Your PAH Story

[On screen text: PAH Initiative, Sponsored by United Therapeutics]

[A storybook titled “Your PAH Story” opens, revealing the following on screen text:

Welcome to your PAH Story - An Interactive Web Journey, brought to you by the PAH Initiative, where knowledge meets inspiration.

The PAH Initiative, sponsored by United Therapeutics, is committed to helping you navigate life with PAH.

Turn the page to begin your journey of exploration, where your story will intersect with others at different stages of their PAH journey.

You’ll learn new ways to think about PAH, your Risk Status, and different pathways for treatment options.

This is a work of fiction based on real experiences of those living with PAH. Any resemblance to actual persons or events is purely coincidental.]

[The book lies flat and the character selection screen appears with four characters to choose from.]

[On screen text: Select your character]

[The page turns to reveal Susan walking down a sidewalk, outside of a library on an autumn day. She encounters Patricia walking a dog on the right side of the screen.

-

[On screen text: Chapter 1: A Nice Day]

[On screen text: Meet Patricia

Patricia. Age: 47, Functional Class III. Has been on two medications (combination therapy ERA & PDE5) for her PAH for about a year.]

Susan: [Inner Thoughts] ”Wow, it’s Pat! I first met her soon after she was diagnosed. I wonder how she’s doing?”

Susan: “Hey Pat! Long time no see! How are things? I’m so glad to see you!”

Susan: “How are you feeling? Has it been almost a year since you were diagnosed?”

Patricia: ”It has, yeah. I’m feeling better. I appreciate you telling me about the doctor you have that specializes in PAH. I love my local doc too so learning that I could see a specialist who would work with my local doc, and I didn’t have to choose between them, was really eye-opening.”

Susan: “What did they have to say?”

Patricia: “She confirmed that I’m doing better, but wants to keep a close eye on things. We were discussing all the tests they do and she told me about an evaluation she does called a ‘risk assessment’. She said it’s a way to pull all my test results together into a single ‘risk status’. Risk status helps give her a more complete view of my treatment progress so far and my life expectancy - specifically, my chance of survival over the next 5 years. She did the assessment, and I found out my risk status is Intermediate Risk.”

[On screen text: Tap to look at Pat’s heart and lungs.]

[A side-by-side comparison view of Patricia’s heart and lungs compared to those of a person without PAH appears. Patricia’s heart is beating slightly faster than a person without PAH, which has a slow steady beat. She has less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are narrower and the blood moves through the vessels more slowly due to increased resistance.]

Susan: ”Intermediate? Is that bad?”

Patricia: “Well there are three levels of “risk status”, Low risk, Intermediate Risk, and High Risk. When I heard that I was Intermediate I was a little scared, but my doctor said while it’s best to get to low risk that any improvement is helpful. And I feel better knowing where I stand so I can make a plan with my doctor to improve.”

Susan: “Did she say what Low Risk means?”

Patricia: “She said as your risk status improves and moves toward Low-risk, you may feel better day-to-day and your chances of survival go up. I’m not gonna lie, that was a little scary to hear. But I’d rather know than not know. And now I can make a plan and adjust if I’m not getting where I need to be. I want to be around and do everything I can to try to get to Low Risk.”

Susan: ”How do you know if you’re getting better?”

Patricia: “Well, she said current guidelines recommend she assesses my risk status and PAH treatment every 3-6 months to see where I am and where I’m heading. And then depending on whether I improve and by how much we’ll adjust the plan to find the right path for me.”

Patricia: “Thanks Susan. [the dog whines] Ruffles needs to go, and I think I’ve talked your ear off today. I’ll see you soon. Thanks for chatting.”

[Autumn scene continues as Susan is walking home along a suburban street and reflecting on her conversation with Patricia.]

Susan: [Inner thoughts] “Risk status. I had never heard of that before!

I wonder what risk status I am? Maybe I should ask my PAH specialist about my risk status...”

[Chapter Ends]

-

[Susan is standing in her kitchen on the left side of the screen, Donna stands on the right. It is winter and snow can be seen outside the kitchen windows.]

[On screen text: Chapter 2: Catching Up With an Old Friend Over Dinner]

[On screen text: Meet Donna

Donna, Age: 54, Functional Class III. Has been on one medication for her PAH for a little more than a year]

Donna: “Thanks for having me over tonight. This kitchen smells wonderful!”

Susan: “Let’s hope it turns out well. I’m trying a new recipe - always a bad idea to experiment on your guests. But if it turns out terribly, please don’t tell anyone else, haha!”

