Day +807

Update from Rita:

Hello Team James,

We apologize for the long delay in writing James' update, but unfortunately 2021 has not been too kind to James. We are in a better place now, but our family has spent the last month and a half in and out of the hospital/specialist appointments/clinic visits, etc.  During these weeks, James has suffered from severe GI issues, headaches, fevers, lack of sleep... the list goes on.  I won't mince words.  The last month has been agony.  But, in true James fashion, his amazing spirit continued to shine through and he has come out on the other side smiling.

So much has happened in the last weeks that it is hard to keep track of all the details, but I will do my best to break it down and not write a crazy long update.  As everyone knows, James' health situation is complicated and always evolving.  Currently, we have 2 main issues that we are tackling.  There are many other issues (alopecia, skin GvHD, elevated medication doses, stunted growth) that we are monitoring and employing a more "wait and see and slowly make changes"  approach.

ISSUE #1:  Build up of secretions/damage to the right lung

You might remember that during his last hospital stay in December, James had a CT of his chest which revealed a build up of something (doctors still are not sure what) in the lower right lobe.  (We confirmed with the team that it is the right lobe, not the left as we had indicated in the last update).  The doctors were concerned that this build up could lead to infection, so the plan was to closely monitor any symptoms, including fever.  

Shortly after the last update from 1/4/21,  James developed unexplained fevers (101-103.3) and wasn't feeling great.  On 1/12/21, we were admitted to the hospital so that James could undergo a bronchoscopy to make sure there was no active infection in the right lung.  We were able to get the bronch done on 1/14/21, after three long days of sitting in the hospital with an irate and very bored 4 year old.  During the stay, the team ran many tests on James' blood and stool and ruled out all sorts of infections and viruses, including COVID.   By the time of discharge on 1/14, James had started to feel a little better.  By the weekend, James was feeling great and was even able to go sledding with his cousins. ​

The bronchoscopy results came back with no sign of active infection.  This was good news, but that still left the question of what is going on in in his lung per the very obvious signs of abnormalities on his CT scan?  Our lung doctor couldn't tell us for certain, but the suspicion is that James' lung sustained some lasting damage from the month and half he was on a ventilator when he was first diagnosed back in 2018.  She suspects the damage to the lung makes it difficult for James to fully clear mucus and secretions (something those of us with healthy lungs do all day long, without even realizing we are doing it).  The concern is that the secretions will start to pile up in the lung and put James at risk for serious lung infections.  It's a bit disheartening to see "new" damage show up and evolve in his lungs... and we're a bit fearful of the fact that these issues are popping up 2 1/2 years since his time on the ventilator.  We do wonder what it means in terms of any further evolution of lung disease...  but no one knows if this is it or if it will worsen.

So, what do we do now that we know the right lower lobe isn't functioning as well as it should?  The lung specialist recommended that James use a percussive vest and albuterol therapy targeted at the damaged part of the lung to proactively help the lung clear secretions.  Due to complications that I will get into later in the update, we weren't able to obtain the vest/albuterol treatments until this week, but so far, James has been such a trooper about the respiratory treatments. We try to make the process a little more enjoyable for him and James picks a show to watch while the vest or his dad rapidly pounds or vibrates his chest.  While all of this is happening, he also has to wear a nebulizer mask over his mouth. The goal is to work up to James wearing the vest and nebulizer for 30 minutes, two times per day... which the doctors know is a very tall order for anyone, especially a 4 year old.  James is allowing about 20 minutes at a time so far, and we are thrilled with how well he is handling it.  Of course we will do anything from James but adding another hour of treatment each day on top of all his current needs is going to be a challenge for all of us.

The plan for James' lung treatment is to monitor his lung function over the next 6 months while he continues his daily vest/albuterol treatments.  Hopefully, we won't have any episodes of coughing, trouble breathing, etc.  At about the 6 month mark, James will have another CT of his chest, and we will go from there.

Below is a video of the vest he will need to wear during these treatments.  For context, this is on the LOW setting!

ISSUE 2:  What  is going on inside James' GI tract?

After discharge from the hospital on 1/14, James had a fantastic 5 day span.  He was medically cleared to return to school and had a blast playing in the snow at Grandma Marilyn's.  He was full of energy, eating and playful.  On Wednesday, 1/20, James developed another fever.  He really began to feel crummy that night, and the next day we were back at clinic with fever, headache, and tummy complaints.  James' blood was drawn, he was given IV antibiotics and fluids, and we were sent home.  Almost minutes after leaving, we received a call that James had tested positive for COVID.  As you can imagine we were extremely surprised.  James always wears a mask out in public, at school and at the hospital- we had no idea how he could have contracted COVID.  We were also extremely worried.  We were worried not only about how COVID would affect James, a medically complicated kid with a suppressed immune system, but also terrified of how it might affect anyone James had come in direct contact with when he would have been most contagious (in the two days before he began showing symptoms, per his doctors).  James actually still had an IV in his arm when he left the hospital as we were supposed to return the next day again for more fluids as well.  Dan removed if from his arm when we got home and James as always was a champ about this.

The entire family immediately went into quarantine mode.  We monitored James closely over the weekend, and he seemed to be having only a "mild" case of COVID, with bouts of coughing and fever here and there.  But on the whole, he seemed to be improving and his fevers dissipated.  Unfortunately, by Wednesday, 1/27, James' diarrhea really began to accelerate. The poor kid was going (and this is not an exaggeration) 20-30 times per day.  It was every hour, then every half hour, then every 15 minutes.  Anyone who has ever had food poisoning or a really bad stomach virus knows how miserable this is for an adult... imagine a 4 year old boy spending hours in the bathroom every day.  The challenge here is that this went on for weeks, not a day... hard to imagine.

