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Kim,

I'm sorry, but I have to go along with your "it isn't right" viewpoint.

How does your cardiologist/pulmo explain the adults and/children that suddenly have primary pulmonary hypertension for no known reason? If you posted this on the general discussion board of the PHA, you will get all sorts of responses from people who didn't take diet drugs, and suddenly had PPH in their 30's, 40's or 50's.

What I would do if I were you is to get the drs. there in Dallas to send a copy of all the testing they did to Dr. Ivy in Denver, CO. Even though your insurance company isn't willing to pay for his services, he will give you an opinion of her medical records for free.

I know this for a fact because I did it previously before I won the appeal with our insurance company to cover Dr. Ivy. It helped a lot in the peace of mind department and being able to know what was the best thing to do for my child.

Best wishes.

Pam (in Texas)

kaclimer <kaclimer@...

Again this was posted on the General message board so forgive me for
repeating myself.
My daughter had her cath done yesterday at Children's Medical Center
here in Dallas. Since it was going to take awhile to get into the
clinic in Houston and insurance wouldn't let us go to Denver, we
decided to have it done here using the Houston clinic's protocols.
These protocols were based on the medication challenge part of the
test. After it was done the doctors came out and the cardio told us
that she hadn't done the final computations but Autumn's pressures
were in the "high" normal range. And even though Autumn has pretty
severe lung damage, she is fine from a cardiac standpoint. When I
asked if the pressures could still rise since she does have pretty
bad lung damage, the cardiologist said that if she were to have PH it
would have shown up by now. People just don't develop PH later in
life unless they have taken diet drugs. Once she left I asked the
pulmo about the anethesia. Since Autumn's cardio/pulmo exercise test
shows us that she is severely effected by extertion, I wondered if
things change when she is up moving about. He said that the cario
added 100% oxygen during the test to see if there were any changes
and there weren't so that told them she was ok with exertion.
Let me remind you that this was done at a hospital that does not
specialize in PH. As a matter of fact, the anethesiologist told us
that this was the second RHC of this kind he had seen. The first one
was last week with the same pulmo. So now if the anethesiologist has
only seen two, then you know the cardiologist isn't very familiar
with them either. I did ask before the test was done if sedation
would effect it and they told me that they do not do caths on
children without putting them to sleep. I can't help but think the
anethesia could have changed things for her.
Help me straighten this out for me. According to this cardiologist,
all of the adults with PH either had it at a very young child
(younger than 11), or you took diet drugs. I don't get it. Now mind
you, my husband was totally excited to hear this news! And I want to
be excited too. But there is something inside me that is saying "it
ain't right". I want nothing more than to put PH behind me, but I am
afraid that even if Autumn is truly in the "high" normal range now,
if we ignore it, it could rise later due to her lung damage.
The pulmologist did say he would send her to Houston for a second
opinion. I don't think we can swing another RHC with the insurance
but at least he can go over all the reports and look at her himself.
Kim in TX
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Again this was posted on the General message board so forgive me for
repeating myself.
My daughter had her cath done yesterday at Children's Medical Center
here in Dallas. Since it was going to take awhile to get into the
clinic in Houston and insurance wouldn't let us go to Denver, we
decided to have it done here using the Houston clinic's protocols.
These protocols were based on the medication challenge part of the
test. After it was done the doctors came out and the cardio told us
that she hadn't done the final computations but Autumn's pressures
were in the "high" normal range. And even though Autumn has pretty
severe lung damage, she is fine from a cardiac standpoint. When I
asked if the pressures could still rise since she does have pretty
bad lung damage, the cardiologist said that if she were to have PH it
would have shown up by now. People just don't develop PH later in
life unless they have taken diet drugs. Once she left I asked the
pulmo about the anethesia. Since Autumn's cardio/pulmo exercise test
shows us that she is severely effected by extertion, I wondered if
things change when she is up moving about. He said that the cario
added 100% oxygen during the test to see if there were any changes
and there weren't so that told them she was ok with exertion.
Let me remind you that this was done at a hospital that does not
specialize in PH. As a matter of fact, the anethesiologist told us
that this was the second RHC of this kind he had seen. The first one
was last week with the same pulmo. So now if the anethesiologist has
only seen two, then you know the cardiologist isn't very familiar
with them either. I did ask before the test was done if sedation
would effect it and they told me that they do not do caths on
children without putting them to sleep. I can't help but think the
anethesia could have changed things for her.
Help me straighten this out for me. According to this cardiologist,
all of the adults with PH either had it at a very young child
(younger than 11), or you took diet drugs. I don't get it. Now mind
you, my husband was totally excited to hear this news! And I want to
be excited too. But there is something inside me that is saying "it
ain't right". I want nothing more than to put PH behind me, but I am
afraid that even if Autumn is truly in the "high" normal range now,
if we ignore it, it could rise later due to her lung damage.
The pulmologist did say he would send her to Houston for a second
opinion. I don't think we can swing another RHC with the insurance
but at least he can go over all the reports and look at her himself.
Kim in TX

Hi kim,
That is how things happened when my daughter Madison had her heart cath,
unfortunately, her pressures were high and unresponsive to everything they
tried, they kept her as an inpatient and started IV flolan, 3 weeks later
she went home with an IV that she has had ever since, that was six years
ago. But your understanding is correct, if her PAP is ok or she responds to
oxygen or Calcium Channel Blockers she could go home the same day of the
procedure. Good luck, I hope all goes well!
Lori Heaton (maddie's Mom)

Hi everyone! I am new to this egroup but not new to PHA. I posted
this on the PHA message board so forgive me if you read it twice. My
daughter, Autumn is going in tomorrow for a RHC. I just talked to the
cardiologist that is going to do the procedure. She said that from
Autumn's cardiologist's report (the echo) Autumn may not have
elevated pressures. But from the pulmologist report (the cardio/pulmo
stress test) she very probably does. That gives me some sense of hope
but I want to remain reasonable.
Tell me if this sounds right. The cardio today said that they will go
in through the groin and into the right heart. If the pressures are
normal and there is no damage done to the vessels, they are finished
and Autumn will be home that same day. If they go in and the
pressures are up the will start by giving her 100% oxygen. If she
responds they are through. If she doesn't, they will then give her
NO. How she responds will determine what course of treatment she will
have. If she does not respond, she will be admitted into the hospital
and started on Flolan.
Does that sound reasonable? I am still holding onto that she might
have normal pressures. The doctor wouldn't give me an opinion on way
or another. She did say that she had a boy in last week that had
normal pressures and the pulmo started him on rehabilitaion.
Thank for your help,
Kim in TX

Hi Lori...this is Celeste mother of Noel.

I was interested in the information you provided on the list about the testing and supplement for Madison and how her health has been improved...

I tried to email you personally and the post came back undeliverable...

I would really like to hear from you regarding this subject....and how it has helped Madison's health...thanks for any info you can provide...much love celeste

Celeste ... sorry that it took me so long to answer you ... this has been a busy week ... tomorrow is Heath's birthday and we are planning his party for this weekend so I have been running around a lot and not checking my mail ....

No, Heath is not on Treacleer ... two months ago in the Hospital they inserted the cardiac cath and did the Flolan test and he reacted so well and so fast (the fasted ever at Eggelston Childrens Hospital) that they felt that something less evasive would work ... so they put him on Nefedipine ... Treacleer was going to be the next step if Nefedipine did not work ... But this seems to be doing the job ..WOOHOO ...The draw-back is Flolan is so fast and the Nefedipine takes longer to work but after 2 months of taking it 3 times a day his pressures have dropped from around 98-100 down to 44-46 ... Nefedipine is taken orally and not with the lines and pump that Flolan has ...
Check out my little man
www.babiesonline.com/babies/v/vernonheath/

Heath's Update Group

Hey everyone,
I've found a supplement that can clinically be proven to improve your immune
system. Madison has been on it for about one year. She has not had a single
cold or flu bug in that time! (having a cold always increases her pulmonary
pressures). The antioxidant levels are measured with a non-invasive scanner
in the soft tissues of your hand, it is so cool. Maddie scored amazingly
high! If anyone is interested in knowing more e-mail me
heaton_lori@... Take care Lori Heaton

Dear Celeste,
I am sorry to hear about the very rough time Noel has been having. We
will keep you in our prayers.
I have also been at the receiving end of a very frustrated teen. Our
daughter, Emily had a hard time with our son Joey's illness and death.
In fact, I think she had the hardest time of any of us.
Emily was 17 when Joey was diagnosed and 19 when he died. She was a
senior in high school, with the typical teenage complaints of wanting
more freedom, a car, etc. She also wanted to be pretty and popular.
Then her little brother comes along with a serious illness and takes
most of her parents time and attention.
Emily wanted to help out as much as possible. But she also wanted to be
hanging around with her friends, not babysitting a two year old on
flolan. She loved us. But hey, she was a teenager, sometimes she didn't
even like us.
She wanted to go away to college and we wanted her to go. But she felt
guilty for wanting to leave. When she complained to her friends, no one
was very understanding, and most of them thought that she was
exaggerating the seriousness of her brother's illness.
But I think the thing that bothered her the most of all was the
unfairness of it. Not just for her, but for us and Joey as well. And
that is what bothered her the most when Joey died.Young people are
idealistic and they want life to be fair. But it is not.
So Emily was very angry at times about the whole situation, and she
would lash out at us, and at me, in particular. This, of course, was
very unfair to me. Finally I told her that I would find her a couselor
if she needed one but that I was unable to cope with her anger, because
my plate was already full caring for Joey. And I just refused to listen
to her and be sympathetic.
A family friend who is a pediatrician agreed to meet with her and they
talked one evening. I don't think that was a magic bullet but I think
she did get a sense that her parents were also hurting, as well, and
there was only so much we could handle.
I'm sorry to take so long to respond to this but we have been on
vacation and offline. I hope things are going better for your family.
Jennifer, mother of Joey, deceased, age 4 PPH

