Tuesday, December 28, 2010

We've Never Seen This Before?

So, how would it feel if the doctors around you say they have never seen this before?  Well, for Scott and I that is our reality with Emma’s condition.  Emma is a complex little puzzle and the doctors are working very diligently to try to put those pieces together to try to get Emma better.  Emma has seen several pulmonologists, cardiologists, intensivists, gastroenterologists, ear/nose/throat specialists, urologists, and nephrologists.  They have poured over her medical history and to be completely honest, are stumped by some of the ways she is responding.  They are not giving up on Emma and neither are we!  Emma has proved that she is a fighter, so we all battle on to try to determine the best plan for Emma. 

Emma is having nutrition problems.  This has been an area that we have struggled with from the beginning.  Emma drains fluid out of her chest cavity.  They have tried several methods to stop/slow down the drainage and have been relatively unsuccessful.  This drainage makes it hard to properly digest important nutrients.  They have just started Emma on a steroid regimen to see if it will help with some of her nutrient loss.  For those of you that know a little about steroids, you know that they are immunosuppressants.  Since this is the “sick time” of the year and this new medicine is known to lower immunity then we will be cutting down on visitors in Emma’s room.  We hope and pray that everyone will understand that this is in Emma’s best interest.  There might even be times when Scott and I can’t be back with Emma.  Emma should be officially listed on the transplant list this week.  Due to being on the vent and other heart meds, she should be listed as a 1A which is at the top of the list to get a heart; however, if she were to become infected then she would be temporarily bumped to a two which would prohibit her from getting a heart until she became uninfected.  So, as you can see, it is SUPER IMPORTANT that Emma stays well.  As hard as it will be to be so secluded, it is to get Emma better! 

Emma had a good day on Christmas and was a happy baby who enjoyed visiting with her company.  See recent photos below of Emma:

Emma's first Christmas!
Emma was visited by Santa!

Specific Prayer Requests:

1.     Pray that Emma can gain weight and nutrition
2.     Pray that Emma can come off the vent
3.     Pray that Emma can have good blood gases
4.     Pray that Emma can stay infection free
5.     Pray that the PERFECT heart will be generously given to Emma
6.     Pray that the Doctors can figure out the best plan for Emma
7.     Pray for the future donor family
8.     Pray for the doctors, nurses, and staff in charge of Emma’s care
9.     Pray for the other kiddos: Ella, Ella, Lyric, Blake, Justin, Haven, etc. who are fighting their own health battles
10. Pray that Scott and I can stay strong through this process

Thursday, December 23, 2010

The Journey Continues

It has been a while since I have updated the blog and for all those consistent followers, I apologize.  It has been busy not only around here but at work, and so now I have some free time to be able to update the blog and share what is currently happening in Sarah, Emma's, and my life.  So, as you may remember, we were going to start going through transplant evaluation.  Well, we have made it through everything now.  We had a lot of meetings with many new physicians and teams of people.  The transplant team checks everything to make sure that transplant is what is right for the person being listed.  The first consult/meeting that we had was with one of the main physicians and some of his team.  He basically just explained to us about what transplant entailed and what to kind of expect as the rest of our evaluation progressed.  We met with a psychologist, who I am guessing wanted to just see how we were doing through all of this and possibly assess how she thought we would do if transplant was the road that we would travel.  We met with child care services who asked us similar questions as the psychologist, but her services were more tailored towards Emma.  We met with neurology who did an assessment to see how Emma was progressing with normal developmental areas for her age.  He watched as she followed her purple elephant around in the dark, how she would follow Sarah and her voice around, and various other tests.  He said at the end that Emma passed with flying colors!!!  And as he was leaving, he waved bye at Emma, and (though probably involuntary) she raised her arm up and waved her hand back at him.  He said she was a "show-off!" Hehe!  :-)  She had a visit from pulmonology who took a look at her lungs.  To him, it looked like Emma was just weak and would need more time before she would successfully come off of the ventilator.  He said her lungs did not look to have a huge amount of lung damage which was really good news.  She also had a gastrointestinal tract test to see if her passage of fluid was obstructed or if anything was abnormal.  Everything from that test came back ok, which is good.  However, we still don't know why she is having trouble tolerating her feeds.  Possibly one of the medications she was on could have been contributing to that, as well as her heart not functioning quite like it should for her GI tract to work as well as it should.  They are still looking into this area.  So, today, they came in and said that they were going to go ahead and list Emma on the transplant list!!!!!!  As many of you probably know, that does not guarantee us a heart right away or anything, so we are going to be working on her main problem at hand right now, and that being the problem of nutrition.  Its very important for her to have good nutrition, not only for her health, but also for whatever path we go down, whether transplant or Glenn procedure.
Right now, Sarah is holding her and she is sleeping!  Such an amazing site to see!!!!  I love them both so much! :-)
So that is what we know right now.  We will keep you all updated as we know more.  We thank everyone for the kind cards and messages that we have received!  As always, I give all the thanks for Emma, the doctors and staff, and all of you, to God!  God is good, all the time!  And Sarah, Emma, and I truly hope all of you have a Merry Christmas!

