Sophia's Sketch

Sophia's drawing.   I labeled it for her.

August 28

Dear Family and Friends,

We thank God for your faithful prayers on Sophia's behalf!  Knowing that you are all praying has been an indescribable source of comfort for us.

Wednesday's CT-scan went well.  Sophia did great.  Although we hadn't really talked with her about the last attempt, she realized that it was important to be still and quiet to get the pictures.  As we pulled up to the hospital, she said, "I'll try not to cry this time."  Bless her heart!  We reassured her that we knew it was hard, and that she was scared, but it would be better this time since she could be asleep.  We told her she was very brave - this time as well as the other!  

As we talked in the car afterwards, I told her proud of her we were. 

Glenn and I have seen the Lord working in her during these trips to Cincinnati - how He is helping her learn more about self-control, and trusting Him to take care of her when she is afraid.  We do rejoice in this - knowing that He is using everything she is experiencing to grow and mature her spiritually, to demonstrate His sufficiency in her life - to His own glory.  To draw her even closer to Himself.

We were very impressed with Dr. Manning.  He spent about an hour with us, showing us the CT-scan images and explaining them, and the indications very thoroughly.  He was kind and gentle with Sophia and talked directly to her, asking her questions about herself.  After much conversation and questions, we have a plan for helping Sophia that we feel is best for her.  

Essentially, her implant is doing a better job than what was at first suspected.  He has not decided yet that he will not replace it with something larger, but he needs to first check the scar tissue that is surrounding it and see how firmly it is holding it in.  He said sometimes it can be pretty "sticky" and cause more problems trying to remove it, than if he just left it for now.  He said that around her peak growing years (about 10-12 years old), there may be more of a need for replacing it - but he was pleased with how good of a job it is actually doing at this point. 

 The slit into her lung is indeed being pressed upon by the aorta - but not as drastically as when she was a baby, prior to the original placement of the implant.  Her heart is not in the normal space, but is instead shifted over next to the implant.  But - he said he was not surprised to see it there since the implant doesn't have the supportive pressure that her lung would have had to keep it on the other side.  He said that replacing the implant with a slightly larger one would help shift it back some, but the heart doesn't respond well to being maneuvered that way, so he wants to provoke it as little as possible.  Her left lung has herniated over into the right space fairly significantly, but again, he wasn't surprised by this and said it is to be expected to a large degree since it is working the load of 2 lungs and has that capability of movement based on the extra space between the implant and the area that her R lung would be taking up.  He told us that lung tissue is much more accepting to being "pushed around" and so he is more comfortable manipulating it, than her heart.

So - he is going to perform a surgery called an aortopexy - and possibly replace the implant at the same time.  Typically, in an aortopexy, the aorta is sutured to the sternum, to provide relief to the airway (or windpipe- as he called it).  So it is suspended above the trachea - attached to the sternum, which also pulls away the pulmonary artery at the same time.  Since so much of her left lung is blocking the sternum, due to the herniation, he feels it would more effort to the tissues  - too far away after he tried to avoid the lung tissue - to attach the aorta to the sternum.  Instead he wants to attach it somewhere closer, perhaps a rib, using the pericardium, the sac around the heart.  He has performed this procedure many times before (isn't that incredible?) - although it is more commonly attached to the sternum.  

Before he does the surgery, however, he wants all the operative information from our hospital from her pneumonectomy (which was done when she was only 6 days old) and from her original implant surgery (when she was 12 months).  He also wants to have an echocardiogram done.  He said it is possible that because the lung is oxygenating all of the blood for her whole body - a job usually performed by 2 lungs - blood pressure could build because of the force of moving so much blood through one lung.  Symptoms include shortness of breath - which of course, she has had.  While he doesn't assume this is an issue, he said it is best to check it before performing surgery.  If it is an issue, it can be managed with blood pressure medication - and he feels it is better to have that information at this point in her physical development, than to not check it and it become a dangerous issue for her later.  Currently, the echocardiogram is to be scheduled the same day as the FEES study she is to have on September 23.  After he has seen the results, the surgery will be scheduled.  Probably about 1-2 weeks following the echo.   Her hospital stay will probably be about 3-4 days, barring complications, and her pain management will be pretty good - she should be on Tylenol with codeine by the time we are home.  He expects she will self-monitor her activity level well on her own afterwards, and hopes that she would be resuming her "norm" by a few weeks.

Sophia sat quietly during the visit.  I ended up giving her my notepad and pencil when she whispered in my ear, "I'm ready to go home now" about halfway through our time with Dr. Manning.  He had been going back and forth from the different scan images as we talked- and we were able to see several pictures of Sophia's chest, her trachea and the aorta and pulmonary artery.  Some of the pictures were in 3-D and he was able to manipulate them, rotate them up to 360 degrees, on the computer.  It was amazing to look at those images - and see what is happening inside of her - in color pictures on a screen.  He was able to do one "layer" at a time - so just the trachea, then trachea plus aorta and pulmonary artery, etc.  

Sophia had been diligently and quietly drawing while we talked.  After some minutes, she stopped, smiled, and held the notepad out to us.  She had written "me" at the top of the page.  Underneath, she drew a picture of her trachea - just as it had looked on the screen.  One main bronchus, with rings of cartilage encircling it from the top down to the 3 little "branches" splayed out like fingers toward the left.  The Dr. was surprised - and smiled broadly.  She showed us other pages, copies of the ct-scan images we had looked at, of the different views, the one with the aorta pressing on the trachea, etc.  All titled simply, "me" at the top of the pages.  Then on the way home, she drew several more, including a cross-section that had the windpipe hole, her left lung, and what I'm guessing is the pulmonary artery.  She was pretty pleased with them.  