Wow this is great news Tracy...Heath is on Treacleer am I correct?

being the mother of a daughter with severe pph and eisenmenger..I just want you to know how happy I am for Heath and your family...much love celeste

OK, ya'll I know that I went on and on about the great Dr. appointment and Heath being able to remove his oxygen today but I just had to tell ya'll about this afternoon when we got home .... For the last couple of weeks Heath has been standing up and taking a couple of steps but that is all he would do ... at the most he would only take four steps and that was wobbly ... well since we got home around 3:00 today until now that child has not stopped walking .... I can not get over the difference it has made in him by removing that stupid tube that was always dragging along behind him ... he is standing up and walking 10 - 15 feet over and over again ... and the steps are so sure and even ... no more wobbly ... I am just so excited ... he has made more progress in just a few hours than he made in 2 whole weeks .... I can not imagine what he will be doing in a week ... thanks for letting me brag one more time ... I am just so amazed that I had to share it ...

Tracey

Check out my little man
www.babiesonline.com/babies/v/vernonheath/

Heath's Update Group

Hey Everyone
We just got home from Heath's Pulmonary appointment .... and GUESS WHAT !!!!!!! ... I am looking at the sweetest little face with no oxygen tubes at all ....WOOHOO.... the Doctor was so pleased ... they removed the oxygen and tested his saturation for 10 minutes and Heath maintained a saturation level of around 95% so that is great ... right now he can go without the oxygen all day and even when he takes a nap as long as it is not longer than 2 hours ... and we do not have to go back to the Doctor for 3 whole months .... I was shocked at that because I really expected to go back in a few weeks but he said that I could if it made me feel better but there was really no need as long as we just kept our eyes out for anything that seems out of sorts ......... It is just amazing how different Heath is acting already ... he has been running all over the house wide open and just gets so excited that he will look at me and start squealing and clapping ..lol... I guess I am going to
have to make some adjustments myself ... before if I left the room I could come back and find him pretty quickly by just following the tube but a few minutes ago I went into the laundry room and came out and kind of freaked cause I could not find him right away (probably all of 30 seconds but it felt like forever) ... he was just behind the chair but I actually had to call him and look around ... guess I better get my old skates out so I can keep up ... Heath's 2nd birthday is next Thursday and we are having a big party next weekend with a clown and everything ... I think he deserves it after the year that little one has been through ....

Thanks to everyone that has been calling and sending notes to us .... it has meant so much to know so many people care ... I will take some pictures this week and let ya'll see how great he looks now ...

Hugs
Tracey, Keith and Heath

Check out my little man
www.babiesonline.com/babies/v/vernonheath/

Heath's Update Group

Hi Bill...great news....we see Liz Mahoney at the support group in burlington, vermont....
we are in upstate NY...my daughter is 19 yo now but one of my fondest wishes is for her to meet someone more in age and with similar problems....
we haven't found anyone up north who does have similar problems....but I think this support group meeting was a great idea....
much love celeste...mother of noel.....19 yo... tracleer and remodulin

About a week ago I asked if anyone was aware of a support group geared to children with PH and their parents. Yesterday Accredo held such a meeting in its Massachusetts office. A special thanks goes to Liz Mahoney for setting this up.

From a parents perspective it was well worth the hour ride to attend. Four families gathered. We shared our experiences, compared notes and enjoyed each others company. It was great to be able to put faces to people we have interacted with over the Web. Just being with people going who are in the same boat was worth the trip.

As far as I can tell, my daughter also so enjoyed the experience. She was a few years younger than the other children, but that did not stop her from interacting with them. All of the children seemed to be enjoying themselves. When I asked my daughter last night what she thought of the meeting, she said "It was cool to be with other kids who have `halos'." ("Halo" is what she calls her pump because angels have halos.)

For me the best part was hearing other parents express the desire to meet on a regular basis. In fact plans are under way for a fall meeting. I know my family will be counting the days until we meet again. In the meantime there is always the web.

Bill

Dear Friends

Please click on the following link for this months edition: http://homepage.ntlworld.com/als.king/

Love and best wishes from Louise xxx

wow....Washington state sounds good...

we are in the northeast..northern NY and where we are now Noel has no socialization.....she is 19 yo old and her friends are all out partying and experimenting......they call her and tell her and she is starting to get more angry...

sometimes when one calls....who has been her best friend she will tell me to tell her she is sleeping...so she doesn't have to hear what she has been up too....

were you already planned to move to Washington or did you do this because it was so bad where you were and after what happened at the hospital?

this is why we plan to move...we do not take our daughter to our local medical center as they know nothing of ph...we learned from experience. we always have to drive an hour and a half to two hours to burlington vermont...for all care..

and I know what it is like to have an emergency and have to get out....wow it sounds like you had some super neighbors...where we are now...back in the woods....we are lucky to even get our regular road out plowed once a day...

we plan to sell this place in the spring and let Noel graduate from her school.....and then we are off....

much love celeste.....Noels mom

This morning I received a copy of a letter that Nicky's consultant
paediatrician has sent to our GP saying that that Nicky's last blood
tests showed HB 22.1 and PCV 65.6 and is boarderline for treatment.
Can anyone tell me just what these numbers mean and what the
treatment might be. He has a complete AV canal defect, PH, chronic
lung disease, gastro problems. and D.S. He will be sixteen next
Friday.
Pauline UK.

Dear Pam,
We've all been basket cases at one time or another. Sounds like you get
through the leaking catheter crisis o.k., in so far as Becky's health
is concerned. If you are like me you get through the crises o.k., then
its time to schedule a little time for the emotional breakdown.
You have to prioritize. When our son Joey was born, I just couldn't
keep up with the kids activities schedule. (That was before he was
diagnosed and we thought he was healthy). Emily was fourteen and
wanting to participate in all kinds of afterschool activities. But I
just wasn't able to cart around a breastfed newborn with me for her
sake. I was still a postpartum mother and I had to get some rest
sometime. And when I returned to my job working nights it just got
worse.
So she only got to participate in one activity a week on my day off. Or
else she had to get a ride from someone else's mom.
She didn't like it, and accused me and her little brother of ruining
her life. But the kids adjusted. I really don't think all the
activities kids do are that essential to raising well adjusted adults.
After Joey was diagnosed, the activity restriction became more severe.
No activities unless you get your own rides. This was not a problem for
Emily at this point, as she could drive and had a car available for her
use. But when she went to college, her brother kept arranging his own
rides. Forrester had grown up accustomed to those restrictions. In
fact, he has continued to do so after Joey's death, even though we are
quite capable of giving him rides. But his best friend had an older
sister who drives, and they had worked out a plan together, and he
preferred not to rely on us.
Fortunately, we had joined the YMCA prior to the diagnosis of Joey's
illness, as had some of our children's best friends. We were able to go
to the YMCA together as a family and invite some of the kids best
friends. Then they would get to the Y, run off with their friends and
ignore us. The nursery at the Y was a little freaked out by having a
child on flolan at first. But part of their mission is to minister to
adults and children with disabilities. I found a young women who worked
on the nursery on weekends whose younger brother had transposition of
the great vessels, and was not freaked out by having Joey in the
nursery. We took portable radios so that the nursery staff could summon
us at any time for a pump alarm or question.
Working out regularly was one of the main ways I coped with the stress
of Joey's medical demands and has been one of the ways I have coped
with losing him. I know it seems counter intuitive to exercise when one
is already so tired. But it helped me keep my sanity, and it is one of
the few things that helped give me the energy to go back to work after
his death. It is hard to get started, and particularly after Joey's
death, I didn't really enjoy it. But I didn't really enjoy much of
anything else, either, and I needed something to fill up that empty
time. And there is a certain release of stress that one gets from the
endorphins produced after working out. Besides, if you are mad at the
world it is so much easier to put some headphones on with loud rock
and have a go at the rowing machine.
Our YMCA has a room for teens that has computers, a pool table, ping
pong, games, etc. so there are different activities for kids to do if
they don't want to or can't go swimming.
Raising kids is not easy, but you have to take care of yourself.
One of my good friends wanted to know what she could do for me after
Joey was diagnosed. And, since she is also a member of the YMCA, I told
her she could exercise with me.It is easier to go if you have someone
else to keep after you to go. So she did.
Now her husband, who is a helicopter pilot with the 101st Airborne, is
in Iraq. And it is my turn to keep after her.
But I have learned a few things from her.
She has two very active sons, but she limited their activities after
her husband was deployed. She says she has learned that their are some
things that it is just too hard to do when her husband is gone, so she
just doesn't do them. Her marriage has survived the first gulf war and
a long separation when her husband was stationed in Korea. So I think
that must be pretty good advice.
Occasionally, I still get some flack from Emily about how she wasn't
allowed to do various activities she wanted to participate in because
she didn't have a ride.
But that is very rare now that she has gotten older and more mature.
So don't feel guilty if you have to limit the other kids activities.
And you might want to consider starting some kind of exercise program
if you don't get any regular exercise. But there isn't any magic bullet
that makes all of this stuff easy.
Even prayer doesn't make it easy but it helps get you through.
Jennifer, mother of Joey, deceased PPH, age 4, and Forrester, age 15
and Emily, age 20