Donna: “My lips will be sealed! Sorry I was late - I ran into traffic coming back from my appointment.”

Susan: “No problem. Believe me, I understand! How did your appointment go?”

Donna: ”It went well. The doc says I’m stable. Honestly, I’m just grateful to be feeling better since the doc finally figured out what was happening and put me on this medication.”

Susan: “That’s great to hear. I’m so glad.”

Donna: “Thanks. Oh! Right before I left, my doc gave me this brochure on how PAH works and the different substances in my lungs that may be out of balance, causing PAH symptoms. Unfortunately, there isn’t a test currently that can tell my doctor which ones are out of balance. It’s very interesting though. Do you want to see?”

Susan: “Sure.”

[On screen text: Read Brochure]

[The brochure unfolds. The brochure is the Your PAH Guide from the PAH Initiative opened to the left page of the PAH Treatment Options section.]

[On screen text: Understanding the balance. The small blood vessels in the lungs produce 3 natural substances that are important for healthy blood vessels.

[Nitric oxide: Helps keep blood vessels open]

[Endothelin: Makes blood vessels contract, or become more narrow]

[Prostacyclin: Helps keep blood vessels open, prevents clotting, and slow the thickening of blood vessels]

[The right amount of each of the 3 substances keeps the blood vessels healthy. This balance allows red blood cells to flow freely through the lungs and carry oxygen to the rest of the body. When you have PAH, the amount of the 3 substances changes and they become imbalanced.]

[The brochure shifts to the right page of the PAH Treatment Options section.

[On screen text: Finding the right treatment plan. You are your healthcare provider will decide which PAH medicines will work best for you.

[Nitric oxide: Phosphodiesterase-5 inhibitor (PDE-5i) Soluble guanylate cyclase stimulator (sGCS) Oral]

[Endothelin: Endothelin receptor antagonist (ERA) Oral]

[Prostacyclin: Prostacyclin-class therapy (PCY) Oral Infused (Pump therapy) Inhaled

[Treating PAH through only one pathway may not be enough for many patients.]

“Susan: “This is interesting. Can I borrow it to read later tonight?”

Donna: “Sure thing!”

Susan: “Thanks! Hey, can you grab some red wine from the basement? I’m going to throw a splash in this recipe.”

Donna: “Can do, I’ll be right back.””

[Donna walks to the basement, she walks offscreen and the scene fades out.]

[Donna returns from the basement, the scene fades back in as Donna is at the door and walks over to Susan. She’s holding a bottle of wine and is moving to sit down, catching her breath.]

Susan: “Are you okay?”

Donna: “I’ll be fine. I need to sit down.”

[On screen text: Tap to look at Donna’s heart and lungs]

[A side-by-side comparison view of Donna’s heart and lungs compared to those of a person without PAH appears. Donna’s heart is beating significantly faster than a person without PAH, which has a slow steady beat. She has less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are much narrower and the blood moves through the vessels more slowly due to increased resistance.]

[Scene returns to Donna sitting in the chair, holding the bottle of wine.]

Susan: “I’m sorry, I didn’t know you were feeling this way when I asked you to get the wine.”

Donna: “It’s okay. This is just something I have to deal with now.”

Susan: “...Your doc said you’re stable?

Donna: “He says I’m doing well. But sometimes I wonder.”

Susan: “Does your doctor see a lot of PAH patients?”

Donna: “Um... I’m not really sure? I may be the only one.”

Susan: “Has he mentioned anything about your risk status?”

Donna: “I don’t think so, no... Why?”

Susan: “I’ve been looking into this more. You know, I’ve been on treatment for less than two years and only recently did I learn about this stuff. If your doc isn’t bringing it up, it might be worth looking into it more and bringing it up with him.”

Donna: “But I’ve gotten better - doesn’t that mean my risk status is good?”

Susan: “Not necessarily. Risk status is a different way of looking at your PAH. Stability looks more at how you’re doing today compared to the past, while risk status is more about your future. You might be feeling better than when you were first diagnosed, but are you still improving?

Susan: “I added a medication at one point after talking with my doctor. I didn’t love the idea of taking more medicine, but it helped!”

Donna: “I didn’t know that. Do you think it really made a difference?”

Susan: “I think so. The way my doctor explained it was that for certain patients adding a therapy can help lead to additional symptom control, so you feel better and you’re able to do more. Every medication has potential side effects though so it’s important to talk to your doctor about that too.”

