February is National Heart Month

Feb 3  2016
February is National Heart Month. I like to say it is Heart Defect Awareness month, but we really only get one week.
I have 3 heart defects, 2 defective lungs, Hemi-vertebrae=Scoliosis, and some other issues. For more information on those go to my very first post, it is MY story.
I have a Patent Ductus Arteriosis, Hypoplasia left Pulmonary Artery and Vein. 
I have a PA and PV, but they are so small, even the dr says absent. Because of the PDA, i have High Pressure in the Heart and Lungs, this is Pulmonary Arterial Hypertension. My PAH, is a more severe form, and I was diagnosed at age 10, Eisenmenger Syndrome. This is because the PDA has Bi-Directional Shunting. (Good and Bad blood mix) This sends Un-oxygenated Blood to my legs and feet. So i tire easily. AND most of the time i have Periwinkle colored toes. This is why Periwinkle is the "Color" for PH.
I also have Pulmonary Hypertension because of the Restrictive Lung Disease. This is very Rare to have both heart and lung PH.
I am on Tracleer for the PH, it is $10,000 a month. I was started on it in 2010. I feel i have reached a Plateau, but Doc doesn't feel it is time for a secondary drug. He says, the other meds have not been tested with Eisenmenger patients. He wants me to let him know when i feel i am declining, and he will consider it.
SIGH!! How do you feel and know you are declining?
It is so relative?

Love diane
#Cardiology, #CongenitalHeartDefects, #EisenmengerSyndrome, #NationalHeartAwarenessMonth, #Patent DuctusArteriosus, #Pulmonary Arterial Hypertension
#1in100, #CHDAwareness,

National Heart Awareness Month, Or Heart Defect Awareness Month

February 2 2015
Hello, It is National Heart Awareness Month, Or Heart Defects Awareness Month as I like to put it.

Some Heart facts/information::  I read, and share into several Support Groups, articles on Heart Defects, and Pulmonary Hypertension, almost every day. Here are some of  those selections.

I have an Interest List "Heart" set up with over 300+ Groups, Hospital Systems, Organizations, Non-Profits, all heart related. These Facebook pages adds into my feeds, and takes forever to scroll through. I also have an abbreviated one, I use most of the time. (I need to downsize it, but can't choose between deleting Rare Diseases, or Genetics)

""Cardiac disease patients bare scars for Kelvingrove exhibition
Friday 30 January 2015
SURVIVORS of congenital cardiac disease are baring their scars in a photo exhibition to raise awareness of heart conditions.
The Scarred FOR Life project will see a number of photos by Herald photographer Kirsty Anderson go on display at the Kelvingrove Art Gallery & Museum in Glasgow for a month from February 13....
Ms Wilson, who had open heart surgery in her early 30s, said the exhibition was being organised to raise awareness of congenital heart disease, which affects one in 125 babies....""
http://www.heraldscotland.com/news/home-news/cardiac-disease-patients-bare-scars-for-kelvingrove-exhibition.117378619

The Pulmonary Hypertension Association has a number of Webinars if you have PAH-CHD.
http://www.phassociation.org/Classroom/Landing/Recordings

AHA Journals Include: Circulation, Cardiovascular Imaging, and others. But the AHA do mostly Non Congenital Heart Defect Research. In other words, it is mostly Coronary Heart Disease. The Journals come out, at least once a month.
http://circ.ahajournals.org/
Then there are articles I wish I could read, but need to sign in to.
Achievements in Congenital Heart Defect Surgery: A Prospective, 40 Year Study of 7038 Patients
http://circ.ahajournals.org/content/early/2014/12/23/CIRCULATIONAHA.114.012033

The American Thoracic Society puts out a Journal, Chest, which I try to read regularly.
http://www.atsjournals.org/toc/ajrccm/current
https://www.facebook.com/americanthoracic

Hospital systems post articles about Patient stories.
http://intheloop.mayoclinic.org/discussion/courtney-kidds-long-road-to-a-normal-life/?linkId=12067626

Also reading the feeds for these 2 medical sites, every day.
http://www.medscape.com/
http://www.medpagetoday.com/

and trying to decide on entering this contest? They said, you don't have to have had Open Heart Surgery, just Heart Defects.
https://www.facebook.com/MendedLittleHeartsNationalOrganization/app_515720611858523

Thank You love diane

#Cardiology, #Congenital Heart Defects, #Heart Defects, #PatentDuctusArteriosus, #NationalHeartAwarenessMonth,

Pulmonary Arterial Hypertension #worldphday

My  other blog has some more information
http://dianehearthearts.wordpress.com/2014/05/04/pha-worldphday/?preview=true&preview_id=254&preview_nonce=2ca97d3df6
Scroll down to the beginning, for my story

Tomorrow, May 5 is World PH Day!! (PH= Pulmonary Hypertension)
here are some places to get additional information
@WorldPHDay,
http://respiratorydecade.blogspot.com/2014/05/world-pulmonary-hypertension-day-2014.html

http://www.phassociation.org/
Pulmonary Hypertension Association

http://www.phaonlineuniv.org/
http://www.phcentral.org/
Pulmonary Hypertension Association of Canada
http://www.phacanada.ca/index.php/en
Pulmonary Hypertension news
http://pulmonaryhypertensionnews.com/
Creating Awareness of PH in South Africa
http://www.phsa.co.za/runtime/popcontentrun.aspx?pageidref=2729
http://www.phaeurope.org/

