[brandybrindlemouse]

Hi there. My sister Mandy is currently being treated for ARDS. She’s 34 years old and has Down’s Syndrome. She has been in intensive care for the last 30 days.

OK technical details – she’s currently on 40% oxygen. She’s on CPAP ventilation mode. She has atrial fibrillation (AF) and has had cardioversion 3 times this week, she also receiving kidney dialysis. We have been told that she has extensive lung damage but because my mother is struggling to cope with the information she is given and in turn cannot provide us with a full summary of what the doctors are saying, I haven’t a clue at this point that that means. Luckily I’m going to the hospital today so I’m going to try and speak to a doctor then.

It has been very hard watching my sister deteriorate over the last month. My parents and my other sister were keeping a 12 hour bedside vigil for her but now she’s been moved so far away my sister and I can only see her once a week. I can tell you waiting each day for update calls is agony. I can’t stand not being able to pop up and give her a kiss and sit with her for a few hours. I also find it hard not having access to the medical staff and being able to ask the millions of questions I always have (I’m based in the UK so the medical system is a lot different to that of the States). Luckily the hospital have been able to find my mam a room so she is with my sister for most of the day.

Having read a lot of ARDS stories, her current symptoms don’t seem to look that good. I do worry that she will die and it will be a long, drawn out, painful affair for both my poor sister and for the rest of us who have watched her in and out of hospital all her life.

Of course my hope, knowing that my sister would probably die before me anyway, was that she would just go to bed one night and never wake up. I never contemplated the indignity of dying with tubes and machines everywhere and her being terrified.

I also find it almost impossible to deal with this illness without having someone able to tell me whether she’s going to live or die. It’s like a horror lottery. I read somewhere that you don’t deal with ARDS you live through it or die. What kind of illness does that?

Anyway, that’s my introduction. If anyone has any comments to make on the symptoms she’s presented with or anything else, I’d be ever so grateful.

Take care
Donna

[Davy9]

Donna,

You sister has multiple problems. When you consider that she has undergone cardioversion X 3 and requires dialysis those are serious enough on their own. Add to that ARDS and the prognosis sadly looks pretty grim. In my experience I suggest that the family weighs the misery of her care versus the best quality of life she may experience if everything turned out for the best. I do not know your sister. She may well be a very high functioning Downs. But, she is in pretty dire straights. Something precipitated all of this. An infection, a trauma, something started this cascade. If whatever started this is identified and controlled and the kidney failure is temporary, and the heart will calm down, then the pulmonary situation can be recovered. 40% O2 on CPAP is not that bad for ARDS. The situation is complex and fraught with both medical and personal conundrums. This is the best I can offer. Keep this updated and I will advance what I can.

[brandybrindlemouse]

Thanks for your helpful reply Davy9.

We're now on day 40. I saw my sister yesterday and spoke to her Consultant and here's where we are:-

She is off dialysis for the time being.
Her heart has calmed down. The heart rate still goes up on occasion but she has required no further cardioversions. Adult Congenital Heart Consultant has confirmed he needs no further input as her heart (notwithstanding her normal heart problems) appears to be fine.
She has significant hypoxemia caused by her lungs.
CT scan confirmed that lungs in fibro proliferative stage and lung damage is extensive. How much is reversible is unclear.
She has lots of lung secretions at the moment which I'm told isn't a good thing (is this because she has too much fluid on the lungs?)
Steroids had been given at various points but unsuccessful. Not suitable at the stage she's now in.
Sepsis has returned. Source of infection not clear yet but temp has reduced from 38.5 to 37.7 this morning.
She had had a line removed but as of yesterday they were going to put another in and start Norad again to support her blood pressure.
Very concerned about organ perfusion. Her blood pressure remains low at 87/40. The perfusion figure is 54 which we are told is low (they would like to see this at 60>)
She remains on 40% oxygen although the ventilation mode has been changed to try and avoid barotrauma (APRV Biphasic).

I noticed you are a retired Respiratory Consultant - how does this look to your trained eye? Whilst I understand pretty much most of what I am being told, the organ perfusion concern is new and I'm a bit lost as to know how significant this is and what the figure of 54 actually means. Would you be able to shed any light on this for me please?

I really am very grateful for your previous response and look forward to hearing from you.

Kind regards
Donna

[Davy9]

Perfusion pressure is a number related to the proper flux of blood supply in the body in general or in any part of the body. Proper perfusion means that blood gets delivered to the body, gets used, then exits, returning to the heart to again be refreshed and recycled. It requires a good pump for the arterial side, good arterial tone, a lower venous pressure to provide a pressure gradient and that is directly related to a low receiving pressure again back at the (right) heart. It is affected when any of those variables get affected. Heart failure backs up the system. Low arterial tone impairs the pump effectiveness and diminishes pressure gradients. A poor pump just can't maintain adequate circulation pressures. It is also impacted by fluid over load.

