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Hey,Lori, I think I could find a therapeutic reason for a trip to
Hawaii.Maybe 800 ft is too high an elevation for our echo. Do you
think I could talk Joey's cardiologist into recommending one in
Hawaii?
Seriously, though, I have a brother who lives outside of
Denver,Colorado. We used to hold family reunions in the Colorado
mountains every year. But we have not been to Colorado since Joey was
diagnosed.Here in the hills of Tennessee 2000-3000 feet qualifies as
a mountain, and us normal people can get a little short of breath in
Denver. I have been afraid to see what it might do to Joey.
Sounds like you're having some fun planning a trip.That way you can
have some fun regardless of how the echo comes out.
Jennifer, Bill's wife, mother of Joey, age 4

First of all I'd like to thank all those who replied publically and
personally to my email to the group. Your info has helped so much!!!
And cheered me up to know that I'm not alone.
New member!!
I've got some of your symptoms too but not so extreme. I do have the
pain behind my shoulder blade (left side) and the numbness on the foot
(left foot). Unfortunately the dr. seems to think it's all "muscle"
related. hmmmmm.
I'll be curious to see what they find out about you.
But the stuff going on with your arms sure does sound alot like
thoracic outlet...but I'm not an expert...
I found that massage therapy has helped the pain behind the shoulder
blade (which could also be coming from the thoracic) and a good back
massage seems to alleviate the foot numbness, but watch out, those
massage therapists are expensive.
Good luck!! Keep in touch.
Jan
New member
I've been having problems for close to a year now, and am currently
seeing a neurologist. Originally I woke up with shooting pains in my
right arm, then it went away. An emg showed "ulnar nerve entrapment at
the thoracic outlet" but the neurosurgeon said "I don't believe in
throacic outlet, have a nice life" and sent me on my way. A few months
later, my doctor put me on darvocet by day, and vicodin by night,
because i was in such pain. This time in BOTH arms.
I am seeing a neuro, who sent me for an mri of my brain and cervical
spine...both came out fine, though she made one comment on the spine
xray about "something there, but nothing to write hme about" - she
just did another emg, and found "something" in my left arm, and is now
sending me for a chest xray.
My symptoms are extreme fatigue in both arms, and the forearm turns
blotchy when it gets sore. My arms tingle, I lose my grip, and putting
my arms over my head hurts to high heaven cuz they fall asleep so
quickly. I've had a pain behind my right shoulder blade for a long
time, but she doesn't think that's related. The top of my right foot
is also numb, but she hasn't looked at that yet cuz she feels it's
probably unrelated and wants to figure out the big problem first.
I'm writing here cuz on the rx for the chest xray it says "dx,
thoracic outlet syndrome" - do my symptoms sound similar to any of
yours?
=====
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Hi Everyone:
I was just reading a lot of your e-mails about Make a Wish and Disney World. We have the opportunity to go with our daughter and Maddie G. ( our 3 year old granddaughter with PH) this fall. We are so excited. What a wonderful organization. Our Maddie is not on flolan but on many medications. She was diagnosed at Children's Hospital in Philly at 6 months of age. She is maintaining stability right now. Any more info you have on Disney World and what to do and see, please let me know. It is the first trip for all of us. God bless all of you wonderful people.
Fran

One of our old college buddies (Yea Sewanee is right!), is
into camping big time. George has a ice chest size
refrigerator freezer that runs off of 110 or 12 volts. This
is not like the coolers like you might see at wally
world but a real freezer. Not cheap, but priceless for
travelling with ice packs. ours was imported by
A R B . they come in three sizes.
when Joey was diagnosed, we bought a 4 wheel drive magazine,
and found an ad for a similar freezer, called the
manufacturer, found a dealer in Nashville and special ordered
it. (yes I did deduct it from my taxes as a medical
expense,) When we go on trips, this big freezer plugs
into a cigarette lighter, and keeps the reserve ice packs
cold. when you get to a hotel, you can bring it in, and
plug it into the wall.
On shorter trips fill a insulated lunchbox or small ice
chest with lots of extra icepacks in there, and you are
good for 24 hours or so experiment in advance, so you
know how long you are good for. (for best results you
want the cooler full of ice packs, I will fill up any
extra space with icepacks from the grocery store).
Bill Nelson

TOCS does not show up on a chest x-ray unless you were born with
an extra rib. If you have an extra rib that will show up. THAT
IS THE PROBLEM WITH TOCS -- ESP WITH REGARD TO WORK COMP THEY
WANT TO SEE SOMTHING TO PROVE YOU HAVE IT AND THE ONLY THING
THAT IS POSITIVE ARE THE TESTS THEY DO. LOSS OF PULSE WHEN ARM
RAISED OVER THE HEAD, LACK OF GRIP STRENGTH, PAIN IN THE NECK,
SHOULDERS, ARMS, AND HANDS. SOME PEOPLE DO HAVE NUMBNESS ALSO,
I NEVER HAD THAT ONLY PAIN PAIN PIAN.

=====
Cathy

I've been having problems for close to a year now, and am currently
seeing a neurologist. Originally I woke up with shooting pains in my
right arm, then it went away. An emg showed "ulnar nerve entrapment at
the thoracic outlet" but the neurosurgeon said "I don't believe in
throacic outlet, have a nice life" and sent me on my way. A few months
later, my doctor put me on darvocet by day, and vicodin by night,
because i was in such pain. This time in BOTH arms.
I am seeing a neuro, who sent me for an mri of my brain and cervical
spine...both came out fine, though she made one comment on the spine
xray about "something there, but nothing to write hme about" - she
just did another emg, and found "something" in my left arm, and is now
sending me for a chest xray.
My symptoms are extreme fatigue in both arms, and the forearm turns
blotchy when it gets sore. My arms tingle, I lose my grip, and putting
my arms over my head hurts to high heaven cuz they fall asleep so
quickly. I've had a pain behind my right shoulder blade for a long
time, but she doesn't think that's related. The top of my right foot
is also numb, but she hasn't looked at that yet cuz she feels it's
probably unrelated and wants to figure out the big problem first.
I'm writing here cuz on the rx for the chest xray it says "dx,
thoracic outlet syndrome" - do my symptoms sound similar to any of yours?

If you have TOCS, the surgery could be top rib removal or
scalenectomy. Since I was not a surgical candidate and was not
allowed to have surgery that was not an option for me...PT was
my only answer. I would suggest that you wait until you see
the Thoracic Surgeon to see what he advises; however, we have
group members who have had top rib removal who can tell you
about it. At this time you don't know what he will advise and
each case is unique in its own way. There is some PT that does
work which takes away the need for surgery. If any other group
members care to comment; please jump in. Welcome to the group.

My Orthopedic is sending me to a Cardiovascular & Thoracic Surgery
Dr. I Had a test done on my blood flow and it came back with a
problem on my left arm. I have done PT (did not help) what kind of
surgery can be done
RC

Hi Everyone,
This is a timely question as we just got back from Disney World yesterday. My daughter Anna is 7 years old and on Flolan and Tracleer. This is her second
time to Disney World and even though we were only there for 3 days she had
a great time. Since Anna is petite for her age a stroller works out well for her
at the parks. You can rent them for the day at the front gate and transfer that
rental to another park if you go on the same day. It seems we ended out at
Epcot for dinner every night. The kids really enjoy eating at all the different
countries. There is also space in the stroller to carry a bag or a small cooler
if you want to bring one with. We would not carry ice with us but head back to our room around 3:00 to take a break (mom and dad get exhausted) and change her
ice. The biggest drawback is that we were having so much fun the kids did
not get to sleep until about 11:00 at night. We are all recovering today. If you
have any specific questions regarding attractions feel free to ask. This trip we went to The Magic Kingdom, MGM and Universal. Have a wonderful time!
Laurie (Mom to Anna)

Luckily we had the International Balloon Fiesta here in Albuquerque this
past week . Alex and his brother came along with me to chase balloons and
help them land and take off . It's really neat to see hundreds of balloons
take off together . This year there was a little over 800 balloons . All
kinds of colors , shapes and sizes , we saw just about all of them . It's a
wonderful show .
Pat and Alex - NM

Hi Jan, I too live in Canada, but on the other side in Vancouver, BC. I was in a
car accident last September and have since developed TOS and RSD, I also have
had a problem with dizziness and was getting treated for it, but I don't think
any of the doctors I have seen thought it was from the TOS but rather from the
whiplash. I am having a bad time of it right now too, last week I thought I had
an ear infection and ended up in the emergency, to cut a long story short it
turns out it's all nerve related. The pain in my ears, neck, face and jaw as
just about gone, but my ears are still sore to touch and feel plugged, almost
like they need to pop, if you know what I mean. They are still sore to the touch
and what's really annoying is that I now have ringing in my ears. I too am
getting somewhat depressed as I have been without income for nearly 7 months as
my disability insurance carrier does not think there has been enough clinical
finding to warrant me being off work. Believe you me there has.
I haven't found anything on the net regarding whiplash and TOS, so I would be
interested if anyone else has. Well Jan I have to run now, but I am glad you
have found the group, I have found it really helpful, not just for info but for
the support, it's nice to know your not alone in this.
Hope you have your power back on, it sure sounds like its been crazy back east
this past couple of days.
All the best, Liz Young.

