Overdue Update for Bugga's 9th Birthday!

Whoa! I cant believe I haven't written an update since 2016!!! Using Facebook to update, living life - outside of a hospital doesn't leave as much time for long updates :)

      Today we celebrate Elizabeth's 9th Birthday!!! Most people close to us and those on the transplant journey all know what a big deal this is.  At transplant the statistics we were given was less than 50% survival at 5 years, she is now over 7 1/2 years post transplant. We don't take that lightly that she is still here with us and doing as well as she is!  The holidays are always a reminder to me of how sick Elizabeth was since it was December 2010 when we were transferred to Houston. December isn't an easy time since its a reminder of how close we were to losing her, how hard she has had to fight to be alive today but its always a time of celebration and remembering just how far she has come and how grateful we need to be for every single day. The holidays and her birthday are also a reminder of how our normal, isn't the same as other families. I sometimes let our lack of normalcy bother me and make me sad for her and our family. But then I have to remind myself that no matter what Elizabeth is loved, and she only knows this life, this is her normal.

  Elizabeth still has a feeding tube and uses it for all her nutrition. So the "normal" holiday meals and birthday cake aren't things that she gets to experience or enjoy. Last summer Elizabeth was also diagnosed with autism, she has alot of sensory issues related to noises and crowds. One of the major issues related her autism is her lack of sleep, she often only sleep 4-5 hours, which means one or both of us also only sleep 4-5 hours.  She also demonstrates alot of other autistic tendencies like flapping, repetitive behavior and over all just wanting to do her own thing. One of the biggest things is  Elizabeth's communication still mainly consists of repeating words/phrases and signing things she hears on her ipad. She can communicate her needs and wants to us, but she isn't conversational. For the most part Elizabeth is completely happy being at home just playing on her ipad, swinging or spinning around in her spinner seat. She loves just hanging out at home, and in all reality that is the best option for avoiding germs especially during the winter.  But at the same time, it makes me a bit sad that she doesn't experience life the way "normal" kids do. I am so grateful for all that she can do, but it makes me sad alot that she may never experience life the way Charlotte will. That is probably super selfish of me, but I would be lying to everyone if I didn't say it or type it out loud.


   An update on Elizabeth's medical status, as of her last checkup in October her lungs are still doing really well. Her heart is still being monitored for the aortic root dilation, she take a few different medicines for that each day and has Echos, EKG, and cardiac MRIs at her 6 mth checkups.  Over the past year her kidney function has started to decline, and she is now being seen by nephrology when we visit Cincinnati, she continues to have monthly bloodwork done and will have kidney ultrasounds at her 6 mth checkups. Kidney issues are not uncommon for transplant kids, the medicine she has to take to keep her body from rejecting her lungs can affect kidney function over time. It was something we aware of but had hoped wouldn't become an issue. For now we just have to monitor her and see what happens.  Another recent issue that was discussed at her October appts is that Elizabeth was diagnosed with precocious puberty. Since she is still very small for her age, there is a concern that going into puberty at an early age will cause growth issues for her. As she goes through puberty her growth plates will close which could result in her staying about the size she is now. (For comparison she and Charlotte wear the same size and are almost the same height and Charlotte is only 4!)  There is some concern as to what caused her start puberty so early, it could be the genetic mutation that has caused all her other medical issues, a result of all the medications she is on, or there could be something going on with her brain/pituitary gland. So she was scheduled for an MRI of her brain earlier this month, but we had to reschedule due to everyone getting a stomach virus. We are planning to go to Cincinnati in January to have the MRI completed. Once we get those results if there are no issues with her brain, we will discuss options for slowing down/stopping puberty or options for growth hormones. As always our main concern is making sure she is happy, and stays as healthy as possible to enjoy the extra time she was given with her angel lungs. We don't know what decisions we may be faced with soon, but we will continue to do what is best for her quality of life.