Some specific prayer requests:
1)  Pray that the path that we are to go on all works out and that Emma continues to improve!
2)  Pray for Emma to get good nutrition and keep it!
3)  Pray for the doctors and staff here at Childrens.  They have many tough decisions ahead.  May God guide them in the way that will work best for Emma.
4)  Pray for Sarah and me and our families as we continue on this journey.

Sunday, December 19, 2010

My Not-So-Happy Baby

Just a Quick Update:

Well, Emma is constantly keeping us on our toes.  Many of you saw the video a posted on Facebook the other day of Emma smiling and very happy.  Well, yesterday my happy baby turned into a cranky baby.  Unfortunately the irritability is getting worse.  She has had several desat episodes (where her oxygen levels drop) and they have had to go up on her vent settings.  When I left Dallas on Friday to go to Oklahoma, Emma was getting to do pressure support trials on the vent (where the vent does not help her) and she was doing great, sating in the upper 80s and did not look in distress at all.  Today, they have upped all of her vent settings and she is slightly retracting when she breathes.  One of her blood gases this morning showed that her CO2 level was 97.  This is BAD!  They have had to give her several doses of sedation meds to get her calm.  We have not had to that in several weeks.  So, they are running every test possible on her to see what is wrong.  Her last x-ray showed more dilation in her stomach and intestines, so they have stopped her feeds for now.  She will be getting a chest/kub x-ray every 6 hours.  She will also be getting an abdominal and chest ultrasound.  They are culturing the secretions in her ET tube (vent), culturing her blood, running labs, and basically testing everything to try and figure out why she does not feel good and why they all of the sudden are having to go up on her vent settings to get the results they are wanting.  So, today my baby does not feel good and it breaks my heart to watch her in pain. 

We received word on Friday afternoon that we officially got insurance approval to proceed with transplant evaluation.  So, on Monday and Tuesday Scott and I will be in meetings all day to answer questions, fill out paper work, and be filled in on what this process entails.  We will be very busy.  We will update the blog on Wednesday to let you know what we find out. 

Specific Prayer Requests:

1.     Pray that Emma feels better soon!
2.     Pray that Emma will be able to come back to low vent settings.
3.     Pray that Emma can tolerate feeds and not have stomach issues.
4.     Pray that Emma will be a candidate for a transplant.
5.     Pray that Emma will receive a heart that is PERFECT for her.
6.     Pray for the family that will make the decision to donate.
7.     Pray that Emma has many happy days ahead.
8.     Pray for the doctors, nurses, and other staff in charge of her care.
9.     Pray that Scott and I will fully trust in God’s plan for Emma.

God is Good!  And, we will praise him in this storm!

Sarah (Mommy)

Tuesday, December 14, 2010

Update On Our Precious Little Princess

Family Picture Time!

Happy Baby in her Christmas Bow!
Well, it has been awhile since I have updated the blog and some things have happened since then.  Emma is doing good as of now.  She is happy most days of the week and loves many things including: her mirror, toys, and being held!  They continued to be concerned about a couple of things.  First, the are concerned with her lungs and coming off of the ventilator.  As you may remember, they think that her not being able to come off of the ventilator may be due to several factors including: her heart, her airway, her strength, etc.  So, they are trying many different approaches and have called in reinforcements to try and increase her chances of coming off of the vent.  They have called in the pulmonologists, which are the lung doctors, to take a look at her lungs.  What they are doing is giving her an inhaled medication called Pulmicort and seeing if this helps her lungs.  They are still doing lung conditioning and trying to cut down her secretions in her lungs.  They have been able to ween her down to very low settings, which is great and means that she is doing a lot of the work on her own.  The second concern is with her nutritional status.  Every time they go up on her feeds (i.e. her calories in her feeds) she doesn't tolerate the increase and starts having diarrhea.  As you can imagine, this is not a good thing since that means she is not only losing nutrition but also possibly losing meds since they are all given orally.  To try and figure out why this is the case, they have called in the gastroenterologists.  These are the physicians that look at the gastrointestinal tract to figure out if there is malabsorption of food, poor breakdown, etc.  They are still trying different ways to feed her to find out what she will tolerate best.

