mom of a daughter with WG

[blessed2bamommy]

Hi. My name is Kim. My 12 year old daughter was recently (Feb 2012) diagnosed with Wegener's. We live near Nashville so she's been to Vanderbilt Children's for her treatments. She was hospitalized most of the month of Feb and into March.

Thankfully we got a diagnosis pretty quickly. Our pediatrician thought she had JRA and sent us to a rheumatologist at VCH. Within 5 min he knew it wasn't JRA and suspected Vasculitis. That was on Friday, Feb 3. Over the weekend Bethany continued to get worse and was admitted to VCH. This stay was 9 days long and that's when we got the WG diagnosis. She was discharged from hospital on 2/13. On 2/16 I delivered our 5th child via c-section (breech baby). On 2/19 baby & I came home from hospital. On 2/20, Bethany was readmitted to hospital. This was a 3 day stay. Then on 2/24 she was admitted again. This was a 2 week stay. During this stay she had seizures and ended up in PICU and had ICU psychosis which meant major hallucinations etc... They say she also has PRES.

She's currently on 40mg of Prednisone (down from 50). While in hospital they did the three pulse of Pred. Those were 1,000 each. She's currently going in once a week for Cytoxan. She has finished her Rituximab. She's also on Bactrum, anti seizure meds, anti hallucination meds, calcium, iron -- seems like there is more but that's all my brain can come up with at this time. :-)

I'm glad to have found this forum for support and encouragement.

[TOBEY32]

There are some younger people that are part of this Forum - I'm sure you will hear from them.

David

[Al]

Hi Kim. It is always heartbreaking when someone as young as Bethany has these issues to face. This forum is indeed a haven of support, and, we hope, useful answers. You have a lot on your mind right now, clearly.

Of course, I cannot comment on the seizures, but I can observe that PRES is often drug induced. In Bethany's case, I would look at the cytoxan warily. In fact, I would ask the rheumy to consult with a pediatric vasculitis specialist. There are a some listed on the Vascultitis website. These doctors are available to consult with local doctors for free. The consensus these days seems to be that Cytoxan is no longer the drug of choice in most cases (particularly, I think, this would be true with young females). Rituxan (rituximab) works as well without some of the really harsh side effects, and it is highly unusual to follow it up with cytoxan.

10,000 milligrams of pred or solemedrol, if that is what it was, is a huge dosage, even for a large adult male. I am wondering that, assuming you have this straight, if that isn't enough to olt her brain out of normal tolerance. (Headaches and convulsions are, in fact, listed as possible adverse reactions.) In all cases, it is important to get the facts. Central nervous system vasculitis is rare but really nasty. If that were the case, you would need the best specialist you could find.

Please do seek a specialist, and let us know how Bethany is dealing with all this.

Al

[annekat]

Welcome, Kim, and I'm sorry your daughter's illness forced you to come here, but you came to the best resource on the web for Wegener's patients. Everyone here is very open and glad to hear your questions and experiences. There are those who know more than I who will have a lot to say. It is upsetting to hear of someone Bethany's age having to deal with this and having a hard time, perhaps with doctors who are inexperienced in the disease. So do follow Al's advice and check the Vasculitis Foundation website for true WG specialists you might be able to access. We will be anxious to hear how Bethany progresses in her treatment, and do share any stories, concerns, and frustrations along with any good news.

[blessed2bamommy]

While Bethany's doctors aren't listed as Vasculitis specialists, one of her main doctors worked at Boston Children's for 10 years and is very familiar with Wegener's.

The seizures may have been med induced but the high doses of steroids were given on our first 9 day hospital stay. The seizures didn't happen until 2/29. My husband was the one staying with her when that happened and he feels it was a combo of the meds and high blood pressure. She's also on blood pressure meds -- I think I forgot to mention that in the intro. She wasn't on BP meds when the seizures happened and my husband feels that had she been the seizures wouldn't have happened. It's hard to know for certain.

