Monday, November 18, 2013

October 2013... 2 1/2 years with Angel Lungs, Trip to Texas, Canceled Make A Wish Trip & Paraflu

October was a busy month for us. We traveled to Texas for her 2 1/2 year post transplant checkup. I am still amazed by the miracle provided to us. 2 1/2 years post transplant! I still worry all the time about Elizabeth's future, but I remind myself to look at how far she has come! Before transplant, I feared she would not see her 1st Birthday, and now are are quickly approaching her 4th. We are blessed with each and every day we have with her. FOREVER grateful for an amazing God, the Ott Family, medical professionals, and the unwavering prayers/support from all of you!

Lungs - 
 We meet with transplant and everything looks good with her lungs at this point. They did not do a biopsy this time, so again we still have no definite idea on rejection other than she is showing no symptoms, and her lungs look healthy. So unless she shows a decline, they don't plan to biopsy.

Growth/Development -
The transplant team is happy with her current growth and development. We know she is still small for a 3 1/2 year only weighing about 28 lbs. But as long as she is growing and making progress, everyone is happy. Developmentally she is doing really well, talking alot more, wanting to be more mobile and walking when you hold her hands or she holds on to furniture to get around. We are hoping to start working with a walker soon so that she can become more independent.

Heart - 
We did meet a new Cardiologist during this trip, one who has experience and conducts research in aortic root dilation patients. So we now have a better understanding of Elizabeth's current heart issues. We have known since her birth that she has aortic insufficiency, her aortic root is dilated, and she has a bicuspid valve. Once she was diagnosed with FLNA mutation, we learned that all of these issues, along with her lung problems, low muscle tone, and the brain changes are part of this condition.  The cardiologist explained things more in depth and we have a better understanding of what the "plan" is for her future. Basically with the aortic root dilation Elizabeth's is currently measuring at 2.5 ( 2 1/2 times larger than what it should be) when/if it gets to 5 she will need to have surgery to have her aortic root replaced. They increased her heart medication to hopefully help slow the dilation down. She will have a cardiac MRI during our next trip in April, to compare to her last one in December 2012 to get a rate of dilation so we have a better idea of how fast this is progressing. We are hopefully that it is a slow progression and she has time to grow and get stronger before she needs this type of surgery, or be lucky enough to not need it. So overall, not the best news ever to have to think about. But we are SO thankful to have a new cardiologist who realizes the importance of monitoring her closely and ensuring that we are doing all we can for the best outcome with her cardiac issues. I have been so concerned about the lack of follow up and concern with our last cardiologist, I left this appt feeling so much better about her care now.

Her airway issues are still a bit concerning but we have decided, and transplant agrees with us that as long as she is showing no problems we will hold out on any surgical interventions for now. The risks just seem to great for a surgery that may not fix her airway. So for now, she will continue to use supplemental oxygen during sleep and will most likely always have a harder time during illness due to her airway issues.

Which brings me to our cancelled Make A Wish trip and the paraflu. We were set to fly out for Disney World on Sun Oct 20th. On Saturday around midnight Elizabeth woke up crying, her heart rate was elevated, and she was running a 101-102 temp, her oxygen levels her a little lower than normal. We called our wish coordinator and cancelled at 2am before our scheduled flight at 8. Our coordinator was so sweet, and understanding. We are hoping to reschedule to trip for spring once the baby gets here. Over the next few days Elizabeth's oxygen levels were lower and she was requiring oxygen during the day and night time at an increased rate. She had fevers up to 102, just felt and looked awful. We had a flu/rsv and viral panel done, which showed she had Rhinovirus (common cold) and Paraflu 3. Since they were viral there isnt really any treatment other than just comfort care, tylenol, extra oxygen and fluids. She was started on antibiotics in hopes of keeping it from turning into pneuomia. She was sick for about 3 weeks, Matt and I were pretty scared during this time, we went to the Pediatricians office 3 times and the Pulmonoligist once. She came close to having to have a bronchoscoy to check for any issues, since it was taking her so long to recover from the viruses.  Thankfully she was able to stay home and not have to be admitted to hospital. She went back to school last week, and her oxygen levels are back to normal now. We pray that this virus hasn't caused any damage to her angel lungs! Germs and illness are always such a big concern for us.

