Thursday, March 28, 2013

Jack, TouchStones, Road Trip and Birthday


Word spreads fast. I met with the social worker from the palliative care program today and she said that Jack's pediatrician heard that the kidney stone was back and thought Jack was likely headed for a hospital admission soon.  The social worker wanted to know if a hospital admission was something I wanted or did I want to switch him over to hospice. Whoa! I shared with her that (1) hearing the word "hospice" makes me physically sick to my stomach; and (2) I don't understand why I would switch to hospice at this point. Apparently, what I envision hospice to mean is much different than what hospice in fact means.  I don't yet grasp the advantages of transferring to hospice (which can be a temporary transfer and not necessarily a permanent placement).  In any event, I told her Jack is not symptomatic, he's happy and feeling well and I'm not ready to make any changes.  Of course, she was fine with that and is not pressuring me to make any changes - she's just making me aware of what my options are.  She also told me she'd stick to only using the word "palliative care" for my benefit. :)

As far as monitoring the kidney stone, Jack's urologist wants another CT scan in August unless Jack starts having problems with pain or otherwise.  In the meantime, we take our cues from Jack and just keep on keeping on.

To respond to Jenn's comment on my last post - Jack has only been on Amoxicillin prophylactically for UTIs and no other meds that would contribute to stone formation.


The first year residents at Phoenix Children's are being introduced to the TouchStones program through Ryan House (the residents are required to participate in an educational program provided by staff at Ryan House).  TouchStones was shared with the first group of residents earlier this month and the feedback I received is that my Dear Future Physician letter moved them to tears (that surprised me) and that they appreciated the information provided in the letter.  I don't think there is any question that most physicians inherently know that showing kindness, compassion and care towards their patients is a necessary part of the practice of medicine.  However, what the residents from PCH shared is that while they know the importance of showing compassion, they don't always know HOW to show compassion.  My letter offered some insight into how a physician can show kindness, compassion and care towards patients and their families.

My letter offers insight from my perspective and is only one example of how a physician should treat his or her patients.  I encourage all of you other parents out there to share your insight and feedback with the physicians who care for your children.  After all, doctors can't read our minds. I'm a big proponent of writing letters and giving feedback to the doctors I've encountered over the years.  I can honestly say that 99% of the letters I've written have been letters of thanks for the care provided to Jack.  I think it's important to let doctors know that we appreciate them and share with them those things they do that stand out as exceptional to us.  Contrary to popular belief, doctors are human just like the rest of us and they appreciate hearing that they are doing a good job.  Conversely, I think it's important to provide feedback - in a respectful and constructive way, when a doctor acts in a less than kind, compassionate and caring way.  We all receive feedback in our jobs - both good and bad and doctors should be subject to the same feedback.  I certainly hear from my clients when I don't meet their expectations and, just recently, my assistant suggested that perhaps I should think about touchstones for attorneys. (I'm not sure if that was directed at me or not!)

A perfect example of how sharing a letter can make a difference involves a good friend of mine.  She has an adult son with disabilities and it has been nothing short of a nightmare as she is transitioning his care from pediatric to adult physicians.  Her son was having problems with his shunt and after several surgeries and many visits to the ED, the neurosurgeon told her that there was nothing more he could do for her son and that he'd just have to learn to live with the pain he was experiencing.  Ultimately, my friend found a different neurosurgeon who operated again and discovered that there was, in fact, an issue with the new shunt that had been placed by the other neurosurgeon.  I pushed my friend to write to the prior neurosurgeon and let him know what happened because it's important for him to know that he was wrong in this case.  My friend eventually did send a very well written letter and shortly after she sent the letter, she received a response from the neurosurgeon.  He told her he appreciated her letter and that he would use the information she shared and the experience with her son as a learning experience.  Kuddos to that doctor for acknowledging that he has room for improvement.  If the letter had not been written, the doctor would never have known.

My point being, doctors need our feedback, especially our young doctors who are still gaining experience in not only the science of medicine, but the art of medicine as well.

Go forth and write those letters!

Road Trip

I spent last weekend in Southern California visiting with my friend Anne.  My friend Jenny, from St. Louis, flew out to Phoenix and joined me on the road trip to California. The original plan (made back in January) was for me to go out to California to keep Anne company while she was on bed rest due to a high risk pregnancy.  But, things have been going better than expected and bed rest hasn't been required.  It was fun to get away and spend time with Anne, her husband Joe, daughter Maia and Jenny. The fact that Anne lives near the ocean was an added bonus.