National Superhero Day #ytcHero

National Superhero Day  #ytcHero
April 28 2014
 Love this quote, but there are several variations.
"The object of lifes journey is not to arrive at the grave safely in a well preserved body, but rather to skid in sideways, totally worn out, shouting, Holy Sh**, What a Ride!!!" — Mavis Leyrer, Seattle
also
""Life should NOT be a journey to the grave with the intention of
arriving safely in an attractive and well preserved body, but rather to
skid in sideways, Champagne in one hand - strawberries in the
other, body thoroughly used up, totally worn out and screaming
'Holy sh** - What a Ride!'" — Mavis Leyrer (octogenarian)

“Life should not be a journey to the grave with the intention of arriving safely in a pretty and well preserved body, but rather to skid in broadside in a cloud of smoke, thoroughly used up, totally worn out, and loudly proclaiming “Wow! What a Ride!’”
It was said by the man — Hunter S. Thompson 1966
whichever??

“We've got some difficult days ahead. But it doesn't matter with me now. Because I've been to the mountaintop. And I don't mind. Like anybody, I would like to live a long life. Longevity has its place. But I'm not concerned about that now. I just want to do God's will.” ~Martin Luther King, Jr

My Superhero pose!!

A friend came to visit and took my for a ride on their bike. Had a Great time!!
Friends help make a difficult journey easier!!

love dek
#CongentialHeartDefects,#MultipleCongenitalAnomalies,

Diane and her Heart Defect Research Quilt

Hello, March 14, Friday.
Wednesday I sent off the quilt to the winner, Mary D. I am sure it is going to a good home. :(
Sniff Sniff, missing it.  :)
I had Ma take one last picture, so you could see what it looks like finished, and a rod in the Rod pocket. But she isn't use to the new fangled cameras and chopped my head half off.
She hasn't taken many pictures since the days of her Brownie.

Diane and the Heart Defect Research Quilt

I have already heard from both organizations, ( CHP's foundation, and UPMC's Development office, for CVI)  and as soon as the monies clear out from Go Fund Me's  We Pay account, i will send off checks to both groups. My $1020, when withdrawn, minus the fees, came to $930.78. Fees than were $89.22,   8.74%. Which is about what they have in the FAQ's.
THANK YOU all for the Support, Donations,  and Love !!
Who knows, if able, maybe I will do it again in 2 years. BUT forget about those 89 little pieces, something a bit easier.

Love Diane

PS 
Plus, Cash on hand, checks, I never added into the G F M, in case they took fees out on it too. $121.
$1051.78  total
But WOW !! THANK YOU AGAIN!!

Heart Defect Research Quilt Mar 5 2014

Hello, March 5, 2014
WOW!! When I started the Heart Defect Research Fund, I expected it would really be like a "raffle", 100 people might give $5 or $10. But for Family, and Friends to gift more, WOW!! I am truly Blessed!!
I am almost to the goal of $1,000, in under a month.
After someone told me, they withdrew some of their monies in their account, of the $400, the amount in the checking account only was $364. The funding source, admits to a 5% fee, plus 2.9 % and $ .30 processing fees, but it was in reality, more like 12% fee. I haven't inputted the Cash on hand, not sure if it would be charged fees also. So I am in reality over my goal amount.
I have emailed both places for information on gifting, and haven't heard back. :(
I need to resend those emails.

The Quilt is almost complete. I had issues with how the binding turned under, over the Rod Pocket, and ripped it out, and resewed it, But I have the hand sewing half done.

WA LA, Rod pocket sticking out on the top, and Binding all stitched on.

The Label I stitched out with the machine, I thought came out well.
I need to hand stitch down the binding and rod pocket.

label stitched out with machine

THANK YOU ALL WHO HAVE SUPPORTED ME!!
http://www.gofundme.com/6psk18
WOW!!!
IT IS OVER!!
Love Diane

Heart Defect Research Quilt

Hello, Feb 26 2014
I think the  FEATHERED HEART APPLIQUE QUILT  is coming along very well. I finished the free-hand quilting, AND the Stitch in the ditch around the blocks. All that is left is the binding and the rod pocket. When there is a quilt show, it is usually required to have a 5" pocket in the back, for a way to hang the quilts. I don't think whoever wins this, will show it, so I am only going to put in a small pocket, for a thin rod, like a small wooden dowel rod.
After I hit the bottom with the Echo-stitching, I still had lots of room in the corners. Normally there would be stipple stitching in there, but my stipple stitching isn't very good. But then the Echo-stitching isn't so great either. As I go out from the center, my points and curved scallops got straighter and less loopy.
I did a small free- hand quilting practice run and told mom I was going to do that in the corners, around small hearts I was going to free-hand quilt. She said I shouldn't, Leave it be. BUT I did it any way. It came out pretty well. I only had to rip out one small part, when I messed up. That wasn't the first time, so no biggie.
So in the corners of the large big center block, I chalk marked around a small heart, stitched around it, plus 2 more times. Then I stitched off of it, and did some loops and did some heart loops. So look close at the free -hand stitching in the corners. I know, the lighting isn't very good, and the flash over exposing it.

Check out the corner where I did some hearts and heart loops.

After i took this, i finished the stitch in the ditch around each block.

