More surgery, seriously....

Today Lily had a sedated MRI and an Echo.  Her head circumference has continued to increase to the point that she was in the 97th percentile at her 6 month well-check yesterday.  Today it was confirmed that she has hydrocephalus.  Her MRI today showed what could be a subarachnoid cyst.  They are not sure if it is a cyst or if her third ventricle is just bulging from fluid.  They also think that hydrocephalus could have developed secondary to her VSDs.

With a VSD, there is additional pressure put on the right side of the heart.  The increasing pressures can result in increased fluid in the lungs and also increased pressures in the blood vessels throughout the body.  If there is increased pressure in the blood vessels, it is hard to remove fluid from the tissues.  This could happen in the brain resulting in extra fluid in the brain.

Whatever the cause, it needs to be fixed soon.  Lily will be having brain surgery on Tuesday or Wednesday to place a shunt.  This shunt will go from the ventricles in her brain to her abdominal cavity.  The extra fluid will drain into there and be absorbed and removed.

Since they think that the increased pressures in her heart might be the culprit, that also means that heart surgery isn't too far in the future.  We haven't seen the official results yet, but I am pretty sure that Lily is going to be referred to the cardiac surgeon to have her VSDs repaired.  The reason they are doing a shunt and not an alternative hydrocephalus treatment (third ventriculostomy) is because they don't want to delay her heart repair too long (at least that is what they told the neurosurgeon.

My heart is breaking for my sweet girl.  I don't want her to need more surgery but ultimately, we will do whatever she needs us to do to be well.  In PT and OT this week, she wouldn't even lift her head during tummy time because it is so heavy from fluid.

Yesterday, Lily had her 6 month check-up.  She received her vaccinations and showed off for her doctor.  She now weighs 12lbs 4oz (below the 5th percentile) and is 24 inches long (5th percentile).  We go back to him in November to get her RSV vaccine and 2nd flu shot.

Now I will leave you with some pictures of my sweet Lily

First Rice cereal

I wish I could make this better

"When it rains, it pours" seems like the theme with Lily lately.  Outwardly when you see her you would think that absolutely nothing is wrong.  It seems like people don't take her precarious health situation seriously because she appears so "normal" and happy on the outside.  She is seriously the sweetest, calmest and happiest baby I have ever been around.  I want people to treat us normally but I still want everyone to understand just where she has come from and where she still has to go. So where do we go from here.....

Lets simplify this and split it into organs/systems (The professor in me writing a blog):

Lungs
Last time I updated she was coughing and wheezing which the pediatrician attributed to reflux.  Since that point, Lily has had her first ER visit because everything got worse.  She had a chest X-ray to rule out pneumonia and reherniation, which were both ruled out.  They concluded that she had a respiratory virus and just needed some albuterol treatments.  Two days later she still was not breathing well so I took her back to the pediatrician. He gave her steroids and referred her to the pulmonologist which is where she went Tuesday.

Her pediatrician's initial diagnosis was correct and it is her reflux that is causing all of her coughing, wheezing and vomiting.  The reflux is aggravating her airway and causing reactive airway disease.  Now armed with new inhalers and a new reflux med, hopefully this will clear itself up.

Heart:
We had a cardiology visit today.  She is gaining weight (now 11lbs 11oz and barely on the growth chart) but not at a rate that the dr wants to see.  When she has her sedated MRI next week, they are taking advantage of that and doing the 2 for 1 special and doing an echo at the same time.  If her Echo shows enlarging of her left ventricle, then he is referring her to the cardiac surgeon.  We will find out results next week right after she has the MRI.

Brain:
She is scheduled for her sedated MRI now on October 4.  We will find out results on the same day and meet with the neurosurgeon.  Hopefully she doesn't have hydrocephalus but if she does, then she will require brain surgery.  The two options are a ventriculostomy or a shunt.

If she needs both the heart and brain surgery, then the brain surgery takes precedence.  If she only requires the heart surgery, it will probably occur within a month of the referral.  Either way, everything is going to be decided before the end of the year.

I am so scared for my baby girl.  I don't want her to have any more surgeries, much less possibly two.  Please pray for her heart and brain so that she doesn't require surgeries.  Please pray for strength for us and we continue on this journey that Lily absolutely does not deserve.

On a happy note, her therapies are helping her so much.  Yesterday she rolled from her tummy to her back for the first time!

I know by this time next year that this will be a distant memory.  I just want what is best for my baby girl.