My seven year old daughter Shannon is being granted a wish through
Make-A-Wish. Her wish is to swim with the dolphins and feed Shamu. She
is currently on Flolan so her doctor has prohibited her from swimming
with the dolphins. We hope she will be able to get up close to the
dolphins and feed Shamu. This is an especially anticipated trip for the
family since we were to leave on a Florida vacation two weeks after she
was diagnosed.
I would like to hear from people who have gone to Sea World, Disney
World, or similar parks. What was your experience? Particularly those
who are on Flolan. How did you keep the medicine cold all day? (Both
the medicine on the pump and back up.) Did you have to carry the backup
supplies with you all day? Did you use a wheel chair? Any information
would greatly be appreciated. We do have one advantage, Make-A-Wish is
paying for the nurse who works with Shannon's doctor to come with us.
Bill form Boston

Hi!
I'm a new member and glad to find out this group exists. :)
I was beginning to think I was the only one with TOS. I hope I can
be of some help to others by sharing my problems.
I was diagnosed with TOS about a year and a half ago when my doctor
decided he could find no pulse in my left arm when he held it up
over my head.
Considering I'd been telling him I was having pain in my lower neck,
near where the shoulder and neck meet, just above the collarbone
area and accompanying dizziness whenever lifting something along
with bouts of off balance since a car accident in July '97, the
doctor knew I had something but couldn't figure out what.
Xrays were done and they found I had an extra rib. Hence the TOS
diagonosis.
I was sent for physio but it didn't help.
They gave me a lumbar puncture (which was screwed up) to find out
what was causing the dizziness but found nothing.
About a year and a half ago I had another car accident. Someone rear-
ended me and all my symptoms increased. I was diagosed with Whiplash
2 and given physio, massage and chiro.
The chiro said I had extremely tight neck muscles - hmmmm isn't that
whiplash? LOL
The physio gave me stretching and strengthening exercises which made
things worse.
I stopped physio and chiro because nothing was helping.
Unfortunately the car insurance guys cut me off massage which is the
only thing that seems to be helping. There are days (rare) when I
feel absolutely normal after a massage but then a few days later,
the pain in side of the neck returns along with dizziness when
lifting things and off balance when walking. At night the arm goes
numb, especially the last two digits.
I do get some weird night terror dreams too as was mentioned on the
list sometime earlier. :( Didn't start getting them until shortly
after that first car accident.
The pain is conveniently right over the area where the seatbelt was
lapped over.
I have had ear tests for balance, hearing tests to find I have
excellent hearing, lumbar puncture, neurological tests etc...
Anyways I could go on and on but I have gotten a wee bit depressed
because my doctor has given up and said "No more tests. You will
have to learn to live with it".
I have given up cross-country skiing, bicycle riding and other fun
activities because of the dizziness. Most of the time my arm is okay
but it does tingle, seems weaker, isn't cold or anything like that.
Just no pulse and it falls asleep when they lift it. The dizziness
comes with little or no pressure on the arm hence why I gave up my
activities. Computer work also bothers the arm and increases
dizziness.
Fortunately because I still have some "normal" days I feel there is
still a glimmer of hope. Although lately things have gone downhill.
I am curious does anyone else experience dizziness when lifting?
I couldn't find anything about that being a symptom of TOS.
I have heard that lots of people develop TOS after car accidents
from whiplash but couldn't find any information on the Net about it.
Anyone know of any sites?
Oh! And I live in Oshawa, Ontario Canada. :) Which is suffering
major rolling electricity blackouts so if you don't hear from me
you'll know why!!!
Thanks for reading my post. Look forward to working with you all.
Ciao for now,
Janet

Hi gang,

All is going well with Trey. He is attending third grade full time and is on grade level in reading for the first time. I was fit to be tied last week when he told me all the kids chase him at school. He looked at me and said Mom "I ask them to". "I love running and being chased". Maybe the Tracleer is working. We are flying to St. Louis Children's tonight for a biyearly transplant waiting list check-up. We hope all goes well as he is activated again and we hope that we will be able to return home and not have to go through with the tansplant wait in St. Louis. Trey says to tell everyone he will be Sponge Bob square pants and carry around a spatula. We do some mild trick or treat stuff. We figure we have enough blood and gore in our lives already. When we return I hope to build Trey a new bed. I want to build a Treehouse/Fort in his bedroom and put his bed in it. I have a cool design to make a full size tree to surround the treehouse. We have 10 foot ceilings in our house.
I figure if the kid can't always go to the outside. I will bring the outside in.My hubbie is less than thrilled. He will probally cancel my Sattelite TV to the home improvement shows Jennifer, I loved the news about Joey. What a perceptive child.

Sunnie and Trey Scott

It's been kind of quiet on the list. I think one of the things is
that people tend to post when they have a problem or want to discuss
issues relating to PH.This means that the topics get heavy.But I
would like to hear some more ordinary stuff.These children are truly
miracles and we should be celebrating some of the ordinary events of
their lives, and valuing the time we have with them.Many of us don't
get the chance to meet another child with this illness so I'd like to
know what the other kids are up to. Like, do they go to school, do
they go full or part time, what activities are they doing, how do
they spend their time. Is Victoria playing soccer again? How is
Maddie doing? How is Sam handling adjusting to life with flolan? Does
he still participate in the same activities? What are people doing
for Halloween?
So, to this end I am posting text from a message about one of Joey's
field trips that was sent to various members of our family. Joey is
four and was diagnosed with PPH at age 2. He is on flolan and
Tracleer.He attends a church preschool two mornings a week, from 9 am
to 12 noon.Bill, my husband ,goes with him and we have portable
radios that we give to his teacher. Then Bill is able to go off and
read in a different area of the church and still be available if
there is any problems with the pump. This gives Joey a chance to be
with other children his own age. He is developmentally normal except
that he is not yet potty trained. He was young when diagnosed, and
between diarrhea from upping his flolan and peeing from his Lasix we
just haven't gotten around to that.If anyone has any experience potty
training a child on flolan, I'd like to know. Joey is on oxygen at
night but not during the day.
Of course, if the class goes on a field trip, Bill goes, too.
Joey went on a filed trip Thursday with his school to Ft.
Campbell.One of the fathers of a child in his class is a helicopter
pilot, who pilots an Apache attack helicopter. This gentleman was
involved in Operation Anaconda in Afghanistan, and stated with
obvious pride that the Apaches were able to turn the course of the
battle.
Bill said Joey was very interested and asked lots of questions. The
Apache was huge and had both cannons and rocket launchers. Bill said
that the Apache had all the fire power of a tank, with much more
mobility.The Apache was also able to fly from the southern border of
Afghanistan all the way to the northern border and 3/4 of the way
back before having to refuel, being outfitted with an extra fuel tank.
"Yes,with this helicopter,"the man said,"we can blow up just about
anything we want."
"Do you blow up any good guys?", Joey asked.
"We try very,very hard,just as hard as we can, not to blow up any
good guys," said the host. "We only want to blow up bad guys."
Joey wanted to be able to play with the rocket launcher but Bill was
not comfortable with him playing with anything that had a big sign on
it saying : WARNING, EXPLOSIVE DEVICES INSTALLED.
The kids also got to meet Mac Gruff, the crime dog, and met some real
life police dogs, one of whom liked to play ball and could jump about
6 feet in the air and catch the ball on the fly.
The dog handler asked if anyone had any big dogs and Joey held up his
hand. The dog handler asked Joey what kind of dogs he had and he said
he had a Gompers and a Satchmo.Bill explained that Gompers was a
Saint Bernard and Satchmo was a black Great Dane.He agreed that Joey
had very big dogs.
Joey had to do a lot of walking with his backpack on for this field
trip and Bill thought he tolerated it very well.
Bill and Joey both enjoyed the field trip a lot.Nursery school has
apparently changed since I was little, we never talked about blowing
anything up.
Friday, Bill took Joey with him while he ran errands.When they were
at the insurance agency obtaining proof of coverage for our
automobiles, he got into a discussion there with someone about our
old trucks. He mentioned that our daughter (age 19) was a princess
and didn't like driving old vehicles.Joey overheard this and took Dad
to task when they got back to the truck. "Don't call my sissy a
princess, she's not a princess!" he insisted.
"She's not?" Bill asked.
"No,she's not," said Joey. "But I am the little king of the playroom."
Jennifer, Bill's wife and mother of Joey, age 4

In the last few weeks we have had several members leave the group. I
am not really sure why this occurs, other than in one instance some
time ago I know why, but I am a strong beliver in free choice and free
will so I never interfer with personal decisions. I guess what I am
trying to say here is, if someone has TOCs and becomes part of the
group; they still have it so why not just stay in the group even if
you choose not to be verbally active. I don't undertstand why this is
happening but I did want the group members to know that I am aware of
these occurences.

