Zach Sobiech: Finding peace through music | Children's Cancer Research Fund

Pretty amazing story about a young man who left his mark on the world before he left it.

Zach Sobiech: Finding peace through music | Children's Cancer Research Fund

On the 23rd of this month Kody had another Cardiologist appointment.  This time he met with the HCM/Arrhythmia specialist at Scottish Rites of CHOA.  I liked the doctor and so did Kody.  We discussed his heart condition and he agrees with us about remaining medication free.  This puts me at ease because there are some doctors that would push for medications even if the cons are worse than the possible pros.  I will add the doctor's notes once they have arrived.  He is also putting us in touch with the social worker about his DNR being in place here in GA.  Kody and I have spoke about things and he does not think living on a machine, considering the path chosen by the FRDA, is something he would want to do.  I respect his opinions and decisions on that.

Christmas went well.  He is pictured here playing one of the few games he got, with his new controller.  He also likes to play some of the games with Rikki.  They giggle and argue about who is kicking who's butt.  It is kind of funny to listen to.

Things with the move have taken time.  I have to call to make an appointment to establish him with the local chapter of the MDA.  It has been a busy last few months for sure.  We have switched out his seat for his manual with the seat from his electric chair to give him a better level of comfort.  He has been having episodes of his legs being cyanotic (blue in color) and we are going to address this with his cardiologist on the 23rd.  He does not complain about much of anything and is a pretty easy to please kind of guy.  Things with his schooling are going well and he loves when he gets to be exempt from assignments that he really did not want to do.  You see, he does not like doing essays because he finds them boring.  I think he just has not quite figured out how to BS his way through one.  He is the type of kid that likes things straight to the point without all the unnecessary wording.  I was able to find a good deal on some sweat pants for him from JcPenny's that fit him well and are easy to get on and off for bathroom trips.  He prefers to remain as independent with his bathroom needs as possible though he does need some assistance with redressing after the fact.  But he has managed to get past being embarrassed about such things with me.  Though he does not ask his dad for help which I find interesting.  You would think it would be easier to have dad help with that stuff but not with him.

 

We were able to get a controller to help him out with his PS3.  This stops him from being bored all the time.  Or at least that is what he says.

This week, on Wednesday, Kody has a writing assessment for school.  The teacher is bringing out a computer to have him do it on.  That might take a while but he will get his point across. I have to schedule his appointments soon and get them taken care of.  I have to remember to email CMS and keep them in the loop for everything just incase his state insurance were to not be working for some reason or another. 

He has been doing good.  Of course mom is his favorite word when I am home.  For the most part it does not bother me except when I walk into the room and answer whatever it is that he is asking and then get into doing something else only for him to call me again.  

Right now his PS3 is out of commission because he needs a new dual shock wireless controller for it and at the moment I do not have the funding to be able to get it for him.  This sucks since he just got 3 months of PS+ and a new game.  Once money is right though we will work towards getting him a new remote.

Kody loves the new dog.  Sadie has blended in well and is spoiled by everyone.

Cardiologist Notes .....

Dear D. Champagne (this is because this letter was sent to us)

Chief Complaint: Friedreich's Ataxia and Hypertrophic Cardiomyopathy

Pertinent Clinical History: Kody is a 17 year old male who is seen for the first time in our practice, having recently relocated from the Tampa, FL area where he was followed by Pediatric Cardiology Associates for his hypertropic cardiomyopathy secondary to Friedreich's Ataxia which was diagnosed at the age of 7 years while the family was living in Michigan at the time.  He is not currently on any cardiac medications, having failed even low-dose atenolol due to low BP (50's/30's) with recurrent syncope.  He was also tried on the investigational medication idebenone as part of a research trial but has to stop it due to an "allergic reaction."  He has occasionally taken lasix PRN when he has leg edema, but he is not on any standing lasix dose due to concerns over his dynamic left ventricular outflow tract obstruction being worsened by hypovolemia.  When he was last seen by his cardiologist in Florida in February 2013, a 24 hour Holter monitor was placed, the results of which are not available for my review.  His mother was told that it revealed "arrhythmic moments" and was as expected given his condition.  His cardiac records have been requested, and his mother will be scheduling him to be seen by neurology, orthopedics, and pulmonary medicine locally.  He denies any palpatations, but he will occasionally have pressure-like right-sided chest pain that is not associated with other symptoms and which does not require any treatment to resolve spontaneously.  He also has significant scoliosis and restrictive lung disease and is wheelchair bound.

