Happy New Birthday, Jaden!!!

It's been over a month since I last posted. It's amazing how life just gets so hectic that there really is no time to take even a few minutes to put my thoughts down. As I started this post, I first wanted to count the number of days since transplant. However, this being the ONE YEAR mark from Jaden's stem cell transplant date, I think we have officially graduated to months or years post transplant, not just days.

Yes, today (okay, technically YESTERDAY July 9, since I'm blogging in the wee hours of Saturday morning) marks the ONE YEAR anniversary of Jaden's transplant. I try and think back to what we were doing, what I was feeling a year ago today. So much of it is a blur at this point, but other moments are so vivid it's as if they just happened yesterday. I recall the day itself--the anxiety, the elation, the prayers, the tears of joy, the tears of fear and dread. Who knew at that point what the coming days or weeks or months would hold? I remember it being so anti-climactic. This is it? A 30 minute cord blood transfusion? This IS transplant?

It was the culmination of years, and I do mean YEARS of planning, searching, hoping. Is transplant the right thing to do? Where is the best place to do it? How do we put Jaden in a position to garner the best possible outcome? I had heard that sibling matches were best. My unending research had shown me that Children's Texas or Duke (or even Cincinnati Children's) were the best places to be for CGD. I had read blog after blog, spoken to families going through transplant, consulted the experts in his disease, seen the best, and read about the worst outcomes. I knew I had to do something. I couldn't imagine Jaden going through life waiting for the next infection or being limited by what he could do or where he could go. When the doctors diagnosed him at 23 months, I remember hearing the words "it used to be a death sentence, but today we have kids with CGD that are living to be 40! They are having families and leading normal lives...". Forty??? Are you kidding me? Of course, this was the best case and didn't take into account the daily medications, the injections, the possibility of infections, hospitalizations, doctors' visits, etc. I couldn't see Jaden doing this. What kind of life would this be for a child...or a young adult...or a middle aged man for that matter?

These were the thoughts that led me to find a solution. This is what lead me ultimately to Duke. I remember the wonder and amazement of the 5200 team when Jaden seemed to sail through chemo and the transplant itself, almost unscathed. I remember hearing Nurse Laura say "Jaden, you're making this look too easy!". I remember the complete disbelief when the team talked about discharge at little over one month post transplant. I remember taking everything off the walls, being ultra-efficient by sending everything back to the apartment in order to minimize what we took out on discharge day. I was up until the wee hours making sure everything was packed up and ready. The camera was loaded and ready for Jaden's big Confetti Parade.

Then it happened...the first signs of the vile and nasty beast that is transplant. His first side effect: Polyoma. I remember the bewilderment of seeing the sight of blood as he urinated. I remember the shrieks that came with the pain. Then the worst news...discharge would be postponed until the polyoma was under control. I remember seeing the look on Jaden's face when I had to tell him we were not going to the apartment just yet. We just need to get through this, Jaden. We'll be back at the apartment in a few days...or so we thought.

The days turned to weeks and the weeks to months. Polyoma was just the first of the side effects. It seemed to open the flood gates for what was to come. Jaden had everything imaginable. The worst of which were his breathing issues--the need for oxygen, first only at night, and then nearly twenty-four seven. I remember the struggle to get him to put the nasal cannula on. I remember the tears, the frustration, the anger. Just when we thought things couldn't get worse, the flank pain began. It was so bad, it brought Jaden to his knees...screaming with unbearable pain. I felt helpless, not knowing what was happening. Even the doctors seemed perplexed for a while. At one point, not finding anything conclusive, they even dared to suggest that it was psychosomatic. But if you had heard the screams, seen the pain in his face, the sweat on his brow, you would know...this is real. This is not just in his mind. Luckily, the cause was identified and the problem rectified over the course of weeks (or was it months?). As I said, the details are somewhat of a blur.

Jaden did have his Parade. He did leave the unit after a record Day +98 in the hospital (which doesn't include the 2 weeks or so pre-transplant he was in-patient). At this point, he could barely walk without pain and fatigue. I tried to encourage his every move, his every step. I remember the beginning of physical therapy, when each step on the stairs was done with such determination. I remember the exhaustion and frustration Jaden felt when he couldn't walk, much less run, down the hallway without extreme fatigue.

I look at my baby boy now and see such hope, such grit, such patience. I use the word "warrior" frequently when talking about him. It surmises all he's been through and all he has yet to go through. Even at one year post transplant, we are not out of the woods yet.

The transplant has done a number on his lungs. Jaden had a full pulmonary evaluation a few weeks ago, including a treadmill exercise test. He needed to go 6 minutes on the treadmill at optimal heart rate. I was so upset because these 6 minutes seemed like an eternity for him. He screamed, he cried, he begged, he pleaded with me "have mercy!" to stop the test. I had to dig deep yet again and be positive, encouraging, uplifting. I had to remind him of the end result. Dr. Hardy needed this information to get an assessment of his current state and to formulate a treatment plan for him.

