Wednesday, November 04, 2009

Emails

Of all the doctors whose emails I share below, only one still remains at St. Louis Children's Hospital and continues to provide care to Jack as a patient. The other doctors have moved to other top Childrens' Hospitals around the country, yet they continue to be there for me and Jack - whether to answer questions or to simply listen and give support.  I am very fortunate to have found such a remarkable team of doctors who have gone above and beyond the call of duty and who have been there to hold my hand and walk me through the last eleven years.


__________

June 24, 1999
Dr. M,
We’ve been home for two weeks now and we’re still home, so that is good news. Jack had a terrible Monday – Sally from the PICU was here as his nurse and he had her hopping. He spent most of the day on the vent. We are not sure why he had such a tough time on trach collar. He has done well the rest of the week, however, his secretions have increased slightly and have gotten a little thicker. They are still clear, so I don’t think he has an infection.

His feedings have gotten a little better, but he still has trouble keeping them down. His first feeding in the morning is his toughest. I asked Dr. H to prescribe Reglan, which he was on prior to being admitted to the PICU. I haven’t given it to him yet, but if he continues to have trouble keeping his feeds down, then I’ll probably give it to him. Any thoughts on this?

That’s our update. I’ll let you know if anything significant shows up.

June 25, 1999
Ann,
Glad to hear Jack is doing so well. Please keep us posted and let us know if we can otherwise help at all.  Go Jack Go!

__________

November 23, 1999
Dr. G,
Jack had a lot of coughing this am with moderate secretions – more like allergy symptoms. I gave him an albuterol treatment this am. Around 6pm tonight he looked a little distressed and sounded collapsed on his right side again, so I have him albuterol, PD and I bagged him for a few minutes to open him up. I turned his rate from 8 to 6 – you wouldn’t think it would make such a difference. Jack must have a low tolerance for change – just like his mom!
__________

March 6, 2000
Dr. G,
During the day Jack has been on a rate of 4 and pressure support of 8. I did turn down his rate to 1 for several hours on Saturday and he did okay. At night he remains at 10 and 10.

To be honest with you, I’ve lost all interest in weaning Jack from the vent. It seems that every time we start making progress he gets sick and we go backwards. It’s easier just to not have any expectations. Interestingly, it has been one year today that Jack has been on a vent – a depressing statistic.

Maybe you can give us a “plan” for weaning – as I’m tired of trying to figure Jack out.
__________

March 27, 2000
Dr. C,
I wanted to let you know that I’ve decided that I will agree to have an MRI of Jack’s brain. Because I’m convinced that there is nothing wrong with him cognitively, I guess I shouldn’t worry about what the scan might reveal. Anything that might get you closer to figuring Jack out is worth a shot.
__________

May 15, 2000
Dr. M,
I received a copy of the report from Dr. B and its states, in part: “there is moderate diffuse cerebral atrophy with prominence of the ventricular system and sulci. There is absence of the inferior vermis and of the cerebellar tonsils bilaterally. The remainder of the posterior fossa appears unremarkable.”

I spent a good part of this afternoon down at the medical library researching what I could so that I can better understand what Dr. C will have to say and to come up with some questions of my own. Like you said, I’m focusing on Jack and his progress, not some textbook explanation of what these brain abnormalities mean.

P.S. Jack has been tolerating pressure support trials of up to 5 hours a day - 2 hours in the morning and three hours in the evening (can you guess who is doing the evening trials?) During the trials his rate is 0, pressure support 4 and peep 5. Maybe we’ll get back on trach collar soon?
__________

May 30, 2000
Ann,
You already know what my advice would be – don’t push him too, too hard. Don’t look at it as how he is compared to his best, look at it compared to his worst. Now that is a long way. I would really encourage keeping a minimum rate and pressure support during sleep until you’re sure he’s really OK on trach collar for a while during the day.

Congratulations! You have really done an incredible job with him. Now, there is no way he can give you any trouble as a teenager.

__________

September 1, 2000
Dr. M,
The alarm that is going off is “volume error”, and, although I appreciate your confidence in me, you guys are giving me too much credit when to comes to vent settings. I have an understanding as to what the range is for his pressure support and rate, but when you suggest that I go up or down on certain settings, I need numbers.