   Thank you to everyone who takes the time to read this, you are the family and friends who have and continue to support us on this journey. Please hug your little ones and be thankful for each and every moment!

5 Years with Angel Lungs

Wow, it has definitely been a while since I updated, which for the most part is a good thing. It means we Elizabeth has been happy and healthy.  And I don't have alot of  "free" time to blog with two busy little girls :)  This post is pretty much a recap of the last year and a report from her recent 5 year post lung transplant check-up. 5 YEARS - for those of you who have been on this blog from the beginning know that 5 years was a super scary number based on the statistics we were given at transplant. The past 5 years have been filled with fears, anxiety, loss of friends we have meet along this transplant journey and the constant question of what does the future hold. But it is also been 5 years filled with memories, love, laughter, and overwhelming pride in seeing our little girl do things we never thought she would be able to do.

           Some days it doesn't feel like its been 5 years, but at the same time the routine of daily medications, checking oxygen levels, making food for the feeding pump, ordering supplies, making dr appts, dealing with insurance is just "another day" here. I worried we would be overwhelmed with the care that Elizabeth needed but you just learn to adapt and it becomes part of our daily life.

     







 Elizabeth has had a pretty good year and has enjoyed being a full time student :) I can't believe her first year of Kindergarten is almost over! I am SO thankful for the teachers and principal at her school, they made this scary transition so much easier. Elizabeth has really taken to one of her teachers, Ms. Tracie, and I am so thankful for her and how much she cares for our little bug. It helps me to know that she has people who care about her so much, and understand our concerns.

Elizabeth overall had a pretty decent school year with attendance and illnesses. She did have a pretty big break in school right before Christmas until the beginning of March. She had a stomach virus right before Christmas, added her already horrible GI system she was was out of school unable to keep any food and losing alot of weight until the beginning of March. We finally have a plan for giving a medication 10 days a month to help with her motility. And she will be having testing done in July at Cincinnati to see if we can figure out exactly what the issue is and a better way to deal with it. I am very thankful for the new GI doctor that she is being seen by at Cinncinati, he understands the connection between all her belly issues and the Filamin A mutation. Even before transplant she has always had these issues, and it just seemed like the different doctors wanted to just treat the symptoms and not look for the underlying issue. I hate that its taken this long to finally have a "plan" but so far she is doing well on the medications and we will hope for an even better plan after the testing in July.

 In April we went to Cincinnati for her 5 year check up. During her check up she had a chest CT, xray, bloodwork, DEXA scan, cardiac MRI and bronchoscopy done. Overall her lungs looked good, and the team is happy with where she is. The main concern that came out of her trip was the issue with the aortic root dilation that we have been monitoring since after transplant. Elizabeth has been on medication since she was 2 to try and slow the progression of the dilation, but it is now at a point where the cardiologist feels that intervention needs to be considered. So we will be planning for Elizabeth to have surgery in June in Cincinnati to replace part of her aortic root. This is a very scary decision for us to make, to put her through a major cardiac surgery. Without this intervention there is a possibility that her aorta could dissect which would most likely be fatal. By intervening now and replacing the part of the aorta, we will hopefully prevent any future issues. As with any cardiac surgery there are major risks, and add in being a immune suppressed kid with a rare genetic condition and the list gets a bit longer. Matt and I both feel that the timing is right for this surgery, and I just pray that everything goes well and she is back to her happy self quickly. We were told to expect 7-10 day hospital stay after surgery. She will be sedated and on the ventilator for the first 24 hours or so to rest and then should be able to be extubated but will most likely be on some pain medications.

   When we first started this journey, we were told its like a roller coaster ride, and that has definitely been the most descriptive way to to explain everything about the past 5 years. As we head for another loop in this ride, I remind myself of all that Elizabeth has endured over the past 5 years. It breaks my heart that I can't fix this or take it away. But I also know that she is so strong, and has fought so hard in the past there is no doubt in my mind that she will continue to do so through this and I have faith that she will wake up asking for her ipad!
    