I may still do a small satin stitched heart in the middle of each small heart applique, I haven't decided on that yet. Technically you are supposed to quilt to 5". This is from the old days when the batting would disintegrate, so you want stitching to keep it together, and in place.

Love ya diane

Heart Defect Research Quilt Feb 19 2014

Hello, Feb 19 2014
I worked hard on the Quilt today. I stitched around all of the 89 little pieces.
Sorry I took 4 pictures, 2 with the flash, and two without, none are really that good. I had hoped at least one would show the stitching a bit better.

Quilting around all the 89 little pieces

Maybe  if you click on it, and make it full size. It is AWESOME!!
The picture from the front, you can see the puffy ness around each piece.
Tomorrow and Friday I will work on the echo- stitching around the shape of the heart.

Heart Defect Research Quilt

Don't forget if you donate to my Heart Defect Research Fund, you have a chance on winning the quilt.
http://www.gofundme.com/6psk18

Thank You
love diane

Hello, Feb 13 2014,
Heart Defect Awareness Week is almost finished. St Valentine's Day is tomorrow.
We were supposed to get our Groceries today, but the weather didn't cooperate.
So I spent the time working on the Quilt. First, I taped down the Backing, then the Batting, and then the Quilt top.
Then I Pin Basted the Quilt. You are supposed to PIN it about every 5" apart. But I was worried about holes showing in the center square.  I then had to move the other Pin Basted Quilt over to the pile of TO DO Items.
Monday I will start the Stitching of the layers together. Free-Hand Quilting.

Pin Basted

Sorry it wasn't the best shot. I couldn't lift the camera up any higher. ::)
Love ya
Don't forget if you donate to my Heart Defect Research fundraiser this can be yours.
http://www.gofundme.com/6psk18
THANK YOU!!
Diane

Hello  Feb 12 2014
I posted this yesterday as a PS on the other days post.::
Well, the backing part today wasn't going  so well. Plan C. Pictures later. Pooped out.
This is who i will contact about the donation.
https://givetochildrens.org/Ways-to-Give/Direct-Your-Gift/Research
THANK YOU !!

TODAY ::
I ended up going with PLAN E. This is what I was going with yesterday, and it wasn't working. I guess, my mind was not functioning in QUILT mode. As I laid there this morning, UH HA, a Revelation came to me.

DUH!!

I was thinking, NOT Clearly, I would sew the 2 sections together, and then do a Diagonal in the middle. I got to that point, and was looking at the 4 seam allowances, and it was   NOT GOING TO WORK,  at least, not for me.
The quilt turned out a bit bigger than planned, 35 by 35.5, not 32.
The pieces I had, were either Fat quarters ( 18 by 22), Half yards ( 18 by 44) or full yards. The ones I had that were full yards, were the Joann Fabrics ones, and one that was mostly white with no hearts on it. The ones from Joann's, were a bit BUSY, but I had to use them. I also had one from Joann's that said, Faith, Hope, and Charity on it, I think it was a BC one. But at least it had hearts !! ( needed lots for the feathered heart, I didn't want the same pieces too close together)

The bigger ones I cut a 20 by 20 and the smaller one I cut 18 by 18, and, well, no good.
This morning, I took the large Moda Marbles Dark Hot Pink, it was barely 34 by 40. I only needed a little more. Yesterday, it got to the point, I DID NOT want to piece the backing, Especially down the center, and have it behind the large Feathered heart. Or only add a small 3" piece onto one side, and look lopsided. SO  I cut 3 -2.5 " strips to use in the border, off of the Hot Pink. Then got JACK ( the ripper) and took apart the seams on half of the above. Chopped off 2- 5" strips and sewed those to each side of the large Hot Pink, now about 28 by 37.
WALA!!

Backing of the quilt !!

WHEW!! By then it was 1pm, ( Home alone) and pooped.
Nappy time, tomorrow or the next day, I will work on pinning the layers together.

http://www.gofundme.com/6psk18

Love DEK

Hello   Feb 10 2014
Well the Heart Defect Quilt Fundraiser is going slow. I did complete the quilt top. Rather then go up to the quilt store, AGAIN, i used as the sashing, the fabrics that I had. It is a little busier than I had planned, but is still BEAUTIFUL!!

HEART DEFECT QUILT

Tomorrow I need to cut 4 pieces to sew together into the back panel, 19 by 19.
It is about 35"  by 35. 1/4".
And find where I put the white cotten batting.
There are a few surprises in it. LOL!! ♥
Just hoping i can manage the feathering in the free-hand quilting.
Keep your fingers crossed for me.

Heart Defect Quilt Fundraiser.
http://www.gofundme.com/6psk18

Love Ya Diane

#CHDAWARENESS
#CONGENITALHEARTDEFECTAWARENESS

In order to find CHD's we are advocating Pulse Oximetry testing of all Newborns. It won't find all, but most. This is SOO important. The Doctors need to find out if a baby has a CHD before it is discharged, so treatment can begin immediately.
http://www.huffingtonpost.com/nancy-brown/heart-defects-babies_b_4737818.html


Story in the local paper about a boy who never knew he had a CHD. He went for an medical exam, and dr heard a Murmur.
http://triblive.com/neighborhoods/yourallekiskivalley/yourallekiskivalleymore/5457115-87/heart-gregory-guzzo#axzz2rutW2Obq

Children's Heart Foundation is doing great work funding research.
http://www.childrensheartfoundation.org/