Donna: “I never knew that. I will ask my doctor about that next time.”

Susan: “If you’re still having symptoms, talk with your doc about your options. There may be more you can do!”

Susan: “Anyway, dinner’s ready. Let’s eat!”

[Scene fades to black]

[On screen text: After Dinner]

[Susan is sitting at the table by herself reading the Your PAH Guide brochure from the PAH Initiative that Donna let her borrow.]

Susan: “This is what the information from the PAH Initiative that Donna gave me says about these 3 pathways...”

[On screen text: Read Brochure]

[The brochure unfolds. The brochure is the Your PAH Guide from the PAH Initiative opened to the left page of the PAH Treatment Options section.]

[On screen text: Understanding the balance. The small blood vessels in the lungs produce 3 natural substances that are important for healthy blood vessels.

[Nitric oxide: Helps keep blood vessels open]

[Endothelin: Makes blood vessels contract, or become more narrow]

[Prostacyclin: Helps keep blood vessels open, prevents clotting, and slow the thickening of blood vessels]

[The right amount of each of the 3 substances keeps the blood vessels healthy. This balance allows red blood cells to flow freely through the lungs and carry oxygen to the rest of the body. When you have PAH, the amount of the 3 substances changes and they become imbalanced.]

[The brochure shifts to the right page of the PAH Treatment Options section.

[On screen text: Finding the right treatment plan. You are your healthcare provider will decide which PAH medicines will work best for you.

[Nitric oxide: Phosphodiesterase-5 inhibitor (PDE-5i) Soluble guanylate cyclase stimulator (sGCS) Oral]

[Endothelin: Endothelin receptor antagonist (ERA) Oral]

[Prostacyclin: Prostacyclin-class therapy (PCY) Oral Infused (Pump therapy) Inhaled

[Treating PAH through only one pathway may not be enough for many patients.]

[Susan reading from the brochure]

Susan: [Inner thoughts] “Hmm...It says here... ‘Today, PAH patients are typically treated with more than one medication. Because patients with PAH generally respond better when more than one substance pathway is treated, it’s suspected that many patients have more than 1 substance out of balance. Adding a PAH medication could lead to additional symptom control, so you feel better and you’re able to do more. If you are still experiencing symptoms, talk with your healthcare provider about your options.’”

Susan: “Hmm. Maybe I should talk to my doctor about this.”

[Chapter Ends]

-

[Susan is standing in a neighborhood next to Linda, who is gardening. It’s a beautiful spring day.]

[On screen text: Chapter 3: Springtime]

[On screen text: Meet Linda

Linda, Age: 47, Functional Class II. Takes two medications (a PDE5 and oral prostacyclin-class medication). Overcame initial challenges with side effects. She is in the same support group as you.]

Susan: “Linda! So great to see you. I’m sorry I had to miss the last patient support group so I didn’t see you last week.”

Linda: “Susan! Yes, I was looking for you. It was a great session.”

Susan: “How’s the garden coming along?”

Linda: “It’s growing nicely... I have some beautiful petunia seeds that I want to plant. Hopefully the rabbits won’t get them this year!”

Susan: “It’s so great to see that you can still garden. You must be doing well.”

Linda: “Overall I would say I’m doing well. I still have to plan my activities and make sure I make time to rest, but I can do more now than I could before, and I can do what I need to do each day a little more easily.”

[On screen text: Tap to look at Linda’s heart and lungs]

[A side-by-side comparison view of Linda’s heart and lungs compared to those of a person without PAH appears. Linda’s heart is beating at a slightly faster rate than a person without PAH, which has a slow steady beat. She has somewhat less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are only slightly narrower and the blood moves through the vessels at a slightly slower rate due to resistance.]

Susan: “What was the biggest change that got you to this point?”

Linda: “Honestly, it was working with my doctor to improve my risk status. We looked at my risk assessment results and we set a goal to help me improve my risk status by adding another medication, a prostacyclin-class medication. I didn’t love the idea of adding another medication, but I wanted to reduce my symptoms so I was open to it after she explained it to me more. And once she explained the impact that it could have if I get to Low Risk - I was ready to give it a try. I want to stick around as long as I can. I’ve got too much left I want to do.”

Susan: ”What kind of medicine is that? Oh wait, isn’t that the pump for very sick people?”

Linda: “They actually have it in pill and inhaled forms too now. I’m on the pill and it’s working well. It’s not just for sicker people - I’m using it before my symptoms get worse.”

