Dread

I have been dreading writing this post for about a month now.  Sometimes medical choices are just not good no matter which choice you make and we are at one of those cross-roads.

Here is the back-story...(it's long...stick with me)

When she was 6 months old she came down with airborne infant botulism.  It had nothing to do with her having disabilities, it was just "LUCKY".

You know how they tell you not to give infants under 1 year old honey?  Well it's because of botulism.  That would be food borne botulism.

And you know how women get botox injected in their faces to help with wrinkles and lines.  That is botulism.  Botox is botulism.

Botulism works by paralyzing the nerve endings.  So in the case of botox they are paralyzing those muscles so they won't move.

Airborne botulism is something you breathe in.  Botulism is everywhere in the world in the soil.  Most of the time it is not a problem.  But sometimes in a dry dusty environment (like Utah) babies can breathe it in and they become paralyzed.  This happens only to babies under 6 months and it happens when they transition from bottle or breast to eating from a spoon.  There are 100 cases of Infant Airborne Botulism in the US per year and 25 of those are treated at Primary Children's.

Ellie was 6 months and we had just fed her her first babyfood from a spoon a day or 2 before.

It was New Years Day 2003 and Ellie wouldn't wake up.  She wouldn't eat and she had no wet diapers.  We tried playing with her, giving her a bath everything we could think of and total zonkers.  My (Mikael's) Mom and I drove to Primary Children's Hospital Emergency Room.

The Dr. in the Emergency Room mentioned Botulism in passing but was running a multitude of tests.  They couldn't figure out what was wrong right away so they admitted her to the floor.  This was her first hospitalization.

On day 3 of being inpatient she coded (stopped breathing).  IT WAS TERRIFYING!  There were almost instantly at least 20 Dr.'s and nurses in her room talking and pushing buttons and doing all they could.  She was intubated (breathing tube down throat).  As soon as they were able they rushed her to the ICU.

The Dr.'s thought that perhaps it was her hydrocephalus (the extra fluid in her brain) causing extra pressure.  So they wanted to do a bedside EVD (External Ventricular Drain).  They drill through the skull and brain tissue and place a straw into the fluid to drain some of it.  They are also able to monitor and see how much pressure there is in the brain.

They had Jason and I step into the waiting room as they did the procedure.  There is a really sweet story about this.  They had to shave part of her head to do the procedure.  One of the residents scooped up her shaved hair from the floor and put it in a biohazard baggie and saved it for us.  It was very thoughtful.  It was her first haircut!

After doing the EVD they did not think this was the problem because the fluid was not under a lot of pressure.

They ran lots of tests including one testing for botulism.  The botulism test is a very strange one.  You must collect a stool sample and inject the stool into a rat.  If the rat dies within 3 days you have botulism.  This was a difficult test to run becuase she was not pooping.  Well at 3 days Ellie's rat was still alive, but it died after that.  So that was confusing to the Dr.'s

There is a medicine you can give to someone with botulism.  It makes the effects of botulism shorter.  You must give the medicine within 3 days of contracting the illness and they helicopter the medicine in from California.  Well, because her rat didn't die within 3 days she was not given the medicine.

The Dr.'s would never 100% say she had botulism, but they also never came up with a better answer, so I say she had botulism.

You breathe in the botulism spores from soil and they multiply in your intestines and travel throughout your blood to all of your nerve endings and block the electrical signals that your nerves are trying to send, thereby paralyzing you.  She couldn't eat, breathe, move, open her eyes, poop, pee, ANYTHING!  The effects of botulism are that you are completely paralyzed.

Airborne Infant Botulism we were told lasts anywhere from 10 days to 1 year.  Ellie's lasted longer.  The botulism does not leave your body, you just have to wait for your body to grow new nerve endings.

Because she was completely paralyzed she was on life support.  She had the breathing tube in her mouth/throat connected to a ventilator which was breathing for her.  She had a feeding tube through her nose into her belly giving her nutrition.

After being in this situation in ICU for three weeks and having no additional answers and not a lot of improvement in her medical condition the Dr.'s decided it was time for us to go home.  They told us that it is not good to have a breathing tube in your mouth and throat for longer than 3 weeks (it causes damage) and that we couldn't go home while being intubated (breathing tube).  So they wanted to give her a trach.  This is a hole they cut in your throat and a tube is inserted into your trachea and you breathe through the hole/tube.  I DID NOT WANT TO DO THIS.

They told me she would get better in 10 days to 1 year.  I was hoping that she would start improving any day.  After several days of them trying to convince me (AKA wearing me down) and sending in many many specialists all telling me that I needed to do this.  Jason and I finally consented.

She got the following surgeries done (these were her first surgeries)
-a trach
-ear tubes (which she needed anyway)
-gtube in her belly (tube to feed her right into her belly) (because remember she is paralyzed and can't eat),
-nissen where they tighten the top of your stomach to prevent reflux (because once again she is paralyzed and won't be able to keep the food in her stomach without refluxing)

When you have a child with a trach you must have a trained adult awake and with them at all times.  This is their only airway and if something is wrong it must be corrected immediately.

This meant that we would need nurses to come in every night and watch her so we could sleep for a few hours.  If a nurse couldn't come, Jason or I had to stay awake with her.

Jason and I had to have extensive training on how to take care of her.  She would be coming home with a trach and a ventilator.  We knew we would need help, so we asked our parents to be trained also and they did.  Our parents are awesome!  What grandparents would do that?

While waiting for nursing to be set up she had to go to South Davis Hospital for 3 weeks.  It was a scary and sad place.  I think it was probably only scary and sad because I was in a scary and sad situation.  Not the actual facility or nurses or Dr.  But those few months were the hardest of my life to that point.  South Davis helped us get our first adapted stroller with a special tray for the ventilator

We came home by ambulance on Mar 1, 2003.  Terry the RT and Sunny the nurse from Homecare were there to meet us.  They helped us get situated and brought us a ton of supplies that we would be needing.  They were so kind and helpful.

