G is for Gangsta

G is for Gastrostomy, a G-Tube.  The G-tube is an amazing little button that is surgically implanted through the abdomen and into the stomach.  Alessandra needs this because of her oral aversion and she can only tolerate small volumes at a time.  

Last Monday Alessandra had her operation to implant the G-tube, correct her intestinal malrotation and remove her appendix.  The surgery lasted about 3.5 hours and half of that was spent trying to place her Central Line.  Her surgery was laparoscopic, leaving behind minimal scars and some interesting photographs.  Everything went well and recovery was surprisingly quick.  The first 3 days were pretty rough, but she was as good as new after that.

The G tube is pretty easy to get used to.  It still freaks me out a bit that she has an incision there (belly button rings freak me out).    The video below shows us giving her meds through the tube in case your wondering how it works:)

~Melissa <3

Surgery # 3

Surgery number three is on the books for August 19^th. Why so soon? 

Before Ali was born we knew there was a likelihood that this heterotaxy would affect more than just her heart. It had been discussed that we would have issues with her other organs as well, specifically her intestines and spleen.  As a newborn, a G.I. study was done and showed that the spleen was absent, but her intestines seemed normal. We were so relieved that she wouldn’t need additional procedures.

After Ali’s heart surgery we had major issues with reflux (vomit, spew, barf, puke, blaaaaaaaaaghhghghgh).  This called for an upper G.I. study at 5 months of age, which did reveal a slight malrotation of her intestines.  However, the malroation has no correlation with the spew.  Her malrotation is of no trouble to her at all, for now.  There is always the possibility that stuff (stuff=guts) could get fused together or twisted, in which case an emergency surgery would need to be performed.  Fixing her malrotation as a precaution seems more sensible given that we have the good fortune of meeting with the surgeon beforehand. 

Still, why so soon?

Ali has been fed from an NG tube for about 8 months now.  She takes zero nutrition orally.  She is open to bottle feeding but her swallow study reveals that she aspirates if she swallows any liquid that is not the consistency of nectar.  We thicken with rice/oatmeal (the only thing approved for her at this age), but the amount of work that it takes to drink that stuff disinterests her.  People food completely offends her at this point (see below)….

The NG tube has served its purpose for the time being, but its time to switch to a G-tube. The G-tube will be surgically inserted into her abdomen and will be a better solution for long term help with nutrition.  Since we are ready to make this transition in Alessandra’s journey it only made sense to have her Ladd’s procedure (to fix the malrotation) done at the same time…..and while they’re at it, throw in an appendectomy as well.

Why so soon?

If we have to do this, I want it done before fall, a.k.a: germ season.  We are expected to stay 3 days in the ICU for recovery, given her need for cardiac assessment after the procedure.  The operation itself should only be about 2 hours.  The risks are relatively minor.  Of course there is a risk for infection, and that risk is increased since she is lacking a spleen, but is no different from the risks we faced with any of her other procedures.  We will have to get used to her new “button” for feeding.  The benefits are that we won’t have to worry about future emergencies and she will have a feeding “button” that will be concealed to all but those with x-ray vision.  This means she can go out in public without stares, points and whispers. 

I have to say… Ali has taught me so much.  I used to think that having a child that people stare at would be the worst thing in the world. It’s really not. Most people are amazing! Some look, some smile, some comment indirectly hoping not to offend but are still curious, some will have experience with similar equipment and will blatantly ask “what’s up with her?” some will have no experience but will still ask “what’s up with her?”  Truth is, I don’t mind any of it.  It has never been difficult for me to talk about it.  I have no issues explaining what the NG is for or what the 02 was for.   Still, for the sake of mobility and comfort it will be much better for Ali to have the G-tube.  This way her chubby little cheeks can be free of tape and she can blend in with the masses ;)

I know this surgery will be standard and successful.  She will be fine.  Still, I’m a mommy and this is my baby going in for an operation and I’m scared.  Our family would greatly appreciate any prayers on 8/19 that no complications arise and that she will recover strongly.  She just started crawling and I want her back on her hands and knees as quickly as possible!!

<3

~Melissa

One Year

Well guys, its been one year.  A year ago tonight, I weighed a million pounds and checked myself into Banner Good Samaritan to have a baby.   (Yep- I looked totally showered and excited to give birth!)