    Thank you for your continued prayers and support, most of you who will read this listen to me vent and stress all the time and I know I don't say it often enough, but I appreciate your support, love and understanding. And most of all I appreciate your love and prayers for Elizabeth!

New Year - New Germs

I never got around to updating the blog after Elizabeth was discharged back in October. We were SO ready to be home and get back to normal.  We were able to spend Christmas and her Birthday at home. Her birthday this year was very special to me. Elizabeth and I were able to spend the day together. I took her to get new big girl glasses, and we went to a trampoline park. She LOVED me jumping and bouncing her. IShe seemed just like any other kid running around wanting to climb and bounce. I was so proud of and for her!  Elizabeth tends to do her own thing and not really want to interact in public places, so this was a huge deal for her and me. Then she was able to go to the book store and pick out some books. I think she really enjoyed her Birthday this year.
Unfortunately, since we returned home Elizabeth continued to not feel so great and in December we were back in Cincinnati for her 3 1/2 year checkup. Thankfully everything looked ok with her lungs, biopsies showed no signs of rejection. Her aortic root dilation is about the same and ENT was not concerned with her airway. There were a few changes made to her medications to make life a bit easier as far as her medicine schedule goes. After Elizabeth returned home in October she was having major digestive issues, vomiting numerous times a day randomly. She was losing weight, and not able to ever have a full feeding we were finally able to get her in with GI here at home and she was given anti fungal medication and medicine to help with motility. Thankfully today she was FINALLY able to tolerate a full feeding. Yes, you read that right MONTHS of throwing up and not tolerating her feedings. Talk about miserable. Lets add to that that she has recently had RSV, and ear infection, and her lungs were starting to look really hazing (signs of early pneumonia).  And lets add that her IGG levels have been low, and she has needed IVIG infusions since October. We decided to try a different version of IVIG that can be given at home subcutaneously. She ended up having a reaction to it and was sick for 24 hours from it. This honestly has been the worst winter for her since transplant with illness. We are just hoping that all this illness will not cause any damage to her lungs. She has missed alot of preschool this winter. We are in the process of scheduling her transition meeting for Kindergarten and Matt and I are really concerned about allowing her to go to public school. We want her to have a normal life, and be able to be around other kids and experience things, but we have to also consider how susceptible she is to getting sick. So we will discuss our options and then decide what we think will be best for her. So months of having a sick kids results in months of mommy or daddy - in this case mostly daddy having to be home or work from home to take of Elizabeth.  Due to being out of personal time, I was not able to call in as much as I wanted too but thankfully Matt was able to stay home to care for her. I am so thankful for him!

We will head back to Cincinnati this week for a followup with the transplant team. Since the pneumonia in October and all the other illnesses this winter could set her up for decline. The team wants to see her more often. We are praying for only good news and a short visit!

Ready for Home

Elizabeth has continued to make progress. She is still on 1-2 liters of oxygen to maintain her levels, but thats been normal for her when she gets sick. She finished her last dose of IV antibiotics today and also weaned off the methadone and ativan. So now her biggest issue is that her anti-rejection medication levels are low, one is so low its almost as though her body is not absorbing it. Her med doses have been changed multiple times throughout the past week to try and get her levels back in check. Before this illness she had pretty much maintained her levels with no real changes to the doses. Of course, this is really concerning to us and the doctors. She is also having to take magnesium supplements due to her levels being low. So for now the plan is to be released tomorrow, knowing that she will have to continue having labwork done when we get home, possibly multiple times a week until her levels are back to normal. And returning back here in a few weeks for a follow up and then more frequently until we kind of know what kind of damage, if any, this major illness has caused. She will be checked again for signs of rejection when we come back and I am praying for only good reports.

