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!

Leap of faith

As many of you know, we went to Pittsburgh to have Michael evaluated for a double lung transplant. It was a lot of information and emotions to process. I feel I am now ready to give the details of the evaluation so here goes; First and foremost, the doctors at Pittsburgh do not feel that Michael is sick enough to need a transplant right now but they do agree with our doctors here that it won't be long. The thing that stuck out in my mind with that information is that we will have to watch him get sicker before we can do anything for him and as a mother, that thought breaks my heart. I am glad that we have the option of a transplant but I do not want to watch my child get sicker and struggle even more. There were so many things that we (or maybe just me) were naive about. I knew that transplant is a HUGE risk and is by no means a cure but I had no idea how many pros and cons we would have to weigh. Here are some interesting things we learned: If Michael gets a transplant and a cure for CF comes later, it won't do him any good. Basically, once lungs are transplanted, they will start to degrade at some point. It's just not knowing when. Lungs are complicated because they have access to the outside world. So once he goes through the transplant we have essentially started a ticking time bomb and won't know when it will go off. That is why we are not listing him now. The longer a person can go with the lungs they were born with, the better. His chances are 50/50 seven years post transplant. We know he has a 0% chance if we do nothing so that 50% is looking pretty good. Also, we found out that there is a 10% chance that he could develop Lymphoma due to the T cells being supressed for transplant. That is a type of cancer that responds well to chemo but I had no idea that was a risk. We also asked the best case/worst case scenario question and were told that the best case scenario is that he does well and lives another 10-20 years. The worst case scenario is that he goes into instant rejection and doesn't make it out of the hospital. The good news is there is nothing stopping him from getting a transplant. He doesn't have B. Cepacia or any other bacterias that will keep a transplant from happening, his heart tests came back looking good and all the other test results were well within what would be normal for a person needing transplant. We also found out that Michael would be in-patient for about 2 weeks post transplant (assuming all goes well) and we would have to stay in Pittsburgh for 4 weeks following that so he can be followed closely and checked for rejection. At the 6 week mark, they would take a tissue sample from the new lungs via bronchoscopy and check for rejection. If there are no problems, we could then bring him home. They would follow up with taking tissue samples at 6 months, 9 months, 12 months, 18 months and 24 months post transplant. Pittsburgh did give us a few recommendations of things we could change around for Michael and he seems to be responding well to the changes. He is holding steady with Pfts around 40%, he is no longer receiving TPN due to the damage it was causing his liver and the risk of sepsis from having an open line but we are now having to do tube feeds around the clock. Michael is tethered to a pole for 20 hours a day so he can get his daily recommended 3000 calories. He does eat but there is just no way he can consume enough calories for him to gain weight when his body is burning them working so hard just to breathe. When we got home, Mike and I had a discussion about whether we wanted to go forward with transplant when the need arises and luckily, we both agreed that we want to pursue it. We both feel that we need to give Michael every possible chance. I'm so glad that we were in agreement. It made things at least a little easier. I think the scariest part is not knowing what is going to happen. Obviously, no one can see into the future and tell us exactly what Michael's outcome is going to be but sometimes you just have to take a leap of faith and believe that things will work out as they are meant to be.