Pam,

Been there, done that, bought the T-shirt, Twice!!!. Trey's last hole in his broviac came at 2 AM when we lived in rural Maryland. I had the Flu so bad I had to craw to his room when he called me. Acreedo was NO HELP and said they couldn't tell me how to do a change over and to get to the ER asap. So we open the front door and there had been a snowstorm with a foot of snow and no snow plow. We woke up the neighbors, all 4 houses and they helped to shovel the 1 mile road, all up hill, to the highway. We raced to D.C. National Childrens' Hospital, (through unplowed roads and deer season) only to find out that they don't do Triage between 2-7am. So we sat in the waiting room with kids barfing and one child bleeding a puddle of blood and waited. At 5am I bought the janitor, who was sweeping, a cup of coffee and he triaged Trey and put us in the back room. I fell asleep against the hospital bed after the doc did a 3 minute assessment and left us. At 7am they woke me up to say
Trey had been missing since 5 am and did I know where he was. Trey figured he wasn't being treated so he left and found a empty waiting room with a TV. The fixed his line and then asked me What the heck is Flolan anyway??? 12 hours after we got home the moving company came and moved us to Washington State. Made me wish we were still in Houston. One other thing. We joined Starlight organization. They are kind of like Make A Wish but the have something called KAN. Kids Area Network. They have fun things to do a couple times a month. Trey loves getting together with other kids who are "sick" and being normal. They have tons of things. The kids in DC got to eat at the White House and have a special tour. We went to a dude ranch for a day. Tonight we are leaving for Seattle for a Sleepover. They rented out the Embassy Hotel and Nintendo is having a video party, We have gone to Broadway style shows. It is all Free. Although we don't participate often it is nice to know we can if
we want to. It is also nice to get together with other parents and kids in the same boat as our kids.

Sunnie and Trey

Hello everyone,

If you are hoping to read an upbeat e-mail, this probably won't be it.

This morning my daughter, Becky's Flolan catheter was a little loose from the connection and started spouting blood on her clothes and the floor, and scared us both today before I got it corrected. Ironically, this comes right as I am getting ready to go see the psychiatrist that I see for me.

Then this afternoon just as I finally am laying down to rest for awhile before dinner, Becky comes running in telling me that her Flolan line is leaking. It had leaked all over the bottom half of her shirt. My husband, John, finally found the leak - a small hole in the catheter itself!

After talking to Aimee, one of Dr. Ivy's nurse pracititioners, we found out she had to be taken to the emergency room of Texas Children's, be put on an I.V. to get Flolan temporarily, and then they had to repair the cathether line. My husband took her, thank goodness. Anyway, he has called me and she is o.k. other than being mad at the I.V.

In the meantime, I find, as I think I've said here before that ever since Becky has been on Flolan, I am always tired, to the point of having to either get a nap in the afternoon at some point to get by or else struggling on the days I'm at work and can't get one.

The psychiatrist has me on a mild sleeping pill to make sure I sleep soundly enough, was on one antidepressant, and now he has added another one to see if it will help with the tired business, since the one antidepressant and the sleeping pill together didn't do it. Now I am getting scared how I am going to have enough energy when my other two kids start school in 3 weeks, and there's homework, and activities, and driving back and forth to school!

Sorry to sound like a basket case, but somehow I seem to have turned into someone who isn't the me I know.

Thanks for listening if you were able to get this far.

Pam (mom to Becky - 14 - severe PPH, congenital heart disease; scoliosis, ADD, anxiety disorder, depression, etc.; Johnny - 11 and Laura - 8

I hope that this OK to do here but I wanted to let the Georgia families know of a new group for friends and family of persons with Downs Syndrome .... I am not advertising this to take away from this group by any means but I think that we can never have enough information or support ...

This is a new group and just getting on their feet ... you can apply to become a member at

Congressman Offers Support Because Of Constituent's Request
PHA is pleased to announce the most recent co-sponsor for the
Pulmonary Hypertension Research Act of 2003 (H.R. 1316), this time
because it is a reflection of the hard work and dedication of the PH
community. Congressman John McHugh of Minnesota signed on as a co-
sponsor of H.R. 1316 shortly after receiving the request from a PH
patient, Celeste Daniels.
The growing support for H.R. 1316 (the number is now at 11)
demonstrates the power of our collective voice. As the Advocacy and
Awareness Manager for the Pulmonary Hypertension Association, I
continue to thank you for your efforts to build congressional support
for this legislation.

Bill,

If you should discover a group like that, I'd like to hear about it. I have never found one.

Pam (Houston)
"O'Donnell, Bill - ITSD Desktop" <odonnellw@...

Accredo has set up a pizza party for children with PH and their parents. I am from the Boston area. Does anyone know of a support group out there geared specifically for children with PH and their parents? There is a possibility that Accredo will turn this into a regularly scheduled group meeting.

To unsubscribe from this group, send an email to:

Accredo has set up a pizza party for children with PH and their parents. I am from the Boston area. Does anyone know of a support group out there geared specifically for children with PH and their parents? There is a possibility that Accredo will turn this into a regularly scheduled group meeting.

We've Hit Double Digits!
Two More Co-Sponsors Bring Us to a Total of 10!
We are half-way to our goal of 20 co-sponsors for the Pulmonary
Hypertension Research Act of 2003. Congressman Dennis Kucinich (OH)
and Congresswoman Grace Napolitano (CA) are our newest supporters.
With nearly 1,000 letters from the PH community circulating the House
of Representatives, the message is spreading. Keep up the good work!
Let's continue to call on our representatives to support this
important legislation.
Reminder: If anyone is interested in visiting Capitol Hill to lobby
for H.R. 1316, or you would like information about how to visit your
member of Congress in person, please call Cara Ugolini at (301) 565-
3004 x113.

Hi Bill, rop
Wow! That is a dramatic drop in pressure, you must be thrilled! It's nice to
heat news like that,congratulations! take care Lori heaton (mom to Maddie,
age 10 is on flolan, tracleer and hopefully sildenifil when her insurance
approves it)

My daughter Shannon returned home from Children's Hospital Boston yesterday. She had a right heart cath done on Monday. The cath showed that her pressures are way down. Her pulmonary pressure is down to 26 and her pulmonary resistance is down to 3. Two years ago when she was diagnosed her pulmonary pressure was around 100 and her pulmonary resistance was 29. Her doctors are amazed that her treatment is working so well.

Shannon is on Flolan, Nifedipine and Coumadin. Her doctor, Mary Mullen, increased her Nifedipine to 30mg a day using and extended release pill.

The Flolan dose has not been increased since last October. Since she has grown since then her dosage has actually gone down. We decided to wait a while before lowering her Flolan, but we hope to be able to in six months.

I hope others will also receive great news like this during the summer.

Bill O'Donnell

Celeste,

My heart aches for you. My daughter, Becky, who is 14, hasn't yet gotten that difficult to be with. However, she tells me she wants to be rid of "that box" (Flolan), and that it isn't fair that she can't swim. She went to a swimming party the other day, and came home telling me "I am not going to another swimming party until I get rid of this box."

Then one day she started telling me she wanted to start taking ballet again and was really insistent about it. That would be fine, except that she still spend half the day breathless and has to have a nap in the afternoon to get through the day. I know, like your Noel, she just wants to do normal stuff.

It makes me feel sad for her, and of course, like you, there is very little that I can do about it, other than to love her and take the best care of her that I can.

Maybe you could get out of the house for a couple of hours and do something you like, maybe that would at least give you a little break - sometimes I just go to the bookstore and poke around because I love books.

Take care.

Pam (mom to Becky - 14 - congenital heart defects, severe PPH, scoliosis, polysplenia, ADD, anxiety disorder, etc.)

pyrfect <pyrfect@...

Hello....

this is celeste ...Noel's mom....

well.....we went through two or three weeks of Noel not being able to walk....

the card said it was gout and had her on some pain med that started with an i.....and now on allopurinol..

she has had gout before and always has knee pain for at least....well for yrs. anyway..

now ...it seems as if ...whatever is wrong with her leg is back...

she is at the limping and hoping point now..sometimes she cannot move her leg...her foot is not as swollen as it was before yet but ...I hate to say this....her foot is very swollen and red purple...her ankle is swollen and her knee....the other foot is a bit swollen...the last time it was so swollen that her toes looked like little stubs...it hasn't gone that far yet...

she is getting mean....

wow...i hated to say this...

but has anyone else been at the recieving end of a very frustrated teen....Noel can get mean to me but now she is also getting meaner with others...

last night I spoke to her about it and she said she can't stand it anymore....everyone else can at least walk...she said....look at Warren...my 4 yo grandson...she said....he can run all over and he feels good...

I can't even walk.....she tells me she dosen't want to be mean but sometimes she just can't help it...

I feel it has been hard for me lately...all the feelings....not being able to make things any better...

although ....the card checked her heart by echo the last time and it is looking better since the addition of remodulin...so this was wonderful news....but now its her leg...

sometimes she cannot even move it....and it was so bad last time we ...anyone could not even touch it...

has anyone else had to deal with any similar issues......has anyone else experienced their child going through this...