One year ago, Alessandra was born.  Unlike most mothers in labor I didn’t want to “push.”  As long as she was in me... she was safe.  The moment she was born, she was going to have to start fighting for her life on her own.  She came into this world ready for the challenge even though I wasn’t.

One year ago I watched my husband become a father.  Within moments after Ali was born I couldn’t be with her.  She was taken to the NICU for observation and I was forced to the recovery floor.  If I had any feeling in my legs at all I would have ran after her.  I had to sit back and let Mark take control.  From that moment forward we became a team.  We have spent many nights this year separated by hospital circumstances, but always a team.  Always working together to take care of our family.

One year ago I watched my beautiful Bailey become the most responsible big sister in the universe. I would have lost my mind by now if it wasn't for all her help.

Alessandra has put up quite a fight this year.  We have spent a cumulative of 3 months in the hospital undergoing heart surgery, thoracic duct ligation, heart failure, lung disease, chylothorax effusions and plueraldesis procedures (2x).

At one year old, she is the happiest little girl.  She is catching up on her milestones and improving every day.  It takes work. It takes weekly therapy and various doctor appointments to get her caught up, especially with eating.  She is moving in the right direction and is becoming less and less dependent on her O2.  Feeding therapy is a hair pulling process but every day she makes progress in new ways that surprise me.  I hope to keep up with this blog to chart her improvement as well as the effort it took for us to get there.

Things I acquired this year:

·      Super acute ability to hear a sneeze/cough/sniffle within a 10-mile radius.

·      Hand sanitizer. Gallons of it.

Things I learned this year:

·      Life can hurt worse than I ever imagined.  The amount of pain to be endured is limitless and yet… life goes on.

·      Things get better.

·      Some people leave. Most stay and show support to no end.

·      I LOVE nurses.

·      Keep your cardiologist close to your heart (I learned this from a fellow heart mom and I just love it).

<3

~Melissa

Thank You

So much has gone on in the last 6 weeks or so and I have been terrible at posting.  Firstly, I am so humbled by all the help and generosity from friends, family and complete strangers.  February was CHD awareness month and we celebrated it true to form… in the hospital.  That time was much more difficult for us than our prior admissions and thankfully we got through it.  Thank you so much to my amazing friend, Lexi, who set up a donation account and wrote about our family’s story on her blog.  Thank you to everyone one that has reached out in one-way or another. It means more to us than you could ever know.

We were released from the hospital 2.5 weeks ago, and a few days before our discharge I did something stupid.  I know this sounds silly but there are a few things that I’m really superstitious about. One of those things is tempting fate without knocking on wood.  A day before Ali’s release, I dared brag about the fact that she has never been ‘sick’.  She has been through heart failure and lung disease so she has been chronically sick in that regard, but she never had a virus/infection type of illness in her 8 months of life.  Less than 24 hours after I spoke those words there was snot….lots of it.  We were released from the hospital nonetheless with some new equipment to manage her health at home. 

List of equipment:

·      Darth Vader sounding o2 machine and nasal cannula

·      Home snot suction kit (its badass)

·      NG Feeds (we had this before)

·      Pulse ox and heart rate monitor

·      An entire pharmacia

The snot has lasted this entire time.  She started to show signs of improvement and then seemed to come down with some type of secondary virus.  She has had good days and bad days, today was a little of both.  We knew over the weekend that she was having a difficult time.  We desperately wanted to avoid the ER over the weekend so Mark took her to the pediatrician early this morning (I was at work, I think its AWESOME that my husband is so in tune with Ali and is so good with her care).  Her pediatrician recommended that she go to the ER so that she could get better suctioned and tested for viruses.  So that’s what happened…luckily it was only about a 4-hour stay though, record time for Ali.

Our plan for the next few moths is this:

1.     Get her through this dumb cold

2.     Replace her disgusting non-fat formula with breast milk (once cardio gives us the okay)

3.     Replace her NG feeds with oral feeds (by sippy cup, bottle, spoon, syringe, ANYTHING that works)

4.     Slowly come down on the o2

5.     Slowly come off cocktail of medication she gets served several times a day

Things are going well, Ali loves being home.  She loves playing with her sister and sitting outside.  She loves to poke the dog and roll all around.  We keep her home mostly, except for doctors’ appointments and trips to the park.   When she’s feeling good she is nothing but smiles.  I hope she will wake up tomorrow feeling like a brand new baby.  I always hope for that.