I saw someone mention a dr in Houston. But no name was given. Does
someone have that persons name?
Mark

Dear Kathi,
What a sad story. My heart breaks for you. Please accept our
condolences on the loss of your daughter.
But I do not think that seeking a transplant is in any way
challenging God, any more than starting Flolan or bringing your
child to see a doctor is challenging Him.These are all difficult
decisions, and everyone deals with them as best they can.We have no
way of seeing the future, except to know that we all must face our
own mortality someday. I do not believe that my faith in God will
ensure that my child lives a long life or ensure that he will avoid
suffering.Jesus did not live a long life by modern standards, and He
suffered terribly on the cross.If God is not cutting any breaks for
His closest relatives I'm not sure that I deserve any.So do not
berate yourself for a lack of faith or feel guilty for your decision
to have the transplant.
But this is what is mind boggling to me:Why would an all powerful God
choose to become human and then choose a path which includes
suffering and dying? A path that included not only suffering and
dying as a person, in the form of Jesus, but must have also included
suffering as a parent,a Father Who witnessed the murder of His only
Son.
The only explanation that makes any sense is that God loves us enough
to suffer with us when we are suffering,because those who haven't
suffered can't relate to it very well.
God Bless You, too.
Jennifer, Bill's wife and mother of Joey, age 4

Lori,
Our prayers are going up for you and Maddie. I can't begin to imagine the
pain your family is in! You are right though it does mean flolan fails.
God Bless You
Gelene
Victoria's mom double lung TX 8/20

Dear Bill and Jennifer:
How I wish I would have read your letter a year ago. You are so very right to believe that transplant is the WRONG option.
Our precious daughter was diagnosed at 5 months with primary aveolar failure. They told us that the only hope would be a heart and lung transplant.
To make a VERY long story short, at 2 years old, we got the call.
It was the worst nightmare we ever could have imagined. This precious child suffered for 6 long weeks after her transplant. Tied to her bed, not understanding what we were doing to her. Surgery after surgery to correct the mistakes they made. It remains to be painful even as I write this. Transplanting lungs in a child is WRONG. They DO NOT live. Our daughter was vibrant and wonderful....on only 1 liter of oxygen. Running, playing, happy....the transplant killed her on August 7th, 2001. 8 days after her 2nd birthday. My advice after going through this would be....do not transplant your child. God works in mysterious ways...do not challenge him. I wish we had more faith and would have believed in him....
Our daughter is gone....it is the worst thing you can ever imagine. Nothing can change this.
I am writing a book. I have to tell parents...DO NOT DO THIS. These children are guinea pigs. Please trust in the Lord...he will provide.
Thank you, and God Bless You,
Kathi

Hi Liz, Pain killers were never even offered to me by Harold
Kleinert, M.D. so that was not an option I had. My step father,
a surgeon, who practiced with Kleinert, told me they were all
addictive. I don't know anything about the drug you mentioned.
Based on your email, I would think that the surgeon would be the
best judge of when you should return to work and for what length
of time you should work. I know that if I been permitted to
return to work, I was not, that some of the restrictions by the
M.D.'s are set at work 15 minutes and take a 5 minute break.
You won't really know what will happen until you go back. If
you have constant pain, which I did, when you are not
working...then you can imagine what will happen if you go back
to that type of work.
I don't know if this is a work comp case or not or why you have
not been paid in 7 months. Your surgeon should also make up a
list of work restrictions for you and sign it which should be
given to your employer. Also, I do know that under the labor
laws, if your M.D. says you cannot return to a job that is
repetitive, or the job you were doing, the company has to find
you a job that you can do. I did not know that until it was too
late.
No one really understands the pain of TOCS unless they live with
it. It is impossible to explain it to someone. Unless they
have it, don't expect them to understand; they cannot.
Sometimes they might be supporitve and other times they might
not be. I was fortunate to have an M.D./surgeon in the family
who could understand somewhat.
I am not sure that this has been of any help to you; perhaps
someone else in the group will have more info. Also, check out
the websit on Harvard Graphics to see if anyone else with tocs
has taken that and look it up on the internet and see what it is
used for. Since it seems that you have already had the
surgery; maybe in time all will get better. Again, I don't know
anything about
what happens to most people who had the surgery and returned to
work. I did not have that option. Hopefully someone else will
be able to assit you more.

=====
Cathy

You should check out the Thoracic Outlet Syndrome forum at the neurology
web forums at Mass. General Hospital
http://neuro-mancer.mgh.harvard.edu/cgi-bin/forumdisplay.cgi?action=topic
s&forum=Thoracic+Outlet+Syndrome&number=98&DaysPrune=1000&LastLogin=. I
have seen some postings there on younger patients, I know one mom that
posts whos daughter was 16 when she first started with TOS and is now in
her 20s. The forums have a search function that may also be helpful.
Also, be very careful of any pt that is making her worse, anything that
causes more pain with tos is not a good sign. There is a pt that has
been developed specifically for TOS called the Edgelow method,
http://www.cwce.com/feinbergarticles/tos.htm, and it is not supposed to
cause any pain, there are many that have had at least some success with
this. I would like to say i have, but i have just started with it and am
too new to it to comment, but i was very impressed by Peter Edgelow, the
methods he uses make a lot of sense after they are explained. I'm not
sure about age specific treatments, I am 29 and have been wrestling with
this for longer than I know(first symptoms in high school, diagnosis at
24) and I have yet to see different treatments based on age. But I do
think that support and understanding is important at any age, one of the
best things i have found is getting educated in all aspects of it, and
that learning is never ending.
You may already be familiar with this but there is also a forum for
Ehlers-Danlos Syndrome at MGH,
http://neuro-mancer.mgh.harvard.edu/cgi-bin/Ultimate.cgi?action=intro&cat
egory=2&BypassCookie=true.
hope this helps, God Bless.
...katie
On Wed, 30 Jul 2003 01:53:58 -0000 "shadowgato2003"
<shadowgato2003@...

Dear bill,
We have always considered lung transplant as a last resort. the reason we
chose flolan over transplantation is because the survival statistics were
better with flolan. despite her high pressures, she does very well adding
the fact that Maddie has had cancer, she would have to be on
immunosupressive drugs after transplantation.being an oncology nurse I know
the risks of suppressing the immune system when there could be the
possibility of cancer cells still lying dormint in her body. Of course we
have checked out Lung transplant programs and have even change to an
insurance program that covers transplants if it ever came to that. For the
time being we are grateful that she feels well and is very functional. we
take each day one day at a time. although Dr. Ivy does want us to spend
some time at a lower elevation then get an echo to see if the altitude makes
a difference in her pressures. we have noticed that when we have taken her
to sea level, she acts like she has more energy. where we live in Utah is
about 4500 feet above sea level, and where she gets her echo's in denver is
about 5500 feet. Take care, Lori

Dear Gelene and Laurie,
We are not listed anywhere for a transplant.This is not
because the option has not been presented to us, it has been several
times, including when Joey was originally diagnosed, when a cardiac
cath revealed the actual pressure in his lungs to be 110.Since that
time it has climbed to 140, if the pressure as estimated by echo is
correct.We have not done another cardiac cath since Joey's original
diagnostic one.This is because we won't let our cardiologist do one,
not wishing to expose him to the risks of anesthesia without a clear
sign of benefit to Joey.We have also rejected the option of lung
transplantation.
We cannot speak for anyone else, these are very difficult
issues and all situations are not alike.We hope for the best for
Victoria.And it is a very different decision with an older child.
But Joey was not yet three years old at the time he was
diagnosed.He lives from day to day and has no concept of deferred
gratification.He has a young child's view of death, although that is
changing as he ages. Elisabeth Kubler Ross, whose life's work was on
death and dying, stated that young children do not fear death, that
what they fear most is mutilation and separation from parents.Lung
transplantation would involve both. We already know how hard it was
for him to be separated from us when he was in PICU, even with
liberal visiting hours, there were times that we were asked to step
out.
But a major reason is that the statistics on lung
transplantation are not that hopeful. And when you get down to very
young children they are downright dismal. Lungs, unlike other organs,
have to be matched closely in size to the recipient.Transplants on
individuals who have reached an adult size (even a small adult size)
are much more sucessful.Vanderbilt, where we receive our care, does
transplant lungs on adults and does a variety of other organ
transplants on children.I feel that we have been able to get accurate
information to make a decision.
We feel that the only treatments that make any sense for Joey
are those which would add to the quality of his life.Right now , he
is not suffering and has a very high quality of life. Young children
have an amazing ability to compensate.He is at risk for sudden
death.But with some statistics showing a 15% 3 year survival rate for
lung transplantation on children under the age of five, it is not
clear that a lung transplant would add to either the quality or the
length of his life.
As Christians, we do not feel that death is something to be
feared.We just don't want Joey to suffer unnecessarily.If we see
evidence of suffering and evidence that lung transplantation could
improve his quality of life, our decision could change.Our cardiology
team is aware of our feelings and has been very supportive. For now,
we will rely on Tracleer, Flolan and prayer (not necessarily in that
order).
Bill and Jennifer Nelson
parents of Joey, age 4

Does anyone know of a teenager with tos? I have a 16 year old with
Ehlers-Danlos Syndrome who has dislocated her shoulder 8x and now
has tos as a result. I looking for info on tos treatments specific
to her age group. She's been in pt for 5 weeks and is getting worse.
I'm concerned about the surgical option. Any info you can provide
would be appreciated.