Sick on Halloween - No trick or treating but she still dressed up :)
She is ADDICTED to the Ipad now

We want her to have normalcy and be able to go to school, play with other kids and enjoy this gift of life she has been given. But there is a very thin line that we have to walk with her. Every illness she has could damage her lungs, and lead to rejection. Some people don't realize how serious any illness is for her, and we try to educate everyone around us about her. So please, know that if I question you, or tend to tell you about it alot, its only to protect Elizabeth.  We explained all of this again to her teachers at school and they have been doing a good job with making sure her hands are cleaned and we are notified if any of the other kids are sick. And our family/friends are always so great with staying away or letting us know if they have been sick. We appreciate everyone for all you do to help us make sure that Elizabeth stays as healthy as possible!

We hope all of you are staying healthy and can enjoy the upcoming holidays with your families. We hope to enjoy the holidays at home celebrating our Miracle Girl and soon the arrival of her little sister :)

Sunday, September 15, 2013

Thank You Complete Training and Team E

I continue to be amazed by the people God has placed in our lives along this journey. When we returned from Texas after Elizabeth's transplant in 2011, I started doing personal training at a local gym in an effort to try and get rid of some the weight I had "collected"  from my pregnancy with Elizabeth and the almost year long hospital stay after.. I was very lucky and found an amazing trainer Will, who took the time to listen to our journey and seriously has become almost like a counselor for me. He was very understanding of our situation, and allowed  and encouraged me to bring Elizabeth with me to the gym when I needed too. He always takes the time to play with her, and treats her just like any "normal" kid. The co-owner/trainer Kyle is also so great with Elizabeth when she is there. They help give me some normalcy with being able to do something as simple as go the gym, which isn't always easy with a special needs child.  And over the past year, I started taking Zumba classes at the gym as well, and have become friends with our awesome instructor Jodi and many of the women in our class. The gym recently started building a new facility, and Will asked me about doing a charity event to raise money for Elizabeth's continued medical expenses as part of their grand opening. We were so thankful for Will, Kyle, and Jodi who made their big day, a blessing for our family. We just want everyone who attended to know how grateful we are and how much you mean to us. Even though we aren't currently in "crisis mode" this journey is still not an easy one. And the love and support you continue to provide us means more than you will ever know!  I have definatley learned over the past 3 years, that God places people in your life for a reason. 

Some of the Team E members after our Zumba Class
Kyle & Will at Grand Opening

And of course, I have to add in an update on our miracle girl. Elizabeth is doing good with everything. She has started eating more and more by mouth, talking ALOT more and in general being a funny, spunky 3 year old with attitude :) Last week she had an orthopedic and cardiology appointments. Ortho didn't exactly have the best news. Her hips are still not going into socket the way the need too. So the Dr thinks sometime within the next 2 years she will need to have surgery to help place her hips in socket where they need to be. For now we will continue to work on weight bearing/standing/walking. We are hoping to get her a walker ordered within the next month or so to help give her more stability. Cardiology didn't have alot to offer, Elizabeth fought like crazy this time during the echo. So they were not able to get any good pictures of her heart. After discussing everything with the Dr, we plan to try and transition cardiology to Texas. That way if there are any major issues they are handled at the hospital that knows her best. And since her FLNA mutation is so rare and TCH seems to be leading with diagnosing it, it makes sense to try and be where the most knowledge is.   So we are hoping the remainder of September is good for Elizabeth. October will be a busy month for us. We travel back to Houston for her 2 1/2 year lung check up and then we will be heading to Disney. Elizabeth is being granted a wish trip through Make A Wish, we are excited for her to have a trip that doesn't involve Drs or hospitals. She has been saying "mouse" alot lately, so  I think she is looking forward to it. Thank You for your continued prayers and encouragement!

Could you still smile like this after ALL she has been through??

LOVES Oreo Cookies

Bananas are a new Favorite

Monday, August 26, 2013

Texas Trip.. Learing about our Donor Family and Surgery Options

Our trip to Texas was definitely filled with emotions this time. We are back home in TN now, as we decided to wait and think about the surgery more before making a decision.

