What to be worried about, what questions to ask?

[different]

I'll try to make this concise with primarily relevant details.

Well my dad is currently in the ICU because he felt extreme weakness and was coughing up blood. He was diagnosed with Wegeners and started treatment for it about 8 months ago, but it has been causing problems since about 12 months ago with his sinuses and a thing in his lung. He was taken cytoxan and prednisone for treatment, things were looking good so they lowered the prednisone, but his anca and sedimentation rate were not improving, but he felt fine. The doctor wanted to start him on Rituxan because those levels were not perfect. The doctor wanted to wait some amount of time between treatments and due to some miscommunication he was untreated for 5 weeks no Rituxan or Cytoxan, but still on 20 mg of prednisone, once a day. On the 5th week his Wegeners flared up with coughing up blood, his blood count went down to 8.4 he was admitted to the ICU but was released 4(thursday morning till sunday afternoon) days later since his blood count went up to 8.5, but still coughing up blood. 2 days later(the following wednesday morning) he felt extreme weakness and told us to call 911, in the emergency room they found out he was only getting 40% oxygen level, they thought it was pneumonia at first, the scaring in his lungs spread to both sides, and his blood count went down to 6.5. They intubated him, gave him a blood transfusion and started plasmapheresis, with high doses of steroids(60 mg of prednisone x4 a day), along with a Rituxan treatment once a week, also gave him a lot of sedatives to keep him calm? His kidneys are doing fine, and I believe there was never any sign of kidney failure.

It's been 8 days, his blood count is going up at 9.8 currently. There are no longer any, or very little signs of his lungs bleeding. He's been off the sedatives for 36 hours and still not waking up, they gave him a diuretic to get rid of accumulated fluids in his body. He has been going under plasmapheresis every day, they are switching to every other day now. I just learned today that plasmaphersis is considered a "last resort treatment". So I am a bit more concerned. From my knowledge and searching the past 8 months, most people die of wegeners because of "renal failure", and his kidney's are fine.

Some other things is he naturally has high blood pressure. So they work on controlling that as well. The machine is giving him 35% O2(down from 65% when he first was admitted, normal air is about 21% oxygen) and peep level is at 5% down from 10%. Not sure on other important factors about his tube machine. Looking at the actual measurement they might increase it back up to 40% or 6 peep level, since it's sticking around 90-93%. I'm leaving out his age since I don't want agest results. Our family has decent genes which cause our organs to be younger and perhaps stronger than others, also he doesn't smoke or any other risky behaviors. Last joke a nurse told him was "your heart sounds good, you have the heart of a teenager".

So I ask you guys, what questions should I ask the doctor? What results or numbers should I be worried about?

[annekat]

Welcome to the forum. Others can probably do a better job of answering all your questions, but you've come to the right place. We have lost a couple of forum members due to other reasons than renal failure, such as unusual, opportunistic infections that can take hold in a person whose immune system is being suppressed by our standard WG meds. But that is rare. And people can develop kidney involvement later, but if it is caught and treated in time they can avoid things like renal failure. Perhaps the plasmapheresis, which I know little about, was done because of that 5 weeks he went untreated, and the low oxygen level. I'm not sure why it would have been necessary to wait between treatments with immunosuppressants, as some patients are given RTX and others at the same time, in some circumstances. Does he see someone with a lot of experience in treating WG? If there's any doubt, you could refer to this list of known specialists who are available for free consultation with your doc if you are not able to see one yourself: VF Medical Consultants . In any case, the fact that his lungs are no longer bleeding like they were seems like a very good sign that the treatments are working. It takes time at this initial stage, and extreme fatigue is very normal. His ANCA and SED rate should be going down, but those, too, can take some time. Although ANCA is not always a reliable indicator, and SED rate can be high for other reasons than WG, such as infections. But again, it could just be taking some time, and I'm sure the docs are on guard for anything unusual or alarming. I hope others can address the things I didn't do thoroughly. Keep us posted on how he does.

[drz]

No bleeding is very good sign. Plasma exchanges are good form of treatment and several of us here have had them and believe they contributed greatly to our recovery. Infections and signs of low O2 levels may be be the main concerns if anemia is under control. Age is an important consideration in what numbers are normal so let the doctors advise you so you don't over react. Recovery from intubation is a slow process and even slower for older patients so be patient and expect it will take longer than you like. Often Wegs is roller coaster ride too with many ups and downs so bad days and apparent setbacks often occur so try not to get alarmed if they happen.

[Green Grass & High Tides]

Sounds like the 20mg pred was subtherapeutic. I've had issues where subtherapeutic doses of pred helped minor pains but aggravated my wegs - cns, lung involvement. Synergistic HPA Axis Suppression helped me concurrent with cytoxan. Raising the prednisone helped as it seems to have done with your pops too. Age is important factor.
I would ask:
1. What symptoms should we chart daily?
2. What nutrition, exercise and restorative sleep suggestions / assistance can be offered?
3. How can you counteract the Paradoxical Effect of glucocorticoids? Especially subtherapeutic doses, like the 20 mg he was on when things got worse (assuming i read that right).