The overall perfusion pressure can be acceptable while the perfusion pressure for a particular organ isn't. If the brain, or the kidneys or the heart are not receiving their proper perfusion they will fail as individual organs.

For now they seem to be maintaining barely adequate perfusion and her kidneys are perfusing well enough to function. But it sounds as though there are some permanent changes in her lungs as a result of her ARDS and how it had to be clinically managed. ARDS has an acute phase that requires delicate but aggressive measures to survive. It is possible that the proper level of treatment needed can itself lead to bad outcomes. It is sort of damned if you do or damned if you don't. Don't take the steps and the patient dies or take the proper steps and live with their potential consequences. The lung fibrosis is one of those consequences. It commonly has some degree of permanence. The exact level of impairment is individual. You will have to wait and see how bad the lungs are and if they will allow her to wean from mechanical support. The only cure I know of for severe fibrosis in the lungs is a lung transplant and that would not be a considered option in her situation.

Her situation remains critical but they are doing the best that can be done. She has (they have) managed to overcome some serious obstacles and now face more obstacles.

[brandybrindlemouse]

Davy 9. Here is the latest update on my sister's condition (apologies but it is my rough typed notes from last night!). I wonder if you would be kind enough to let me have your comments.

Were told that my Mandy had shown good progress last week. However there were no guarantees Mandy's recovery would be a continuous path. There was always the possibility of further complications.

He said at the tail end of Sun/Mon and Tues her oxygens levels had been between 40-55

He said she is now having 65/70/75/85

Said she was on a higher level of support and not far from requiring full support (PSV 12 PEEP 12)

He said it is not clear why this is.

Number of possible reasons:-

Could be the fluid imbalance is slightly wrong. They took an x-ray which showed her chest had not changed in 7-10 days and in fact might be worse. There is quite dense shadowing which indicates significant scarring.

Could be infection. Thinking it is unlikely to be a bacterial infection as all tests had come back negative. Thinks it could be a virus called CMV. They could do a couple of tests to check for it - a telescope examination which Mandy could tolerate but it would be unpleasant or a lung biopsy which Mandy wouldn't be able to tolerate. He said they would treat Mandy as if she has the virus because the harm from treatment is less than the if Mandy has the virus.

Could be progressive scarring of the lungs causing the problems. It could be the lungs are too stiff to work properly.

I said I found it very difficult sometimes to guage how worried I should be and how serious her issues were.

He said if he was brutally honest he felt the best case scenario for Mandy is that she will always need ventilator support whether that be from a CPAP mask at night or through a tracheostomy he doesn't know.

He said long term prognosis is that lung scarring will not go away. She is not eligible for a transplant.

He confirmed she could still die.

He said if this was a temporary blip and Mandy rapidly gets better within 4 days then we can put it down to just a horrible week.

If she doesn't respond or gets worse then she has so little reserve to be able to fight that we could be back to the beginning.

I asked which one he thought this problem was and he said it was impossible to know.

He said if she improves then she could get to a stage where she maximises her potential (i.e. the halfway bit between not surviving and being the same as she was pre-ARDS). Whether this could be treated at home or in managed care or whether she would become unstable again he said they would have to decide on balance.

I asked whether if Mandy gets worse we might get to a situation where we are given the decision as to whether to turn off Mandy's vent. Dr Jones said yes. He said it wouldn't be a situation where we were given the decision. He said the hospital would be advising us as to what to do.

I feel he sounded fairly pessimistic me and seemed to say a lot of 'I need to have open and honest dialogue' which makes me feel like things are really serious again but I do find it really hard to understand how serious this is. I think it may have something to do with the unconscious believing she may not make it and my conscious which won't allow me to think she won't.

Many thanks
Donna

[Davy9]

Donna,

Some of your data does not make sense to me. Generally it sounds as though she is is suffering from either a worsening of her lung scarring status or from another opportunistic lung infection. At this point you should focus on how much oxygen is required to keep her adequately oxygenated and the pressures used to adequately ventilate and oxygenate her. With diffuse lung fibrosis the lungs become stiffer, more rubbery. When a mechanical ventilator is used this means that they need to hold the lungs open using Positive End Expiratory Pressures (PEEP) and that higher pressures are required to shove a breath into the stiffening lungs. It sounds as though she is on pressure support ventilation with a back up respiratory rate of 12 with a PEEP of 12 cmH2O. But how much does she breath on her own and what are the peak pressures used to deliver the backup tidal volumes? Oxygen delivery tensions above 50% and high peak delivery pressures, on top of the PEEP level will only exacerbate the lung scarring further. This is totally aside from combating any acute viral or bacterial infections.

