Finally....a Molly update (via auntie Nicole)

It's been awhile since Amy updated the blog, so while I am here with Molly I am going to do it (this is Amy's sister here giving an update).  Thursday night, Tim got a call at 2:30 am saying that they needed to get over to the hospital that Molly's potassium level was critically high, her heart rate was low and her CO2 was high.  She's always critical, but with her potassium being so high she could have gone into cardiac arrest.  Then her kidneys started to fail, wasn't producing urine and she is in acute kidney failure.  She has been on diuretics to pee and get fluid off of her, but they weren't working.  Her belly swelled up, alot, and was pushing on her lungs.  Initially they thought she had what's called abdominal compartment syndrome.  I've heard of this in legs and things like this, but personally have never heard of this in the abdomen.  So something was swelling, still don't know what, pressure builds up and presses on everything else.  Likely, this was impeding the function of the kidneys which caused the acute kidney failure.  Treatment of this is to open her belly and relieve the pressure.  So the attending physician wanted to do this surgery, she had the mindset that this is what would do Molly in for lack of better words.  She felt she might make it through the surgery, but would surely get some sort of infection that would kill her.  So she gives the option at this point, do  Amy and Tim want to go ahead with surgery or do they want to just make "Molly comfortable".  We know what they meant by this, however, they never say it outright and never give a prognosis.  So the surgeon comes to evaluate her and doesn't believe the pressure was high enough to warrant surgery.  It's always two sided here and none of the physicians are on the same page and certainly seem to not communicate with each other.  So the next option, put Molly on dialysis which pretty much does the functioning for the kidneys. 

DIALYSIS: a process for removing waste and excess water from the blood, and is used primarily as an artificial replacement for lost kidney function in people with renal failure.  When healthy, the kidneys maintain the body's internal equilibrium of water and minerals (sodium, potassium, chloride, calcium, phosphorus, magnesium, sulfate).


So here we go with the dialysis.  She would need what's called a portacath placed into a main vein which hooks up to the dialysis machine.  She only has two options of where this can be placed because all her other main veins are currently being used.  So two options, her femoral vein or her jugular vein.  Possible complications of both:  femoral vein is a long vein that goes into the kidneys, however, since it is long then it may not make it all the way to the kidneys to dialyse.  Plus, with all the swelling and the edema, there was potential for that portacath being "squished" and not functioning as it should.  Onto the jugular vein.  You have two, one on each side of your neck.  The right one has been used previously for her broviac, but is the better option.  Ultrasound showed that it was occluded and so could not be used.  So we are onto the left jugular.  It is open, and usable, but possible complication.  It doesn't work, closes up, and the blood backs up into the brain.  This means possible stroke. 

After Tim and Amy discuss this and I chime in, we talk to the surgeon who says jugular is better choice but they can wait and come up with a decisive choice.  Then he speaks with the attending and says this is critical and she needs this procedure.  So, the decision is made to place the catheter into the jugular vein.  The surgeon talks about complications and all that and gets the consent from Amy.  He's all encouraging and tells the risks of the procedure, but is cordial about everything.  Then the anesthesiologist comes and needs parents consent.  At this point, the room is ready for the procedure, Amy and Tim were talking to Molly and giving her kisses (I was able to give her smooches on her lips before I left the room, first time for that).  So this anesthesiologist is going over risk and possible complications and then tells Amy and Tim that she is aware that Molly will not survive this surgery.  She didn't say it was a possible risk. She said SHE WILL NOT MAKE IT THROUGH THIS PROCEDURE. Well this was a shock as no one had mentioned the fact that they didn't expect her to survive the procedure.  This threw Tim and Amy (mostly Amy) into a tailspin.  I was watching from a far at this point.  Voices were raised at this point and Tim and Amy were furious.  They come over to me, I ask that the surgeon come over.  He just happened to walk by at this point.  He says hmm, well I wasn't aware of this, with a very puzzled look on his face.  As I said previously, these doctors DO NOT speak to each other and so you hear something from one and then hear something completely different from another.  Anesthesiologist comes over and I ask where she got this information from about Molly not making it....she says the NURSING staff.  What the?  You didn't speak with the attending physician about this?  Amy was pissed.  She was almost up in the anesthesiologist's face at one point saying something like she wanted to punch her in the face or something.  It's all sort of a blur because we were all pissed at this point....even Tim and he even made me afraid lol. 