The past few weeks have been hard, after being home and "normal" for 3 1/2 years except for a two short hospital visit.  I honestly question how we made it through those long months in the hospital before/after transplant. Elizabeth absolutely hates the hospital! She refuses to talk to anyone or participate in any of the therapy services that have stopped by.  I actually feel kind of bad for how much she ignores them. But with all she has been through, she just wants to go home. She hasn't talked very much and hasn't stood up or walked much. She is still pretty weak from being so sick, so we know it will take some time for her to recover.  We are hoping being back home and getting back to her normal routine will really help her with getting back on track.

Making Progress

Elizabeth is doing SO much better! She was able to wean off of the ventilator on Friday! She was put on 12L of high flow oxygen and since Friday has been able to wean down to 2L of oxygen via regular nasal cannula. So during the course of last week on top of germs in her lungs another culture came back for pseudomonas from the bronchoscopy that they did here. So she is now getting IV antibiotics, inhaled antibiotics and a vest treatment to loosen up all the gunk in her lungs. On Friday she was very tried and weak and slept alot after they removed the breathing tube and ventilator. She has started watching her movies on the Ipad, smiling and has said a few words. She is also weaning off of the sedation and getting ativan and methadone to help with that. Her little voice is pretty raspy from the tube being in there,  so she hasn't said a whole lot yet. She was able to start sitting up on her own today and moving around some. She did try to walk earlier to Matt and it was SO sweet. She is still pretty weak and it will take a bit for her to get over this. We are beyond thankful she was able to fight this and is recovering. The plan for now is to hopefully be moved from PICU tomorrow to a regular hospital room, finish out the IV antibiotics this week and possibly be discharged home on Friday if she continue to do well and doesn't need more oxygen. We will have to come back in December for the evaluation and testing that was originally scheduled for this week. Her next appts will include a appointment with ENT here to get a second opinion on her airway, and hopefully some good news options for fixing it.  And we will also meet the new cardiologist and get a check on the aortic root dilation. After her checkup in December we will most likely come back more often for a while to be sure that this illness hasn't stirred up possible rejection. Rejection is a big fear for us, and we pray that this illness was just that a bump in the road for her amazing gift of lungs will be undamaged and continue to provide her with MANY more years!

This was one of the scariest times since transplant for us. Its so easy to get caught up in the everyday things when she is well. This was just another reminder to all of us just how quickly things can change, and why we need to make the most of each day we have with her. Just like all of us need to remember its not the quantity of life that matters but the quality. Things like money, houses, cars, and what you have really don't matter at the end of the day. This amazing little girl, her sister, Matt and our family/friends are the important things in my life. I really hope that anyone who knows Elizabeth, or any of our transplant friends, learn from these amazing kids to appreciate all you have in life and know how quickly it can all change!

NO REJECTION!!

We talked to the Transplant team after they reviewed Elizabeth's biopsy today. Her biopsy shows NO rejection. We are thankful for this answered prayer, but cautious as well. The biopsy was several small pieces of lung from her middle right lobe. Lungs are a huge area in comparison to the small amount that was biopsied. There were some cell changes in her lungs that will continue to be monitored closely. She was still treated with the 3 day steroid dose for acute rejection, just be on the safe side and in hopes that the steroids may also help her lungs with the current illness. But we are thankful at this point the team thinks this illness just hit her really hard. We have no idea how long it will take her to recover from it or what her baseline, as far as needing oxygen will be once she is over it. But we will watch her even more cautiously when she is over this for any changes. She has remained stable today and we hope they can make more ventilator changes tomorrow to work towards getting her off of it soon.

Transfer to Cincinnanti

I apologize in advance if my lack of posting had anyone worried, I have gotten a lot of texts recently. The last few days have been beyond exhausting and worrisome for us.  For those who haven't heard on Monday, Elizabeth appeared to be getting a bit worse. After talking to her transplant team Matt and I decided that we would feel more comfortable with her being with  the her team in case she were to get sicker or needed to be checked or treated for rejection. 