Dear Gelene,
Maddie has not been listed because in 1999 she was diagnosed with Acute
lymphocytic Leukemia and has recently completed a 2 year course of
chemotherapy(in addition to PH!)they won't consider her until she has been
in remission for five years. We have checked out the living donor program at
UCLA, and will probably go that way if it ever came to that. to be honest
with you the thought of a Lung Transplant scares me, because that would mean
she has failed on the flolan. It sounds like your daughter is doing well, I
wish you the best. Lori(maddie's Mom)

I agree these children are exceptional. Has your daughter been list for
transplant? If so where? If not why?
gelene
Victoria's mom double lung TX 8/20

Melissa:
I had blood clots in my subclavian veins also. One month following my rib
resection, my doctor did a venogram/angioplasty to see if my veins were now
open. I had the left rib resection done in October and the right done in
February. Following my second venogram, my surgeon said that I am now "clear"
and should never encounter any problems. YEAH!!! He said that sometimes even
after removing the rib, the vein needs to be opened up with a balloon.
DENISE

Hello I am new here. Two weeks ago I had a rib resection in my left
arm because I developed a blood clot in my subclavian vein. I was
wondering what type of therapies (treatments) I could look into to
pevent this problem from occuring im my other arm. Thank you
Sincerely, Melissa G

To: PHA members and friends:
It's now time to call your Senators!
As you probably know from PHA's recent note two important legislative
acts (H.R. 4013 and H.R. 4014) were passed in the House of
Representatives on Oct. 1.
Now, the Senate is going to vote on this legislation, and we just
received word from the National Organization for Rare Diseases (NORD)
office that the vote will likely come as soon as this week.
Please call both your state's U.S. Senators as soon as possible to urge
them to vote in favor of these two pieces of rare disease legislation:
the Rare Diseases Act of 2002 and Rare Diseases Orphan Product
Development Act of 2002 . Don't forget to give them a personal
perspective on why this is important to you and your family as citizens
connected to PH.
The Capitol switchboard (202-225-3121) can put your call through to your
Senator's office. To find the names of your state's Senators, go to
http://www.senate.gov/senators/senator_by_state.cfm
These bills together will:
----codify the NIH Office of Rare Diseases (write the office
into law so that it cannot be eliminated in the future)
----authorize $24 million annually for rare-disease research through the
Office of Rare Diseases
----authorize $25 million per year for the FDA's Orphan Products
Research Grants program, which currently gets only $12 million.
The House Bills passed on voice votes. That means that no record is
kept of who voted yes or who voted no. While we are not sure whether
the Senate will vote on these measures by voice or roll call, you should
still let the person you are talking to know that you'd like to be
notified know how your Senator votes on the two bills.
Also, after you make your calls, please send an e-mail to
rino@... and let us know your name state and the names of
the Senators you contacted so we can continue to track our
effectiveness.
Thank you.
Rino

Hello,
Madison just returned from the cardiologist in Denver yesterday. we were
very hopeful that maddie's pressures would be lower. She has been on flolan
for five years and Tracleer for 6 months. we haven't had an echo since she
was started on Tracleer in March. we were quite shocked when her readings
were between 140-155! She is feeling better than she ever has. Even the
doctor was surprised because she looks so good. sometimes it takes a trip to
the Cardiologsit to remind me I really do have a seriously ill child, even
if she doesn't act like it. I just need to focus on how dramatically her
symptoms have improved since being on flolan and tracleer, rather than the
actual numbers they get from the echo. I am grateful for every minute I have
with her. I think there is something very special about our PH kids that
makes them strong and in turn gives us strength by their example. Take care
Lori(maddie's mom)

Hope all goes well with Sam's echo.But you know what? We've never had
a good echo. Every time our son Joey gets an echo, they estimate his
pressures even higher.But Joey is doing great. We've seen a great
improvement since he started Tracleer but it was flolan that saved
his life first. Tracleer takes 6 months, I am told, to reach its
maximum effect. When your child is passing out you can't wait 6
months.
We had rounds of good days and bad days when we started flolan. We
have been on flolan since Christmas 2000.Its real hard to comment on
side effects because Joey was 2 years old when he started. Jaw pain
and diarrhea were the noticeable ones, but a 2 year old child isn't
going to tell you about joint pain or nausea.Joey seems much more
stable since he started Tracleer, and his activity levels are
better.We have been able to reduce his flolan levels some.He does get
migraines, but there is a family history of them and that is
something he may have had anyway (he had his first migraine after
starting flolan but before starting Tracleer, but his siblings get
migraines also, though not as frequently.
Joey's presenting symptom was sudden and complete respiratory
arrest.Nothing we have gone through since was as bad as doing CPR on
Joey in a parking lot in the rain.This is a very serious illness and
coming to terms with it means one must accept the fragility of life.
But Joey is not only alive, he is an absolute hoot.He enjoys life
more than our teenagers and has been known to take them to task when
they are griping too much.Forget about teaching him to work his pump,
we are still working on things like "don't stand on your pump" "don't
throw your pump at other people if you are mad""tell mom or dad right
away if crash your tricycle, fall down and clamp your line off".
As far as I can see, Joey is doing a lot better than when he was
first diagnosed.He is active and happy and has had many more good
days than bad since starting Tracleer.I hope for the best with Sam's
echo.But hang in there. It is certainly possible for Sam's symptoms
to improve, even if his echo doesn't.
Jennifer, Bill's wife and mother of Joey, age 4

Thank you Shawna for keeping the Group posted on your status. I
am glad you are home and all has gone well. I wish you a
magnificant recovery. I also want to thank all the Group
members who have emailed this week and shared info. Sometimes I
feel like no one is out there; all I did was create a room on

Well I had my surgery on Tuesday. I was released from the hospital
today. My surgeron performed a Rib Resection (Thoracic Approach). I
am doing well. You know my arm has not fallen asleep one time since
the surgery. My left hand is a little blue, but the Dr. said that it
would go away soon. The veins are not near as appearant as they
were. The pain isnt as bad as I thought. To be honest I have taken
1 pain pill since yesterday. That was just to get to sleep. The
worst part was I was so nauseated from the oxicotin (sp?) and
morphine. So they took my off of that yesterday. Boy do I feel a
hundred percent better. The drainage tube was not too pleasent, but
he removed that yesterday. I asked him about the vein that was so
large in my venogram. He said that he was work in a small area and
it was a definate presence that made him nervous, but got through it
fine, and hopefully it will shrink down some since the compression
was no longer there.
Just wanted to let you all know I am fine and glad I had it done.
=)
Shawna

Congratulations and thank you!
I am pleased to tell you that over 100 contacts were made with Members
of Congress by PHA members in less than 24 hours.
I am even more pleased to report that both H.R. 4013, the Rare Diseases
Act, and H.R. 4014, the Rare Diseases Orphan Product Development Act,
passed the House of Representatives today.
Working together with the members and friends of other rare disease
organizations, we made a difference today.
Thank you letters or e-mails to John Shimkus, Mark Foley and Henry
Waxman will be much appreciated. A letter or e-mail to your
representative thanking him/her for continuing support would be
wonderful as well. Shimkus, Foley and Waxman worked very hard for the
rare disease community to make this happen. Contact information can be
found at: http://www.house.gov/
The next step for these bills will be the Senate. We will let you know
when votes are scheduled.
Rino

PHA is a member of the National Organization of Rare Diseases (NORD).
We have just received the following important information from them. We
encourage you to take immediate action on it.
The two rare disease bills, H.R. 4013 and H.R. 4014, will be brought to
the floor of the House of Representative tomorrow, Tuesday, October 1.
The vote will occur after 12:00 noon.
If the bills pass the House, they will be brought to the Senate where
Senator Ted Kennedy has promised to bring them to the floor for a vote
as soon as possible.
If H.R. 4013 and H.R. 4014 are passed, they will codify the NIH Office
of Rare Diseases (write the office into law so it cannot be eliminated
in the future). It will authorize $24 million annually so the Office
for Diseases can fund rare disease research. The bills will also
authorize $25 million per year for the FDA's Orphan Products Research
Grants, which currently gets only $12 million.
It is extremely important that you contact your congressman's
office in the House of Representatives before noon Tuesday and
ask him/her to vote for H.R. 4013 and H.R. 4014. The Capitol
switchboard (202-225-3121) can put your call through to your
congressman's office.
Let the person you speak with know how living with a rare disease like
PH affects you and your family members and ask them to notify you on how
your Congress member votes on H.R. 4013 and H.R. 4014.
Please send an e-mail to rino@... after you reach your
Member of Congress' office. Please let us know your name, your state
and the name of your Congress member. This will help us track our
ability to reach members of Congress and their responses.
Thank you.
Rino

