many time viewer - first time poster

[Daggar]

Hello Everyone,

I have been reading a lot of your posts over the past few months since my 14-year-old daughter Holly was diagnosed with WG (nov 2010). I just want to thank you all for helping me ask the "right" questions and make the "right" decisions with her treatment from those early days. There is a wealth of valuable information here and I've used it numerous times.

We are thankful, and lucky, that Holly has received extremely good care from her medical team at the Alberta Children's Hospital since the time of diagnosis to present. Our medical system in Canada is overburdened, to put it mildly, and our medical professionals have their hands full in dealing with the "crunch" so it makes us all the more grateful for their care.

To lessen their burden I'm being proactive in gathering as much information as possible through websites, and folks like you, so that I become more of an asset with her illness. I'm hoping to gain more knowledge from you and maybe even share a piece of information from our experience that may be helpful in return.


Weggie Dad

[Thakator]

Welcome, Weggie Dad - - and kudos to you for taking an active role in your daughter's new journey. Our hearts go out to you as do our hopes for only the best regarding her treatment and road to remission. Ron

[pberggren1]

Welcome to the Forum Daggar. I too am from Canada, Swift Current, SK, about 5 hours down the road. I hope Holly is doing well. I know it is very hard for a parent to see your child get ill like that and to not know what to do. It sounds like she has good docs.

[elephant]

Welcome and ditto to what Thakator said. Love to hear your daughters story!

[Daggar]

Wow, I didn't expect such a quick response!

HOLLY'S STORY -- I believe Holly's story began at Christmas in 2009 when she had a cold and her sinuses took a while to clear up. Our FP gave her some antibiotic nasal drops which seemed to do the trick and nothing further came of it. Along came spring/summer and Holly started to develop swelling in one of her ankles which we wrongfully assumed was due to wearing the flat-soled boots that were popular amongst many of the girls at that time. She also had a tendency of not tying her sneakers up throughout the years and walking on the heel area which caused the foot to collapse in. Our FP checked her over and agreed that this was probably the cause and signed her up for some physiotherapy to try and strengthen the tendons/ligaments. She went through a month of that and it seemed to be doing okay until the Fall when the pain in her joints worsened and she started to experience unusual fatigue. Our FP was at a loss and we weren't getting the answers needed so we took her to emergency at the local Children's Hospital where she was admitted and the real story began. Her kidneys were failing and the the Nephrologist ordered a kidney biopsy to confirm his findings from the blood/urine tests. Everything moved at warp speed from there with the request to give her the chemo/steroid treatments asap to lessen further damage to her kidneys. We were shell shocked at that time but thankfully they stressed the importance of catching it asap! The sad part of the story is that Holly had a slower progression of the disease and a high tolerance for pain/discomfort which in the end caused more damage. We're just not sure how much at this point but remain hopeful.

COMMENTS -- There are moments in your life that you never forget and remember every little detail -- like the birth of both my daughters -- and I'll never forget when one of the nurses who had grown fond of Holly in the couple of days that she tended to her began to tear up when I asked her if there was any news on the diagnosis. I really thought the worst and when they had me call the wife and 2nd daughter back into the hospital to meet with a "team" of doctors my bp went through the roof! What a roller-coaster ride!!! At first you realize that what they're telling you is that she doesn't have a "terminal" illness and you feel elated. Then as they begin to explain the treatments you soon realize that this is still "very serious" and the real fun begins once you start to deal with the mix bag of complications due to WG itself, the damages from WG and the bonus ones due to the actual treatments.

MOST IMPORTANT THING THOUGH!! She's doing okay and she is getting excellent care!

Thanks for the warm welcome!

[Sangye]

Hi Daggar, welcome to the group. I'm so sorry your daughter has Wegs. It's hard enough to deal with it as an adult, but 14..... I'm glad you found our group, though. We have several members who got Wegs at a young age. They have much to share that will help you and your daughter. Meanwhile, feel free to ask questions or let us know how we can help you.

