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Grandma2Katie
03-21-2009, 08:40 AM
Hello Everyone. My name is Cindy. I live in North Carolina, USA. I joined this forum because my sweet, little granddaughter, Katie was just diagnosed with WG. She is only 7 years old.

In February, she had her tonsils and adnoids removed and surgery done on her nasal passages which had completely grown together. Her recovery has been slow. One night, she ended up in the emergency room because she was throwing up blood and blood clots.

She was referred to another surgeon and a hemotologist. After tests, tests, and more tests, the diagnosis finally came this week. Now the doctors want to biopsy the nasal tissue, kidneys and lungs.

None of us knew anything about WG until last week when the doctor suggested testing for it. We all have a lot to learn so that we can make informed decisions about what is best for Katie. So I am here to learn as much as I can from the folks that know best what she is going through.

My best to all here.

Cindy

Sangye
03-21-2009, 09:25 AM
Hi Cindy,
My heart just sinks reading that your granddaughter is only 7 and has been diagnosed with Wegs. It sounds like they've only diagnosed it through blood work, is that correct? No biopsy yet? Is she on any meds? Where is she getting medical care?

I'm so glad you found this forum. Everyone here can give you lots of info and support. There's also a Yahoo group for parents of kids with Wegs. I don't know the group name, but you can call the Vasculitis Foundation. The group only allows parents (I'm sure grandmothers, too!), and the VF screens members for them.

Grandma2Katie
03-21-2009, 11:25 AM
Hi Sangye. No, Katie hasn't had a biopsy yet. That should be scheduled on Monday the 23rd. She goes in for more blood work then. As far as I know, she is taking antibiotics for the strep throat she came down with and the supplement, folic acid??, to help increase her red blood cells. No treatment has begun for WG.

She is VERY anemic and has lost 15 pounds since Feb. 4. She doesn't want to eat. Food makes her nauseous. She has had muscle pains in her legs at night. Her sinuses are completely closed off and one side of her nose has already grown back together.

The doctors told my daughter they suspected either Von Willenbrand's or Wegener's. The test for Wegener's came back positive on Wednesday. So we are just getting started with this.

Anything that you know of that is essential for us to know when first starting out would be greatly appreciated. Katie is being treated in Charlotte by Dr. Eric Mayer. Her parents are very confident in him.

Thank you for replying......... I know there is a wealth of information and friendship here.

Cindy

Sangye
03-21-2009, 11:50 AM
(I wonder why they're thinking Von Willebrand's-- that's a hereditary bleeding disorder. It only causes easy bleeding, but would have nothing to do with her sinuses closing off, etc....)

It's necessary to do a biopsy to confirm Wegs. Personally I've never heard of them doing a biopsy of sinuses, kidneys and lungs all at once. Are her kidneys involved? (They'd know from urine and blood work) If not, I don't know why they'd biopsy them. Usually they go for a tissue sample that is both easy to obtain and also the most likely to give the best result.

Have her parents tried giving her smoothies? They can add protein powder and other nutrients. Don't make them with ice or dairy-- hard on the stomach. For an itty bitty kid losing 15 lbs. is a lot.

Sorry to bombard you with questions! It's just that Wegs can move quickly and so many of us lost time with doctors who weren't familiar enough with it. It's very important that they do the biopsies as soon as possible. If it's Wegs, they have to start treatment right away.

I'll keep all of you in my prayers. :)

Grandma2Katie
03-21-2009, 12:41 PM
The doctors will biopsy the nasal tissue first since the nasal passage has grown together again. Von Willenbrand's was ruled out before the diagnosis of WG. I can't answer your question about the lung and kidney biopsies. I'm four hours away and get all my info from my daughter.

One thing I read which is eating at me: the chemo drugs will make her sterile and it is recommended that steps be taken to preserve eggs or ovary tissue for later use before starting the chemo. Do you know anything about this?

How do you make smoothies without ice or dairy?

Cindy

crackers
03-21-2009, 01:22 PM
hi cindy.first as a grandfather then as a WG sufferer my heart goes out to poor katie.have you introduced her parents to this forum? if not you need to do so.from personal experience,in a crisis,it needs grandparents to oversee the situation.it may be that katies parents are focusing on her and it may need you to focus on the docs to get the treatment that she needs as quickly as possible.don't take a back seat cindy,shout and holler as much as you want for katie.
john.

Sangye
03-21-2009, 01:39 PM
It must be so frustrating to be far away, getting info secondhand. I bet Katie's parents are on total overload, too, making it even harder to keep you updated.

Yes, the chemo and sterility. A consultation with a pediatric oncologist is a must, because no one knows better than them what the options are.

You can make great smoothies using rice milk instead of regular milk. Rice milk is very easy to digest and doesn't create phlegm. (It's not a dietary substitute for milk, since it's made from a grain, but it is quite nutritious.) You can also use yogurt instead of milk. Much easier on the stomach, and might actually help it heal. As long as the ingredients are refrigerated, there's no need for ice. It won't have the same texture--more like a shake. Protein powders help bulk it up and will give her nutrients she needs.

Soups are a good option, too, just not the cream-based ones.

Grandma2Katie
03-21-2009, 01:59 PM
Hi John. I have sent several email messages to my daughter, one containing a link to this forum but I will send it again. You are right..... they are distraught to say the very least. Since the diagnosis was only made on Wednesday and they don't have another appt. until Monday, I'm trying to wait it out to see what the next move is by the doctors. I have only been reading up on Wegener's for about a week and don't want to overload them with what I have read. I really would like for them to seek the info for themselves. I will push for a membership here. The best info comes from the people who live with it everyday!

Thanks for your input.... keep it coming!!

Cindy

Grandma2Katie
03-21-2009, 02:01 PM
Sangye,

I would like to copy and paste your message into an email to my daughter. Would that be ok with you?

Cindy

jola57
03-21-2009, 05:32 PM
Dear Cindy, my heart is with you and your family. How is Katie doing? This must be so difficult for her to understand. With the chemo, I'm with Sangye, seeing an oncologist is a must. If you have the time, read the posts, there are some members that also have had this since childhood and are still here. There are other ladies that have had children after being treated for WG. Please do not lose hope and let your children know that we are here for them as well as for you.
Sending warm hugs,

Sangye
03-22-2009, 12:21 AM
Sangye,

I would like to copy and paste your message into an email to my daughter. Would that be ok with you?

Cindy

Of course!

I agree with John that they might really need help from you because they're on overload. I was diagnosed as my doctor drove me to the hospital. All I knew about Wegs was what they told me. They weren't Wegs specialists, so it was pretty inaccurate info.

I didn't have computer access for months, and none of my friends did internet research the way I would. I didn't know of this group for 2.5 years! I was completely shut off from information that would have helped me tremendously. I was so overwhelmed with phone calls and visitors, I couldn't ask for help. And, I could barely function being so sick and weak.

It may be difficult for them to ask for your help. It's best to just offer it.

So please share what you've learned with them in your computer travels. You can repackage it to be easy to read. Ask if they need/want it-- would it be helpful. If they're not ready, hold onto it--they will be soon, I guarantee you.