Hi Linda,
Alex was diagnosed in March . We live in Albuquerque , New Mexico and
travel to Denver , Co. for treatment . He see's Dr. Ivy . Alex hasn't
really had any side effects other than jaw pain . Alex's flolan dosage
shouldn't go any higher according to his doctor . They say if anything
they'll start to lower his flolan rate since he's doing so well with the
tracleer .
Good Luck to you guys and I hope Sam's echo goes well .
I'll let Alex know about Sam and see what he says about writning to him
.What is Sam's e-mail address ? Just in case.
Best Wishes
Pat - NM

Gelene,
Wow, what a story. Thank-you for sharing what must have been an overwhelming time for you and your family. I was amazed that Victoria had no symptoms and yet her disease was so severe. (I too am a nurse and felt very guilty that I didn't detect something wrong with Sam sooner) I'm so pleased to hear that her transplant was a success. I pray that she continues to do well. i really appreciated the info about flolan dosing. I feel much more informed and ready to ask questions at our next clinic visit. I suspect Tracleer will be considered for Sam eventually. I've been researching it. Please keep us informed of "life after PH". Hopefully all of our children will be cured someday.
God bless you and your family,
Linda (Sam's mom)

I wish that I was able to answer your questions. Perhaps other
group members will respond to you and be able to give you added
support. I cannot advise you how long to continue the treament
you are but if you are having any improvement whatsoever I would
recommend you stay with it. I don't know anything about the
effects of acupuncture on TOCS, maybe it will be a positive
outcome. I don't know that anyone can understand the effects of
TOCS unless you have it. I long ago gave up the attempt of
trying to get people to understand what is impossible. If you
don't have TOCS it is truly impossible to even begin to
understand the pain and the frustration you have with being so
limited. With PT or surgery it usually does improve, over time.
I don't lift more than 1/2 gallon of milk and even that is
difficult after lived with this for 8 years. I do vacuum
although the vacuum cleaner at first felt like a two ton truck.
Quite often you have to learn to do things another way; your
way. It sounds like you are getting good medical treatment in
your country; maybe it is better than what we have in the U.S.
I do hope the group can be of support to you. I sleep on my
back with my shoulders shruged. When I was first diagnosed I
was told to sit at night with both arms elevated on two pillows
(this raises the shoulders and takes the pressure off the
nerves) on the couch for ate least on hour before I went to bed.
This created numbness in the arms and hands which should permit
you to sleep longer before awakening from pain. I was only
given anti-inflammatories for treatment. I can only say that
you are not alone, many of us have this and live with it daily.
Be positive and be determined--that will get you a long distance
at a slow pace...I hope the other group members will be respond
to you and offer you some support. Living with TOCs is
difficult and it might greatly change your lifestyle, depending
on the severity of it, but ... accept what you cannot change and
over time, you will learn to live with TOCS and it will become a
part of your life but hopefully, with medical treatment, not be
the number one pain it is right now.

=====
Cathy

Hi Pat, Thanks for responding. Was your son just diagnosed in March? If so we are really in the same boat! Where do you go for treatment, who is his doctor? I am still amazed at the dosage numbers (Sam is only on 27 ng but has had difficulty with side effects) But he is no longer as short of breath, no chestpain and most importantly, not passing out. I'm glad to hear that your son and his brother have taken on responsibility for the illness. That was one thing I tried very hard to do for Sam. He is very competent at priming, changing pump settings, etc. He is very knowledgeable about the disease and its treatment. Has Alex decreased the flolan dose since on Tracleer? Glad to hear he is doing so well. We go for first echo since treatment. Pray for a good report. Thanks again, hope to hear from you soon.
P.S. would Alex be interested in writing / e-mailing to Sam? I think he would like to correspond to some one his age.
Best wishes,
Linda Mercuri (and Sam)

Hi ,
I have just been diagnosed a few weeks ago with TOS . I sleep on my
back with difficulty . I have it on both sides bt oredominantley on
the left . I started physio about 3 weeks ago and acupuncture +- 2
weeks ago .A few weeks ago I was given excersizes to start. I have
difficulty carrying relatively light items. How long should I give
conservative treatment which I feel has started to help but slowly .
A few weeks ago I could not even type or write . I have a wife and 2
kids who have difficulty understanding what I am going through .
Any suggestions on coping mechanisms , how long I should give
conservative treatment and your experience with the problem . I live
in Cape Town South Africa.

It seems that it is necessary for me to post this message. If there
is any disagreement among members in this group, disputes, or taking
sides; I will ask members to leave this group. I started this group
and if any of you have any reasons you choose not be here you are free
to leave. AGAIN I WILL STATE THAT I DO NOT MONITOR THIS GROUP
CLOSELY, I DON'T HAVE TIME AND THAT IS NOT MY PURPOSE, BUT I DO
RECEIVE EMAILS. IF I SEE ANY MEDICAL INFORMATION ON HERE THAT I AM
POSITIVE IS INACCURATE I WILL COMMENT ON THAT. AS A PAST EMPLOYEE
WITH OVER 25 YEARS OF EXPERIENCE IN THE MEDICAL FIELD AND MY STEP
FATHER BEING A SURGEON...IF I SEE ANYTHING THAT I KNOW IS INACCURATE
IN REGARD TO TOCS, I WILL COMMENT ON IT, OR I WILL ASK YOU ABOUT IT.
I WILL NOT PERMIT INACCURATE MEDICAL INFORMATION IN REGARD TOCS HERE.
YOU CAN DISCUSS ANY TYPE OF TREATMENT OR SURGERIES THAT YOU HAVE HAD
THAT HAVE BEEN SUCCESSFUL BUT I WILL NOT PERMIT MISINFORMATION. I
HAVE NOT MADE ANY RULES UP UNTIL NOW BUT IF YOU DO NOT WISH TO ABIDE
BY THIS RULE AND YOU CHOOSE TO CREATE CONFLICT OVER IT YOU ARE FREE TO
LEAVE THE GROUP.

Lori,
thank-you so much for the nice response. I'd like you to know that you and your daughter have already touched our lives. We read about Maddie's story in the Feb issue of perisitent voices. You did a beautiful job of telling others about life with PPH. I only hope that I can be as strong as you have been through all of your daughter's difficult times. I suppose we are just beginning as far as the Flolan goes. Sam is only at 27ng and has had difficulty going any higher due to side effects. Hopefully we will see some evidence of a response at his next clinic visit. Thanks again, we have been truely inspired by your story and deeply appreciate your support. I'm sure you'll be hearing from me again with more questions!
Take care, Linda and Sam

Dear Linda,
I feel like a veteran parent, we have been through a lot with my nine year
old daugher. Maddie has been on flolan for five years, and has been through
chemotherapy for leukemia during that five years also. She was started on
Tracleer six months ago. She is in remisson with her Leukemia and is doing
great. In regards to the Flolan side effects. When Madison first started on
flolan, we were increasing the dose weekly. the thinking at that time was
that you pushed the dose until they had side effects (HA, rash,etc) but now
according to her cardiologist, they feel that it isn't necessary to push the
dose until the poor kids feel miserable. Since starting on Tracleer, we have
been able to decrease the Flolan substantially. In fact, when she does get
Flolan side effects we decrease her Flolan rate some more. hang in there,
the major lifestyle changes that you make for a child with PH soon just
became routine and things do get easier. Take care Lori

Dear Laurie,
My name is Lori Heaton, my daughter Madison was diagnosed with PH when she
was four, she is now nine. She has been on Flolan for five years and
Tracleer for 6 months. My husband and I have five daughters. Jessica 21,
Janae 19, Chelsea 13, madison 9, Randilyn 8. I believe we met at the PHA
conference this summer. I check the group board almost daily but I don't
post very often. I love to hear about everyone's situation, and I will post
a reply when i feel my input will help someone else. I agree with Bill,
Sometimes will don't hear from each other, only when ther is a crisis. It
would be nice just to chat about everyday concerns. It is hard sometimes to
find the time. We all know how involved you can get caring for the needs of
your PH kid, and the needs of the rest of your family. Madison sees Dr. Ivy
in Denver. We are going there next week. It will be her first evaluation
since she was started on Tracleer. We are hoping for good news! Please
everyone keep posting when you have a minute. It's nice to feel your
support!
Take care Lori

I am glad for you Shawna; that you will soon have the surgery
and hopefully feel much better. I was no a surgical candidate
so ...I did not have that choice. I have not had any headaches
from TOCS and I don't know what he means by a sluggish
diaphragm. If they take your top rib out you might feel a
little differently down there until you get used to it I
guess--beats me, ask him what that means...hey maybe you won't
even notice or have a slugghish one...Good luck and keep the
group posted.