Her prognosis remains poor. I would agree that she may become vent dependent owing to the lung scarring. That keeps her in the same catch 22 where more mechanical support can worsen the lungs status. There is 1 value you can follow. It is static lung compliance. The static lung compliance is a number that relates to how stiff the lungs themselves are. It is the trending you want to know about. Is her static compliance remaining the same or getting better or getting worse? The good news is the compliance improving. Less good news is the compliance not getting better. The worse news is an ever worsening compliance indicating that the lungs are getting stiffer and stiffer. They should be tracking her compliance values on the ventilator flow sheets.

[brandybrindlemouse]

Hi Davy 9

I went to see Mandy today so have a few more accurate details. I wasn't able to speak to her Consultant or the Registrar today but I understand that the problem was the CMV infection to which she's responding well to the antibiotics. She has some thick, creamy secretions this evening which they've said is either an infection brewing or is being caused by the furesemide they are using to help her pee. Her support seems to be remaining high - what do you think:-

Ventilator setting CPAP/PSV

21 CMH20 Peak
12 CMH20 Pmean
11.5 L Total VE
0.43 L Vte

CMH20 PSV 6
CMH20 PEEP 10
L/min Flow Trig 0.8
Fi02 55%

Her heart rate is 102
BP 107/59 (75)
AR 19
02 92%

I can't say enough how much your information has helped me understand what is happening. It is sometimes hard enough to take in what the Consultants are saying so it's such a help to be able to go away, digest and then seek clarification of what some of the terms actually mean.

Once again thank you.

Best wishes
Donna

[Davy9]

The furosemide (Lasix) is a diuretic and would not directly cause secretions of the type you describe. If they are considering a new infectious process they can capture and culture those secretions.

Her ventilator settings have nominally improved but the level of supplied O2 needed to achieve her 92% blood saturation has risen to 55% and that is worse. Her situation has not changed much since your last post.

I will add that it is not easy to accurately digest precisely what is happening. Her care is complex and you do provide useful but limited data. For now it would seem that she remains in difficult straights. I would make certain that they are taking measures to address gastric acids, etc.... because stress ulcers under these circumstances are common. They also need to be getting adequate and proper nutrition into her either via feeding tube or a major vein. Has she been trached (breathing through a tube in the neck)?

[brandybrindlemouse]

HI Davy9

Another week has passed and I doubt an awful lot has changed clinically. On the positive side Mandy has been moved to a longer term respiratory part of the hospital who will try and undertake what looks as though it could be a long wean.

Mandy has had a good week generally only yesterday was having some difficulty with pain in her tracheostomy (which answers the question in your last posting - she's been trached for the last couple of months). Her support needed to be turned up because she was struggling with her breathing. She's also started to need more support at night too. I spoke to a registrar earlier on in the week who confirmed there has been no change in Mandy's condition. She was a little surprised that Mandy caught an infection called CMV. She said it was quite common in people with compromised immuse systems. The only thing she could think of was that Mandy had an operation to correct her tetralogy of fallots 10 years ago and perhaps that could have left her susceptible. She appears to be responding well to antibiotics.

She confirmed that recent chest x-rays show some chronic permanent damage but she said it is still impossible to give an answer as to what Mandy's lung function will be. I find this so frustrating as we're 2 months in and we still don't know what caused the ARDS, don't know how damaged her lungs are and have no idea what the future holds. She said they would continue to be cagey about an outcome until they were sure - although she has no idea when this will be aarrrgghhh!

She is on a new ventilator and the settings are completely different from the ones in ITU. I wondered if you had the time to explain some of the more significant ones? I can see she's on 50% oxygen but I don't know what the equivalent PEEP and PSV settings are and how they compare with the settings last week.

IPAP 18cm H20
EPAP 8 cm H20
Rate 16 bpm
I-time 1.20 sec
Rise 3
02 50%

Breathing seems be a mixed between exhale/spontaneous/timed
Rate 24 bpm
VT 305-445 (it varied between these figures)
VE 6.8-11.6 (again it varied)
PIP 18-20 cm H20
Pt trig 97%
TI/TTOT 27%

Sats 90%
HR 91
Resp 23

Thanks again Davy9.

Kind regards
Donna

[brandybrindlemouse]

Sorry Davy 9 I forgot to include the fact that she's started to puff up a lot again. Her face is huge at the moment. I asked if they were giving furosemide to reduce the swelling but they confirmed they weren't as Mandy is already quite dry and they are worried about the prospect of having to put her on dialysis if she dries out too much. How will the swelling reduce?

Thanks
Donna