Wegeners journey

[jojoizzy]

Maybe I can write this now... I am the mother of a 14 year old and her story begins from start of her life. At 4-6 months she constantly had runny nose/cough. By 2 years old, had so many ear infections, bronchitis, croup, that they sent me to have Cystic Fibrosis testing (negative). The explanation now for all symptoms is allegies At 2 yrs, found to have sinus infection. By 3 yrs, found to have low level conductive hearing loss. By 4.5 years, she has had ear tubes( hearing loss recovered), adenoids removed, and sinus surgery that had polyps removed. Over the next 10 years, we will battle infection after infection bronchitis, ear will ooze blood and pus, nosebleeds that look like she's been in a car wreck and hemorrhaging, wheezing but no explanaition why, sinus infections that followed for MONThS and don't respond to antibiotics. By age of 13, she will have had 8 ( yes I said 8!) sinus surgeries! Now doctors will begin to question that she needed these surgeries because that is unheard of. Yes, I agree. The problem is that for the last 3 surgeries I had begun to sense something was not right and taken her to 3 different ENT for separate opinions. They all said she needed surgery. I even began taking her to Infectious Disease who fought the sinus infections for 6-8 months with no progress.
Dec. 29, 2009, she has her 8 sinus surgery. She usually ounces back within days. This time she won't get out of bed 2 weeks later!!! At follow up I ask if she could have MONO because of this crippling fatigue and the fact her throat feels like it is swollen and ahe can't talk right. Dr. aCTUALLY scolded me for being overly concerned and told me it takes awhile for a child to heal after surgery and sleeping is typical for a new teenager!! On way home, my daughter ( who is quiet stoic and has huge pain tolerance). Looked at me with tears and said " I don't care what he said mom i' m bad sick and I need to go to another doctor!". The next day she was diagnosed with High levels of EbV/ atypical lymphocytesand diagnosed with MONO. By March ( just 3 months later CT scan shows she is occurred and mucosal thickening in sinus again!). New ENT recommends a 2 nd opinion and suggest someone in Dallas. I go home and google ENT and find the top ranked place is johns Hopkins. Within a few months we are flying there to see ENT , IFectious Disease, Rheumatology. Don't even know how we ot all these appointments ( sometime I think our destiny is not so random!). Long story short, she has a working diagnosis of limited Wegeners. Pediatric Rheumatologist at JHH, presented case to Bayview and based on clinical evidence feel this diagnosis is correct. My daughter case is still a working diagnosis because she has clinical picture but never ANCA positive and biopsy (showing chronic inflammation) were pulled from last 2 surgeries and whatappeared to be granuloma, but no artery or vessel attached to seal the
deal so to speak. She responded to Methotrexate and we had NO sinus symptoms for 5 months ( first time in her life). She came off meds over summer and by the 6 th week all symptoms beginning again. sinus infection not responding to Augmenting, biaxin, Levaquin, throat hurts, coughing so bad doctors can hear audible wheezing ( but PFTs are always good?). JHH doctor consulted and beginning Methotrexate again even though local doctor doesn't see how she really has Wegeners.
There is so much left out of this story, especially the emotional toll this has taken on mot only my daughter, myself, but our whole family! Many a day I have questioned if I might have Manchasuen?? Thankfully even a doctor told me I could not make up the obvious problems! My daughter looks beautiful and has been a State level swimmer. She gets told she can't be sick or she wouldn't be able to swim at this level. Well fortunately other people are winning because she has been reduced to not be able o swimming at her level because she can't breath. No doctor still can explain shy she is having hard time with breathing while exercising and doesn't respond to inhalers for exercise induced asthma. I know time will complete the picture and I wil now thati have tried everything a mother can do to fight this beast. Some doctors won't believe what they can't see or measure ( and even then some won't believe what is righting front of them because it's not supposed to be there?!)

[elephant]

I am sorry you are going thru this and feel bad for your daughter. At age 15, I was misdiagnosed and my kidneys were failing and they could not find anything. So they guess I had some type of autoimmune diesease, but could not properly diagnose me. I was on prednisone for a long time. During my hospital stay I was given cytoxan, but I don't know how long. I was suppose to take cytoxan as a teenager but stop taking it. It is important to find a good pediactric Rhuematologist for now and then get her to A Rhuemy who specializes in Wegs ( John Hopkins, Cleveland Clinic, Mayo, ...). You are doing all the right things and you appear to be on top of it. Don't give up, there are some Mom's on this forum who will help you too.

[Dryhill]

Dear Jojoizzy,

My heart goes out to you. As a parent I know how terrible it is when your child is ill and there seems so little you can do. The bad news is the concerns of a parent is a life time commitment, my daughters are 29 and 32 and still I worry about them.

Hopefully your daughter will now start on the road to remission.

[vdub]

I don't think I've heard of someone so young having Wegs.... What a sad way to start life. On the good side of things, they can treat wegs pretty well now and, now that you have a diagnosis, maybe things will start looking up for you and your daughter.

I'm glad you were persistent in pursuing the problem. My PCP once told me that it would have taken longer to diag me if I hadn't been so persistent and pushy.... :-)

[KimH]

What can I say to comfort you!?!? I too have a daughter that has been seriously ill for 17yrs. She did not become ill until 13 yr but for the yrs inbetween her illness has affect the entire family. I remember being in the hospital one time and our pastor had come to visit. He ask me how I was. I was confused? I was fine...Liz (my daugher was NOT). Many people do not undertand what it means to the family to have an ill child...at that time I was one of those people. I wanted her to be well..normal.