Susan: “Don’t prostacyclin-class meds have side effects though?”

Linda: “Sure. The side effects vary some depending on how you take it, but common side effects can include headache, diarrhea, nausea, jaw pain, and flushing.”

Susan: “Does everyone get these?”

Linda: “No, but they are pretty common. My docs were familiar with these though, and they helped prepare me.”

Susan “Interesting. I’ve been talking a lot with my doctor lately about my risk status as well. It’s great to hear you got to low-risk! Way to go!”

Linda: “Thank you so much Susan. It’s been a hard journey sometimes but I feel it is worth it in the end. My doctor put it this way, the goal is to have fewer symptoms and to continue with my daily activities for as long as I can. My doctor told me people who get to low risk have a better prognosis. These flowers need me! They aren’t going to water themselves!”

Susan: “That’s a good way to think about it.”

Linda: “I know you said you’re pretty happy on your medications, but if you’re still having symptoms or find out you’re not getting to low risk status you should talk to your doctor about adding a therapy. It could make a difference for your future.”

Susan: “Yeah, that’s a good idea. It never hurts to see if there’s more I could be doing!”

Susan: “Well, I’m going to head to the store. I’ll see you at the next meeting!”

Linda: “See you, later, Susan. Thanks for stopping by.”

[Chapter Ends]

-

[Susan standing back in her kitchen, reflecting on the past year when she has learned so much.]

[On screen text: Chapter 4: Your Story]

Susan: [Inner thoughts] “What a year - I’ve learned a lot.”

Susan: “Takeaway #1: Find a PAH Specialist”

Susan: “It’s good to know I’m doing the right thing by seeing a doctor that specializes in PAH. PAH is a complex disease, and it requires the expertise of a PAH specialist. I’m glad Pat is seeing a PAH expert also!”

Susan: “Takeaway #2: Ask about risk status”

Susan: “I am feeling pretty good about my current treatment plan, but it might be time to ask my doctor about risk status to see if there might be more I could do. I’m glad Pat mentioned risk status to me and it was really helpful talking things through with Donna, but now it’s my move. My doctor is helping me in this but ultimately, this is my life, my PAH, and my opportunity to get to Low Risk status.”

Susan: “Takeaway #3: Maybe there’s more I can do”

Susan: “It was so great running into Linda the other day! And our conversation really got me thinking. Since I’m still having symptoms, there may be more I can do to improve. I’ll talk to my doctor about my goals and what we can change. Adjusting my dose or adding a medication may help better control my symptoms.”

Susan: “Takeaway #4: Ask about prostacyclin”

Susan: “I think I am going to ask my doctor about prostacyclin-class medicine. Linda said it’s available in pill or inhaled forms, as well as a pump. She wasn’t waiting for her symptoms to get any worse!”

Susan: “If prostacyclin-class medicines have been shown to help control symptoms so I can do my everyday activities and improve my risk status, I should ask my doctor about them.”

[Chapter Ends]

-

[On screen text: Your Story: What will your story be?

Learn more about PAH, Risk Status, and available treatments.

Find a PAH specialist.

Learn more about prostacyclin-class therapies and what you can do to fight PAH.]

[On screen text: PAH Initiative, Sponsored by United Therapeutics]

[A storybook titled “Your PAH Story” opens, revealing the following text:

Welcome to your PAH Story - An Interactive Web Journey, brought to you by the PAH Initiative, where knowledge meets inspiration.

The PAH Initiative, sponsored by United Therapeutics, is committed to helping you navigate life with PAH.

Turn the page to begin your journey of exploration, where your story will intersect with others at different stages of their PAH journey.

You’ll learn new ways to think about PAH, your Risk Status, and different pathways for treatment options.

This is a work of fiction based on real experiences of those living with PAH. Any resemblance to actual persons or events is purely coincidental.]

-

[The book lies flat and the character selection screen appears with four characters to choose from]

[On screen text: Select your character]

[The page turns to reveal Joseph walking down a sidewalk, outside of a library on an autumn day. He encounters Patricia walking a dog on the right side of the screen.]

-

[On screen text: Chapter 1: A Nice Day]

[On screen text: Meet Patricia

Patricia. Age: 47, Functional Class III. Has been on two medications (combination therapy ERA & PDE5) for her PAH for about a year.]

Joseph: [Inner Thoughts] ”Wow, it’s Pat! I first met her soon after she was diagnosed. I wonder how she’s doing?”