Meanwhile, keep collecting info. It's necessary to know everything you can about Wegs. Judging from many of our experiences, you might save Katie's life.

coffeelover
03-22-2009, 02:41 AM
Dear Cindy,

I am sorry for you and sorry for Katie having such a devasting illness at such a young age. I know from experience as my neice suffered from a rare liver disease when she was 7 years old and went through what no child should ever have to go through for the next few years.
I hope they can take care of her sinus problem soon, so she can feel like she can breathe. I too suffer from the sinus problem to some extent and have tracheal stenosis and a trach in to help me breathe...all due to WG.
Sangye is right...we want you to get treatment right away, as soon as possible, because the sooner she gets treatment the quicker she can get in remission.
I too was afraid to go on the Chemo drugs, but you have to do what you have to do. Hopefully her parents will get some consults and options for Katie and as a grandma, I would encourage you to stay inovolved and pro active. YOU may be the only one thinking rationally at a difficult time like this. I know from our family experience, that "grandma and grandpa" were what got my neice through the toughest of times and their support of the parents was a much needed avenue of healing for all.
Best of luck and keep on chatting with us.
Lisa Coffeelover

andrew
03-22-2009, 08:42 AM
Sangye,

I would like to copy and paste your message into an email to my daughter. Would that be ok with you?

Cindy

I'm sure I can speak for Sangye when I say go for it.

andrew
03-22-2009, 09:03 AM
We use Sustagen for people that need to eat but aren't up to solid food. You probably have same or similar there. Check here for what I mean: http://www.sustagen.com.au/

Regarding the biposy, they'll probably just pick one area i.e. sinus, lung or kidney to biopsy rather than all three I would have thought. Probably they'll do the sinus as I would assume it would be the easiest to get to. I would also think that they would have started her on Pred at least by this time?? They are doing the right thing though in getting a proper diagnosis via biopsy.

Agree with Sangye that a Pediatric Onc. is a must-do ASAP if one hasn't already been summoned. He/she will know what's best for someone so young.

Thoughts and prayers are with you, your Granddaughter and the rest of your family.

Grandma2Katie
03-22-2009, 03:11 PM
Thank you all so much for your responses. I'm not getting anything from daughter & son-in-law at the moment. I did copy Sangye's post and sent it in an email to them. All I can do at the moment is send them info. It is very frustrating from this end. I'll let you all know when I hear anything new.

Cindy

Grandma2Katie
03-23-2009, 12:18 PM
Just got an email from my daughter. They have had a very rough weekend with Katie: nausea, vomitting, fever. They have gone ahead to Charlotte tonight because the appt. in the morning is at 8:30. They are seeing a pediatric oncologist/hematologist. I am praying that he will admit Katie to the hospital until they can conclude her tests and start her on meds.

Laura, my daughter, did reassure me that they will address the fertility questions with the doctor. Right now though, they are very alarmed at how fast Katie is going down hill. Any of you so inclined, I ask for prayers for Katie. We are all worried sick about her.

Once again, many thanks to all of you for sharing your information. I am passing it along to my daughter. Perhaps when things settled down a bit, my daughter will be able to join.

Blessings to all,
Cindy

Sangye
03-23-2009, 12:36 PM
I will certainly keep Katie in my prayers. This is so troubling to hear.

I think they have to admit her to the hospital. Her parents need to be firm about it, and not just hope that the doctors will do it. You might be the only one who really understands how serious Wegs is. Please tell them to insist that she be hospitalized and that a biopsy be done immediately. Any procedures happen faster if you're hospitalized, rather than an outpatient. She's lost so much weight, she has no appetite, and now she's vomiting, too. She needs to get on IV fluids and get nutrients in her. I'm not even going to apologize for sounding bossy--this is serious business and she's heading downhill.

Most often pediatric specialists do a good job with things like this. But there are many cases where they didn't, and the child was put in jeopardy as a result. It happens more frequently with Wegs, because so many of the symptoms can be attributed to other things.

Hang in there, Cindy. I'll keep checking in with you.

Grandma2Katie
03-23-2009, 03:25 PM
Thank you Sangye. I will call Laura early in the morning.

Cindy

crackers
03-23-2009, 08:31 PM
hi cindy.i agree with sangye that katie needs hospitalisation so that her condition can be monitored closely.she sounds to be in decline and this needs addressing immediately.you're all in our thoughts
john.

Grandma2Katie
03-24-2009, 12:25 AM
We got very bad news this morning. Katie was rushed to Presbyterian Hospital this morning at 2am. She is now in the pediatric ICU. Her lungs are bad and her kidneys have shut down. She is receiving fluids and blood transfusions. Doctors are being called in from all over the state to help. She will start cytoxan today.

Cindy

Sangye
03-24-2009, 12:40 AM
Oh, Cindy, this has me in tears.

As I read your post last night I thought that was the case. It's not as obvious as you'd think to have the kidneys shut down. On the plus side, there are many people (I know they will chime in here) who had their kidneys shut down and the kidneys recovered.

It's good that they're getting docs from all over, but they must have a Wegs specialist. I bet you need something to do today to keep from worrying. Please give VF a call and see if they can help you get hold of a VF doc who could advise Katie's docs right away. I can assure you her parents are not able to do this. They're bombarded with doctors coming and going, nurses, procedures, info--all this happening to their little girl so fast. They don't know that Katie's doctors don't know.

You can email me if you want to talk and I'll send you my phone number. Please don't wait to call VF.

Grandma2Katie
03-24-2009, 01:08 AM
Sangye,

Will call VF right away. I am pleased though with how assertive Laura and David are being with the doctors. I reminded Laura to keep notes on treatment. She has gone back to Forest City to get clothes and necessities and make phone calls. She seems to be holding up pretty well considering how fast all of this has happened.

Thank you again..... will check in here off and on today.

Cindy

coffeelover
03-24-2009, 03:05 AM
All I can say is I am so sorry! I hope the transfusions and cytoxan helps quickly. I will be praying for her recovery!
Lisa

crackers
03-24-2009, 04:38 AM
cindy as quickly as the symptoms have progressed they can be halted with the proper treatment and medication which hopefully she is now receiving.everyone here has katie and all the family in our thoghts.
john

andrew
03-24-2009, 05:25 AM
Hi Cindy...
Sounds similar to my situation. My kidneys shut down and my lungs were almost full of blood (small section in the top of my right lung was clear). With the correct, aggressive treatment, kidneys and lungs came good. My kidneys are now just a pinch above normal and I can wee like an elephant.

It's good that she is getting a heap of attention too. I and my parents (who are probably reading this) will prayer for a quick turnaround in Katie's condition.

Grandma2Katie
03-24-2009, 05:41 AM
Thank you all. I just spoke with my daughter and son-in-law. Katie is being air lifted to Baptist Hospital in Winston-Salem where the specialists are waiting for her. Her kidney function has improved some and her dad said she is looking better. I will be able to visit her perhaps tomorrow because she will be only 40 minutes away. I am relieved that she will be in a hospital with doctor's who have treated this disease before.

Cindy

andrew
03-24-2009, 06:28 AM
W00T!! Doin a happy dance :D That's good news both that she's responding to treatment and that she's going to be closer to you. That hospital looks like a good one too from my 'Googling'.

Sangye
03-24-2009, 07:02 AM
Very good news!!! Please keep us posted. I've discovered a lot of hospitals have computers you can use, so maybe you can update us from there also.

(Hey, what's WOOT? I see it all the time, and think WHAT?)

andrew
03-24-2009, 07:09 AM
(Hey, what's WOOT? I see it all the time, and think WHAT?)