I turned his sensitivity setting up from 3 to 5. He did not alarm at all last night, but he was positioned on his side and his night nurse did not reposition him at all. But this morning, when I repositioned him on his back, he did alarm, but only once. So maybe changing the sensitivity did help some. Should I adjust his tidal volume next? If yes, give me a number. Doesn’t tidal volume only come into play when he has a rate. With a rate of only 8, how does changing the tidal volume make much of a difference?

September 1, 2000
Ann,

Regarding;

The alarm that is going off is “volume error”, and, although I appreciate your confidence in me, you guys are giving me too much credit when to comes to vent settings. I have an understanding as to what the range is for his pressure support and rate, but when you suggest that I go up or down on certain settings, I need numbers.

OK, 1, 2, 3 … sorry, just kidding.

I turned his sensitivity setting up from 3 to 5. He did not alarm at all last night, but he was positioned on his side and his night nurse did not reposition him at all. But this morning, when I repositioned him on his back, he did alarm, but only once. So maybe changing the sensitivity did help some. Should I adjust his tidal volume next? If yes, give me a number. Doesn’t tidal volume only come into play when he has a rate. With a rate of only 8, how does changing the tidal volume make much of a difference?

. . .

It would really help to have someone stand there and turn the dials and see what is happening. Do respiratory therapists still come out? Can they call us next time they are there? I would suggest you bring him by the hospital but I know how much you love to visit (NOT!) and besides, if it only happens when he sleeps, stopping by may not help.


__________

November 13, 2000
Dr. C,
I had emailed Dr. H regarding his progress on the research he was doing with respect to Jack’s muscle tissue. I have forwarded his response I received. Could you please tell me what the results sent to you said? What did they test for that showed up as “normal”? I’d like to know what diagnoses can be excluded. I realize you are busy and have many patients, but my life is Jack and I live daily with his condition and not knowing where we are heading. I’d appreciate receiving all information you receive regarding Jack. Unless someone tells me that there is no hope of him getting better then I am going to pursue any and all avenues relentlessly ruling out treatments rather than just assuming they won’t help. I’d still like to know why we can’t try IvIg treatments. The steroids clearly helped some, but they didn’t put him in remission if that is a possibility. I need to know that it won’t help, not just assume it won’t. Dr. S said that you felt side effects from steroids were less than with IvIg treatment. Don’t you think that should be my call?
__________

March 23, 2001
Dr. G,
Jack is not doing so great. Lots and lots of secretions, coughing, puking, suctioning (you get the picture).  It seems more like allergy symptoms to me, since it’s so much upper airway congestion. His secretions still remain fairly clear and once you get him all suctioned out his lungs sound clear. Not much we can do I guess except to try and dry him up. I called Dr. R’s office after hours on Wednesday to see if they had any suggestions for meds to help him. I got Dr. L – who was a jerk. The first thing he asked me was whether Jack was a full code! Apparently, he felt if I was concerned enough to call a doctor, then it must be serious enough to call 911. Dr. L had no suggestions for helping Jack and he said that kids Jack’s age don’t get allergies and that I should call my pulmonologist. Now you can understand why I prefer to deal only with you. In all fairness to Dr. R, I’m sure he would have handled it differently. I really didn’t see any reason to call Dr. S, mostly because I would not have gotten her anyway, I would have gotten a resident who doesn’t have a clue about Jack.

Sorry to vent. I’m sure we’ll get through this like we always do. Have a great weekend – hope you get to spend it outside of the hospital.
__________

April 10, 2001
Ann,
I can appreciate your frustration about Jack and getting answers. I wish there was a solution to the problem. Believe me, though it is not my area of expertise, if I come across anything that might shed light on Jack, you’ll be the first to know. But, as you must know, if people like Dr. C and the people at Wash U’s neuromuscular disease center are ultimately stumped, there is little that mere mortals like you and I will discover. But it needn’t stop us from trying.

__________

May 8, 2002
Dr. M,
Just wanted to give you a Jack update – he is doing great. Dr. S increased his tidal volume because he was dropping his sats at night and seemed to have something going on with his right side. It was only supposed to be a temporary situation, but you know Jack, once you give him more support he almost never wants to give it back. Doesn’t look good for him ever getting off the vent, eh? Really wish I could get a prognosis on the kid. I broke down and made an appointment with Dr. C before we leave – don’t know why I don’t like to see her, guess because she never has any good news.