Joseph: “Hey Pat! Long time no see! How are things? I’m so glad to see you!”

Joseph: “How are you feeling? Has it been almost a year since you were diagnosed?

Patricia: “It has, yeah. I’m feeling better. I appreciate you telling me about the doctor you have that specializes in PAH. I actually just started seeing this doctor who sees lots of PAH patients. I love my local doc, and so when I realized I could see a specialist who would work with my local doc, and I didn’t have to choose between them, it was really eye-opening.”

Joseph: “‘That’s interesting. I appreciate you telling me about that. I’ll look into it.”

Joseph: Did you learn anything new?

Patricia: “She confirmed that I’m doing better, but wants to keep a close eye on things. We were discussing all the tests they do and she told me about an evaluation she does called a ‘risk assessment’. She said it’s a way to pull all my test results together into a single ‘risk status’. Risk status helps give her a more complete view of my treatment progress so far and my life expectancy - specifically, my chance of survival over the next 5 years. She did the assessment, and I found out my risk status is Intermediate Risk.”

[On screen text: Tap to look at Pat’s heart and lungs]

[A side-by-side view of Patricia’s heart and lungs compared to those of a person without PAH appears. Patricia’s heart is beating slightly faster than a person without PAH, which has a slow steady beat. She has less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are narrower and the blood moves through the vessels more slowly due to increased resistance.]

“Joseph: ”Intermediate? Is that bad?”

Patricia: “Well there are three levels of “risk status”, Low risk, Intermediate Risk, and High Risk. When I heard that I was Intermediate I was a little scared, but my doctor said while it’s best to get to low risk that any improvement is helpful. And I feel better knowing where I stand so I can make a plan with my doctor to improve.”

Joseph: “Did she say what Low Risk means?”

Patricia: “She said as your risk status improves and moves toward Low-risk, you may feel better day-to-day and your chances of survival go up. I’m not gonna lie, that was a little scary to hear. But I’d rather know than not know. And now I can make a plan and adjust if I’m not getting where I need to be. I want to be around and do everything I can to try to get to Low Risk.”

Joseph: ”How do you know if you’re getting better?”

Patricia: “Well, she said current guidelines recommend she assesses my risk status and PAH treatment every 3-6 months to see where I am and where I’m heading. And then depending on whether I improve and by how much we’ll adjust the plan to find the right path for me.”

Patricia: “Thanks Joseph. [the dog whines] Ruffles needs to go, and I think I’ve talked your ear off today. I’ll see you soon. Thanks for chatting.”

[Autumn scene continues as Joseph is walking home along a suburban street and reflecting on his conversation with Patricia.]

Joseph: [Inner thoughts] “Risk status. I had never heard of that before! I wonder what risk status I am? Maybe I should look into seeing a PAH expert as well...”

[Chapter Ends]

-

[Joseph is standing in his kitchen on the left side of the screen, Donna stands on the right. It is winter and snow can be seen outside the kitchen windows.

[On screen text: Chapter 2: Catching Up With an Old Friend Over Dinner]

[On screen text: Meet Donna

Donna, Age: 54, Functional Class III. Has been on one medication for her PAH for a little more than a year.]

Donna: “Thanks for having me over tonight. This kitchen smells wonderful!”

Joseph: “Let’s hope it turns out well. I’m trying a new recipe - always a bad idea to experiment on your guests. But if it turns out terribly, please don’t tell anyone else, haha!”

Donna: “My lips will be sealed! Sorry I was late - I ran into traffic coming back from my appointment.”

Joseph: “No problem. Believe me, I understand! How did your appointment go?”

Donna: “It went well. The doc says I’m stable. Honestly, I’m just grateful to be feeling better since the doc finally figured out what was happening and put me on this medication.”

Joseph: “That’s great to hear. I’m so glad.”

Donna: “Thanks. Oh! Right before I left, my doc gave me this brochure on how PAH works and the different substances in my lungs that may be out of balance, causing PAH symptoms. Unfortunately, there isn’t a test currently that can tell my doctor which ones are out of balance. It’s very interesting though. Do you want to see?”

Joseph: “Sure.”

[On screen text: Read Brochure]

[The brochure unfolds. The brochure is the Your PAH Guide from the PAH Initiative opened to the left page of the PAH Treatment Options section.]

[On screen text: Understanding the balance. The small blood vessels in the lungs produce 3 natural substances that are important for healthy blood vessels.

