Cyclophosphamide

[staystronglivelong]

Hi Guys. I went with my husband to his most recent infusion of cyclophosphamide ( his 6th infusion). Its good news as the consultant said he is very pleased with his progress. :w00t: He told us that his ANCA was 84 when he was diagnosed and its now down to 25. Sounds good but personally don't really understand what that means. :ohmy: Although I did ask: If Mark was in remission would his anca be nil? And he replied not necessarily - Mark could be in remission and still have a positive anca reading. Anyway, enough waffleing. My query for you good people is: If on IV Cyclo how long were you on it? We understood that he would be having 6 Infusions - so, Marks been on it for 3 months and now they are saying he will be on it for another 3 months as its more likely he'll maintain remission than just having the initial 3 months of it. They're saying if they took him off it now he's more likely to relapse than if they give him another 3 months of cyclo. They haven't actually said hes in remission so I don't think he is yet. :sad: Also he is now on 10mg of pred. He gets headaches if he wakes in the night and thinks its when the pred has worn off - cos he takes it in the morning. Hope I've made sense. :tongue1:

[elephant]

Is he having Wegeners Symtoms( shortness of breath, crusting in the nasal/sinus, joint pain, fevers, rash) or feeling better. Your Rhuemy is right, that is what my Specialist said too about the ANCA's and mine is in the 30's and has not come down.

[Geoff]

Hi there SSLL!!,

I can only speak from my own experience with cyclo about a year ago when I 'flared' (ANCA went to 170) I had 4 infusions then they stopped although like you, a total of 6 visits was mooted. I slowly managed to drop my ANCA although its stuck about the 80 mark and just bubbles up and down! Did get pred down to 5mg about 3 months ago but back up to 20 at the moment. Perhaps if I had continued to go for the 6 infussions it might have worked out better. Having said that I trust my doc to make the right calls...he introduced me to the word recalcitrant to describe my ANCA. when alls said and done ANCA values are just part of the overall diagnosis but it sounds as if Mark is going along nicely!

[Jack]

I think that 6 months is not at all unusual and I would agree that it is best to stay on it until Wegs activity is really low. On the other hand, it is best not to take it for more than 6 months due to the increasing risk of side effects. 12 months should be considered to be a maximum.

[Sangye]

They should not be making a treatment decision based on the ANCA. It's been discredited as being an accurate indicator of disease activity and is primarily used in initial diagnosis. Some people are in remission with sky-high ANCA's, and some have active Wegs with low or (-) ANCA's.

They should be basing their decisions on his inflammatory markers (ESR and CRP), kidney function (if he has kidney involvement), imaging (eg lung CTs showing resolving hemorrhaging and/or nodules) and resolving signs/ symptoms.

The length of time they use the ctx is as Jack described.

[staystronglivelong]

Is he having Wegeners Symtoms( shortness of breath, crusting in the nasal/sinus, joint pain, fevers, rash) or feeling better. Your Rhuemy is right, that is what my Specialist said too about the ANCA's and mine is in the 30's and has not come down.

Hi Elephant. Are you in remission? He's not having wegeners symptoms. He always feels abit unwell after the pred has been lowered - the consultant says that is pred withdrawal. He's not had any bad side effects from the cytoxan. He feels tired the next day and gets tummyache. But four days after the last IV Cyclo he was very unwell with constant being sick and diarhoea all day - he couldn't keep anything down not even water and was red hot. He's usually fine and I never know whats ok and whats not because they say take him to hosp (out of hours) if his temp hits 38 or more or if he's got an infection. He's got a helpline number but they don't listen to it until 11 am every day. Then they don't ring you back. We've got no way of contacting his Rhuemy, only his secretary - until 3.30 every day. So, I was quite worried about how ill he was. I rang our GP, who rang back and checked that his medication would of stayed in him which it would of and said he doesn't think its a side effect of the chemo - he thought it was a bug. I was thinking it might be a side effect of the accumulated chemo but I guess we'll never know. He was ok next day - just a very tender tummy. How long have you had wegeners elephant?

[staystronglivelong]

Hi there SSLL!!,

I can only speak from my own experience with cyclo about a year ago when I 'flared' (ANCA went to 170) I had 4 infusions then they stopped although like you, a total of 6 visits was mooted. I slowly managed to drop my ANCA although its stuck about the 80 mark and just bubbles up and down! Did get pred down to 5mg about 3 months ago but back up to 20 at the moment. Perhaps if I had continued to go for the 6 infussions it might have worked out better. Having said that I trust my doc to make the right calls...he introduced me to the word recalcitrant to describe my ANCA. when alls said and done ANCA values are just part of the overall diagnosis but it sounds as if Mark is going along nicely!