~Melissa

Post Op Update

The past few days have been a blur and I expect that my spelling and grammar in this update might be pretty hazy as well.  Alessandra had her thoracic duct ligated last Thursday along with the pleurodesis procedure.  The surgery took about 3 hours and she has a 2.5-3 inch scar on her backside in between her ribs.  A painful location, without a doubt, more painful than her sternal incision.  She was extubated shortly after surgery but was struggling.  The purpose of the procedure was to close off the duct that was causing all of that leakage in her chest.  It was a last resort since the other less invasive measures did not work.  For double assurance, after the ligation, the pluerodesis chemical was inserted through her chest tube into her chest cavity to inflame the tissue and seal up the leaking channels.  Another painful procedure.

The first day after surgery was a typical post-op first day.  She was heavily medicated and monitored to get her through the worst part of recovery.  By nightfall she had taken a turn for the worse and needed to be re-intubated.  She wasn't getting enough oxygen on her own and her lungs were working too hard.  Her heart rate was high and she was just so uncomfortable.  The idea was to give her some additional support for the night so that she can rest and regain strength.  We hoped to see improvement by Saturday but her lungs were worse.  The reason for this I don't know and can't understand.  It seems that her body reacted to the pain and invasion by clamping down and producing secretions, almost like RSV or a respiratory infection.  Part of her right lung collapsed and she started to run a low grade fever.  Her sats were terrible and her lungs sounded like crackling wax paper.

Since Ali is intubated, she needs to be heavily sedated to keep her from hulking out and rejecting the breathing tube.  Poor kiddo has been sleeping for days.  Now, we have had many ups and downs and trying times through this but last night was by far the worst night we have had.  Even though she's sedated, she was completely intolerant of anyone messing with her.  When the nurses try to suction her or simply re-arrange her position she clamps down so hard that the ventilator cannot support her and her sats drop.  Last night they dropped really low, like 15-18 low.  I've heard that the pulse ox isnt the most reliable measure, especially for anything below 60 and by looking at her she certainly didn't look that low, but still.... it was a new complication and low numbers that I have never seen before.  Wanna know how to turn your mom into a crying lunatic? Drop your sats to 15.

Today has been a little better.  We have her on a new sedative that she seems to be more comfortable with.  It has suppressed the hulk so far.  We started steroids today and the suctions seem to be more productive.  I guess we will know more with her x-ray tomorrow.  The goal is to get her off the ventilator as soon as we can since her Glenn physiology does not respond will with the positive pressures of the ventilator.  For now, she needs the support and the rest.  Poor kid has been through a lot and needs time to peacefully heal.

Of course there are other complications that come with the territory.  The combination of her response to surgery, going one day without diuretics and retaining IV fluid has caused her to be very swollen.  Everything in medicine seems to be a trade-off.  What fixes one thing, screws up something else.  We are trying to flush the fluids out of her with diuretics but she's still positive everyday, and of course low on potassium since the electrolyte balance is thrown off.   The second issue is that she has an line in her femoral artery for IV meds (I had a weepy rant in one of my posts about this line the last time it was placed because of the risks being blood clots...) well, this line has caused a blood clot.  The good news is that its small and not likely to break off.  She's getting shots of  (iforgotthenameofit) to help break down the clot and to keep it from growing much bigger.

Ugh- So that is where we are right now.  I will be sure to update once things get a little better. I know they will get better.  We always knew that it would be a rough first year.  I never expected it to be this rough.  We would really appreciate any and all prayers for Ali.  She is going through so much.  She such a sweet little girl and I love her so much. I cant wait until she can open her eyes again and feel good again. 
Thank you for reading,
Melissa

CHD Awareness Month

Hospitals, Holidays and Hearts

I swear we don’t live at Phoenix Children’s Hospital, but it seems that whenever I find time to post an update….we are at PCH.  I will get to all that but first allow me to backtrack so this record has some sort of order. 