On our 1st day of appointments, Elizabeth had her monthly bloodwork done and we met with the Transplant Team.  Back in April, I had written a letter to our Donor family expressing our gratitude for their decision to donate and saving Elizabeth's life. I realized when I wrote there was a chance we may not hear back from them. But I really wanted them to know how important they are to us, and what a difference this gift is making in all of our lives. During our appt with transplant, our coordinator gave me a letter from our Donor mom. I was so nervous to open it, but so happy to hear from them and know that they had received our letter. So as we sat in the waiting room waiting for Elizabeth's CT scan, Matt and I read the letter together. As we were sitting there worried about Elizabeth's pending procedure, this letter was a reminder of hope, a reminder of what has gotten her this far.  Our donor mother, Samantha and I have been on contact via Facebook and I asked her permission to share some information about Elizabeth's angel with you all. Elizabeth's angel is a 4 month old little boy named Elijah, he was born Dec 23, 2010 just days after we were transferred to Texas to start Elizabeth's wait for the "perfect lungs".  I have seen pictures of this precious boy, and it still amazes me that his family was able to make such a huge decision during their time of loss. We are and will forever be grateful to them. We truly hope that learning about Elizabeth gives them some comfort in knowing that Elijah continues to make an impact on others, even from heaven.

As for the remainder of Elizabeth's trip/appointments:
After the Bronchoscopy
CT Scan - We learned that she now apparently has a reaction to contrast. She broke out into a red rash all over, but thankfully it went away. But now she will need to predosed with medication prior to future testing. The CT scan showed that her aorta and innomiate artery are both enlarged and that is what is causing the major compression of her airway. Enlarged vessels/arteries are one of the complications/concerns with the Filamin A mutation she has. Scan also showed there is some slight compression where her trach used to be. Her bronchoscopy showed the same issues and gave a different view of the compression from inside her airway. We were able to look at all these images as the doctors explained them. We knew of these problems from all the previous testing at Vanderbilt but just never had such "good" images to really show us how bad the compression was.
After her CT Scan

So we were given 4 options with regards to treatment/surgery and the risks/complications:

p1~ Do nothing, continue with supplemental oxygen during sleep and hope that she doesn't get worse or run into any complications. The doctors all agreed that this is not the option they prefer. They feel something needs to be done to correct the problem. The biggest concern with her airway collapsing is that it can cause air trapping in her lungs, basically killing parts of her lungs and decreasing her lung function. With Elizabeth we need to preserve these angel lungs as long as possible! The survival rate at 5 years is less than 50% - which they reminded us of during this chat. So we need to be sure we do everything we can to not cause damage to the lungs.
2~ Replace the trach, it will keep her airway open and provide peep to keep her lungs open. The downside- due to the innomianate artery being dilated the trach could rub on it causing it to erode over time, leading to internal bleeding. And the major risk of infection having a trach causes could potentially damage her lungs more. This option was also voted "not the best option."
3~ The option that the Doctors recommended most - Basically relieve the compression on the airway by surgically replacing part of the innominate artery with a graft and moving the position some so it was no longer on the airway. The graft would be smaller and possibly out of the way. Sounds easy enough right. The downside, since during transplant the Dr lifted her aorta to attach it to her sternum in hopes of relieving the compression that now makes it difficult to open her chest up. Basically he said the hardest part of this procedure would be ensuring he does not cut her aorta when he opens her sternum. Replacing part of the innominate artery would mean affecting the bloodflow to the right side of her brain/arm and potential for stroke. The possibility that these dilated arteries will not "hold" a graft and start leaking. The fact that she is immune suppressed and healing could be difficult for her. And the potential for needing to go on heart/lung bypass during the procedure causes other risks. Add to all of that, that this may not fix the compression. So basically this was our "best option" with some pretty serious risks. THEN, once she recovers from this major surgery, possibly have another surgery to fix the floppy area where the trach was by removing a piece of her rib and placing it in the area to strengthen and hold it open.
4~ Our last option which is definitely the last resort, would be to completely remove the floppy/compressed parts of her trachea and to try sew the remaining sections together.  And I wont even go into the risks of this one, since we basically have no plans to consider unless we have no other options left.

