Transplants and Train rides

For those that don't know, Michael got his call for new lungs on September 6th and went into the OR at 1:04 am on September 7th.  He was in Pittsburgh for almost two months post and came home on Halloween.  Now that we are home and I am not in survival mode anymore, it has all hit me what he went through.  It was the hardest thing I've ever had to do watching my child go through that.  I was blindsided by all the stuff I didn't know and completely overwhelmed and unprepared for the stuff I thought I knew.  I've realized there is no way to ever prepare for something of that magnitude and until you go through it, you have no idea what it is going to be like.  It took me 45 minutes to work up the courage to see him after surgery.  I walked down that hallway many, many times and every single time, I would lose my nerve and turn around because I just couldn't face seeing him like that.  When I finally did see him and he was laying in the bed, a raw incision across his chest, blood draining out of him through tubes, hives all over his face from a blood product reaction and attached to a machine that was breathing for him, it literately dropped me to my knees and my first thought was "My God, what have we done??"  I didn't know if we had made the right decision and I had so much doubt and confusion.  I'm so glad that he was sedated because I was sobbing.  I never wanted him to see me scared or confused.  Sometimes, he would wake up from sedation and every single time his eyes would be rapidly searching the room and he would be panicked.  The anesthesia they gave him had an amnesiac effect and he would forget where he was or what had happened.  I had to constantly reassure him that I was there, the surgery was over, he did well and that I was SO incredibly proud of him.  One time, they came by with a floor polisher that was really loud and he got really agitated and scared.  I had to tell the nurses if they saw the floor polisher coming to close his door. I've never had an experience in my life where I wasn't completely in touch with myself and my emotions and I was just so completely lost through this process.   I cried for 3 days straight after he was transplanted and I can't even really tell you why.  I don't know if I was scared, thankful, relieved.  I don't know.  I just know those were the most powerful emotions I had ever felt in my life and I couldn't even define them.  I couldn't put a name to them.  I had no idea what they were.  I felt like I was on a runaway train barreling towards some unknown destination.  There were so many things I didn't know about transplant and I wish I had talked to more people that had been through it.  I wish I had done more research.  I wish I had known more.  I always saw transplant as black or white, it either goes well or it doesn't.  I didn't know there were so many shades of grey where things can go wrong.  One of Michael's doctors described it as a roller coaster ride through the dark because there are ups and downs and you don't know when they are coming.  I think that's a good analogy   When we were going through the transplant process everything was so vivid and so real, too real.  Now that we're home, it feels like a dream that you wake up from and you know it was very vivid but the details are blurry.   I know I sat in a waiting room for 20 minutes not knowing if he was going to live or die after he became unresponsive and that was my absolute lowest point.  I know at some point, I sat in the basement of the hospital and held his old lungs in my hands.  His old, hard, rubbery, rough lungs.  Those lungs I fought so damn hard to keep healthy.  That was such a bittersweet moment.  I felt relieved that they were out of his body, I felt sad that they needed to come out, I felt angry that they were trying to kill him and I felt like I had failed him in some way because maybe I didn't do enough.  I didn't know for sure whether we had made the right decision until I sat in that lab and had the pathologist tell me that "with lungs that sick and scarred, I would be surprised if he had made it past Christmas."  That's when my heart shattered.  I knew my little boy was sick and I knew he had less than a year but I didn't know it was so much.  I didn't know his time was SO close to running out.  I didn't know how close we really were to losing him.  So while I think the roller coaster ride through the dark is a good analogy, I would describe it more as a train ride. A train ride that has a dark, long tunnel.   When you first board the train, it's exhilarating as the view speeds by and you can tell if the train would slow down, the scenery would be beautiful.  Then the train slows down to go through a tunnel.  A long, dark tunnel.  You don't know how long it is or where it's taking you.  After some time, you get used to the view and darkness in the tunnel.  Then when the train comes out of that tunnel, the brightness stuns you and since the train has slowed, you can see the scenery and it's breathtaking.  Seeing my little boy not have to struggle for every breath, cough until he's blue or vomits, relying on oxygen and bipap, sitting on the couch and watching life pass him by is my new scenery and it is breathtaking.  And since I've been so busy fighting for his every breath with every one of mine, I had forgotten how important it is to just slow down, take in the view and enjoy the ride. 