It used to be 'cool, daddy-o', but now it's w00t. I say it to sound more modern and hip and oh so groovy yeah baby. :D

Urban Dictionary: w00t (http://www.urbandictionary.com/define.php?term=w00t)

Grandma2Katie
03-24-2009, 12:44 PM
News is worth at least two woots! So, Woot, Woot!! :-D Lastest news, more procedures tomorrow. They are going to do a kidney biopsy and filter the kidneys. Sorry, I don't know the correct term but it is similar to dialysis. There is blood in the lungs but she is breathing on her own and they have her on oxygen. The prednisone was started this morning. She was sleeping when I spoke with Laura a little while ago so all seems well for the night.

Thank you all for getting a frantic and frightened Grandmother through the longest day of my life. BTW, Sangye, the Vasculitis Foundation called me today and offered any help we might need. Thank you for the suggestion.

Blessings to all,

Cindy

crackers
03-24-2009, 12:54 PM
that's very encouraging news cindy.that poor girl has been on my mind all day.we have a grandson about the same age so i know what we would be going through if it was him.our thoughts are with you all.
john

andrew
03-24-2009, 01:03 PM
That's gerat to hear. Brings a smile to my face. I've been hanging out for news since the last post and very happy that she is responding so well and has such great people around her!

Sangye
03-24-2009, 01:32 PM
THANK GOODNESS Katie's getting everything she needs. Sounds like they're right on it. The pred will work very quickly to alleviate symptoms. You won't believe how much better she'll be in a few days.

Thank you so much for keeping us posted. She's been on my mind all day.

Grandma2Katie
03-25-2009, 12:32 PM
After wandering through a maze of corridors and searching for the proper elevators, I finally found Katie and her mom. Katie was sleeping when I arrived but woke fairly soon afterwards and the tears started to fall as soon as she realized she was still in the hospital. She let the whole ICU staff know in no uncertain terms that she wanted to go home NOW. She is one unhappy little girl. She thinks everyone at the hospital is mean and won't let her eat or sit up.

Her lung x-ray was not good today and her kidneys are only working 14%. I was hoping for a big improvement today but that doesn't seem to be the case. She is having a great deal of trouble breathing and hates the oxygen tube.

At least I know she is getting the medication she needs. She is also getting quite a bit of other meds but I don't know what they are. They put in the cathater for the dialysis, but that won't be done until tomorrow.

I wish I could take her place.

Cindy

crackers
03-25-2009, 12:47 PM
cindy i know this must be very frightening for her but she is in the best place for her right now.hopefully once the meds kick in things will improve.
john.

Sangye
03-25-2009, 01:07 PM
Hi Cindy,
Thank you so much for keeping us updated on Katie. I bet it was a mixed bag to see her today-- relief and sadness. Wegs and the response to treatment isn't linear, so it's to be expected that she's still in bad shape. Don't get disheartened. If she isn't losing ground at this point, that is progress. It's kind of like reversing a speeding train-- gotta slow it down, then stop it, and then you can put it in reverse.

Don't underestimate the power of youth. Her body is programmed to build and repair at a much greater rate than we older folks. And it sounds like she's feisty. Very good sign. :)

Grandma2Katie
03-25-2009, 01:15 PM
Fiesty, indeed she is. She has dubbed the entire medical staff as "rockheads."

I met her rhumatologist today. I asked about how long we could expect Katie to be in ICU. Her guess was one to two weeks. Does this sound reasonably to you all?

I am one bewildered grandma.

Cindy

Sangye
03-25-2009, 01:53 PM
(Well, fiesty is good, but I don't know that it'll help her to call the people keeping her alive rockheads. By fiesty I mean she's got a lot of life in her and a strong will to live.)

I think anything is reasonable, considering the shape she's in. She'll get the most attentive and skilled care in ICU, so if they'll keep her in there, I say let 'em.

We know it's confusing. That's why we're here to help fill in the gaps, so maybe you won't have to go through as much confusion as most of us did.

coffeelover
03-25-2009, 02:33 PM
I am glad Katie is getting the care she needs! I have been thinking about her along with the rest of the WG gang. ICU is not fun, all that poking and prodding, but I like Katie's spunk. She needs to find a way to accept this disease, a very tough task for such a wee one.
Grandma! Keep up the good work! You are more needed than you think!
Keep us informed when you can
Lisa

Grandma2Katie
03-25-2009, 03:05 PM
Thank you all for being here. I was rather depressed when I got home from the hospital. I have to trust that Katie is where she needs to be.

Katie has always been spunky! Her mother and I talked her out of giving the nurse a "knuckle sandwich." So perhaps "rockhead" isn't quite so violent. She is just so miserable. The doctors give her sedatives to calm her down when she gets so distraught. I wish they could find a way to just let her sleep through the whole ordeal but I know that isn't possible with the lungs as compromised as they are.

We are all hanging on to the kind thoughts and good wishes from all of you. They mean so much.

Cindy

Doug
03-25-2009, 05:53 PM
...and it is plasmapheresis! That is the procedure like dialysis that your granddaughter had. It was developed through research done by MDA funding. When I researched it, discovered it was MDA money that helped make it possible, realized that I, in a sense (and at age 55, then), was one of Jerry's kids, I bawled: it saved my life! Drugs weren't pulling me out of severe WG, which I had ( and your granddaughter has), but dialysis, then plasma pheresis did. I was on dialysis for a week, had a day off, then had dialysis followed by plasma pheresis, a day off, and a final shot of dialysis followed by plasma pheresis. It is painless, though, my heart goes out to your granddaughter, your family, and you because the all of the preparations getting to the painless part are, no doubt, scary to a child, perhaps even a bit painful. You've been given excellent advice so far. I'm particularly glad you made contact with the Vasculitis Foundation, and that they've given you good support.

Sangye
03-26-2009, 12:10 AM
Hi Cindy,
These first couple weeks are some of the toughest times you'll have. Katie went from a healthy child to a very sick one overnight, and it will take all of you awhile to get your minds around it. And since Wegs is a chronic illness, it's a whole different thing. Right now is the time for gathering info and getting her started. Once the dust settles a little, you will all (including Katie of course) have some grieving to do. Because this is like a death--losing the "dream" you all had about Katie, which certainly never included something like Wegs. If you lean towards such things, finding a good therapist now will help you in the months ahead. It has kept me alive to share that.

One other thing : It's possible the hospital staff will tell Katie's parents about CaringJournal, but just in case.... It's a free website service from a non-profit that provides ready-made, very easy to use templates and hosting for people like Katie. The basic idea is to save you from having to call/write/email everyone with every update. You can write a daily journal entry, put Katie's story, announce and organize fundraisers, post pics and videos of her, etc.... Loved ones can send messages in a guest book to keep in touch, too. You can even keep changing the template with a click for different holidays, etc...

I don't use it myself--wish I'd known about it in my early very overwhelmed months, though. I keep up with many friends via theirs. Anyway, the link is
CaringBridge. Free Websites That Support And Connect Loved Ones During Critical Illness. (http://www.caringbridge.org).

Be well,
Sangye

Lotte
03-26-2009, 12:12 AM
Hi, cindy i was 11 yrs when diagnosed with WG. Obviously all my parents could think about was getting me well again. Naturally everything else came second including whether or not i could have children in the future. I went through the menopause at the age of 22 probably due to chemo. Everyday my husband and i miss not having children. Life goes on and we do not dwell, we love our dogs instead. Katie is a lucky girl having you to look out for her. If you can give Katie the choice of having children in the future, it could mean a lot. Things were different in my day and my parents were wonderful and strong for me in fact i never knew how ill i was thanks to my parents, i just thought i was a super star. I really don't know how my mum did it but we just laughed through the good and bad and thats what i remember the most, the laughter. So its not all bad. Try and keep smiling, i know it's hard.