So, if you've made it this far in this post, you realize that this decision is NOT an easy one for us to make. The risks are very big with such a large procedure and the chances of something going wrong are pretty significant. Matt and I just felt, now was not to the time to decide and the Dr told us it was not emergant that we decide during this visit, that we could take time to think and discuss it. And of course, since this mutation she has is pretty rare, there aren't any cases to really look at and see how well this procedure has worked in the past. So we wanted to come home, spend more time with Elizabeth, talk to our families about it and hopefully find some guidance. Elizabeth also has an appointment coming up with Cardiology, and I really feel we need to check the status of her heart and artery dilatation with him to be sure there aren't any other issues going on that may need to be resolved/checked first.  And then we will follow up again in Texas in October with transplant for her lung check up. So, all that to say, please pray for guidance on this decision for us, and continued stability for Elizabeth's airway. And of course that her angel lungs continue to do well. Thank you to everyone who called/texted/messaged us during this trip. It was definitely the most stressful and worrisome since transplant for us, and I think Matt an I both are really concerned about making the best decision for Elizabeth.
Excited to be home with her toys/books~ I let her destroy the room :)

Sunday, August 11, 2013

Hope and Fear

Just wanted to do a quick update on things around here. Most of you family, friends, and those who I am friends on Facebook with already know our big announcement. But we are expecting another little girl in January 2014. We put a lot of prayer, thought and research into our decision to have another child. We met with the genetics Doctors at Texas and a genetics counselor here at home and were told the chances of Elizabeth's condition reoccurring were 1-3%. Due to Elizabeth's genetic mutation being X-linked, and me not having any of the genetic abnormalities they think the mutation Elizabeth has was sporadic. We realize that life with Elizabeth can sometimes be difficult with the care she needs but we also realize that life must go on for us and Elizabeth. We can not live in fear of the unknown (easier said than done!) But have faith that God will give us no more than we can handle. And that is how I am handling this pregnancy and the fear I feel daily. I am excited to be a mommy of 2, and even more excited for Elizabeth to have the chance to be a big sister, and for this baby to meet, and love Elizabeth just as we all do. So with all of that, we went into this pregnancy more educated than when I was pregnant with Elizabeth. In an effort to be as prepared as possible, I decided to have an amniocentesis done with this pregnancy, I had one with Elizabeth and was assured the results were normal. But now we know what genetic mutation to look for specifically. So last Monday I was seen by the high risk doctor here in Chattanooga. They did an anatomy scan, and the amnio on the same day. Based on the ultrasound this baby appears normal and healthy, there are NO abnormalities visible like the ones we saw with Elizabeth. The results from the amnio will take a few weeks to come back, but we are hopeful for only good news. We chose to do these tests so we could be best prepared to care for our little girl, regardless of the outcome we will love her just as much.

As for Miss Elizabeth, she is doing so amazing! She has continued to make progress with saying more words. And has started trying to walk when you hold onto her hands. She really is making the most of those angel lungs :) She started her 2nd year of preschool last week in a new classroom. Thankfully most of the same kids are in the class, just new teachers. Every milestone she meets truly another blessing for us, there were many days I worried she would never make it this far. We head to Houston this week for testing and possible airway surgery. Matt and I both are so unsure of what to do, what decision will be best for Elizabeth. We are hoping with more information from the tests and the consultation with the surgeon, we will have more information to make this decision. We want what is best for her, sometimes I really do hate having to make these decisions that affect her life so much. I know thats part of being a parent, but sometimes it is a lot to take in - having to make choices that could mean life or death for your child or choices that could totally affect their quality of life. But with all the other decisions we have had to make, I try to remind myself that not matter what WE decide, someone bigger already knows the outcome.

I will update more as we learn more during our trip to Texas, and as we get the results on Baby #2. Thank you for your continued prayers and support! We will definitely need them as we embark on this new stage of life with Elizabeth!

Tuesday, July 9, 2013

Gearing up for a trip to Houston

In my last post I mentioned that we were and have been waiting on decisions regarding Elizabeth's airway issues. We will be going to Texas in August for a repeat CT angiogram, rigid brochoscopy and consult with surgery, ENT and the transplant team. They will give us our options for airway surgery, and if we agree to the it she is already scheduled for surgery while we are there in August.

