The Life and Times of Camille

So thrilled to see Camille sitting up in her wheelchair this morning when I came in.  It’s the first time she’s been out of bed since she got here.  Everything is going well and  we are hoping to get her home next week.  She is on antibiotics until Sunday and they still have to do her vent wean.  They want her to have a couple of stable days on her home regiment before they send her out.

Dr Thomas was able to get her dental cleaning in yesterday.  Camille got a good report card - no cavities!  They did not have to pull any of her baby teeth either.  They just did xrays/cleaning and sealants on her molars.

GAPP is once again trying to decrease the number of private duty nursing hours Camille receives.  Just prior to this hospital admission we received notice that they were going to drop her down to 70 hours eventually.  We requested a review and a hearing in front of an administrative judge to appeal this decision.  Thankfully she is locked in at 84 hours/week until the hearing.  I’m relieved for that.  As if it is not stressful enough trying to take care of Camille and her needs - we constantly have to fight to keep the nursing care that has been quite successful at keeping her out of the hospital.

Very excited today - Camille was stable enough to be moved out of the Pediatric ICU to the Technology-Dependent ICU.  The nurses there know her well and were thrilled to see her.

Camille actually had infections from 2 organisms - pseudomonas in her urine and kleibsiella in her blood.  Both cultured out as resistant strains so she will have to get a full 10 day course of 2 IV antibiotics.  Her arterial line, central line and catheter had to be reinserted on Thursday because she was back on dopamine and epi for a period of time.  She seems to have stabilized and they were able to wean her dopamine off yesterday.  The art line and catheter came out this morning and she was transferred to TICU this evening.

She looks great and is acting like her old self again.  Smiling, waving her arms, making her grumpy face when they give her a bath and turn her on her left side:-))). I’m so relieved - the last 2 weeks have been so stressful I’ve run out of energy.  Just thinking of all she’s been through makes me want to cry.  I’m so glad she is doing better.

Camille will be in the hospital at least another 7 days for the IV antibiotics.  She also is going to have her yearly dental on Thursday.  Dr. Thomas (her dentist) has agreed to do it if she is stable.

I had an extensive conversation with one of the pulmonologists last week and she said the only way that bilateral spontaneous pnuemothoraces could have happened is that Camille had to have gotten a large mucus plug in her trachea.  When she coughed against this plug she generated so much pressure in her airways that it blew out both of her lungs.  Before we go home, they plan to do a CT scan of her lungs that will tell us if she has any cysts (blebs) on her lungs and if this will likely happen again.  Dr Brooks said the incidence of recurrence was anywhere from 10-18%.  I know Jim, Robin (one of her home nurses), and I hope we NEVER see another episode like this one.

Camille had a great day yesterday.  The esophagram showed that the esophagus was still intact.  Her blood pressure was stable and she really seemed to be turning the corner.  Dr Hamre did say that she was worried about Camille getting an infection and was going to try to get her invasive monitors out.  They d/c’ed the arterial line and the foley catheter yesterday but it evidently wasn’t soon enough.  Last night there were nitrates in her urine and today they cultured Pseudomonas (a gram negative bacteria) from her bladder.  Dr Hamre had them immediately pull the central line and culture it also.  It came back positive for gram negative rods also.  Sooooo - she now has a blood infection which is creating problems with her blood pressure again.  They just restarted the dopamine and a couple of antibiotics.

Difficult to follow the last 2 posts:-))).  Jim is pretty incredible.  He has basically no medical background yet has learned to take care of an ICU level patient in Camille.  His way of interpreting data frequently provides a new insight or way to improve her care.

When I started Camille’s blog I envisioned it as a way to allow friends and family to check on Camille’s status anytime of the day or night.  However, it has become an invaluable source for me and for her physicians.  On the recent Sunday night that she was so unstable, I was able to find a post from 2007 that described the exact same type of “shocky” situation.  This prompted the intensivist to put her on a couple medicines that we may not have thought of initially.  It also us with provides an easily accessible reference point. How long was she on dopamine last time?  Has she ever been on max doses of epi?  How long did it take to wean her from the vent last time?  The intensivist and the nephrologists said that they have really never managed anything quite as complex as her sodium and urine output on this admission so perhaps if there is a “next time” we’ll have the info as to what treatment worked and what did not work.