So what we know:
So what we know is that both of these concerns could be due to her heart.  And we are reminded about this almost daily.  This means that we are needing to get on the transplant list in case this is the cause of her problems.  However, we are having some trouble clearing it with our insurance company......  Hopefully it will clear very soon and we can continue down the road to getting put on the list.  They are not giving up on her heart she has now though since the cath of her heart looked pretty good.  So, we will keep trying to feed and grow her and see how she does.  If she doesn't progress like they would hope, then by that time hopefully we will be on the transplant list and be getting a new heart.

What to pray for:
1)  Pray for her to become stronger and get the nutrition she needs.
2)  Pray for her lungs.
3)  Pray for the doctors, nurses, respiratory therapists, etc. in charge of her care.
4)  Pray for Emma's progression to go in the right direction.
5)  Pray that everything will work out with this transplant, if it is the path that we are to go on.
6)  Pray for Sarah and me as we continue on this journey with Emma.

Somedays it is tough not knowing which direction Emma is going.  But, we have wonderful physicians and medical staff striving to get Emma well, no matter what direction that takes to get her there.  We are very thankful that Emma is doing well now though and is happy!  :-)

God is good!  All the time!  And all the time!  God is good!

Thank you for all the thoughts and prayers!

Daddy Scott

Gramma holding Emma for the first time!

Grampa holding Emma for the first time!

Tuesday, December 7, 2010

Plans Changed: Please Start Praying

Chilling in our bouncy seat for the first time! :)

Dear Family and Friends…….

It is amazing how much can change in just a few short hours.  We are constantly updated on Emma’s care plan and needs.  Today is no exception to that.  Today, during surgical rounds, we found out that perhaps, Emma’s heart/lungs are not performing exactly how they should.  Yesterday during the cath they found that the pressure in Emma’s ventricle when it relaxes is a 13.  Today, Dr. Forbess informed us that ideally that pressure would be a 6 or 7.  We also found out that Emma’s right lung did receive quite a bit of damage from the restricted atrial septum.  While this damage should not be unconquerable, it is hard to overcome when your heart is not quite functioning as it should.  While Emma’s heart is “fixed” like it should be, it is not relaxing as it should.  In a baby without lung issues, this would not be a huge problem, but with a baby like Emma, it can be.  So, what does this mean?  Well, basically this means that it is in Emma’s best interest at this point to be put on the heart transplant list.  This was somewhat shocking to us today.  We have known from the day we met Dr. Forbess that this was a possibility for us, but we really wanted to Emma to do well with the three-surgery process.  As we are reminded daily, what WE want is not always what happens.  The doctors are working hard to understand what will be best for Emma.  We are supposed to meet with the transplant team, cardiologists, and the cath lab doctors sometime this week to discuss this further.  Emma is stable and that is a good thing.  They will continue to monitor Emma and see what will be best for her, but at this point, all signs seem to point towards transplant.  Please keep us in your prayers as we take this next step.  Emma is such a fighter, but it is hard to know how to pray because getting Emma on a transplant list means that our potential gain is someone else’s loss.   Please keep the prayers coming. 

Mommy Sarah  

Monday, December 6, 2010

The Cath

The Cath went well.  Emma only went into A-Tach once and they were able to pull her out of it.  To give a little clarification, her heart looks good for this stage of her recovery. The right pulmonary veins are pumping more blood than the left but receiving less oxygen due (probably) to the drainage/wetness of her right side. Also, the left side had a little narrowing that they tried to fix with a balloon but it needed a stint, but Dr. Forbess wants to wait until the Glenn to fix it since it is really not causing any issues.  The drs. tell us that this was to be somewhat expected due to the restricted atrial septum in utero.  However, her heart is NOT in failure and now we just need Emma to gain weight, get stronger, and lungs to heal up.....the answer right now is TIME!  Thank you all for your prayers.  Keep praying, as this journey is not over yet.

To the CATH lab!

Emma will be going to the CATH lab around 1:00pm today to assess heart function. Please pray that this procedure goes well and that we will have some answers as to why Emma is not progressing as she should. Also, please pray she has no arrhythmias during the procedure.