So we go out to the waiting room.  We do not know what to expect.  After an hour, the Surgery Fellow said she made it through fine and they were cleaning everything up.  Phew....relief.  Finally the surgeon comes out and says everything went fine.  So....she did okay over night, they were able to stop her diuretics and is off her dopamine and norepinephrine.  All good things.  She is peeing now, her labs look pretty good.  Kidney doctor says likely another 24 hours and then take her off the dialysis and see if her kidneys function on her own.  This is where we are at at this point.  I asked about a liver transplant, when would she actually be able to get on this list.  But all you can get out of these people is "today she is not able to get on the list".  Okay, but what would be the criteria for her to get on the list?  Nope, never a straight answer. 

At this point, no one knows what caused the kidney failure or the acute swelling.  I had initally planned on coming up here today to see Molly in action.  She had been moving and was awake and off the paralytic.  At least she is not paralyzed, just super doped up.  I was able to see eyes open a little and she cries when she needs her methodone, which is so sad to see.  She's such a sweet little girl. 

So right now it's day to day, almost every 4 hours by every 4 hours, because that's when the next set of labs are drawn.  The short term goal right now is to get her off the dialysis machine and get her kidneys functioning.  Then after that, not sure.  They need to see what's up with her intestines because it may have all stemmed from this. 

Right now, she is still here with us, not in pain, and doing okay.

Day 238

We have spent 238 days in the hospital. Hard to believe. But, I think we have finally found our miracle drug....omegaven. This has been such a great week. I got to hold my peanut for 1.5 hours on Tuesday. It has been at least 6 weeks since I had held my daughter. Way too long. She has been tolerating her pedialyte like a superstar. She has been pooping. And yesterday her eyes were as wide as they have been in awhile. She was staring at her block hanging over her head. I grabbed it and moved it from side to side and up then down and she tracked it the whole way. This may not seem big to anyone reading this. But we had another head ultrasound last week and it showed progressive atrophy of her brain. So, this is huge. Also, Tim and I have been suctioning Molly's trach and doing trach care. We have a checklist of things (these 2 included) that we have to perform before we take trach class #2 which is trach change. I am scared. But I was scared of trach care and it no longer seems scary and that is after only 2 times.

One last piece of news and a huge piece is that Molly's direct bilirubin has gone down 4 points in the last 2 days. 4 points!!! I think we are finally over the spike in levels we expected to see at the beginning of omegaven. I think it's all downhill from here. I am hopeful!  And hopeful feels good. 

Pictures include Molly's kitty slippers from cousin Alani, Molly block watching, Mommy and Molly, Molly's beads of courage, and Molly blowing the biggest mucus bubble I have ever seen. Enjoy!

Molly video!

https://www.youtube.com/watch?v=UhcjgkPfKeg&feature=youtube_gdata_player

Omegaven - week 2

I apologize immensely for not blogging before now.  Sometimes, I just don't feel up to it.  I will try my best to do it daily or every other day.  Because now, there is so much to catch up on. I will start with her labs.

Omegaven was started on 5.6.14 and I believe I posted those results.  Most of her liver function labs are Mondays and Thursdays now. Anyway, here are her labs from

5.15.14:

ALT - 129
AST - 204
Alkaline Phosphotase - 319
Total Bili - 36
Direct Bili - 25.70
Albumin (5.12.14) - 3
INR - 1.3
CRP - 2.49
Platelets - 61

Labs from 05.19.14:

ALT - 236
AST - 369
Alkaline Phosphotase - 298
Total Bili - 41.4
Direct Bili - 29.40
Albumin (5.12.14) - 3.1
INR - 1.4
CRP - 3.02
Platelets - 95

Her bilirubin levels are as high as I have ever remembered them being. Everything I have read about Omegaven (I have read about everything there is out there), her bili levels will rise before they drop. It usually is not this fast though. So, we can only have faith in Omegaven and hope those levels go down. Some good liver news is that her INR is still in the good range, her Albumin level has increased and her Platelets just keep rising and rising.  These numbers are very good.  And, Molly's GI doctor (the omegaven doctor) says that based on these numbers, her liver is holding stable.  They are concerned about her bilirubin levels, but they are kind of stable.  We just have to pray and hope that the Omegaven does like it has proven to do, and that is to start seeing a drop in those numbers.  Her CRP is up, (2.49 to 3.02), (up is not good). But, she had infections in both PICC lines. 

