Wednesday, June 27, 2012

No Answers

Jack saw the orthopedist today and had an x-ray of his spine.  According to the ortho,  Jack can't have a compression fracture because his spine is fused.  I'm guessing he knows what he is talking about, but I wish he would have spent more than 2 seconds looking at the x-ray to say from his perspective, he can't find the source of Jack's pain.  The hardware in Jack's back all looks good and, apparently, the x-ray didn't show any obvious breaks or anything else that might explain Jack's pain.  I trust this doctor, but I guess not enough that I'm won't seek a second opinion.  I intend to get a copy of the x-ray on disk and send it to our St. Louis ortho for his opinion.

I was able to arrange the CT scan today so that we went right from the ortho appointment to the radiology appointment for the CT (both places in the same building).  I called the urologist's office after we got home and was told that Jack's urologist is out all week. Grrrr!  I asked if anyone else in the office could pull up the CT and read it.  I was told that they haven't received the report yet and that they will call once they receive the report.  I then informed the person at the other end of the phone that Dr. Z (Jack's urologist) doesn't wait for the report, he sits down at his computer and pulls up the actual film and looks at it!  To which I was told that "our process is to wait for the report and then call".  To which I wanted to respond .... you are wrong because Dr. Z does NOT wait for the report to call me.  Ugh!  Sometimes it's just not worth the argument.  So, my plan is to call tomorrow and tell them to fax me the report and I'll read it!  At least I'll know if something is going on before next week when Jack's urologist will be back and can call me.


I have a real fear that the CT scan will show nothing and then we are sitting here not knowing what is causing Jack to be in pain.  Then what?  I guess we discuss how to manage Jack's pain because if we can't find the source, we certainly aren't going to ignore the pain.

Time will tell .... in the meantime, we continue to bag Jack through his periods of distress and I'm stocking up on the wine.

Monday, June 25, 2012

From What I Can Remember ... an Update of Sorts

In my last post I said I had a lot to update on and that I'd get an update to you this week. Now, I don't remember everything I had in mind as far as an update.  I need to start making myself notes! Well, actually, I already make myself notes, email myself reminders from home to work, from work to home, and have yellow stickies all over my desk. I guess I now need to start writing down my blog ideas as they come to mind, or else, "poof" they are gone.

Anyway, here is what I remember that I can update on:


Jack is still experiencing pain and it's hard to know what exactly the problem is.  Jack was pretty miserable most of our vacation - which was not fun for him or for us.  We know that he has residual stone in his kidney based on the x-ray following the lithotripsy.   The day after we got back from vacation, we took Jack down for another KUB (x-ray of kidney, ureter and bladder).  The urologist called me to let me know that there is "fading" of the remaining stones compared to the last x-ray. When I told him that Jack still has periods of pain, he said the only way to really tell if there were stones blocking something was to get a CT scan.  I asked him to please order one - and I'm now waiting to hear from scheduling at PCH.  

The other potential issue is GI.  I never did give you the follow-up on the c-diff.  Both cultures came back negative for c-diff, as did the culture for any type of bacteria.  The issue pretty much resolved on its own, but he's still not 100% back to his normal self as far as his GI tract and it makes no sense when you consider that he has been getting the same food for well over 10 years -- nothing has changed in his diet that would account for a change in his GI tract.  It's a mystery.  I decided to keep the appointment I could get with the GI doc in August.  If nothing else, I want to meet the guy and have him meet Jack so that we are no longer "new patients" who have to wait forever and a day to get an appointment.  Oh, and before Jack's issues resolved themselves, my pediatrician wanted to admit Jack to the hospital for a GI work-up because we couldn't get in to see a GI before August.  Needless to say, I wasn't too keen on the idea.  He said to think about it over the weekend and let him know on Monday.  Fortunately, things improved over the weekend (this was several weekends ago) and the urgency of the situation was greatly diminished.  Thank God, because I don't think I could have dealt with another hospital admission just to sit around and wait for tests to be ordered.

Another theory for Jack's pain as suggested by my doctor friend, Anne (the founder of Cure CMD and an expert in congenital muscular dystrophies) is that Jack might be suffering from compression fractures in his spine.  She said she has seen this with another CMD child who is about the same age as Jack.  In thinking about it, it could be a real possibility given the fragility of his bones and all the moving around he has been through the last several months.  I called Jack's ortho today to get an appointment and was told the earliest they could get me in was July 11th at a different office ... to which I told the woman, "I am not waiting until July 11th and I'm not going to a different office, let me talk to Patty."  (Patty is our ortho's right-hand-woman.)  I talked to Patty and she said "do you want to come in today or Wednesday?"  It sure helps to know who to go to when you need to get something done!  Anyway, I'm taking Jack in on Wednesday for x-rays.  I'm anxious to see what they show.  

Jack has had a very rough 2012 and it just breaks my heart that he is hurting so much. It's written all over his face, is reflected in his breathing and his sats.  He's requiring bagging almost every day and I relate his drop in sats to pain.  I sure hope that not only can we figure out the source of the pain, but also find a way to resolve it once and for all. 

