Sunday, November 11, 2012


We celebrated Jack's birthday last Sunday at Ryan House.  Jack and I stayed at Ryan House last weekend while Mark was out of town with Hilary. Jack hadn't stayed at Ryan House since March and I wanted to get him back there so they didn't forget him (not really!)  Actually, I had lots to do last weekend and without Mark or nursing, I'd have been stuck without Ryan House.  What I love about Ryan House is that they have separate rooms for the family - which are just like hotel rooms. I can hang out in my room and relax, read and/or sleep and be there to check up on Jack or answer any questions they might have, but still have time to myself. Or, I can be gone all day (which was the case most of last weekend) and come back and stay there the night with Jack.  It's just an awesome place all the way around ... with really awesome people who work there!

The family rooms have names that encourage one to rest, relax and sleep! 

I woke up the morning of Jack's birthday to find this on the door of his room:

When I peeked in on Jack he was asleep and his numbers were all good, which made for less guilt as I left to go to my sister's house for my niece's engagement brunch.  My niece Bridget and her fiance, Joe, live in New Jersey and almost didn't make it out for their engagement party because of Hurricane Sandy.  They didn't get out as early in the week as they had hoped, but they made it out by the end of the week and the engagement party went on as planned:

Congratulations Bridget and Joe!

After the engagement brunch, everyone headed over to Ryan House to celebrate Jack's birthday:

Ryan House graciously allowed us to use their kitchen area/dining room for Jack's birthday celebration.  

The big appointment of the week was with Jack's new pediatrician.  Her speciality is children with special needs/palliative care/hospice.  I love her!  She knew what questions to ask, she understood where I was coming from and she helped me (and Mark) make some difficult decisions now and gave us some difficult decisions to consider that will have to be made at some point in the future.  The best way I can explain how I felt after meeting with her is that for so many years, I've been leading the "charge" for Jack. I decided what I wanted for Jack and did everything in my power to make it happen (to the best of my abilities).  I was the captain of the ship and I was making all the calls - at least I'd like to think I was. :)  This last year, however, has not only taken a toll on Jack, it's taken the wind out of my sails.  I'm tired and, if I'm honest, I'm a bit lost.  I'm willing to release my grip on the reins and let someone else lead as we continue to navigate this journey with Jack.  I found this person in the physician I met on Thursday.  She came highly recommended and I was not disappointed. She was also not taking any new patients, but, thankfully, I had a "connection" who got us in.  I feel such a sense of relief knowing that I now have a pediatrician on board who will manage Jack in all aspects of his care and will not refer me out when the going gets tough.  

In other news, I received an email a few weeks ago from my friend and the co-founder of Cure CMD, Dr. Anne Rutkowski, letting me know that the world renowned physician/researcher at the NIH and an expert in the field of congenital muscular dystrophy (the same physician who met Jack a few years ago in San Diego) agreed to enroll Jack in whole genome sequencing to identify the causative gene for Jack's form of muscular dystrophy.  I was put in touch with the person who would arrange for us to get Jack's blood delivered to the NIH for testing.  I was told that they like to get the parents' blood as well when doing the testing. I mentioned that I had a daughter who we though might be mildly affected because she has low tone (Hilary), so she said to include Hilary's blood as well.  Knowing we'd be down at PCH for scheduled blood work that the nephrologist wanted, I asked if they could get us the supplies for the blood draw as soon as possible because the only place we'd be able to get Jack's blood drawn was PCH because he is such a difficult stick.  Getting PCH on board to draw Jack's blood for the NIH was a challenge. Getting someone on the other end of the phone to understand what I needed (someone to collect the blood from Jack and put it in a specimen tube I would provide) and what I didn't need (someone to spin, freeze, test or ship the blood) was the first hurdle.  After that, figuring out if they could even do it because how would they bill it? became the next issue.  I'll spare you all the details of our multiple phone conversations, but it was ridiculous all that was involved in just getting them to draw Jack's blood for me. Actually, they didn't agree to draw Jack's blood for the NIH, what they did agree to was that after they finished drawing Jack's blood for the nephrologist's order (which they could bill for), they'd fill the extra tube for the NIH.  As for the rest of us, my PCP's office agreed to draw our blood without hesitation and for no charge. (Thank God for the people who go out of their way to help us because they are the ones who replenish the energy depleted by those who go out of their way to put up hurdles.)  In any event, we managed to get all the blood drawn and shipped off the same day we were down to PCH for the appointment with the new pediatrician.

drawing the liquid "gold"

As Jack's neurologist pointed out, "giving Jack's muscular dystrophy a name does not benefit Jack, but it definitely helps us understand a very severe form of muscular dystrophy which may benefit others."  