[Nitric oxide: Helps keep blood vessels open]

[Endothelin: Makes blood vessels contract, or become more narrow]

[Prostacyclin: Helps keep blood vessels open, prevents clotting, and slow the thickening of blood vessels]

[The right amount of each of the 3 substances keeps the blood vessels healthy. This balance allows red blood cells to flow freely through the lungs and carry oxygen to the rest of the body. When you have PAH, the amount of the 3 substances changes and they become imbalanced.]

[The brochure shifts to the right page of the PAH Treatment Options section.

[On screen text: Finding the right treatment plan. You are your healthcare provider will decide which PAH medicines will work best for you.

[Nitric oxide: Phosphodiesterase-5 inhibitor (PDE-5i) Soluble guanylate cyclase stimulator (sGCS) Oral]

[Endothelin: Endothelin receptor antagonist (ERA) Oral]

[Prostacyclin: Prostacyclin-class therapy (PCY) Oral Infused (Pump therapy) Inhaled

[Treating PAH through only one pathway may not be enough for many patients.]

Joseph: “This is interesting. Can I borrow it to read later tonight?”

Donna: “Sure thing!”

Joseph: “Thanks! Hey, can you grab some red wine from the basement? I’m going to throw a splash in this recipe.”

Donna: “Can do, I’ll be right back.””

[Donna walks to the basement, she walks offscreen and the scene fades out.]

[Donna returns from the basement, the scene fades back in as Donna is at the door and walks over to Joseph. She’s holding a bottle of wine and is moving to sit down, catching her breath.]

Joseph: “Are you okay?”

Donna: “I’ll be fine. I need to sit down.”

[On screen text: Tap to look at Donna’s heart and lungs]

[A side-by-side comparison view of Donna’s heart and lungs compared to those of a person without PAH appears. Donna’s heart is beating significantly faster than a person without PAH, which has a slow steady beat. She has less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are much narrower and the blood moves through the vessels more slowly due to increased resistance.]

[Scene returns to Donna sitting in the chair, holding the bottle of wine.]

Joseph: “I know that feeling. I’m still struggling with stairs as well. I’m sorry. I shouldn’t have asked you to do that.”

Donna: “It’s okay. This happens all the time.”

Joseph: “...Your doc said you’re stable?

Donna: “He says I’m doing well. But sometimes I wonder.”

Joseph: “Does your doctor see a lot of PAH patients?”

Donna: “Um... I’m not really sure? I may be the only one.”

Joseph: “Has he mentioned anything about your risk status?”

Donna: “I don’t think so, no... Why?”

Joseph: “I’ve been looking into this more. You know, I’ve been on treatment for less than three years and only recently did I learn about this stuff. If your doc isn’t bringing it up, it might be worth looking into it more and bringing it up with him.”

Donna: “But I’ve gotten better - doesn’t that mean my risk status is good?”

Joseph: “Not necessarily. Risk status is a different way of looking at your PAH. Stability looks more at how you’re doing today compared to the past, while risk status is more about your future. You might be feeling better than when you were first diagnosed, but are you still improving?

Joseph: “I’m on two medications and I feel like I’m doing ok, but I recently heard current guidelines suggest adding an additional medication may be beneficial for people who have not yet reached low-risk status.”

Donna: “That makes sense. Do you think it really makes a difference?”

Joseph: “I think so? It could help get symptoms even more under control, so you feel better, you’re able to do more, and maybe have more “good days”. I know guidelines are built on the collective wisdom of PAH docs across the world so there must be good data behind it. I’m thinking of asking my doctor about it.”

Donna: “I never knew that. I will ask my doctor about that next time.”

Joseph: “If you’re still having symptoms, talk with your doc about your options. There may be more you can do!”

Joseph: “Anyway, dinner’s ready. Let’s eat!”

[Scene fades to black]

[On screen text: After Dinner]

[Joseph is sitting at the table by himself reading the Your PAH Guide brochure from the PAH Initiative Donna let her borrow.]

Joseph: “This is what the information from the PAH Initiative that Donna gave me says about these 3 pathways...”

[On screen text: Read Brochure]

[The brochure unfolds. The brochure is the Your PAH Guide from the PAH Initiative opened to the left page of the PAH Treatment Options section.]

[On screen text: Understanding the balance. The small blood vessels in the lungs produce 3 natural substances that are important for healthy blood vessels.]