Typically, children draw animals, their mommies and daddies, etc., Sophia drew her airway, aorta, lung.  :)

We can't adequately express our gratitude with words.  God's obvious care of her - His wisdom and guidance regarding her doctors, the visible fruit of His Spirit at work in Sophia.  And your prayers for her.  It seems I really can't say it enough.  But, again.. thank you!  

We will certainly feel most relieved when the surgery has been successfully accomplished, but God's peace prevails.  

Hope you are all well- We send our love to each of you and your precious families.

Love,

Kelly for the Orr's

Fall 2009

August 17

Hi Everyone,

After talking with another nurse today at Cincinnati CH, Sophia's CT-A has been moved up to August 24th - a week from today at 9:30 am.  Her appointment with Dr. Manning is now Friday, August 27.

We're quite relieved.  

She's complained of a sore throat this week and is sniffling, her breathing is a little raspy.  We're hoping she's not coming down with a cold.  

Please continue to pray that God would sustain her health, and for wisdom for the doctors who are in charge of her care.

Thank you,

Love,

Kelly for the Orr's

August 11

Hello Dear Family and Friends,

Just a quick update to give you the dates to be praying toward for Sophia's next tests.  Sophia's CTA - ct-scan with angiography - is scheduled for August 26 in Cincinnati.  We are on a cancellation list - should an appointment come available sooner.  Afterwards, she will see the cardiovascular surgeon, Dr. Manning, the following Friday, September 3.  He was ready to see her this Friday - but needs those scan images first.   Lord willing, once they have the information from the scan, they will know better the course which will be best to help her.  

Dr. Wood did review last Friday's scopes again and noticed the presence of lipids in Sophia's lung, which is an indication she may be aspirating.  That means she is choking on food or liquid and it is going into her lung instead of her stomach.  Because it is hard to swallow when you are breathing rapidly, it is easier to aspirate.  One study used to find out more information is called a FEES (fiberoptic endoscopic evaluation of swallowing) and she is scheduled to have that on September 23.   She will also do a video swallow the same day which should yield more information about aspiration and whether or not reflux is an issue as well.

We're thankful that school helps keeps things normal and busy around here.  Latin declensions have never seemed so appealing. We still continue to be blessed with an overall sense of peace, which we thank God for, knowing that He is in control of all of it.  And thankful that He is.  

Thank you for your continued prayers for our family.  You are all such a blessing to us!  We thank God upon every remembrance of you!

Love,

Kelly for the Orr's

Virginia Beach 2009

August 7

Good Morning Dear Family and Friends,

I'll try to be as succinct as possible - yet give you as clear a picture as I can about where things stand currently.

Due to some miscommunication, the CTA (CT scan with angiogram) on Thursday couldn't be done.  When we got to the hospital Thursday evening, they told us that it had to be scheduled  and although they had her orders for it, she hadn't been put on the schedule.  So we called the next morning on the way to the hospital for the scope and they put her on for 11:00 am.  Unfortunately, because Sophia didn't wake up from her surgery well - she was crying and unhappy, when we went down from the recovery room to Radiology, they weren't able to complete the scan.  They were patient and tried for about 45 minutes and at one point, it looked promising - Sophia was quietly laying in the scan machine on her back, arms above her head holding Glenn's hands as he talked to her.  But when the tech put the contrast solution in the IV port in her hand, she felt the warmth as it went in and started crying hard and pushing up her chest.  The tech had to stop right then.  The contrast solution takes only 10 seconds to  work and during that 10 second period, Sophia needed to be perfectly still and breathing quietly.  Due to the composition and side effects of the contrast solution, it can not be re-administered for 24-48 hours.  So we have to reschedule the scan - and the next time they are doing it with sedation.

The scopes with Dr. Cotton and Dr. Wood went well - they were able to see that her tonsils aren't hindering her ability to breathe, Dr. Cotton didn't even feel they were grossly enlarged.  They could see that her upper airway and the branching down in her lung look normal - "pristine" is what Dr. Wood said.  They don't have any excess fluid in them, etc., they aren't abnormally shaped.  But at the point of the slit, Dr. Wood said that he was able to see pulsing - which he feels is the aorta pressing against the airway.  He had talked with us prior to the scope surgery yesterday morning and said that he suspected the trachea is twisting around and being pressed upon and pulled away from the opening into her lung by the aorta.  So what he saw inside seemed to confirm his suspicions.  However, the CTA images are critical for him to be able to know how to proceed.  He needs a clear picture of what is happening *outside* her airway, now that he knows what the inside looks like.  The very good news is that he doesn't feel it is "the fire alarm I expected it to be" - meaning she is not in immediate danger; we are not as under the gun timewise as we were concerned.  He took a piece of paper and rolled it up and blew gently through it - saying that is like breathing through a trachea.  Then he said that for Sophia, because she has a "dynamic" airway - the floppy cartilage, which is also being compressed- when she has increased physical activity which requires her to breathe harder, hers would look more like what would happen if you took the same tube of paper, and blew very hard and fast through it.  As he demonstrated this to us, the paper he was breathing into was collapsing inward and crumpling, and then opening up again. So he has told us to try to keep her activity level low for now.  So - as long as we can keep her quiet and not very active, she will be comfortable.

Crystal(Dr. Cotton's nurse) called us on our way home from Cincy and told us that we need to get the scan images very soon, as well as see the cardio surgeon, Dr. Manning.  She gave me the number for Dr. Manning's office.  After I talked with her, I called his office and was told she was already on the phone with their nurse making arrangements for the scan to be scheduled the same day as a visit to Dr. Manning.  Dr. Manning's nurse called me back to let us know they are working to get Sophia in as soon as possible - and they would schedule her CTA and her visit with Dr. Manning and try to let me know on Monday.  

