Sunday, February 26, 2012

Miracles Continue

Elizabeth continues to do well. She is super happy and smiles alot. Her appointment with ENT on Feb 14th, went ok, of course it would have been better if the news was great but at least it wasn't horrible. We know she has alot of floppiness in her airway, and they were going to decide on a plan to try and fix it. Elizabeth is scheduled for surgery on March 13th at Vanderbilt. They will take some of the tissue out of her airway, inject steroids in it, and use a balloon to dilate her airway. They will check it 3 weeks after the procedure and if it seems to have helped they will repeat it, and again in another 3 weeks. The Dr said 3 times would probably be the max he would do it, if it helps great, if not we will decide on another route. I hate that she is has to go through this, I know in the grand scheme of things this is a small thing compared to her other surgeries. But it still sucks. We are really hoping that it helps and they wont have to do anything more invasive to "fix" her airway issues. Other than that she is still doing amazingly well. She had continued to gain weight, she is now up to 18lbs 13ozs, still well below the normal range for age, but shes gaining so we will take it! Her anti-rejection levels have still been a bit elevated, so we have decreased her dose again, and will be doing labs more frequently until she is at a good level. She continues to get monthly synagsis vaccinations to prevent RSV, thankfully the season will be over in March/April and she wont have to get the shots and we can venture out more without the fear of RSV and Flu.

  She is still making progress with therapy. Still no crawling, walking, or talking (other than the occasional momma, dadda, or bye-bye). She still has no desire to eat by mouth. But, she is moving around alot by scooting on her back to get to her toys. She babbles alot, and seems pretty content. Any progress is great, and we will take whatever she is willing to give! We are planning to add speech therapy within the next few weeks.

 The weather was really nice a few days last week here,  but was it was super windy. I took her outside just to sit on the steps at our house. It was so sweet to see her reaction to the wind. She would make the cutest face, and snuggle up close to me, then she would laugh and smile about it.  It really is the small things that mean SO, SO much. And I am so thankful that I am able to share in those moments with her. I remember this time last year, when I worried we would never make it to this point.

We continue to be amazed at her progress and her love of life!! Everyday we are thankful for her and the lessons she is teaching us. Its so easy to sometimes get caught up in the "downside" of things and how different our lives are, and the constant what ifs of her future. But different WITH Elizabeth is the only life I want to know!  The what ifs keep me in check and are a constant reminder to cherish the gift of her life.

~We recently (I know late) had some pictures taken for her 2nd Birthday, I posted a few of them below. LOTS of thanks to Boyd&Olson Photography for their awesome work! And my friend Amy for the amazing cupcake!! :) ~

Wednesday, February 8, 2012

9 Month Checkup in Houston

We traveled to Houston Jan 29-Feb 2 for Elizabeth's 9 month checkup. Thankfully she did well with flying and slept the entire time on all of the flights! We were fortunate enough to be able to stay with our wonderful friend, Shelley and her family during our stay this time instead of at the Ronald McDonald House.  She is one of the amazing nurses that took such great care of Elizabeth during her stay at TCH.  During her visit Elizabeth had lots of labwork done, a brochoscopy,  and a chest xray. We met with the transplant team, dietician, social worker, and genetics during our visit.
Thankfully all of her lab work looks good. They were happy with all of her nutrition levels - which makes me feel better about the home blended diet we are doing. At least I know we are mixing the right foods for her. They did decrease her steroids and one of the anti-rejection meds due to higher levels. The downside of having the higher levels is that her immune system is even lower than "normal" for an immunosuppressed kiddo, so we have to be extra careful until her levels are within normal range again. All of her labs will be repeated here at home sometime next week. During the bronchoscopy her lungs looked good. The Dr is still concerned about her floppy airway. We will be heading back to Vanderbilt to see the ENT Dr there and we will discuss the plan for either fixing her airway (surgery, stenting, dilation) or waiting it out. Thankfully the transplant Dr is planning to discuss everything with ENT, so that everyone is on the same page as far as what needs to be done and then deciding where it needs to be done at.
     We met with genetics during this visit and again discussed Elizabeth's diagnosis with the FLNA mutation. I know in the past I have mentioned it and the fact that it is what basically caused her medical problems. It is such a rare and underdiagnosed condition that there just isnt much research availabile, or much knowledge regarding it. We asked lots of questions about other medical issues it causes, we want to be sure we know what to ask our Drs to look for.  Now that we have "fixed" the lung issue. We have to focus on the other problems that this mutation can cause. Cardiac issues are one of the biggest concerns, so we have to be sure that her Cardioloigst at Vanderbilt is looking for the right things on her echos and that they are being done frequently. She will be seeing him in March, and we plan to discuss everything and make sure he is up to date on this disorder and what needs to be looked for.  Other issues that we were told to monitor closely are her hearing and vision screenings. And of course the hypermobility that this mutation causes, she will need therapy services for a very long time. We also discussed the possiblites of having more children, and the chances of this mutation occurring again. We were told that the possibility is extremely low, the chances of this occurring again would be the same chance of it happening to anyone else in the general population.

   So all in all her 9 month evaluation was very good. The transplant team is happy with her progress, they just want her to gain some weight :) We are very thankful for a good report, and a safe trip. We will travel back to Texas in April for her 1 year evaluation and if all goes well then, her evaluations will be moved to every 6 months instead of every 3. Kind of exciting and scary to me. I almost like knowing that they see her so often right now. But not having to travel that far every 3 months (for a not very fun trip) will be good for all of us.  I am so thankful that she is doing so well, and just hope and pray that she continues too!!