I know it seems like I've been out of town every other weekend lately.  The fact is, I need time away.  The cumulative effect of caring for a medically fragile child for fourteen years coupled with the intensity of last year has drained my reserves.  I'm easily depleted and restoring my reserves is essential to my sanity.  Call me a wimp, but I understand the importance of respite and I take every opportunity I can to make it a priority in my life.  I'm fortunate to have nurses who I trust and who will work extra hours when I'm out of town and a husband who never begrudges my time away.  Not that I need to justify my time away, but I do get the raised eyebrow from some when I skip out of town by myself.  I know Jack appreciates the fact that I take time away because I'm a much more present and happy mom when I get back.

Respite for everyone!

Anyway ....

The ocean view from the second floor balcony of Anne's house

Jenny, Anne (and baby) and me

nothing like the serenity of the ocean to ease your worries

(photo courtesy of Jenny)


And last, but far from least ...

Mary celebrated her 20th birthday last week.  If Mary had her way, she'd have skipped 20 and jumped right to 21.  Ah, the naivety of youth - always wanting to speed up the clock.  If they only knew.

Okay, it's almost 1am - time for me to end this and catch a few hours of sleep.  Until next time ... peace my friends.

Wednesday, March 20, 2013

The Consensus of the Ologists

The nephrologist called me Monday morning.  She told me she had talked with Jack's urologist and he suggested trying to treat the kidney stone non-surgically by giving Jack medication that would increase the acidity of his urine and potentially dissolve the stone.  The problem with this approach is that the drug they use to increase the acidity of the urine causes problems with the lungs.  Not the best side effect for a kid on a vent with diminished lung function to begin with.  But, the nephrologist said she'd talk with Jack's pulmonologist and get his thoughts and then call me back.  She said she was concerned that the pulmonologist would think she'd lost her mind to even consider it.  My concern was whether the stone would dissolve into a fine substance that could easily be passed or would it just break off into smaller pieces that would have to pass.  If it broke apart into smaller pieces, then the pain of passing the pieces of stone would be sheer hell for Jack.

The nephrologist called me back this morning and said that Jack's pulmonologist wasn't completely opposed to putting Jack on the medication, that he said he could follow Jack closely and increase his vent settings to compensate for any breathing issues.  But, the pulmonologist did want the other ologists to be sure that if we went this route, that the likelihood of successfully dissolving the stone was high.  The nephrologist said that the drug they were considering was typically prescribed to prevent kidney stones, not dissolve them and that the urologist thought it might work, but really couldn't be sure. (I give the guy credit for thinking outside the box.) The final consensus of all the ologists is that it's just not worth the risk.

So we are back to trying to determine (1) how to handle the newly formed 4.7 cm kidney stone from hell; and (2) how to prevent the stone(s) from forming in the first place.  I should add that I received the radiologist's report from the CT scan in the mail today and in addition to "extensive calcification in the upper right renal collecting system", there is "tiny new nonobstructing calyceal stone in the upper pole of the left kidney."  So, now we are also dealing with kidney stone formation in the left kidney - which, for some reason, the urologist failed to mention to me (and is the very reason I request copies of all Jack's reports).  In any event, the nephrologist said that one of the meds she put Jack on is doing the opposite of what it should be doing.  So, she's taking him off the one potassium med and putting him on another one.

When I asked the nephrologist if she had any ideas as to why Jack is having so many problems with kidney stones and why the medication that should be working to prevent them is not, her response was "you mean, other than the fact that he is a very sick and complicated kid?".

I think that's code for "I don't have a clue."  

This is where perspective comes into play.  At this moment in time, I don't see Jack as a "sick kid".  I'll give her the "complicated kid" label - although I think how complicated a patient is to a doctor is largely a factor of that doctor's experience (or lack thereof).  From my perspective, for the majority of last year, Jack was a sick kid. Today, he's not in the hospital, he's not in pain and his numbers (sats and HR) are stable.  Does she see him as a sick kid solely because he's on a ventilator?  Or because he's on a ventilator and he's complicated?  The quandary is, in being offended when a doctor who doesn't know my kid that well views him as a very sick kid, I'm contradicting my own position that Jack is now sick enough to transition him to palliative care.  Obviously, I'm still struggling with accepting the decisions I've made.  There is nothing about this journey that has been easy, but decisions regarding Jack's care at this juncture are brutal.  They are as difficult or even more difficult than the decisions that had to be made fourteen years ago.