Sophia was just happy to be coming home.  She didn't say much at the hospital except, "I don't like today."  She has been my little shadow yesterday and today, constantly holding the little stuffed crocodile she calls "Alexander", that a nurse gave her yesterday.  She and Isabella were sitting next to me on the couch yesterday afternoon, and Isabella said something about wanting a little toy horse like the one Daddy gave Sophia at the hospital.  Sophia looked at her very quietly and seriously and said, "Isabella, it's ok to wish you could have my toys, but don't wish you were me.  I had to have things done to me today that hurt me.  They hurt my arm and my hand.  And the worst thing was that Mommy and Daddy had to go away.  They couldn't stay with me and I was scared."  Then she looked at me, and leaned against me quietly and said, "I can't talk about it anymore."  

It's hard to explain our emotions right now.  The anxiety of feeling that she could have respiratory failure at any minute is greatly relieved.  But I think I was hoping that if we could just get her there, once we did, they would know exactly what to do and just do it - yesterday.  But, we do feel we're where God wants us to be right now - on the right track with people who aren't intimidated by her issues.  Dr. Wood did tell us briefly that he is already considering possible courses of action - replacing the implant is one, but he's not sure it's enough, or slicing her trachea and pulling it out from behind the aorta and reconnecting it on the other side of the aorta so it can't be pulled over again.  But he didn't talk at great length about them - instead told us that he needs more information before he can proceed.  And other doctors to consult with about her.  One nurse there told us yesterday that Dr. Wood is typically very conservative and won't say much until he is sure of his course of action.

Thanking you for praying for her seems an inadequate way of expressing to you all how much we do appreciate it and desire your prayers.  Truly - your prayers are so important to us- and frankly, knowing that you are praying is so encouraging - so thank you for telling us you are.  

We love you all,
Kelly for the Orr's

late summer 2009 - twin love

August 4

Good Afternoon Sweet Family and Friends!

Just wanted you to know that things are set for Thursday and Friday.  Since the Radiology Department doesn't require a scheduled time for the chest x-ray, and they're open till 11:00 pm for registration, we decided to leave after dinner tomorrow evening.  Glenn and Sophia and I will go straight to the hospital and get the x-ray, then go to Ronda's brother's house to stay the night.

Friday morning, Sophia will have a flexible bronchoscopy with Dr. Wood, an associate of Dr. Cotton, as well as a laryngoscopy/bronchoscopy with Dr. Cotton.  Presumably, after they've looked inside themselves, they will be able to discuss with us their recommendations.  We'll let you know, of course.

Thank you, again, for your prayers.  Truly, the Spirit has brought us great comfort and peace this last week or so.  We know that your prayers on behalf of our family have been heard, and answered.  

Love,

Kelly for the Orr's

last fall 

July 31

Hello dear Family and Friends,

I'll try to give you as many details as possible, but not make it too lengthy.

Yesterday (Friday) morning, I called Dr. Cotton's office and spoke to his nurse, Crystal, again.  She told me she would be presenting Sophia's case next Wednesday to an entire team of interrelated specialists at the Cincy hospital - pulmonologists, airway specialists, ENT's, nurse practitioners, GI, cardio-thoracic, to name a few.  She said they all meet once a week - Wednesday - to discuss new and current cases -  something called an Aerodigestive Team.  Then a week later, would make recommendations about each case and start a general comprehensive testing.  We would begin a 3 day period of introduction by first meeting the team members who would be working with her.  She told me about the various tests they would do on Sophia - gastric reflux testing, Ct- scan, esophagus study - as well as her trachea, speech pathologist, swallow study - where they would use an endotracheal tube in her nose, otolaryngology, bronchial washes, and more that I didn't write down.  All on an outpatient basis, but requiring us to be in Cincy for 3 days consecutively.  She said that if she couldn't call me next Wednesday, for me to call her Thursday.  It looks as if doing all of that could take a few more weeks, and then Dr. Cotton would scope her - another bronchoscopy like what our Dr. Sufi did this past Monday.  

Our former pulmonologist - who I have told you about - Dr. B, has called me every day from his cell phone to offer suggestions and encouragement.  He has been invaluable - and inspiring.  He told me that he has been thinking of Sophia daily and all throughout the day.  He reminded me that as her parents - we are her strongest and best advocates.  That we have power regarding her care.  Need I say how much we miss having this doctor for Sophia's pulmonologist?  As he was talking to me yesterday, Dr. Cotton's nurse Crystal beeped in.  She told me that Dr. Cotton was to be in the office at noon for a brief time, she wasn't sure she could talk to him, but she would try.

I then talked to Dr. Young's nurse practitioner, who told me she had sent an urgent email to their entire staff of pulmonologists and airway specialists at 9:20 Friday morning.  She had spoken with one of their pulmonologists and he knew Crystal had Sophia's info.  They decided that at this time - having Crystal take the lead in Sophia's case would be best for Sophia - and they would follow along and work with the rest of the Aerodigestive team.  She told me to call her if I needed anything. 

At 2:30 I was in the car with the kids when Crystal called on my cell phone.  She told me that she had in fact been able to speak with Dr. Cotton for a few minutes at noon.  Then shortly thereafter, he called her into his office to ask her for more information about Sophia.  Per their discussion, the course of action has changed significantly for Sophia.  Dr. Cotton and one of his partners are going to do a bronchoscopy on Sophia next Friday. We will go on Thursday to meet them, as well as their cardio-thoracic surgeon, and have an angiogram performed on Sophia - they want to make sure there is nothing that with her heart that might complicate the scope on Friday.  They are also checking her for reflux, to make sure that they don't have any problems that way while the scope is in her airway.  In essence, Crystal told me that they are switching the usual order of procedure for new patients in Sophia's case.  The comprehensive testing will be done after the scope, because Crystal told Dr. Cotton she feels Sophia has an "unsafe airway" and should be seen immediately.  She told me that they would rather know how severely Sophia's airway is compromised, and then backtrack and do the comprehensive testing, then wait any longer than necessary while Sophia's airway could me further twisted.  She said they do have inpatients on trachs who have serious illnesses, but their airways are safe, Sophia's isn't.  