Thursday, December 2, 2010

Still Trying to Figure Out Some Things

Recently when Emma was extubated, she failed very quickly and had to be reintubated.  This was very disappointing since the doctors and all of us thought she looked ready to come off.  Well today, they made an attempt to find out some more answers as to why she did not do well coming off the ventilator.  They took her down and performed a procedure to look at her airways and see if there is any swelling, obstructions, other problems, etc.  What they found out was that she had a little swelling but other than that nothing much really.  The OR people thought it was more than likely a lung related issue, not an airway issue.  So now, we are back trying to figure out what caused her to fail quickly.  We have yet to talk to the ICU doctors about what they think about these results, and what they think will be the best plan to proceed with.  This is disappointing to Sarah and me because we really don't know a whole lot more today after the procedure, as far as why she failed coming off the vent, than we did before the procedure.  Hopefully we will get some answers sooner than later.

Please pray for her lungs to heal if they are damaged and to improve so that she can come off the ventilator soon. 

Daddy Scott

Wednesday, December 1, 2010

Recent Happenings!

Recent Happenings:

Well, here is what is going on in Dallas.  As most of you already know, the doctors have told us that we will be in the hospital until the Glenn procedure (2nd surgery).  So, yesterday I went to Yukon and with the help of Mom, Jana, Stacey, Steve, Stephani, Patty, Amy, and Tony, we packed up our rent house.  They were a huge help and we got A LOT accomplished.  For those of you who offered to help…thank you as well.  This was my first time out of Dallas since September 11th.  It was hard being away from my little girl, but it was also good for me to get out of the hospital for a little while.  I even had the chance to visit with my “helpers”, sweet neighbor Brenda, and dear friends Kelli and Stephanie.  Oh, I cannot forget getting to see Skippy JohnJons!  She was very excited to see me. 

Emma has not had it easy these past few days.  They were able to get her heart arrhythmias under control, which is great.  They switched her back to a PO (by mouth) form yesterday and she seems to be tolerating well.  They have also switched her to a different formula called portagen.  (http://home.intekom.com/pharm/bm_squib/portagen.html)  which is a step up from the tolerex.  This formula actually has fat in it.  They are trying to get her up to 30kcals, but Emma keeps having diarrhea when the amount is increased above 24kcals.  Please be praying about this because she really needs optimal calories in order to gain the weight necessary to get stronger and bigger for estuation and the next surgery.  Also, diarrhea increases loss of oral medications, which we found to be very detrimental in Emma’s health. (e.g. A-Tach).   Also, they have had to go up on Emma’s vent settings since her extubation/a-tach episodes and we are unsure why this is.  Before extubation, Emma was on a rate of 4 (how many breaths per minute) and oxygen at 21% (room air).  Now Emma is on a rate of 20 and oxygen at 30%.  They are planning on taking Emma to the OR tomorrow around noon to do what is called a BRONC (http://www.webmd.com/lung/bronchoscopy-16978 ) This procedure will give them an idea about whether or not Emma has any airway issues that are contributing to her having problems coming off of the vent.  Please pray that this goes well and that we will have some answers as to how to help Emma come off the vent. 

So, to make a long story a little shorter, Emma’s heart function is doing very well.  Most definitely an answered prayer; however, there are problems that need to be fixed.  Emma NEEDS to come off the vent.  The longer she is on it, the more dependent she is on it.  We feel like we get really close to getting her off of it and then we take several steps backwards.  It is hard to watch her struggle with this and gag and cough on her tube.  Also, her feeds need to increase and be tolerated.  Emma is not too fond of touch and this breaks my heart.  We are trying to get to hold her more and do more with her, but she is 8 weeks old and has only been held about 5 times.  Imagine for a minute watching your baby cry, gag, struggle and touching her only irritates her more. 

I love my baby so much and want her to get better SO bad.  We need prayers.  This going “backwards” business is NO FUN!  It can be wearing on us.  Also, Scott starts working on Monday, so Emma and I will need to learn to do this without him on a daily basis and neither one of us is looking forward to that.  

God is Good!  He fearfully and wonderfully made Emma!  Although we cannot always understand his plan…..he has one!

Specific Prayer Requests:
Pray that Emma will tolerate higher calorie feeds and GAIN MORE WEIGHT!
Pray that Emma will be able  to come off the vent SOONER THAN LATER!
Pray that Emma will be more adaptable to touch and holding!
Pray that Emma’s oxygen saturations will increase on lower vent settings.
Pray that Emma will start having more “good” days instead of “no progress” days.
Pray that Emma’s heart will be free of arrhythmias.
Pray for Scott and I to stay strong in our faith and strength.
Pray the BRONC will go well tomorrow and we will get some answers.
Pray for the doctors, nurses, and other staff caring for Emma.

Pray for our friends Taylor, Ella, Ella, Emma, Lyric, Lane, Kaytee, Blake, Haven, and many others who our on a daily health journey.

Sarah (Emma’s Mommy)