=====
Cathy

Hi Linda ,
My son Alex is eleven and has been on flolan since March , he was put on
tracleer in May . He's been doing great with both of them . In a couple of
weeks his flolan will get bumped up to 100 ng . His doctor says this is as
high as he's going to get ( I guess for now.) There has been some talk about
getting him off the pump , but that won't be until next year .
Alex also has an identical twin brother named Felix . Felix at first had a
hard time dealing with this , but now he's ok with it but is very protective
of his brother . Even though they're only eleven they both know how to prime
and change the flolan cassette . They wanted to learn just in case something
happened in school . They both attend Garfield Middle School .
All in all everything has been going good for both of them .
Good Luck to you guys.
Pat - NM

Saw my Dr. on Tuesday. We scheduled it for this Tuesday (7/15/03) at
12:45 pm! I could kick myself in the a$$ for not telling our
schedulers to block that time out for me, it was clear all morning
and I get to the dr. and BOOM all booked up!
He said the incision will be about an inch above my collar bone. He
also said that he hopes he can have me back at work in a few weeks
(me too cuz I cant afford to be off for more than 3 weeks).
He also told me that my Diaphram will be kind of sluggish for a
couple of days. Does anyone know what this means?
Also, do any of you suffer from headaches from TOS? I have horrible
headaches on my left side (same side as my TOS). I asked the dr.
about it and he said that it could cause headaches. Just wondering
if any of you have these too?
Hope everyone is well!! If I dont talk to you before, I will talk to
you after my surgery.
=) Thanks for being a great resource for information!
Shawna

I guess I should introduce myself since I have just sent my 2 cents out to the last couple posts. My name is Gelene (soft G). We are originally from upstate NY but currently live in South GA. A car accident disabled my husband and shoveling snow became too much of a burden. I am a RN working as a case manager. A wonderful job that allows me to work at a home office.

About 13 months ago our daughter Victoria was diagnosed with PPH. There is no family history. She has no other medical problems. She did not ever have any symptoms. She was playing soccer and is a second degree black belt in Tae Kwon Do. She was winning national tournaments. One day we were shopping and I noticed a very slight blue tinge on Vicki's bottom lip. The next day she had a cough and I brought her to her doc. Everything sounded good. Vicki was sent for a chest x-ray to rule out bronchitis. On the film it was noticed her right heart was 2 sizes larger then normal. She was diagnosed with PPH. Her pressure at this time was 150. We transferred care to Gainesville Florida as there is not pediatric specialist near us. Vicki was started on ccbs and her pressure went down to 90. No change in her heart. Flolan was started and a transplant work up completed.

We kept going up and up on the flolan and added the adult dose of tracleer. No response. Victoria was changed from shaded to the active list. We got our call on 8/20 and Victoria underwent a double lung transplant. She is recovering fine. Did have a bout of mild rejection and we will know if that is over in a month.

Victoria no longer has PPH. She feels great and is breathing free. She is back in TKD and looking forward to soccer. We not longer mix flolan but as they say exchanged one set of problems for another. We our trusting in our Lord and Savior Jesus Christ for a long and healthy life.

Gelene

Victoria's mom

I am sorry we have not communicated sooner but the last 7 weeks have been amazing for us. My daughter was on flolan for a year. At transplant she was on 78 ng. My understanding is dosage is determined by patient presentation and response. No 2 people are the same.I have heard about chidden going up to 100 ng. Please don't say you are guilty of avoidance - it is OK to want a normal life. Things have children changed for your whole family but the focus needs to be on living life. I pray your son shows a good response.

Gelene

Victoria's mom 11 double lung TX 8/20

With all that has happened in the last 7 weeks I also neglected to post. It
grieves me so that anyone has to have PH but especially our children. I
have one child - Victoria 11 yo- and one step child. I cannot address the
sibling issues except from a somewhat clinical point of view (Psych nurse
specialist - scary huh?). The feelings the other children get - fear,
anxiety, jealousy, etc. are all normal. One child may be sick but this
disease consumes everyone. It is not something you can take a pill for and
not think about until the next dose. It is there in your face all the time.
I would suggest counseling for the family. I'm sure money is an issue so do
you have a pastor, priest, or county agency (maybe united way) that can do
this for free?
My prayers are with you all,
Gelene
Victoria's mom 11 double lung TX 8/20

Shawna, I hope it all goes well for you. Based on the people I
know, their surgery went well but they did have some pain at
first. It goes away after time. I would have had the surgery
if I could have; I was not a surgical candidate. I would be
more worried about the rib coming out than the tube; chances are
you probably won't even know its there. They will put you out
first, then the tube and the rib removal and the tube will come
out when you are in recovery. You probably won't know it was
even there...
What city are you having this done in? Be positive!!! My
brother is out of town for two weeks and the person who was
suppossed to water his grass did not do it. He has a huge
yard...
I can tell you that my TOCs in a flare since I decided to move
the hoses around and water the grass for him. I just cannot be
pulling and moving long heavy hoses like that...I learned it the
hard way. Take care.

=====
Cathy

Welcome Laurie and hello to all fellow parents,
I feel a little guilty because I have been reading (and learning) from so many of you for months but have just never responded. This is a wonderful support network so I'd like to now join "officially". My name is Linda Mercuri, and our son Sam was diagnosed only a few months ago (PPH). He is 9 years old, and until last December been perfectly healthy. His symptoms started with a syncope episode, then a couple of what we thought were seizures (actually pulmonary hypertensive crises caused by the lack of oxygen and decreased carrdiac output). He also had the typical SOB, fatigue, and activity intolerance but somehow he coped and we had no idea how sick he was. After an MRI showed evidence of the changes of PH, I frantically searched the internet for info. To make a long story short, I was overwhelmed both with too much bad news too soon and the frustration of not understanding this complicated illness. It was the PHA that saved my sanity. A special thanks to Pat Patton
in Florida who answered my first plea for help, then Nancy Frede who comforted and educated me tremedously.
We ended up at Cleveland Clinic (Ohio). Sam has been on Flolan since June. he is doing well but as all of you know, we are still in the adjustment phase. Some days he seems great and others he is wiped out. And then there are the days when the side effects of the Flolan seem worse than his disease. (nausea, headaches, joint pain, etc). His dosage is still being increased gradually. I'm still not clear how the "right" dose is determined. Do children continue to require higher dosages? Do they ever reach a max? Any responses to this would be helpful.
Also, Sam's physician wants to see how he is responding to the Flolan first (his first follow up echo is in 2 weeks) before considering other therapies. Would like to hear more about Tracleer and how it is used with/ without Flolan. Has anyone weaned off Flolan entirely and switched to oral meds?
I can relate to Bill Nelson's comments totally. Although it is wonderful to talk with other parents, I find very little time to do so. By the time the kids are in bed, medication is mixed, and all the other misc. daily chores done, I am exhausted. As he said, our entire life seems to revolve around this terrible illness. And I am also guilty of just avoiding it sometimes. So I will go for weeks without getting online, only because it can be painful as well.
But I do find the list helpful and encourage others to join. I still have so much to learn and appreciate the support and knowledge of the "veteran PH parents" out there. God bless you all for sharing!
One other comment. Bill asked how PH has affected other siblings. We have just 2 boys, Sam and his 13 year old brother Jake. initially we thought his brother was coping well, but then he started to have symptoms of anxiety similar to panic attacks. Just like a father to be may have "sympathy" pains, he was having spells of chest pain and SOB. he was convinced that he had PH too. We had planned taking him to th PH center at Sam's next appt for evaluation and echo but it was in his best interest to reassure him sooner. So our local cardiologist did an echo that was thankfully completely normal, and now he is fine. That reasurrance was all he needed. Never underestimate the effect this (or any chronic illness for that matter) can have on siblings. Keep them informed with appropriate info (our son said he felt left out initially and everyone but him knew what was wrong with Sam.). Help them to be involved but don't throw too much responsibility on them. Jake looks
out for his brother but can be a bit overprotective at times. Sam thinks we all worry about him too much! I think it was very helpful for our other son to go to one of Sam's clinic visits. It allowed him to better understand what Sam has to do and he could ask questions, meet his PH team, etc.
Hope I didn't take up too much space! Thanks to all for listening and sharing.
Linda Mercuri (LMerc512@...)