I know it must all be part of the exceptance of the disease...and the coping is hard.....and it is hard for the parents.....

sometimes I cry...much love celeste

mother of Noel...

pph, eisenmenger, congenital heart defects, ds, legally blind...

currently on tracleer...125 mg 2x/day

remodulin....10%..... 45.5

pepcid....20 mg

synthroid......allopurinol.....O2..which she will not wear anymore due to peer pressure...unless she is really ill....

oh yes and her girlfriends call her and are telling her how they ride in the car with boys....experimenting with everything...dating....all of it...and Noel can't do these things due to her health...she does not like what she is missing...

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Celeste, My heart goes out to you. Yes, I am going through the same thing mentally with my son Randy who is 21. He gets very angry, slams things, yells. He says it just isn't fair. Well.......it isn't! He hates that he doesn't see himself getting better. I beg to differ. He is able to do a lot. This is the first time I have heard of gout. Randy gets pain in his heels and lower legs but never to the extent as your daughter has. I hope this is remedied very quickly. I will keep you in my prayers. Jane, Mom to Randy 21

Pam, my son Randall has been on flolan since May of 1993. It took many months before we saw an improvement in his activities. I think it was at least six months. When Dr. Barst did a cath and tested him for the flolan he didn't even show signs that it would help him at all. Randy is now 21 and skis, bikes trails, golfs, and swims. All of a sudden you will see a huge improvement in your daughter. Randy could hardly even walk to the bus stop and now he is very busy. Sometimes he scares me with how much he does. I hope I helped answer part of your question for you. I know each child reacts differently, but I've heard from others that it does take some time to notice a change. Feel free to ask me any questions you may have since we have been dealing with PPH since 1992.
Jane (Mom to Randy age 21) upstate NY

Hello....

this is celeste ...Noel's mom....

well.....we went through two or three weeks of Noel not being able to walk....

the card said it was gout and had her on some pain med that started with an i.....and now on allopurinol..

she has had gout before and always has knee pain for at least....well for yrs. anyway..

now ...it seems as if ...whatever is wrong with her leg is back...

she is at the limping and hoping point now..sometimes she cannot move her leg...her foot is not as swollen as it was before yet but ...I hate to say this....her foot is very swollen and red purple...her ankle is swollen and her knee....the other foot is a bit swollen...the last time it was so swollen that her toes looked like little stubs...it hasn't gone that far yet...

she is getting mean....

wow...i hated to say this...

but has anyone else been at the recieving end of a very frustrated teen....Noel can get mean to me but now she is also getting meaner with others...

last night I spoke to her about it and she said she can't stand it anymore....everyone else can at least walk...she said....look at Warren...my 4 yo grandson...she said....he can run all over and he feels good...

I can't even walk.....she tells me she dosen't want to be mean but sometimes she just can't help it...

I feel it has been hard for me lately...all the feelings....not being able to make things any better...

although ....the card checked her heart by echo the last time and it is looking better since the addition of remodulin...so this was wonderful news....but now its her leg...

sometimes she cannot even move it....and it was so bad last time we ...anyone could not even touch it...

has anyone else had to deal with any similar issues......has anyone else experienced their child going through this...

I know it must all be part of the exceptance of the disease...and the coping is hard.....and it is hard for the parents.....

sometimes I cry...much love celeste

mother of Noel...

pph, eisenmenger, congenital heart defects, ds, legally blind...

currently on tracleer...125 mg 2x/day

remodulin....10%..... 45.5

pepcid....20 mg

synthroid......allopurinol.....O2..which she will not wear anymore due to peer pressure...unless she is really ill....

oh yes and her girlfriends call her and are telling her how they ride in the car with boys....experimenting with everything...dating....all of it...and Noel can't do these things due to her health...she does not like what she is missing...

Hi everyone,

Hope you are o.k. It is certainly hot here (Houston).

I was wondering if those of you with children on Flolan or who have been on Flolan, could tell me about how long it took before your child seemed to have a lot of improvements in terms of being tired and not having shortness of breath very often?

I am trying to see if I am being unrealistic in thinking that Becky would be better than this by now. She started in late May.

Thanks.

Pam (Becky's mom and mom to Johnny and Laura)

Having read some of the recent posts, I am reminded that there are
some amazing stories out there that need to be told! I also realize
completely understand and respect any desire for privacy, but if you
WOULD like to tell the story of the PH patient in your life, we are
trying to build the "Our Journeys" section of the website
(http://www.phassociation.org/Our_Journeys/) and would love to
feature your stories and pictures there to to help raise awareness
around PH.
Please feel free to email me at kaaryn@... and thanks
for all you do in the fight against this disease!
Kaaryn Sanon
Director of Internet and Print Services
Pulmonary Hypertension Association
http://www.phassociation.org
850 Sligo Avenue, Suite 800
Silver Spring, MD 20910
(301) 565-3004 ext. 109

Hello..I recently heard from our Congressman...John M. McHugh...he has also agreed to join as a cosponsor of the legislation...YEAH!!!!

much love Celeste...Noels mum in upstate NY

Another Co-Sponsor is Added to H.R. 1316!
D.C. Lobbying Urged for Summer Visitors
I am pleased to announce that Michael Honda (D-CA) is signed on as a
co-sponsor for the Pulmonary Hypertension Research Act of 2003 (H.R.
1316). We're now at EIGHT for representative support. PHA anticipates
that more co-sponsors will be added in the near future.
PHA set a goal to have 20 co-sponsors in the House of Representatives
this year. We are well on our way! Once again, many thanks to the PH
community for rallying together in support of our letter-writing
campaign- Almost 1,000 emails and letters have been sent to U.S.
representatives nation-wide! This is a powerful statement for a
campaign that began less than three months ago. With your continued
support and advocacy actions, we will reach our goal on this bill and
also make significant impact on the Medicare bills. The following is
an opportunity for special action in regard to securing additional co-
sponsors for H.R. 1316:
Planning a trip to DC?
At this point in our campaign to get H.R. 1316 out of committee and
onto the floor for a vote, I am encouraging you, if are visiting the
Washington, DC area to plan a visit with me to visit your member of
Congress or his/her legislative aide on Capitol Hill.
Personal visits are powerful in lobbying for a bill. It makes an
issue real to a congressional staffer who, on an average day, is
faced with hundreds of bills. If you are in the Washington DC area
and are interested in taking this action, or if you would like to
find out more about how to visit your local congressional district
office, please contact Cara Ugolini at (301) 565-3004 x.113.

Hi Pam
My heart goes out to you for all you are going thru. Like you I seem to be the strong, handle everything parent in our family. I have previously been on antidepressants also, I found I was waiting everyday for Tara to die. Had to really pull myself together and the anti depressants helped me a lot. Took them for about 6 months.
Don't know about the ones you take, perhaps they cause the tiredness? Perhaps you are aenemic? Have you seen your Dr and explained how you are feeling?
I do agree that we become very exhausted from the ongoing stress of having a PH child which gets much worse when they become ill as Becky has been. I seem to handle everything in front of other people, then at night or when I'm alone that's when my worst feelings surface. I have Tara who's 4, then Kyla age nearly 3 and Nelson aged 19 months. They keep me extremely busy, but I am thankful for that because I don't have time to dwell on the negative side. PLUS I refuse to let my mind be constantly thinking about Tara's poor prognosis. A bit easier for me when I am occupied with my 3 little ones.
Hopefully as Becky's flolan dose is worked out and you all get used to the new way of life bought on by flolan, you will feel more confident, and in control of things. All I can really advise is concentrate on the positives happening at the moment, like you winning the battle for Becky to see Dr Ivy. And also take yourself along to your Dr and have a talk about how you are feeling.
Hope you are feeling on top of the world soon.
Best wishes
Chris
mum to Tara

Hi Pauline...I am Celeste and live in upstate ny...my daughter recieves all her care in vermont.

My daughter Noel is 19 yo, ds, primary pulmonary hypertension, eisenmenger, congenital heart defects, hypothyroid, legally blind, nystagmus...etc.

I have tried to write you several letters to welcome you and don't know if I had sent one yet..and the others were getting carried away...

some day I am going to write out our experience...and then I can send it to people...it would make for an interesting read....at least I think.

WELCOME to our list...I am sure that you will learn alot here.

My daughter was dx at 18 months of age....with severe pph...she is currently on tracleer (bosentan) and remodulin..

she is also supposed to wear her 02 at night and she used to wear it well but with a bit of peer pressure and teasing now she won't wear it unless she is practically down and out....

her sats run around 78-80 at rest...with the meds....although she is not cured the meds have made a big difference with her.

she still gets tired....

she has her happy times but is also dealing with the facts that her friends are out running high wide and handsome...dating, experimenting, etc...she is now going into the..no man will ever want to date her with ph and a remodulin pump...it breaks my heart...but what can I do..I try to deal with things the best that I can.

I am looking forward to hearing more from you....I will be happy to discuss my situation....and answer any of your questions...

Noel has been through quite a bit.....her sats went down to 38 with an irregular pulse of 36 before the tracleer...she has experienced....hemoptysis and gout...the top part of her heart quit working in 1998...but I am happy to report that with remodulin it is starting to show some movement..her vena cava is smaller ..her hematocrit is down to 57.5...whoohoo...and probably something else....

