Sunday, August 24, 2014

Overdue update and signing off

Another unfulfilled promise to update this little blog.  At least no posts means no necessarily bad news to report.  Keegan has been stable over the summer, and we've done our best to keep him that way.  There isn't a lot going on with him medically that is concrete enough that I felt I needed to record it here, which is good and not entirely good.  There have been milestones and hiccups, but he has stayed home through it all.  Hang onto your hat; it's gonna be a long one.  Here's the scoop:
  • Keegan is OFF steroids!  Yep, you read that correctly.  It was the longest, slowest, two-steps-forward-one-step-back steroid wean in history, but it finally paid off.  Keegan has been off steroids successfully for an entire month with no flares or major side effects.  He hasn't had any viral exposure to test this theory really in the last month, but so far, so good.
  • Without steroids, Keegan's intestinal failure has worsened.  This was expected but still difficult to see.  The intestinal failure is caused by his immune system attacking his GI tract as part of his underlying (and yet still unidentified) global autoimmune disorder.  We knew that the anakinra wasn't helping this one aspect of the disease, but it works for keeping the big, life-threatening flares away.  The intestinal failure is manageable with TPN and IV supplementation, so it's not as much of a priority.  We haven't jumped the gun to add more TPN on yet, but it will happen soon. 
  • Since the steroids came off, his anemia and neutropenia have made a slight comeback.  It's too early to say if this is related to the steroids.  It's not actionable yet, just something to watch.
  • For the first time, the chronically hypertensive child who has been on no less than three blood pressure medications his entire life has been fighting with fluctuating blood pressures.  It happened once at the beginning of the summer, but he quickly went back to his "normal" high numbers.  Then two weeks ago, his blood pressure tanked again.  Instead of running to the ER, we started his TPN immediately at a higher rate to get some fluid on him and held his blood pressure medications.  He seemed stable enough to stay home, and a quick visit to the transplant clinic didn't show anything immediately concerning.  So for now, he's still off the medications.  He is making a much slower return to his higher numbers, and I anticipate we will be back on his blood pressure medications before long.  This could be a result of the steroids being off, or it could be Keegan keeping us on our toes.  Another thing we will have to wait and watch.
  • We are waiting on a bone density scan in the coming weeks to make sure there is no permanent bone damage from the years of steroids.  Keegan has not grown more than 2cm in the last several years, which we attributed to the steroids also.  If we don't see more growth in the next 4 to 6 months, we will see an endocrinologist to determine if we need to help Keegan's body along.
  • Still no results from Keegan's genetic mapping back yet.  His rheumatologist was meeting with the researchers last week, and we are hopeful that the results will be done soon.
All in all, Keegan has been stable at home and enjoying summer.  We've had a few fun family outings around town and have been swimming once about every other week.  Although the medical team wouldn't endorse swimming with a central line, we made the decision to allow Keegan to do so after careful research.  We only swim in my parents' pool; we cover his line as best as possible and do a dressing change immediately after he gets out.  I wouldn't say that we can call it completely safe after the limited amount of swimming we have done, but (knock on wood) he's done well with it so far.  A little bit of normalcy where we can get it.

The last major point to update is Keegan's neurological status.  At the end of the school year, Keegan's teacher and ABA therapist both independently approached us about his apparent inability to make any gains in more abstract areas of thought, anything he couldn't memorize.  This was a red flag we had been told to look out for by neurology.  With this information and the end of the steroids, we met with neurology to determine the next step.  We ultimately decided not to do another MRI at this time.  He is not having any more strokes or seizure activity, so new films would only give us another baseline to determine the rate of aging/decline/gliosis/whatever-we're-calling-it.  It wouldn't change our treatment of him at this time.  Instead, Keegan's being referred to a new neurologist specializing in this area.  

I guess this is a good time to reiterate that Keegan's medical history, disease history, strokes, and medications have left him with a brain that has "dried up" and was two years ago, roughly similar to that of a 65 year old.  The worst damage is not in an area of the brain where Alzheimer's usually occurs, but rather, we must watch for signs of early onset dementia in a child who never reached normal development in the first place.  If you can imagine learning the subtle signs of dementia and stroke activity in a normal 65 year old, imagine the difficulty in determining these signs in a child who never spoke or acted "normally" in his 6 years.  This new neurologist will hopefully be able to help us distinguish between existing damage and the signs of dementia that his teachers and therapists (and family) may not initially see.  We believe she will be able to help us determine when it's time to start various neurological/dementia medications that may help Keegan cope over time.  I won't even try to explain how difficult the idea of watching your child go through dementia before he's a teenager is to swallow.  However, the good thing is that we are surrounded by doctors, friends, therapists, and family that are committed to helping him (and us) through it.