As you can imagine it's been a very difficult road. Dealing with WG alone is hard enough but having just had a baby has made it all even more difficult.

Bethany loves to draw but her hand tremors make that very difficult and frustrating right now.

[Al]

Bethany has high blood pressure at age 12? You didn't mention any kidney involvement, but that could be a reason for the hypertension.

I still recommend a WG specialist be involved, and to be asked about the cytoxan. Considering your daughter's age, that worries me.

Bethany may have to work on an alternative style of drawing until the pred dosage is tapered way down. Most of us know those tremors...

Al

[blessed2bamommy]

Al,
Yes, she does have kidney involvement. That has been the organ the most affected. Her creatine levels are finally coming down.

Her lungs look great. She's had the joint pain and swelling thus why the ped initially thought JRA.

Back in Dec of 2008 she got a double ear infection and sinus infection. She had never had an ear infection prior to this. She ended up getting tubes in and adenoids removed. She has continued with ear and sinus issues since then. The ENT never suspected or mentioned WG.

Now for the first time since all of that started she can breathe through her nose and her sinuses are feeling better. We are switching ENTs over to one at VCH.

[annekat]

The double ear infection and sinus infection, followed by a couple of years of recurring sinus and ear problems, is exactly what happened to me back in Oct. of 2008. I had never had an ear infection, either, and I also had the joint pain which seemed like RA. I was 56 years old when it all began, instead of 12. Same thing with the ENT not mentioning or suspecting WG until things got really bad. Interesting that Bethany and I are on a similar timeline, though I was diagnosed almost a year earlier. I'm doing pretty well. But I haven't had the kidney involvement or seizures and didn't have to spend any real time in the hospital. I had lung involvement which cleared up within a few months. The sinus and ear stuff is taking longer but is better, though there is some permanent damage. It seems early in Bethany's treatment to expect a whole lot of improvement very soon, but it's encouraging to hear her sinuses are better and her creatinine levels are coming down. Congrats on the new baby, and I can understand the difficulty of dealing with both.

[Al]

Great about the lungs, Kim! But the kidney business is serious. Creatinine coming down is excellent news, but Bethany needs all the reserves she can get. If I were you, I would request a consultation at UNC, which is not that far from you and has one of the best nephrology;vasculitis programs on the planet.

Al

[drz]

Great about the lungs, Kim! But the kidney business is serious. Creatinine coming down is excellent news, but Bethany needs all the reserves she can get. If I were you, I would request a consultation at UNC, which is not that far from you and has one of the best nephrology;vasculitis programs on the planet.

Al

Doctor Ronald Falk in nephrology department contact info:
FALK, RONALD J., M.D.
DJ Thurston Distinguished Professor
Director of the UNC Kidney Center
Chief of Nephrology and Hypertension
University of North Carolina
125 MacNider Building, CB# 7005 UNC-CH
Chapel Hill, North Carolina 27599
Phone: 919-966-4615
Fax: 919-966-5775
UNC Kidney Center (http://www.unckidneycenter.org)

[BrianR]

You are getting good advice from Al BTBAMommy (Kim?). This is a terrible thing for your daughter, you and your family to be faced with. I do not need to tell you that Wegener's is a serious disease that you need to stay on top of and become very involved in the treatment. This I am sure you already know.

When my wife was diagnosed with WG she, like most others, was very, very ill. Not as bad as some but very ill nonetheless. I tell you, I was a complete wreck. For whatever it's worth, it does and will get better. This forum is a wonderful place to be for support AND information.

Have faith that things will get much better (it will!) and stay very involved in your daughter's treatment. Please get a WG specialist - you will need him/her for a long time until (not if) they come up with the cure.

If so inclined, please visit www.weggiesunite.blogspot.com (http://www.weggiesunite.blogspot.com)

Peace.

[annekat]

I remember a young girl, I think her name is Holly, around 14, maybe, whose parents were on the forum awhile back. She had Wegs with kidney involvement and was doing pretty well, if I remember right. I wonder how she is doing.