AHA does some CHD research, but the bulk is adult Heart Disease, not Defect research.
American Heart Association information on CHD
http://www.heart.org/HEARTORG/Conditions/More/CardiovascularConditionsofChildhood/Commonly-Asked-Questions-About-Children-and-Heart-Disease_UCM_311917_Article.jsp

Congenital Heart Information Network
http://tchin.org/

Adult Congenital Heart Association
http://www.achaheart.org/

Mended Little Hearts Local is Southwestern Pennsylvania
http://www.swpa.mendedlittlehearts.net/

The other item we are ADVOCATING for is the ECG Screening for students going into sports.
Next time i will try to add some links for friends i have that are doing screenings, raising funds to do more across the US.

diane

Hello
Feb 3 2014
Ma and Pa were away to their Class reunion get together. SO home alone, I worked hard on the Heart Defect Awareness Quilt center, the Feathered Heart. WHEW!!  Very difficult and forgot about the main thing with appliques, you gotta turn it upside down, so when you trace it and then stick it to the background it is correct side up or orientation. So the numbered pieces were not on the correct side. I had to WING it big.
I had my desk lamp under the kitchen table and opened up the center section for the leaf, that way I could see the Heart outlines through the pink fabric.

Heart outline with the table center opened up

That big thingy on the right is Ma's fryer she bought. $$.

89 little pieces. which didn't go together as well as i had hoped

The little pieces didn't go together that well, there was some Pink showing through. But when I stitch around them all, hopefully all you see is the stitching. Of course, my free -hand outline stitching is SOSO.


Will do my best, that is all I can do.

This is the fundraising page, please support me.
http://www.gofundme.com/6psk18

Thank you.
Love diane

Heart Defect Research Fund Raising Quilt

Jan 26 2014
Heart Quilt
Ever since realizing that they were doing Catheterizations as early as 1958, but my doctors didn't do mine until i was 10, In 1967,  I have wanted to raise funds for heart defect research.
Well, even before that.
Why did they wait? Because of all my issues? Hemi-vertebrae scoliosis, Absent upper left lung lobe, Hypoplasia lower lung lobe, Patent Ductus Arteriosus (PDA), Hypoplasia left Pulmonary Artery and vein.
Officially
hypoplasia /hy·po·pla·sia/ (-pla´zhah) incomplete development or underdevelopment of an organ or tissue.hypoplas´tic   from The Free Dictionary

So I started ANOTHER QUILT,  Do I have unfinished projects?  SURE!!
But this was going to be all  BRIGHT  pink or red fabrics that had HEARTS on it, only I couldn't find enough fabrics. I guess, St Valentine's Day is the season, and I was looking in October, which is when I can get to one or two quilt stores.  (Birthday month)
As I worked on the heart, I decided I needed more. I managed to find some and order online more pinks and some had hearts on them, from a local store. January was SOOO cold, I wasn't going out much. I don't want to get sick, so if it is below 30, NOT going out. Then they rescheduled my Heart dr appt twice, and HAD to go out when it was 8 out there. Thankfully the high was 34, and it warmed up later, after I was home. ;(

I had found a GREAT idea in a Quilt Magazine a few years ago, a feathered heart. Only 89 pieces, TINY pieces.
But very Beautiful.
So going to post the Jpegs I did, i hope I don't get into trouble:

Beautiful!

It is supposed to be a shadow-work type applique. But just want the main layer with all kinds of PINKS, since I couldn't find very many reds.
But ended up using a few of the reds, because I needed more variety. I didn't want the same fabrics to be too close together.

89 little pieces

HMM?? Can i do this?

All marked out on the steam a seam

I tried to put all the ones for the same fabric together, but as I was going around, the pieces for the same fabric are going to be too close together. I found I NEEDED more fabrics, the more the merrier. 
Utilizing 7 fabrics,  pieces  1-8-15-22-29-36-43-50-57-64-71-78-85

I tried several different ways, using programs in the computer and on Mom's Computer, but i finally went to the market and used their copy machine. The Heart base design needed to be enlarged 200%.

Layout of heart applique enlarged 200%

What am i going to do in the corners? Possibly paper piece some hearts?

I wanted to be done before i started the fundraising site, BUT not going to be near done.
About 30" By 30".
Can i raise  $1,000?
$500 each for 2 research projects?

I started the fundraising here. Please support me.
http://www.gofundme.com/6psk18

TRYING!!
Love dek

What would the difference be??

Sept 30 2013
Through the years, i have slowly been finding out more and more about ME.
Once in doing a search i found this list::
http://www.nlm.nih.gov/archive/20061212/mesh/jablonski/syndrome_toc/toc_a.html
Multiple congenital anomalies. WOW!! SO many?
Why not a list without those with Mental retardation syndromes too?
Recently, in doing some research i found another type of list, Multiple Chronic Conditions!
http://cmcd.sph.umich.edu/managing-multiple-chronic-conditions.html
WHAT WOULD THE DIFFERENCE be between the two??
Congenital VS Acquired?
This has some ideas::
 http://www.hhs.gov/ash/initiatives/mcc/index.html
"pose a significant and increasing burden on the health of Americans"

"" Chronic conditions are conditions that last a year or more and require ongoing medical attention and/or limit activities of daily living.^3""