We don’t have any appointments down at Children’s before we leave, they are all at Missouri Baptist. We are planning a “Thank you” party for everyone who has been a part of Jack’s life here in St. Louis – details to follow.
__________

January 31, 2004
Dr. S,
Have any desire to move to Phoenix? Jack is in desperate need of someone to manage his vent. He hasn’t seen Dr. P since May and his vent settings haven’t been adjusted for over a year. Jack is doing very well all things considered. It is my mission to get one of the hospitals in this city to recruit a pulmonologist who specializes in kids and vents. Although my current mission is planning a conference that will take place in June in St. Louis for families of kids with trachs. SLCH has agreed to be one of the sponsors for the event and we are hoping to get a panel of doctors from the hospital to speak on various topics that are relevant to our kids. Are you available?

On another note, I’d like Jack to see you when we come to St. Louis is June. Do you see any benefit in updating his PFTs and/or checking for any change in the movement of his diaphragm? I don’t want to have a bunch of tests run just to do them, but if you think we can gain some useful information from the tests, I like to have them done. If you’d be willing to adjust his vent settings that would also be good!
__________

July 19, 2005
Dr. M,
We are home and Jack is doing great. I think he tolerated the last two weeks better than the rest of us. It was good to see you and I was very happy you were covering the PICU for part of Jack’s stay. I really appreciate you letting us bail from the hospital even though you weren’t 100% comfortable with Jack’s x-ray. He seems to be fine with no signs of a pneumonia. I’m tempted to stop the antibiotic, but I won’t.

We’re supposed to come back in 2 months for a repeat bronch. I can’t even think about that right now. There has to be an easier way to get Jack to St. Louis, I just haven’t figured it out yet!
__________

July 19, 2005
Ann,
Glad to hear you made it OK and that Jack is holding up.

Well, you know we would not have released him to just anyone.

__________

August 19, 2005
Dr. M,
Dr. C called me today and after six plus years and two muscle biopsies, we can now give a name to Jack’s condition: Congenital Muscular Dystrophy secondary to reduced alpha dystroglycan. Try saying that 3 times fast! I know giving it a name doesn’t change a thing – it just means I can’t be in denial any longer. Most people would think, how in the heck could I be in denial – all you have to do is look at Jack and it’s pretty obvious he’s got some serious issues going on. But, it’s amazing what you don’t see when you don’t want to (not that I saw a perfectly healthy kid who was going to get off the vent and run a marathon some day). I guess he is officially one of “Jerry’s Kids” – a club I’ve tried to avoid like the plague.

Anyway, life goes on. We’ll be back to St. Louis the first week of October. Let me know if you’ll be around on the 5th of October – Jack has a bronch that day.
__________

January 13, 2006
Dr. M,
I need help trying to figure out Jack and since Dr. S is still incognito, I thought I’d ask you. I won’t give you the specific details of what’s going on with Jack because I’m not asking you to diagnose him via email. My question – can a kid have pneumonia even if his sats are fine (> 95) and his secretions are not colored or excessive? The only symptoms are a high heart rate and he looks like he’s working to breathe. (Ok, so I gave you a few details).

I’m lost and frustrated and Jack is not a happy camper.

January 13, 2006
Ann,
Speaking hypothetically, yes, one can have pneumonia without those findings. On the other hand, just based on heart rate and increased work of breathing, a pneumonia is not the only thing to consider in a child with a trach (I’m thinking airway obstruction at some level).


January 13, 2006
Dr. M,
Hypothetically speaking, I’d rather have my toenails ripped off one by one than deal with the doctors in this City. You mention airway obstruction and I’m thinking ENT - we are in big trouble because I’ve pretty much run through all the ENTs here. So, my only option is to wait until the issue becomes acute and then take him to the ER. It’s Friday the 13th and there’s a full moon – the ER is definitely the last place I want to be tonight!

Thanks again – I’ll let you know if it’s anything serious.
__________

February 9, 2006
Ann,
I was invited to a conference of the peds neurologists the other day. The discussion was about predicting outcomes with neurologic disabilities, ethical issues and how to speak to families, etc. AC actually mentioned her experience with you and Jack, without your names of course. But, I knew who she was talking about. You see, Jack (and you) continue to teach us and hopefully make things better - at least in terms of how we communicate.

__________

August 1, 2006
Dr. M,
I’m too emotional right now and will probably do something stupid like cry. I’ll be fine. It’s just the BS politics of being on the floor. No one can make a decision around here – too many teams involved, but no one will own the problem. If we were in the PICU, the issue just gets resolved.
__________

February 4, 2007
Dr. M,
As far as our recent experience at SLCH, I’m sure the dreadfulness of it all was amplified, in my mind, by the absence of you and P. I just hate it when I’m forced to make decisions that I don’t agree with – which was the case with the broviac/vanc. But, it’s done and over with and time to move on.

