Monday, August 30, 2010 6:07 PM, CDT

Elizabeth had her bronchoscopy this morning, she did well during the procedure and after during recovery. The pulmonary Drs came and talked to and explained that Elizabeth has a floppy airway, know as tracheomalacia. Which "could" be a contributing factor in her rapid declines with her oxygen saturations. We know that her lungs are underdeveloped but by having a airway that is floppy and doesnt always allow the oxygen down to her lungs could cause her lungs/heart to work hard and need more oxygen, causing her to have the pulmonary hypertension crisises. The Ear, Nose, Throat Drs just came by and want to do a CT scan of the airway to make sure there are no vessels or anything pressing on her airway causing this or if her airway is just floppy. The upside to this "new issue" is that most of the time kids can just grow out of it, but since she already needs so much support there is  a chance she will need a tracheostomy to ensure her airway stays open. For now we are not sure what route we will need to go. We are having a Care Conference tomorrow at 2, which is when we will meet with all of her doctors here, pulmonary, cardiology, neurology, nutrition, gentics, and anyone else I forgot :)  This way we are sure that everyone knows her current status and everyone is on the same page as far as treatment plans and issues. We "may" get to go home this week depending how the CT goes and they also plan to do another Echo and Upper GI to make sure there are no changes with those things before we leave.

Sunday, August 29, 2010 11:14 AM, CDT

Elizabeth was moved out of PICU on Friday and is a regular room now. They would like to do a Bronchoscopy to check her airway to see if that could be causing any of her issues. She had a sleep study done last night to see if that could be causing anything. At this point they are just trying to rule out any other cause to her declines since they dont think its her heart.  Other than that no new news to report.

Thursday, August 26, 2010 2:00 PM, CDT

Elizabeth was able to wean off the nitric oxide around 1130 this morning. She had an echo done when she was at 1ppm of nitric and then again when she weaned off. The cardiologist came by and told us that she could not see a difference on the echos based on being on and then off nitric, so she does not feel that a cardiac cath is necessary. Her dose of Sildenafil has been increased today and will continue to increase over the next 2 days in hopes of helping keep the pulmonary hypertension controlled. SO, our question is what caused this decline and what are the chances it will happen again? Since it doesnt appear to be her heart that has caused it next thing is for the Pulmonologist to decide if there is more that they think needs to be done on the lung side. We are grateful that her heart doesnt seem to be worse, but now worried that we still dont have an answer.
She is still in PICU until she can be weaned down and off of the high flow oxygen but they plan to start weaning that today, so we may be in a regular room within the next day or two. Once cardiology and pulmonology get together and discuss the findings from today we will maybe have more news.

Wednesday, August 25, 2010 4:35 PM, EDT

Cardiology came by today to try and decide on a plan of action for Elizabeth. They did an Echo on her heart, will know the results later once it is read. The cardiologist I spoke to seemed really great, but maybe thats because she kinda is doing what I hoped for. She thinks it would be best to increase the Sildenafil medication for pulmonary hypertension over the next 3 days and see if that makes it easier to wean off the nitric oxide. Once they are about to totally wean off, they will do another echo when she is on 1 of nitric oxide and then an echo when she is off and see if they can determine just how well she responds to nitric. IF they can gain enough info from those echos she wont have to have the cardiac cath done. Which would be great, I am very fearful of the cath being done since there is such a high risk invovled with it. That being said this "plan" will definatley be a very slow process meaning at least towards the end of next week before she "might" be off the nitric. And then we still have to determine a treatment plan. It makes me feel a bit better that they are not rushing to do a cath without trying all other possible means first.
Of course her IV has went bad and a new one needs to be started! This will be her 3rd one in a little over a week! They tried earlier and had to stick her twice with no luck. We requested that they give her something to keep her calm since she gets so upset and her heart rate gets too high (in our opinion). So when they come back to try again, they plan to give her some medicine first to calm her.
So thats the news for now, pulmonary will be by later to discuss anything they have decided to do/change but for the most part I think things will remain the same until she comes off the nitric.

Wednesday, August 25, 2010 11:53 AM, EDT

We spent all day yesterday waiting for a room to open at Vanderbilt and last night around 630 we got the word that a room had opened. Around 830 Vanderbilt's Lifeflight arrived and a swarm of people were getting her ready to transport, including her flight Dr, nurse, and resp therapist. Thankfully I was able to fly with her and Matt had enough time to drive to Nashville before we arrived. Elizabeth rode in an ambulance from Erlanger to Chattanooga Airport and then we got on a small plane flew to Nashville airport and took another ambulance ride to Vanderbilt. She got into her PICU room around 1030. She did really well on the plane ride, she slept the whole way :)
Once we got here they got her settled and the PICU Dr's came by to check on her and get her history. It was a LONG night since we got her so late, they still had to draw blood and check her vitals every 2 hours and monitor her oxygen and nitric levels. Once of us is able to stay in the room with her, and we were also assigned a sleep room on the same floor and Matt went there to sleep for the night.
  TODAY~ So far the plan is to wean her nitric by 1 every hour until we get to 10. She started at 20 ppm and we are already down to 17. They think it would be less risky to do the cath if she can come off the nitric before hand. They have also increased her Sildenafil (viagra) medication for her pulmonary hypertension to see if that helps any. We are waiting for Cardiology and Pulmonology to come by today.