^{"concurrent chronic conditions""
Well, they both sound VERY alike to me!
Ok, Diabetes, Cancer, Alzheimer's, Arthritis, Osteoporosis, Heart Disease for a few.
As opposed to, Heart Defects, Lung Defects, Osteopenia, Pulmonary Arterial Hypertension, Hemi-Vertebrae Scoliosis.
Drugs that are $6,000 a month.
HMMM??
I was told
"}The difference is that "chronic" conditions may exist at the time of birth but they may also acquired later in life. So, a congenital condition may be considered a chronic disease but not all chronic diseases are congenital.^{" 
SMH!
Sounds to me like, More of them, so they are funded.
Hospital systems should have Departments for those with MCA! The PCP has a hard time keeping up with ALL of our issues.}

More About Me

Diane’s  Story ::

My mama told me I wasn’t a blue baby, but I always think of myself as a neon aqua blue. At 6 months of age, my pediatrician suggested I go to Children's Hospital of Pittsburgh for Cardiology and Orthopedic issues.  The Pediatrician must have been able to hear something amiss in the heart, and see the spine curving.  CHP Cardiologists told my parents, they could or would not do a Cardiac Catheterization until I was 10 years old, at which time they were going to fix the Patent Ductus Arteriosus.  At 10, in 1967,  they were all ready to fix it, but when they did a Catheterization they found the internal heart pressure was high, ie: Eisenmenger Syndrome a more Severe form of Pulmonary Arterial Hypertension. They then knew there were other issues. In poking around, they said I had an Absent Left Pulmonary Artery, or one that was possibly very tiny.  It wasn't until years later, in a Cardiac MRI, that it was decided, I did have the artery, but it and the Left Pulmonary Vein were Hypoplastia, (teeny tiny). Doctor said, they were as big as a Pen tip, as he scribbed on a piece of paper. It was decided to leave the PDA alone, they felt the PDA would act as a back flow pressure relief valve, or pop up valve, in effect keeping me alive. 
I recently saw an Adult Congenital Heart Doctor, he got out of Iron Mountain my 1967 Cath report. It stated they did an AngioGram. WOW! They found 2 Arterial Branches off the Descending Aorta, and they supply blood to the Lower Lung Lobe.
What REALLY bothers me is they suspected in 1958 the Left Lung was Hypoplasia, but NEVER bothered to tell Ma or Me when I was old enough. They mention that the heart is shifted over to the Left side, and that the PDA was BiDirectional, mostly shunting blood right to left. ( this is Eisenmenger Syndrome)  This is why the doctor now says, Oxygen Therapy won't help, the un-oxygenated blood shunts to the legs and toes. I always show the doctors my toes, because they are Periwinkle.

AND ::

"MAIN PULMONARY ARTERY PRESSURE WAS 93/48 AND THAT OF DESCENDING AORTA WAS 94/47"
SO even then the Internal Pressure or PAH was HIGH. But all they ever said was I had Eisenmenger, never the PAH part. At one point, years ago, when we asked about the Eisenmenger, the dr said, not to worry, it only means high pressure in the heart. 

Growing up, I was the only girl who did the boys push ups, ( with 4 brothers and 1 sister that was what I knew)  I did not run fast or play sports, i tired fast. I was the last chosen for dodge ball, or the first chosen for the OTHER team. In high school, I was excused from gym class.  While in grade school, the school district  offered a family swim.  I was sent home early because my lips would turn blue. I did do a little “sports” with the family, like going after the Tennis balls that escaped the fence, Ping pong, and wiffle ball out in the back yard.  I was more into chess, puzzles, and always helped my mom in the school library. It wasn’t so much I was told NOT to do things, but I couldn’t, I never had much energy. Of course then there was MAMA, yelling to GET DOWN OUT OF THAT TREE, when I tried to climb one. ( with 3 younger brothers staring up at their BIG sis ) So ok it was only about 3” in diameter, my Dad having trimmed off any lower branches off the BIG trees.

When I was 12, they decided to fix the scoliosis, which was because of a hemi-vertebrae, missing the left half. ( Everything affected is on the left side)   Leaving the 6^th grade early, in the middle of April, I was hospitalized until the beginning of July.  The school had a surprise Going Away party for me, with all the 6^th grade girls. I was given 30 presents, to open - ONE A DAY. Mom would come every couple of days on the bus, and Great Aunt Margie would come from work, pick her up and take her home. Mom would bring a few when she would come visit.

Once they came in and needed blood work. I remember the Vampire sticking me at least a hundred times, then asking a nurse to hold me down, she wanted to try a leg vein. The Nurse refused, and told her to call another Vampire to come and try. The second one, she got a hit with only 3 tries.
After many tests, like Spinal tap, and I am sure there were breathing tests, there came the day the doctor took me over to the Boys ward and showed me a young boy in the Halo-Traction. I got a bit upset, and we had to call Mom to try to calm me.

The Orthopedic doctors decided to go with the Halo-Femoral Traction, rather than the Risser Jacket (which was a body cast from chin to pelvis with pressure pads ), because of the heart and lung issues.
I call the Halo-Femoral traction the Ancient Medieval Torture Treatment.  They put 4 pins into my skull and a halo, where they hooked weights, and then 2 pins, one into each Femur, just above the knee, which was then connected to weights.  After weeks of this “straightening”, I was as straight as they could get, and balanced, they set about planning the surgery.
BUT  I caught a cold, at first they suspected allergies.  I got so bad they had to take the weights off so I could cough, and at one point, I needed to be resuscitated. I had to spend a week or so in an Oxygen tent. They sent me off to the other section, Metabolic Unit, for a few weeks.  In the end, they decided it was Acute Bronchitis from the cast dust, due to several new girls in the girls ward, needing casts.  After restarting up the weights, and spending more time in the traction, I eventually did have the surgery, where part of the spine was fused, and they inserted a Harrington rod, which is the size of a pencil.  The rod is connected to the spine with pins, screws and other lovely hardware.  