The ugly truth

Michael had a partially collapsed lung, spent one night in the PICU and two weeks total in-patient.  This was after he spent almost the whole month of May in the hospital.  He has had increased exacerbations, lower pfts, lower sats, increased hospitalizations, has been needing more support from antibiotics and oxygen, had a pneumothorax and is struggling more to breathe.  The doctors told me that this is what happens when a person enters the end stage or terminal phase of the disease.  Michael needs to be activated on the transplant list.  We are awaiting word from his transplant team in Pittsburgh as to when they are going to list him and what his LAS (lung allocation score) is going to be.  His LAS will determine where he is on the list.  I'm assuming he will be pretty low on the list to begin with but may get bumped up as we see how this plays out.  Once a CF patient has a pneumothorax, it's possible it could keep happening.  If that happens with Michael, it will increase his LAS and move him up on the list.  It's also possible that it never happens again but either way, Michael is (as the doctors put it) desperately ill.  Each lung collapse is a life-threatening event.  So here we are.  A place we had hoped we would never be.  A place we had hoped the doctors were wrong about.  A place we naievly thought we'd be prepared for.  We can no longer fool ourselves into thinking that Michael will prove them wrong.  We can no longer pretend it won't or couldn't happen because it is.  We have had the rug pulled out from under us in an instant.  Our journey from here on is into the unknown and it is terrifying.  We are going to be faced with many, many tough decisions along the way.  Decisions no parent should ever have to make for their child.  I was SO scared about making those decisions because I don't want to make a wrong one.  You can only be wrong one time with this and it could be fatal.  But then I got to thinking and I believe if all our decisions are based on what's best for Michael and our love for him how can we make a wrong one?  So I am a little more at peace now.  Michael is ready.  He knows his body is failing him.  He is scared but excited to see what life looks like on the other side of transplant.  He's ready to be able to run, play, do karate, swim and just breathe.  He wants to experience life outside of a hospital room, without being strapped to an oxygen tank, without being hooked up to a feeding tube 20 hours a day.  He is looking forward and I should take my cue from him and look forward too.  It's going to be a hard road getting there but we will.  I HAVE to believe that because I just can't face the alternative.   I wasn't going to make this public until we heard from Pittsburgh and we (as a family) needed some time to process it but everyone has been so supportive offering up so many prayers and so many kind words that I felt that I needed to share.  You may share in my sorrow but please do not feel sorry for me.  I  love Michael beyond words and I feel blessed to be the one chosen to walk this path with him.  I would do it a million times over if I had to.  Please continue the prayers for my son's health, our strength and wisdom, and for successful outcomes. 

I am aware

May is national Cystic Fibrosis Awareness month and usually I would post something every day on Facebook but this time, I just don't want to.  I am so damn aware of it that I'm sick of it.  It almost feels like a celebration of all things CF and I am just not there right now.  How can I celebrate the monster that is slowly but surely stealing my child from me??  I know it's part of who he is and I should celebrate that part but I just can't.  The people who go on Facebook and get to read the daily little tidbits of information, hardly ever even hearing of the disease, are the lucky ones.  You get to slowly raise your awareness level and learn about the disease.  Not us.  We (all CF parents) get thrown into it.  Tossed like a rag doll.  We have to land on our feet and hit the ground running.  We have to learn quickly how to take care of our children.  We have to learn terminology like PICC, port, nebs, FEV1, G tubes, NG tubes, GJ tubes, Vests, Sputum, Postural drainage, Pancreatic insufficiency, etc. etc.  We have to make decisions for our children based on the little medical knowledge we've gained since diagnosis and it's hard to know we are doing the right thing.  Is a G tube better than a GJ tube or should we start with an NG and work our way to a G tube?  The list goes on and on.  I remember when Abby was a baby and I would creep into her room to make sure she was still breathing.  I do that now with Michael and it means something completely different because back then I didn't REALLY think she would stop breathing.  It was more of an excuse to see her in all her blissful innocence.  I can't say the same now.  I really DO check on him to see if he's still breathing.  So yes, I am CF aware.  I'm aware that my son needs a new set of lungs or he will die.  I'm aware of the moments I have where I think of what it truly means if he dies.  It means gone forever, no longer with us and it stops my heart, steals my breath and leaves me paralyzed with dread and fear.  I'm aware of the many, many tears I've cried for him and all my children.  I'm aware that I may have to bury my children and I'm aware that it shouldn't be that way.  I'm aware that it's impossible for my children to have a normal childhood because so much of it is interrupted by treatments, hospital stays, medications and illnesses. I'm aware that my daughter is scared as she watches her brother wasting away from the same disease she herself has. I'm aware that someday Aiden may be an "only child".  I'm aware that there is no break from this disease, no remission.  I'm aware that it can take a person quickly and without warning and you can't EVER let your guard down.  I'm aware that my heart aches so bad I think it's going to burst out of my chest when I hear of a parent that lost their child to this disease.  I'm aware that a cure is so close we can almost reach out and touch it but I'm also aware that it won't do Michael any good.  CF has ravaged his body and left his lungs too damaged to be cured.  I'm aware every single minute of every single day.  Oh yes, I am CF aware.  Are you??     