Kody has a past medical history and family history are documented in the medical record.

A 10 system review was performed and is documented in the medical record.

Medications:  No current outpatient prescriptions on file.

Pertinent physical findings: 

Pulse 100, Resp 18, BP RUE 100/78 mmHg, BP LLE 112/0 mmHg, Wt (wheelchair bound), Ht (wheelchair bound)

Constitutional: no distress, wheelchair bound with profound muslce weakness and difficulty speaking, cachectic, and small for age.

Head: normocephalic, atraumatic

Eyes: normal sclera, conjunctiva, and lids

ENT: inspection of nares, gums, oral mucosa, and external ears appears normal

Neck: mobile with weak muscle tone

Chest and lungs: clear to auscultation and fair air exchange without wheezes with asymmetric chest shape.

Cardiac: normal active precordium, regular rhythm, normal S1, physiologically split S2, no diastolic murmurs, clicks, rubs, or gallops, brachial and femoral pulses are 2+ and symmetric without delay, normal distal perfusion with brisk capillary refill, no jugular venous distention and no clubbing, cyanosis, or edema; grade I-II/VI medium frequency systolic ejection murmur maximal at the left sternal border and radiating along the sternal border.

Abdomen: no masses, no hepatamegally, no splenomegally and soft and nontender / active bowel sounds.

Muscoskeletal: severly decreased muscle strength diffusely; sever scoliosis

Extremities: decreased muscle tone in extremities

Skin: clearn, no rashes, no lesions.

Neurological: cooperative, able to speak a few words at a time without becoming out of breath.

Laboratory:

ECG: due to his known hypertrophic cardiomyopathy, a 12 lead electrocardiogram was performed in order to assist with the evaluation of his rhythm and ventricular forces.  This was obtained and demonstrated: normal sinus rhythm at 96 bpm with a normal corrected QTc interval of 401 msec.  There was inferolateral T-wave inversions as previously reported with ST segment elevation in leads V1-V3 as previously seen.

Echocardiogram: Given his past hypertrophic cardiomyopathy I did recommend an echocardiogram in order to evaluate for any worsening of his ventricular function or left ventricular outflow tract obstruction.  This study was obtained and demonstrated severe concentric left ventricular hypertrophy with a IVSd of ~18-19 mm and a LVPWd of 14 mm.  There was a near LV cavity obliteration in systole with systolic anterior motion of the mitral valve resulting in very mild mitral regurgitation.  There was no effusion and no evidence of cor pulmonale.  There was good biventricular systolic function but evidence of significant left ventricular diastolic dysfunction based on tissue Doppler imagine. Overall, the left ventricular wall thickness seems compariable to his report from his previous study in Florida.

Impressions:

1) Friedreich's Ataxia

2) Severe hypertrophic cardiomyopathy without significant resting LVOT obstruction.

3) Systolic anterior motion of the mitral valve (SAM) with very mild regurgitation.

4) No pericardial effusion.

5) Marked left ventricular diastolic dysfunction with "normal" sytolic function in the setting of marked left ventricular hypertrophy.

Discussion:  At the present time given Kody's stable clinical status, I have not recommended any cardiac medications since he was intolerant of beta-blockers and idebenone.  I did review the importance of keeping him well-hydrated as hypovolemia could potentially result in increased dynamic left ventricular outflow tract obstruction.  I would also suggest that he be followed in our hypertrophic cardiomyopathy clinic and that the family establish care with a local pediatrician as well as a pediatric neurologist, pulmonolgist, and orthopedic surgeon.  His mother is in agreement with this plan.  Overall, it would seen that his prognosis is poor given the severity of his heart disease,  There is a notation in his records from Florida that a DNR order was in place.  This should reasonably be re-addressed once he has been seen by our HCM clinic and by his local subspecialist.