This leads me to Lucas Finley (how’s that for a segue way??).  Actually, Lucas is better known as the Loop of Henle which is a part of the kidney responsible maintaining a specific concentration gradient of electrolytes.  This gradient allows the kidneys to concentrate urine based on the needs of the body.  The nephrologist thinks that Camille does in fact have Central Diabetes Insipidus and responds to DDAVP.  Because she was so shocky for several days, Camille ended up getting fluid replacements (with electrolytes) of up to 11 liters/day.  This is an unnatural situation for the kidney and she thinks that the massive amts of fluids & electrolytes basically destroyed the concentration gradient in the Loop of Henle.  We started seeing huge urine outputs along with high urine sodiums and this led the doctors to think she actually had Cerebral Salt Wasting Syndrome (CSWS).

Why does this matter asked my EE/JD husband? (had to get in @ least one dig)  It matters because CSWS occurs due to cerebral edema.  Cerebral edema occurs if there is injury to the brain.  If there was no CSWS or significant cerebral edema, then it is much more likely that Camille will come out of this neurologically intact.

The intensivist this morning started out with “let me tell you what I am worried about.”  It seems that someone (not clear who it was @ this point) decided to change the naso-esophogeal tube whose purpose is to drain secretions from Camille’s stapled off esophagus.  In this morning’s chest xray it appears that the tube is down in the abdomen.  If that is so, then the new tube has perforated the esophagus.  Camille’s spinal rods make it difficult to visualize areas on the xray so they have decided to do an esophogram where they will inject dye into the esophagus and see if it leaks into the abd.  We’re going down for that right now.  More later.

Look who’s awake!!!  Apparently SHE understands!  (not that I’m still bitter or anything…)  When I walked into her room and said hi, she opened her eyes, smiled, and waved her arms.  Go Milley!!!!

Hello!  This is Jim.  For some reason I’m filling in for Dena today.  Why?  Well, I don’t really know.  Dena called me and said “update Camille’s blog when you are at the hospital with her.  Keep it short, because you don’t understand.  I’ll post a detailed explanation tomorrow.”  Ummm, what?  Keep it short because I don’t understand?  Hoookkkaaayy.  Now, I know I’m not necessarily the brightest guy out there, but I do have a degree in electrical engineering and I did manage to graduate near the top of my class in law school, so I think we could at least start with the assumption that I might understand.

Anyway, after she called to tell me to keep it short because I don’t understand, she babbled on for about 20 minutes on a cell phone with sketchy reception.  Do I know what she said?  No.  But, then again, do I ever?  Personally, I’m blaming it on the cell phone.  Anyway, for you non-medical types, here is what is going on.  The med-heads will have to wait for Dena’s “detailed explanation” tomorrow.

Ok, so here we go.  Apparently, somewhere in Camille’s kidneys there is something that, on a garbled cell phone, really sounds like a “Lucas Finley.”  Ol’ Lucas really likes a certain balance of electrolytes, which he isn’t getting right now.  So, in the grand tradition of NFL players, pilot unions, and the New York City Sanitation Department, Lucas has decided to quit working until conditions improve.  In other words, he’s on strike.  Now, Lucas has one of those thankless jobs that nobody notices until it’s not being done.  He concentrates urine.  Since he is on strike, fluids are just rocketing through Camille’s kidneys.  So, according to the nephrologist (aka “the kidney doctor”), the issue isn’t that Camille doesn’t respond to the DDAVP that they have been giving her, the issue is getting Lucas back to work.  Ha!  I bet that Dena thought I didn’t know what a nephrologist was!  After 12 years with Crohn’s and 7 years with Milley, I have my ‘ologists down pat, thank you very much!!  Anyway, back to our buddy Lucas.  In a nutshell, they are going to try to get the proper balance of electrolytes restored so that Lucas will end his strike and start concentrating her urine again.  Unfortunately that doesn’t do anything for the current NFL lockout, but I’m sure Camille will be happier.