Its a tough disease that doesn't always make sense. I'm no expert and my suggestions are rudimentary and i hope helpful but please discard them if not. Prayers to you and your family.

Sent from my SM-G925V using Tapatalk

[Pete]

I would add the following:
1. What is the basis for diagnosis? What tests have led to this? What else could it be? This last one was crucial for me because their answer indicated that they had diagnosed me through process of elimination. They left the most definitive diagnostic of an open lung biopsy up to me. I decided against it as I just wanted out of hospital and to get on with treatment. The biopsy would have meant another week in hospital, a greater chance of infection, and significant discomfort.
2. What is the treatment plan? What dangerous side effects should I be on the lookout for? How soon should I feel better? What if I feel worse?
3. Do you have a plan B? What is it?

I would also want to gauge the doctor's experience level in treating Vasculitis. Most doctors may see a handful of weggies during their career. A doctor at a large teaching hospital such as Ohio State may see a few dozen. Better, but still not optimum. A doctor at a hospital such as Mayo or Cleveland may only see Vasculitis patients. This is best, but sometimes even they can be baffled by how our dumb disease can impact someone. The nephrologist and the pulmonologist at Ohio State who were on the team that diagnosed me said they might see 25-30 Vasculitis patients in a year. My rheumatologist at Cleveland Clinic sees only Vasculitis patients - 12 patients a day, 5 days a week, probably 46-48 weeks a year. And she sees many, including me, on a recurring basis (I'm down to twice a year.).

Hope this info is helpful. Best to your Dad!

[different]

Dad was diagnosed awhile ago. We're with kaiser in southern california his doctor for treating this is a Rhumatoglist who only saw about 20 wegners patients in the past 20 or so years. Her name is Dr. Akitson(not sure if right spelling).. My dad has full medicare coverage due to retirement from JPL..So he is with kaiser, has been for a long time.

Anyways he's in the icu still, the changes since I made this post is he finally seems more lucid and responsive after 5 days no sedatives, but the tube is in him still. Also his blood count has been dropping slowly for the past few days and today it's at 6.8 from 8.4 the previous day. They will give him 2 more units of blood. No more obvious bleeding from lungs.. So he must be bleeding from somewhere else..I have yet to see the doctor today and when I do I might update this post if there are any significant changes in his care.. A few other things is he has have been struggling a lot lately as if something is bothering him. Everyone is assume it's just the tube down his throat.. His heart rate goes way up as a result like 100-123 and stays around that high for a few couple of hours..His blood pressure was really high they have difficulties controlling that as well. The dyastolic blood pressure(bottom number) has been pretty low through out his whole stay here.


What I'm looking for with this update is possible solutions to why his blood count is dropping..How to convince doctors he doesn't need to be intubated anymore. Lastly perhaps other wegners doctors who are covered with medicare.

[Pete]

Here's a list of Vasculitis consultants VF Medical Consultants from the Vasculitis Foundation. Most of these will consult with your local doc at no charge. Perhaps you live close enough to one so you can get an office visit. I drive 2+ hours one-way to go to Cleveland Clinic.

Good luck!!

[annekat]

As to your specific question on dropping blood count, I'm sure others know more, but I did a quick Google search and was reminded that chemotherapy can lower blood counts (Mayo said by damaging the bone marrow?). The immunosuppressants we take, such as CTX, are forms of chemotherapy, though we generally take smaller doses than cancer patients. I've heard RTX called chemo, though I'm not sure it technically is considered so; I have heard it called a biologic. I know RTX is used in some forms of cancer such as leukemia and lymphoma. It may have some of the same effects of lowering blood counts. I guess this could be a question for your doc, and you could also do some searches, including here with the forum's search function, which might bring up posts and threads where this was discussed. I don't blame you for being concerned that your dad's blood counts are dropping again after showing improvement.

As for the high heart rate, mine was around that in the early stages after diagnosis. It still isn't very low most of the time. Probably more exercise would help with that. My blood pressure was not overly high the whole time, nor was it particularly low. I always got higher readings in the doc's office than at home.

I can't answer you on the intubation, but know there are others on here who have been through that and know more about the conditions under which one needs it and when it can be stopped.

As for WG docs who take Medicare patients, I'm sure a lot of them do, and people on here who go to the big specialist centers mentioned above can tell you if those places do. The problem could be if you needed a referral, and Kaiser might not give you one. I've heard of people with Kaiser having trouble with that, as they prefer to keep you within their network and might not pay for an outside specialist. I'm on Medicare, and am aware that you technically don't need referrals unless the prospective physician demands one. If he is on a Medicare Advantage Plan through Kaiser, it might work more to your benefit than not being on one. Or if you could handle it through Medicare on your own, and pay whatever copays apply. All this is still very confusing to me, and I've just gone to independent docs who take Medicare patients. I have not been admitted to a hospital since being on Medicare. I only know a little about Kaiser from being on it long ago in California, when I didn't have any serious illnesses. Best of luck to you in figuring it all out. And maybe someone from your area can recommend docs to you, if you will indicate where you live. Best wishes to your dad.