I wish...

Michael's birthday is coming up.  The birthday that by all accounts, could be his last.  I wish I could say I'm not angry and scared. I wish I could say that I am handling it well but I'm just not.  I wish I could say that I'm not taking out my anger, fear and frustration on the people that love me the most and just want to support me through this. I wish he didn't blow a 27% on his pfts yesterday and he wasn't laying in a hospital bed away from home tonight.  I wish he wasn't dropping his sats and needing oxygen support.  I wish none of this was happening and I could just wake up and have it all be a horrible nightmare.  I wish it didn't hit me so hard when he makes an innocent statement of what he wants to be when he grows up.  I wish I could take him in my arms, protect him and keep him safe.  I wish it was as simple as when he was little and a kiss could take away the boo boo and make things all better. I wish I didn't have to worry about what our life will be like once he gets a transplant.  I wish I didn't have to worry if I'm making the right decisions for him.  I wish I could stop time and his disease from advancing.  I wish I could trust God with all of this.  I'm trying.  I really, really am.  I wish no parent had to go through this, EVER.  I wish I could trade places and take it all away from him.  I wish there was a cure.  Most of all, I wish I didn't have to imagine what my world would be like without my little boy in it. SO many things, I wish...     

Transplant and Talks

We took Michael to Pittsburgh on March 14th for his follow up from his transplant evaluation and here is what they had to say...Michael's X-rays showed advancement of his disease and more scarring and damage than his last X-ray. He has advanced to the point where the Vertex medications will not help him. We have no hope now except for transplant. There is just too much damage already done. Michael's PFTs have dropped from 45% to 37% this past year. The only idea they had for bringing them back up was to put him on continuous low dose steroids but before they do that, they want him to have a DEXA (bone) scan to ensure that he doesn't have osteoporosis already. If he does,the steroids won't be an option because they can cause bone problems and stunt growth. Also, they are presenting his case to the whole transplant team on Tuesday and Dr. Spahr is going to consult infectious disease about keeping Michael on a long term antibiotic called Zyvox. We have already lost so many options with antibiotics because Michael's bacterias have grown resistant to most everything. If we use this and lose it as an option, it could be catastrophic when he does get a transplant. They gave the time frame for transplant at about a year but said that we are one major event away from having to list him. Michael is at high risk for pneumothorax (collapsed lung) and hemoptysis (coughing up blood) because of how damaged his lungs are. Should either of those things happen, he will be placed in the intensive care unit here and Pittsburgh will go ahead and list him as a priority one which means he would be at the top of the list. They also want Michael to have an EKG which will tell them how his pulmonary hypertension is. If that has advanced as well, it could push them into listing him sooner so his heart doesn't sustain more damage. He would also then be placed on oxygen around the clock. I will hear from the transplant coordinator after the team meets on Tuesday so she can inform me of the decisions they made. Basically, they are not ready to list him quite yet but we are steadily moving towards it.
I had a conversation with Michael about what happened in Pittsburgh and it went like this: "Michael, do you have any questions about Pittsburgh and what the doctors said?" "No, I don't have any questions but I've decided that I don't want the transplant." "What made you come to that decision?" "The doctor told me that recovery is a slow process and I just want to run and play like a normal kid." "Well Michael, you will get there someday it will just take some time. Do you understand what will happen if you don't get the transplant?" "Yeah, I'll be fine. I've been taking my meds." "It's great that you comply and take your meds but your lungs are very sick and at some point they will stop working. Do you understand what that means?" "Does that mean that I will die?" "Yes, honey, it does. Daddy and I have made this decision for you because we don't want to lose you and we want to give you the best possible chance but we aren't going to force you into anything. It's not happening any time soon so you have some time to think about it." Hardest conversation I've ever had to have in my life! I had to walk outside and compose myself because I don't want him to see how heart wrenching this is. What do you do when your 8 year old doesn't want a procedure that will save his life? Do you force him into it because he's a minor and under your care or do you respect his wishes and let him die? I just don't have the answers but I pray every day that we are saying the right things, doing the right things and making the right decisions.