A very dear friend, Ronda W., has a brother who lives in Cincy and she has contacted him - with the result that he has offered for Glenn and Sophia and me to stay at his house while we are there.

At this point, we are waiting for the scheduler from Dr. Cotton's office to call (most likely Monday) to give us details about time, etc. for next week.  Crystal told me that she had had Sophia's chart all day up to that point and hadn't had a chance to give it to their insurance rep, but she was not expecting issues with precertification for Sophia.  Thank goodness the whole health bill fiasco hasn't messed everything up yet!

So you see, how God has directed everything to His pleasure- through your prayers on Sophia's behalf.  We can't thank you enough.  Please continue to be in prayer - and to ask others to pray for her as well.  We are watching her closely - and I have begun to see some mild retracting in her abdomen the last 2 days - evidence of how hard her body is working to get air.  Her sats are still normal - her heart rate is slightly elevated.  When she talks, she takes a deep, audible breath, then says something very quickly and breathlessly, then pauses and repeats the process.  Her little clavicles come into relief when she is exerting energy - after going up and down the stairs, for instance.  But she is happy - she is singing in her high, breathless voice, continually.  And she has been really producing lots of drawings - of princesses, and mommy and daddy, and frogs.

We are so thankful for all of you - for your prayers, and also for your words and email of encouragement.  Dr. B said to me today that I sounded strong - I don't feel it.  But I know that God has sustained us this week (can it really have only been 5 days so far?)  - we feel supported and encouraged - and hopeful.  We know the Spirit's leading has blessed us - and we know that your prayers have been instrumental.  

Please call or email with any questions.

Love

Kelly for the Orrs

July 30

Hello all,

Yesterday, I was able to talk directly with one of Dr. Cotton's nurses and with Dr. Young's nurse practitioner assistant.  The Lord blessed both the words that He gave me to say to them, and He moved their hearts in such a way that at the close of each conversation,  each nurse told me that she was going to impress upon the doctors that she feels Sophia needs to be seen ASAP. 

Dr. Cotton's nurse asked me many medical intake type questions about Sophia and then asked me to tell her what is happening now.  She said they have all the medical records and Glenn was able to fax our insurance info, too. Yesterday, our pulmonologist's office was able to email scanned copies of Monday's bronchoscopy.  I talked to them, too and they told me that they had been unable to send the bronch report from Monday because "it hadn't come up right away, for some reason.."  Good thing I called.  Dr. Cotton's nurse told me that Dr. Cotton - or Dr. Rutter (if we wouldn't mind) - would very likely do another bronchoscopy themselves right away, as well as other assessments which would mostly be outpatient.  She said only one of the initial tests may require an overnight stay.  We will talk to their anesthesiologist as well when we go, due to the risk of having Sophia under general anesthesia.  She said Dr. Cotton would probably want a team of specialists to help Sophia since her issues are complicated.  As we were getting off the phone, she assured me that she was going right then to "find Dr. Cotton" - that she would call me today (Friday) with more information about when Sophia can be seen.  She said she was going to tell him that she feels he should "expedite" their efforts to see Sophia.  I talked about the tonsillectomy and our concerns with doing that when they are not presenting as serious a problem as the twisted slit - and she agreed and said that Dr. Cotton would most likely want to take care of the twisting first.

Dr. Young's assistant, who is a nurse practitioner, said that she and Dr. Young had already had a brief discussion about Sophia per info from the short messages I had left the last 2 days.  Dr. Young was thinking, even prior to my conversation with the NP, that Sophia may need a team of interrelated specialists.  I told the NP about our contact with Dr. Cotton's office and that they have her records and intake information.  She asked me the name of the nurse from Dr. Cotton's office that I had spoken with  - and she knew her, and said she would contact her.  Dr. Young is out this week, but the assistant has daily contact with her by phone.  At the close of our phone call, the NP said she felt sure Sophia should also see one of their airway specialists, who she said were "fabulous".  She also said she would call me today with news from Dr. Young.

Sophia is going along as usual, but the sucking in at her throat almost takes my breath away because it is so noticeable.  I asked her not to run today- until she feels better and it's not so hard for her to breathe.  She has largely scaled back her activity, these past few weeks especially, but occasionally tries to run and play in the house with Isabella and Olivia.  She told me, "But I want to run, Mommy"  and I asked if she could just wait until it's easier.  I have begun checking her stomach for signs of retraction when I see her breathing rapidly, so far she's been ok, as far as I can tell.  Her oxygen sats continue to be in the normal range, even when lying down at night.  

The kids and I are continuing to do school and try to follow our normal routine.  Glenn and I are so thankful for your prayers - please continue!  God is already at work answering them.  Please contact us if you have any questions.

Love,

Kelly

July 29

update on July 29....

Good morning, Family,

A quick update.

I had a fruitful conversation with Dr. S - he told me that they are doing much to push to get Sophia to Dr. Cotton and that Dr. Hamaker will be especially helpful due to her regular communication with him.  He has spoken with her and Dr. Abraham today about Sophia.  Dr. S. feels she is not "emergent" - but certainly urgent.  She is able to be here at home and is not currently intubated at the hospital.    He does think she should see Dr. Rutter and most likely some others there in Cincy.  He told me they are the best and know more than he does.  He stated he is happy to help - and has had the Peds Pulmonology staff continue to have phone contact today with Dr. Cotton's NP.  I asked about the slit - whether it is rigid or fixed, or whether it was collapsing due to the bronchomalacia he had mentioned on Monday.  He said it is not rigid - it is twisted and because it is soft, it is collapsing.  He still feels the implant is going to be the best option.  He didn't mention performing the tonsillectomy.   I also asked him about her heart and what the CT-scan showed regarding its position.  He said the scan in early June showed the heart in the correct place, that it was not rotating as it had when she was a baby.  