Check back tomorrow to see how much of my “limited understanding” explanation actually pans out.

Camille has a new moniker.  The nephrologist who saw her for the first time yesterday has anointed her the “girl who pees too much.”  She lived up to her name last night.  After about a 12 hour period of stability yesterday her sodium shot up and the urine output followed.  This resulted in a drop in BP and an increase in her dopamine drip.  They gave her a dose of DDAVP and by the time I arrived this morning she was stable again and on just a pittance of the dopamine.  She keeps flipping in and out of DI and CSWS.  The nephrologist feels that it is all centrally mediated and that her kidneys are functioning fine.

I’m trying not to get too stressed out over her neuro status at present.  They turned her muscle relaxant off and she does respond to the sound of my voice by trying to open her eyes.  She is still on a fentanyl drip for sedation so I’m hoping once we can turn that off we’ll see a better response.  Would love to see one of her big smiles or even what her nurses and I call “Camille’s grumpy face.”  The home care nurses get a grumpy face if they try to give her a shower too early in the morning instead of letting her take a nap :-)))

I had an opportunity to speak with the pulmonologist yesterday.  I ask him if he had any insight into why Camille developed the spontaneous pneumos with no history of lung disease.  He said the fact that she has had numerous aspiration episodes along with being on the ventilator at night has probably caused some damage to her lungs.  He surmised that she probably has some cystic areas in her lungs that are not apparent on xray and these weakened areas are susceptible to rupture.  He says it takes around 2-4 weeks for the lungs to heal.  Camille’s xray looked greatly improved yesterday with most of the subq air gone.  They did clamp her chest tubes and her lungs stayed expanded which is good.

Camille is still having periods of BP stability mixed with periods of instability.  They feel this instability is related to the swings in her urine output and the cerebral salt wasting.  I asked if there was any other reason she would be putting out so much urine and the PA said her kidneys could have taken a hit with the hypoxic episode.  However, he thinks that CSWS is the most likely explanation based on her urine sodium concentrations (they are high).  I actually found an article yesterday in the Pediatric Neurology Journal about kids with both Central Diabetes Insipides and CSWS.  It is obviously quite rare but the mean amount of time these kids in the study were in CSWS was 6 days and the range was 1-9 days.  Camille developed this around day 3 so we are about 4 days into it.  Also, the kids in this study all had normal brains prior to the insult that caused the CSWS.  Since Camille’s brain is abnormal, it wouldn’t shock me if it takes her longer to come out of the CSWS.

Camille is back on a dopamine drip for her blood pressure.  After being fairly stable for around 12-18 hours she started putting out copious amounts of urine again.  A normal urine output is 1-2cc/kg/hr.  Camille weighs approx 22 kg so they would expect her to pee around 22-44 ccs/ hour.  Yesterday evening for three consecutive hours she put out over 1000 cc/hour.  They are replacing her urine 1:1 with fluids but with those types of numbers it is easy to get behind.  That in turn affects her blood pressure.  They have been able to manipulate her fluids such that even though she is still spilling sodium into her urine with the Cerebral Salt Wasting Syndrome (see ICU Day 3), her sodium in her blood is staying within normal range.  I have no idea how long this pattern may continue -  I would assume until her brain swelling has resolved.  Unfortunately her neurologist is on vacation this week so I can’t ask him.

They did take her chest tubes off suction yesterday.  The next step will be to clamp them off and see if her lungs stay inflated.  This would indicate that the holes in the lungs are healing.  I don’t think they will do anything like that while she is still unstable with her blood pressure though.

It’s difficult to keep my mind from the “What If” game?  What if this CSWS doesn’t resolve?   What if Camille can’t be weaned from the vent?  What if her neurological status doesn’t return to it’s previous level?  What if this happens again?  What if? What if? What if?  After 7&1/2 years of caring for Camille, I am well aware of the futility of this type of thinking.  I try to combat the “what ifs” by  concentrating on living in the now and present with a “it is what it is” attitude.   Jim has a book about consciously living in the present entitled “Wherever you go, there you are.”  With Camille in our lives, I think our title would be “Wherever Camille takes us, There we go.”