Pfts and Legos

Michael is SO excited to go to a Lego event coming up soon.  I was hesitant to let him go because of the germ exposure but I talked with the doctor and she asked me if I would regret letting him go or regret not letting him go.  I would feel horrible if letting him go means that he ends up in the hospital but that question made the answer pretty clear so he is wearing a mask and going.  He will probably end up in the hospital soon anyway so I don't think keeping him away from something he loves is the right thing to do.  I talked with the people hosting the event and they did tell me that the Legos are sanitized after each session and that Michael being in a wheelchair and on oxygen wouldn't be a problem.   Michael has been doing as well as can be expected lately. His pfts are staying steady at 37% but we used to see anywhere from 40-50% in the past year and a half. The doctors informed me recently that they can't do anything more for him to bring his numbers up. 37% is his new baseline. They did tell me that "it's possible for him to float along like this for years" but I'm just scared that's not going to happen because of how much he's declined in the past year. I'm trying to balance optimism with realism and it's a very hard thing to do.  Also, he has been losing weight lately and has dropped from a BMI percentage of 78 down to 37 so we have some work to do with him.  He needs to be as healthy as possible for transplant and they like to see a BMI percentage at 50 or above.  He has been vomiting quite a bit lately and sometimes vomiting blood.  We were told the blood is from his sinuses and now have started doing sinus rinses on him.  It seems to be helping some.  We are traveling back to Pittsburgh soon to see what they have to say about the time frame for transplant.  We don't want to wait too long to list him since his blood type is common and that could make the wait for lungs longer but we don't want to do it too soon either.  The longer he can go with the lungs he has, the better.  Traveling has become a hardship for us since Michael is getting more and more dependent on machines but we are hoping to take a family vacation to Gatlinburg in April.  It is going to be quite a job packing everything up but we are determined to go have a good time and forget about transplant for awhile.  I get tired of having this dark cloud hanging over my head and not knowing what is going to happen.  It is exhausting and frustrating and I think I have gone through the full scale of emotions numerous times in the past year.  Despite everything, I'm thankful that God chose me and I continue to marvel at Michael's caring, giving ways, his fighting spirit, his wonderful sense of humor and how much joy and laughter he has brought into my life and I continue to feel so incredibly blessed that he calls me Mom.     

This is stability?

All 3 kids were admitted to the hospital on September 4th.  Matthew stayed for 2 weeks, Abby and Michael both stayed for almost 3.  They actually got discharged early since our insurance company refused to pay for any more days.  They claimed that the kids were "stable enough to be cared for at home".  So home they came.  Michael has been struggling since he got home.  He has been running low grade fevers more days than not and he is needing his oxygen all day long instead of just at night.  He has been coughing up and vomiting up gobs and gobs of mucous.  He has moments where he struggles to even finish a sentence because he can't catch his breath and he is struggling with going up and down the stairs.  He has been grumpy, emotional and just plain hard to please.  I don't know what all of this means and I'm scared.  The only thing I know for sure is that we are inching closer and closer to transplant.  When we went to Pittsburgh, the pulmonologist actually said if Michael continues on the path he's on (gaining good weight and blowing pfts in the 40s) he might never need a transplant because pharmaceutical companies are working on a correction of the CFTR defect for his mutation.  If he can hold on for four years, this pill could be a miracle drug for him.  The doctor said he is an optimist and that is the path he hopes Michael takes.  But it's not the path he is taking and I knew in my gut when he said that it was not reality.  I talked to his doctors here and they agree with me.  They are sticking to the 2 year prognosis.  I hope and pray that Michael proves them wrong but I don't want to be an ostrich with my head buried in the sand and not think about all the possibilities. I sincerely wish someone from the insurance company could come to my home and see how "stable" Michael is.  I hate how they make judgements without knowing what is truly going on.  Michael is sick.  Michael has been sick for a long time.  Michael is not going to get better.  He is only going to get worse and we have to watch it happen. I wish I could explain to them how heart wrenching it is to watch your child struggle for breath, cough until he vomits, have people stare at him when we are out and about because it's unusual for a child so young to be wearing oxygen, how I have to answer his questions about why people are staring, how when I tuck him in at night and lean in to kiss him, I breathe in his scent and try to memorize every detail of his face because I don't know how long we have. But I don't think I am capable of putting it into words that they would understand because they don't know and love Michael like I do. To them he is just a case number and an expensive one (I'm sure).  One of the good things that happened while Michael was in the hospital, he got his last hepatitis A and B shots.  He was supposed to go to the pediatrician for them on September 17th (That was when the last shots in the series were due) but after me going off on a handful of residents, it got done.  He was up to date on his shots but when the team in Pittsburgh did his blood work,. they discovered that he wasn't making antibodies to hepatitis A or B so they requested that he repeat the series.  That was the last thing on the list that they asked us to do so I will be calling the transplant team tomorrow to see where we go from here.  I will, of course, keep you posted.