[Palmyra]

Hi Kim,

I too, am the parent of a 'child' with Wegener's, although my child is now 26!! She was first diagnosed w/ Crohn's when she was 14, then Wegener's at the age of 20. We live in Texas, but I took her to the Mayo Clinic, Rochester, to see a Doc there that only treats vasculitis.

Several members have recommended that you take your daughter to one of the major Vasculitis centers, and I could not agree more.

Even if it is for one initial visit, these specialists see nothing but vasculitis and have so much experience with this rare condition and are on the cutting edge regarding autoimmune science. I wish you and your family all the best during this stressful time.

I hope you have future time to contact the Vasculitis Foundation as well. They have been of great help to my family:
http://www.vasculitisfoundation.org/

You have found this group, and I think it is second to none regarding support.

Best Wishes,
Jane, mom of Alison

[Dryhill]

Blessed2bamommy, welcome to the forum as you have already discovered this is a good place to get advice. As a parent I do not envy you, having a child with such a nasty disease and giving birth all at the same time, I know you ladies like to prove you can multi-task but I do think this is taking things a bit too far. :confused1: I hope you and your baby are doing well and that your elder daughter gets the treatmentr that she clearly needs.

Jim

[blessed2bamommy]

Blessed2bamommy, welcome to the forum as you have already discovered this is a good place to get advice. As a parent I do not envy you, having a child with such a nasty disease and giving birth all at the same time, I know you ladies like to prove you can multi-task but I do think this is taking things a bit too far. :confused1: I hope you and your baby are doing well and that your elder daughter gets the treatmentr that she clearly needs.

Jim

Jim,
I agree that this is taking multi tasking too far. You made me laugh at that. This is definitely not what we had in mind.

[jeriorleans]

My son, A.J. was diagnosed in September, 2009 at age 13. He is now 15 and was doing very well until recently. A very large chlosteotoma was discovered in his right ear that extended through the middle ear into his sinuses and all the way to the base of his skull. All of the bones in his ear had to be removed. He is still recovering and it is unknown at this time how much hearing he will have. He has been wearing hearing aid since his diagnosis as the Wegener's attacked his ears and nose. he is currently on Imuran and his rheumy just raised his dosage to hopefully prevent this from occurring in his left ear.

This disease is very hard on teenagers. Kids tease about everything from the acne the steriods cause to having hearing aids to the weight gain.

[annekat]

My son, A.J. was diagnosed in September, 2009 at age 13. He is now 15 and was doing very well until recently. A very large chlosteotoma was discovered in his right ear that extended through the middle ear into his sinuses and all the way to the base of his skull. All of the bones in his ear had to be removed. He is still recovering and it is unknown at this time how much hearing he will have. He has been wearing hearing aid since his diagnosis as the Wegener's attacked his ears and nose. he is currently on Imuran and his rheumy just raised his dosage to hopefully prevent this from occurring in his left ear.

This disease is very hard on teenagers. Kids tease about everything from the acne the steriods cause to having hearing aids to the weight gain. Jeri, my heart goes out to your son for all he's been through. I remember the pain of being teased in school, but I didn't have Wegener's. Now, I have hearing aids, and it is not a big deal, but I can imagine how it would have been having them as a teen. This new development sounds very scary, and is more than most of us with ear involvement have to endure. I can only hope that he is young enough to see a cure for Wegener's and other autoimmune disorders in his lifetime.

[NicShaf]

I'm glad you've found this site for support and information. All our best to you and your daughter.

[Sangye]

Welcome to the group, blessed2beamommy. I can't imagine how you're managing all this! I second what others have said about getting a Wegs specialist involved. It's hard to overstate how important that is when treating a highly variable, incredibly sneaky disease like Wegs. And she already has a complicated case. Get the very best doctors involved.

I'm sure your daughter must have been on 1,000 mg solumedrol (not 10,000) as that's the highest dose. They typically give it 3 days in a row. Truly horrible--I started on that dose and have great sympathy for anyone else who has to have it.