[Nitric oxide: Helps keep blood vessels open]

[Endothelin: Makes blood vessels contract, or become more narrow]

[Prostacyclin: Helps keep blood vessels open, prevents clotting, and slow the thickening of blood vessels]

[The right amount of each of the 3 substances keeps the blood vessels healthy. This balance allows red blood cells to flow freely through the lungs and carry oxygen to the rest of the body. When you have PAH, the amount of the 3 substances changes and they become imbalanced.]

[The brochure shifts to the right page of the PAH Treatment Options section.

[On screen text: Finding the right treatment plan. You are your healthcare provider will decide which PAH medicines will work best for you.

[Nitric oxide: Phosphodiesterase-5 inhibitor (PDE-5i) Soluble guanylate cyclase stimulator (sGCS) Oral]

[Endothelin: Endothelin receptor antagonist (ERA) Oral]

[Prostacyclin: Prostacyclin-class therapy (PCY) Oral Infused (Pump therapy) Inhaled

[Treating PAH through only one pathway may not be enough for many patients.]

[Joseph reading from the PAHI material]

Joseph: [Inner thoughts] “Hmm...It says here... ‘Today, PAH patients are typically treated with more than one medication. Because patients with PAH generally respond better when more than one substance pathway is treated, it’s suspected that many patients have more than 1 substance out of balance. Adding a PAH medication could lead to additional symptom control, so you feel better and you’re able to do more. If you are still experiencing symptoms, talk with your healthcare provider about your options.’”

Joseph: “Hmm. Maybe I should talk to my doctor about this.”

[Chapter Ends]

-

[Joseph is standing in a neighborhood next to Linda, who is gardening. It’s a beautiful spring day

[On screen text: Chapter 3: Springtime]

[On screen text: Meeting Linda

Linda, Age: 47, Functional Class II. Takes two medications (a PDE5 and oral prostacyclin-class medication). Overcame initial challenges with side effects. She is in the same support group as you.]

Joseph: “Linda! So great to see you. I’m sorry I had to miss the last patient support group so I didn’t see you last week.”

Linda: “Joseph! Yes, I was looking for you. It was a great session.”

Joseph: “How’s the garden coming along?”

Linda: “It’s growing nicely... I have some beautiful petunia seeds that I want to plant. Hopefully the rabbits won’t get them this year!”

Joseph: “It’s so great to see that you can still garden. You must be doing well.”

Linda: “Overall I would say I’m doing well. I still have to plan my activities and make sure I make time to rest, but I can do more now than I could before, and I can do what I need to do each day a little more easily.”

[On screen text: Tap to look at Linda’s heart and lungs]

[A side-by-side comparison view of Linda’s heart and lungs compared to those of a person without PAH appears. Linda’s heart is beating at a slightly faster rate than a person without PAH, which has a slow steady beat. She has somewhat less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are only slightly narrower and the blood moves through the vessels at a slightly slower rate due to resistance.]

Joseph: “What was the biggest change that got you to this point?”

Linda: “Honestly, it was working with my doctor to improve my risk status. We looked at my risk assessment results and we set a goal to help me improve my risk status by adding another medication, a prostacyclin-class medication. I didn’t love the idea of adding another medication, but I wanted to reduce my symptoms so I was open to it after she explained it to me more. And once she explained the impact that it could have if I get to Low Risk - I was ready to give it a try. I want to stick around as long as I can. I’ve got too much left I want to do.”

Joseph: ”What kind of medicine is that? Oh wait, isn’t that the pump for very sick people?”

Linda: “They actually have it in pill and inhaled forms too now. I’m on the pill and it’s working well. It’s not just for sicker people - I’m using it before my symptoms get worse.”

Joseph: “Don’t prostacyclin-class meds have side effects though?”

Linda: “Sure. The side effects vary some depending on how you take it, but common side effects can include headache, diarrhea, nausea, jaw pain, and flushing.”

Joseph: “Does everyone get these?”

Linda: “No, but they are pretty common. My docs were familiar with these though, and they helped prepare me.”

Joseph “Interesting. I’ve been talking a lot with my doctor lately about my risk status as well. It’s great to hear you got to low-risk! Way to go!”

Linda: “Thank you so much Joseph. It’s been a hard journey sometimes but I feel it is worth it in the end. My doctor put it this way, the goal is to have fewer symptoms and to continue with my daily activities for as long as I can. My doctor told me people who get to low risk have a better prognosis. These flowers need me! They aren’t going to water themselves!”

Joseph: “That’s a good way to think about it.