Noels gout is better.....something I learned recently....gout does not always start in your toe....this time it was in NOels knee and both her legs and feet were swollen...BIG....and they were both reddish purple.....

looking forward to hearing more about you and your son...celeste...mother of Noel...

Hi everyone,

Jennifer, thank you for your prayers. I certainly appreciate them, and your kind understanding as well.

As of last night, Becky's stomach problems from Flolan seemed to be subsiding, which is good. We are scheduled to go up by 1 on the rate tonight - from 62 to 63. Hopefully, such a small increase won't cause her more problems. Dr. Ivy is trying to keep increasing, but with more days in between and smaller intervals, trying to minimize the side effects. We'll see how it goes, I guess.

I am having a problem with myself that seems connected to all this stuff with PPH and Becky, but I have tried and tried and can't make much sense out of it. Hopefully, one of you will have some ideas.

I am taking an antidepressant - Lexapro - 40 mgs. per day. It seems to work pretty well. Before I was put on it, I had been tired all the time and wanted to get in bed constantly, was very irritable, yelled at the children, etc. Lexapro seems to work pretty well. I don't yell much anymore and am considerably less irritable with my family. However, it still seems like I have to have a nap every afternoon (except for the days I work when I struggle through the afternoon) and I seem tired a lot. As my 8 year old says, "you always need a nap". We used to go to church and for about the last 4 Sundays or so, I don't wake up on time to go and we miss it. I feel bad about it, but after I get up it feels like I needed the sleep.

I do know that I have been under a lot of strain since February when Becky started getting worse. Then I had insurance wars over Viagra and then over trying to get them to cover Dr. Ivy's services at in-network so we could see him. There were also a lot of struggle with our PPH doctor at the time, who was really unhelpful and dragged his feet about doing things Becky needed. Then Becky was hospitalized and was really bad (almost to point of losing her), and then there has been all the stuff with the Flolan. And then we went to Denver to see Dr. Ivy.

So I am trying to figure out if I am just exhausted from too much stress or if I am still depressed and need a better antidepressant or what. Normally, I am the person in my family who holds everything together and is strong, so I am really puzzled.

Sorry to go on so long about all this, but thanks for listening.

Pam (mom to Becky - 14 - severe PPH, congenital heart disease, scoliosis, anxiety disorder, polysplenia, ADD; Johnny - 11 - healthy; and Laura - healthy - 8)

Hi Pam....I am sorry to hear that Becky is having trouble with the Flolan.

I will keep her in my prayers that she is feeling better soon...much love celeste..mother of noel..19yo on tracleer and remodulin..

Hello. This is my first post but I have been reading your posts for
a couple of days. My son, Nick, is nearly 16, he has DS and
Eisenmenger syndrome, he is totally tube fed and has a permanent
oesophagostomy due to other medical problems. At the moment he is
keeping well, he is not on any medication, he is not blue and his
sats are usually in the low to mid 80's, he is fairly active but he
can't run. Its hard to know just how far he could walk as he is
incline to be lazy so always have to take his wheelchair with us when
we go out. I wasn't told until he was 7 that he has Eisenmenger so
of course it was much to late to operate at that stage. His
cardiologist is hopeful that he has 10 - 15 GOOD years left,
considering he was not expected to live to be two then 5 then a teen
he is doing wonderfully well. I am looking forward to learning a lot
about PH from you all. We live in the UK.
Best wishes, Pauline.

Hello everyone,

My thanks to everyone who gave me advice about travelling to Denver with Becky (my daughter) and all her Flolan supplies.

We returned yesterday afternoon late. The trip went well, and Dr. Ivy's staff treated us very kindly and went beyond the call of duty. We didn't find out anything really new and startling. He wants to try to slowly increase Becky's Flolan. She is on red tops now, and is having a lot of trouble with stomach cramps, nausea, etc. But unfortunately she is still tired and out of breath a fair amount of the time, so obviously her Flolan dose is probably not high enough yet. She is not happy.

Dr. Ivy's plan is to try to slowly increase the Flolan and in six months or so to try to get her on Sitaxsentan as part of a study in addition to the Flolan. We'll see how it goes. Right now I am worn out with her distress, the trip and my concerns about her PPH. Today I am at work. I've received an e-mail from my supervisor complaining about a fairly minor matter, and I feel like sending her one back asking her to let me know when she gets a real problem. But of course I won't, since I need my job.

Anyway, thanks for your help and for listening to me.

Pam (mom to Becky - 14 - congenital heart defects, severe PPH, scoliosis, polysplenia, ADD, anxiety disorder, etc.; Johnny - 11 - healthy; and Laura - 8 - healthy)

Dear Celeste,

I bet Bob would be the best to ask in the pha community, (not here in pha parents) as to road trips as he and his wife Wanda who has PH travel extensively.

I know when we travel we have to stop about every 2 hours and walk around etc, but by then we're all bored anyhow so it works for us. I think if you planned a road trip, plan half the trip for travelling....make sure you hit the scenic roads or at least something Noel can enjoy. I remember she is legally blind, so scenery may not be what she is able to see, unless you're up close and personal.

I pray noel's legsd are getting better, and she is feeling better too. I am off, it is HOT here and I have tons to do before we leave tomorrow.

Co-Sponsors Needed for New Medicare Bill!
The Medicare Patient Access to Drugs for Rare Diseases Act of 2003
Introduced!
On Thursday, July 10, Representative Chris Cox (R-CA), working
alongside our friends, the National Organization for Rare Diseases
(NORD), introduced the Medicare Patient Access to Drugs for Rare
Diseases Act of 2003. This important legislation if passed will
ensure that Medicare recipients continue to have access to life-
saving therapies through the Medicare Hospital Outpatient Prospective
Payment System (HOPPS). This bill is a response to new regulations
that drastically cut the Medicare reimbursement rates for drugs
covered by HOPPS. The result is that hospitals will have to pay much
more than they receive from Medicare for some intravenous and
injectable orphan products, including Flolan and Remodulin, two out
of the three treatments approved for treating pulmonary hypertension.
In many cases, hospitals may simply stop stocking those products. The
proposed legislation in Congressman Cox' bill will exclude orphan
products from the existing regulations at HOPPS.
PHA has prepared a sample letter for you to send to your members of
Congress, asking them to co-sponsor this bill. To participate, please
visit http://www.phassociation.org/Take_Action/ and click on the
Action Alert titled "Co-Sponsors Needed for New Medicare Bill." Feel
free to make changes to the sample letter and decide if you want to e-
mail it or mail a hard copy. Below the letter, fill out the relevant
information and click "Send Message." As always, please ask family
and friends to participate.
Thank you for your continuing support of PH Advocacy!
Sincerely,
Cara Ugolini

CONGRESSIONAL SUPPORT FOR PH INCREASED!
The CDC and HRSA encouraged to expand their relationship with the PH
community in 2004
Thanks to the support and effort of Rep. Kevin Brady (R-TX), Rep. Kay
Granger (R-TX), Rep. Jesse Jackson, Jr. (D-IL), and PHA members, our
community has taken an important step forward in our goal of further
involving the Centers for Disease Control (CDC) in expanding PH
awareness.
Anne Caesar, MD, a PH patient and PHA member, testified before the
House Appropriations Committee on May 14, 2003. Following her
testimony, Congressman Dave Weldon, who was chairing the session,
remarked that, as a physician who had a partner in his practice
diagnosed with PH, he was familiar with the devastating aspects of
the illness.
On June 25, 2003 the House Appropriations Committee marked-up its
FY04 appropriations bill for the Departments of Labor, HHS and
Education. Included in the report accompanying the bill is a
committee recommendation encouraging CDC to expand its PH awareness
activities, working in partnership with the PH community. The
language is as follows:
The Committee continues to be interested in pulmonary hypertension, a
rare, progressive and fatal disease that predominantly affects women,
regardless of age or race. The Committee looks forward to reviewing a
report (as requested in the FY03 omnibus appropriations bill) from
the CDC outlining the progress that has been made in establishing a
pulmonary hypertension awareness campaign focused on the general
public and health care providers. Moreover, the Committee encourages
CDC to give priority consideration to supporting a cooperative
agreement with the pulmonary hypertension community designed to
foster greater awareness of the disease.
On June 26, 2003 the Senate Appropriations Committee marked-up their
Labor-HHS bill and PHA's CDC language is included in their report as
well.
In addition, the Senate Committee accepted PHA's language within the
Division of Transplantation at HRSA calling for the agency
to "enhance its partnership with the pulmonary hypertension community
aimed at increasing awareness about the need for increased heart and
lung donations." This language was also included in the House Labor-
HHS bill.
Submitted by:
Cara Ugolini
cara@...
PHA Advocacy and Awareness Manager
June 27, 2003

I would like to hear more about blood clots and traveling by car...

with noel we always have to go by car because she cannot fly.....due to her ph etc....

we do take frequent brakes when we go anywhere...but no one has ever discussed anything with us about this...

and I would like to know what is the proper precedure when you have to go by car....like how often should you stop etc...we do try and stop every hour....or so..

we did get a minivan...the wc fits and the tanks...and noel has much more room esp to stretch her legs..

please share with me any info you have about traveling by car...I would love to go somewhere....and take Noel....she hasn't been well enough lately...but I am planning on her getting better.......much love celeste

I can see Joey removing that clot...sounds like my boys when they were young...I have removed strange things from teh boys noses when they were about 2 1/2 to 4 years of age....

I will be thinking of you two this week!