Doug
03-26-2009, 02:51 AM
Sangye is right. This website is probably the best way to keep everyone interested in your granddaughter's progress informed. You don't have time to make all the calls you'd need to make or take all the calls you get without this sort of help. It certainly will reduce stress and save you repeating the same thing over and over. When I was at my worst and my friends and family were their least informed about my disease, the last thing I wanted to do was talk over the phone to let them know what was going on. I didn't have anyone to do this for me. It was very, very, very tiring.

Grandma2Katie
03-26-2009, 03:03 AM
Doug, what a beautiful prayer. Thank you. It is a comfort to know that Katie's family has grown to include such wonderful, caring people.

Cindy

Grandma2Katie
03-26-2009, 03:13 AM
Lotte,

I wish treatment could have waited long enough to address the fertility issue but Katie was critical when she entered the hospital. Her parents were aware of the fertility issue ahead of time. We all thought that saving her life was more important.

I hope Katie will grow up, marry and have a family like my parents did. You see, I was an adopted child, a very lucky adopted child. So even though she will not have the option to have her own children, she can still have children of her own.

Cindy

Grandma2Katie
03-26-2009, 03:16 AM
Sangye, what a great idea. I will check it out. It would save a lot of time, especially for Laura and David. Thank you for the suggestion.

Cindy

Sangye
03-26-2009, 03:21 AM
Cindy,
I spoke to a friend whose daughter underwent chemo for a rare cancer which began at age 3 and metastasized within a year. She had cyclophosphamide-- probably the same chemo Katie is getting--but of course at the "cancer" level. She had many, many rounds for months. Her docs occasionally did imaging studies of her ovaries to check the size (not sure what/how). Hers actually grew during the process, so the docs believe she may have retained her fertility. She is strong and vibrant today--no sign of cancer or anything else. I see her and can't believe she's the same kid.

I say this so that you won't think it's a done deal. Anything is possible. Little bodies do heal.

And like you said, either way she can be a mommy with children of her own.

Lotte
03-26-2009, 03:33 AM
Unfortunately in England someone with WG isn't fit to adopt a child. Not where i live anyway but i know we are sometimes a bit behind the times, other areas may not have come across this problem. I didn't lose my fertility until (they think) my 3rd cycle of Cyclophosphamide which i had over a few years. I was in Great Ormond Street hospital for a year before i was diagnosed with WG. I had been in quarantine at my local hospital 3 months prior. I hope things have changed since my day!

Grandma2Katie
03-26-2009, 04:37 AM
Just spoke with Laura. Good news! Katie may be able to move out of ICU tomorrow!!:):):)

They are keeping her more of less sedated to try to keep her blood pressure down. The first para paresis (?) was done today with another scheduled for Friday and Sunday.

I passed on all the hugs and well wishes to Laura and asked her to give Katie a giant hug from all of you wonderful folks.

BTW, thank you all for the birthday wishes. I have gotten the best birthday present of all; improvement in Katie's condition.

Cindy

Doug
03-26-2009, 05:45 AM
Cindy- I copied some information on plasmapheresis (I thought it was two words) to save you some time looking it up. Please note that I am not endorsing MDA or suggesting you get out your VISA and help a few people with other autoimmune diseases or suggesting that this treatment is an end all be all treatment. It brought about a remarkable turnaround in my condition, I know from specific, personal experience.

Facts About Plasmapheresis
Muscular Dystrophy Association


Updated 07/05

http://www.mda.org/publications/images/fa-plas111.gif
http://www.mda.org/publications/images/clear.gif Plasmapheresis and Autoimmune Disease
Many diseases, including myasthenia gravis, Lambert-Eaton syndrome, Guillain-Barré syndrome and others, are caused by a so-called autoimmune, or self-immune, process. In autoimmune conditions, the body's immune system mistakenly turns against itself, attacking its own tissues. Some of the specialized cells involved in this process can attack tissues directly, while others can produce substances known as antibodies that circulate in the blood and carry out the attack. Antibodies produced against the body's own tissues are known as autoantibodies.
Treatment with medications that suppress the activities of the immune system and/or reduce inflammation of tissues has been the most common approach to autoimmune disease for more than 30 years. Many new immunosuppressants have become available since the 1960s, but all the medications used to treat autoimmune disease have serious side effects when taken in high doses for months or years.
In the 1970s, with the support of the Muscular Dystrophy Association, researchers developed a new approach to the treatment of autoimmune conditions. Instead of trying to change the immune system with medication alone, they thought that it might be possible to mechanically remove autoantibodies from the bloodstream in a process similar to that used in an "artificial kidney," or dialysis, treatment. The procedure became known as plasmapheresis, meaning plasma separation. It's also known as plasma exchange.
Medications that suppress the immune system or reduce inflammation are often combined with plasmapheresis, but they can usually be given in lower doses than when used alone.
Today, plasmapheresis is widely accepted for the treatment of myasthenia gravis, Lambert-Eaton syndrome, Guillain-Barré syndrome and chronic demyelinating polyneuropathy. Its effectiveness in other conditions, such as multiple sclerosis, polymyositis and dermatomyositis, is not as well established.
What is plasmapheresis?
Plasmapheresis is a process in which the fluid part of the blood, called plasma, is removed from blood cells by a device known as a cell separator. The separator works either by spinning the blood at high speed to separate the cells from the fluid or by passing the blood through a membrane with pores so small that only the fluid part of the blood can pass through. The cells are returned to the person undergoing treatment, while the plasma, which contains the antibodies, is discarded and replaced with other fluids. Medication to keep the blood from clotting (an anticoagulant) is given through a vein during the procedure.
What's involved in a plasmapheresis treatment?
A plasmapheresis treatment takes several hours and can be done on an outpatient basis. It can be uncomfortable but is normally not painful. The number of treatments needed varies greatly depending on the particular disease and the person's general condition. An average course of plasma exchanges is six to 10 treatments over two to 10 weeks. In some centers, treatments are performed once a week, while in others, more than one weekly treatment is done.
http://www.mda.org/publications/images/fa-plas-photo.jpg
Plasmapheresis removes the fluid part of the blood, the plasma, from blood cells. The cells are returned to the person undergoing treatment, while the plasma is discarded. The procedure takes several hours and can be uncomfortable, although it is normally not painful.
A person undergoing plasmapheresis can lie in bed or sit in a reclining chair. A small, thin tube (catheter) is placed in a large vein, usually the one in the crook of the arm, and another tube is placed in the opposite hand or foot (so that at least one arm can move freely during the procedure). Blood is taken to the separator from one tube, while the separated blood cells, combined with replacement fluids, are returned to the patient through the other tube.
The amount of blood outside the body at any one time is much less than the amount ordinarily donated in a blood bank.
Are there risks associated with plasmapheresis?
Yes, but most can be controlled. Any unusual symptoms should be immediately reported to the doctor or the person in charge of the procedure. Symptoms that may seem trivial sometimes herald the onset of a serious complication.
The most common problem is a drop in blood pressure, which can be experienced as faintness, dizziness, blurred vision, coldness, sweating or abdominal cramps. A drop in blood pressure is remedied by lowering the patient's head, raising the legs and giving intravenous fluid.
Bleeding can occasionally occur because of the medications used to keep the blood from clotting during the procedure. Some of these medications can cause other adverse reactions, which begin with tingling around the mouth or in the limbs, muscle cramps or a metallic taste in the mouth. If allowed to progress, these reactions can lead to an irregular heartbeat or seizures.
An allergic reaction to the solutions used to replace the plasma or to the sterilizing agents used for the tubing can be a true emergency. This type of reaction usually begins with itching, wheezing or a rash. The plasma exchange must be stopped and the person treated with intravenous medications.
Excessive suppression of the immune system can temporarily occur with plasmapheresis, since the procedure isn't selective about which antibodies it removes. In time, the body can replenish its supply of needed antibodies, but some physicians give these intravenously after each plasmapheresis treatment. Outpatients may have to take special precautions against infection.
Medication dosages need careful observation and adjustment in people being treated with plasmapheresis because some drugs can be removed from the blood or changed by the procedure.
How long does it take to see improvement?
Improvement can sometimes occur within days, especially in myasthenia gravis. In other conditions, especially where there is extensive tissue damage, improvement is slower but can still occur within weeks.
Does MDA pay for plasmapheresis?
MDA supported pioneering research to develop plasmapheresis. However, payment for this procedure is not among the many services included in MDA's program. A number of health insurance plans do cover the procedure.
Where are plasmapheresis treatments offered?
Plasmapheresis is performed at many major medical centers across the country. MDA clinic directors can offer advice about the availability of this treatment and its use for specific conditions.