I think that was most of it.

I also talked to Jenni, Dr. Hamaker's nurse practitioner, and she said Dr. Hamaker is out until Friday.  But she remembered Sophia when Dr. S came on Monday to discuss her at their office.  She was quite sympathetic to me and listened to me - encouraging me that indeed, Dr. Hamaker will be very beneficial in helping us to get Sophia in quickly to Dr. Cotton.  She said that it will be important for Dr. Hamaker to assess Sophia in person on Monday, to give her more info to share with Dr. Cotton.  Jenni told me that they realize Sophia is in a delicate condition - they want to help us and they understand our concern.  

The nurse practitioner for Dr. Lisa Young - Director of Pediatric Rare Lung Diseases Program at Cincy, called while I was talking to Jenni, so although I couldn't talk to her in person today, we have left messages for each other.  Dr. Young is the long-time pulmonologist for a family we know intimately.  My friend told me to call Dr. Young's office and let her know that they have referred us to her.  My friend's son is currently in Dental School with his tuition paid in full, due mostly to the letters of recommendation and assistance of Dr. Young.  I thought it might help Sophia if she can see Dr. Young - but minimally, I would appreciate talking to her about Sophia if I'm able.  

I did talk to Dr. Abraham's office, too.  Sarah B is out this week, but providentially, her replacement used to work in the NICU and remembered taking care of Sophia - particularly because of the rare diagnosis of Lobar Emphysema.  I explained Sophia's situation and that as a patient of Dr. Abraham's, we were hopeful he could also take steps on her behalf to see that she is seen quickly in Cincinnati, by contacting Dr. Cotton's office himself.  Dr. Sufi told me later that after talking with Dr. Abraham again today, they both agree strongly that she should go to Cincy and that Sophia should benefit from as many as possible.

I did also leave 2 messages today with Dr. Cotton's nurses - both times the receptionist said that they would be calling me very soon.  So I'll try again tomorrow morning - 

I want them to see that I am concerned, but I don't want to annoy them so that they avoid talking to me - it seems a bit of a fine line.

I have found myself looking at Sophia more often than usual - not just to assess her breathing, etc., but just looking at her.  At the beautiful way God has made her - and thinking how she has grown from a baby to such a sweet, little girl.  Although she is reserved at church and with people she doesn't know well, in her home she is always singing, playing the piano, pretending to be a horse - or a ballerina, drawing almost constantly (especially pictures of mommy and daddy) - she is filled with life.  It's good of God to give me something to remind me of the preciousness of our children, because I do take them for granted so often.

Thank you for praying for Sophia and for your encouragement - we don't just appreciate it, we need it.

Love,
Kelly

2 sisters expecting - and lots of cousins- Sophia in the middle

prayer sent from an unknown saint

a dear friend and her family wanted to help by asking their blog readers to pray for Sophia. One precious reader, a sister in Christ (Leah) - whom I may never know this side of heaven - posted this prayer by Martin Luther.  I read it almost every day...

 from the Lutheran Book of Prayer:

“At Times of illness in the family…

Almighty God,with whom all things are possible, hear our prayer as we come to you in trouble and distress. You are our hiding place. You are our very present help in trouble. We have no other refuge in an hour like this. Gracious Lord, You can heal. Your grace can restore to health and give us strength to carry on. We know that you are compassionate, for you have sent Your beloved Son Jesus Christ, to suffer the death of the cross in order to redeem us and make us your own. As you did not forsake Him, surely you will not abandon us in this time of great need. As You raised Him from the dead on the third day, so we ask that you would also raise us up with him from this present danger. O Lord, we humble ourselves before you and confess our many sins. We pray, merciful Father, for your grace hour by hour. Forgive us and restore us, for You have made us Your children in Christ. Remove the worries and anxious fears that would crush us. Give us grace to trust in You whose will is wiser than our own, for we have been baptized into the death, burial and resurrection of Jesus Christ, our Lord. Amen.” Martin Luther

July 28

to family and friends.... July 28...

Good morning all,

Glenn and I want to keep you all as updated as possible about Sophia. 

I emailed our old pulmonologist, Dr. B. on Monday afternoon to tell him about the bronchoscopy and seek his help.  He took excellent care of Sophia until he moved to NJ 4 years ago.  I have had just one correspondence with him since then, but after my email to him, Monday, he has been a huge help.  He sent me his phone number and we talked yesterday.  He encouraged me and Glenn to be as assertive as possible on Sophia's behalf, urging us to trust our instincts about her.  He wants us to get her to Cincy to a couple of docs there immediately.  He actually called back twice yesterday, to tell me what I need to take of Sophia's records, and to give me immeasurable help and encouragement about how to get her there quickly.  Because he isn't privy to her scope, he has to go on what info I can give him - so there are questions he has that I don't have answers to.

I also talked with our pulmo's office several times, first to ask their help in getting Sophia quickly to the specialists.  They were able to contact the main dr's office., Dr. Cotton, and faxed her medical records, scope pictures, and other important info to his nurses, who are supposed to call me.  Although, per Dr. B's urging, I am going to call them persistently to speed up the time they might otherwise take to get to me.  Hopefully, today, I will be able to talk to someone who I can impress the urgency of Sophia's condition to sufficiently that they will be an advocate to Dr. Cotton for her.  