Unlocking the mystery of Jack?

After spending a good part of the day down at PCH on Thursday, I thought I was done for awhile.  However, I received a phone call Friday morning when I was at work from the nephrologist's office telling me that Jack's blood results came back with an extremely high potassium level and I needed to get Jack back down to PCH for a repeat blood draw stat.  I was also told that the blood that was drawn the day before had hemolyzed and, therefore, that could have skewed the results. Needless to say, I was not happy about having to leave work, pick up Jack and make the half hour drive back down to PCH!  My mood didn't get any better when I arrived at PCH and was greeted by a security guard before I even got to the entrance of the parking garage and was asked "what brings you here today?"  Really? What brings me here today?  With an exasperated tone (because, I really was not happy at this point), I proceeded to ask him, "what do you THINK brings me here today?  This is a Children's Hospital, is it not?" I mean, really?  I wasn't there to do my grocery shopping!  I have an issue with PCH's security anyway because you'd think it was the Pentagon with all you have to do just to get in the parking garage.  I understand having to sign in and get a badge once you get inside the hospital, but the third degree at the parking garage is unnecessary!  After we entered the hospital, I discovered why the question prior to entering the hospital grounds -- Garth Brooks and other country celebrities were on campus for a fundraiser/publicity event.  

We made our way through the crowd of cameras, lights and people to get to the lab on the second floor. The lab tech tried twice to get blood out of Jack without success. They have a "2-stick" rule and after that they have to call the ordering physician's office, have them send a request for the IV team and then the IV team is paged and we wait for hours until the IV team can make its way to the lab.  Two plus hours and three sticks later, they finally got their vial of blood from Jack.  

I was told to hold Jack's dose of Potassium Citrate until they called us with the lab results.  No one called me on Friday with the "stat" lab results, so I have no idea if Jack's potassium really was high or if it was, in fact, due to the bad blood from first blood draw.  I'm inclined to think that his levels were fine with the repeat test.  I'll call tomorrow and find out.  

This week we have more appointments - with the nephrologist and the pulmonologist.  I swear, I am so over appointments with doctors!  I'm vowing to take the rest of the year off.  Think I'll be successful?

That's all the "stuff" from here. Have a great week friends!


Vicki said...

What a frustrating, difficult week for all. I am glad you had a good weekend &the that the new Dr is so good.

Sarah said...

Ann I'm so glad you found such a wonderful new Dr! So wonderful to just step back for a little while and let her lead. So frustrating about the blood draw. I don't understand why it takes them so long to get the IV team to even show up. Insanity. I know how stressful the waiting can be especially for one little vial that should have been taken care of properly the first time. Lots of loves and hugs as always as you hit up your next few appts. I feel you as we have 5 scheduled over two days this week. Peaceful night's rest!

Anonymous said...

I'm so glad that you have found a pediatrician that you connect with--it makes all the difference in the world!

I'm sorry your hospitals are challenging. I think that all children's hospitals should follow a similar model so the experience is the same or close to the same at every one. It's not fair to the kids or their families when treatment and care varies so wildly and widely between facilities.

Have a good week. I hope Jack remains stable and comfortable:)

kadiera said...

I think I'd just start having the doctor fax the IV order for each blood draw.

The ER here at our home hospital is on notice now that nobody does a draw or IV on my kids unless they're from PICU or anesthesia after our last few failures. We don't need to traumatize them because someone thinks they can get it

CS said...

Oh goodness. Some of that "stuff" sounds more like "hell". I'm glad you found an advocate for Jack AND you. Huge hugs, Dear Ann.

Susan said...

Ann I am so glad that you were able to pull some strings and get the help of a good pediatrician. I can only imagine how nice it must be to finally feel you have an ally. Wouldn't it be great it you could just proclaim you are done with doctors appointments and that was that?!

I'm glad that you were eventually able to get the blood samples. Hooray to you for all the work required to make it happen. You're amazing! I hope that some good will come of it. I'm going to send you an e-mail.

I'm so glad you have the Ryan House. What a blessing! I'm glad Jack had a nice birthday.

ssouth said...

Geeze Louise, what a time you've had. I sure hope the blood sent to NIH didn't hemo-whatever too. I'm so glad you've found a great pediatrician that will hopefully help keep the whole picture in focus and take some of the burden off your shoulders. I'm afraid I'm just going to have to add you to the permanent prayer list here.

Christy said...

I'm so glad you have the Ryan house! And that's awesome that you found that pediatrician! I really hope that you get some relief in all the decision making.

Love ya!