Jack
03-26-2009, 07:21 AM
Unfortunately in England someone with WG isn't fit to adopt a child. Not where i live anyway but i know we are sometimes a bit behind the times, other areas may not have come across this problem. I didn't lose my fertility until (they think) my 3rd cycle of Cyclophosphamide which i had over a few years. I was in Great Ormond Street hospital for a year before i was diagnosed with WG. I had been in quarantine at my local hospital 3 months prior. I hope things have changed since my day!
Not sure where in England you live Lotte, but due to problems caused by chemo, I have one adopted daughter and the other by doner. I had no problems at all with the process and the officials involved thought it was a great outcome.

Grandma2Katie
03-27-2009, 06:08 AM
Doug,

Thank you for the information on plamapheresis. Most helpful.

Cindy

andrew
03-27-2009, 06:22 AM
Hey Cindy, is there any update on Katie for us?

Doug
03-27-2009, 07:34 AM
Doug,

Thank you for the information on plamapheresis. Most helpful.

Cindy

Cindy, you are welcome. It sounds like it may have helped Katie if they are looking toward taking her out of the ICU! Your family must be very grateful to the medical team who brought her to this point! We all can agree on that!

Grandma2Katie
03-27-2009, 09:07 AM
No update yet. Called Laura but she hasn't returned the call. Will let you know ASAP!!

Cindy

Grandma2Katie
03-27-2009, 03:46 PM
I got a phone call this evening from Katie! She sounds much better. The staff had to threaten her with a feeding tube down her throat to get her to eat but that is not unusual for Katie. She has never been a good eater. Today she had chicken and corn. A big improvement. The x-rays of her lungs showed improvement.

I talked to Laura after I spoke with Katie. That is when I got very concerned. There was good news; lungs improved and kidneys haven't sustained too much damage. BUT, even after the biopsy of the kidney, these people can't nailed down a firm diagnosis. The doctors said it didn't matter, the treatment would be the same. They will give Katie 6 chemo treatments and 6 plasmaparesis treatments. They intend to send her home with no cytoxan. I don't know about the predinose but her dosage was lowered today.

What is the deal here? Do these doctors have a clue what they are doing or am I over reacting?

Cindy :) :mad: :confused:

Doug
03-27-2009, 04:15 PM
Doctors need objective evidence before they treat or change treatment. It's one of the frustrations of dealing with doctors, but it helps to observe for yourself that improvements are happening: Katie's getting better!

Each Weggie, as you've probably discovered reading our many stories, has treatment experiences in common with other Weggies and other treatment experiences that are very different.

I hope these differences in treatment suggest questions you can pose to Katie's doctors.

jola57
03-27-2009, 05:32 PM
Hello Cindy, I am so glad that Katie is responding to treatment so well. I do know the frustration that doc's feel when diagnosis is not as simple as 1, 2, 3. Autoimmune diseases are so similar yet affect different parts of the body and other illnesses have same symptoms. Did they do a biopsy? Lung or kidney at this point would be the best choice. Biopsy would definitively show granulomas and confirm WG. The standard Treatment for WG is helping Katie so lets hope that that is all it is.
Warm hugs to you and family and especially Kaite

andrew
03-27-2009, 07:30 PM
My guess is that they are not giving her Cytoxan to take away because the chemo treatments sche's scheduled for will do the job. Might be too big a hit of chemo for such a young kid if she were to be taking Cytoxan pills as well.

As far as the Pred is concerned that will probably stay for a while and be gradually reduced over some time. What dosage is she on now? As long as the treatment is working, that's a good thing.

The other thing my docs thought I had was Goodpastures syndrome but WG won the day. Both are the same treatment anyhoo. I hope they nail down a diagnosis soon if at least for something to call it!

Jack
03-27-2009, 09:46 PM
The vagueness of the diagnosis is not at all uncommon and tends to be made on the basis of probability. ie. If the results of 3 tests are positive and 2 negative then the probability of disease X = so many % It is not the exact science that you would expect it to be! :)
Sounds like the treatment is working, so I would say that was good enough for now.

Sangye
03-27-2009, 11:28 PM
I agree with what everyone has said. There are a number of rare autoimmune diseases with overlapping symptoms, all with the exact same treatment. This isn't uncommon, and is more of an intellectual difficulty than an actual medical one. Many tests (blood, biopsy) become more clear as the treatment progresses.

It sounds like they're doing IV chemo? So she wouldn't be leaving with that. And, she did have plasmapheresis, which can be very effective at putting the brakes on. It's also normal to begin decreasing the pred even though she's still very sick. You can't stay on the very high doses for long. I was only at 1,000 mg IV pred (the highest dose there is) for 3 days.

Having said ALL that (!!!) if her parents have any intuitive feeling that something's not right, they should insist on a VF consultation, or get Katie to a Vasculitis Center. We can get caught up in the info and tune out our intuition. And heck, even if they don't feel something's askew, asking for her docs to consult with a VF specialist would not be out of line.

Grandma2Katie
03-28-2009, 10:34 AM
Hi Everyone,

Report today was good. Katie is out of ICU. She had her second plasmaparesis treatment today. Also, a definite diagnosis of WG. I am still a bit unsettled about the home treatment and will discuss this with Laura and David.

All in all though, things are greatly improved.

Cindy

Doug
03-28-2009, 11:49 AM
I know children even younger than Katie can develop WG, but I've never had contact with any child with it, regardless of age. I hope you can maintain your Vasculitis Foundation contacts for advice. I hope you have gained sufficient guidance from this site, too, so that you feel a little more expert and a little more prepared for Katie's home challenges. Your postings will be great information for the next family member with a child with WG: it's new territory for this site, as far as I can tell, and the running narrative of Katie's admittance to the hospital through today, even, is a remarkable document. Have you had time to go back through it yet? Whew! Sometimes I forget that we are English, Canadian, Australian, and American on this site. That's a significant part of the English-speaking world. We can do a lot of good for Weggies around the world.

andrew
03-28-2009, 11:53 AM
Well said, Doug. This will be a great journal for future parents/relatives of kids with the WG.