Matt, Charlotte and his dad & step mom all drove up and were here before Elizabeth and I arrived. Elizabeth and I flew out of Chattanooga around 2/230am on Tuesday morning.  It took a while for transport team to get her set up on their equipment. I did get a bit scared while enroute from our home hospital to the airport the Transport Team ran out of oxygen. The ambulance had extra tanks, and the team had to bag here with an oxygen tank to keep her oxygen saturation up.  As a police officer, I am used to riding in a vehicle with lights and sirens. But when its your child in the back and your not sure if she's going to have enough oxygen to make it to your next location, its a whole different story! Thankfully we made it to the plane and they were able to get her transferred onto the oxygen inside of it quickly. She did really well on the flight. Once we got to Cincinnati around 3am the PICU team and our Transplant Team were all waiting for her and us. We have been really impressed here with the care she has gotten and are grateful to feel we made the right choice for her.

Once she was settled they re-cultured everything and did more lab work just have their own records her. She has been able to maintain her oxygen levels and has not required any extra support besides the ventilator and sedation. Her sedation was increased when we arrived to make sure she is comfortable and resting. They have also added more meds to try and get the extra fluid off of her. Transplant felt that another bronchosocpy needed to be done, and also a lung biopsy so that they could determine if she has underlying rejection. She had the bronch/biopsy yesterday and thankfully tolerated the procedure well. We should have those results later tonight/tomorrow.  We are praying she is not in rejection, and that this illness won't set her up it. The Dr did say when he was looking at her lungs they were full of thick mucous from the pneumonia/infection. He was able to remove alot of it and she is now starting airway clearance therapy to help loosen some of that up. Her oxygen requirement has went down to 55% from being on 70-90% before we got here. So we are hopeful that she is really improving and its just taken getting some of that junk out of her lungs and getting fluid off for her to start improving. We still worry about rejection, and as a precaution they started treating her with a 3 day round of high dose steroids that are usually given when someone is suspected of being in rejection. She will get her 3rd dose today. And that may also be what is helping her right now too. So for now the plan is to continue to wean the ventilator until she can come off of it. Start the percussion therapy to loosen up more of the junk in her lungs. Wait for the biopsy results to know for sure about rejection. Starting her feedings back today. And lots of prayers and waiting for our precious girl to get better. She a fighter for sure!

Love my Precious Izzabug

Elizabeth had a little rougher day yesterday. Her oxygen saturation levels started dropping. She dropped as low as 77 but will come back up to 98 with increased support. They think its because of the extra fluids she had to get to help get her blood pressure stable, that Elizabeth's lungs are too wet now. This happened often during transplant, where she needed extra fluids, and then ended up getting out of balance. So it will just take some time to get her fluid levels back in balance, she was started on diuretics to help with that.  So all night her oxygen saturation has been up and down. They have had to adjust her ventilator settings some and as her oxygen saturation fluctuates they have had to adjust the amount of oxygen she gets. So its been up and down a lot.   Her sedation meds were also increased to help keep her calm. They were able to start feeding her a small amount of her regular formula through her feeding tube in addition to giving her TPN (IV nutrition) yesterday. Her blood pressure has been decent and they have been able to get the dopamine down to very low settings and even turned it off earlier this morning but are still watching her blood pressure. She had an echo on her heart this morning so we will meet with Cardiology here at some point to discuss what it shows, I don't expect it to be much different than her what we saw in Texas back in April.  She is still opening her eyes every once in a while, and I feel so bad for her. I hate that she going through all this again, but I know they are taking great care of her here.
I am very thankful that alot of the nurses, resp therapist and all of the doctors in PICU here at home remember her from before transplant. They have been great about answering our questions and doing the very best to care for Elizabeth. We have also been beyond impressed with how great our transplant team from Cincinnati has been as well. They have been in contact with the PICU and with us daily, to ensure we felt comfortable with the care she is getting and to answer any questions or provide any assistance we need. Matt and I have been taking "shifts" at the hospital I stay at night with her and he stays here most of the day. That way we can also spend time with Charlotte. I feel so bad for her, I know she misses Elizabeth and doesn't understand what is going on. Our families have been great about helping out with watching her and sitting with Elizabeth when we need a break. We continue to be thankful for your prayers and support. Elizabeth is a little fighter, and will overcome this!