Glenn was able to pick up 4 cd's with all her chest x-rays and ct-scans since birth - which Dr. B told me to take when we go.  (I mentioned that to you all before)

Dr. S also called and although he is open to us getting the 2nd opinion (his idea originally on Monday), he did not offer to do anything to help us get her to Cincy, and restated his opinion that she should have the tonsillectomy first.  I am planning to call him back today to plead with him to help us get her to Cincy, using his position as her physician.  Hopefully, God will give me the right words to say that will help encourage him to be more active in helping us.

I also have a friend, Terry, who has a strong connection with the doctor who is the Director of Peds Pulmonology at Cincy, so I am going to try to make inroads with her today, using Terry's name as a reference.  This doctor works with the other specialist - who is a pulmonologist - that Dr. B - and Jason E have told us to see.  He has done pioneer work in airway reconstruction which is so new, it isn't published yet.  His name is Dr. Rutter.  

In addition - I am going to call the assistant to the cardio thoracic surgeon who did Sophia's implant.  I have been on friendly basis with her since Sophia's hospitalization when she was given the implant - mostly due to the fact that we both have twins - hers are 6 months younger than the girls.  I am going to ask if she or Dr. Abraham can also talk to the Cincy people and persuade them to see Sophia right away.

Also - Sophia has a 10:30 appt Monday with the ENT who would do the tonsillectomy.  This ENT has regular communication with Dr. Cotton -and could very well be a big help to us.  Dr. B has suggested to me that Sophia shouldn't have any procedures done - including the tonsillectomy until she is able to be comprehensively examined by the specialists.  He said since she isn't having any issues related to the tonsils - no loud snoring or sleep issues - to take them out could prove more harmful than helpful at this point.  He said that there are so many complications that could arise from the tonsillectomy that we would certainly lose precious time getting the very serious issue of the slitted opening to her lung and whatever structural complications are present there that are unknown right now.

So please pray that the Lord would be gracious to us - and that He would cause the right connections to be made, and the hearts of the right doctors to be moved in Sophia's favor.  That they would be able to see her immediately.

Love,
Kelly

the girls' ballet recital - Coppelia

July 26

email to a friend - who is also a pulmonologist -  July 26....

Glenn and I wanted to write to you to tell you about Sophia.  We began to notice in late March and early April that she was not returning to her "normal" health in between respiratory illnesses.  She was sick all winter - and on antibiotics and Orapred every month from November through May of this year.  After noticing other physical symptoms which puzzled and concerned me, I took her to her pediatrician and talked with him about her.  At the time, she was sick with yet another respiratory infection and needed antibiotics and steroids again.  She hadn't been to Peds Pulmonology for 6-8 months.  The ped. felt that she was struggling and told me to take her to Dr. Sufi.  After seeing her in late May, he ordered CT-scan and chest x-ray which showed that she was having twisting of her airway again.  This morning, he and Dr. Abraham performed a bronchoscopy on her.  They found several things - first that her tonsils and adenoids are extremely large, blood vessels in her airway show symptoms of an asthmatic, she has a nodule on one of her vocal chords, and some little bumps in her airway that are typically present in patients with reflux or allergies.  She is having collapsing at her pharynx and he said bronchomalacia is present as well.  But the most concerning thing is that the opening into her lung is a slit.  He is contacting Dr. Hamaker about a tonsillectomy to give immediate help, and then once she has recovered, he and Dr. Abraham are each to see her - about 3 weeks after that procedure.  He feels Dr. Abraham needs to replace the implant with something bigger, and that it will hopefully widen the opening.  But he isn't sure it will be enough.  He says that since not many physicians have experience with Post-Pneumonectomy Syndrome, he wants to bring as many experts on board as possible, so he will most likely send us to Cincinnati.  He says stents haven't been successful in children, so we may need to try something experimental.

June 6

Hello Everyone!

Just wanted to send you a quick update.

Sophia's adenoids are not enlarged - a blessing - so I don't know whether or not the pulmonologist will still want her to have her tonsils and adenoids removed, but I assume that he will not order a sleep study to check for apnea.  Praise God for that!

However, more sobering is that the CT scan does show a narrowing of her main bronch.   In other words, the implant is not continuing to provide adequate structural support in her chest cavity, so there is shifting inside of other organs (probably heart and lung - the nurse couldn't tell me) which is causing her floppy airway to be twisted, causing her breathing to be impeded, and the airway to collapse more intensely.  It will have to be addressed.  The pulmonologist's office called me and said they would be faxing the info to the surgeon and calling the cardio-thoracic surgeon's office.  Within 10 minutes I received a call from the surgeon's office saying that he was in the OR until late tonight, and had a few cases in the morning, but as soon as possible would review her case and call back to let us know what he plans to do and the time frame.

Here are my and Glenn's assumptions based on what we know and my conversation with the pulmonology nurse:

She does require surgery - but at this point the surgeon will have to decide if the implant he placed in her at 12 months has the capacity for more fluid.  And if so, will it be enough (we wonder) for her adult body?  If he has to completely replace the implant with another, which is obviously more risky, will it receive an amount of fluid that will continue to provide support into her adult years?  I don't know if they know.  I do remember the surgeon telling us immediately after her implant surgery that he had tried to put more fluid in the implant, but she had become unstable, so he pulled some back out until she was stable.  But that is all I know - I can only assume that they would put a small implant into a baby - and I know that breast implants come in different sizes (although my knowledge does not come from personal experience) - so I'm confident there are bigger sizes they could use.  We know that adding fluid to the existing implant - as long as it would work successfully in the long run, is the safest scenario.  So - alot of unanswered questions yet to be answered.  