Thanks for your reply Geoff :biggrin1: I would say recalcitrant could be used to describe WG ! :thumbsup: Are you in remission at the moment? I just worry about him getting more chemo than is necessary obviously because of the long term health issues. One evil to cure another is what I say. :rolleyes1: I perhaps shouldn't question what the Rheumy says but its because of all the info I read on the net. I do think Mark's Rheumy is to be trusted - been told he's a specialist in WG. Last time Mark went for his Cytoxan there was a couple from Derby there - the lady has Wegs and had been referred to Mark's Rheumy because he's meant to be good - not saying he isn't. He was impressed with my list of questions I asked him and he gave me very good answers.

[staystronglivelong]

I think that 6 months is not at all unusual and I would agree that it is best to stay on it until Wegs activity is really low. On the other hand, it is best not to take it for more than 6 months due to the increasing risk of side effects. 12 months should be considered to be a maximum.

Hi Jack. My minds happier now having heard you say 6 months is not unusual. I'm not questioning Mark's Rheumy ( not to his face ), just curious, I think we misunderstood when it was initally explained to us we were thinking 6 infusions not 6 months. Mark has complete faith in him so thats good. And Mark is doing everso well. :biggrin1: I am compiling a list of questions for Mark's next visit! Like Mark says, if he thinks of anything he wants to ask when he gets there and sits down he can never think of it then!

[elephant]

Hi Staystronglivelong, Yes I am in remission! I started out with symtoms of WG around 12 ( red eye, joint pain, bronchitis symtoms), I am 45...so abouat 32 years. I was not diagnosed until 2008. Just keep an eye on with prednisone withdrawl, it should only last a few days. Are they doing anything for Marks diarrhea?

[staystronglivelong]

Hi Staystronglivelong, Yes I am in remission! I started out with symtoms of WG around 12 ( red eye, joint pain, bronchitis symtoms), I am 45...so abouat 32 years. I was not diagnosed until 2008. Just keep an eye on with prednisone withdrawl, it should only last a few days. Are they doing anything for Marks diarrhea?

He's ok thanks, he only had the diarrhea for 1 day. Blimey, you were only diagnosed 2 years ago. I hate Wegeners Granulomatosis and it breaks my heart when I read about children having it. The Pred withdrawal symptoms only last a few days like you say. Hope you stay in remission forever. :biggrin1:

[staystronglivelong]

They should not be making a treatment decision based on the ANCA. It's been discredited as being an accurate indicator of disease activity and is primarily used in initial diagnosis. Some people are in remission with sky-high ANCA's, and some have active Wegs with low or (-) ANCA's.

They should be basing their decisions on his inflammatory markers (ESR and CRP), kidney function (if he has kidney involvement), imaging (eg lung CTs showing resolving hemorrhaging and/or nodules) and resolving signs/ symptoms.

The length of time they use the ctx is as Jack described.

Hi Sangye. My way of explaining things is confusing. I don't have waffle on :razz: Sorry about that.

Yes I'm sure they are not basing there decision on the ANCA - they know what they are doing.

[Sangye]

LOL-- I'm glad his doc is a Wegs specialist.

[mickoko]

Hi staystronglivelong,
Just to let you know i was diagnoised with WG 10 years ago and had 13 infusions of cyclophosphamide at doncaster royal infirmary in south yorkshire, i was told i was to have about 8 infusions but they decided to give me more in the end.
All i can say is it was bad, (all day jobs) but was well worth it in the end. i am now as my doctor says, " back to normal for you".

[staystronglivelong]

Hi staystronglivelong,
Just to let you know i was diagnoised with WG 10 years ago and had 13 infusions of cyclophosphamide at doncaster royal infirmary in south yorkshire, i was told i was to have about 8 infusions but they decided to give me more in the end.
All i can say is it was bad, (all day jobs) but was well worth it in the end. i am now as my doctor says, " back to normal for you".

Hi Mickoko.
Thanks for your reply. Are you in remission?
Staystronglivelong.:biggrin1:

[mickoko]

Yes i`m in remission and have been for a few years, but the years of other tablets and medicenes have took there toll. I have bronchiectasis, which at the moment is nearly as bad as WG (coughing and spluttering, bad breathing and breatlessness ), problems with nerve damage in my feet and just generally falling to bits. But don`t let that worry you, i had a bad attack of WG, and I get on with day to day living just like everyone else. All i can recomend is live for every day and try and have a smile on your face every day.:hug3:

[mickoko]

I also take mycrophenolate everyday for the WG and also amlodipine, allopurinol, losaprin and atenolol for other things, and a prednisalone injection for gout about 2-3 times a year. Inhaler and nasal spray for the Bronchiectasis also.