I sat down with Dr. Hardy a week later and she told me that although the results were normal in many areas, there were two areas of concern: Jaden's small airways were obstructed (72% flow rate) and he had significant air trappings (37% where the norm is 20%-23%), meaning he could not fully expel all the air in his lungs. She felt that this was a result of the chemo and the transplant so no bronchodialators (ie albuterol) or steroids (ie pulmicort) would make things better. With one fell swoop, she stopped ALL his breathing treatments. I was so excited! This would free up so much of our day. No more washing breathing masks! No more running home for breathing treatments!

As I probed further about what this meant for Jaden and what the long-term implications were, I was once again blind-sided. Dr. Hardy told me that this was the best Jaden's lungs would be. Although there was some hope that his lungs would grow over the next year (Jaden is only 7 and the lungs are fully developed by age 8 or so) and potentially mitigate some of the issues, the reality of the situation is that Jaden may just have to live with the limitations. He wouldn't be running any marathons. "Choose activities that require short bursts of energy," Dr. Hardy had said. Jaden will never be a great distance runner. He will need to rest periodically and catch his breath. Altitudes will cause issues for him since the air is thinner and he will have a harder time breathing as it is. I couldn't believe what I was hearing. I needed to absorb it, soak it in, mull it over. I immediately started to pray. Pray that his lungs grow. Pray that he overcomes this hurdle and can be a normal boy, jumping and playing, running and chasing.

I was dealing with the harsh reality presented to me by Jaden's pulmonologist when I started to notice a cough. First I thought it was just a little cold. It came with a some congestion and a little runny nose. Paranoid parent that I am, I brought it up immediately with Nurse Sherrie in clinic. She listened to his chest and told me all seemed fine. She said we'd watch it and ensure things didn't get worse. Are you sure? Then the words slipped out before I could reign them in "If we were at DUKE, we would have....". Oh boy, Dr. Prasad had made it very clear to me that Dr. Walters wanted to be in charge once we were in Oakland. I could not second guess his decisions or revert back to Duke. I had vowed not to mention Duke if Dr. Walters, who is a colleague and close personal friend of Dr. Prasad's, would accept Jaden as a patient. Now I'd done it! I couldn't help it. I knew if we were at Duke, Dr. Prasad would have run at least an RSV to check for a virus if nothing else. I felt helpless...

Well, I don't know about worse, but the cough was definitely persistent. It was a wet, nasty cough that seemed like it should bring a good amount of phlegm with it. However, Jaden never spit anything out and when he coughed, it seemed to clear. I continued to bring it up in clinic. A chest x-ray was ordered but showed no major areas of concern.

The cough persisted and at the last visit, Nurse Lizzie said she heard some crackling in the right lower lobe. I wasn't concerned at first because "crackling" seemed to be Jaden's baseline post-transplant. Had it gone away and now returned? Nurse Lizzie had no basis for comparison since she had not heard him before. We waited again...

Last week when I took Jaden for his clinic visit, Nurse Lizzie listened again. The crackling was progressing and a chest CT was ordered. She said the team had talked about Jaden in their morning meeting and Dr. Hardy's report coupled with the sounds in his chest suggested Jaden may have bronchiolitis obliterans, or GVH of the lungs. Graft vs. Host of the lungs? At this stage of the game? The words stung my ears. My heart sank... I didn't know what to think. I told myself not to jump to conclusions. Let's just get the scan done and see what happens.

Well, the next day, I got the call from Nurse Lizzie. The chest CT in fact confirmed their suspicion. Jaden did have GVH of the lungs. I was stunned. I didn't know what to say or what to think. I had so many questions, and Nurse Lizzie patiently answered each one. What did this mean for Jaden? Would this improve? What was the prognosis?

Nurse Lizzie explained that essentially this was Gia's donor cells recognizing Jaden's body (mainly his lung area) as foreign and attacking them. GVH can be in any area of the body--skin, gut, etc. In Jaden's case, it was the lungs. This brought to mind our pre-transplant consult with Dr. Kurtzberg and Dr. Prasad. It was very clearly stated that in Jaden's case, if there were to be any issues, they would in the lungs or in his gut--the two main areas in which Jaden had prior infections. Well, they were spot on. The bulk of his problems have been and continue to be with his lungs.

The net net of it is that Gia's cells may, in a few years, adapt to their new environment and Jaden would not really see any effects of the GVH. The other end would mean Jaden would be on breathing treatments long term and "wouldn't be able to play any competitive sports". WHAAT???? Not play sports? Jaden LIVES for sports. He watches ESPN religiously. He played soccer, basketball, baseball. He knows every player on every team, where they were drafted, their stats, their vitals...Jaden IS sports. This was a huge blow.