[different]

I bolded the important words since I kinda rambled and told an emotionally charged story instead of pure diagnostics.

I'm happy one person keeps on replying.. I do wish to hear from some others more often, I'll read a few more threads on this forum in hopes of answers..Update is kinda the exact same as last update.. Though there were some changes in the between but everything turned out to be almost the exact same as far as I know..

He was extubated the next day after I made the last post I believe.. The nurse that day gave him another batch of sedatives just because she felt like it, but he didn't wake up till the next day even though they were supposed to be short acting sedatives. The doctors told the nurses to stop giving him sedatives since it kept lowering his blood pressure too far and they want him lucid to try to remove the tube. After he was extubated we could finally talk and he could respond.. Problem is he lost his memory and much of his mind.. That worries me greatly.. Kinda similar to what I believe an alzheimers patient would act I believe.. He remembered my mom, but he didn't really remember me and many of his friends.. if I had to guess based on who he remembered and his speech and what he said, his memory was in the 70's to 80's.. That was very tough on me the thought of him losing his mind.. since I rely on him so much.. He isn't himself with out his mind intact..He was afraid he would be abandoned and forgotten so I stayed the night to keep him company.. The next night he was very delirious and thought mom ate some poison and needs the antidote.. and told me to sit over there and be quiet or go get mom and bring her back to the hospital to get her the antidote.. They also considered him well enough to move to upper floors, so I told the nurse to tell him his wife is getting treated downstairs if he asks, and I decided to leave him alone for the night, since he was worried about mom more than himself so I thought to let him be for the night so he would be a bit more calm and not worry so much.. But that was my mistake, something happened and he relapsed again. I don't know if it was neglect on nurse's side or it was just bad timing.. His oxygenation apparently kept going down throughout the night till they needed to keep him on 100% O2. I got a call from the doctor at 9 am telling me they need to put him back in the icu, 10 hours after leaving, and they needed to intubate him again and give him a bronchoscopy to see what was wrong. They found a bleeding site that opened up again and flushed it with saline and sucked it out. Their treatment plan is to up steroids, give him his rituxan chemo therapy a few days early, they found out the next day his blood was back to 6.5 and needed 2 more units of blood. The doctor also decided to give him a platlet transfusion.

Now his ventilator settings is pretty much the exact same as the day before he was extubated. The doctor even said "we would take the tube out, the bleeding seems to have stopped, his lungs sound good. But to be cautious we will keep it in for a couple more days" So we're pretty much back to how he was 5 days ago. Lucid, responding, with a tube in him at the lowest settings with his own breathing. Low blood count getting blood transfusion. Difference seems to be higher doses of steroids..


Also I really don't like all these blood sugar tests and insulin injections.. He's not diabetic, and he never took any before when he was taking steroids or when he was in here before and given steroids for other things..

What I'm worried about is his mind if it will return, and will he stop relapsing.. it's already been 24 days since this all started...Is it supposed to last this long?..

[Green Grass & High Tides]

Hi Different - sounds like a scary time. I had memory loss and times in the hospital where everything seemed to be out of control. Hang in there. It takes time and persistent / insistence from my family. They eventually found wegeners causing bleeding in my brain and gave me 1000mg Solu Medrol per day for three days, then 80mg pred/ day...Rituxan was too slow acting for me at that time and the wegs was rolling hard in many areas of the body. The steroids probably saved me but did take a toll which I won't go into here. It took 3-4 months for me to see anything from Rituxan. Blood sugar tests and insulin might not be worth worrying about now. Let them do it if they feel it's necessary. The steroids have strong effect on blood sugar. 24 days is not a long time. I would follow Pete's advice #7 above and get a specialist involved. This case sounds so severe that I'm actually afraid to respond since I'm not a doctor and you seem to really need consultation with a wegeners specialist more than anything else. Don't take anything I say as medical advice, please, I'm no where near qualified. It's just my experience.

As far as the "mind" coming back. I say "KEEP THE FAITH!"
A good neurologist can help answer this better. I definitely was not as sick as your describing. My brain healed rapidly with a good daily regimen and proper wegeners treatment. After 3-6 months I was walking fairly well. What hasn't healed is out of my hands. When the bell rings we answer. I hope it's not your dads time but I certainly offer my prayers and healing wishes.
Tom

================
From #7 above:
Here's a list of Vasculitis consultants VF Medical Consultants from the Vasculitis Foundation. Most of these will consult with your local doc at no charge. Perhaps you live close enough to one so you can get an office visit. I drive 2+ hours one-way to go to Cleveland Clinic.