My Big Sister's Story by Her Little Sister

[Squish]

15 days ago she was diagnosed after being admitted to the hospital.

Currently she is in ICU hooked up to a ventilator, ECMO (extracorporeal membrane oxygenation), and on continuous dialysis due to pleural effusion and partial renal failure. She has been this way since this past Sunday with no change.

She only had one incorrect diagnoses of RA and that was only 12 days prior to her hospitalization.

Her Wegener's has a cardiac aspect that is causing additional complications.

I have read all the diagnostic, and research medical material I can handle.

At this point my family could use some first hand knowledge from y'all.

What has been your experience with situations like this personally or with family members?

Thank you,

A Fearful Little Sister

[drz]

Some of us on here have been intubated too for a couple weeks, had kidney damage that resulted in dialysis or kidney transplant, and spent lot of time in ICU and recovery programs. Most (estimates run from 85-92% from some research I read) survive their initial treatment for Wegs but it usually is a major life changer and it can take awhile to adapt to it afterward just like any major illness or serious accident. Degree of recovery varies greatly according to age, health, severity of attack, treatment received, support available and attitude of patient.

Be supportive and don't read any research or info about Wegs more than a couple years old cause treatment has improved greatly which results in better prognosis today. Many people do recover their kidney function, (I got about half of mine back), and many people live OK today with a transplant kidney. And have a much better appreciation of their life so they can enjoy it more.

[andrew]

Yep I echo what DRZ says about research. Limit to the last year or two.

Your sister is in a similar situation to me back in 2003 - minus the cardiac issues. I got the 'sickest person in the hospital' award. I was on a ventilator with renal failure and it wasn't looking promising at all. I spent around two weeks in an induced coma and about five weeks in ICU. Thankfully my kidneys came back and are still with me. They have 50% function but I like to think that between them that makes one perfect kidney :) The darkest days are at the start when all is new and unknown. You're doing great to research as much as you can but don't let all that information bring you down or make you feel helpless. If it does just stop and hang out with your sister.

Is your sister under a Wegener's specialist? Which hospital is she in? How long had she been having symptoms?

[Squish]

it can take awhile to adapt to it afterward just like any major illness or serious accident.

This wouldn't be her first life adjustment due to major illness or injury about 5 years ago she lost a leg due to an injury.

[Squish]

Is your sister under a Wegener's specialist? Which hospital is she in? How long had she been having symptoms?

No she is not. She is at Memorial Herman, and depending on what the doctors are actually considering her symptoms to be could be months to years. She has always had fairly frequent nosebleeds and upper respiratory problems but never anything to the extent of pneumonia or pneumonia like. As for things like excess fatigue that has been since she lost her leg about 5 years ago which everyone including her just chalked up to the fact that things are a bit more difficult when you have one leg.

[drz]

Yep I echo what DRZ says about research. Limit to the last year or two.

Your sister is in a similar situation to me back in 2003 - minus the cardiac issues. I got the 'sickest person in the hospital' award. I was on a ventilator with renal failure and it wasn't looking promising at all. I spent around two weeks in an induced coma and about five weeks in ICU. Thankfully my kidneys came back and are still with me. They have 50% function but I like to think that between them that makes one perfect kidney :) The darkest days are at the start when all is new and unknown. You're doing great to research as much as you can but don't let all that information bring you down or make you feel helpless. If it does just stop and hang out with your sister.

Is your sister under a Wegener's specialist? Which hospital is she in? How long had she been having symptoms?

Mine in 2010 is about the same as Andrew's, I had no cardiac issues but type one diabetes which compounded my problems and care. Doctors often commented on how my survival and later degree of recovery was rather miraculous. Recovery can be slow but most people get better with proper care and effort. Some never attain a good remission but still have less symptoms with treatment.

I under stand the stress and problems of one leg now since i have a broken ankle that I cannot walk on or put any weight on that leg and it does make every thing slower and more difficult which creates lots of extra problems and stress.

You can get good or at least adequate care at other facilities than the expert ones if the staff have some experience with Wegs and consult with experts about proper treatment. My treating staff at a large University Hospital did that and I survived. I might have done better or had fewer complications and recovered quicker at Mayo but they wouldn't accept me at that time for insurance reasons.

[Squish]

I might have done better or had fewer complications and recovered quicker at Mayo but they wouldn't accept me at that time for insurance reasons.

Because of insurance she literally cannot go to any other hospital. Her nephrologist has been consulting with a specialist though.

