The Gastrostomy

The day we have long awaited with both eagerness & trepidation finally arrived.  Amber went into surgery this morning at Starship Children's Hospital to have her Mic-Key Plug feeding tube inserted.  This will enable us to administer fluids & medicines directly into her stomach via a valve in her abdomen.  We carefully considered the pros & cons before putting her through this & unanimously decided that once she heals the benefits far outweigh the risks & initial discomfort she'll experience.  We'll no longer have to worry about reinserting her nasal gastric tube which, although tiny, undoubtedly causes some irritation in her throat exasperating her persistent gag reflex.  Additionally we were keen to get the tube out of her airways as she already has her muscle deterioration from the disease which compromises her breathing.  As mentioned in my previous post, an adnoidectomy was planned for the same time with further hopes of improving her breathing.

Our pre-op consultations with the gastro team & ENT doctors were pretty straight forward but the anaesthetist expressed some concern with how she would cope with the recovery from both procedures.  Cauterising her adnoids while being tubed under anaesthetic could place additional stress on her breathing & pain management could be challenging afterwards.  There is a hesitation with the overuse of morphine as it causes respiratory depression & the last thing anyone wants is for her to end up in ICU.  We always knew that there would be a question mark over whether the gastro could be performed successfully due to her hepetasplenomegaly (enlarged liver & spleen).  Once in theatre, if the surgeon decided there was not enough room for the procedure to be done safely the decision would be made to not proceed.  With Niemann-Pick being a venture into the unknown for all of us involved we were aware this could be a little more complicated than the average gastro.

Hanging out at Ronald McDonald House prior to surgery

Imagine our surprise when we received a phone call an hour later informing us that everything had been straightforward & Amber was starting to wake up in the recovery room.
Naturally she has not been her usual self since "coming around".  We have been shifted to the HDU (High Dependency Unit) on the ward so she is monitored carefully & we manage her pain.  She has been showing considerable distress at times with only relatively short periods of sleep.  The plan is to administer fluids intravenously today & then start her on a small amount of Pedialyte through the new tube tomorrow.  We'll gradually build up to our "normal" intake with hopes of being discharged on Monday pending everything going smoothly.

Her pain is being managed with small doses of morphine as & when she needs it without exceeding the recommended 24 hour dose.  She's also been introduced to paracetamol suppositories which go...well, you know where.  Additionally there are some eardrops for her left ear which was filled with wax & showing signs of a small infection.  Surprisingly though both her tubes (grommets) were still in place.
Initially the morphine was not working & her heart rate was rising above an acceptable level with her oxygen levels dropping intermittently.  She is receiving oxygen via nasal prongs & as long as her pain is controlled her heart rate remains normal.

The Mic-Key Plug looks like a valve for blowing up an inflatable toy although it has a temporary drainage tube in it at the moment.  There is also a small incision below her belly-button from where the surgeon inserted the plug laparoscopically.  It all looks quite daunting but I'm cautiously optimistic that we are over the worst of it.

belly incision, Mic-Key Plug with drainage & nasal prongs for oxygen

While my summary of today sounds very clinical, I assure you that I have been feeling very anxious about the surgery for quite a long time.  A big hurdle.  As they wheeled her out of pre-op there is a fleeting though of "what if this is the last time I kiss her goodbye".  Anything could happen.
I could not sit still while she was being operated on & took an extended walk to the small shopping area in the hospital hoping to find something bright & cheerful for Amber.  Not a lot of luck there but it kept me occupied.

Dylan got on Skype to his family as well as finally downloading a series of photos & videos from his mobile to his laptop.  Among them was a clip of Amber on the day of her diagnosis.  Watching this was nearly my undoing.  I'm even tearing up now thinking of how robust she seemed smiling & responding & being cheeky.  How innocent she seemed before Niemann-Pick took a firm grip on her body.  How can this happen to my baby who was so full of life?  I feel sick thinking about how this horrible, horrible disease is bit by bit taking her away from us.  It was such a reminder of how much she has changed & it breaks my heart.  What a cruel, cruel process this is.

I know in the future I will be thankful for the memories we have created but for now we still have a long way to go.