[Daggar]

Thank you Sangye and yes 14 is damn young to have to deal with something this serious! Funny enough Holly has been the strongest of all of us and we find her trying to reassure us that everything will be okay. The nurses and doctors were shocked by her personality and demands to give it to her straight from the beginning. She may be 14 but she has an "old soul".

From what I've viewed on this website you, Jack and a few others seem to be the "Weggie Gurus" so I'm sure I'll have questions to ask in the coming days, weeks and months.

A couple for now -- has anyone experienced issues with their liver from WG or is it usually the treatments that elevate liver enzymes? And -- just curious as to how many WG patients need to be placed on an ACE inhibitor to control blood pressure while taking high dosages of prednisone? Holly has experienced a spike in her blood pressure of late but she's not at that point yet.

[drz]

Happy to hear treatment is going well and that they caught it early enough to avoid even more serious complications. I can't imagine what it is like to watch your child deal with Wegener's at its worst. I believe it was much harder for my family to watch me than it was for me to deal with this horrible disease. The Vasculitis foundation is worth investigating. Canada is having a big vasculitis meeting this spring too.

[DEE]

Welcome to the forum Dagger im also sorry your daughter has WG but hang in here, the people who post here are the best whether your having a good ot bad day
Ask as many questions as you need there is always someone that can find you an answer

[ArlaMo]

Sorry about your daughter! I have a 16 year old daughter and I can't imagine!
As for the hbp and pred, my nephrologist just put me on Lisinopril in January. I have had relatively low blood pressure all my life, but after 9-10 months on prednisone, it spiked. He also said it would help protect my kidneys (I have kidney involvement as well.)

[Jack]

Hi Daggar, blood pressure problems tend to be associated with kidney damage and I've been through the whole range of options over the years including ACE inhibitors. I now only take a Beta blocker to regulate my heart rhythm, so you can consider this treatment as being typical of the add ons that we tend to gather. Keep an eye on the increasing number of pills that your daughter will tend to gather and keep questioning if they are really necessary. None of them come without their own side effects, but many can't be avoided.

You are not alone in trying to find an innocent explanation for your daughter's symptoms in the early days, problems are far more likely to be caused by something common and no one expects a rare disease. My own daughter was complaining of chest and back pain a few years ago which we were convinced was due to indigestion. It turned out to be a collapsed lung! A year later, she was complaining again and we told her it was just some twinges from her previous surgery. It was the other lung collapsing! After a rough year or two she is fine again now. :biggrin1:

[Daggar]

Hello Jack,

I've followed some of your threads over the past few months and I've come to think of you as the "Weggie Godfather".... the fedora helps!! lol The one thread where you took a leave of absence (broken bone?) and everyone was concerned about your health truly shows how much the folks on here have come to appreciate your input.

Yes, we've come to terms with the fact that Holly's kidneys are probably the main cause of the elevated blood pressure but the prednisone probably doesn't help. They've also started her on a montly dose of iv methylprednisolone to further combat any possible inflammation that may still be present that may do further damage. The most frustrating thing was when we were told that we were doing everything "right" as far as diet and nutrition and that her blood tests were "excellent"..... we had that false hope that she may of been one of those rare few that beat it early. The blood pressure and elevated liver enzymes have given us a reality check and we need to look at things in the long-term. Our team of doctors are concerned about the liver enzymes because it has been a gradual increase over the past month of blood tests and they're not sure why. Drug reactions tend to be more of a sharp spike -- much like what I read from Raj on this website. There are no signs of infection so it is a mystery at this point and that is the scariest part of WG - the not knowing!!

I hope you don't mind me asking you questions over the next while....

[Daggar]

Hello ArlaMo,

Hopefully they caught the WG before it did too much damage to your kidneys? The bp meds may still be necessary for Holly -- she's doing a 24-hour monitoring starting tomorrow and we'll know soon enough.