And this is where I'm incredibly fortunate to have the friendship, support and guidance of several of Jack's St. Louis doctors (current and former).  These doctors have been with us from the very beginning - they know me, they are invested in Jack and they truly care about both of us. As I try to reconcile taking a palliative care approach with how to manage the new/recurrent kidney stones, the insight and concern of these doctors has been invaluable.  I can't say enough how blessed Jack and I are to have such amazing people on our team.

At this point, the nephrologist has done her part (from her perspective) by changing up Jack's meds.  She is deferring to the urologist for any further treatment.  I left a message with the urologist to talk about what the plan is for monitoring the stones outside of waiting for Jack to let us know (by way of pain) that something must be done immediately.  I don't think there is any doubt that the large stone has to be removed at some point. The question is when. The honest answer is, I don't want Jack to have surgery at all, ever.  But, I'm not sure it can be avoided indefinitely.  I also don't want to find ourselves in an emergent situation again and then have to wait weeks to get the surgery on the books because of all the scheduling issues at PCH.  And the bigger issue is, if this stone is eventually removed - how do we keep it from coming back again?  The "your kid is a very sick and complicated kid" response isn't really the answer I'm looking for.  I have to wonder whether I'd get the same response from the nephrologist in St. Louis.  Or maybe my kid really is complicated and there is no discoverable source or treatable condition.  It certainly wouldn't be the first time I've heard that when it comes to Jack.

For now, I guess we wait, watch and take our cues from Jack.  The last week and a half Jack has been doing great and I'm just happy to have a happy Jack.  I'm trying to enjoy the moment and not look too far into the future.  Easier said than done, but I'm trying my best.

I imagine this blog post is way more information than the average blog reader cares to know.  But, I'm a detailed kind of person and that's how I write.  So, if you are still with me - thanks for checking in and thanks for caring.

Friday, March 15, 2013

Not Happy

I left a message first thing Thursday morning for Jack's nephrologist to call me.  I received a call from her nurse letting me know the nephrologist was out of the office, but she would let her know I needed to talk with her.  The nurse first asked if I needed to make a follow-up appointment and I told her that I did not intend on schlepping Jack down to Phoenix Children's for an appointment when the doctor didn't need to see him to answer my questions.  When I didn't receive a call by noon today, I called again and again reiterated that I needed to talk with the doctor.  I received a call from a different nurse in the office wanting to know if I just needed to know the results of the CT scan.  The answer to that question would be "No".  Why these people feel the need to screen my reasons for wanting to talk with the doctor is beyond me, it doesn't matter - my son is the patient, I am the parent and she is the doctor!  In any event, I again explained why I needed to talk with the doctor.  The nurse said he'd pass the message on to the doctor, who WAS in the office today.  It's now after 7pm and I didn't get a return phone call.

I'm not happy - and that is using my nice words to explain how I really feel.

You know, the things I wrote in my "Dear Future Physician" letter back in 2008 have not really been an issue for me personally for many years because I've been fortunate to avoid having to deal with doctors for the most part until last year.  But, as I say in my letter, when I call a doctor and ask for him or her to return my call, I really need him or her to return my call.  And not a week later! There is no excuse for Jack's nephrologist not calling me back today.  Jack's urologist has always returned my calls the same day, even when I'm told he is out of the office.  He usually calls me after 5pm when he is in his car, but he calls me.  It's really okay for a doctor to make a phone call after 5pm. I can hear some of my doctor friends grumbling right now! But really, my friends, put on your parent hat (especially those of you with medically complex kids) and I know (hope) you understand where I'm coming from.

It's Friday evening and I really need to set aside my anger because I don't want it to consume me over the weekend.

'Nuff' said!

It's St. Patrick's Day weekend and there's no time like the present to start celebrating!

I'll leave you with a few of my favorite St. Patrick's Day quotes:

Some may say the glass is half empty,
Some may say the glass is half full,
But the Irish will forever say
"Are you gonna drink that?"


As you slide down the bannister of life, 

may the splinters never point in the wrong direction!

Slainte´ my friends!