I believe the last time I posted an update we were re-admitted to the CVICU at PCH because Alessandra had a chylothorax effusion (I have been saying ‘infusion’ instead of ‘effusion’ for the past month, I only recently realized that was incorrect.  I hope nobody noticed.)  The EFFUSION was caused by her heart surgery and fluid was accumulating in her chest cavity due to some cut or disturbance to her lymphatic system.  Several steps were taken to remedy this: 1) Diet change from breast milk to a gross non-fat formula... Didn’t work. 2) Aggressive diuretics to flush the fluid out of her system....  Didn’t appear to work.  3) 8 days NPO (without food) so that she can heal without stimulating the thoracic ducts while processing fat intake....  Didn’t work.  4) A procedure called Pluraldeses (sp?) to expedite the healing process by swelling the chest cavity with a chemical inserted by chest tube. ... Sorta worked. 

The pluraldeses (sp??) procedure was done a few days before Christmas and we expected good results.   A few days after the procedure Ali’s x-rays looked great and Dr. Nigro (Ali’s surgeon) surprised us by releasing us on Christmas Eve.  That was the most amazing gift ever! We were no longer hostages and were free to spend time as a complete family.  For so long Mark and I had to trade shifts. We would only see each other for a quick switch-off and poor Bailey really missed her sister (RSV restrictions at the hospital will not allow kids under 12 to visit).  The holidays were so wonderful and Ali’s development was rapidly catching up.  She was interacting more, sitting upright on her own, rolling from tummy to back.   Her being home makes such a difference.  The past month of my life has been so special.  It really brought us back to the basics and it’s a lesson I want to carry with me.  Sitting around the dinner table each night with the fam is the best thing in the world. Even if the baby is crying and throwing her food.  Even if the pre-tween is complaining about how gross squash is.  Even if I know there is a million things I have to do once dinner is over…It’s a few moments out of the day when I feel restored and we are all where we are supposed to be.

As outpatients we were not out of the clear. Alessandra had weekly x-rays and doctor appointments to monitor the fluid.  Unfortunately, the fluid came back.  At first we were watching it and treating it with diuretics, but no real progress was made.  She seemed fine so the plan was to wait it out and continue with the diuretics.  Well, for the past week her breathing was becoming more rapid and she was doing more chest-breathing instead of tummy-breathing.  Her cardiologist was keeping close tabs on her and her breathing pace was to be expected considering the fluid in her chest.  Yesterday morning we noticed she was slightly grunting with each breath, which didn’t seem right.  It’s hard to tell if she was coming down with a respiratory sickness (ugh, I can SEE the germs crawling everywhere this season), or if the fluid was giving her trouble.  We called the doctor and it was recommended that we admit Alessandra back to the CVICU for observation.

Coming back to the hospital is very familiar.  We know and love all the staff and doctors in this unit.  They are all polite and accommodating.  The doctors and nurses go out of their way to answer any and all questions.  Ali is in good hands.  She’s a rockstar when she’s here.  She has people standing on their ear to make her smile.  She won’t give it up too easily, although I know she is secretly amused.  For her, the poker face is important.  She’s learned that someone can be her friend one second and then poke her with a needle in the next second.   She is very keen on the subject of human behavior.  In fact, last weekend we took her to the zoo for the first time and when I approached an exhibit with her on my hip she had no interest in looking at the animals, but wanted to look at the people looking at the animals.  I think she will grow up to be a lady of science.

Anyway, so we are back at the hospital waiting for the next step.  It is likely she will get cathed tomorrow morning to verify that the fluid is chyle and not due to a blockage in her cardiovascular system.  I’m a little nervous for this because her last cath brought out the Hulk.  I think we know what medications work, and she is much more stable since her surgery so that incident shouldn’t repeat itself.  What I’m REALLY hoping for is that the IV Lasix did the trick and we will get an x-ray in the a.m. that will say all of the fluid was miraculously lifted and we can go home without any invasive procedures!  I know it’s a long shot but I have to always hope for the best.

On a closing note, CHD awareness week is Feb 7^th through the 14^th and I plan on celebrating the heck out of it.  I hope that you will join me.  CHD is such a struggle but treatment methods and surgical practices have come such a long way.  It was only a few decades ago when babies like Ali wouldn’t have had any chance at all.  Practices that began with baboon heart transplants for single ventricle babies have evolved into some very efficient surgical plumbing.  I’m so grateful for the research that has been done and there can always be more.  Spread awareness!!!

 ~Melissa