I also wanted to share something, a few weeks ago Matt and I attend a marriage retreat at Winshape in Rome, GA. One of the songs that was played while we were there reminded me SO much of Elizabeth and her fight, and of how I have had to learn to have faith. I was crying so much while it was playing, and I just knew people thought I was crying because of my marriage ;) But it was because I kept remembering all Elizabeth has been through and all she may still have to endure. I really think God placed this song to be in my head and on my heart to help prepare me for this dip on the roller coaster ride of Elizabeth's journey. I have played it for her a few times while we have been here.
 Blessings by Laura Story:  https://www.youtube.com/watch?v=1CSVqHcdhXQ

Such a strong little fighter!

Just a quick update on how Elizabeth is doing. She was able to weaned down on the dopamine for her blood pressure and her ventilator settings! Prayers were answered today! She seems alot more comfortable today.  Her temperature has been back to normal and her heart rate is alot lower. She seems to be responding well to the antibiotics that were started, and we hope that she continues to improve. She is still sedated and resting, but moves around and opens her eyes every now and then. The bacteria that is growing in her lungs was identified as streptococcus, so the antibiotics are working on that bug. The paraflu is just a virus her body will have to fight. Being on the ventilator will just help her body rest and be able to fight these nasty germs easier. Her immunoglobulin (IGG) level was checked when we got her Thursday and  results came in today showing it was low. So she relieved IVIG today, blood product that will help "boost" her immune system. She used to get infusions every month after transplant but her levels were stable for a while and she didn't need them.  We are hopeful that with the IVIG it will help her be able to fight this even more, and hopefully boost her to be able to stay healthy through the rest of the fall/winter season. So for now the plan is to continue the antibiotics and watch for anymore bacteria to grow in the cultures. They will continue to wean the dopamine and ventilator as she can tolerate it. We are hoping she will be able to start being fed again soon. And Monday they will do an echo of her heart. We know that the aortic root dilation can cause some issues with blood pressure and they just want to check heart to see if that could be what is causing all the low pressures or if its just because she is so sick. Thank you for the prayers and for checking in on us!

3 1/2 Years Post Transplant - And - In the Hospital

Most of you have seen my recent update on Facebook about Elizabeth's current status. Thursday morning around 1am we noticed that her heart rate was higher than normal. That is usually our first sign she is getting sick. She didn't have a fever and her oxygen level was normal so we just assumed she was getting a virus and would follow up with the Doctor once the office opened. Around 530 am Matt woke up and found her to have a 102-103 fever. We gave Tylenol and she stayed awake and seemed to feel better and was playing. From 530 am until around 1230 her need for increased oxygen went from 1L to 4L. Her color was not good, and we knew this was more than an regular virus. She was admitted to the PICU here at home with a fever of 105. After being admitted pulmonlogy did a bronchoscopy to check her lungs and get some fluid for testing. They also drew blood and got urine to test for infections, FLU/RSV test and a viral panel. She was intubated and placed on a ventilator to give her lungs and body a rest. She has some sedation and has been sleeping. Her oxgyen levels have been good and she seems to be tolerating the ventilator well. Her biggest issue has been low blood pressure, and she is currently on medication to help maintain a good pressure.

Her chest xray shows pneumonia and her viral panel shows that she has Paraflu, the bronchoscopy of her lungs shows some bacteria growing, which we are still waiting to be identified. So for a post transplant, immuno-supressed kid this is not the news we wanted to hear. But we know how hard Elizabeth has fought in the past and we know she can do it again. These angel lungs are strong and we know all of her family and friends are praying hard for her!

