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.   

I do but I don't, I will but I won't

We had an amazing trip to the beach!!  Michael and Abby (due to the generosity of some very kind people) got to take surf lessons while we were down there.  The salt air is so good for them because it replaces some of the salt they are lacking.  In fact, one of the medicines they are on was discovered by surfers in Australia.  It is a 7% salt solution called hypertonic saline and being at the beach is just like getting a treatment of it.  One of Michael's dreams was to learn how to surf so it was wonderful to see it come true.  I can't even explain how I felt seeing him on that surfboard.  I admit it, I cried.  When we were done with surfing at Wrightsville Beach, we went back to my parent's home at a different beach and were just in time to see some baby loggerhead turtles being born!  It was moving to see them start out on their journey.  While we were standing there watching them be swept into the ocean, Michael took my hand, looked up at me and said "This is a day in my life I won't ever forget."  I'm sure I won't EVER forget it either.  When you are living day to day and moment by moment, it means so much to be able to give your children a memorable day.

I can't even describe how difficult this journey has been for us.  I personally struggle a great deal with Michael's prognosis.  This is the most contradictory set of emotions I have ever had.  I want to move forward to know what is going to happen and yet I want time to stand still.  I want to know how much time we have and yet I don't want to know. I know in my heart that I should just enjoy the time we have with him but I have this endless loop of questions and "what ifs" that run through my mind.  I am strong one day and overwhelmed, paralyzed with fear the next. It feels like we are just spinning our wheels and it is maddening.  I want to move forward and I don't because moving forward means transplant.  It means putting Michael through a huge, risky procedure.  It means trading this life for a completely new one.  It means not knowing what is on the other side.  It means no cure will help him.  It means a long, drawn out hospital stay away from home.  It means a risk of lymphoma.  It means switching to hospice and changing his full code status to a DNR if lungs don't come in time.  It means losing my child and I can't face that. Yet it's something I face every day.  Most parents don't question it to hear their child talk about what they want to be when they grow up because they just assume that their child WILL grow up.  Michael talks about what he wants to be when he grows up and I don't care what he wants to be, I just want him to have the opportunity TO grow up.  Some days I feel strong and some days I don't.  Maybe tomorrow I will feel stronger and maybe I won't.

Hurry up and wait...

Michael continues to do well.  He has been running intermittent fevers lately but they seem to go away on their own.  I am finding this the hardest time for me.  After his prognosis, there was always something to get done, something to get through and now all I have to do is think and think and then think some more.  After the doctors told us two years was what he has left, we went to Pittsburgh for the transplant evaluation and I was busy planning that, then he was in and out of the hospital, then we went back to Pittsburgh for a follow up, then he was in the hospital again.  I didn't have time to sit and process this all.  It has hit me like a ton of bricks lately and I am finding myself unable to sleep some nights because my mind won't shut off.  There are so many scenarios and I try to picture them all so I feel somewhat prepared but the many variables make it hard.  Sometimes I feel paralyzed with fear because I don't know what is going to happen with Michael.  It's like being told you are going on a trip.  You pack your bags, head to the airport, and are told your flight has been delayed AND rerouted.  So now you have no idea where you are going, when you are leaving,  and what time you will get there.  The only thing you know for sure is that you are going on a trip.  You have no idea what the journey will be like.  You have no idea what your destination will be like.  You don't know if you will be happy in the place you end up.  Will this trip be the best decision you ever made or the biggest regret? 

We did it!!

I am happy to report, we have reached so many milestones this past few months.  Michael turned 8 in May and I was so happy to see it!   When he was so sick with his liver and his heart, I was scared he wouldn't make it to 8 but here we are!  The only thing he wanted for his birthday was a green butterfly net.  Simple, right?  I looked ALL over town for months for one and finally found one at Toys R Us.  They had JUST gotten a shipment in and only had one green one left by the time I got there.  His face lit up when he unwrapped it and that moment will stay with me always.  I'm so glad I could make him so happy! 
Michael just got home from a 3 week admission.  It was a planned admit but the day he was to be admitted, his sats dropped, his heart rate increased. he needed his oxygen and he spiked a fever.  It turned out to be viral but I'm glad he was going to be admitted anyway.  It always scares me when he gets that sick that fast.  The admission went well..  They switched his tube feed formula from one that has 1.5 calories per ounce to one that has 2 calories per ounce and he put on 4 pounds.  I weighed him when he came home so I could get a baseline on our scale and he weighed 49.4 pounds. It has been my goal for the past year and a half to get him to 50 pounds (those of you with CF kids know how difficult this can be).  I even made a deal with him:  If he started eating well and doing his tube feeds (he sometimes unhooks himself so he can play), once he passed the 50 pound mark, I would pay him $15.  Well, I weighed him tonight before bed and he now weighs 51 pounds!!  I am going to (gladly) pay him his $15 tomorrow or we may just go to Walmart and buy him the Beyblade thing he's been wanting.  Abby is currently in the hospital for a tune up and we are hoping to get her out on the third of July even though her 21 days aren't up until the fourth.  They are not going to gain or lose anything with 24 hours so we are fighting to get her home on the third so she can be with us to celebrate on the fourth.  That's all there is to report.  Things are going well here.  I hope and pray that they stay that way!