Grandma2Katie
03-28-2009, 12:40 PM
I wouldn't have made it through this week without you guys. You have been great and have shared your stories and information so freely. Thank you from all of the Linders and Cravens.

Cindy

Grandma2Katie
03-28-2009, 12:50 PM
Just wanted you all to know who Katie is. This is a picture of the whole family. Katie is the older and taller of the two girls. Her sister is Roxanne, 5 going on 25. A real catbird! That's Laura and David with the girls.

Cindy

Doug
03-28-2009, 01:03 PM
Thanks for sharing!

(p.s. Pardon me for duplicating my thoughts, but I've been fighting Internet connectivity problems all day. I thought the remarks above were lost. Turns out I was just in a different part of the site trying to duplicate what I thought was lost.)

Doug
03-28-2009, 01:21 PM
I just remembered another thing about the drug effects: food tastes a lot different. Maybe people still dealing with Cytoxin/Prednisone can amplify on this thought. After months of no appetite, then five weeks of medical treatments, I returned back to Nebraska with a voracious appetite. I decided to celebrate my return by ordering my favorite food, a taco pizza (don't ask! haw!). I barely made it through a small piece: it had a horrible taste! The next day, we had something for supper that I'd ordinarily pour lots of catsup on: catsup was my favorite condiments. Ugh! Another taste disaster. It turned out, after lots of careful eating over the next few days, that anything with tomato or vinegar in it just did not work for me! On a happy, if impractical note, chocolate and sweet tastes were just fine. Fruit and plain vegetables tasted OK. I still don't eat a catsup on much of anything any more (a good thing!). Most foods started to taste ok, if dull without herbs and pepper, after I stopped Prednisone, if I remember right, though the regain was slow.

If Katie was a fussy eater before, there is a good chance she will have new food issues after she comes home. Be patient with her if she complains about taste.

crackers
03-28-2009, 01:23 PM
great news cindy.things are getting better but at the same time things are only just starting.make sure that they are monitoring her condition,and her meds, on a regular basis.wishing you all well.
john.

Sangye
03-28-2009, 01:27 PM
I'm trying to remember turning down any food--I can't think of anything! I ate everything. I didn't know for several weeks it was the pred. I thought it was the chemo requiring me to have more food in me so I wouldn't feel nauseous or something.

My first docs were so bad that way. Can you believe they never told me it makes you eat like a fiend?? I didn't have internet access, so I didn't find out for months!

Doug
03-28-2009, 01:44 PM
Sangye-

That's right! I forgot about that eating like a horse! Do you suppose it was the prednisone that made food taste bad? All I know was I thought the hospital food tasted like it came from a three-star resterant. Yeah, sure!

andrew
03-28-2009, 01:51 PM
Ha! When I was on the Pred I don't think food was in my mouth long enough for me to taste it. I sucked down anything that wasn't nailed down :D Absolute food-vaccum. No recollection of taste being affected.

Grandma2Katie
03-28-2009, 05:51 PM
Thanks Doug for the food tip. I will pass that along to Laura. Katie thinks ALL foods should be very sweet. Maybe we will get lucky and she will crave chicken and veggies!

Cindy

Grandma2Katie
03-28-2009, 05:55 PM
John, I am concerned about Katie's regimen when she goes home. She lives 4 hours away from the hospital and doctors. How often should she be checked once she gets home?

Cindy

Doug
03-28-2009, 06:31 PM
Chocolate rabbits are available again! Haw!

Jack
03-28-2009, 08:23 PM
Revisits -
So much depends upon how she is doing and how stable she is. At first, I think I was on weekly visits, but this dropped as my condition became better and for most of the time I have been on 3 months. However, on more than one occasion, I had to ring up because I was unwell and when I went in to be seen, they would not let me go home again! :mad:

Luce
03-28-2009, 09:45 PM
I'm six months down the line from diagnosis and am still been seen every 2-3 weeks with weekly blood tests in between.
I believe it was the oral chemo pills that made food taste funny, I went off so many of my favourite foods and picked up some cravings for things I'd never enjoyed before. For example I swapped coca cola for tea!!
When I came off them most of my normal preferences returned but I still don't like coke and I've just started the pills again so will report any oddities.

Doug
03-29-2009, 01:08 AM
Cindy, I don't know how the hospital care is organized back there.

Here, I started out at a local general hospital. The local hospital has an affiliation with a regional hospital, 58 miles away, where there are a variety of specialists (including the pulmonologist I still go to). The two hospitals "share" the specialists through a specialty clinic at the local hospital. The specialists each come up twice a month, in rotation, on different days, so one can see specialists locally five days a week.

For a while, in the early stages of my illness, I was going in to either the specialty clinic at my local general hospital to see my pulmonologist or having to go to his office at the regional hospital for check ups. Either way, he was in consultation with the rheumatologists in Denver, who were satisfied he had control of my care, based on reported test results.

I only had to go back to Denver once (2005) to the University Hospital to see the rheumatologist who managed my care there in 2004. My Denver rheumatologist after a very thorough examination (2 hours!), was very happy with my progress and said he wouldn't need to see me again if I continued to heal the way I was.

Other than the cANCA test (which I have on a six-month schedule or if I have symptoms suggesting a flare, as needed), the lab at my local hospital does my lab work and reports it to my pulmonologist. Usually I get that lab work done the same day I see him here so that's part of my regular lab work routine. I'm on a six-month schedule for that now, too, unless I have reason to think I might be having a flare. I thought I was having a flare a bit over a year ago. It turned out to be a false alarm, but it happened a month earlier than schedule for my routine cANCA test. That reset my routine appointment schedule by a month, but that still is a six month thing, mostly done at my local hospital.

Katie's doctors will determine how often and where she needs to go for check-ups. Does she have one doctor you've identified as her primary care doctor, say the first specialist she saw who ultimately sent her to the hospital four hours away from home? That will probably be the doctor who manages her maintenance care. If that doctor is at the hospital four hours away, Katie's parents should ask about the possibility of managing her care through a doctor they and the specialist have confidence in who is closer to their home. Katie's progress will determine whether that is practical or safe.

jola57
03-29-2009, 01:11 AM
I have been on the chemo pills and prednisone since September, blood check is every 2 weeks, and I see rheumy every 3 months. When I was diagnosed in 2006 it was the same but I had so many questions about symptoms that a I just picked up the phone and went to to the docs any time I wanted. Its the prednisone that makes you eat like a vacuum. From comfortable 155 lb at 5'7" to 215 lb. Yah Andrew its like everything thats not nailed down just goes whoosh and yam yam. I have always been a picky eater, now EVERYTHING tastes great and yummy. Its not enough to have one sandwich or one slice of pizza, its 3 or 4.
As I remember it took a couple of months to get that hunger, it wasn't right away. Chemo did not make me sick or put me off my food, and I didn't lose any hair but it curled - I call it my special perk.

coffeelover
03-29-2009, 01:39 AM
I agree with Jolanta. I did not feel the extreme hunger ( in fact I was was the opposite) for about 2 months....but then, when it hit...IT HIT! Now I am constantly struggling with my hunger and what I put in my mouth.
I am keeping the anti inflam foods in the house so if I do reach for something it hopefully will not be junk. It is a constant battle and one I intend to win!
Lisa Coffeelover

Doug
03-29-2009, 02:21 AM
Yeah, I wish someone like Sangye had been around to advise me when I was at that stage. There is something to be said for the holistic approach to healing. And eating to be healed!