Finally in early July, I was discharged in a body cast, knee to chin, with a steel frame, encompassing the halo, and the skull pins.  I remember being told, I was the seventh person to undergo this procedure.  Several others had had to have their fusions redone, hence the need to stabilize the spine, immobilize the spine for 12 months.  The plan was for me to be in the big cast, pins and all, for 6 months.  But after four months, the lower legs started swelling, and I had to have the leg pins removed, and the lower few inches of the cast cut off.  I told the Doctors the upper thighs hurt, but they ignored me. Later, when they cut the big cast off, there were scars on the upper legs where my skin was pinched, when the legs had swelled. 
During that summer, I remember several of the girls from Bon AIr Grade School coming to visit, we played some board games, and cards. Relatives  brought lots of books for me to read, and Needle crafts to do.

In November,, the right forehead pin started sliding, when I could no longer see out of my right eye, we returned to CHP.   The Doctors took me out of the Body cast and pins. It was a month early, then planned, but I think, my dad was glad.  Because I had had that cold/Bronchitis, he had had to rotate me EVERY DAY, so I spent one hour on my stomach each day. At this time, I was put into a smaller cast.

In September, the school sent two teachers for my home bound tutoring. I had a Math teacher come twice a week, and an English teacher come 3 days. She taught me English 2 days, and Spelling one day. They had to change the Math Teacher after about a month, she couldn't make it up and down our hill. 

In December, two weeks before Christmas, I caught another cold.  Pediatrician came to the house, and said that I didn’t have enough room to COUGH, in the smaller cast. It was too tight across my chest.  Back to CHILDREN’S, with Acute Bronchitis, and weeks in the O2 tent.  This time I spent about a month in the clink.  The local Lions club sent me a gift, thanks to a school teacher member, he heard i was on the clink. I also received a few from other organizations. When I was discharged, they built the cast with a lot of room up front, the chest portion stuck out a few inches. Except for the monthly trips for X-rays, I didn't need to return.  In April, they put straps on one side of the cast, and hinges on the other.  AND I slowly relearned to WALK!!  WALK, WOW!  That summer I was slowly allowed to take the cast off, for larger increments of time, as my muscles strengthened. I couldn’t wait for the day when I was finally allowed to go out on an excursion, and leave the cast home alone.

I returned to school for my 8^th grade. The school district was concerned about me being bumped during the room changes, so I was allowed to leave each class early, to allow time to get to the next classroom.  I was also allowed to have 2 books for each class, one for use in the classroom, and one to use at home.  This saved me from having to lug all the books around.  Every school year I would get terrible colds, and  would be absent from school for about 2 weeks.

At 16, I started seeing the Orthopedic Doctor at his office, rather than the Children’s Hospital Orthopedic clinic, and stopped going when I figured he retired.  When I tried to see a Scoliosis Specialist about 9 years ago, I found out my old doctors office had pitched my files.  Never knew there was some law, if you haven’t been in to see the doctor for 7 years, they HAVE to pitch your files. I asked them, WHY DIDN'T YOU CALL ME, there had to have been several boxes of stuff on ME, with all the X-Rays.  But they said, we would have to call hundreds of people. I don’t understand though, someone with a simple bone breakage, with a skinny file, being equal to a lifetime of files.  (or at least 29 years)

My Mother has approximately 3,000 books. While in grade school she worked as a Building aide, working in whichever grade school building needed assistance. On the occasions she was needed at the  Bon Air Library, I would help her. At some point through the years, she was hired as the High School Librarian aide, assisting in adding, sorting, maintaining, and inventorying the books. In Junior and Senior high I always volunteered in the library. In College, I didn’t have much extra time, but after graduation I did start to volunteer in the local People’s Library, and volunteered more than 20 years. Mom was on staff then, and I helped card, file books, and started their Revoked Privilege list. This was the people who refused to return the books they had signed out.  I also assisted in the book sale. For months in advance of the sale, I was in charge of sorting, storage, stacking, and filing, of the paperback books. During the book sale, I kept the sales tables organized, and restocked.

I had received a BVR Grant ( Bureau of Vocational Rehabilitation ) which paid for my Seton Hill College tuition, and room and board. Before my college senior year, the School BVR Counselor informed my mom, that due to her salary, in addition to my dads, I would not be eligible for the full grant for my senior year. She quit, in order for me to finish, and graduate. He had told Mom, in my High School Junior year, if I wanted to go to College, they could see that I did. I knew that I did, it helped in the planning ahead.

I still see the Cardiologists at Children’s Hospital; I use to see whoever was doing clinic, but have seen the same Doctor the last 22 years.  Once I asked him, if I was his oldest patient, he said, no, and each visit thereafter, he would tell me, I was not his oldest patient. But he hasn’t mentioned it lately.  They recently started an Adults Congenital Heart Defects Center, so I see him through that clinic. Plus, now they have an officially trained Adult CHD Doctor, I see him also.