[Alysia]

Hang in there sister. We have a weggie friend on facebook in a very similar condition. Started about 6 weeks ago. She is getting better but it is in baby steps, with ups & downs. Sending many prayers to her ♡

[gilders]

Hi Squish.
We can all give you our own Wegener's story and there will be similarities between them, but it is not a disease/condition that follows a set pattern for each patient.
There has been many of the members on this forum (including myself) that have had the initial onset of Wegener's take us to death's door, but so long as you get the appropriate treatment, the out come is usually very. It is something she will have to live with and relapses/flares are common, but treatment usually works well.

The 3 main things at the moment are that she has been diagnosed, has her sister and family there to support her and finally (I hope this is the case) is getting the correct treatment.

I'm sure your head is in a spin, so may not know the answer to this, but in what way is Wegener's affecting her heart?

Keep us informed of your sister's progress.

[drz]

Because of insurance she literally cannot go to any other hospital. Her nephrologist has been consulting with a specialist though.

I would highly recommend this expert listed on the Vasculitis site. I believe my nephrology team consulted with him.
North Carolina
UNC Kidney Center & Vasculitis Center
Chapel Hill, NC
Doctors: Ronald Falk, Patrick Nachman, Julie Macgregor, Will Pendergraft III
919.966.2561

[Squish]

...in what way is Wegener's affecting her heart? Keep us informed of your sister's progress.

It is a form of cardiomyopathy. Her heart at random times is just not strong enough to pump blood through properly and it is for only a moment. It is strange but you can see it when you go look at her charts.....everything else will be perfectly fine...then suddenly her blood pressure drops drastically for a pump then starts to go back towards normal. They are still trying to figure that part out. When she wakes up they will be bringing in a cardiac specialist for her. on that one.

As for an update as to how she is doing she has been weened off the ECMO and completely off now for about 16 hours. They are waiting a full 24 hours before they even consider weening her off another support.

[Alysia]

It is a form of cardiomyopathy. Her heart at random times is just not strong enough to pump blood through properly and it is for only a moment. It is strange but you can see it when you go look at her charts.....everything else will be perfectly fine...then suddenly her blood pressure drops drastically for a pump then starts to go back towards normal. They are still trying to figure that part out. When she wakes up they will be bringing in a cardiac specialist for her. on that one.

As for an update as to how she is doing she has been weened off the ECMO and completely off now for about 16 hours. They are waiting a full 24 hours before they even consider weening her off another support.

She is getting better then. Thanks God.
It is possible that the heart is reacting to the lungs.

[Squish]

It is possible it is a reaction to the drugs but the cardiologist seems to strongly believe differently. I am not sure if there is something in her chart or history that makes him believe as such but till such time as she is off the medications that could be causing that she is notated s having a cardiomyopathy.

[gilders]

Hi Squish, if they've taken her off ECMO, hopefully that means she's improving.
The reason I asked about her heart is because I have Left Ventricular Dysfunction (my heart wobbles rather than performs a good pump). I still have no answers as to why I have this, although I suspect Wegener's is responsible in some way.
Is she still on continuous dialysis. I would have thought the initial dialysis would have removed the pleural effusion and restricted fluid intake should keep the excess fluid around the lungs down. If she has partial renal function I would have thought a few hours dialysis every day at the most would be sufficient, rather than continuous.

Hope she's showing some improvement.

[Squish]

Hi Squish, if they've taken her off ECMO, hopefully that means she's improving.
The reason I asked about her heart is because I have Left Ventricular Dysfunction (my heart wobbles rather than performs a good pump). I still have no answers as to why I have this, although I suspect Wegener's is responsible in some way.
Is she still on continuous dialysis. I would have thought the initial dialysis would have removed the pleural effusion and restricted fluid intake should keep the excess fluid around the lungs down. If she has partial renal function I would have thought a few hours dialysis every day at the most would be sufficient, rather than continuous.

Hope she's showing some improvement.

She is actually semi awake now....by semi awake I mean they still have her sedated but instead of being in a technical medically induced coma she is simply sleeping and responds when they come in to do things.

She is still on the continuous dialysis as she is producing very little urine on her own....So her kidneys are still on the wavering side of things....but her lungs are on the up and up.....another 24 to 48 hours and they will consider getting her off the ventilator and letting her try breathing on her own again.

In the mean time we take turns going in and reading some of her favorite light hearted things from growing up to her. Nothing like reading your sister through and adventure through the 100 acres woods as adults.

[drz]

Glad to hear there has been some progress. My doctors always told me if you are not getting worse, then you are getting better.

[gilders]

Hi Squish. Hope there's been some positive progress with your sister since we last heard from you.

[renidrag]

She is blessed to have you. Through all this your Sister will be in need of an advocate for her, I assume it is you. Make sure to take time to also take care of yourself. Hope she continues to progress. Love your screen name by the way.
Dale