The journey is not over

I thought that once we got Lily out of the hospital that things would be "normal" so to speak.  She didn't need a feeding tube and was taking full feeds by mouth.  She only needed oxygen for a little while.  She is on minimal medication and has no signs of pulmonary hypertension, which plagued her early days and was the reason for her needing ECMO.  As far as CDH babies go, we are pretty lucky.  However, Lily's journey is far from over.  Every CDH baby has a different journey.  That was something that I didn't fully understand before we started.

Among the specialists she has to see are neurosurgery and cardiology.  On her post ECMO MRI they found ventriculomegally (enlarged spaces in her brain).  During her initial head ultrasound when she was placed on ECMO, they found a resolving Grade 1 brain bleed (probably from cord compression at birth).  Because of these two things, she has been monitored by neurosurgery since she left the hospital.  Every 6 weeks we checked her head circumference and plot it on the growth chart.  From her last appointment in July her head circumference went from the 60th percentile to the 95th.  They are suspecting that she has hydrocephalus.  Lily is going to have a sedated MRI done on October 5th followed by an appointment with the neurosurgeon to decide what to do.  If its hydrocephalus, the two options require brain surgery.  I am so scared for my sweet girl.  Please pray that the MRI shows no hydrocephalus.

Lily also has two unrepaired VSDs and sees the cardiologist every 4 weeks.  Almost 3 weeks ago at her appointment, the dr said that if she didn't gain sufficient weight in 2 weeks (she had not gained in a month) that he was going to refer her to the surgeon to have her heart repaired.  Well Lily heard the threat and gained almost a full pound.  She is still a little bitty thing (11lbs 9oz at 5 1/2 months old) but she is feisty.  At her next appointment she will have another echo and chest x-ray to see if her heart needs to be repaired yet.  At this point it is not a matter of if but when.  Her moderate size membranous VSD is not likely to close on its own.

Lily has recently developed a cough so I took her to the pediatrician today.  He thinks that it is just worsening of her reflux.  However, if it doesn't improve over the next few days he wants to send her to the cardiologist sooner than her next appointment.  The cough she has could be a sign of heart failure from her VSDs.  The VSDs cause the left side of heart to push more blood to the right side of her heart which pushes more blood to her lungs causing fluid to accumulate in her lungs.  She takes a diuretic currently to help combat the extra fluid.  Please pray that we can wait longer before her heart needs to be repaired.

We also had her developmental screen on Tuesday and she is about 2 months behind in her physical development.  This is not surprising and its why I take her to OT and PT twice a week.  Please pray for big strides in development

Otherwise, when you look at my sweet Lily, you would have no idea she has been though and is still going through so much.  She is such a joy to be around and is always smiling.  We are enjoying having her at home and so is her big sister.  Now I will share some pictures with y'all!

Dr Big Sissy

 Visiting the SC aquarium

Family picture on the beach in Edisto


 Baby toes in the ocean

Beach baby rocking the bikini (you can barely see her scar)

Sweet sisters

 Big baby smiles

Beach girls

Beach cuties in the sun

Lily with her big sissy and cousin Caleb (also a CDH survivor)

Please continue to keep Lily in your prayers.  Also she has several CDH buddies that need continued prayer.

Home Sweet Home! (lots of pictures too)

Lily came home on June 11 after spending 73 days in the hospital.  Take that 50% chance of survival!  The last few days in the hospital were spent on feeding by mouth.  She was able to leave the hospital taking full feeds by mouth and without a need to tube feed!  She was discharged on oxygen and a monitor.

Since she came home we have been to multiple follow-up appointments every week.  I will give a run-down of everything.

Weight:
Lily barely weighs 10lbs today.  She left the hospital weighing 9lbs 8oz and in the 7th percentile.  Now she is 10lbs 1oz and in the 2nd percentile.  She has had trouble gaining weight since she left the hospital.  This is not uncommon with CDH but when you add in the VSDs then she just has a double whammy.  She was getting hindmilk with protein added but I was having trouble keeping up with the demand.  She is now getting breast milk with protein and formula powder added in to hopefully help her gain.

Lungs:
Lily is now off of oxygen as of Thursday.  She has an appointment with pulmonology next week so they can more closely monitor her lungs.  This appointment was not made when she left the hospital but  was a recent referral.

GI:
Lily tolerates feeds very well.  She has reflux but rarely spits up or vomits as long as we make sure her zantac dose is correct.  When the dietician added in the formula powder to her feeds she did have trouble with the particular brand they gave us.  We have since found a brand (Target gentle) that she tolerates well.