We were supposed to be headed to Cincinnati on Sunday for her 3 1/2 year lung check up but those appointments are being postponed until she is better. Some people have asked about us transferring there for current care. As long as she is maintaining her current condition, we will continue care here at home. But if she begins to decline we will be transferred to Cincinnati to be where the transplant team can be more involved in her care.

Please continue to pray for her! We hate seeing her so sick and know she can overcome this too!

Celeberating our Time

So grateful for a busy and illness -free summer! In May we traveled to Orlando for Elizabeth's Make A Wish trip. I can't believe I am just now updating about it!

It was so nice to have a "break" it was our first trip as a family that did not involve medical appointments. Elizabeth and Charlotte both did great and we were able to have alot of fun. We are very thankful to Make A Wish and Heatech who sponsored Elizabeth's trip. Especially since everything had to be rescheduled after she got sick back in October. During our trip we stayed at the Give Kids the World Village. We were able to visit Disney's Magic Kingdom, Animal Kingdom,  and Universal. I think Elizabeth's favorite part was getting to see Dora. They had a character parade where Dora and all her friends came out, Elizabeth was able to meet them all and have her picture made with everyone. Then after the parade was over the had all the wish kids meet up and let them spend more time with the characters. I loved how special they made the kids feel and I know she enjoyed meeting her favorites :)

Elizabeth is doing SO well developmentally and making great progress. She is talking alot more and walking everywhere now. She did get some new inserts for her shoes to help her gait. Ortho still thinks she may need surgery on her hips at some point but we are holding out as long as we can. She is in her last year of preschool! I can not believe how fast she is growing, and she will be starting kindergarten next year. We will start meeting with the school district sometime after the first of the year to decide what school will be best for her and her needs. We really hope we feel comfortable with whereever they offer.

The biggest news as far as Elizabeth's medical care is that we have decided to change hospital/doctors. After MUCH consideration we have decided to move her transplant and other major care from Texas Children's to Cincinnati Children's Hospital.  I had mentioned here before that her primary transplant pulmonlogist and nurse coordinator had moved to Cincinnati to start a transplant program. We have been in contact with them and feel very good about moving her care. Cincinnati is significantly closer to home 6-8 hour drive VS 13-15 hour to Houston. While we have and will travel anywhere we need to get her the best care. Now that we have Charlotte we also have to consider long term, if Elizabeth needs another transplant or were to be hospitalized again we need to be close to family/friends. As well as the trips every 6 months, being closer means missing less work.  We have always had a good relationship with Dr Schecter and are excited that he will now be Elizabeth's primary care doctor again. We realize Cincinnati's program is new, but know the Doctors/Surgeon/Nurse Staff are all from TCH and have significant experience with transplant care. We also feel that being in a newer program, the dr-patient ratio is smaller and may provide for more personal care - at least in the beginning. This was a HUGE decision for us, and we pray that we have made the right one! We will be traveling for her first appointments in October. She will be seen by transplant, cardiology, ent and orthopedics while we are there. We have a packed 4 day schedule during our visit there. We ask for continued prayers for only good news during this trip.

Everyday I see Elizabeth making progress and I can not help but think of how lucky we are that she is still with us! SO many prayers have been answered over the past 3 1/2 years. Some days its easy to get caught up in the routine of just living our lives. I still worry alot about her future, the constant fear of her going into rejection or that her heart will continue to get worse. There are so many unknowns with transplant and her genetic condition. But I have to remind myself Its not up to me to question when, why, or how things will happen. I found this bible verse recently and printed it out. I read it every morning when I get ready.  Its my daily reminder, there is a time for everything. We have and continue to experience many "times" with Elizabeth and now is the time to enjoy life and make the most of the memories we are making with her.
"For everything there is a season, and a time for every purpose under heaven: a time to be born, and a time to die; a time to plant, and a time to pluck up that which is planted; a time to kill, and a time to heal; a time to break down, and a time to build up; a time to weep, and a time to laugh; a time to mourn, and a time to dance; a time to cast away stones, and a time to gather stones together; a time to embrace, and a time to refrain from embracing; a time to seek, and a time to lose; a time to keep, and a time to cast away; a time to rend, and a time to sew; a time to keep silence, and a time to speak;
a time to love, and a time to hate; a time for war, and a time for peace. He hath made everything beautiful in its time."