The elevated liver enzymes is the one that really scares the crap out of me. It's hard enough dealing with kidney issues without adding the liver into the mix. Holly's diet has been kept below 2000mg of sodium per day and she only eats fish - no chicken/beef/pork. All of her blood counts are bang on (except creatinine) and nutrition isn't an issue so it continues to be a mystery.

[Daggar]

Thanks pberggren1!

A fellow Canuck ..... wish it were hockey we were chatting about!

Have you had any issues with getting the right doctors? I've heard a lot of nightmares from the clinic we go to regarding adult diagnosis and care. It is horrible for Holly to have acquired this at 14 but from all accounts the Children's Hospital is very good at dealing with WG.

[Jack]

I'm not sure that I deserve such an elevated description! I've just managed to survive with this disease longer than many here, although there are one or two who go back even further. Due to the nature of the disease and because I was a bit of a guinea pig in the early days I have experienced a lot of the problems, but of course, my experience can never transfer directly across to a different case. Everyone has a different story to tell and their own experiences to add.

Liver problems is one area I know nothing about so can't be of much help at all I'm afraid. From my own experience with my daughter's lung collapse, I can only sympathise with your position and know that you will do everything you can for your own daughter.

Best wishes to you all,
Jack.

[BrianR]

Hi Daggar,

My wife Marta deals (dealt) with elevated liver enzymes. They were sky high. Now they are normal. She is clearly one of the "exceedingly rare" cases that have liver involvement. The Dr wanted to stop her Cytoxan due to her elevated liver enzymes until they figured out what was causing it. We both felt that it was WG and rather than stop Cytoxan we had it increased by 50% (from 100mg to 150mg) and her enzymes started dropping almost immediately and now they are in normal range. Now, I am not saying this is the case for your daughter. I am just pointing out that the drugs can either lower or raise liver enzymes, depending on the circumsatance/condition. We were VERY worried and are now VERY relieved. If your daughter's high enzymes are due to WG then they will drop when the disease is controlled. If they are due to drugs then they will find an alternative. Just stay very, very vigilant and ask questions and pay attention to the trend in her liver enzymes against her medication. I do not pretend to know more than the doctor(s) but you may notice a correlation before they do. After all, you only have one patient to pay attention to.

Your daughter will absolutley improve.

[marta]

A couple for now -- has anyone experienced issues with their liver from WG or is it usually the treatments that elevate liver enzymes? And -- just curious as to how many WG patients need to be placed on an ACE inhibitor to control blood pressure while taking high dosages of prednisone? Holly has experienced a spike in her blood pressure of late but she's not at that point yet.

Hi again Daggar,

I have liver issues. I have felt all along that my liver enzymes go up when my disease is active. They were high before/during diagnosis (this is before I was on any meds other than some antibiotics that the docs here were giving me for my supposed ear infection) and they went up again when I flared in October/November. The docs actually took me off the chemo drugs because they were afraid that they were the cause of the elevated LFT's but I felt it was a flawed experiment on my butt - pardon the expression. It takes about a week for the chemos to kick in and in reverse it takes about a week for them to get out of the system. They kept putting me on (at my request) and then taking me off several times with about a week or so in between. Things would just start to stabalize and they'd take me off. Finally I put myself back on as I felt the Wegs dog doing mean and nasty things inside my head. I told my docs that there is significant chance of permanent serious damage (loss of hearing, loss of sight, saddle nose) if we keep experimenting and not treating the disease - stuff I felt going on in my eyes, ears and nose. As soon as we went back to the cyclophosphamide and then increased it in January due to my increased weight my liver enzymes have completely normalized. I was at over 350 GGT and my last blood test was 51 GGT. 152ALT and last test was 21.