Heart:
This is our biggest area of concern right now.  Lily has both a muscular and a membranous VSD.  She has had two appointments so far with cardiology.  Right now she is on lasix because she was starting to accumulate fluid in her lungs.  This is common with larger VSDs (the membranous one is moderate in size).  The blood is shunting from the left side of her heart to the right side.  This is causing the right side of her heart to work harder and causing something similar to congestive heart failure.  That is why she is accumulating fluid in her lungs.  There has been no definite decision on whether or not it is going to require surgery to repair.  The cardiologist is keeping a close eye on her and will make the decision on whether it needs to be repaired before she turns a year old.  I really don't want my baby girl to have another major surgery so hopefully she won't need to.

Brain:
Lily sees the neurosurgeon as well.  On her post-ECMO MRI they found that she had ventriculomegally (the spaces in her brain that circulate cerebrospinal fluid are larger than they should be).  The neurosurgeon is monitoring her head circumference to make sure she is not developing hydrocephalus.  If her head circumference all of a sudden jumps percentiles and deviates from its current curve, then they will do an MRI.  So far everything is stable there.

Development:
Lily had a babynet (early intervention program) evaluation the other day.  She is not qualified to receive PT and OT through babynet because she is not considered developmentally delayed!  She is on the low end of normal but I am thrilled that she is not delayed.  The areas of concern were with her vocalizing and favoring turning her head to one side (thanks to ECMO).  Yesterday she started "talking" more and actually laughed!  I am going to get the pediatrician to refer us to MUSC PT/OT so I can at least get her some therapy.

Randoms:
When she is about a year old, she is going to have her ear tag removed by the plastic surgeon.
She is very social and loves to be talked to.  She especially loves her big sister.
Her favorite things currently are her playmat (she loves to try to pull the bird off), chatting with mommy, smiling at her big sister and goofing off with her daddy.

Now for the fun part: pictures!
Last day in the hospital

 Ready to leave!

 Leaving MUSC!

 Can you tell I am crying?  I wasn't sure that this day would ever come!

 In the car ready to go!  I cried almost the whole 15 minute drive home.

 Big sissy helps feed Lily

 Izzy loves her little sister

 First family picture at home

 Big sister wants to change a diaper

 And big sissy wants her baby doll to match her sister

 Goofing off

 Get the camera out of my face mommy!

 First bath at home

 Daddy and his girls

 Story time on father's day

 Mommy and her girls

And our family pictures done by my best friend Tish of Tish Hitesman photography (https://www.facebook.com/tishhitesmanphotography)

I promise to update more frequently.  She still has many things going on but is doing great overall!

Starting to see the light (Day 51)

I am liking this calm after the storm.  Lily is now off of CPAP and on a regular nasal cannula!  6 days after she was extubated and she is already on a nasal cannula.  This girl continues to amaze us and the doctors!  Over the course of the week she has gone from 1 liter of flow and is now down to a half liter.  From a respiratory standpoint she is doing phenomenally.

Her morphine has been weaned every day this week.  At the beginning of the week she was getting a relatively high dose every 4 hours.  The dosage was eventually decreased and then the doses were spaced out.  Yesterday she was getting a small dose every 8 hours.  Today that has been changed to every 12 hours.  If she tolerates that change well, the next step is once every 24 hours and then no more!  The next step after that will be to get her off of her twice daily ativan.  The last time they tried to change the ativan to once a day, she did not tolerate it well at all so hopefully this time around (since she is off fentinyl), she will do better.

We also started trying feeds by mouth starting with nursing before trying a bottle.  The first couple of days were awful with her absolutely refusing.  The third day she latched for 5 seconds and the fourth day she latched for a whole 30 seconds.  Hopefully if she can learn to nurse I can kick the pump to the curb.  She is continuing to tolerate her feeds well with very little, if any, reflux.  That might change once she is taking full feeds by mouth but we will take this right now.

A couple of problems have cropped up over the past week.  As a result of being on ECMO, she has a sore from where either a portion of the cannula sat on her head or from the sutures that were holding the cannulas in (not quite sure).  It was healing fine but the scab that was covering it fell off.  As a result, the skin was open and got infected.  She is on IV antibiotics right now so hopefully that will clear up soon.  It is already looking so much better.

