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PH Awareness Blogging Day!

Tuesday, November 18, 2008
Please grab a cup of coffee and click on the following link. You will enter the most amazing stories of the most amazing phriends and caregivers.


I thought I'd share some specific info on the medicine that I use that allows me to function and live a somewhat "normal" life. I remember how poorly I felt before I started on Flolan. As many of you know, I was diagnosed with Primary Pulmonary Hypertension on June 29, 2004 (this also happens to be my beloved husband's birthday). I began using Flolan two weeks later. With Flolan therapy I was able to move from a Class 4 to a class 3 and sometimes even a class 2!

Class 1:Patients with PH but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.

Class 2: Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.

Class 3: Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, chest pain, or near syncope.

Class 4: Patients with PH with inability to carry out any physical activity without symptoms. These patients manifest signs of right-heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.

An infusion pump infuses (pumps) Flolan into my system through a central line.

I was going to actually take a picture of my site today, but I couldn't quite figure out how to do it modestly. But, this drawing will give you an idea.

My site is located on the right side, about 8 inches down from my collar bone. I also have two lumens (tubes with connectors) so that if there is a clog in one line, I can get the Flolan going through the other. That's important because Flolan has a short "half-life." That means that it only stays in your system for a short time. I can't go for more than 8-10 minutes without Flolan before I could possibly have a rebound effect and go into heart failure. Flolan also needs to be cold in order for it to work effectively. So, I always have a couple of ice packs right next to the Flolan pump in the fanny pack that I always wear around my waist.

I also have to always have a cassette of Flolan mixed and ready to be used, along with an additional Flolan pump in case of emergencies. I actually had my pump fail once at the end of the day during my last year of teaching. My teammate, principal, nurse and I had practiced what to do in an emergency. I can proudly say that we all stayed calm and I got everything switched out in less than 10 minutes.

Every night between 9-10pm, I mix my Flolan for the next day. I first take the cassette out of the refrigerator that I mixed the night before. I hook that up to my extra pump and then switch out the pump I used since last night. Then I mix a new cassette that I will have in case of an emergency. If I don't have an emergency, then that cassette is used the next night and so on and so on....

Thankfully, the only time I've had "an emergency" was that one time at school in 2005.
The picture above shows all of the things I need to "mix my medicine" every night. You may notice the neat little tray with the holes cut to hold the bottles of medicine and the cassette. My beloved husband made this little tray to help me with my mixing. Without it, I was having a hard time keeping everything from falling over.

I am so very grateful that Flolan is an effective treatment for me. But, along with the medicine comes the side effects. The side effects that I experience the most are flushing (I always look like I have a very healthy glow to my cheeks and red rashes on my chest and arms), headache, jaw pain (this is an odd one) and joint and muscle pain. These side effects are manageable with pain meds (except of course for the flushing).

Well, I think that's it for now.

No, I forgot something very, very important. I would like to thank you all for your continued prayers for our phriend, Mason.

I can happily report that Mason has been opening his eyes a bit, he also sticks out his tongue, and he's moving his arms. His mom has had to lay on Mason's arms because she just knows he'll rip out the ventilator tube if he gets a hold of it. They think that is the biggest reason why he is still sedated.

Mason's doctors have said that Mason had internal bleeding in his lungs, which is now stopped. The only conclusion they have come up with as to why this happened is that Mason had a very bad reaction to the chronic rejection treatment he received last Monday and Tuesday. They do not know which part of the treatment caused this reaction, but they are working to figure that out. However, Mason will not ever have the photo again.

Mason's doctor is optimistic that Mason will pull through this, but it's going to take a very long time because Mason's lungs were damaged.

Mason's mom has also said that the doctors are discussing the possibility of Mason being listed for another transplant. He'll need to pull through this and gain some strength, but it sounds optimistic.

So thank you, thank you, thank you for your prayers for this special young man. Continued prayers for healing are greatly appreciated.

My dear sweet husband saw my tray of supplies laying out tonight and thought he'd surprise me and mix for me! I caught him in the act! What a sweetheart!

Thanks for reading.

9 comments to PH Awareness Blogging Day!:

Teddybear said...

WOW - didn't know all that about Flolan, but I am not on it either.

Thanks for sharing your nifty tray with us. Your husband did a great job on making it - could he patent it so others could use it?

I have to admit I was lol when you said you wanted to use a real picture but...

It is amazing how different everybodies blog is today. Blessings to you, Deb

Nancy said...

The tray your husband made is wonderful. Do you mind if I share a photo of it with my support group members who are on Flolan?

My co-leader demonstrated Flolan mixing at our last meeting. It was very informative for everyone.

Colleen said...

I've never seen the medicine being mixed. For some reason, I was picturing huge vats of substance being stirred together. Not that I am thinking you, or anyone on Flolan, are witches. LOL!! Thank you for sharing all this info, Annette!! This was a great post!

Colleen :)

Anonymous said...

What a great post..Annette:))
and you added the link to the Bloggers:)

Great tray that Rod made and what a keeper he is;)..Thx for the Flolan info.

And I am continuing to pray for "our" Mason...

Take care


paula54 said...

Very informative. But where, oh where, was Rod's mask? Do you not use a mask when you mix? I've been mixing flolan for almost 10 years! It's just part of my daily routine.

Kathy said...

Do you have extra trays? I would buy one off you...flolan users would love that!

TW(Terry) said...

Of all the blogs and stuff I've read over the years, this is without doubt the most informative and best written that I have ever read. I had often wondered about the mixing of the drugs and everything else that goes along with being on Flolan. I thank you for writing such a great blog, I feel like I've been educated and now can see what Flolan users go through. I'll never complain about taking my oral meds again.

Thanks Again

phtony said...

Hi Annette,

I echo TW's words: I found your post to be VERY educational & worthy of a website all it's own. It seems there's some teacher left in you yet :-)

Thanks for sharing & take care,


Annette Markin said...

Deb, you crack me up! I actually shared the tray design 4 years ago, shortly after Rod made the first one. I put a picture and description on the PHA discussion boards, but no one was interested at the time.
Nancy, feel free to show the photo of the Flolan tray to your support group.
Jen, I think our prayers are starting to help "Our Mason."
Colleen, thanks for realizing I'm not doing any "hocus-pocus" over here. Of course, if I could do "hocus-pocus" I'd "h-p" PH out of our lives. POOF!
Paula, when I was trained by the home health nurse 4 years ago, she didn't have me use a mask or gloves, just lots of handwashing and antibactirial(sp?) junk.
Kathy, shoot me an email and we can talk about any extra trays.
Tony and Terry, thank you...you two made my day.