At my last doctor visit, she said to me after looking at my bloodwork. I now agree with your assumption that your liver is involved with your disease. I totally get that they want to protect my liver, I only have one, but if it comes at a cost of not treating the disease, then I'm not so into it. I also have a pretty good sense of my own body. Now things are a little different and I have to re-learn a bunch of stuff, but I used to be quite active in a bunch of different sports and was very finely tuned into what everything inside me felt like. When I finally demanded to be put back on the cyclophosphamide I ended up printing up a bunch of papers where WG patients had liver involvement. Not sure if my rheumy at the time put me back on because I convinced him or because he just wanted to get me off his back, but it all turned out for the best.

As for our previous correspondense - anytime you need a mountain vacation, you're welcome. We look forward to meeting you and your family.

[marta]

Ha! Brian and I were posting at the same time. He's a lot more eloquent than me. I just babble on and give way too much info. That's why we work so well together.

Best of luck.

[NicShaf]

Daggar,
I'm sorry to hear about your daughter's diagnosis of WG, especially being so young. I was diagnosed at 26 (just a couple months ago) and I thought I was young for this disease. On the plus side, I was told by all my doctors that being young and healthy (except for WG, of course) should help me in the fact that there is no reason for my body not to respond to the treatment and achieve remission...this should go for your daughter too.

As for the liver, for the past two weeks my liver enzymes have gone up, they were in the low to mid 20's (units/liter) and they have gone up to 36u/liter. My pulmonary is the doc who pointed it out, I saw him this week. He thinks it's the Cytoxan, and we need to keep an eye on it, but I'm barely outside the normal range, so if it stays there, he's not too concerned. I go in for weekly blood tests and I see my Rheumy next week, I'll ask about it then and see what he says....hopefully it's the same still.

Getting all the information you can on WG is the best way to go, and everyone here will be able to help a lot...as I'm sure you can already tell. I have quoted people from this site to my doctors, telling my doc I read it in an article or something, and they are usually right on the money.

Best of luck to you, your daughter and your family.

[ArlaMo]

Daggar,

Thanks! I was close to renal failure when I was finally diagnosed, but the cytoxan got my kidneys under control pretty quickly. Unfortunately, my sinuses are still out of control and it looks like I might have some lung involvement (bronchoscopy tomorrow) so we're looking at changing to Rituximab after the results are in.

My husband is a fellow hockey-loving Canuck, displaced to the south a bit since he started hanging out with me (almost 20 years now!)

[Sangye]

Daggar, it's wonderful that your daughter has you in her corner. You do so much to educate yourself about Wegs and her particular case. That's what will make a difference in her recovery.

Have you gotten a VF specialist to consult with your docs? Canada has some, and you can also access the ones in the US.

[Daggar]

Thanks for all the responses.... the wealth of knowledge from the people who have gone/are going through it is "invaluable"!

Holly is one of those kids that just doesn't like to talk about her pain or things that are bothering her - complete opposite of myself! If I'm not performing up to par I'm all over it! lol

She told me last night that she has been having ear popping sensations over the past 2 days and I'm starting to wonder if there was some disease activity within the inner/middle ear to cause an infection or previous inflammation that the standard dose of oral prednisone hadn't dealt with? Seems odd that this just started happening since the iv methylpred treatment on Monday. She has been dealing with a cold over the past week or so as well which she has dealt with quite well.

Her Nephrologist has said that it is either an infection or a slow reaction (toxicity) to the cyclophosphamide or sulfatrim/cotrimoxazole. Her rise in AST, ALT and LDH have been very slow and consistent over the past 2 months. They are now above normal and appear to be continuing the upward trend very slowly.

Sangye -- yes, Holly was lucky to have been able to go to the Children's Hospital in Calgary where they have a "team" of specialists that have dealt with the WG issue. She has a team of Nephrologists and Rheumatologists as well as an Ophthmologist, Gynecologist, Dermatologist and Paediatrician. Some of the doctors are professors at the University of Calgary due to the close proximity and working relationship. I've also been in touch with a Dr. Brogan (Jack's neck of the woods - Birmingham, England) for some needed input on the course of treatment. I did not want to make any "wrong" choices at the start so it was awesome that he responded!