Her diagnosis was clear. It was lupus. Our Rhuemy sent us and her records to many different places and dr's. There were unexpected things that happen like angio-edema of unknown etiology...meaning her throat swelled up and closed for no apparent reason. It apparetnly was something to do with her compliment. Yet she was intubated in ICU for a week. The stories of her illness and all the complications could fill a book.

I know your nights of worry. The days waiting for test results. The hope that remission willl come and all will be well. There were many different drs we had to deal with after the Rheumy..nephrologists, hemotologists, ENT's, gastroenterologis, neurologists, gynecologist....At times it was a nightmare to get them to talk to each other and each one had to be asked if they had had all the info needed to make the necessary decisions for her next procedure. We had been spoiled that her Rhuemy had acted as her case manager.. When the Lupus went into remission her main care was under nephrology and then the transplant team at hartford hosp.. i had to become the case manager and the most annoying parent ever!

Please know that i am thinking of you! All my best!

[Sangye]

Hi jojoizzy, welcome and thank you for sharing your daughter's story. How very sad to read what she has gone through for her entire life! I breathed a big sigh of relief that you sought care with JHU and that the Vasculitis docs at Bayview were consulted. I encourage you to follow JHU's treatment plan and have them continue to oversee her care. Doctors who are not Wegs specialists think Wegs has a classic look-- sinus, lungs, kidneys. Since her case doesn't look like that they can't believe it's Wegs. Too bad. They don't get a vote! This child has already been put through the diagnosis wringer. You will likely have to educate them as you go, but I suggest you don't stay with any doctor who thinks they know better than the experts, or who won't allow the experts to provide the treatment plan.

Regarding her difficulty breathing, there may be many factors. I've had extensive and repeated lung involvement and tons of lung clots, but my PFTs look good anyway. Yet I have difficulty breathing and no one knows why. It is common with Wegs. Some of the meds cause lung irritation as a side effect. Isn't that ironic? I do believe that your daughter will find a way to swim competitively again. It might just take more time.

BTW, my therapist told me if you're wondering if you're crazy you definitely aren't. I bet the same thing applies for Munchausen's. :wink1:

[jojoizzy]

Yeah that is what my therapist said too.. Truly crazy people never question their sanity. Whew what a relief!
I have some questions about lung issues. Is this normal to have trouble breathing but normal PFTs? She has desaturated twice on exercise stress tests most recently to 79%. Also found to have hyperinflated lungs when put in body box. I believe her RVC was like 214% which demostates some air trapping. She states her chest tightens up and while competing her legs go numb if she pushes herself.

[Al]

Dunno from normal, but I can tell you that my lung tests have always been pretty good. Heck, I had the pulmo's assistant, the one hired to walk me up and down several flights of stairs, severely winded, and he was a young guy. On the other hand, this was the case even in the worst of the hemoptysis. So the tests don't tell us everything.

You have taken on quite a job. But, as any parent will tell you, one that we would not think of not doing, to the best of our abilities. Thing is, though, it can be utterly draining, especially when you have to re-discover fire at every turn. When you need a little help in that regard, please lean on us....

Al

[elephant]

I have air trapping, part of my lung are like a 80 year old...this was confirmed from my lung function test. My pulmonologist said it was from my Wegeners disease.

[jojoizzy]

Can anyone explain how air trapping is related to Wegeners? I stay so confused...

[Pmarsh]

It sounds like your daughter has similar breathing and exercise issues to me. I was a competitive swimmer growing up and throughout university. More recently I have turned to competing in triathlons and bike races. I was diagnosed with Wegeners almost exactly a year ago - mainly with chronic sinus issues and acute lung inflammation and bleeding. Over the past year I have tried to get back into shape and keep up with regular exercise. In May I had a flare and they found granulomas (lesion) in my right lung. My lung capacity is certainly not what it used to be. I use inhalers when I exercise but find they don't help a great deal. I'm still convinced it's a matter of getting the Wegs more under control and my lungs will improve. I practice some breathing exercises each day that might help. Just some deep breathing and using a respirometer from time to time.

Hopefully your daughter can get back in the pool and to top form soon. All the best...

[jojoizzy]

Sangye,
Could you explain what you know PFTs being normal range, but TLV/ RVC being elevated (216% showing hyperinflation/ air trapping)? I still don't understand why or how lungs can be affected but never have the PFT show abnormalities despite obvious wheezing, short of breath, etc

[Sangye]

I'm sorry, I don't know enough about PFTs to explain that-- a pulmonologist should be able to.

[Trudy]

I still don't understand why or how lungs can be affected but never have the PFT show abnormalities despite obvious wheezing, short of breath, etc

The PFTs are alot of different tests that they use as many equations to determine results as were in our high school algebra books. I have had asthma since childhood and my PFT can be normal. The TLV is Total Lung Volume and the RVC is Relaxed Vital Capacity (all the air expelled from the breath you took in). In a healthy lung 1/3 of the air stays in the lung when you breath out the other 2/3. In a diseased lung this changes. In a healthy person if you were to take a deep breath and hold it, your lung would be hyperinflated; so in a diseased lung of diminished capacity (or from pressure from inflamed tissue outside the lung) the same result could occur. The result is shortness of breath(SOB).

I have found that for a few hours (max 4 hours for me) after taking Prednisone that I am not SOB. I imagine this could alter a PFT result. I know medication can alter my blood test results. I was taking my weekly Vitamin D the day before my blood test and it looked OK for 2 months. The next month I decided to take it after the test and my score was below normal. What I am trying to say is examine when meds are taken in relationship to the testing.

Hope this did not confuse you even more!