We are scared, nervous, and worried about the potential surgery. Not knowing if what they recommend will help or how recovery from this surgery will go for Elizabeth, if we chose to do. The advantages and quality of life changes from having a successful surgery would mean that the opening in her airway where her trach used to be, can finally after almost 3 years be closed. This means that Elizabeth would be hopefully no longer need oxygen during sleep, she could breath freely and unlabored, she would burn less calories helping her grow. She will able to participate in water play at school, go swimming, play in the sand, take a REAL bath and be able to play and splash in the water like all the other kids do and hopefully be able to do aqua therapy which would help strengthen her muscles. This could be a chance to add a little more "normal" to her life. But the risks of such a major surgery are also very obvious, she is immune suppressed, will have to be sedated/paralyzed for part of recovery. The risk of infection and just being in a hospital again scare me to death. She is getting older and I really think she understands alot more of what is happening and I know she will be scared and it breaks my heart. We are unsure how long recovery will be for this surgery but were told to plan for a couple of weeks. The thought of being away from home again, and not knowing for how not a good feeling either.

Please pray that we are able to make the right choice for her regarding this, and that the Surgeon and Doctors can be confident in their treatment recommendation. We still don't know for sure what options will be given to us but we want to do what is best for Elizabeth.

Saturday, June 15, 2013

Again I am totally slacking on updating this blog. Life has been busy for us!

April was a rough month for Elizabeth, she got pretty sick for about 3 weeks, needing oxygen 24 hrs a day, vomiting and fevers. She ended up having metapneumovirus, its pretty similar to RSV. We were lucky that she was able to stay out of the hospital and recover at home. As with any illness/infections/virus she gets our biggest concern is that it can cause her to go into rejection or cause damage to her lungs. Some illnesses/viruses can cause rejection immediately (acute) and others can just cause long term damage which leads to chronic rejection. Thankfully we are not seeing any immediate damage from this illness, but only time will tell with long term damage.

 We traveled to Houston for her 2 year check up~ AMAZING I know. I cant believe it has been 2 years since she received her gift of life. Her checkup went pretty well, it was a busy week for sure, she was seen by ENT, Neurology, Genetics, PT, OT, Social Work, Nutrition and the Transplant Team. She had a CT - angiogram, labwork, and a brochoscopy done while we were there.  Her lungs seems to be doing well, and she is showing no signs of rejection at this point, which we are very thankful for! Her airway is still the main concern, and based on previous discussions with Texas and Vanderbilt we have decided to pursue treatment at Texas instead of Vanderbilt. We have been waiting for all the doctors to meet and discuss a plan for her. Last week I received a phone call that she will need a repeat CT-angiogram and rigid brochoscopy done in Texas. Once that is done, the Surgeon can make a better decision on how to move forward with any type of airway reconstruction. We are scared, but ready for "this issue" to be treated and resolved. The biggest fear with her airway being so floppy is that if she gets sick like she did in April it could cause her airway to close off. So for now we are waiting for a call back with an appointment date, hopefully within the month to travel for these tests.

 April was also the month we celebrated Elizabeth's 2 year anniversary with angel lungs, as well as remembering her donor and their family. We celebrate the gift of life she was given, and the milestones she has been able to accomplish all because a family in their darkest day choice to give her life.  We were blessed to share the day with our family/friends and again this year released butterflies in honor of her donor.

  Developmentally Elizabeth continues to make progress. I wont lie there are days I get jealous of other kids her age and even younger who are doing things that she can't do yet. But I remind myself she is alive, and progress will come all in "Elizabeth Time". She has started saying more words, and still uses sign languae and can communicate her wants/needs pretty well. She is now pushing/pulling to stand pretty frequently. Her favorite place to stand is in front of the TV, watching Dora. She will stand for long periods of time and ask for help when she wants to sit back down. She is side stepping some while holding onto things. So we are hopeful that she will be able to walk within the year. She is doing really well at preschool, playing with her new friends, playing on the playground at school and doing lots of big girl things.

Elizabeth at school :)

There are days when I just watch in amazement at the little girl she has become. She is truly a miracle and we are so thankful for her. Everyday I remember and "relive" some of her days in the hospital when she was so very sick. It breaks my heart to look back at those times, but it keeps me grounded in the now, knowing that any given day we could be back there. We have to cherish today, and make the most it!