Hugs,
Evan

Dear Pam,

This is what Sean and I did once he was on Flolan, and we HAD to fly home....(he couldn't be in the car that long-blood clot possibility, etc.)

I mixed the extra Flolan cassette and put it in the cooler from Acredo, and then when we boarded the plane told the attendant that I had life saving meds on hand and they needed to be kept cold, at a constant temperature. She (a wonderful lady) took the flolan cassette and the cooler, the whole sheebang and put it in the stow spot for something else. She took the meds and put them on ice as well as the ice packs. They were kept at a constant temperature while we were on the plane. I did have the emergency kits we had assembled, and it was with us on the plane plus an extra 2 days meds. I did this because I had to make sure he had what we needed.

The people on the plane were great, Sean had oxygen on, had to have it, and there were a few staring eyes, I think it was because he was so big and burly....and Sean slept all the way home. I did the pinch the nail thing to m ake sure he wasn't losing saturation and all that, and he about slapped my hand once or twice, but everything was okay. Once we got home, the wheelchairs and everything were waiting as well as additional oxygen.

You may want to check about oxygen for Becky. DIA and Denver are higher than the elevation of anywhere in Texas and she may experience some SOB, so the oxygen might be a good idea. Tell Dr. Ivy's office or your regular doc's office you need oxygen. Period. They will give you the information needed to get it.

We bypassed security lines and went to a specific area for people with needs or whatever, and I think the first class flyers checked through there too. Tell them to NOT x-ray the Flolan. Tell them what it is the pump, etc, and that X-ray will damaged it. They will look it over and if you explain a bit about the pump, etc., they will listen and do what you want. You know me and my aggressivenes, er, I mean gregariousness, I made sure that nothing was touched except what needed to be touched in his meds. I told the guards they'd need to wear gloves to touch anything that was belonging to the medical end of what we were carrying on- I didn't want their skin to come into contact with Sean's supplies. They just looked the bags over then....(hee hee) and I told them we had sharps and everything. It was okay going through security and when we boarded the plane I gave the backpack with the sharps to the attendant so they'd feel okay about everything. It is stupid to have to be so
worried about it, but better safe than have problems. Once she realized we were okay etc, she gave me the back pack back, and I stowed it under the seat.

Tell the airlines you need wheelchari transportation, at the airports. I don;t know what airlines you're both flying on, but in DIA it is an ENORMOUS walk from point A to point B. We had a guy push Sean all over the place. I tipped him because he had to ride the trains and everything with us. I don't know if you need that too....

Okay, I think I have told you all about our trip flying. If you need any more information, please email me privately, you've got the address.

I wish you and Becky and great trip to Denver with Dr. Ivy.

Warmest Wishes!!!

Evan

Evan White (pronounced Eve-anne)
Mother of Sean the flolan addict
DX 10/18/02, PPH
Peace to everyone who reads these words
Working to help find a cure to this silent killer

Pam,
Good luck with your trip. We never flew with Joey. For
long day trips we took one of those insulated flexible
lunch boxes, and just filled it up with Ice packs , and the
thermal mass made them last longer. Dry ice would be good,
but I have never messed with it. On longer car trips we
took a 12v dc-120v ac refrigerator freezer we saw advertised
in a 4 wheel drive catalouge.
It was expensive, but made traveling easier (yes I claimed
it as a medical expense on my tax return). If set on
freeze it keeps those ice packs rock hard.
As well as our emergency kit, which contained all the stuff
needed to mix two doses, we also had a plastic parts
organizer tray (from a hardware store filled with more than
enough for a day's mix, we took this with us, along with
a large cookie sheet when we went to a doctors appointment,
or anytime we might not get back home in time for medicine
mix. Traveling you have two concerns, the first is that you
have all the stuff you need, and a little extra, in case
you mess up. The second is that the stuff you have is
organized so you can find what you want when you want it.
When traveling timing medicine mix is a important
consideration. Jennifer has mixed flolan in the back seat
of the suburban (parked), and once at a picnic table at a
rest area.
We had these plastic containers with a carry handle from an
art supply store, that held a bunch of plastic trays with
lids. we organized a week supply of meds into one of
these cases each monday, and did inventory as the container
was stocked. we had an extra, so when we traveled (by
car-this doesn't really relate to flying) we could stock up
the two week supply of meds we were supposed to have with
us at all times. I don't know how you would carry all
that stuff flying, I would be worried some as the medicine
has a narrow range for storage temperature.
Joey's emergency kit was rather extensive . It was a Lands
end bag made to be a large daiper bag. it had a real
nice daiper changing pad (never used as such) that we would
have used for an emergency mixing surface . We kept some of
everything in that bag, Jennifer, being an RN even kept an
Iv starting kit, stethoscope , and a blood pressure cuff in
there , extra batteries. The bag ended up weighing close to
40 lbs. but we kept finding more stuff to put in there.
I know this is not specific to air travel, but I hope it
helps some. The containers really helped organize the needed
supplies, which helped make mixing medicine at home or away,
faster and less stressful.
good luck with your trip.
Bill Nelson

Hi,

When we fly which is frequently, I pack 3 days of supplies, I.V. Start kit, and paramedic instructions in our carry on and the rest in our checked bag. I pack a small lunch kit (lunch box for yankees) with ice packs and dry ice (if I can get away with it) and put that also in my carry on. If you have a doctors letter of medical necessity you can carry up to 1.5 lbs of dry ice on the plane. Other wise I pack the dry ice in the lunch kit and throw it away before we board then we have about 10 hours of frozen ice packs. Last year we got stuck in Salt Lake City airport for 12 hours because of a security breech. Ben and Jerry's Ice Cream kept Trey's Ice packs frozen. I have found that a shoebox holds 3 days worth of everything and scans nicely. That way if something happens I can just grab the box. We keep it by the door when at home so we can grab it on the way to the hospital. Pam, I know some people use the steril drapes to mix on. We have a small plastic cutting board that we
use for medicine only that I take with us if I am out of sterile drapes. Also I wrap alot of the stuff in the plastic bags that they come in because they have the perscription label. Take care and have a good visit.

Sunnie and Trey

Hello everyone,

Hope you have had an enjoyable 4th of July.

For those of you with children on Flolan, I need a few tips for travelling please.

I am taking my daughter, Becky (14) to see Dr. Ivy in Denver, CO next Wednesday. We're going by plane since we live in Texas. I am having Accredo ship 3 days of supplies so I don't have to take all the supplies on the plan with us.

However, I still need to take the backup pump, something to mix on (I use a t.v. tray at home), some alcohol to sterilize the mixing area, the backup cassette, and her ice packs.

What I'd like to know is what do you use to mix on while you are travelling; what do you keep the various things in for travelling; and how do you keep the ice packs cold while you are travelling? If this sounds stupid, please excuse, but we haven't travelled since Becky started on Flolan. It will just be she and I since we are taking Miracle Flights for Kids and they only allow one parent with the child to have the flights paid for; so I am rather nervous about not forgetting something or not being prepared somehow.

Thanks.

Pam (mom to Becky - 14 - PPH, congenital heart defects, scoliosis, ADD, anxiety disorder, etc.; Johnny - 11 - healthy; and Laura - 8 - healthy)

URGENT ACTION ALERT!
THIS JUST IN: Research Progress May Come to a Halt at NIH!
Next week the House will be deciding on a funding proposal for the FY
2004 Labor-HHS-Education Appropriations bill which would stop medical
research progress in its tracks. The Senate will decide on similar
bill later in the month. The proposed funding level for the National
Institutes of Health (NIH) in the Senate bill would allow for no
growth in its budget, while the House bill would actually result in
funding cuts for medical research.
PHA is working with the American Heart Association, the National
Coalition for Heart and Stroke Research Colleagues and several others
to prevent this bill from passing with lowered support for NIH. The
goal of our quickly formed coalition is to have 500 or more health
organizations involved in this action.
A sample letter has already been prepared for you to send by mail or
by email to your local representative and state senators. Here's how
to participate:
1. Go to http://www.phassociation.org/Take_Action/
2. Click on the Action Alert titled, "Research Progress May Come
to a Halt at NIH"
3. Enter your zip code in the box
4. Two letters will appear, one on top of the other. The top
letter will be sent to the House of Representatives. The bottom
letter will be sent to the Senate
5. As always, feel free to make changes and add personal
sentiments- the letters were written from the perspective of a
patient, so please make the appropriate changes if you are a family
member, caregiver or friend
6. Underneath the letters, fill in the relevant information and
click "Send Message"
7. Please invite friends and family to send letters and emails
As always, thank you for your participation, especially on such short
notice.
Sincerely,
Cara Ugolini
Advocacy and Awareness Manager
Pulmonary Hypertension Association
(301) 565-3004 x.113
cara@...

Evan:
I will be saying lots of prayers for your family and Sean when you go to San Diego. That will be nice to make a mini vacation out of the trip. Try and enjoy as much of the trip that you can. Good luck on the appointments!
Connie

Hi Everyone,

I received a call yesterday from UCSD (University of California, San Diego) and Sean has the evaluation next week. I have mixed feelings, but terror and anxiety top the list. We see Dr. Yung, who is a specialist in lung transplant and pulmonary hypertension. He has written many articles with Dr. Channick and few with Dr. Ruben, so I know Sean is in great hands. It is just the whole deal that scares me. Like waking up and being on Mars. Okay it isn't that bad, but it sure feels weird. I have had a lump in my throat ever since the phone call..... The good part about all of this is that we will have a mini vacation while we are in San Diego, hubby has never been to Sea World, and Cameron, Sean's brother has a birthday this month too, so it will be a mini vacation celebration.