Im still here, I haven't had my surgery yet. I just got back from
vacation on Monday and didnt want to risk feeling icky while out of
town. I see my surgeon next Tuesday to get ready for surgery. I am
guessing that it will be around July 15 somewhere (at least that is
when I am going to try to get it schedule).
He did do a transcervical 1st rib resection about 3 weeks ago. I was
glad I wasnt the first hehe. I ran into him a week later and asked
how it went. He said that it went very well, that he believes that
it had helped all her symptoms. Said that she was in a lot of pain,
and told me to be prepared for that. I also talked with the nurse in
the room and she said that it went very well also. I guess I have an
advantage here because since I work in the surgery department I can
ask all kinds of questions. I also get to pick my OR staff for my
surgery. You know what is bad, I am more nervous about being
intubated than the surgery itself. I dread the thought of having
that tube down my throat! haha!!
=)
Hope all is going well with all of you!!
Shawna

Welcome Laurie.
I will respond on list , as that may help get at least
one more post. My wife Jennifer and I have three children,
ages 19, 14, and four. our four year old , Joey has PPH.
Jennifer is a float nurse at Vanderbilt Children's Hospital,
in Nashville Tn. I used to be an Automotive Technician
before Joseph was diagnosed.
Joseph was diagnosed just before Christmas, a year and a half ago.
His presenting symptom was respiratory arrest. His pressures
were very high at diagnosis, and have climbed since to
approximately 140, but he looks very good, and has a lot of
fun. He has been on Flolan since diagnosis, and has been
on Tracleer for eight months.
We are very blessed in our medical care. Joey's current
primary care physician, who first saw Joey in the local
emergency room, knew something was unusual, and insisted we
follow up with him, two days later. Picked up several unusual
heart sounds, and packed us off to Vanderbilt immediately .
My guess is that there are not many posts for the same
reason it has taken me days to finish this response. lack
of time, and or mental or physical exhaustion. Also when
one has some time, it is probably wise to spend it in some
way that can let you pretend to forget about ph for a
few minutes (while you listen for a pump alarm).
I have an interest in how the PH ordeal affects other
siblings. MY 19 year old daughter at times is resentful
that everything revolves around Joey. At other times she is
worried and upset by his prognosis. She decided over the
summer to change her college classes to fit the pre-med
path. Joey's older brother is a super big brother, and
will follows Joey around a lot, playing four year old games.
He seems to take all of this in stride.
I think that this list is very valuable, as the folks on
this list have an understanding of this condition. It is
very strange to have your entire life controlled by a
child's medical needs.
We have noted that this list seems to get used only when
someone is facing crises. Because of that, we can be
happy when there are no posts, but this really limits the
usefulness of this list for people who are just getting
into this boat, who might see the inactivity on the list,
and decide it isn't worth writing an introductory letter.
This would be a shame, as there is a lot of hard won
experience on this list. Also it seems that many of us
have seen a long string of miracles, and grace has been
provided to allow us to recognize them even though they
have been surrounded by hard adversity.
Bill Nelson

Hi, welcome to the group. I think you find lots of help here, I know I have.

Wanted to say hello, read through posts. Tried searching for info
last fall when dx'd with this trouble, but never stumbled onto this
group before today.
I'll post my troubles and questions later when I have more time, but
I wanted to say hello and read some old posts first.

Hello Laurie,
My name is Pam Adams. I also have 3 children - ages
13, 11 and 7. In our case, my 13 year old daughter,
Becky, is the one with PPH as well as congenital heart
defects. She takes Tracleer and has done well on it.
I am really glad to hear that there is a Parent
Coordinator for PHA. I guess my worst frustration in
regard to other parents of PPH children is that it
seems hard to have very much contact with other
parents. On this list for whatever reason, the
parents don't seem to post very often. Maybe they are
too busy. On the message board on the PHA website,
there are hardly ever any postings. I am not sure
exactly what PHA or you could do about this, unless
perhaps there was a list started for people who have
the time and want to participate more regularly. Any
thoughts you have would be appreciated.
Thanks.
Pam Adams (Houston, TX)

Hello Everyone. I just wanted to introduce myself. My name is Laurie Jeter
and I am the newly assigned Parent Coordinator for the PH Association. I would like to give you a little bit of information about my families background. We have three children, ages 11, 9 and 7. Our 7 year old daughter was diagnosed with PH 3 years ago. She is on Flolan and took part in the Bosentan clinical trial last year through Dr. Barst. She is currently doing well on both Flolan and Tracleer.
I am interested in knowing how the Association can further help parents and families
with support. You can either comment through this list serve or email me
directly at mjeterl@.... The best to you all, Laurie

Please send me the details so we can send a card.
Gelene

From: lorimarque [mailto:Lorimarque@...]
Sent: Saturday, August 31, 2002 10:47 PM

What a blessing that things have gone so well for Victoria. We hope
her new lungs will allow her to experience a life blessed with good
health.Please keep us posted. Bill and Jennifer Nelson, parents of
Joey, age 4, pph

From: lorimarque [mailto:Lorimarque@...]
Sent: Friday, August 30, 2002 3:38 PM

Cathy,
Appreciate your response but I did go to the doctor as well and was
referred to a specialist last year but I decided to wait to see if it
would get better or not on its own.
This is definitely TOS however in my case it started out with the
tingling sensations for about a month or two in either arm and hand
and I lost strength esp. in my right arm/hand. My Aunt happened to
have something similar. It tends to get worse at night esp. if you
sleep the wrong way at least in my case. My Aunt had something
similar. I know this is also stress related however I am not
depressed or mentally ill. My Aunt recommended acupuncture and I
tried it and it helped a little but is obviously not a cure.
I could live with mild TOS but the problem is the Night Terrors which
I know are due to it. The only time I ever get jolted is because my
body must be releasing adrenaline to wake me up suddenly whenever the
blood to feed the nerves in my arms is pinched. I do not have
nightmares. I don't dream half the time. It's not like that.
Anyways, I am looking for others with the exact problem so please if
anyone has anything similar please post here. I found one person on
the Night Terror board who had the same thing happen and he described
his arm falling asleep somehow before his night terror occurred. In
my case, it feels like my arm is dead in every instance I get woken
up and it takes me awhile to get the feeling back and the tingles to
go away. This is not just sleeping on my arm because it happens when
I'm not sleeping on my arm and it is something which initially
happened nitely a year ago and go more spread out as time progressed.
I'm just looking for answers. I know not every case of TOS is the
same. Not everyone shares the same issues.
-Joel

From: lorimarque [mailto:Lorimarque@...]
Sent: Friday, August 30, 2002 3:37 PM

This is for Joel; from Cathy, Group Moderator...
I do not know if you have TOCs or not but the symptoms you have
of tingling are the beginning symptoms of TOCS or CTS. This
could be from computer typing, repetitive strain injury, all day
long. I recommend that you get a complete medical exam if you
suspect you have TOCS or CTS. A brief ER visit is certainly not
enough to diagnose or treat TOCS, however, you may be in the
beginning stages. I have not experienced any night terrors, and
based on my medical knowledge, this is not related to TOCS. I
recommend that you see an M.D. about this or at least a
Therapist, Counselor, or Psychiatrist. It could be related to
some meds you are on or it could be there is something bothering
you that you need to work out. You can tell you M.D. about it
first and see if he can refer you to someone or call your local
mental health hotline for info.

=====
Cathy

Hi, I'm new here. I'm glad this board is here because this is a
REAL problem. I'm a computer consultant in Chicago who types a lot
in front of a computer and plays the piano a lot but I don't have
carpal tunnel synd...
I was diagnosed with TOS last summer by an emergency room physician
who did the TOS pulse test and said it was the most conclusive test
he's ever done on anyone.
I didn't believe him however I believe I know how I got this. I
went to the gym and was on the row machine last summer after years
of not working out. I put too much weight on it and pulled the
nerves in my neck/arms somehow to cause this condition. Ever since
last summer, I have been having Night Terrors or Sleep Terror
Disorder as they call it. I wake up suddenly during REM screaming
(more like shrieking so hard my voice box hurts afterwards) at the
top of my lungs and feeling like I'm going to die. One of my arms
is always tingling either left or right, my heart races, and it
feels like the blood is gone and it is the scariest feeling in the
world and it seems I have little control over it. It takes several
violent minutes to get my heart racing to get the blood back to the
nerves in my arm (right or left depending on which side I slept
wrong on -- i guess my neck or arm or something is pinching the
nerves due to thoracic). My doctor told me to sleep on my back but
I tend to move around a lot at night. This is not a solution. Only
sometimes during the day do I feel tingling in the extremities of my
pinky fingers in my hands and I have some weakness esp. in my right
with loss of strengh since I'm a righty and it should technically be
stronger than my left!... last summer it was the worst with tingling
sensations constant but now it only happens at night when I sleep
wrong or have tension in my neck so it may have gotten slightly
better over the course of the year. (I stayed away from weight
lifting of course.)
Last night I had two terror attacks at 2 am and 5 am and if you
heard me yelling you'd think I was a mental patient from the movie
One Flew Over the Coo Coo's nest but I swear I'm a sane individual.
Maybe a bit stressed from work, but this has happened on and off for
a year now. My girlfriend and neighbors are starting to despise me
ruining their sleep and scaring the bageebees out of them not to
mention how terribly embarrassed I am that I do this seemingly every
other night. Sometimes I'm afraid to go to bed as if I'm in some
scary Nightmare on Elm Street film!!!
Now I realize that this condition is not going away. It is a
PERMANENT problem that I need fixed despite my reservations for
doctors and surgeons esp. neuro and vasular surgeons. I don't want
surgery or someone to screw with my nerves but I do need help
badly. I haven't slept well in over 1 year. I am now tired all the
time and I am agitated and nervous a lot and stressed due to bad
sleep patterns caused by this TOC!! Only for brief periods has it
seemed to disappear only to reappear again and again.
I am here on this board a bit embarrassed to be honest but looking
for answers to what I now see is a more common injury than I
originally thought and it totally affects your life and most doctors
have no idea what this is.
If anyone has been experiencing these Night Terrors with symptoms of
TOS, please let me know. I hope I am not the only one here looking
for answers and thinking I am having mini-strokes or mini heart
attacks at night which I'm not. I'm only 28, my heart is fine, my
mind seems fine, and my doctor took X-rays and says circulation is
fine, spinal structure is fine, etc, etc. but that I may need to
lose a little weight. I have very slightly elevated blood pressure
but nothing major. This is a physical dilemma!!!
Good luck to all searching for answers. Please email me if any of
you have anything similar to me and if anyone has a solution or can
refer a specialist somewhere in the US.
-Joel
Chicago, IL

It has been a while since I have posted to this group. The school year started without incident. Now that Victoria is in a new middle school I expected to have to start the fights all over - we were so pleasently surprised. The principal is wonderful and actually knew the law!!!