Wednesday, April 3, 2013

Worry, Fear, Excitment and Progress

I havent updated in a very long time! But Elizabeth continues to amazing, I am sure no one ever doubted that :)

I think the last time I updated was in October so here is a brief rundown of the past few months:

November: I was gone on active duty with the Navy for 4 days at the beginning and then 20 days at then end of the month.  My first time being away from Elizabeth. It was hard, but of course Matt did awesome taking care of her. Also I ran a 1/2 Marathon in November for Erlanger Children's Foundation, raising money for one of the hosptials that cared so much for her in the beginning. Family and Friends worked a water station in her honor, and promoted Organ Donation Awareness.

December: Right after I returned from active duty, Elizabeth had a seizure. It was 4am and Matt woke me up. Elizabeth still sleeps in our bed due to the oxygen and monitor needs during sleep. He felt her moving/shaking and thank God it woke him up. We were nervous because she seemed to seize for so long, so we called an ambulance. Our first 911 call (and hopefully last) throughout all of her illness! We know that the FILAMIN A mutation can cause her to eventually have seizures but it looks like her blood sugar was the cause for this one. We spent one night inpatient, but it required an IV in her head, due to how quickly they needed to get her blood sugar back up, it was below 20. She has been doing well since. And of course Christmas and then her 3rd Birthday, which were both pretty great. This year she started realizing how to open gifts, so it was nice to see her understanding a little more. We also spent some time at Vanderbilt, Elizabeth had more testing done to look in the airway reconstruction surgery.

 January: After Elizabeth turned 3 she transitioned out of the Early Intervention Services and fell under the County School System. We had multiple meetings and thankfully she was able to stay at Siskin Children's Institute. She transitioned to 5 full days of preschool instead of 3 1/2 days. It was more of a challenge for me than for her :)  Matt and I had our first trip away from Elizabeth together. And we went to Houston, I know of all places to go! I ran in the Houston 1/2 Marathon in honor of Elizabeth and to raise Awareness for Organ Donation. I was lucky to have with me one of her nurses from TCH PICU and the former PR person from LifeGift, who helped me share her story when we were waiting in Texas. It rained the entire time, and the first song on my Ipod when I started running was "Bring on the Rain, by Mercy Me".

 Bring me joy, bring me peace
Bring the chance to be free
Bring me anything that brings You glory
And I know there'll be days
When this life brings me pain
But if that's what it takes to praise You
Jesus, bring the rain

 It reminded me of when we started waiting for Elizabeth's lungs in Jan 2011, and the way I felt the whole time we were waiting. I learned alot of myself, and my faith during that time and I continue to learn now. Elizabeth is brought me closer to God and sometimes I think maybe thats part of her purpose in my life.

 We are still unsure of what needs to happen and it will be discussed further at our appt in April in Houston. She underwent 3 procedures to check her lungs, airway, and GI system.

February: Nothing major other than her FINAL IVIG infusion for the winter. She was having to spend one day month at the hospital for the infusion after getting labs drawn. She HATED it because of having to get an IV after being stuck for labs. She will continue to get labs drawn and if her IGG level is low or she starts getting sick we will restart the infusion. But we are hoping she wont need them again, at least till winter. She also randomly started using the potty, we are hoping that she will eventually get potty-trained, of course all on "Elizabeth Time"

March: Elizabeth started standing on her own! Another miracle, and its so amazing to see! We are thankful for this blessing and know how hard she has worked and continues to work to make this progress.

April: National Donate Life Month ~ a time to remember the gift of life that was given to Elizabeth and continue to raise awareness for the need for Organ Donors. We encourage EVERYONE to let your family know of your decision, share Elizabeth's story and save someone's life!!

I wont lie, the past few months have been emotionally draining for me. I tend to hold in all the emotions of the past 2 years until... I just can't. I am so thankful for ALL of you who support us, and continue to follow us on this journey. I know everyone else has issues, and problems in their lives as well, but you continue to "be there" for us. You will never know how grateful we are for the prayer, encouragement and love. We are blessed to be chosen to care for and love such a special little girl. She is reminding me daily, to keep the faith and never give up!