Wednesday, August 24, 2011

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 ::
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 6th 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 6th 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 8th 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, 

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