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.


Susan said...

F indeed Ann!!! I just don't know what to say except I am SO SORRY. As always you are an exceptional advocate for Jack. I hope you have some time before surgery needs to be done. I'm glad Eric had a great birthday. When did he get braces? Anyway, you'll be in my thoughts. I really wish I lived closer and could give you an ear and a shoulder. Hang in there.

Anonymous said...

Oh Ann! I don't like this news about Jack at all! I hope there is a less complicated solution than last year. You are in my prayers.

Anonymous said...

Ugh!! Thinking of you today, Ann, and praying for strength for you. I'm sure it's frustrating to have to deal with that darn kidney stone again. And I know firsthand what you mean by feeling like you are going to throw up when you think about any more surgeries for Jack. Our Jonathan has had about 45 surgeries already. We are contemplating a never-been-done-before surgery for Jonathan (his innominate artery is pressing on his trachea AND his trach tube has eroded his trachea on the inside...a fatal combination if not addressed surgically) and it makes me want to vomit! And when you say you are tired...I understand. I really get it. It's pure exhaustion....both physically and emotionally. But, we do it because we love our boys more than anything. Please don't ever give up, Ann. Jack is where he is today because of you. And please know that you have so many people who care (even people like me who don't know you personally!!). I appreciate the honesty in your posts. It shows me how I can pray for you specifically. And I do.

Keep pressing on,


E Fischer said...

I think the word your looking for is Fubar.

Sarah said...

Oh Ann. I can't believe this! Why do our kiddies have to have the most outrageous things? I simply can't believe the rate at which these stones are growing!! I'm simply in shock. Hoping the Dr gets back to you quickly. Please let me know if you need anything. Loves and hugs!

Ann said...

Eric, I had to look that word up, but you would be correct!

Ann said...

Amy, the same thing happened to Jack. He has a right aortic arch his aorta sits right up against Jack's trachea. When they bronch Jack, they can see the aorta pulsating from inside the trachea. Back in 2005, during a routine bronch, they discovered that Jack's trach tube was eroding through the trachea and if it did, it would puncture the aorta and that would have been the end. The pediatric cardiothoracic surgeon talked with the adult CT surgeons and they came up with the idea of putting a pericardial patch between Jack's aorta and trachea to create a barrier in the event the trach tube were to erode through. What are they proposing for Jonathan? Jack's neurologist did share with me that one of the top causes of death for her neuromuscular patients who are long time trach users, is the trach tube breaking through and hitting an artery causing a massive bleed. Not a pleasant thing to consider, that's for sure. I hope they figure something out to help Jonathan.

Thanks for your support.

Anonymous said...

Ann, The doctors are planning to do a graft of Jonathan's innominate artery to lengthen it and then put a piece of muscle (from his neck, because it needs to have a blood supply) in between his artery and trachea. The cardiac surgeon does grafts all the time. He just hasn't done this particular surgery for this reason. As you probably know, they often find out about a tracheoinnominate fistula when it's too late (and the patient bleeds out) so they don't really have any experience with this in a preventative sense. Anyway, we had it all planned for April 22 and now one of our longtime doctors just randomly said "Let's not do it!" So now we're forcing face-to-face communication between this doctor and the cardiac surgeon (and possibly the ENT) (which should have already happened, in my opinion, in a rare case like this). Anyway, I know the severity of the situation and will go ahead with the surgery even without the "blessing' of our longtime doctor. We've done it before and are not afraid to do it again. I have seen the reports regarding what's going on and I feel like we don't really have a choice.

I read your most recent post (Fri) and am frustrated for you!! I don't understand when doctors do not return calls...especially to parents of kiddos with special needs. I joke that we are still waiting for the on-call pulmonologist to call us back from a call I placed one evening in approximately 1999!! It's really not funny...but after that many years I have let it go :)

Hope things start looking up over there and that you get that phone call on Monday.