My Wegs doc at Johns Hopkins (Dr Seo) has told me that they have to use higher doses of pred in kids. Apparently Wegs is more tenacious in young people. Another reason to get an expert involved.

[Sangye]

Jeri, I'm so sorry to hear about your son's new complication. How awful! :sad:

[blessed2bamommy]

Welcome to the group, blessed2beamommy. I can't imagine how you're managing all this! I second what others have said about getting a Wegs specialist involved. It's hard to overstate how important that is when treating a highly variable, incredibly sneaky disease like Wegs. And she already has a complicated case. Get the very best doctors involved.

I'm sure your daughter must have been on 1,000 mg solumedrol (not 10,000) as that's the highest dose. They typically give it 3 days in a row. Truly horrible--I started on that dose and have great sympathy for anyone else who has to have it.

My Wegs doc at Johns Hopkins (Dr Seo) has told me that they have to use higher doses of pred in kids. Apparently Wegs is more tenacious in young people. Another reason to get an expert involved.

I'm sure you are correct. I went back and edited my post to reflect 1,000 not 10,000. :-)

She's at VCH right now for another dose of Cytoxan. My husband is going to talk to doctor about consultant a specialist.

[blessed2bamommy]

My son, A.J. was diagnosed in September, 2009 at age 13. He is now 15 and was doing very well until recently. A very large chlosteotoma was discovered in his right ear that extended through the middle ear into his sinuses and all the way to the base of his skull. All of the bones in his ear had to be removed. He is still recovering and it is unknown at this time how much hearing he will have. He has been wearing hearing aid since his diagnosis as the Wegener's attacked his ears and nose. he is currently on Imuran and his rheumy just raised his dosage to hopefully prevent this from occurring in his left ear.

This disease is very hard on teenagers. Kids tease about everything from the acne the steriods cause to having hearing aids to the weight gain.

I'm so sorry for all your son is going through. Thank you for introducing yourself.

[pberggren1]

I'm so sorry AJ has to go through this. I was totally deaf for a year so I can kinda relate. The pain must be crazy too. I was deaf for a year but now have cochlear implants. This may be an option for AJ depending on his status.

[watersedge]

Hello Kim, sorry to read about your daughter, she is so young to have to face all this, it is so unfair on her. I hope things get better for her with the right treatment and doctors. Congratulations on your new baby, you have a lot going on right now and hope you are managing to get some rest yourself after giving birth, getting up for night feeds and with all the worry you must have for your daughter at this time.

[Sangye]

I'm sure you are correct. I went back and edited my post to reflect 1,000 not 10,000. :-)

She's at VCH right now for another dose of Cytoxan. My husband is going to talk to doctor about consultant a specialist.
That's great! You won't regret it.

I just commented about the 1,000 vs 10,000 because other readers might think they're being undertreated!

[Natalie]

Hi there sending some love to you and your daughter . tough times mate All the best and please keep in contact of your progress xx

[Al]

That's great! You won't regret it.

I just commented about the 1,000 vs 10,000 because other readers might think they're being undertreated! Let's see....10,000 translates into early 3 gallons, so, yes, it apeared to be a typo....

Let us know if you find a specilist. I have a hunch that most specialists would recommend RTX, rather than cytoxan, especially for young women.

Al

[Jaha]

Kim,

Welcome to the forum. I'm so sorry to read about your daughter, getting dx at such a young age. This is a great place for any info you need. I wish your daughter and family all the best with her tx's and care.
Jana

[Psyborg]

Welcome to the forum, my daughter is 12 as well so I can only imagine what you are going through. I hope you find plenty of answers to your questions.

[HopeinTN]

Hello Kim. Thanks for posting your story. I sent you a private message/messages about being a fellow Vandy patient. I hope you, your daughter and your family are doing well. Good luck and you are in the right place for sure.

Katrina- Nashville, TN