Wednesday, March 13, 2013

Well, F

I'll refrain from inscribing the word that first came to my mind when the urologist called to give me the results of Jack's CT scan today.

Yes, the thing that showed up on the ultrasound is, in fact, the return of the kidney stone from hell.  It's at least 4cm in size or 80% of the size of the stone when it was discovered a year ago.  The urologist said he's never seen a stone grow back so fast.  The thing acts like it's a tumor.

If you recall, back in November when we got the results of Jack's 24-hour urine analysis, the results showed a couple of things in Jack's urine that were concerning. The most significant finding being a very high oxalate.  At the time, I decided not to pursue further testing because Jack had been through so much already and he was just starting to feel better.  Based on the return of the stone, I think we are going to have to pursue further testing.

I did do some limited research on high oxalate, also known as hyperoxaluria.  There are several potential causes for hyperoxaluria. One is diet, which we know is not the case with Jack (he doesn't eat leafy vegetables).  There is also a rare genetic condition where an enzyme in the liver is defective which causes an increased amount of oxalate to be produced.  I'd be inclined to say that it is very unlikely that Jack would be born with two congenital conditions - Muscular Dystrophy and Primary Hyperoxaluria.  But, the unlikely has been known to happen when it comes to Jack.

Where we go from here is that I hope to talk with the nephrologist tomorrow.  The urologist said he was calling her today and I figured I give her a day before I called.  I'm just hoping I don't get any push back from the office when I call and tell them I want to talk to her today.  I will not wait days to talk with her and I don't see any reason to haul Jack into the office when she doesn't need to see him to discuss what is going on.  I'm guessing she will order the blood work she had in mind last November.  High oxalate can also show up in the blood - which is bad news because it can affect the heart. In fact, Primary Hyperoxaluria can affect heart function.  I'm not ready to make any connection regarding Jack's decreased heart function given that he also suffers from muscular dystrophy, but my interest is peaked.

As far as what we do about the stone at this point in time - we wait and watch.  Jack is much better than he was last week - he's off oxygen and feeling good.  I don't think his most recent episodes of distress were related to the stone.  Until he starts showing signs that the stone is causing him relentless pain, we will monitor the stone every 3 months with CT scans and also check for UTIs on a regular basis.  Neither we nor the urologist are anxious to subject Jack to another surgery.  The only way to get the stone out will be another percutaneous nephrolithotomy.  The stone is too big to break up via the non-invasive lithotripsy.  I cannot even contemplate another major surgery for Jack right now.  In fact, it makes me sick to my stomach just thinking about it.

I will probably contact both the St. Louis urologist and nephrologist to get their thoughts on this.  I don't know either one of them very well and they don't know Jack well, so it's not the same as dealing with Jack's regular St. Louis docs.  But, based on my past experience, they are both very receptive to helping Jack.

On a happier note, we had a great visit with Peggy last week.  We wish she could have spent more time with us, but we'll take what we can get.  I know most of you have heard the story of how Peg came into our lives, but for those who don't know, you can read about our dear friend Peggy HERE

Happy to see each other

Enjoying the beautiful AZ weather

In birthday news, Eric celebrated his 11th birthday on Tuesday - from breakfast to dinner, the day was his day!  The added bonus is that he's always on Spring Break for his birthday and that makes it an even better birthday from his perspective. 

Birthday muffin for breakfast

playing his new game

dinner celebration at the restaurant of his choice 

That's the update from here.  I'm exhausted just writing it all out.  Living it is just about killing me.

Wednesday, March 06, 2013

Say It Isn't So

Jack had his kidney ultrasound on Tuesday.  You know, the one that was supposed to show that the added meds and extra water are keeping the kidney stone from hell at bay?  Well, maybe not.  When the ultrasound tech said she needed to check with the radiologist to make sure he didn't need additional images and she was gone for well over 20 minutes, I knew something was up.  When she returned and took images from a different angle, I could easily see that something was taking up about 1/2 the space in his kidney.

Jack's urologist called me this afternoon and said he compared the latest ultrasound to the one taken just about this same time last year when the stone was first discovered and they don't look the same.  He's not convinced that what the radiologist is calling a stone is in fact a stone.  They measured it at 4cm already and the urologist said he can't imagine it coming back that fast.  I'd like to believe him.  I want to believe him.  The only way to know for sure is for Jack to have yet another CT scan of his kidney.  I think this will be about the tenth CT scan in a year's time.