All-in-all, she is in good hands and she'll be doing some further tests later today and early next week to try and figure out the next step.

Marta -- thanks again ... you are too kind and Holly and I are looking forward to meeting you in the future!!

[marta]

I'm posting this to hopefully put your mind at ease a little. I had the ear problems at first, and when I went on the drugs it took a while but I also experienced the popping in my ears. From my understanding and discussions with my ENT, the early blockage and hearing loss and fluid in the ear is due to inflamation and the shutting down of the eustacian (sp?) tube. Once the inflamation starts to subside (and I imagine this is happening quickly due to the extra IV methylpred) the tubes start to open up slowly and the gunk built up in there starts to move around - hence the popcorn in my head feeling. It was shortly after that, that things in my ears started to normalize and I started to get some of my hearing back.

[pberggren1]

Thanks pberggren1!

A fellow Canuck ..... wish it were hockey we were chatting about!

Have you had any issues with getting the right doctors? I've heard a lot of nightmares from the clinic we go to regarding adult diagnosis and care. It is horrible for Holly to have acquired this at 14 but from all accounts the Children's Hospital is very good at dealing with WG.

Hi there Daggar:

I am very fortunate now to have a Wegs specialist right in town here. His name is Senyo Tagboto and by far the best doc I have ever had and I have seen just over 60 docs in the last 8 years. His specialties are Internal Medicine and Nephrology. He also comes from Jack's neck of the woods and worked with Dr. Adou and Bacon and Savage. He said back in the UK he was involve in diagnosing about 1 case of Wegs a month on average over 18 years. And on top of that he saw Wegs patients in the clinic by the hundreds each year. So he definitely knows Wegs. If you run into any problems with docs or are not sure about something just let me know on here or send me a private message. I am more than willing to help. I'm sure my doc is even willing to consult if need be.

[Daggar]

Thanks Phil... I'll keep that in mind. Hope you're doing well!

Finally got some numbers back from the lab work done yesterday and it all looks "good"....

Holly's liver enzymes are on the way down and her serum creatinine level is at the lowest (101) since being admitted to ER back in November 2010. Urea level is also normal for the first time since then.

Her blood counts have almost all climbed/dropped into the normal range (RDW still a bit elevated).

C-Reactive Protein still flatlining at 0 and the nasty Proteinase 3 antibody believed to be linked to all the problems has dropped to its lowest point 5.5 (just above the 0-5.0 normal range)

So we're cautiously optimistic for her and it is truly amazing how a test result can elevate your spirits! Hopefully they continue.

Have a good weekend everyone!

[jeriorleans]