TouchStones of Compassionate CareTM

In other news, the CHOC-Touchstones program was invited to present at the 2012 Patient and Family Centered Care Conference in Long Beach, California which was held last Friday.  We joined "nationally recognized faculty providing presentations related to Best Practice in delivering care that is truly Patient and Family Centered."  Pretty cool, huh?  It was a great experience - notwithstanding my complete fear of public speaking.  Everyone who attended our presentation seemed very interested in bringing the TouchStones program to their hospital.  I'm hopeful that we will be invited to share information about the program with other medical institutions.  Who knew my "Dear Future Physician" letter would take me where it has.  I owe a great deal to my friend, Sarah, who I met on the message board many years ago.  Sarah was moved by my letter and she is the one who pushed to make the program a reality at CHOC.  Sarah is on the Parent Advisory Committee at CHOC and she worked on people there for well over a year before someone would listen.  She is awesome - as is Dr. Katz at CHOC.  In order to get the program off the ground anywhere, we need what I call our "physician champion".  It was two of Jack's former doctors who supported me and helped me to get the message in my letter out there  - they were my "physician champions".  It was Dr. Katz at CHOC who went to bat for us to help us get CHOC on board. It's definitely a team effort and every team needs a physician champion to make it happen.

It's exciting stuff!

I don't know if I mentioned on my blog (I know I did on Facebook) that I've registered the "TouchStones of Compassionate Care" program (which includes the name, the stones, my letter and my video) with the United States Trademark office and once my application makes its way through the process, it will be a registered trademark under the category of "educational services".  More coolness! :)


Well, that's about all I've got for now.  I'll leave you with some pictures of Eric from one of his swim meets this summer. He is doing really well - especially considering a year ago he wouldn't even get in the swimming pool he was so afraid. 


I'll update after Wednesday and let you know what the x-ray showed.


Edited to add:  One more thing I wanted to share, Part II of my essay, "Management of a Ventilator Dependent Child - A Caregivers Perspective" was published on the Cure CMD website.  Here is the link - it's the "personal perspective".

Part II - The Personal Perspective

Tuesday, June 19, 2012

I've Been Remiss

I am so sorry for the lag in blog posts.  We just returned from a week long family vacation in the San Diego area. It was a family reunion of sorts - it was me, my sisters, brothers and our kids and my dad who all met up in California for the week.  I do have lots to blog about, but I'm too tired tonight to think.  I will share some of my favorite photos from our trip.  All my photos were taken with my iPhone, so they aren't the best, but they are pretty darn good for phone photos.  I've got a busy week/weekend coming up, but I promise a more substantial update next week.

We stayed at Camp Pendleton - Del Mar Beach.  Because access is restricted to military families - the beach wasn't super crowded, which was great.

The "big" cousins helping the "little" cousins build a sand castle

Kite flying

Jack was not a big fan of the beach, but we made him go out a couple of times.  The beach wheelchair does not work for Jack because he has no tone and can't sit in the chair.  My nephews, brothers and brothers-in-law were great in helping carry Jack (in his wheelchair) out onto the beach.  It took four guys to carry him out.

Eric got good and dirty and had the time of his life!

Some in the group went to Sea World for the day 

Eric and his cousins at Sea World

Seaport Village in San Diego (one of the few days Jack was happy)

Mr. "GQ" 

Sunset on the last day of our vacation - awesome!

Thanks for checking in.  More later ... I promise!

Wednesday, June 06, 2012

The Kidney Stone Saga Continues ....

We were back down to Phoenix Children's today for another procedure under anesthesia to have the stent removed.  The urologist said that the KUB (x-ray of the kidney, ureters & bladder) showed that there are still some small pieces of stone remaining in the kidney AND as a result of causing a hole in the kidney (either from when the neph tube was place or during the surgery to break up and remove the stone) pieces of stone leaked out and now there are pieces of stone in Jack's peritoneal cavity.  Based on an article I read: "Extravasation of irrigation fluid during percutaneous nephrostolithotomy is a major complication that can result in severe morbidity."  Fortunately, as of now - Jack seems to be okay with these pieces of stone floating around his insides.  The urologist said they shouldn't cause him problems - or pain. He also said he doesn't even know how he'd remove them, as he's never encountered this before.  The urologist also doesn't think that the small pieces of stone remaining in the kidney will cause him pain.  I guess that remains to be seen.  It's times like this that Jack's inability to communicate and express how he feels makes it really, really difficult - for him ... and for me.  

The plan is to have a repeat KUB in 6 weeks and if there are still pieces of stone in the kidney, the urologist will do another lithotripsy to try and break them up more.  

As for the composition of the stone, it was determined to be "carbonate apetite".  I got on PubMed to see what I could find out about stones with this composition and found a recent article that discussed the composition of kidney stones in patients with musculoskeletal anomalies - which includes individuals with muscular dystrophy.  I could only get the abstract on PubMed, so I emailed one of the authors and he sent me a copy of the complete article.  On a side note - I sent an email on Saturday night, received a response from the doctor who co-authored the article on Sunday morning and first thing Monday morning - I had a copy of the full article along with another article the doctor thought might be relevant and of interest.  How cool is that?  Just another example of a doctor who cares!  

Based on the article, kidney stones in individuals like Jack - individuals who are unable to move -  were once thought to be caused by infection, but now they are finding that many are of metabolic etiology - possibly due to high urinary pH.  I was initially told that Jack's type of kidney stone was likely caused by infection. However, after removing the stone, the urologist said that it didn't look like it was due to infection. So, Jack's is likely a metabolic stone. They are now testing his urine and we should know more in a few days.  Problem is, according to the urologist, the treatments they have used to lower urinary pH have serious side effects. 

There you have it - more than you ever wanted to know about my kid's kidney stones! :)

And in the meantime  - we will continue to do what we do best .........

Keep calm and carry on! 

(okay, the keeping calm part might be a bit of a stretch.)