Also, Lily is not gaining weight.  Her weight has been the same for the past 2 weeks.  She will make little gains, then she will lose and so on.  We are now trying a hindmilk intervention to try to get her to gain some weight.  It could be that since she has been extubated, she is now having to breath on her own and, thus, burn more calories.  Hopefully adding in the additional calories from hindmilk will help her gain.

She had a repeat echo recently to reevaluate her VSD.  From the echo report her VSD is still there and is moderate in size.  We don't know if it is going to close on its own or if this will be something that has to be repaired surgically at a later date.  The best thing is that she is no longer showing any pulmonary hypertension.  The PPHN is what landed her on ECMO so thank God that is gone.  Her right ventricle is still showing the side effects of PPHN with some hypertrophy (increased size) but it is mild.

We are also going to have some genetic testing done to see if she has some kind of microdeletion that could have caused the CDH.  She has a few things that could indicate some kind of genetic mutation.  She has CDH, a VSD, trouble gaining weight and an ear tag.  It will take a while to get those results back but I am hoping for no mutations!

Lily is also going to have an MRI this week to make sure she has no major side effects from being on ECMO since it can cause brain bleeds.  When they did her head ultrasound on ECMO she had a resolving grade 1 bleed but they think that happened at birth and not from ECMO.

If Miss Lily keeps amazing all of us and kicking CDH booty, we should have her home soon.  How soon, we don't know but there is a light at the end of the tunnel now!

And seriously, I will eventually get to updating her early days.  I still haven't even told the story of her getting off ECMO, surgery and early post-surgery.

Long overdue update

I really am the worst at updating a blog!  Now that the semester is over I should have more time to catch up on everything that has happened.

As of now here are the big things:

Lily has been extubated!  This happened on Wednesday (May 9) and she is now on CPAP.  She was on the ventilator for 5.5 weeks and is now breathing on her own for the first time in her life.  She is doing well on CPAP and they have started turning down the pressure.  Hopefully in a few days she will be on a nasal cannula.

She is also now off of her Fentenyl, which is a really strong narcotic.  As part of the weaning process she is on scheduled morphine that they will slowly wean down.  It is not good to completely discontinue fentenyl without a step down narcotic.

Lily is now on full feeds through her feeding tube.  We are going to start on feeding by mouth soon.  She started with occupational therapy this week and they said she is doing great.  They were actually surprised at how well she is doing.

Today they are going to pull her PICC line out because she has nothing else going through it.  All that she is attached to now are CPAP, her feeding tube, the leads that monitor her stats and the pulse ox!

For Mother's day she gave me big REAL baby smiles.  These weren't gas-induced smiles at all.

And finally a family picture

Big milestones!

Lily has had made some big steps this week.  Yesterday (day 25) they started feeding her!!  With all of her gastrointestinal tract previously being in her chest, she was not being fed because they needed her bowel to stay decompressed so it wouldn't compromise her lungs.  Since she had her repair last week, everything is now in the right place and settled in (surgery was a week ago yesterday).  They felt comfortable enough with everything to start feeds.  She will be getting 5ml every 3 hours for a few days to wake up her digestive system slowly.  Her first feed didn't go so well.  There was a residual left in her stomach that was almost the full volume of her feed.  However, her second feeding was great with barely anything left in her stomach.  So that means that everything is starting to work.  All of her subsequent feeds have been great with very little residual left.  Hopefully she will be able to tolerate that volume and they can increase her feeds.

Today (day 26), they did another wean on her ventilator.  She is going to have little weans done every day as long as she tolerates them.  So now she is taking baby steps to getting off of the ventilator.  Also today I finally got to HOLD her for the first time.  I have been anxiously awaiting the time when she would be stable enough for me to hold her and today was the day.  She had her chest tube (from surgery) taken out yesterday which was the last thing holding us back from holding her.  For 3 glorious hours this afternoon, I finally held my baby girl!  She did get upset at one point when I had a tickle in my throat and coughed.  I think it startled her and maybe hurt her a little.  Seeing her silent cry broke my heart.  If she could have made a sound it would have been really loud baby screams.  They ended up giving her morphine to help ease her pain.  She is already on a fentinyl drip but can get morphine to help if her pain gets worse.  Since that chest tube just came out, she is probably sore and she is still recovering from a pretty major surgery.  Once we got her pain under control she settled right back down.  With her being on the ventilator still we can only hold her once a day and for a minimum of 2 hours because of how hard it is to move her.  It took 3 nurses to move her and all of her "accessories".

Now here are some pictures from the past 2 days:

After her surgical dressing was removed

Holding my baby girl!!!

-Liz