Monday, August 23, 2010 4:05 PM, EDT

Matt and I discussed things and decided that Vanderbilt would be our choice for Elizabeth. Right now the PICU doctor plans to talk to Cardiology here and ensure that Vanderbilt is completely aware of her issues and ensure a plan of action is prepared. We are waiting for that discussion to happen and then plans to transfer will begin. We have been assured that Pulmonolgy at Vanderbilt is onboard with what the lung specialist at Cincinnati has recommended as far as her lung disease goes, so we know that we will get the same treatment with regards to that no matter where we go. We were also told that Vanderbilt is just as good of a choice for Cardiology as Cincinnati, so we are hoping thats the case. Both hospitals have great doctors and facilities that will be available for a high risk cardiac cath, if she ends up getting it.   One possible issue is that as of last night Vanderbilt's PICU was full, if thats still the case and they dont have room for her, then we will try for Cincinnati. So basically we made a choice but still not sure where we will end up :-/
    I will update more as we find out, but since its already 4pm here I doubt much else will be accomplished today.

Monday, August 23, 2010 12:21 AM, EDT

Nothing much to update today, Elizabeth is still in PICU on high flow oxygen and nitric oxide. Tomorrow (or today I guess) will be a big decision making day for us ~ where do we go~.

  I sometimes feel like all of this is not real, how is it that someone so little has to suffer and struggle so much. When Elizabeth was born I remember thinking ok, shes gonna be in NICU for a few days and then be just fine. But those few days turned into a month. And then I thought ok she just needs oxygen for a few weeks and she will be ok. I dont think the severity of her illness hit me until she was on the ventilator in March and I realized there was a good chance that things may never be "normal" for Elizabeth. It seems like every month since then we have something new or worse to deal with, I try hard to find a "good" thing or progress made each day to give me hope, but sometimes thats hard to do. I hate not being able to make things better for her.
  Some of the choices we have had to make and will continue to need to make scare me but knowing that we could make the wrong choice scares me even more. I hate knowing that other families have to struggle with these same choices daily and it makes me so sad knowing that other kids are going through the same and even worse situations than Elizabeth.
 I am starting to realize that all we can do is try our best to make the most of the resources we have and learn as much as we can to provide her with the best care and best life possible.

Saturday, August 21, 2010 2:26 PM, EDT

I posted on Thurs that we planned to have Elizabeth transferred to Cinncinati, but after further discussion with the specialist there and other doctors we were also again told that Vanderbilt would be a good option. SO, over the next 2 days Matt and I have to decide where we want her to go, so that she can the transfer process can begin on Monday. Basically the Lung Disease Specialist cant really do anything for Elizabeth at this point since her current issues seems to be her heart and pulmonary hypertension, so where ever we go the main "purpose" will be to see Cardiology and most likely have a cardiac cath done on her heart. She has never seen cardiology at Cinncinati but has been seen and is establised as a patient at Vanderbilt. But we want to be sure of which hospital and doctors are the BEST possible option for doing the cath and for providing the possible care needed depending on the findings of the cath. All of the doctors here have great things to say about both hospitals and both cardiology departments, so the choice has to be ours.
Elizabeth is doing well right now, she is stable on the high flow oxgyen and nitric oxide and the plan is to just continue on those things until she is transported to make sure she remains stable instead of trying to wean her back down to a regular oxygen flow.

Thursday, August 19, 2010 11:11 PM, EDT

Elizabeth had a somewhat better day. Her mood was alot better, she was happy and playing alot today. She is still on high flow oxygen and nitric but they were able to wean it down some. At this point there are a few things that need to be done in order to make Elizabeth more stable and to have a better chance at letting her lungs grow.
In order to get Elizabeth what we believe to be the best possible care,  Matt and I have requested to have her transferred to Cinncinati Children's Hospital. Since her conditon has gotten worse, we feel that being at the hospital with the doctor who specializes in her lung disease and has seen children with very similar issues would be the most beneficial thing for Elizabeth. She also needs to be seen by Cardiology to assess her pulmonary hypertension again and most likely have a cardiac cath done on her heart. There are some other things that may need to be done and I will write about those as the decisions are made.
The plan for now is to keep her stable and keep weaning nitric and high flow oxygen amounts. The staff and doctors here are planning to work on getting things in order for us to transfer tomorrow. Best case is that we will be heading up there early next week.

