Monday, August 30, 2010

Where to start?

I'm sitting here wondering where to start?


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)

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