Establishing a Cardiologist In Atlanta

We had an appointment this past Thursday with Sibley's Cardiology in Atlanta.  The appointment was only two hours long which was not bad for an establishing appointment.  The doctor asked us to bring him back in three months to be seen by the doctors who specialize specifically in Hypertrophic Cardiomyopathy.  First impressions of the doctor and the clinic is that we liked them.  Kody was included in conversation and treated with respect.  Of course he liked all the pretty nurse techs.  The doctor came into the room where he had the echo done and explained everything he was seeing on the screen and asked if we had any questions.  At this point his heart has not gotten better but not gotten worse in comparison with the notes from the last appointment in April.  They have not yet reviewed his full chart because it had not been received by the clinic.  It had to be sent via snail mail because of the amount of information.  

Summary of report:

*Mitral valve regurgitation is present and is mild. This means the valve between the upper and lower chamber of the heart on the left side does not close properly so the blood being pumped out trickles back into the bottom chamber.  

*The right portion of the mitral valve when opening has a tendency to suck up to the right side of the wall.

* Thickness of the heart muscle is such that though it is pumping efficiently the walls touch when the chambers fully contract.

I made mention of the aorta narrowing but did not ask about it when he had the sonogram screen in front of me.  

I will call on Tuesday to make the appointment for three months from now.  Because of the HCM he has to be seen on a Monday and at the clinic in Atlanta verses their location in Marietta which is closer to us.

I set up an appointment for 8/26 @ the new cardiology clinic in Atlanta.  The plan was for Kody to go to Sibley Cardiology in Marietta GA BUT when I called to make the appointment I was told he has to be seen at this other place because they SPECIALIZE in HCM.  Yes, this is the first time ever we have dealt with someone who specializes in Kody's heart condition.  Yesterday I had to fax the request for the old clinic to send ALL of his records to the new clinic.  This should be interesting.  Will update at a later date.

Camp Pictures

I have put together a "story book" of some camp photos from Kody's final year at MDA camp.  Click on the photographs below to be able to view all the pictures.  He had a blast and they really did love the heck out of him.... was sad to close that chapter of his life.  

Day 2 At Rotary Camp ..... in Brandon, Florida

In these pictures Kody seems to be in a contest ... I thought it was drinking liquid but apparently he is trying to suck jelly through a straw.  I would not be surprised if he managed to get it done ..... lol ... go Kody. 

The Special Mother by Erma Bombeck

The Special Mother 

by Erma Bombeck 

Did you ever wonder how mothers of disabled children were chosen? 

Somehow I visualize God hovering over the earth selecting his instruments of propagation with great care and deliberation. As He observes, He instructs His angels to make notes in a giant ledger. 

"This one gets a daughter. The Patron saint will be Cecelia" 

"This one gets twins. The Patron saint will be Matthew"

"This one gets a son. The Patron saint.....give her Gerard. He's used to profanity"

Finally He passes a name to an angel and smiles. "Give her a disabled child".

The angel is curious. "Why this one God? She's so happy"

"Exactly," smiles God. "Could I give a disabled child to a mother who does not know laughter? That would be cruel!"

"But has she patience?" asks the angel.

"I don't want her to have too much patience or she will drown in a sea of sorrow and despair. Once the shock and resentment wears off, she'll handle it. I watched her today, she has that feeling of self and independence that is so necessary in a mother. You see, the child I'm going to give her has his own world. She has to make him live in her world and that's not going to be easy."

"But Lord, I don't think she even believes in you"

God smiles, "No matter, I can fix that. This one is perfect - she has just enough selfishness"

The angel gasps - "Selfishness? is that a virtue?"

God nods. "If she can't separate herself from the child occasionally she won't survive. Yes here is a woman whom I will bless with a child less than perfect. She doesn't realize it yet, but she is to be envied. She will never take for granted a 'spoken word'. She will never consider any 'step' ordinary. When her child says "Momma" for the first time she will be present at a miracle and will know it. I will permit her to see clearly the things I see...ignorance, cruelty and prejudice...and allow her to rise above them. She will never be alone. I will be at her side every minute of every day of her life because she is doing my work as surely as if she is here by my side"

"And what about her Patron saint?" asks the angel, his pen poised in mid air.