But we are thankful for what God has given us so far.  So thankful and relieved that Sophia has a condition that can be addressed.  Thankful that He brought the issue to light by presenting symptoms for us to see.  Thankful she is older now than when the first implant was placed and she is also healthy!  When she received the current implant - she had an undetected bacterial infection in her lung that immediately blossomed into ARDS (which you can read about http://www.pulmonologychannel.com/ards/index.shtml)  

I read quite a bit online about post-pneumonectomy syndrome this morning - to refresh my memory, since that was the diagnosis which precipitated the implant surgery.  A pneumonectomy in itself is rare; in a child more rare.  Her original diagnosis of congenital lobar emphysema - which was the reason for the lung resection is also extremely rare - none of the staff had seen it in real life - only in textbooks.  Post-pneumonectomy syndrome is rare as well- since most patients who have a lung removed are adults - (due to carcinoma) and do not have "soft" insides.  I didn't read anything at all yet, (I'm still looking) about having the implant replaced due to problems.   So we have a rare, beautiful little sweet pea who the Lord has seen fit to bless us with - and who He has seen fit to bring glory to Himself in a rare way.  It's humbling to see Him work through her life in this way for His purposes.  We trust His Infinite Wisdom and Sovereignty in all of it!  What a loving, merciful, steadfast God He is!  We wait in faith to see His will accomplished through this new episode in her life - and our lives.  Also - we are incredibly thankful for you all - and for your faithful prayers on her behalf!  Thank you!!

We will update you as soon as the details about the surgery are made known to us.  

Hope you are all well!

Love

Kelly for the Orr's

Olivia, Sophia, Isabella

June 1

email to a friend ... June 1...

Sophia is the same - she had a CT scan today and I have to take her back for a neck x-ray.  She is not sick - but her breathing remains the same.

We're hopeful for answers soon. Thank you for asking!

Love,
Kelly

Alix holding Sophia at a wedding

May 26

email to friend on May 26..

Yes, we are better here - well, Sophia continues to have noisy breathing - I have no idea why.  Wondering whether I should call a dr. about it - it just won't go away.  And otherwise she seems healthy.  But that is not how she should sound normally...

Hope you are all well, too!

Love,
Kelly

Happy girl!

At Grandma's and Grandpa's house

Where to start?

I'm sitting here wondering where to start?

hmmm

Sophia.  She's why I decided to finally take the plunge and start a blog.  I have no idea if I'll actually stick with it - but I've decided it is really a good way of letting people know how she is doing, without sending long emails to people who really just wanted a short, "she's ok", instead of all the gory medical details that I feel compelled to include in the updates about her current medical issues.  Actually, those long emails are mostly for me - and for Sophia.  They help me keep information somewhere safer than my memory, and I hope one day they will be a way for her to look back and see how well God has taken care of her - over and over.  How well He has taken care of our family.  His mercy to us - to her.

In case some of you are coming in late, I'll try to succinctly flesh out some background that will, hopefully, help keep things understandable.

7 years ago I was pregnant with twins.  We were soooo excited!  In between 6 miscarriages, we had had 4 other fantastic children - Luke, Alix, Kate, and Noah.  I was sure I would make it to 36 weeks gestation at least.  Well, I was wrong about that.  The girls were born at 32 weeks exactly - after I was rushed to the hospital via ambulance due to the placenta previa I had.  Sophia Grace was 3 lbs  13 oz and Isabella Faith was 3 lbs 10 oz.  Both did great initially.  But by day 6,  Sophia was struggling to keep her oxygen levels up, and she began to have swelling on her right side.  I arrived at the NICU, just in time to see them finishing an x-ray of her.  Glenn was at work.  An hour later, they called me back to a room to show me some "very unusual results."  She had something called Congenital Lobar Emphysema - a very rare condition.  Her right lung was inflated to extremely large proportions - it had shoved over her heart and left lung, and caused her trachea to look like a "C", instead of a straight line down the middle of her body.  The alveolar sacs in the lung were taking in oxygen, but unable to give off carbon dioxide.  They were inflating like balloons.  She was in serious danger.

They operated on her, our tiny little baby, within 2 hours and ended up having to remove all of the right lung.  The neonatologist did come out once to tell us that it was "touch and go".  They didn't know how her left lung would respond to taking on full oxygenation duties for her whole body.  But God was merciful.  He orchestrated things perfectly - she did great.  Her left lung worked beautifully.

Over that first year, she developed concerning symptoms and by the time she was 11 months old, after several hospitalizations for pneumonia, etc. (including 2 when we were in different states on vacation),  an ambulance ride to the hospital because she turned blue in my arms at home, she was admitted for croup.  And within 3 weeks, diagnosed with another rare condition - Postpneumonectomy Syndrome.  Because of the space left in her chest after the lung was removed, her left lung had grown extremely large, and pushed her heart to the extent that it malrotated to her lower abdomen.
Her trachea was soft and collapsing inwardly when she inhaled - a separate (rare) condition called tracheamalacia.  Because of its "collapsibility"  (word?), the lung was able to shift as well as the heart, and it was being squeezed between her spinal column and her aortic arch.  She was almost in respiratory failure.
Surgery was performed and a saline breast implant inserted into the cavity where her right lung had been.  As the cardiovascular surgeon filled it with fluid, it pushed back her lung, heart, and airway in a more midline position.  She did have some very serious complications after the surgery, including ARDS and drug withdrawal.  But again, God was merciful.  He gave wisdom to her doctors, and strength to her little body.  She recovered.

Here is a copy of an email I sent to a friend recently, remembering that time..