In contrast to essentially every post over the last year, I will not wrap this up by promising any new posts in the immediate future or apologizing for a lack of posts.  It has taken me about a year to admit that I don't want to update the blog anymore.  Gray and I have made a commitment to actively pursue quality in Keegan's life, as there is no guarantee of it's quantity.  It may seem counterintuitive, but Keegan's stability has made us much more acutely aware of how limited our time with him may be - whether it is chronological time or neurological time.  That means that we are actively committing to living a more authentic, unplugged life with our children.  I want my kids to know that I am living each day with them and for them, not to have something to post on the blog or Twitter or Facebook.  Additionally, the changes in Keegan's health and overall condition have been more subtle over the past year, making it more difficult to put into writing.  

So where does that leave us?  We'll still be here.  There probably won't be much over there on the Twitter feed.  In fact, that will likely go away soon.  K's for Keegan is still alive and well.  The next race will be the Turkey Trot in Frisco on Thanksgiving Day, which I will relay more information about soon.  Every one of my own races is a K's for Keegan race, but my race season has taken a hiatus this summer as I dealt with some personal health issues.  This blog was never about me in the first place though.  When the posts do come from here on out, they won't be about anything but Keegan anymore.  

To that end, there won't be many posts here, but I'm not shutting it down.  If there's something important to note because Keegan's struggling or he's inpatient (or oh-my-word, he's at Disney World on his second attempt at a Make-A-Wish trip!), I will come back.  In between that, the day-to-day pictures and life updates will stop.  No news is not bad news.  We are so grateful for the continued support, love, and prayers that everyone has given Keegan and our family over the years, that I couldn't possibly retreat into our shell for good.  I am a firm believer in the power of prayer and intercession.  I would never take that blessing out of Keegan's life voluntarily.  It's just time for our family to be our family.  To disconnect for awhile and make the memories that we will need one day when our family isn't four anymore.  No one can tell us how long that can be, so we're going to make the most of it.  I wish we were stepping away because things were just so peachy keen we didn't need to update anymore.  Maybe I'll regret stepping away 10 years from now when things are exactly the same as they are now, but for now, this is where we, as a family, need to be.  

So, thank you again.  And please don't give up on us.  We'll be back soon.  

5 comments:

Greg said...

Thanks for your post - I was curious what was happening.

I completely understand your perspective on posts.

We have different reasons for coming to a similar conclusion.

Our child has a rare chronic medical condition, and we've chosen not to publicize it, at all. We don't talk about it on any social media, and we tend not to publicly participate in charitable drives (races, etc.), although we do support them financially.

Our child will have to deal with this condition for the rest of their life, and we want to leave it up to them as to if and how they want to publicize it when they get older.

In the meantime, we've tried to make life as close to normal as humanly possible, and we don't think that posting updates on social media is compatible with that (although others with children with the same condition would disagree).

We've never met, but for some reason, I've become vested in hoping that you and your family persevere through the many challenges you face. As a selfish result, I look forward to your updates.

That said, however, because I do hope that you and your family persevere, I think you're making the right choice in limiting your time here.

Time is fleeting for every one of us - we should all enjoy each moment we can.

Greg

Ben and Abby said...

Love you all, Maddie. We continue to support, love, and care for your family. Even though we don't see each other as often as we used to, your family is still in our thoughts, prayers, and memories. We have so many pictures of the kids as 'babies' that we cherish. :) Such cuties... :)
xoxo.

Lauren said...

As always, I will keep your family in my thoughts!

Lauren Wood

Cody said...

Thank you so much for sharing and know that we will continue to support your family with caring thoughts & prayers.

We have so appreciated all of your updates, photos and the education that you have provided as you began your life journey with Keegan. It allowed us to be aware of Keegan's status as his life was so very touch and go for so long.

Along the way we have also been able to get a glimpse at Miss A. What a beautiful family you have.

So very happy that you have been able to experience some stability with Bug's health, and be able to enjoy your family.

After reading this entry, I totally understand why it took you so very long to put it in writing, and why you are choosing to sign off for now.

Maddie - we support your decision, and will remain your caring family friends from Washington State.

With love & prayer - Cody, Gary & Griff

Tari Anne Roth said...

Maddie...with my year anniversary coming up working with Southwest Transplant Alliance...I want you to know I think of Keegan A LOT. He, and any child or person that needs an organ transplant to live, is what keeps me going. I find it so strange the things that link us together (although we've never met). My cousin, Matt, who went to Jesuit with Gray, our love of running, our faith in God, and organ transplantation. You, your family, and Keegan are inspirations to me and I pray and think of you all often. I hope that I can do the Turkey Trot with K's for Keegan!!!