God smiles "A mirror will suffice"

MDA Camp

On Monday we took Kody for a visit with the MDA doctor to get his physical and clearance for camp.  I had to make sure to bring the clearance from his cardiologist.  He is excited about going to camp this year and this will be his last year since he is 17 now and the age max 17.  The doctor was quite impressed with the strength level he has in his extremities.  It just confirms what we do for him is working in some respects.  He is not babied but rather left to do as much as he can.  He tires easily but does manage to do some of the essentials by himself.  He can bathe and transfer to the toilet on his own but sometimes needs help if he missteps.  For his bath we run his water and put his poofy and soap within his reach.  I am proud of him for not complaining about what he does not have or is not able to do .. but rather does whatever he can do on his own with pride.  

His halter monitor came up as the doctor expected it to.  He is still medication free and that is what works for him.  Life is life for him and he does not complain about much except when I interrupt his gaming time.  

FRDA has altered how his life is going but has not taken away the essence of what makes him .. him.

You Keep Surprising Me

17 years ago today I was scare of the outcome of giving birth to Kody.  He had been trying to be born since I was 28 week pregnant and here it was 5 weeks and 5 days until my due date and my water broke.  You would think he was in a real hurry for something and that he would have been born quickly but he waited almost a full 24 hours before making his entrance into the world.  Thank God for epidurals!  There was a time when I did not think he would be with us at this point in his life.  Doctors have given us timelines due to the FRDA and he has lived past them all.  So I guess doctors are not always correct and they are just giving an educated estimation.

Yesterday Will took the kids to see Iron Man 3 and to have an early dinner at Applebees.  Unfortunately I was unable to attend due to an injury.  Kody was upset with me for not coming but later on after he and I chatted he understood why.  

Happy Birthday Kody!!

April's Halter Monitor

Today we went to St. Joseph's to have a halter monitor place.  McKenna wants to have another one to compare this last one too that was done about a month ago.  He will call me with updates on results within about a week or so after the monitor is sent back into him.  I used to hold my breath with things like this BUT I have come to learn that stressing myself out does nothing good for any of us.  So until we have the results .... it is what it is.

Yes Another Halter Monitor

On Friday (4/12/13) we will travel back to St. Joseph's Hospital in Tampa for Kody to have another halter monitor placed to give McKenna another recording to compare the last one to.  We also have to have his camp physical form filled out by McKenna due to the level of heart damage he has.  This is to okay him to be stable enough to attend camp this summer.  This will be he last year attending MDA camp because he will have aged out.  We are also looking at the options for school for him next year.  Do we continue with Hospital Homebound or consider virtual classes?  There is a few things to consider since he is getting older.  I cannot believe he will be 17 next month.  I remember when the cardiologist said they would be surprised if he seen 16 and he blew right past 16 with ease.  Now onto the next milestones ... and paths .... God will take the wheel and show us what is next.  Just have to trust in him and cherish the time we have .....

Dr McKenna Called About Holter Monitor Results

First off, I have learned when the doctor himself is calling you that the news is not what you want to hear.  Secondly, I was right in the first sentence.  His 24-hr monitor showed more atrial fibrillation and tachycardia.  McKenna suggested we start him on a small dose of Atenenol (beta blocker) that he said may or may not work.  This is something we tried before and the medication made him sick, vomit, faint, and blood pressure drop.  So, we will not be putting him back on that medication.  I could hear the concern in his voice as he was talking to me.  It is an unmistakable sound in voices when they are concerned.  I had figured there was not much change because I hadn't heard from him about the monitor and it has been a little while.   

Seems when we think things have leveled out with Kody .... things change for the worst.  At the moment I am still working to process this new information .... not sure how to feel about it all.  We just came back from an amazing vacation and then I get a call from his doctor that is just like wow.  

((HUGS))

Kody Champagne: Cardiology Visit

Doctors Visits Tend To Make Me Nervous

Yes, I admit it that they make me completely nervous.  I think more so because this is a CARDIOLOGY visit so it will give us a look at how the Hypertrophic Cardiomyopathy is doing.  It has been about six months since he seen doctor McKenna.  The one thing that I do know is with every appointment there has been progression, even if it only a little bit.  I will post a video blog tomorrow on everything since today is going to be crazy busy. 

Kody Update 1/2013

Kody updates