Because of the space left after her right lung was removed, and because she has a rare condition where her whole airway is soft and collapses when she breathes, her left lung got bigger and moved over towards the space.  It also pushed her heart around and rotated it.  By the time doctors realized what was happening (because she kept getting very sick with respiratory infections, pneumonia, bronchitis, etc.) her airway was being flattened between her spinal column and her aortic arch.  She almost died - turned blue in my arms at home one day.  We rushed her to the hospital - and she was admitted, but because it is hard to see that type of movement on a typical x-ray, it took a couple of weeks before one doctor tried a spiral ct-scan - which showed it.  Meanwhile, she was on oxygen, I stayed there with her all the time because I was still nursing her and Isabella.  At any rate, I wouldn't have left her - she needed me to taker care of her.  I didn't expect the nurses to stay with her around the clock. The diagnosis was Postpneumonectomy Syndrome - a very unusual result of having a lung removed.  Our doctors had never seen it before- but the literature that was available said the best results were to use a breast implant in the space, which when filled with fluid, would push back the heart and lung and straighten her airway, lifting the aorta off of it.  It worked very well.  But she's grown alot since then - and because her airway never has firmed up (like they hoped), it is still susceptible to the shifting of her inner organs.

She almost died during her pneumonectomy, and then again after that implant surgery.  She had the start of a bacterial infection before they put the implant in, and no one knew, so because of the incredible stress to her body during surgery, it grew like lightening and she had a very serious condition called ARDS (acute respiratory distress syndrome) by the time the surgery was finished.  Her lung was filled with fluid - worse than what you would see with pneumonia.  She was on total oxygen support for about a week (I can't remember exactly) and then they gave her blood transfusions, which carried oxygen rich blood into her lung.  God gave them wisdom in their efforts - and used the antibiotics, etc - helped her body recover, get more sacs with oxygen than fluid in her lung. 

 Then she went through drug withdrawal - to keep a child sedated and unconscious all that time takes alot of drugs because they have high metabolism.  I'm not sure why a drug wean schedule wasn't established, but within hours after she was eventually discharged from the PICU to a regular floor (about 2 weeks after that surgery) she was in withdrawal.  It was terrible.  I didn't know what was wrong with her and I spent hours trying to calm her down.  It was her pulmonologist (the other one who has since moved, but who we still talk to) who came in around 10 or 11:00 pm to check on her and saw me with her.  After a few questions, he knew right away what was happening.  He gave orders to the nurses, (they gave her some meds), then took her from me and held her and soothed her till she fell asleep - it was a long time.  He stayed in the hospital all night - coming back to check on her throughout the night.  

When she did finally come home, she came home on oxygen. We had a big tank in our bedroom where her crib was - I had a portable tank that I had to take everywhere with us for a few months.   We had really long tubing for it, because she was crawling around, not walking yet, at the time.  I had to change it regularly - the nasal canula that is - and keep it taped to her cheeks.  She hated when I changed it!  So did I.  There were monitors at night to make sure her oxygen levels didn't dip too far - which they did pretty frequently.  When they did, we had to turn up the tank controls, etc.   

>>>>>

<<Oh- I forgot to finish the drug withdrawal part - 

They put her on a very strict wean schedule for 2 of the drugs she was addicted to - and I actually finished the wean schedule with her at home after she was discharged.  The pulmonologist said he had never sent a patient home on a drug wean - so we were sooooo grateful he let us.  It was alot of upheaval to our family - she and I had been at the hospital for about 7 weeks.  I came  home once during that time for Luke's birthday - and we had our babysitter stay at the hospital with her while I was home.  So for him to let us go home for the drug wean, instead of staying there was such a gift.  I almost panicked on the way home from the hospital that day, though, because I couldn't get the drugs at either of the 2 pharmacies I tried (they weren't able to sell those drugs) and it was close to the time when they were to be administered.  I ended up going back to the hospital, a nurse came out and met me on the curb and gave me the drugs.  I was like a maniac about the time - because they told me how important it was - and I administered the drugs to her at the exact time that was printed out for me for her schedule.>>

<<There are soo many ways I have felt guilty over the years - from the time I was told about the postpneumonectomy syndrome, til now.  If only I would have had the doctors look at her more closely when she was a baby, over the latter months of that first year.  I remember she looked pale, seemed to lose weight, although she didn't actually.  Her breathing was labored, she eventually had retractions in her abdomen (which is very, very serious) and had a "barky" kind of sound (stridor) when she sneezed or coughed.  I even took her to the doctors during that time period, for regular visits, and they didn't notice anything noteworthy.  I was so inexperienced concerning her physiology.  I had healthy children - I didn't know what to expect with a child who had 1 lung.  I assumed some of it was normal.   But still - I have always felt I should have known.  I should have been more assertive - asked more questions.   Ever since that first surgery, I think I have always been subconsciously looking for those symptoms again.  Assuming it would never happen again, but not able to forget it.  When I realized in late March/April that she wasn't sounding normal between her rounds of antibiotics and steroids, between the respiratory infections, and also noticed that she seemed thinner, yet wasn't losing weight- it was such a red flag, a feeling of deja vu.  

I remember that night in the hospital, when she was in withdrawal, talking with her pulmonologist.  He was essentially warning me that she might die.  And I told him that I knew God was in control.  I trusted Him.  I really had no other choice.

She is His.  So are all the other kids - we are thankful for every day He gives us with them.  Every minute.  >>>

<<


Not sure anyone is still reading at this point.  :)  So much for being succinct.
But, as I said, this is mostly for our family.  For Sophia.  Someday, I hope she'll read this, and be on her knees in thankfulness, again.

The reason I have started this blog at this point is probably starting to become clear.  Sophia is currently struggling with postpneumonectomy syndrome again.  I'm going to see if I can figure out how to post the other emails I've been sending to my family and our close friends the last couple of months.

So even though this might seem a little backward, it's the way I'm doing it! :)

here she is after her pneumonectomy (she's about 2 weeks old) - see how little she is compared to the passy

the girls - ready for a trip somewhere - Sophia's oxygen tubing in place - about 2 months after the saline implant surgery (they are about 14 months old here)