A better day than yesterday (not many things could make it worse).

Our friend Caroline, who is a nurse manager, spoke with the ICU director first thing this morning.  We met Caroline when she was the first nurse to care for Jonathan after coming out of heart surgery in super critical condition.  Though that time we each learned and taught each other a lot.  We have kept in touch with her.  She was almost as disappointed and upset as we were after leaving work yesterday. She participated in the intubation, preparing the crash cart medications in the event Faith went into cardiac arrest.

Dr. K, the ICU Director came down to Faith’s room this morning, stood at the end of her bed, looked at her and us and let us know in uncertain terms how dissatisfied he was with the situation.  He only returned from California Wed night. We had a long talk with him and he understood everything we had to say and was taken by surprise at some of the things we had experienced.

Here are the changes he promised and we have already seen:

1. He will be point man for the team delivering her care, coordinating services and getting her what she needs and deserves.
2. Pulmonology (Attending, Fellow & Resident) were up at her bedside and agreed that the FULL airway needed further examination via rigid bronchoscopy as even with the endotracheal tube in there is still some unusual noise lower in the trachea.
3. Neurology has ordered an MRI to rule out two know contributors in Down Syndrome patients that may have neurological component in her respiratory issue.  The first is simple and all DS kids are screened for this in early childhood (Yes, Faith was at 3 and it was OK).  It’s called Atlantoaxial Instability.  Basically the first and second cervical vertibae (C1 & C2) can shift back and forth causing airway instability.  The second is something called Arnold–Chiari malformation (http://en.wikipedia.org/wiki/Arnold-Chiari_malformation)
4. ENT Attending visited the bedside also today.  They want Neuro to clear her before they attempt any surgical interventions.  They will scope her again thoroughly without any steroids on board which have masked the problem in the past.  It will be done in the OR under controlled circumstances because they will need to take out the ET tube, scope and either leave it out or need to re-intubate based on findings.  He believes more and more it may be a problem in the larynx (no specifics yet).
5. ENT has also contacted an outside expert with specialty in airway problems in children with Down’s Syndrome, he is away and will be back and in on Monday to see Faith and review what has been done.

Our confidence is almost restored that everything and anything is being done to find the RIGHT solution to fix the problem Faith has been struggling with for over a month.

“Our lives begin to end the day we become silent about things that matter.” – MLK Jr

BAD Night, VERY BAD Day

Last night the plan was to try to get Faith to tolerate CPAP as a means of helping her airway obstruction. Let’s just say EPIC FAILURE.  Even with the inclusion of sedation that should have been able to keep a horse calm she was able to breakthrough and rip it off.  The dose was the highest allowed without already being on a respirator, at a slightly lower dose she was trying to get out of bed.

At 9AM we had a very confrontational bedside meeting with ENT and the ICU team. We told them all where this was going and the fact that she needed immediate assistance as she had been working way too hard to breathe since late last night.  Her breathing was obviously very difficult and her heart rate was going very high.  Apparently albeit repeated attempts it fell on deaf ears.

At 12 noon she was again intubated and returned to a ventilator.  The intubation procedure was very smooth; there was no loss of oxygen or cardiac events.

There is so much more to the story that I may go into at a later time, but to summarize we are very disappointed in the level of care she has received from an institution we once revered.

Without any studies to tangibly support it they have clinically diagnosed her problem with the ubiquitous “upper airway obstruction”.  Their solution to remedy the airway problem is tracheostomy.  We are not convinced that such a radical procedure is necessary without knowledge of the underlying cause, nor are we convinced it is appropriate without knowing the root cause of her breathing difficulty.

We have been asked if this is a life-threatening condition, the simple answer is the reason one is in an ICU is because their condition is life threatening.

“What doesn’t kill us makes us stronger”

A relatively quiet night (for Faith), the ICU was very busy and we were not in an isolated room so sleep was not so good.

OK, Its not an abscess, the x-rays were compared to previous which also showed the same space and she has NO clinical symptoms of an infection (confirmed by blood work).

With the promise of ice cream Faith did let ENT pass a small scope up her nose and down her windpipe for a quick look and nothing abnormal was found although it was admittedly difficult to get a full clear picture in a kid who was awake and not happy about the procedure.

For today the team knows she responds well to systemic steroid as they reduce the inflammation in her windpipe and ease breathing.  They come with a whole bunch of other problems in the long term.  For tonight we are going to “try” to get her to accept using CPAP while she is sleeping.  CPAP will keep some positive pressure on the airway and prevent collapse or obstruction while making it a bit easier to breathe.

The odds are less that 50% we will make much progress tonight convincing her this is good for her and she should cooperate.

For those who have asked what the final goal to getting her healthy his.  Quite simply to be able to breathe on her own without assistance or O2 while both awake and sleeping.

More tommorrow.

We are back in CHONY PICU original bed. Easy transfer no incidents.  Faith slept most of the way.

Tests and consults by ENT and pulmonary.  Talk of doing CT scan tonight.

Updates in morning if anything new.

We are awaiting transportation back to CPMC/CHONY.

Faith started developing respiratory issues again on Sunday. They have not improved adequately since then.  A neck X-ray this morning MIGHT indicate an abcess pressing on her windpipe.  The experts here are split. This facility is not
setup for acute care. So we go where we need to until this all gets sorted out.