Marguerite
Active Member
My sister W rang this afternoon. brother in law and J's kids were called in to a meeting with J's specialist last night, they were given the details of the diagnosis and prognosis. There are a lot of us in the family (siblings etc) to call, brother in law just didn't have the time or energy to get to me, so W promised him she would. I'm cool with that. I actually suggested that maybe we should set up a communication chain so he doesn't have to feel he's got the load on his shoulders to tell everyone personally.
Anyway, W told me the diagnosis for J is Hamman-Rich Syndrome. As I expected, this is still the original diagnosis of Idiopathic Pulmonary Fibrosis, but is a subset of it, a sort of fine-tuned diagnosis. Hamman-Rich Syndrome is also known as acute interstitial pneumonitis. PF is also a form of interstitial lung disease.
The info on this form of PF is that it is much faster to develop than usual PF and similarly progressive and fatal. However, I found a glimmer of hope - there was a mention that occasionally a patient can recover, especially if prednisone or cortisone can arrest or turn around the deterioration. And this seems to have at least partly happened with J. Those who recover from an episode of this generally don't get it back, and can recover lung function completely.
I really didn't think it was going to be possible for her to come back from this. I thought that once the lungs were fibrotic, that would be it. But apparently not necessarily. However, the odds are still against her surviving.
She is now fully aware of her condition and I hear very depressed about it (understandably). She remembers a near-death experience at some stage over the last couple of weeks. Due to the medication she was on, her memories are thankfully hazy, but a few things she does remember, including my last visit. But she also remembers having the choice to let go and die, or fight and come back. She remembers choosing to fight but has been saying, "Now I wish I had let go when I had the chance offered."
Despite this, she is a fighter and has a strong determination. But I think she just doesn't want to suffer any more.
What is happening now - she's off the ventilator, off all drips, central line is out. Still on lung drainage (recovering from the inevitable pneumothorax from last week's open lung biopsy). She's getting oxygen via oxygen prongs only, but has good sats. She's lost a great deal of muscle tone, is very weak and tires easily, but all medications and all nutrition is now oral.
Her current improvement could be due to the massive prednisone causing a short-term improvement in lung function due to a localised reduction in inflammation. Or it could be a genuine recovery beginning. We don't know. But now we have a confirmed, detailed diagnosis, plans can be made. If she continues to improve, there is a chance after all that she could recover completely. I don't dare hope. But while she is improving, even though it's slow, and while there is still even a faint hope of eventual recovery, there will be no suggestion of lung transplant. I would think that if she begins to deteriorate again, they will at that point put her on the transplant list. But not before.
So now we wait, and try to keep her spirits up. I can't get over to visit her until probably next Friday (Good Friday) so I hope she hangs on until then. We need her to be as positive as possible, to give her every chance. It is so hard for her to breathe, it would be so easy for her to give up. And after what she has already been through, I could hardly blame her.
But she has her three (adult) kids to live for, and a very young grandson. A twin sister, plus her husband and the rest of us all barracking for her.
She's definitely not out of the woods yet, but things look a lot better than they did a week ago.
Marg
Anyway, W told me the diagnosis for J is Hamman-Rich Syndrome. As I expected, this is still the original diagnosis of Idiopathic Pulmonary Fibrosis, but is a subset of it, a sort of fine-tuned diagnosis. Hamman-Rich Syndrome is also known as acute interstitial pneumonitis. PF is also a form of interstitial lung disease.
The info on this form of PF is that it is much faster to develop than usual PF and similarly progressive and fatal. However, I found a glimmer of hope - there was a mention that occasionally a patient can recover, especially if prednisone or cortisone can arrest or turn around the deterioration. And this seems to have at least partly happened with J. Those who recover from an episode of this generally don't get it back, and can recover lung function completely.
I really didn't think it was going to be possible for her to come back from this. I thought that once the lungs were fibrotic, that would be it. But apparently not necessarily. However, the odds are still against her surviving.
She is now fully aware of her condition and I hear very depressed about it (understandably). She remembers a near-death experience at some stage over the last couple of weeks. Due to the medication she was on, her memories are thankfully hazy, but a few things she does remember, including my last visit. But she also remembers having the choice to let go and die, or fight and come back. She remembers choosing to fight but has been saying, "Now I wish I had let go when I had the chance offered."
Despite this, she is a fighter and has a strong determination. But I think she just doesn't want to suffer any more.
What is happening now - she's off the ventilator, off all drips, central line is out. Still on lung drainage (recovering from the inevitable pneumothorax from last week's open lung biopsy). She's getting oxygen via oxygen prongs only, but has good sats. She's lost a great deal of muscle tone, is very weak and tires easily, but all medications and all nutrition is now oral.
Her current improvement could be due to the massive prednisone causing a short-term improvement in lung function due to a localised reduction in inflammation. Or it could be a genuine recovery beginning. We don't know. But now we have a confirmed, detailed diagnosis, plans can be made. If she continues to improve, there is a chance after all that she could recover completely. I don't dare hope. But while she is improving, even though it's slow, and while there is still even a faint hope of eventual recovery, there will be no suggestion of lung transplant. I would think that if she begins to deteriorate again, they will at that point put her on the transplant list. But not before.
So now we wait, and try to keep her spirits up. I can't get over to visit her until probably next Friday (Good Friday) so I hope she hangs on until then. We need her to be as positive as possible, to give her every chance. It is so hard for her to breathe, it would be so easy for her to give up. And after what she has already been through, I could hardly blame her.
But she has her three (adult) kids to live for, and a very young grandson. A twin sister, plus her husband and the rest of us all barracking for her.
She's definitely not out of the woods yet, but things look a lot better than they did a week ago.
Marg