And, please, stop giving me so much credit for keeping Jack alive. I appreciate your kind words, but let’s be honest, it’s a damn machine keeping Jack alive, not me. I suppose the fact that I know how to keep the machine running helps some.

February 4, 2007
Ann,
You are taking me literally about Jack. Of course the machine is “keeping him alive”. What I’m saying is that I’ve seen a lot of medically fragile kids either just wither and die or not have the kind of life you’ve provided for him – in spite of the machines. And with regards to whether doing all this was the right thing, only your heart can answer that. Watching the latest video clip of him – obviously happy – answers the question in my mind. But it’s your mind and Jack’s mind that matters.

__________

April 15, 2008
Dr. H,
I want to share a letter with you that I wrote several months ago as part of a first year medical student’s project for her “Reflections on Doctoring” course. I shared the letter with Dr. M and he felt it was worth sharing with the physicians on the Pedsccm.org website. I am passing the link to the letter your way in hopes that you might feel its worth sharing with the physicians in training who you work with. It is my plan, as an extension of this letter, to have pocket stones with the words “kindness”, “care” or “compassion” engraved on them and provide a stone to young physicians to carry in their white coats as a reminder of what’s important when interacting with their patients and patients’ families. Let me know of if you are interested in being part of the pocket stone project.

April 16, 2008
Ann,
I am intrigued by your “Pocket Stone” project and would be very interested in partnering with you. We spend an enormous amount of time trying to teach compassion and professionalism to young physicians.  I have long maintained that there is very little I can add if it wasn’t given to them in their formative years by their parents and yet, I am learning with time that you can teach some old dogs new tricks! Let me know what I can do and how I can help.

This was the perfect email to finally get to at the end of a long 36 hours in the PICU. It helps to remind me that we can all make a difference.
__________

October 14, 2008
Dr. S,
We just returned from a week of appointments in St. Louis, including a sleep study. The report will go to AC, who will provide the report to me to give to Jack’s pulmonologist here in Phoenix. I’d like to provide a copy of the report to you and get your thoughts. Jack had to be admitted for the sleep study and we saw Dr. S. - he asked why I don’t see the pulmonologist at Phoenix Children’s. When I explained my reasons why, his comment was that I’ve just been “spoiled” by you. Very true! (and I sure miss having an advocate like you on my side).

Jack is doing well, all our appointments went well in St. Louis and all things considered …. Life is good.

October 15, 2009
Ann,
It was an honor to "spoil" you and Jack.
__________

June 18, 2009
Dr. S,
I wanted to let you know that I’ve become involved in a newly formed organization called Cure CMD. One of the things Cure CMD is trying to do is put together a list of “top docs” for kids with CMD as part of their standard of care initiative. I was asked to recommend a pulmonologist to add to the SOC effort. Of course, you are at the top of my list. I just wanted to give you a heads up in case you should happen to receive some information from Cure CMD.
__________

September 8, 2009
Ann,
Just in case you haven’t heard, I am on the CMD standard of care committee. I am the US pulmonary group leader. Thank you for mentioning my name. Didn’t you tell me that they had identified what form of CMD Jack has? If so, could you remind me which one? It will help me put some of the information into perspective with the other CMD’s we are working on. Hope all is well with your family.

2 comments:

ssouth said...

Shoowee! And that's just a tiny glimmer of the picture. I know you don't like to take credit, but credit, credit, credit to you!! I wish I could be so forth right with doctors. I've only e-mailed one and that was after you encouraged me.
Happy Happy Birthday to Jack. I hope the whole family enjoys this day.

Melisande said...

Ann, you know this is your life in these letters. I think this might be good stuff to include in a book or something. I know letters like this myself! I've written a few. I hope our docs know the power nature of these exchanges in the kind of care we can give our kids. I've only been able to do what I did for Donovan with any confidence with the support of Dr. C. Without all that, I would have never had the kind of confidence to do what I needed to do, at least for me. It's a huge, huge thing. Thanks for sharing as always. I'm putting this stuff in my memory bank. If I get to work on my PhD I think there's something to do with it. Maybe there's some stuff worth making scientific enough to make all doctors do what our doctors have done for us!!! My wheels are always turning too. {hugs}