While I wasn't very open to that approach in the earlier stages of my illness, I realize that Western medicine used to pooh-pooh acupuncture, chiropracty, and other "nontraditional" (in the West) herbal medicines only to find they have their place, work in a different way perhaps, and people benefit from those methods of healing.

Until two days ago, the director of my local hospital wasn't an MD but a DO (doctor of osteopathy).

Grandma2Katie
03-29-2009, 02:25 PM
Katie is feeling so much better that she has been able to go to the Ronald McDonald playroom for a while.

Ok, I got it wrong. Katie will only receive one cytoxan treatment while she is in the hospital. She will have six plasmaparesis treatments. Her kidneys are still not responding as the doctors would like but at least they are working.

Laura, my daughter, actually went home today so she could get some uninterrupted sleep. I know she is exhausted.

The hospital is only 2 hours away from their home. (My mistake! :o) For me, it would be 3 hours but then I drive the speed limit. It remains to be determined what will need to be done once Katie is sent home. I hope her original doctor in Charlotte can handle the blood tests but if not, she will be taken to Winston Salem.

If you wouldn't mind, someone point me toward the healing food list. I'm sure it is posted here somewhere thanks to Sangye.

I hope you all are having a great and healthy weekend.

Cindy

Doug
03-29-2009, 02:37 PM
Sangye is the most knowledgeable about that. I'm the one who thinks taco pizza is "food"! Haw! :D

andrew
03-29-2009, 07:48 PM
If you wouldn't mind, someone point me toward the healing food list. I'm sure it is posted here somewhere thanks to Sangye.

Very happy that Katie is bouncing back so well, despite the issues with the kidneys.

Is this the post you were referring to?

http://www.wegeners-granulomatosis.com/forum/weggies-stories/191-wondering-5.html#post1401

I'm almost positive Sangye also had something on the topic but I can't find it.

Sangye
03-30-2009, 12:24 AM
Cindy, any book on "Anti-inflammatory" diet is great. Andrew Weil's stuff is probably the easiest to use, since it's aimed at realistic life changes and cooking for families, etc... His website has a ton of info.

And just in case....Tell Laura not to give Katie herbs or nutritional supplements without a holistic doc involved. Even benign remedies can be quite dangerous to Weggies for a few reasons.

I might not be posting much the next few days. We've had a death at our temple, and it's impacting me a lot.

Grandma2Katie
03-30-2009, 10:45 AM
Sangye, so sorry for your loss. Thanks for taking the time to answer my question.

Blessings,
Cindy

Doug
03-30-2009, 10:47 AM
...me too.

Doug

crackers
03-30-2009, 12:28 PM
my thoughts are with you sangye.
john

jola57
03-30-2009, 04:24 PM
My thoughts are also with you Sangye,

andrew
03-30-2009, 06:56 PM
Ditto as well

Carol
04-03-2009, 10:54 AM
I'm always surprised on this forum to see how many people have biopsys done to see if they have this disease. When I had big purply black lesions on my hand they did a biopsy - cut a cirlce of tissue out of the middle of it and it came back negative. The rheumatolist worked it out without any further biopsys, with the anca and the white spots of my lungs. So the question is are all those traumatic biopsys necessary?

Sangye
04-03-2009, 11:26 AM
A positive biopsy is needed to confirm the diagnosis. If symptoms are obvious and you respond to treatment, they sometimes don't pursue a biopsy. Biopsies can be false-negative.

Cindy--Any news about Katie?

cwcindylou
04-09-2009, 04:51 AM
Is there anymore news on Katie?

Grandma2Katie
04-12-2009, 02:05 PM
Good news everyone. Katie got to go home on Thursday!! She has 10 prescriptions and instructions to be back on Monday for more tests but she made it home for now. Her lungs are clear. Her kidneys are not what the doctors were hoping for but they are good enough so that she will not need dialysis for now. Her BUN level had to come down before the doctors would let her leave. Things are looking better. Hopefully, Monday's tests will be good and she can return home.

BTW, Grandma has been down with an arthritis flare. I got out of bed Monday morning and by lunchtime I could barely walk. Saw the doctor on Wednesday. Pain meds and anti-inflamatory helped until I had a reaction to the anti-inflamtory. Had to stop that med. Hopefully, I won't regress too much before Monday.

Hope you all are feeling great!

Cindy

Doug
04-12-2009, 02:35 PM
A tip of the hat to the team that brought her home! Get some sleep, now, Cindy!:):):):) (Again thwarted by the four emoticon rule- this is a 5-smiley bit of news!)

crackers
04-12-2009, 05:23 PM
great news cindy.please keep popping in to see us all and to let us know how she is doing.
john.

pberggren1
04-12-2009, 06:33 PM
Stress can bring on any illnes, especially these pesky autoimmune ones. Doug is right Cindy, Get sleep now. And de-stress. Easier said than done of course.

Sangye
04-12-2009, 11:06 PM
Yay Katie!!! Once she's home, you won't believe how much better she'll get. Something about being in a hospital makes you lose sight of how much you can actually do.

You take good care of yourself now, okay Cindy? Get something that works well for you, and if it's a pharmaceutical antiinflammatory (even OTC) always take it with a very full stomach.

jola57
04-13-2009, 03:51 AM
Wonderful to have Katie home, now is the time to relax and get rid of the tension. Agree with Sangye, take OTC drugs but eat plenty of food with them.

Luce
04-13-2009, 05:50 AM
Sangye, I am always amazed at your level of knowledge - a brilliant resource for everyone and I applaud you for being so willing to share your wisdom!

Yay Katie, so glad she's home!

Doug
04-13-2009, 05:56 AM
YouTube - Bunnies, 1 (http://www.youtube.com/watch?v=mRwZgVwn5SI)

http://www.youtube.com/watch?v=_DVmqx__BEg

This one's for you, Luce! (I'm not in it....)
This two's for you, too, Luce!

Luce
04-13-2009, 06:36 AM
Doug, thank you for the links but YouTube is telling me those videos aren't available in my country!

Doug
04-13-2009, 08:33 AM
Too bad! They had to do with rabbits as pets, and how they are as intelligent as cats and dogs, etc.

Sangye
04-13-2009, 09:16 AM
Thanks, Luce (4 comments ago)-- I had excellent training as a biologist and then as a chiropractor. Everything I have to share is due to all my teachers.

marian
06-24-2009, 12:18 AM
Hello everyone

This looks like an appropriate thread for me. My grandson was diagnosed last August at the age of 14. He had not been feeling well for months, and various doctors had come up with various reasons: mono, whooping cough, and then pneumonia. As spring turned into summer, and he spent more time sitting on the players' bench rather than playing his usual robust games of basketball and then baseball, all the time experiencing horrible coughing fits. I knew something was far more wrong than the doctors suspected. But, it took until August for his parents to finally agree to my demand that he be admitted to a hospital until someone gave him a definite diagnosis. Even then, in the children's infectious disease ward of a big teaching hospital, most of the doctors were perplexed. By that time, my grandson was a very sick child. Finally, one resident uttered the term Wegener's. I happened to be at the doctors' station when she said it. I saw at least six doctors who had been in and out of Patrick's room look up wide-eyed. They all started racing for their various computer screens. Minutes later, after I went back into Patrick's room where I was spending the night, two doctors came in and told me they were pretty sure of a diagnosis...enough that they wanted to start him on massive doses of prednisone, along with two units of blood. They said the could not afford to wait for an official diagnosis.