Wednesday, August 18, 2010 10:36 PM, EDT

Unfortunately we are back in the hospital! Elizabeth started having problems keeping her oxygen saturation up and needing increased oxygen on Monday and we took her to the ER, she seemed to improve and stabilize on her own while were there so we were sent home. Throughout the night and into the next day her condition worsened and she was requiring alot more oxygen. She went from 3 liters to 5 liters. We made it to  her pulmonologist office and were admitted from there to PICU at TC Thompson. She has been on high flow oxygen and nitric oxide since yesterday. The reason for her rapid decline is still unclear, a test for viruses (like cold or something similar) has been done and we are waiting for those results. She also had another echo done on her heart to check if her pulmonary hypertension has worsened and could be the cause.
    At this point she is doing ok with the nitric and high flow oxygen. The doctors are working on a plan to figure out what the next step will be once she is stable. The rapid decline we see with Elizabeth when her saturation drops so quickly and the fear of not making it to the hospital in time or something bad happening at home has become a major concern for us and the doctors. At this point we are just waiting to see.
    I try not to express my fears on here but the past few days I have been more scared than ever about Elizabeth and her condition. I hope that Matt and I are making the best choices for her. This is the hardest thing either of us have ever had to do and it breaks my heart to her struggle everyday just to breath. I wish more than anything that we could just make her better. I hate relying on other people and in this situation thats all I can do, is hope that the doctors are providing us with the best options and we make the right choices.
  Please continue to keep Elizabeth in your thoughts and prayers, she truly is the strongest person I have ever know!

Sunday, August 15, 2010 9:51 PM, EDT

As most of you know we FINALLY made it home Friday after 23 days in the hospital!!
Elizabeth is doing well, we are still adjusting to the feeding tube and feedings every 3 hours plus all her medications. Matt is planning to go back to work tomorrow and I will go back sometime soon. Just waiting to make sure Elizabeth has adjusted well and she has numerous appointments here at home, to get her back on track with her doctors and with physical therapy. We have followup appointments scheduled in Sept at Vanderbilt so it looks like we will be making monthly trips up there. Elizabeth still has a long road ahead but we are thankful for the accomplishments she has made so far and hopeful that she is headed in the right direction

Sunday, August 8, 2010 9:44 PM, CDT

Its been a few days since I last posted, things are stressful and we are ready for Elizabeth to feel better and for us to be able to go home and have a "normal" life. In addition to Elizabeths rare lung disease, pulmonary hypertension, failure to thrive issues, we now have another diagnosis to add to the list.
We got the results from her brain MRI which shows she has a neuronal migration disorder called subependymal heterotopias.  This disorder can cause seizures, developmental an intellecutal delays. There is nothing to do as far as treatment for the disorder unless she starts having seziures and needs medication. At this point she will just be observed by neurology and we will continue with her physcial therapy to ensure she does not get farther behind developmentally. Knowing about the disorder in advance will better prepare us for any other possible delays she may have. And if she does ever have seizures we will know the cause and can be prepared. We are trying to take this new issue as it comes, just because the disorder says these things may happen doesnt mean that they will.
We were planning to go home tomorrow (Monday) but Elizabeth had a slight fever on Friday and it appears she has an infection arouond the gtube incision on her belly. So they wanted us to stay a little longer and start antibiotics to make sure she was doing better. Hopefully we will be leaving Tues or Wed now.
Matt and I both truly appreciate everyones concern and support. We are beyond grateful to our parents and sisters who have taken time out of their normal routine to make NUMEROUS trips and overnight stays to be here with and for us. We have had someone with us everyday since we have been here! We know there are lots of people who are not fortunate enough to have that kind of support and hope everyone knows how much we appreciate it.

Wednesday, August 4, 2010 9:35 PM, CDT

Elizabeth has done well with the gtube. She was given pedialyte throughout the day yesterday to see how she tolerated it and started on formula feedings today. She has done very well and hopefully will be released within the next day or so. She also had occupational therapy today and did very well with it. We did get her MRI results and I will post more about that when I get time and can provide more information about it.  Thank for your continued thoughts prayers and support!

Monday, August 2, 2010 9:00 PM, CDT

Elizabeth had surgery this morning and did well with it and coming off the ventiator. Her surgery was to put in a gastric tube (gtube) and a procedure called nissen fundoplication where they wrap the top part of her stomach around to make the opening smaller so that she cant reflux. The reason she needed the nissen was to keep reflux from causing her to aspirate and irritate her lungs more. She was in recovery for a little while, and came back to the room where we are now . She has done well all day she is in some pain when she wakes up so she has been on pain medicine and sleeping alot. She hasnt had anything to eat since last night and they do not plan to start feeding her until the morning to give her stomach time to get used to the tube. They will start by just giving her pedialyte through the tube to make sure she does ok with it and then we will move on to giving her formula. She will be here for at 3 to 4 days for her body to adjust to the tube and for observation to be sure she is able to tolerate the feeding and make sure we understand how to use and care for the tube.
HOPEFULLY this will be the last major thing she needs done and this will put her on the right track for gaining weight and getting her lungs to grow.