In the year 2000, I started noticing when we would go to the local mall, whereas before I could walk around one store and go out to the main mall, and walk from one end to the other, and back again. BUT now I had to stop for rest breaks. I also explained to the Cardiologist, I had to take rest breaks sooner in climbing up our hill. But it wasn’t until, January of 2009, when the cardiologists intern asked him if he wanted a new Pulmonary Lung Function Test done.  When he hesitated, I asked about seeing a Pulmonologist. My Cardiologist said, he didn’t know any, and instead suggested I see a Pulmonary Hypertension expert. He set an appointment up with the head of the UPMC Presbyterian Hospital Pulmonary Hypertension Center.   

The expert there told me, that in the last 10 years there have been a lot of research, with currently 8 drugs approved for PAH, with 3 different classifications. When he told me, in order to get on any of the drugs for PAH, he would have to “drill into my Jugular with just a local, but he would be there to sweet talk me through it“,  I balked and said I would have to think about it.  After months of telling myself I should move up the appointment, in Feb 2010, I had two episodes of getting VERY out of breath with little exertion. I emailed about moving up the appointment, they called and gave me one within 2 weeks.  

April 1, 2010, I saw the PH expert and a Pulmonologist. I was also scheduled for a Pulmonary Lung Function Test, a Six Minute Walk test, and later the Pulmonologist ordered a Ct scan on my lungs. 
On April 12, 2010,  42 years after my first Catherization, I had my second Catherization.  They did a Right Heart Cath, by going down into the Jugular and poking around in the heart.  After him saying he would be there, he had his partner do it. Dr Rene told his assistant 3 times, call MIKE. Dr Mike finally came an hour and half afterwards. He told me, I had Pulmonary Arterial Hypertension, (formerly Secondary PH, PH secondary to CHD) and said my Hemodynamic pressure levels were very high, the highest he had ever seen. I am currently on PAH Drug therapy, Tracleer (Bosentin).  Tracleer can cause liver damage, and after one month, my liver functions were high, so I had to remain on a lower initial dosage.  They were checking the Liver Functions every 2 weeks. Normally, after the first month of Tracleer, on the lower initial dosage, you start on a higher dosage, but that was postponed until the levels remained within normal limits for one month. I have since started on the regular double dosage and the Liver functions have remained within normal limits.  I was SO glad when I no longer had to go in for the blood work every 2 weeks, and they returned to monthly. Me, who hates blood work, has to have tests every month.

On May 6 2010, I returned to see the Lung Doctor, to discuss the Ct scan.  "Seeing" my lungs was VERY interesting.  After 52 years of wondering, did the left one work, and thinking it was there, just didn't work so well. The CT scan showed the right one is larger, with the upper section being larger with an over extension, which mushed the heart to the left side. The Left Lung is barely a half lung, the upper Lobe of the lung is not there, and only a small portion of the bottom half gets air and works. On the monitor it looked like the size of a quarter. Officially, Hemi-thorax- Left Lung Lower Lobe- Hypoplasia, and Agenesis of  Upper Lung Lobe.  When I next saw the Children’s Hospital Cardiologist, I asked him if he had seen the Lung Ct Scan, he said, it didn’t show him anything he didn’t already know. I was stunned. He didn’t have an answer when I asked him why I had never been told about the lungs. I guess, with their clinic, I was going in each year and seeing a different doctor each time, each thought the other had explained it.  But I had been seeing him for almost 20 years. Plus, there is this mentality, I am a HEART doctor, can't discuss the lungs.

The Pulmonologist said both of the lungs showed signs of PAH. But with the way the PH works, there has to be a blood source to get the PAH into the left lung.  They had always told us, Absent Left Pulmonary Artery.  But the Children’s Hospital doctor’s report always said there was a Possible Hypo-plastic left PA.  But it has yet to be seen in an Xray or echogram. I have asked about an MRI or Ct scan, but have always been refused.  I am hoping to someday visualize the Blood source, or possible Hypo plastic left PA or ( the PAH Doctor said) sometimes capillaries can form instead.  It wasn't until August 2013, that I had a Cardiac MRI. Doctor report said  Small Left PA, and PV.  But from the images Isee what the 1967 Cath report called, 2 Arterial Branches off the Descending Aorta. One could be the PulmonaryArtery and the P Vein, he mentions in the report.  PDA is a large 1.1 cm.

They have also refused me when I have asked about a left arm Ultrasound or Ct Scan. The left arm is about a half inch smaller, the thumb didn’t  form correctly, and doesn’t bend, ( no knuckle); and there is no pulse at the wrist. I explain it by saying, a messed up Vascular system.  If the pulse is from the radial artery, it is either absent or also Hypoplastic.  The nurses can get a pulse higher up the arm, and the Blood pressure is faint.
I had hoped the MRI would include what went from the Heart to the Left arm, but no.

Before the Tracleer, I wasn’t able to walk very far after eating, Doctor explained it by saying, I couldn’t multi-task. IE: I couldn’t digest my food, and exercise.  Heart needs to digest the food, and can only work on one thing at a time. Within 3 weeks of starting Tracleer, I wasn’t as tired, and was able to walk further, even after eating, though I still need a nap in the afternoon.  And when I over do it, I can still get exhausted, it is like I hit a brick wall, and need to sit down NOW!  I have never fainted, only a couple of times i have gotten a wave like of dizzy ness.