[Jack]

Hi fellow Bronchiectasis sufferer. :)

What have your specialists said about this condition? I associate mine with the Wegener's and reading all the info on the web this would seem to fit, but the lung people disagree and I can find very little published material that links them. As you say, the symptoms can be pretty bad with constant coughing and lung congestion in spite of the inhalers.

[mickoko]

Hi Jack,
The speciaist for the Bronchiectasis says it was caused (he thinks), when my lungs collapsed when I had my big flare up. But my WG specialist, who personally I think is brilliant, thinks it could have been caused then but is not too sure. I don`t think they really can confirm this or not. My X-rays and scans years ago showed not much damage but it has got worse as the years have gone on. Either way I just get on with it, just another doctor to see every 2 months, and now I think I know everybody personally in both the hospitals I go to, Nurses, Doctors and even the canteen staff too.

[barry2010]

Hi staystronglivelong Im Barry I was diagnosed 4 years ago had 10 infusions of cyclo was only supposed to be 6.I went in to remission a little over a year ago and am pred free now .

[Jack]

The link between Wegener's and Bronchiectasis seems obvious to me, but not to the specialists. Either I am missing something or they are being deflected because there is no recognised connection published.
Everything I read says that Bronch. can be caused by severe lung infections and we all know how good Wegs is at mimicking infection and how it can attack the lungs. I really don't believe in the coincidence of having two major conditions that are unrelated. I can't be that unlucky!

[mickoko]

Totally agree with you jack. It seems obvious to me too, but like you, when you ask any of them, they are not sure. I too don`t believe in that much of a coincidence that we are both that unlucky, but you never know.

[me2]

I was curious what you guys were talking about so I looked it up on Wikipedia. Below you find a clip from Wikipedia. If Bronchiectasis can be a complication of RA , Crohns, and AIDS then why not another less studied autoimmune disorder? How about another more general theory- it sounds to me like that things that can damage your lungs (like WG) may indirectly cause bronchiectasis. But don't listen to me , I'm not a doc.



Acquired causes
Acquired Immune Deficiency Syndrome (AIDS (http://en.wikipedia.org/wiki/AIDS)) is the leading cause of bronchiectasis, especially in children. AIDS predisposes patients to a variety of pulmonary ailments, such as pneumonia (http://en.wikipedia.org/wiki/Pneumonia) and other opportunistic infections (http://en.wikipedia.org/wiki/Opportunistic_infections).[3] (http://en.wikipedia.org/wiki/Bronchiectasis#cite_note-2)
Tuberculosis (http://en.wikipedia.org/wiki/Tuberculosis) is another major cause. Endobronchial tuberculosis (http://en.wikipedia.org/w/index.php?title=Endobronchial_tuberculosis&action=edit&redlink=1) commonly leads to bronchiectasis, either from bronchial stenosis (http://en.wikipedia.org/wiki/Stenosis) or secondary traction (http://en.wikipedia.org/wiki/Traction_bronchiectasis) from fibrosis.[4] (http://en.wikipedia.org/wiki/Bronchiectasis#cite_note-3)
Bronchiectasis can sometimes be an unusual complication of inflammatory bowel disease (http://en.wikipedia.org/wiki/Inflammatory_bowel_disease), especially ulcerative colitis (http://en.wikipedia.org/wiki/Ulcerative_colitis). It can occur in Crohn's disease (http://en.wikipedia.org/wiki/Crohn%27s_disease) as well, but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungus (http://en.wikipedia.org/wiki/Fungus) spores (http://en.wikipedia.org/wiki/Spores).[5] (http://en.wikipedia.org/wiki/Bronchiectasis#cite_note-4)
Recent evidence has shown an increased risk of bronchiectasis in patients with rheumatoid arthritis (http://en.wikipedia.org/wiki/Rheumatoid_arthritis) who smoke. One study stated a tenfold increased prevalence (http://en.wikipedia.org/wiki/Prevalence) of the disease in this cohort.[6] (http://en.wikipedia.org/wiki/Bronchiectasis#cite_note-5) Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.

[staystronglivelong]

Hi staystronglivelong Im Barry I was diagnosed 4 years ago had 10 infusions of cyclo was only supposed to be 6.I went in to remission a little over a year ago and am pred free now .

Hi Barry. Thats great news that you are in remission and pred free. Long may it continue. :thumbsup: Yes, that sounds right, same as my husband - supposed to be 6 but he will be having 10.