Within two days, they had their test results back from the Mayo clinic, Patrick was feeling perky, and his color had returned. He was back home in three days. He was relieved that he didn't have cancer (he told me he thought he was dying of cancer), but bummed that he'd been on medication for a long time.

By late September he was playing football and this spring he was back playing baseball. However, when his hips started hurting, the doctors suggested he not do things that would be painful.

The problem with being a grandparent is that we have more time to study all the effects of the disease and the medications. The parents tend to want to not discuss things with me because they know that I worry too much.

Meanwhile, I think that Patrick has a tendency to hide some of the problems he is experiencing (both emotionally and physically) because he doesn't want to bother his parents.

Meanwhile, we've been playing golf and yesterday he laughingly told me that the lab technician botched his bloodwork yesterday.

So, he is taking it all in his stride and is living his life the way his doctors have suggested...that he's just like any other kid except he's on medication.

He has always been the sweetest kid...the youngest of three boys. His oldest brother is very carying of him, while the middle brother still beats up on him.

I want to be able to talk to Patrick to see if he has cares, concerns, fears and if he'd like to communicate with someone his own age (I have found another 14-year old). But, he seems to be doing well without anyone discussing it with him.

Thanks all for listening...i mean reading...

Marian

Jack
06-24-2009, 01:22 AM
Hi Marian,
Sorry to hear about Patrick, how is he at the moment?
I'm sure that I won't be the only one on here who will be advising you that he needs to see a specialist, most doctors have little idea about the control of this disease. What medication is he taking? Prednisolone alone will probably not be enough to maintain remision and the long term effects are not good.

marian
06-24-2009, 01:51 AM
Hi Jack

Thank you for responding. A rheumatologist at Dartmouth-Hitchock center in New Hamphsire is in charge of Patrick. He started out on prednisone and cytoxin but has been off both for several months. The doctor put him in charge of his medication and he is very careful and thorough in taking everything. He has been on methotrexate once a week ever since he stopped taking p and c. He also takes folic acid. He seems to feel quite well, except his hips started hurting during sprints in baseball practice.

I guess what I question is the difference between his parents' attitude and my own. I guess that difference stems from the fact that they are dealing with their young son, while I am looking at the disease as a whole.

They do not want anyone in town thinking of Patrick as "sick" Patrick, so nobody discusses it, although his teammates all knew that he was on medication for "something" and that his face swelled up.

I would like to open up the discussion and spread the word about Wegener's and autoimmune diseases. The disease has been very rare, but it seems to me that there is a lot more of it now. One man in town who has an autoimmune disease claims that he can make his condition flare up simply by going to the golf course and coming in contact with the chemicals that are sprayed on the greens. I have been taking Patrick and his brothers to play golf since they were little more than toddlers. Is this just a coincidence? I'd love to know more. I sometimes watch golf tournaments on TV and I shudder when I see a golfer stick his tee in his mouth!!!

I do defer to his parents' wishes and I have never been an interfering grandmother, so I mostly keep my thoughts and findings to myself. But I do want to keep Patrick and his parents as fully informed as I can about so many issues of Wegeners. I want Patrick to take it all seriously so that he will know how to handle any issue that seems out of the ordinary to him. I would like them all to attend some vasculitis conferences, but they say they will wait until Patrick is ready for such events.

By the way, his rheumatologist has one other WG patient. But she is in constant contact with specialists in Boston and Cleveland.

It's really hard to know the best way to handle the condition in such a young person.

Thanks again for your response.

Jack
06-24-2009, 07:17 AM
It all sounds like good news to me. An early diagnosis, no permanent damage and now in remission with little in the way of medication. :)

I understand your concern about his condition. You obviously know that it is potentially a very serious disease and flare ups after remission are common. So long as he is aware of this and is prepared to act at the first sign of problems, there is little more you can do. The cause of the condition is still unknown and I've never heard of anyone being able to stimulate flare ups. I've read many papers on the subject, but they all seem to rule things out rather than point at potential causes.

jola57
06-24-2009, 09:23 AM
Hello Marian, I too have never heard of flareups being stimulated by anything. The young have much more resiliance than us. It is a life long illness but one that fortunately can be handled. You are a caring grandma and one day he will know that for sure. For now I guess I would let him be just a boy, the time may come when that will end and he will need your support but for now keep his illness in confidence, he has enough teenage angst without additional baggage. Let him enjoy carefree happy days without all the baggage of having to go to meetings, symposiums and such. One day he may want to know more and if you continue to gather information for him you will be there with it when he needs it most.

marian
06-24-2009, 10:47 AM
Thank you Jack and Jola for your responses. They are good responses with good advice to me.

I spent the day with Patrick and his older brother. During the past six months, Patrick has developed from being a child a little shorter than I am, 5 ft 4 in, to being a young man as tall as his older brother (6 feet).

During this past year, I have been interested in knowing how the medications for WG affect the body during such a growth spurt. I was first afraid that he might not grow, but that fear has been allayed for good. Now, my thought rests in wondering if the medications have affected the quality of the growth. I guess time will tell.

He looks fine, but I could tell by the end of the day he was feeling very tired.

My fondest wish is for some dramatic scientific development within his lifetime that will eradicate these autoimmune diseases. I really want to tell him to go into the field himself and see what he can do. His scholastic interests are all in the scientific area.

Again, thank you both for your comments. You've been very helpful.

Doug
06-24-2009, 12:49 PM
Marion-

I think you are on track, Patrick is on track, yet continue to search for information. At some time Patrick will need to understand just how lucky he is, in some sense, to go through the early stages up to and including diagnosis with relatively little harm done to his body or life.

People die from this disease.

Many people on this forum would love to have Patrick's medical history, up to this point, because they have had to have kidney transplants after renal failure. They have endured "saddle nose" and have reconstructive surgery on their noses ahead of them. One Weggie is near death, perhaps has died (and I missed the news). Lives have been altered drastically.

A positive attitude is very important, perhaps a no-brainer for a young guy like Patrick. A realistic understanding of the disease process and the worst possible effects a Weggie can experience, too, is important. That knowledge is what helps the Weggie ask his or her doctors the right questions in a timely matter. That knowledge, too, helps the Weggie look dispassionately at new symptoms through the point of view of what is important for his or her doctor to know.

A long life is possible with WG, but your grandson needs to understand he is the new owner of a very high maintenance body, one that will rebel if he fails to address WG-related issues in a timely manner. As a Weggie, it is safe to say, every change in his health needs to be viewed by his doctor and him through the WG filter.

Sorry if I've become a bit preachy, Marion, but that's kind of what we do in this forum, a bit from each point of view, as we feel we can offer some insight or bit of knowledge that will help the family or Weggie (not patient-a Weggie is someone who has WG, but may or may not have active symptoms). Enough for now. I have visitors and am a bit distracted from this more important mission. (p.s. Every Weggie needs a family support person as actively involved as you! Good job!) :)

cwcindylou
07-02-2009, 06:56 AM
Any more news on Katie?