On a major positive note, the doctor at LA said Sean doesn't have to come back for three, yes, three months! That is a good thing, it means we are therapeutic at this Flolan level and Sean knows that when he has to increase then he can call and they will take care of it.

Today we go to the infusion center and if they pull the PICC line out anymore then we will be back in the hospital because it was pulled out about an inch last week, and his transplant nurse in LA had kittens! She ordered an immediate chest x-ray and sent an e-mail to the doctors out here...I think I will be banned from this hospital and medical center once we finally teach them what is what. Every Tuesday we get new meds and then a dressing change for Sean. wish us luck and good work by the nurses. Not to mention the prayers. Sean said he will just tell the nurse if you pull it out, we are putting a PICC in YOU TOO!

He has been a bit depressed lately, not getting out much or anything. I think the gravity of this whole situation is finally effecting him. I sure know he has a great big facade about all of this built around him. He told a friend yesterday, "I just show up for the appointments. My mom is my secretary/nurse/advocate." I wish he would do a bit of all of this too....

I pray you are all well, and that it is not too hot for any of you today. I feel like I am in a broiler....welcome summer! If I am sidetracked I want to take this opportunity to wish each and everyone in the USA happy Fourth of July! Be safe, have fun and if you play with fireworks, please remember to keep the water hose right there-only you can prevent wildfires and all the bad things!

God Bless each and every single one of you!

With Love and Prayers,

Evan

Gees we finally get record heat here in the northeast and I have poison ivy..I think.. all over my face....much love celeste

Hello lori, we have had extensive experince with sildenafil while our daughter was in the hospital. Dr. brown of Riley childrens hospital in Indianapolis IN had been using sildenafil on three other patients with pulmonary hypertension on the iuc at the time and did not see any great improvments from it but the children were staying very stabe. He started our daughter on it about 3 weeks after her first heart surgery but she had a very rare disease, pulmonary vein stenosis, and it was a very severe case. the sildenafil mixed with all the other drugs worked to keep the pressure down for Chloe and had no seriouse side effects for her. Chloe did pass away on March 18th of htis year but she was born with many seriouse problems that no one knew about until she was 4mths so it had progressed to far and the doctors had not seen very much of it so they could not help her too much. Chloe lived to be 11mths and that was a lot longer than most of the doctors thought she would, except
for doctor Brown. He had so much faith in her and we trusted him completely. He gave us much more time with our daughter and it wasnt all suffering, we had some great times even in the icu because Chloe only left the icu for 1week out of the 7mths she was there. Now wether this time was given to us because of sildenafil or not we cant be sure but we can say that it was not taken from us because of sildenafil. You could probably get a lot of valuable info from Dr. brown at riley hospital and you could start by going to the rileykids wesite i am not real sure right now what it is but I will email you with more info.

Hi, everyone,
I haven't tuned into "the group" lately because we had a fire at our house,
when I finally checked my e-mail I had 89 new e-mails! Anyway, to update you
on what has been happening, I've had to go back to work fulltime for
financial reasons, I'm the new clinical care specialist for breast care
services at Utah Valley Regional Medical center, what that means is I
contact women who have recently been diagnosed with Breast cancer. I become
her resource person, giving her information on the disease, treatment
options and then coordinating her care with the surgeon, medical oncologist
and radiation oncologist. While I was at work 2 weeks ago, I get a call from
my husband, telling me our house was on fire! Maddie and her 8 years old
cousin were playing with a lit candle and hid it in a doll house when she
was called down for lunch, the dollhouse caught on fire, which then caught
the carpet on fire! luckily that was the extent of the fire damage but there
was extensive smoke damage. We are in a hotel for the next four weeks, while
the clean-up takes place, the good news is, no one was injured and I get new
carpet, freshly painted walls and a great "spring cleaning!" Last week we
took Maddie for her 6 month follow check with Dr. Ivy in Denver. Her
pressures are still high (120-130) but she feels well. she is on flolan and
Tracleer. He started her on Sildenfil. Does any one have expereince with
this medication lowering pulmonary pressures and what side effects it has.
Thanks for listening Lori and Madison Heaton

Lori:
I'm sorry to hear about the fire in your house. Luckily nobody was hurt.
Good luck with your 6 month follow-up with Dr. Ivy. I hope that it goes well.
Connie (Mother to Tony, 12, PPH)

Hi, everyone,
I haven't tuned into "the group" lately because we had a fire at our house,
when I finally checked my e-mail I had 89 new e-mails! Anyway, to update you
on what has been happening, I've had to go back to work fulltime for
financial reasons, I'm the new clinical care specialist for breast care
services at Utah Valley Regional Medical center, what that means is I
contact women who have recently been diagnosed with Breast cancer. I become
her resource person, giving her information on the disease, treatment
options and then coordinating her care with the surgeon, medical oncologist
and radiation oncologist. While I was at work 2 weeks ago, I get a call from
my husband, telling me our house was on fire! Maddie and her 8 years old
cousin were playing with a lit candle and hid it in a doll house when she
was called down for lunch, the dollhouse caught on fire, which then caught
the carpet on fire! luckily that was the extent of the fire damage but there
was extensive smoke damage. We are in a hotel for the next four weeks, while
the clean-up takes place, the good news is, no one was injured and I get new
carpet, freshly painted walls and a great "spring cleaning!" Last week we
took Maddie for her 6 month follow check with Dr. Ivy in Denver. Her
pressures are still high (120-130) but she feels well. she is on flolan and
Tracleer. He started her on Sildenfil. Does any one have expereince with
this medication lowering pulmonary pressures and what side effects it has.
Thanks for listening Lori and Madison Heaton

Two Co-Sponsors Added!
Congressional Support for H.R. 1316 continues to grow
PHA welcomes Congressman Chris Van Hollen (D-MD) and Congresswoman
Loretta Sanchez (D-CA) to the growing list of co-sponsors for the
Pulmonary Hypertension Research Act of 2003. We're now at 7, with
support from members of both parties.
PHA is especially pleased because our headquarters is in
Representative Van Hollen's district.
These new sponsorships are a reminder that our efforts DO make a
difference!
If you've already sent e-mails and printed letters to your Congress
member and you'd like to do more, please consider visiting your
congressional office. Legislative assistants are usually willing to
meet with their representative's constituents. The following are some
tips for visiting a congressional office:
Call the office to set up an appointment
o For phone number and address, visit
http://www.phassociation.org/Take_Action and click "Elected
Officials." Enter your zip code to find the Representative nearest to
you. Click on his or her name to find the office address and phone
number
o Ask for the Health Legislative Assistant, who's name is also
available at the site above, by clicking on "Staff" (underneath the
picture of the Representative)
Tell the Legislative Assistant that you are a constituent
with concern for a bill, and that you would like to set up a meeting
with him/her, or the congress member if he/she is available
o Feel free to tell him/her the name of the bill (H.R. 1316)
and that you are living with a rare illness called "Pulmonary
Hypertension"
In a couple of days I will be posting talking points for a
congressional visit. These will include tips on what to say when you
go in to meet with someone from a congressional office. If you are
interested in participating in this action, please e-mail me at
cara@....
Sincerely,
Cara Ugolini
Advocacy and Awareness Manager
Pulmonary Hypertension Association
cara@...
(301) 565-3004 x.113

Great news, good luck with the home care. Hope you
can rest up and recover from some of that extra stress.
Bill Nelson

MEDICARE ACTION ALERT UPDATE
(articles on House vote below)
Continuing Action Needed on Senate
Thank you to all who participated in yesterday's late-breaking letter-
writing action to several members of Congress regarding the pending
Medicare bill. As you may have already noticed in today's papers, the
bill made it through the House committee, and will soon be before the
full House for further discussion and a vote.
PHA is working with the National Organization for Rare Diseases
(NORD) and other organizations to better understand the implications
of the proposed Medicare billparticularly as it is amended and
altered in the legislative process. As this legislation continues to
evolve in the next several weeks, I will keep you posted as to what
helpful actions our members can take.
E-mails to the Senate should continue to be sent (Sen. Frist, Sen.
Grassley, Sen. Baucus) since a vote is expected any time. You can
do this by going to http://www.phassociation.org/Take_Action/
E-mails to the House of Representatives (Rep. Thomas, Rep. Tauzin,
Rep. Hastert) are no longer needed as the bill has cleared Committee
(see 4 articles below).
Thank you for your continued support,
Cara Ugolini
Advocacy and Awareness Manager
Pulmonary Hypertension Association
cara@...

Great News Evan!!!!

I am so happy to hear that Sean was able to return home...I thought for sure he would be in for a week or two...

good for you doing all his nursing care....you are a great mom...much love celeste...mother of noel

Hi Everyone,

Sean is home. Well it sure finally seems like normal because I got to sleep and hear that concentrator in the hall again.

Welcome to his new nurse for dressing changes and IV infusion therapy, nurse Evan! We ended up in the hospital last night, waited in the ER because of me being unable to get his IV therapy (antibiotics) in him, and when they finally called us (45 minutes later to an IRATE mother) the next thing the nurse said was undress, and get ready for heart monitoring. I said, *&@$^%$ no, that is not why we are here and I am not putting him through all of this again. All I want is help getting his antibiotics in his PICC line and if you can't do that without distressing him, we'll figure it out on our own!