It was hard.  We had a 2 year old boy and now suddenly an extremely complicated little baby.  Our life got instantly hard.

If I didn't get a shower in before the nurse left, I had to bring Ellie and the ventilator into the bathroom with me and shower with my head poking out of the shower curtain every few seconds to check on her.  We had a little camry that was our family car.  Jason would drive.  In the backseat was Ellie in her carseat and our son in his carseat with me squished in the middle while the ventilator was buckled into the front seat and the stroller folded up in the trunk. At my feet was Ellie's emergency supplies (go bag) and suction.

She had the ventilator for about a year and the trach until Aug 2004.  (Much longer than the promised 1 year...it was 1 year and 8 months).

I know that was a really long back-story...but it is needed before I tell you the current story.

After getting off the vent and trach she went almost immediately on bipap at night.  This is a mask you wear on your face connected to a machine that delivers breaths of air.  She used bipap at night probably since she was 2 or 3 years old.

Starting in the Fall of 2015 she started having pneumonia every 6 weeks involving hospitalizations and IV antibiotics.  It was bad.  She had the occasional pneumonia before, but it was increasing rapidly in frequency.  This was bad because #1) people die from pneumonia and she had been close before #2) with so many antibiotics she could develop antibiotic resistance and get a super bug that nothing could fight off. #3) it was really hard on our kids and our marriage.

I spoke with her pulmonologist and suggested that the bipap was pushing the secretions down deeper into her lungs where she wasn't strong enough to cough them out.  She said it was possible but the sleep studies showed that she still needed bipap.  We tried adjusting the bipap settings.  And finally I decided on my own (without Dr. permission) to stop using bipap in fall of 2015.

Once we stopped bipap she did not have a single pneumonia or hospitalization for about 18 months.  It proved that my theory was correct.  She did really well for quite a while.  But the last few months have proven difficult.  She is now needing oxygen almost continually and sounds congested all the time.  She never previously needed o2 during the day unless she was sick.

I met with pulmonology again about a month ago.  We decided yes, the bipap had been pushing secretions down and causing pneumonia.  And we thought that now her lungs were probably partially collapsed because they were never getting blown open by bipap.  This would explain the increased ongoing need for o2.  We decided to do a chest xray.

The xray indeed showed that the bottom lobes (bottom part) of her lungs were closed (collapsed).

I asked if the lungs are never getting open and are not getting o2, do they die.  AKA...have we already done irrepairable damage?  The pulmonologist said no.  You can always blow them back open.  whew!

But then the problem is, if we go back on bipap then we go back to pneumonia leading toward death.  If we do nothing than her lungs continue to collapse, her breathing difficulties increase leading to death.

We brainstormed a lot of ideas including botox in the salivary glands, tying off salivary glands, meds to dry out secretions, lowering baclofen dosing.  If we could not come up with a good solution, then we may need to get a trach and use a ventilator at night..

AHHHHHHHHHH...NOT A TRACH!!!!!  It is hard.  We know how hard it is.  We want to explore all options before we once again feel forced in to a trach.

With the Dr. suggestion we decided to start doing bipap for an hour or 2 during the day while awake and sitting up.  We were hoping that she might be able to control her secretions better if she was awake and sitting up.  We started this about a week and a half to 2 weeks ago.  We saw an immediate difference.  She was now able to go some days without oxygen and just seemed to be doing better overall.  Everyone agreed she was improving.

Which brings us to this weekend...NOT IMPROVING!

Last Thursday she seemed slightly congested.  Friday both ears started draining.  We started eardrops in both ears.  Ear drainage stopped over the weekend, but congestion increased.  Last night was really rough.  I was up suctioning her most of the night.  I couldn't keep sats above 91 (mostly much lower) even on 3 liters of oxygen and using every trick in the bag.  I slept on her floor on a mattress.

This morning she was working a little harder to breathe.  She was needing 4 liters of 02, her heartrate was elevated and her temp was low.

I decided at about 8:30 this morning that I needed to bring her to PCH Emergency Department.  We got here at 10:30 AM.  She met shock protocol (good and bad).  Good because it gets Dr.'s and nurses and tests moving FAST!  Bad because it means she is pretty sick.

The airways in her left lung are collapsed (adelectisis) and small non-specific patches on the right that could be a virus?!?!?!  They found her positive for Human Metapneumo Virus.  This could explain the ears draining and the increased o2 needs and congestion.

We are now in PICU.  (Pediatric Intensive Care Unit).  They have had to put her on bipap and high oxygen to keep her oxygen sats up.  She has been awake however.

The thing I am worried about is we just started doing bipap 2 weeks ago and here we are in ICU.  That could mean one step closer to a trach.  Not happy.  I would do it if we have to, but seriously not happy.  THIS IS THE DREAD!

As for right now, she is doing ok.  She has not gotten worse throughout the day (since getting bipap on).  They did lower her o2 a bit, so I would say that is slightly improved.

I have seen her worse!

Blood pressure was low when we got to the hospital but came up with fluids.  Her sodium is a bit low but trending up.  Calcium was a bit low so they gave her some.  White blood count is low, to be expected with the virus.  She was also retaining CO2 (that you exhale), not good...but will hopefully improve with the bipap.

She also has bacteria in her urine.

They put her on antibiotics which should cover all of her ails!  Thank goodness for modern medicine.

What a way to spend spring break.  Ellie can say "I was away from home all of spring break at a hotel".  We just won't mention that it was the "hotel on the hill!"