am sure that there will be plenty other members chime in to give you encouragement also.

[Pete]

This too shall pass. Your daughter is in great hands with Dr Lebovics. Many of us on here have had experiences similar to your daughter's. Many of us have gotten back to a "new normal" that allows a pretty high quality of life.

Encourage your daughter to get some exercise. She should start slowly and increase her activity gradually. This will give her the strength she needs to cope with our dumb disease.

Best to you!

[whatthewhat]

Hi annmarie,

I am glad you found this forum. My daughter was dxd at age 14 in March of 2013. I find it helpful to read posts here as they cover such a wide array of topics. I also find it helpful to talk with other moms of Weggies (on Facebook) as they really get specifically what my emotions are in any given situation.

And just as a side note, I personally believe there is a link genetically between some anxiety-related disorders and WG (just anecdotally noted.) and addressing the one can alleviate the other to some extent.

Feel free to private message me anytime.
Whatthewhat

[mishb]

Hi Annmarie and welcome to the forum.

What a time your daughter has been through.

Just as Whatthewhat is on here for her daughter, you will find many parents of young kids or teens that have been given a diagnosis of WG.
I think the younger ones tend to bounce back much better than we oldies.

Try not to stress about it and try to help her not to stress either. We have all found, that stress makes things worse.

[annmarie]

Thanks to all of you; your responses are exactly what I need! Mas78, I read your story and it gives me enormous hope that my daughter will continue to do well overall and live with a pretty high quality of life, as Pete said, in her "new normal."

I agree with you, Whatthewhat, about the stress. Dr. Mark Speira at HSS did a study about it a few years back. I'm sure you've seen it. The beginning of 2013 was a very stressful time for us and is right when my daughter began with her sinus infections. I've believed for a while now that the two things weren't coincidental. Ironic that your daughter was diagnosed at the same age in the same year as mine.

One question I have for anyone who may have experienced this: my daughter clears her throat fairly frequently. I've spoken to the drs. about this... over and over again. She had been on enalapril for over a year and I read that throat clearing can be a side effect of ACE inhibitors. In January her nephrologist took her off the enalapril, but she still has the throat clearing, though not nearly as frequent. Dr. Lebovics thinks it's nothing more than a habit she developed and it makes some sense because if I call attention to it, she does it more. Just wondering if anyone else had any similar experience.

Also, during cold/flu season, how do you all go about your business? I don't want to turn my daughter into a germaphobe, but I'm having trouble stopping myself.

Thank you!!!
Annmarie

[Dirty Don]

Continue to worry Annmarie, but in a good way. I had a very similar, despite age difference, experience in that I have anxiety & WG. The docs have noted the rise in my anxiety occurrences with the onset of the WG...and, the lessening/disappearance of the anxiety attacks with WG treatment. Now, they have no direct link, but I agree with you in that the anxiety may have been part of a 'warning system' in my body/head telling me I was becoming very ill. Sounds good, but no proof of it other than anecdotal. Anyway, after nearly 4 years, I am in medical remission and within an inch of getting off the last of my WG drugs (taking another shot at it in 2 weeks!!). Your daughter is young, and sounds responsive to her situation (many teens aren't right away). She will get thru this and move on to a better life...youth helps along with adult care and strength. Best to you both, as Pete noted, your daughter is in good hands.

[MaxD]

AnnMarie, Between Drs Spiera and Lebovics your daughter is not only getting the best care available, but being looked after by wonderful, caring human beings. I know, because I see both of them too. I'm sorry your daughter has to deal with more than just this awful disease. As others have said, it's serious, but treatable and manageable and I'm sure both docs have stressed the importance of regular checkups. Your daughter is young and will bounce back quickly - but let her know it takes time recovering from the steroid withdrawal so not to be impatient - I know, that can be impossible for a teenager.

So this was my first cold/flu season after treatment last year and initially I was nervous. But the docs said not to let that stop me from normal life. I did come down with the flu, and although it hid me hard, my sinuses stood up much much better than in the past - I used to end up with a sinus infection every time I had a cold. This time, no prolonged issues with the sinuses! I'll take that as an unexpected bonus!

As for a parents fears, let them come and let them go. Time will help moderate those worries.

Good luck to both of you!

[annekat]

Welcome to the forum, Annmarie. I agree with all the encouraging statements made above. This forum has made a huge difference in my feeling as comfortable as possible with my diagnosis, that it is not the end of the world and there is still a lot living yet to be done. My experience was similar to your daughter's although I was 58 when dx'd and have not taken RTX, was started on Cytoxan, which worked well. You should probably avoid spending too much time on internet sites that dwell on the most negative statistics and most extreme symptoms of WG/GPA. Instead, keep reading and participating in this forum and get on some Facebook groups such as the ones for parents that whatthewhat mentioned above. That way, you'll be getting your info from real people who deal with WG/GPA every day. I understand your anxiety and hope some of it can be relieved here. I don't know what I'd have done without this forum..

[annmarie]

I feel so much better already! I was advised by my daughter's doctors and by my doctor/friend with GPA to stay off the internet, but sometimes I can't help myself. One really good point my GPA-DR-friend made was this: "About 50% of what you read on the internet is correct and good luck figuring out which 50%." He also pointed out that medical studies, much like anything else in publishing, don't get published if they're filled with boring and normal results. So that helps me stay calm while reading upsetting things.

At MaxD: my daughter sees Dr. Lebovics, but not Dr. Speira, though I've considered going to him just to discuss my daughter. I was referring to a study he did about stress and GPA. Our/her rheumatologist is Dr. Simona Nativ. She's a pediatric rheumatologist and is the one who diagnosed my daughter. She did her fellowship at Columbia Presbyterian in NYC and worked with a lot of kids with GPA. Her father (also a Dr.) has been friends with Dr. Lebovics for many years; since they worked together at the NIH. Dr. Nativ has been wonderful. She consults with Dr. Lebovics and with her partner as well as with the drs at Columbia. She sends some of my daughter's lab work to the Mayo Clinic and keeps her under the care of herself, Dr. Lebovics, a pulmonologist, a nephrologist and a opthamologist.

As far as the flu is concerned we kept our daughter out of school for 6 weeks because her Rituxan infusion was smack in the middle of flu season and our schools were hit pretty hard AND the flu shot wasn't very effective for anyone! Hopefully we'll get her medication scheduled in a way that we won't be doing that again!

Anyway, thank you all for your supportive words! I can't believe I waited this long to post!