An overdue update & 3 year lung anniversary

It has definatley been a while since I have updated, no news is good news right? :) We had a bit of a rough winter with Elizabeth getting sick but were fortunate enough to stay out of the hospital. She missed alot school during the fall/winter months with viruses/fevers/ and extra oxygen. In December we celebrated an amazing milestone, her 4th Birthday! Hard to believe looking back at how sick she was that she would ever make it that far. On Jan 10 2014, we welcomed our newest little bug, Charlotte Marie into our family. She weighed 8lb 3 oz, and is very healthy. We were so grateful for more answered prayers for her safe arrival and good health. Elizabeth is adjusting well to her new role of Big Sister. She asks to hold Charlotte, and to give her kisses. But she also lets you know when she is "all done with the baby". :)

Some major milestones, Elizabeth now walks on her own without any assistance. Its pretty amazing to see, she is all over the place now. She is still a bit unstable and wabbles some but is doing great. She is still enjoying preschool and playing with her friends. She is talking ALOT more and eating more by mouth. She still has her feeding tube for the majority of her nutrition and still requires oxygen during sleep due to her airway issues. She will start her last year of preschool in the fall, we will start discussing her plan for Kindergarten in February. I can't believe how fast she is growing up!!

In April we traveled to Texas for her 3 year post transplant evaluation. I can not even believe its been 3 years! I am so thankful for all she has accomplished during this time, and all the memories we have been able to make with her. I try to constantly remind myself we knew going into transplant that we need to focus on quality of life, not quantity. Make the most of the time we have regardless of how much that may be. She touches the lives of everyone who knows her, and I am so lucky to be her momma.

Her appointments in Texas consisted of transplant team, neurology, genetics, cardiology, labwork, CT scan of her lungs, Xray of her lungs, an another MRI of her heart. The team chose not to do a bronchoscopy of her lungs this time since she not had any issues as of now. Based on the tests/images her lungs are doing well. Neurology and Genetics didn't have any new information, we just follow up to they can track her progress. But our main concern was her heart, and unfortunately her aortic root has dilated more since her last visit. In previous posts I explained how if she were an adult they would already be considering surgery to replace it. But in children they are monitor it closely and set a threshold for surgery. Elizabeth's aortic root is currently dilated to 2.9 CM with 5 being the cut off for replacement. Based on previous images, the Dr told us its dilating pretty quickly. So we are starting her on a new medication in addition to the heart medication is already taking to help lower blood pressure in hopes that it slows down the dilation and gives us more time before she has to have surgery to replace it. We are really hopeful that it will slow down enough that we have years before we have to put her through such a large surgery again. But we really have no idea how long it will be. She will continue to have checkups every 6 months in Houston for her lungs and heart due to her airway issues and the aortic dilation. Most transplant kids at this stage are only going yearly, but we will go as often and as long as they tell us to ensure she is getting the best care possible.

April was also our time to remember Elizabeth's angel Elijah and his family. Remember the gift they gave, and celebrate the accomplishments Elizabeth has made this past year. We pray for comfort for his family everyday and are constantly reminded how precious this gift of life is.

We thank you all for your continued prayers and support! We ask for specific prayer that this new medication will work and slow down the dilation of her aortic root, and that her lungs continue to do well and stay healthy. I also ask for peace in knowing that hard choices are coming and God will give Matt and I the guidance to do what is best for Elizabeth.

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.

Airway-
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 :)

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