For some reason, James' body kept sending him signals that he had to go.  We had countless episodes of sitting on the potty for 15 minutes, getting up and getting tucked back into bed, only for James to be convinced he had to go again minutes later.   It was an awful, never ending cycle of him not only feeling terrible, but also getting very little sleep day after day after day.  We were concerned about his nutrition as well as his body's ability to keep up with all of this day after day.  James' body was telling him he had to go, causing him to strain so hard trying to empty his system.  This eventually caused his rectum to begin prolapsing again.  Dan and I had to explain to James, in a way that he could understand, that he couldn't push too hard because sometimes pushing too hard would cause part of his body to slip out a bit.  It seems too crazy to be true, but James now knows the word "rectum" and how it is a part of your body that you want to keep inside.

On top of the hours being spent on the potty, we were also noticing more and more blood in James' stool- a terrifying sight for any parent.  At first it was a few times a day - and then eventually it was every time he went to the bathroom.  We brought James back to clinic in desperation mode.  We were willing to do anything we could to help him.  The team again tested his blood and stool for anything that could explain the terrible symptoms, but no answers.  In terms of treatment options, we finally got the OK to give him Imodium, but other than that, all we could really do was make sure James did not get dehydrated and wait to be seen by GI.

When the problem still had not resolved by the following week (now 14 full days of this nightmare), we were a bit in panic mode.  We were also all completely exhausted by lack of sleep and trying to take care of 2 young kids, one with horrible stomach issues, all while trying to work and keep ourselves sane.  On top of that, we were all isolating due to the COVID diagnosis.  On 2/9/21, we met with a GI doctor who recommended that James start an antibiotic called Flagyl to help with  any possible infection in the GI system and/or help with inflammation.   The doctor explained the inflammation could be caused by GvHD (although this is still not high on the list of potential causes) or some sort of inflammatory bowel disease like ulcerative colitis.  The typical treatment for inflammation in the gut is steroids, but  James is already on a triple dose of hydrocortisone and we wanted to do everything in our power to keep him from switching back to prednisone (the higher potency steroid he was on for 18 months).   

​As a side note - in addition to this James' skin GVHD was also really acting up from time to time, creating one more stressor for him to deal with.

After 3 days of Flagyl without too much of an improvement, we went back to an inflammatory bowel disease specialist to discuss next steps.  The specialist admitted that he is puzzled by James' pattern.  James had these exact same symptoms back in December and the issues seemingly resolved on their own after about a week.  Now, we were back with the same issues, possibly caused by COVID, but possibly not.  The symptoms were the same this time around, but had lasted 19 days at that point.  

It is and continues to be a mystery.  Because the specialist can't be sure what this GI issue is, he cannot know how best to treat James.  He recommended that James undergo yet another endoscopy/biopsy/sigmoidoscopy (camera down the esophagus and into the GI system) to help confirm the diagnosis.  This was scheduled for 2/18.  The specialist did note that Flagyl could take a few days to work, but if James was not improved by Monday, 2/15, we would move forward with the test.

Thankfully, James finally (finally!!!) got some relief over this past weekend.  He started going less (more like 10 times per day) , not sitting on the potty for so long, and not having to return 2-3 times in one 30 minute span.  His appetite and energy returned, and we got our happy little guy back in full force.  Things continued to improve to the point where our GI specialist agreed that we could postpone the endoscopy until James has to go under anesthesia for his 6 month lung CT or if the GI issues return.  

While we are so relieved that James is doing better, there is  a pit in my stomach about this GI issue.  Why does it keep coming back? What is this and what is causing it? How do we prevent this from ever happening again?  Is this GI issue contributing to the lack of growth?  Right now, the doctors can only guess. The suspicion is that James has some sort of GI/gut inflammation, possibly caused by one or several of his medications, possibly caused by GvHD, possibly something else or a combination of some or all of the above.   The other hypothesis is that it could be ulcerative colitis (not good) which would be yet another complication/medical issue added to the list that James would potentially have to deal with for the rest of his life.  That said, we/the doctors simply do not know what the issue is at this point and we're hoping for more details in the coming months as we're able to see how James reacts to these new treatments and (hopefully0 avoids any further episodes.

Moving forward, we will start to wean James off the Flagyl and back down off his hydrocortisone.  We are anxious to get him on lower doses of these meds as both seem to cause fluid retention and James' belly is very distended.  After what seems like 6 months of setbacks, we are really hoping we can get James back on the road to recovery, and focus on weaning medications and stimulating growth.  

The older James gets, the more I worry that his early childhood memories will be overwhelmed with doctors, hospitals, needle sticks, breathing machines, appointments and waiting rooms.... more appointments... more waiting rooms.  Because of this, we really try to live in the moment with James and immerse him in loving, happy and "normal" early childhood experiences.    James himself is largely responsible for allowing us to live in and cherish each moment with him, as his fun loving and cheerful personality brings us such joy.  When I hear the sound of him playing and making up voices for his Scooby Doo characters (his latest toy obsession) or singing along to music (he loves to sing the Fireman Sam theme song to his baby sister), it fills me up with joy because I know he is truly feeling good.  And that's really what we want for him, to feel good, GREAT even- the way every young child should when they wake up each morning.  

For now, I am so very grateful that we have gotten about one week of those "I Feel Good!" mornings and I cherish every single one.  Hoping the trend continues in this direction.

If you made it to the end of this very long update (apologies!), thank you for reading and thank you so very much for loving and supporting our family.  

Thanks!

Rita