Just to be clear, palliative care doesn't mean we won't take action when issues arise for Jack.  It means our primary focus is on Jack's comfort and not the resolution of every issue that arises because of the progression of Jack's disease.  However, doing nothing when it comes to kidney stones is not a comfort measure.  That being said, we know that if, in fact, there is another large kidney stone, we won't agree to having the same procedure done that was done twice last year because it didn't resolve the issue the first time around. But, we'll cross that bridge if we get there.

Jack has had a rough week.  He's still on oxygen and has needed a lot more bagging than he's required the last few months, but not as much as he needed when he was at his worst.  He was miserable at Phoenix Children's yesterday and required bagging pretty much the entire time.  Not fun at all.  We've been giving him morphine almost every day to get him through periods of pain.  It's hard to know the cause of his pain/distress.  The message I got from Jack's nurse earlier this week was "your son's lungs sounds like crap".  He's improved as the week has progressed, but it's possible he has pneumonia. Or is the distress kidney stone or UTI related? (UA results are still pending.)  Or maybe a combination of all of the above?  It's so incredibly frustrating having a non-verbal, medically complex child who is hurting.

Speaking of "bagging" Jack, a commenter on my last post asked what I mean when I say we are bagging Jack.  Note: all my trach parent friends, you can skip this part as none of this is information you don't already know. :)

When we bag Jack, it means we are manually ventilating him with a hand-held ambu bag.  Here is a picture of the ambu-bag/oxygen set up next to Jack's bed.

Bagging someone is similar to giving them mouth-to-mouth resuscitation, except we squeeze a bag to give him air and we are giving him air through his trach and not his mouth. (And, Jack isn't coding at the time we are bagging him. However, if we didn't bag him through his episodes of respiratory distress, he would likely, eventually code.)

Ambu bag connected to Jack's trach

Bagging Jack gives him more forceful and more frequent breaths than he gets via his regular vent settings.  His oxygen sats are usually in the low 80s and lower before we bag him (after having first suctioned him to make sure the desats aren't due to secretions blocking his airway).

When Jack first came home from the hospital after being trached/vented, we had to bag him quite often because we had him off the vent a lot during the day and he would get pockets of collapse in his lungs because his respiratory muscles weren't strong enough for him to adequately ventilate himself off the vent.  When Jack was about three years old, it was clear he wasn't ever going to get off the vent and we stopped trying to wean him.  Once he was on the vent full time, he almost never required bagging. This having to bag Jack all the time is new within this last year.  As you can imagine, it's incredibly stressful having to resuscitate your child on what is becoming a routine basis.  Pretty soon, someone is going to have to resuscitate me because my body can't take much more of this stress!

Anyway, I'm not going to worry too much about the potential kidney stone until I know for sure that's what we are dealing with.  The difference this time around is that in between his episodes of distress, Jack is really pretty happy.  He wasn't that way last year when this whole saga started.  I'm expecting a call from radiology scheduling tomorrow and I'd guess we'll have the CT scan sometime next week.  Stay tuned.

On a completely different note, I just have to say that Eric is such a good sport.  When I drug him out of bed last Saturday morning at 5am to participate in the Ryan House Run, he asked me "Did I agree to this?"  He actually didn't have a choice. I signed him up, told him I needed a partner and he was it! He really did end up enjoying himself, mostly after the race was over.

After the finish of the 5K race

On yet another completely different note, I received paperwork in the mail this week telling me I needed to get Jack registered for his freshman year high school classes.  HIGH SCHOOL?!  It just can't be possible that Jack would be in high school next year.  Jack should be flirting with the girls,  playing sports and text messaging his friends all day long, not struggling to breathe and feel comfortable.  Sometimes the unfairness of it all really makes me angry.  But, I have to let it go because anger just saps my energy and I've got so little energy to spare these days.

Speaking of sapped energy, I must really look like hell because the child cashier at the grocery store gave me the senior discount when I bought groceries tonight.  Seriously kid, I might look tired, but I don't look 55+,  dammit.

Closing on a positive note, Peg arrives tomorrow and we are so looking forward to her visit.  We've not been able to see her much the last few years we've been out to St. Louis because she's been out of town.  Looking forward to our traditionally long talks into the late night hours and the requisite wine tasting that goes along with it.


Peg and Jack circa 2000