So very sorry to hear about your daughter. My son, A.J. was diagnosed in September of 2009 at age 13. His journey began with an ear ache in Feb. of 2009. His pediatrician said he had fluid behind his ears, no worries it will go away. He was given his vaccinations at that time, which has always made me wonder if this was the "trigger." Things went downhill from there. He had nose bleeds and crusting. He had constant headaches and complained that his "face hurt" and his teeth hurt. He had strange purple like blisters on both ears, a couple on his face, and his chest. Looked like ink marks. Dermotologist said, no worries, its just blood under the skin. Ear aches continued. Fatigue started. First ENT put tubes in his ears in the office without anesthesia. A.J. was in so much pain at the time, he didn't care how much it was going to hurt. Tubes fell out in 2 weeks. Pain continued. Numerous trips to the ER with various diagnoses from swimmers ear to ear infections. Finally in August of 2009 he was seen by another ENT who took him i into surgery to put new tubes in his ears and found the granulation and sent it out for biopsy. Prior to this no one had even mentioned Wegeners. Unfortuntely the ear problems had gone on for too long and done too much damage and he lost about 80 percent of his hearing. He now wears hearing aids. The Wegener's also affected his lungs. Unfortunately, the only pediatric rheumtologist in New Orleans didn't know what he was talking about so A.J. treats at the EMory Clinic in Atlanta. He started with Cytoxan and 60 mg of Predisone. He now is on Methotrexate, Bactrim and Folic Acid. Although he seemed to be doing better, his doctor called me last week and said we are at the 1/2 way mark (one more year of methotrexate) but he wasn't real impressed with his blood and urine specimans. He developed blood and protein in his urine several months ago and it has not gone away. The protein left for a brief time, but its back. They are going to monitor it for another 2 months and then if it is still present perform a kidney biopsy.
Its a very scary ride. I still have not learned not to overreact whenever he stiffles or sneezes. He constantly (and impatiently) reminds me that not ever cold is Wegeners.
I have found that talking to all his teachers and principal first think and apprising them of his situatin is the best thing to do. Fortunately, he goes to a private school (public schools not an option in New Orleans) so they are a little more lenient when it comes to missing time.
Well, I've gone on and on. Feel free to ask any questions. There aren't many young Wegener's patients out there.

[Daggar]

Hello Jeri,

Sorry to hear about A.J. as well. It sounds like he has been through a lot and I hope the doctors in Atlanta have the WG under control. Your mention of kidney involvement scares me because that is what really hit Holly hard. Has AJ's serum creatinine been rising along with the protein in his urine samples? You mentioned prednisone with his initial treatment - what dose is he currently on? Is there a possibility that the WG is still active and is effecting his kidneys or is this a result of what he went through?

I hear you with the "overreaction".... the first month after Holly was released from the hospital was the absolute worst! I became her shadow and almost drove her as crazy as myself!! We're now at a more "manageable" craziness. Holly's school/teachers have been amazing throughout the entire process. She goes in a bit late each day due to the chemo/steroid treatment and she has regular clinics/lab tests to attend so they set aside a couple of periods each day where she can catch up with help from a teacher, vice-principal or principal.

The one thing I've learned from this is that kids are "tough" and surprisingly rise to the challenge better than most of us adults. Still... I find myself wishing they could somehow hook up an iv to Holly and I so we could switch places.

[jeriorleans]

My thoughts exactly as far as changing places. I think as kids they hide so much pain and I wish that just for one day I could take all of that away. I don't think we can even imagine what they must be going through mentally and physically. It only takes them googling Wegener's to know that this is a very dangerous disease.
A.J. is not on steriods at all anymore. He was weened off after about a year. I'm really having a bad feeling though, he is sniffling and picking at his nose like in the early stages. (I say this while at this exact time he is outside playing basketball). Overreacting? I hope so.
There are other sites oriented to parents with children with Wegeners on yahoo and facebook but I really don't find them very useful. THe yahoo group seems to get way off subject and not offer much useful information. Feel free to email me personally at [email protected] if you would like to chat.
MAke it a habit to check this site reguarlly. It has invaluable information and advice from great people.

[Palmyra]

Hi there Dagger,
My daughter also has WG...she first developed Crohn's disease at the age of 14, and WG at the age of 20. She is now 25, and I am in the current position of pulling back on the parental controls, and encouraging her to enter this board and make more of her own medical decisions. That hasn't been easy, as she is still of the mindset to be 'normal' like all of her peers. She is only just now beginning to accept the fact that these are chronic conditions, and she needs to take advantage of any programs that may be of assistance.

In retrospect, coming from someone that has has a child with serious disease issues during the teen years, it is important to advocate for all of the educational and government provided advantages you may have available. I learned to navigate physicians (they can be angels or nasty buggers,) and schools alike (really hard in this State!) to try and provide the best services I could muster. Things are different here in Texas, USA, but emotionally all very much the same when you have a child with a serious illness.

Best wishes to you, and glad that you found us....there is literally a "world" of information on this site!