In the short term, Lizzie said that Dr. Walters was going to be conservative. She described Jaden's case as "mild". The CT was concerning, but they had seen much worse. Dr. Walters wanted Jaden back on Albuterol twice a day. They were also adding Symbicort (similar to Pulmicort) twice daily as well as azithromycin (antibiotic). If this didn't work, the next step was to put Jaden back on steroids.

Steroids??? Say it isn't so! It took forever to get Jaden OFF of steroids! This can't be happening!!! I had to tell myself to take a step back. This is yet another lesson in patience for me to learn. This is God's way, yet again, of telling me I have no control of the situation (control-freak that I am). All I can do at this point is pray and hope for the best. The rest is Jaden's destiny and in God's hands.

Coming back to the annual review...it seems like we take one step forward and two steps back. Jaden has made such tremendous progress in other areas. He's running and playing. He looks so much healthier in the nearly 3 short months since we left Durham. He is looking like himself again--although there is a little bald spot on the top of his head where Jaden actually pulled his hair out. Reason unknown.

My biggest challenge has been keeping him isolated for the most part. He so desperately wants to go places and do things. I have become a little more lenient--allowing a play date with his special friend (even in their home!), taking him to an afternoon matinee (with mask on and no food or drink allowed at the theater), and letting him play with the siblings at Devin's baseball games. It's outside and it's not a "crowd". It's just a few boys throwing the baseball around.

Meanwhile, Jaden is becoming more and more belligerent when it comes to following rules, especially as it pertains to wearing his "duck" mask. I caught him with it around his neck, instead of over his mouth and nose, as he played catch with some kids in the park. He had moved away from my immediate line of sight and cleverly put it back on as he walked towards me, thinking he had pulled one over on me. I had to remind him that I have eyes in the back of my head and quickly called him out.

It's tough being the gate keeper and the enforcer. I'm not Jaden's favorite person. As a matter of fact he's made it quite clear, I'm not the one he wants to be around much of the time. When I protest, he calmly points out that he's "bored" with me. He's with me "all the time". Jaden's looking to find new company and new surroundings. He jumps at the chance to leave the confines of our house--the same house he begged to come home to after 10 long months in Durham.

The hardest has been his inability to leave the area, specifically to go to Los Angeles to see his beloved cousins. Per Dr. Walters, we are not to leave the area while Jaden is on immunosuppressants. The plan was originally to keep him on these medications until September and then begin the wean. However, with the latest lung issues, I'm told we will not jump to wean and it will be much slower than anticipated. Realistically, Jaden will once again miss another year of school and be taught at home by a district-assigned teacher. I have not even mentioned this to Jaden for fear of completely shattering his hopes.

Jaden speaks frequently of 2nd grade. He wants to know which teacher he will have, which friends will be in his class, etc. He wants to GO to school, play at recess, run with friends, interact, engage...In short he wants his OLD life back. He continuously asks me if he'll EVER be able to swim again or if he'll EVER be able to go to LA again...it just breaks my heart.

I have explained to him the circumstances surrounding the two medications that are keeping him from doing things and he seems to understand. However, at the tender age of 7 (even though Jaden is mature beyond his years), it's difficult to grasp and digest it all. I remind him of how far he's come and yet he dwells on all that he is not able to do. I tell him that "soon" he will be able to resume his "normal" life again. To him the "soon" is not soon enough. It seems like an eternity.

I can't blame him. Poor thing has been in isolation for a year. He's missed out on friends, school, family events and gatherings. He seems at his breaking point, but I have to keep him going. I have to show him the future that we have all worked so hard to create for him. The future that is free of daily medications, shots, doctor's visits, hospital admissions....

I keep coming back to the positives. This past year has shown us what determination and strength is. It has shown us the importance of family and close friends who stepped up, traveled across the country (sometimes at a moment's notice), stayed in our home and took care of our kids. It's the grandparents, aunts, and cousins that bonded together and got us through the toughest year of our lives. It's the dear friends that sent care packages to Jaden and uplifting words to us that showed us that it truly does take a village to raise a child. We have all endured so much, grown so much, and learned so much...but we've done it together.

I want to acknowledge each and every one of you, without who's support we would not be at this pivotal point in our journey with Jaden. Although I know there are many bumps in the road ahead and I'm not even sure if this road will ever be smooth, I know that if we got through the past year, we can get through just about anything.

Next week Jaden and I leave for Durham once again. We will be back in clinic at Duke. Back to familiar surroundings. Back to the comfort and safety of our new home away from home. I'm excited to show the bmt team how far Jaden has come. I'm anxious to hear what Dr. Prasad thinks of the latest findings and what he projects for the coming year. Most of all, I'm amazed that we will have gone full circle--back to where it all began.

Stay with me. I can't wait to see what the next phase of this journey holds. I can't wait to share it all with you...