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

Here we go again...

As I type this I’m sitting in an empty hospital room.  The only other time I have sat in this empty room was when Alessandra had her heart surgery.  All the same feelings from that surgery have returned.  The stomach twisting anxiety, the slow moving clock and I’m holding on to the only thing I can to keep myself together: an unshakable trust in God.  I have to trust.  

I know it’s been awhile since I posted so I’ll try to bring you up to speed.  After we were discharged from PCH on the 12/1 we had several follow up appointments as outpatients.  One of those appointments was an x-ray that revealed some fluid in Alessandra’s chest.  Return of the chylothroax.   She was admitted back to the CVICU to have a chest tube placed and the fluid drained.  Chylorthorax is a thick milky substance that is produced by the lymphatic system. Sometimes, during heart or lung surgery, the “vessels” that this liquid travels through can be cut causing a leakage into the body.  I added the quotations to vessels because I believe they are more like canals or tissue grooves and not necessarily a vessel.  Anyway.   The issue with her chyl was caught shortly after surgery, but the drainage tapered off, the tube was removed and we were sent home with an ng feeding tube and some non-fat formula that we were to continue for 6-8 weeks.  However, since being readmitted to the hospital the chyl output is still high. Too high.  Ali's cardiologist and surgeon discussed an aggressive measure to remedy this.  The decision was made that she would be off formula and completely NPO for a period of a few days.  A catheter will be inserted into her femoral artery and she will be given IV nutrition for several days. 

Right now she is back in the lab, being put to sleep so they can insert this tube.  Of course before we sign the consent form for any type of procedure, the risks are discussed which usually leaving me white in the face and trembling as I sign the forms.  The risks for this procedure include blood clotting, and she would not be a candidate for the medicine used to treat blood clots because of her physiology. It really sucks.  I didn’t go to medical school and for this particular procedure the decision was made quickly. I didn’t have time to google or chat with fellow heart moms.  Not that the internet and the experience of others makes me a qualified mom-doctor, but it helps me to know what questions to ask, what to expect and sometimes what doctors to specifically request.  This was one of those times where I have to just trust.  Trust that the doctors know what they are doing, trust that they will handle her with the same care they would use for their own children and trust that God has a plan for us in all of this.  I don't know what else I can do.

This is all to solve priority problem number 1, her chylotharx.  Unfortunately that is not the only after surgery side-effect that we are dealing with.  Ali has been throwing up a lot since surgery.  Several times a day.  It appears to be a mystery since no known cause can be identified. We worked with some GI doctors the other night and they did study and which found some surprising results.  Ali has malrotated intestines.  We knew during my pregnancy that with a heterotaxy diagnosis that this was a possibility, however she had an anatomy scan after birth and we were told that her intestines were fine.  We never thought to challenge that because her eating and gi functions were perfectly normal.  From birth to about 4 months she nursed like a champ, gained tons of weight and had zero gi issues.  Well, now we know. To make it more confusing, although her intestines are malrotated, they are not kinked in anyway and are therefore not her reason for vomiting.  Which is pretty obvious to us anyhow since she didn’t vomit prior to surgery.  This is all very confusing but it means that she may need an additional surgery on her intestines at some point and the cause for her throwing up is still unknown.

The past few days have been so horrible, and not just for us.  An entire nation weeps this weekend over recent events.  The uncommon desert rain outside seems fitting.  Things feel dark and sad.

A New Heart

 I have been meaning to post something about the outcome of Alessandra’s heart surgery but it’s been hard to find the time.  Her surgery was last Monday (11/19) and everything went perfect!  Her surgeon, Dr. Nigro, came to the hospital on the Sunday before to go over some final details and to answer any questions we had. He came into our hospital room when I was getting out of the shower. I thought I heard his voice so cut myself shaving, threw on some clothes and swung the bathroom door open (I almost slammed him with the door…{gaaah!}).

The entire surgery lasted about 3 hours.  It was the longest 3 hours of my life but we had a ton of support from family that showed up at the hospital to wait with us.  The procedure was better than expected; Ali didn’t need bypass or any blood products.  We were able to see her shortly after the operation and she looked so beautiful.  Her lips and fingernails were so pink!  CHD babies tend to be dusky in color and I had grown so accustomed to her purplish tint that it was incredible to see my sweet baby peacefully sleeping with her new rosy complexion.  A few hours after the operation she was extubated and breathing on her own without trouble. 

For several days the amazing and brilliant nurses at PCH have carefully managed the worst of her pain.  We are still in the hospital as she recovers but she has been weaned off most of her pain meds.  She is now down to just Tylenol and ibuprofen (along with heart meds and antibiotics, of course) and is doing well.  She smiled for the first time today.  She is so strong!!

The only complication we have had is with her chylothorax.  During surgery, her thoracic duct must have been disturbed and now she has a leakage of chest fluid.  This is a common issue after heart surgery and she still has a chest tube in place to help drain the fluid.  The sad part is that the treatment for chylothorax is a diet of non-fat formula for six to eight weeks.  This means that she can no longer take in breast milk. I wasn’t ready to stop nursing and the Enfaport formula doesn’t seem to sit well with her.  I’m still going to try to pump and store breast milk, hopefully my supply can last several weeks this way.  Also, since Ali isn’t quite meeting her feeding quotas she needed to have an NG tube placed (a thin tube that goes through the nose and into the stomach) for feeding.  She hates it and I feel so bad for her.  I know she will get used to it the way she has had to adapt to all these terrible things.  Hopefully she won’t need it for long.

These past two weeks have been so rough for Ali.  Thank you all for your healing thoughts and prayers.  They have been well received and she really needs them.  She now has a pretty neat scar down her chest that we are very proud of.  I will post pictures once she’s able to lose some of her leads and plastic accessories.  Poor kiddo has been through more in these few weeks than many will endure in a lifetime.  The fact that she is already smiling shows the magnitude of strength in the human spirit.  I’m so proud of her.  

I’m also so proud of Bailey! Poor kid has been living out of her backpack for two weeks being bounced around back and forth between my mom’s house and her dad’s.  Every time she visits she shows only her unselfish concern for her little sister.  What an adaptable kid, and if you don’t mind my bragging I want to mention that she got all A’s on her midterm this semester (!!!!!!!!!!!!!!!!!!!!)!

I can’t wait until we can all be home again.

~Melissa

night before

Ugh, this is tough.  We have been here nearly a week now and for the most part it has been HARD but not bad.  It's stressful seeing my sweet baby with oxygen tubes and a variety of leads taped to her, but once she got used to it so did I. It's scary when alarms go off every time she desats.  Its really scary to see her numbers drop to the 30s.  I can't imagine what that must feel like for her.   Until now I dreaded the idea of heart surgery.  I know she's ready for it and I'm ready for her to get better. 

Today was probably the hardest day since we have been here.  She needed her pre-op blood-work drawn which is extremely difficult for a baby like her. Not only does she hulk-out, but she's chubby and her veins are hard to find.  Another challenge is that her blood is thick and clots quickly rendering some samples obtained useless. She's had several rounds of pokes today and it was rough.

Like I said, it has been really hard but not bad.  When she desats, its just momentarily and then her levels climb back up.  She has been tested for viruses and infections, all of which have been negative.  Since being here she has reached new developmental milestones.  We are blessed that our experience here has been good.  I wanted to share this video.  I think it shows how amazingly resilient these kiddos are.  Ali will overcome this with squealing laughter.

I really appreciate all of you who have been following our journey and keeping Alessandra in your thoughts.  Tomorrow around noon she will have her heart surgery and I would really appreciate your prayers. 

Thank you <3
~Melissa