We then snuck by the security guard and headed down to where Sean had been for the past five days and although no one could touch him, they helped me out as I did everything and it worked perfectly (I could have killed Sean...). So now I am getting my eyes open to do it again, eight hours later and ready to change a dressing too, that is the yuckiest part, so I won't go into that with everyone...

Sean will be on IV therapy (antibiotics via the PICC) for several weeks, until July 17 or close to that, and then blood work one more time... I will be the dressing change nurse until it heals from the inside out....(GROSS). But I am SOOOOOOOO happy he is home and I can see him sleeping instead of having to drive to see him sleep.

They did an echo when he was in the hospital to check for infection in his heart and the tech guessed his PA pressures were at about 93, that is way down from the original 110 from last October. I hope she was right. That means the Flolan is working!

San Diego called yesterday and he is playingphone tag with them, and so did the hospital in Los Angeles to do another RHC. They'll have to wait. Everyone wil have to wait. I am not going anyplace yet. Give me a few days to compose myself and get this nursing routine down! All the calls have been made and we are doing a-okay. Thank you all for your prayers and concern, without the prayers, I truly believe Sean would still be in hospital and I would be freaking out.

Much love to Each and Every One of you,

Evan

The URL below is incorrect. My apologies. To Take Action! please link to
http://www.phassociation.org/Take_Action/
Thank you.

CONGRESS TO VOTE ON MEDICARE PRESCRIPTION DRUG BENEFIT TODAY!!!
Between now and the end of July, Congress is expected to consider a
long-awaited prescription drug benefit for Medicare recipients. Last
Thursday, the Senate Finance Committee cleared the way for
consideration on the Senate floor (S. 1). On Tuesday, June 17, the
House Ways and Means Committee will consider its version, which adds
greater reforms, and requires traditional Fee-for-Service (FFE)
Medicare to compete against private plans. Both the Senate and House
bills provide for a prescription drug benefit to be offered by
preferred provider organizations (PPOs).
PHA is working with the National Organization for Rare Diseases
(NORD) to get the best version of this bill the Prescription Drug
and Medicare Improvement Act of 2003 (S.1) passed.
WHAT DOES THIS MEAN FOR PH PATIENTS?
As written, this bill will, for the first time EVER, include
an oral drug prescription benefit plan. This is a good thing.
However, there is a BIG difference between the House and Senate
versions of the bill.
The House version of the Bill is much more generous in its
prescription coverage.
Every senior citizen, and every person with a disability who
is enrolled in Medicare, should be provided with guaranteed,
affordable and comprehensive prescription drug benefits under
Medicare.
We believe the House version brings us closer to this vision.
We also believe:
Rare disease Medicare beneficiaries should have
pharmaceutical insurance benefits at least equivalent to the average
privately insured American who receives their health insurance
through their employer or through the Federal Employees Health
Benefits Program (FEHBP). There should be no coverage gap that
exposes beneficiaries to large out-of-pocket costs.
This is currently not in either bill.
There should be no co-payments required once a beneficiary
reaches an expenditure level that would trigger a catastrophic
benefit. This is particularly important for rare disease patients
whose therapies can cost $100,000 or more. After a rare disease
patient has reached the threshold for catastrophic coverage, the
program should cover 100 percent of the cost of drugs.
This is not in either bill.
WHAT CAN I DO?
1. Go to the Take Action! Website at
http://www.phassociation.org/Take_Action.
2. Click on the Action Alert titled, "Medicare Prescription Drug
Benefit Considered Today!"
3. In the zip code box, ENTER ONE OF THE FOLLOWING ZIP CODES.
This is important, because there are 6 key members of Congress who
are critical to this decision. Entering their zip codes will ensure
that your message gets to the right person:
a. 50309 Senator Charles E. Grassley- Iowa
b. 59101 Senator Max Baucus- Montana
c. 37205 Senator Bill Frist- Tennessee
d. 93309 Representative William M. Thomas- California
e. 70737 Representative Billy Tauzin- Louisiana
f. 60510 Representative J. Dennis Hastert- Illinois
4. After you click "GO," match the zip code with the names
given. For example, when you enter zip code 50309, two names will
appear. Click on Grassley only.
5. Using the talking points, select the paragraphs you wish to
include. Note that the sample letter assumes a patient's perspective.
If you are a caregiver or medical professional, please make the
necessary changes. As with all previous action alerts, you may feel
free to change or add anything to the letter. It would be good to add
some personal details about yourself.
6. Enter the relevant information requested below the talking
points. Click "Submit."
7. When asked to select a topic, scroll through the list and
select "Medicare."
8. If you can, please spread the word to family members and
friends TODAY. Also, please go back and send e-mails to each of the 6
members of Congress listed under #3 of these directions.
NOTE FOR MEDICAID RECIPIENTS: It is possible that a similar amendment
will be put into affect for Medicaid in the future.

Evan,

Bless your hearts! Having your child in the hospital is such a difficult situation. I'm glad your husband is good and supportive, though, that does help.

I hope Sean proceeds along now, since the holes have been repaired. I know you'd like to be home soon.

Take care of yourself, as much as you can.

Pam
Lady Evanne <ladyevanne@...

Hi Everyone,

Thanks for the words of encouragement, the mother tiger continued today after getting much needed rest.Pappa tiger was on watch until three today and he was the best he could be! I am proud of my sweet husband! He did really well, fighting off doctors with attitudes and everything else!!!

The cath was finally removed between 3 and 6 this morning- there are two gaping holes in Sean's chest, I know they are gaping, I saw them intimately tonight! Sean started bleeding out of the larger incision tonight to the point he was soaking thru his dressing hourly. The surgeon came in and I got to assist him in repairing the bleeder, and then doing the dressing. It was COOOL!!!!! Okay I know some of you out there must think I am nuts, but I was in heaven helping (especially since I am NOT a nurse). Sean's temperature is fluctuating up and down like a yoyo, so he won't be going any place soon. I was hoping he would be home in a day or two, but that is to still be seen.

Dietary blew it tonight and forgot Sean's supper so he got junk food, well a big ol' Carl's Junior burger and fruit, I went and bought supper, so he was happy eating that instead of hospital food.

Thanks again for everything each of you have done, especially the prayers. To all you Dad's out there in cyberspace, Happy Father's Day, you are loved much and greatly needed in our lives these days!

I'll keep you all posted on how we're doing!

God Bless!

With Love and HUGS,

Evan

Hi Everyone,

Thanks for the words of encouragement, the mother tiger continued today after getting much needed rest.Pappa tiger was on watch until three today and he was the best he could be! I am proud of my sweet husband! He did really well, fighting off doctors with attitudes and everything else!!!

The cath was finally removed between 3 and 6 this morning- there are two gaping holes in Sean's chest, I know they are gaping, I saw them intimately tonight! Sean started bleeding out of the larger incision tonight to the point he was soaking thru his dressing hourly. The surgeon came in and I got to assist him in repairing the bleeder, and then doing the dressing. It was COOOL!!!!! Okay I know some of you out there must think I am nuts, but I was in heaven helping (especially since I am NOT a nurse). Sean's temperature is fluctuating up and down like a yoyo, so he won't be going any place soon. I was hoping he would be home in a day or two, but that is to still be seen.

Dietary blew it tonight and forgot Sean's supper so he got junk food, well a big ol' Carl's Junior burger and fruit, I went and bought supper, so he was happy eating that instead of hospital food.

Thanks again for everything each of you have done, especially the prayers. To all you Dad's out there in cyberspace, Happy Father's Day, you are loved much and greatly needed in our lives these days!

I'll keep you all posted on how we're doing!

God Bless!

With Love and HUGS,

Evan

EvanL
It sounds like you have had a very rough day. That's good that your husband made you sleep.
It sounds like Sean is doing better and I'm very glad to hear that! Hopefully the hospital stay won't be too long.
With thought and prayers,
Connie

Hi there everybody!

My husband told me last night...this morning..some time during the dark time that I was NOT driving any more and I WAS going to bed if he had to duct tape me to the mattresss. So I slept. It was hard but I was freaking out.

Sean's Groshong Cath, well the subcutaneous part and his body became close friends. So close that he actually absorbed it as a part of him. It was literally attached to his skin like it was a part accepted by his body. So after dealing with a resident surgeon, and telling him I thought he was a boob....another one came in and said, wow I have never seen this. We will be back with operating room equipment-to perform this delicate procedure (it's not a surgery when they do it bedside). So the doctors started at 2:00 this morning to remove the thing and they finished at 6:30, with a break in there to retrieve appropriate equipment from the operating room. DOH! They had to literally "dissect" the cath out of him until they reached the vein.

I told them the should listen to me, and this was the norm for us- whatever can be abnormal for the world is just a part of our life as who we are and PH. Now he has cotton packing there and he will have it changed twice a day until it heals. It is supposed to heal from the inside out

Then there was another round of hives and antibiotics...Glad I was passed out here. I had earlier explained Sean was different from every other patient. He has a different metabolism. What takes 30 seconds in one person can take up to an hour in him. Sure enough, the hives came. The nurses listen, but the doctors? Well that is a different story. Some think I don't know my own kid. I wish they were a little more listening and hearing than just the "I know everything" and you don't because you're not an MD attitude. It would really help in situations like this.

It is hard to