When I returned to the PH Center in July, 2010, I was able to walk further in the 6 minute walk test. The doctor insisted it was from the meds, NOT because this time it was at 9 am, not 1 pm. AND before this test I hadn’t had to Hike from Falk lung center up the hill to Presby. ( there is a internal bridge connecting the buildings)   In Jan 2011, I was able to walk exactly the same distance as in July, even though the test was scheduled for 1 pm. I told them I am getting tired after I eat, they don’t seem to understand this. They just like to see that the patient has an increase in the distance walked.

He did impart some good news, this PH center doesn’t feel it is necessary for a Cath every year. ( YIPEE) BUT, if he feels I need a secondary drug therapy, he has to check those Hemodynamic Pressure levels. Since then, he has told me, It would be tooo invasive, and he would not do a cath on me.

I would love to know, in a fetal growth timeline, was my non-existent left thumb knuckle ( malformed thumb ); possible missing left radial artery ( no pulse in left wrist);  Left arm  1/2” smaller; Hypo-plastic Left Lung; ALL  forming at the same time as the Hypoplastia left Pulmonary Artery/Vein and left half of the Hemi-vertebrae that never formed??? Did something Zap me???  
But more then likely each formed, or malformed at their own individual times.
In the reports i have read on VACTERL, Dr Ben Solomon has mentioned it is possible it is from a Genetic Malformation, Mutation, Or a Domino Effect.  
Did the Hemi-Vertebrea caused Scoliosis, cause the Lungs to split funny? ( I read somewhere they start as a whole, and split 3 lobes on the right, 2 lobes on the left. BUT mine split 3.5 on the rt and less than .5 on the left)
Because there is no Upper Lung Lobe, ( I read the Pulmonary Artery forms from the Lung TO the heart. ) So with no Upper Lung Lobe no Left PA formed?
Since then, i have read that Lungs can Hyper Inflat? So they wouldn't have split funny, but the right one would have grown larger on its own. AND There is a Left PA, just a teeny tiny one.
Because of the Scoliosis, the Left Kidney got squished down into the Pelvic region?  When i was having some issues, i had a Pelvic Ultrasound and was told the Left Kidney was above the Ovary.
BUT this really doesn't explain the Left Arm issues.
I use to want a WHOLE body MRI to see if there were any other issues, on the left side. But after the Heart MRI  I DO NOT WANT ANOTHER ONE!! I HATED it. No i am NOT claustrophobic, just hated not moving for over 2 hours. It took me 5 minutes to try to sit up after it. Legs kept twitching, the restless leg part just can't stay still for long. My back was aching.
I keep busy.  I have made about 25 quilts, mostly small, but several large ones. Several were sister projects, and reside with her. I won a blue ribbon at the Butler Quilt show in 2007, with my first Junglesong Rainbow Quilt. A People’s Choice award at the Tarentum Library Quilt show in 2005, with my Memory Quilt, i did for the folk's 50th Wedding Anniversary.  The Second JungleSong Quilt I had entered in the Butler show, the judge said, I needed more work on the Piecework. ( Because it was made out of flannel, it gave me problems, and would stretch. This caused the seams cross hairs to not match.) But it did win a prize at the Tarentum Library Quilt Show in 2012.

I helped with the Class reunion, doing most of the computer work that was necessary, helping to look up  current address of the classmates.  I scanned in and uploaded some of the class's yearbook pages, and pictures from the reunion to my Facebook page, group page, and the Microsoft Live Page I started for the Class. I send out occasional emails when a classmate passes, or is in the news.
I have two blogs, one for the medical and reference information, and one for my quilts. I have 2- Microsoft Live Space, with Skydrive storage space. I love working on the family tree and researching Genealogy information, and am working on uploading that information into the Skydrive, for any unknown family members to find .

In 2009 I attended a Pulmonary Hypertension Association, On the Road, conference, and have joined several PH Support groups  on Facebook.  I have also joined several Congenital Heart Defect support groups, and working to push for the Mandatory Pulse Ox Testing of newborns across the country. Also looking at how to spread the word, on the need for ECG testing of school students going out for sports. There is a need for increased funding for the NIH ( National Institute of Health ) and the CDC ( Centers for Disease Control and Prevention), for more research into CHD and Lung defects. Allegedly, AHA only gives pennies to the dollar for Heart Defect Research, but from what I have read, it is more like 8%, which is still not enough. 
Sometimes I think about starting a foundation and insuring the monies goes to Heart and Lung Research, but i don't think the SSI and Medicaid would like that. Besides, I don't know many people, who would donate?

DEK Updated  11/16/2014

Patent Ductus Arteriosus, Hypoplastia Left Pulmonary Artery and Vein,  Right lung - over extension, Opacification of the Left Lung, Hypo-plastic left Lower Lobe Lung, Agenesis Left Upper Lobe, No pulse at left  wrist-possible missing/ Hypoplastia left Radial Artery, Left thumb is malformed, smaller left arm - Ulna, Hemi-vertebrae- scoliosis. Pelvic-Cystic Left Kidney.

#CongenitalHeartDefects, #HeartDefects, #Scoliosis, #MultipleCongenitalAnomalies, #Lung Defects, #EisenmengerSyndrome, #PulmonaryArterial Hypertension, #PAH-CHD, #Cardiology, #PatentDuctusArteriousis, #LungHypoplasia,