We got "the call" for Victoria on 8/20. She had her double lung transplant. She has been in transplant housing with us a couple days. Unfortunately no medications worked for her and her pressures stayed dangerously high. She is a new person now. No more pph and so far no problems with the new lungs. Life will definately be different. We were told that this tranplant went easier then any they have done and she was out of the hospital in less then 2 weeks. We were also told that Victoria was alive by the grace of God - this we knew - we also know that by the grace of God this new life will be long and healthy.

Gelene

Victoria's mom former 11 yo with pph - now double lung TX

Hurray for Victoria!! Congratulations on her new lungs. I am so glad things went well.

Michelle M-W (Rayni's Mom, 5 years old, SCIDS, 4 years post cord blood transplant, pulmonary fibrosis, secondary pulmonary hypertension, on double lung transplant list at Childrens Hospital in St. Louis, zantac, septra, predisone, IVIG, calcium, oxygen 24/7, j-tube 10 hrs/day, flolan)

Dear Sunnie,
Iwas very happy to read that you and Trey had a good experience in Denver.
Sounds like your on the right track with getting him feeling better. We
have all been wondering how things went and how Trey is feeling. thanks for
sharing with us. Take care (Maddie's mom)

Hi,

Thanks to everyone for all their support. Denver was able to put Trey back together again. We were super happy with the care we received. All of Trey's 4 meds he takes were out of whack! Most of them were still at the same rate as when he was 3 y.o. and 28lbs. His pressures have dropped from 130 to 82. And the pressures were only 40 when he was on Oxygen. After 4 days in Denver he felt good enough to go without Oxygen and we went to the baseball, soccer games, and 2 parties and the museum. Dr. Ivy said "Hi" back to all those that e-mailed me and said "Hello" to him, Maddie, Alex, etc. Things are good!

Sunnie and Trey ( 9 y.o.)

Hi Bill,
Yes, I noticed it is very quiet on the list, too. I
am glad to hear that Joey is doing well.
Hopefully, some more people will post again soon.
Pam (Houston, TX)

It has been very quiet on the list. I hope that
means every one is doing well.
Joey has been doing well. The site of his new
central line looks good.
We have decreased his flolan rate a second time, due
to muscle aches and restlessness at night, which Dr Doyle
believes may be a side effect of the combination of
Traceleer and Flolan. The decrease in the flolan rate has
had no effect on his energy levels or his color. Those
symptoms showed up after seven months on Tracleer, and
reducing the Flolan rate cleared them up.
It has been cooler here finally, Joey actually got
to play outside in his sand pile on Thursday, we may let
him play outside this afternoon. He has open house for his
preschool on Sunday, and his preschool starts on Tuesday.
He also has a appointment with Dr Doyle on Tuesday
afternoon, so He will be a busy boy this week.
Has anyone heard from Sunnie ? She and Trey have
been in our thoughts and prayers. It takes a long time to
deal with line infections, and it must be tough a long
way from home
Bill and Jennifer Nelson

Shawna:
I had venous TOS, which is a very RARE form of TOS. I had my left
rib removed in October and my right rib removed in February of this
year. My doc did a transaxillary resection (the scar is at the
bottom of each of my armpits). I had absolutely no complications,
and the pain was not nearly as bad as I thought it would have been.
I had had blood clots in my left subclavian, brachial and cephalic
veins back in 2001. The docs who took care of me had no idea of what
caused it. I did research on my own (having been a medical
transcriber) and heard about TOS. I then found a doc who specializes
in it. He was the first doc out of 8 to confirm my suspicion.
Because I had VENOUS TOS, I had very little pain with my TOS. Do you
have any pain? How did your TOS present itself?

this is for Liz....You can really aggravate that TOCs without
knowing it. You have to find a new way to do things. I put 24
begonia plants in the yard but I only put in two a day and it
took me 2 months but my yard looks great. Over time you will
learn what aggravtes it. IF IT HURTS...OR BEGINS TO HURT STOP.
Most likely it was the weeding you did that set it off.
Sometimes just sleeping the wrong way or turning the wrong way
can set it off. Somedays are stable but if I ever begin to even
think hey its better; boom it hits me. I know it is there
sometimes it is just a little be more asleep until it WAKES UP
AND ZAPS ME. A lot of living with TOCs is sometimes an hour at
a time and it is always a day at a time. YOu don't know about
work until it is time to do it. There is no way to know but if
you worry and get yourself all stressed about it your mucles and
tighten up and put more pressure on the nerves. I am so sorry
to tell you that this is TOCs. I can only say that I am glad I
started this group for you and be sure to know you are not
alone, we are all in the same boat with you. What is inside
your head will have a great deal to do with living with TOCS.
YOu have to be determined and you have to find new ways to do
things. I have to break everything I do down into small jobs...
and find another way to do it. Be patient with yourself as this
is all new to you and; it may get better, who knows.

=====
Cathy

Shawna,
I also have the same problem as you and have wondered about having the
surgery. I was hurt at work and also had 4 disc removed in my neck and have TOS.
If
you get any e mails from anything that has had the surgery would you please
forward them to me also. I'd appreciate it, I heard it is a hard surgery, don't
mean to scare you but best of luck to you. With mine I am also having
soreness over my left chest do you ever get that in the top of your breast area?
I
just had heart surgery and they put in a stent and don't know now if it is pain
from TOS or the surgery. Best of luck to you!!!!
Thanks, Rose

Best of luck with the line replacement. I know the center in Denver
has a good reputation.Sometimes our PH nurse has contacted them with
questions. I hope all goes well for Trey and that you are able to
find some good primary care for him when you return home. You need to
have a good pediatrician who will work with your PH doctors, and not
pretend to know everything, and will be available when you need him
or her. We are blessed to live one hour's drive from Vanderbilt
University Medical Center, where our son's pediatric cardiology team
is, but we have had several emergencies where we have roused our
pediatricians in the middle of the night and met them at the
E.R.Sometimes these emergencies can be handled at our local E.R.,
with the pediatricians consulting with our cardiologists, and
sometimes we have been transported to
Vanderbilt by ambulance.Our pediatrician has insisted that our son
never see an emergency room doctor, and has discussed him with the
seven other pediatricians that share call with him.Without good
primary care it is difficult to access the medical system rapidly
when you need it.PH is life threatening, so you don't have time to be
messing around with people who are unfamiliar with this disease or
your child.
We were quite lucky to have found our pediatrician. Joey's
pediatrician had retired from private practice before he was
diagnosed, and the pediatrician I had chosen for him was out of town
the night that Joey began having color changes and we wound up in the
e.r.We wound up seeing his current pediatrician, who happened to be
on call that night, and recognized that something unusual was going
on. After we wound up at Vanderbilt, the pediatric cardiologists all
but insisted that we continue following up with his current
pediatrician, rather than the one I had originally chosen for him.(I
would not have been able to choose his current pediatrician had we
not wound up with him in the e.r. as he already had an established
practice and was not accepting new patients.)We have been very
pleased with our primary care doctor, and find that he works well
with our cardiologists, and that our cardiologists trust his
assessments.
Perhaps the people in Denver could recommend a pediatric cardiologist
or other specialist in your area that they work well with, and you
could then get a recommendation on a good pediatrician or primary
care doctor.This is going about it backwards, but you need to have a
whole team that is familiar with your son.My son's pediatrician wants
to see him on a well basis in between our cardiology appointments,
and we keep these appointments. That way if we have an emergency, he
is familiar with Joey's baseline status and quick to detect
changes.Best of luck. You will be in our prayers. Jennifer Nelson,
Joey's mom, age 4. pph, flolan, tracleer, pepcid, lasix, digoxin

I went to my Dr. last week for another follow-up, and he said that he
thinks surgery will be my best bet. I have Venous TOS, so there
really is no other alternative. First he was talking about removing
my clavicle, but luckily he changed his mind and decided he is going
to remove my first rib. He is going to do this by going in through
my neck. Has anyone had the procedure done this way before? What
can I expect? I am not having it done until next month because we
are going on vacation at the end of this month.
Hope all is well for all of you!
=)
Shawna

Hi I was wondering if anyone can give me some advice.
Last weekend I was actually pain free, I really thought I had cracked
it and that I was going to get back to work. I felt so good I did
some weeding