Onto the infections. On Saturday, she spiked a temp. So, they started her on 2 broad spectrum antibiotics (vancomycin and zosyn) and did cultures. Today they were able to identify the infection. It was staph. It thankfully did not get into her blood stream. In fact, the cultures they drew the day after they started antibiotics are negative.  So, it appears as though the infection was caught in time and they have now started her on an antibiotic specifically for staph infection.

She has been started on pedialyte.  A very small amount, just to wake up her gut. She has tolerated it beautifully so far and has even pooped!  Yay for poop!  I almost took a picture of it.  Gross, but when your daughter had her intestines hanging out of stomach for 3 months and pooped in a bag, this is great news!

The nimbex (paralytic) is being weaned daily. She is now starting to open her eyes and stay awake for more and more each day. She wiggles her hands and feet, too.

Here's the overall plan:  Continue to wean the nimbex. When that is gone, start to wean the nitric oxide (as long as her echo is okay). That will probably occur over the span of a week. After that will come weaning the narcotics. She is currently on drips of fentanyl and versed. She is on scheduled doses of methadone and valium. Eventually, the methadone and valium will be increased until maximum doses are reached, then the fentanyl and versed will be weaned until she is off of them.  Intermittently, the vent will be weaned and the pedialyte will be increased until she is on the full amount. After that, we will switch from pedialyte to neocate
(formula). So we have a LONG road ahead of us, but we will certainly get there. Just have to have patience and pray and enjoy each and every day with our cute little peanut!

Please don't ever leave Molly out of your prayers. Even though she may be stable, we still need prayers to keep her that way.

Thank you to everyone reading this!

Amy

My first mother's day

I am sitting by Molly's bed right now. She is the sweetest little girl that ever lived. I am truly the lucky one having her as my daughter. She has taught me so much. Patience. Unconditional love. Faith. Strength. She is the bravest, strongest, most stubborn little peanut I have ever had the privilege of knowing, much less be the mother of. We have had one of the toughest months we have had to date. But, the past several days have been uneventful. Uneventful is good. She has been stable and holding steady. We couldn't ask for much more than that right now. I love my peanut so much.

Omegaven day 1

Sorry for the lack of updates recently. It has been a rough month. Molly has been fighting her infections and recovering from them. Currently she is being weaned off the paralytic, on fentanyl, versed, methadone, and valium, and on 20 ppm of nitric oxide. She was supposed to have started the omegaven last Friday, but of course we hit a snag. This drug has been the hardest thing to get. And that is so sad. So many babies can benefit from it and yet the parents (not the doctors) have to fight for their baby to get it to save their baby's life. I need to give props to my sister Nicole for writing a letter to the head honcho of the hospital. Without that letter, we would be waiting another 2 weeks of valuable time to start the omegaven.

Molly is stable at this time. We were given a grim prognosis yesterday. Her lungs are getting worse because of the pressure from her belly which is full of fluid, or ascites. Her heart is working overtime to compensate for the pressure in her portal vein. In my opinion, the cause of all of her problems is the liver. Omegaven has to work. We have no other options. We need as many prayers as we can get that omegaven is the answer we have been looking for. It is not a miracle pill in that it doesn't work overnight. It will be at least a month before we start seeing any improvement. In fact, her numbers will get worse before they get better. We also need prayers that she stays infection-free. I don't know if her little body can handle another infection. I don't want to find out either.

Please pray for the omegaven to work and that she stays infection-free. These things have to happen so that one day we can take our peanut home.

I know that probably nobody knows what these numbers mean, but I am putting them here for reference. They are Molly's pre-omegaven numbers.

Total bili: 23.0
Direct bili: 15.7 (normal is <2.0)
Liver enzymes:
      ALT - 146 (normal is around 50)
      AST - 138 (normal is around 50)
      Alkaline phosphotase: 163 (currently     in normal range)
Albumin  - 3.9
Platelets - 29,000 (very very low - normal is > 150,000)

These are just some of the numbers we will continue to monitor on the omegaven. The most important one that we will watch is that direct bili. It's a good indicator of her liver function.