Linda: “At our meeting you said you are feeling better than you did, but still wish your meds did more to help your symptoms. You should ask your doctor about your risk status and about adding a therapy. There may be more you can do and it could make a difference for your future.”

Joseph: “Yeah, I know I should. I just figured she’d let me know if there’s something else out there, you know?”

Linda: “I hear you. Like we were talking about at the last meeting though, it’s up to us to be our own health advocates. Sometimes we’re the ones who have to start that conversation.”

Joseph: “Yeah, that’s true. I appreciate the encouragement.”

Joseph: “Well, I’m going to head to the store. I’ll see you at the next meeting!”

Linda: “See you, later, Joseph. Thanks for stopping by.”

[Chapter Ends]

-

[Joseph standing back in his kitchen, reflecting on the past year when he has learned so much.]

[On screen text: Chapter 4: Your Story].

Joseph: [Inner thoughts] “What a year - I’ve learned a lot.”

Joseph: “Takeaway #1: Find a PAH Specialist”

Joseph: “I wonder if I should look into finding a PAH specialist. I really like my doctor, but Pat said she was able to see a PAH expert and still keep seeing her existing doctor.”

Joseph: “Takeaway #2: Ask about risk status”

Joseph: “I still have some challenges with everyday activities; I think it might be time to ask more seriously about my risk status. I’m glad Pat mentioned risk status to me and it was really helpful talking things through with Donna, but now it’s my move. My doctor is helping me in this but ultimately, this is my life, my PAH, and my opportunity to get to Low Risk status.”

Joseph: “Takeaway #3: Maybe there’s more I can do”

Joseph: “It was so great running into Linda the other day! And our conversation really got me thinking. Since I’m still having symptoms, there may be more I can do to improve. I’ll talk to my doctor about my goals and what we can change. Adjusting my dose or adding a medication may help better control my symptoms.”

Joseph: “Takeaway #4: Ask about prostacyclin”

Joseph: “I think I am going to ask my doctor about prostacyclin-class medicine. Linda said it’s available in pill or inhaled forms, as well as a pump. She wasn’t waiting for her symptoms to get any worse!”

Joseph: “If prostacyclin-class medicines have been shown to help control symptoms so I can do my everyday activities and improve my risk status, I should ask my doctor about them.”

[Chapter Ends]

-

[On screen text: Your Story: What will your story be?

Learn more about PAH and Risk Status.

Find a PAH specialist.

Learn more about prostacyclin-class therapies and what you can do to fight PAH.]

[On screen text: PAH Initiative, Sponsored by United Therapeutics]

[A storybook titled “Your PAH Story” opens, revealing the following on screen text:

Welcome to your PAH Story - An Interactive Web Journey, brought to you by the PAH Initiative, where knowledge meets inspiration.

The PAH Initiative, sponsored by United Therapeutics, is committed to helping you navigate life with PAH.

Turn the page to begin your journey of exploration, where your story will intersect with others at different stages of their PAH journey.

You’ll learn new ways to think about PAH, your Risk Status, and different pathways for treatment options.

This is a work of fiction based on real experiences of those living with PAH. Any resemblance to actual persons or events is purely coincidental.]

-

[The book lies flat and the character selection screen appears with four characters to choose from]

[On screen text: Select your character.]

[The page turns to reveal Michelle walking down a sidewalk, outside of a library on an autumn day. She encounters Patricia walking a dog on the right side of the screen.

-

[On screen text: Chapel 1: A Nice Day]

[On screen text: Meet Patricia

Patricia. Age: 47, Functional Class III. Has been on two medications (combination therapy ERA & PDE5) for her PAH for about a year.]

Michelle: [Inner Thoughts] ”Wow, it’s Pat! I first met her soon after she was diagnosed. I wonder how she’s doing?”

Michelle: “Wow, funny running into you here! How long has it been?”

Michelle: “How are you feeling? Has it been almost a year since you were diagnosed?”

Patricia: “It has, yeah. I’m feeling better. I appreciate you telling me about the doctor you have that specializes in PAH. I actually just started seeing this doctor who sees lots of PAH patients. I love my local doc, and so when I realized I could see a specialist who would work with my local doc, and I didn’t have to choose between them, it was really eye-opening.”

Michelle: “‘That’s interesting. I appreciate you telling me about that. I’ll look into it.”

Michelle: “How did it go?”

Patricia: “She confirmed that I’m doing better, but wants to keep a close eye on things. We were discussing all the tests they do and she told me about an evaluation she does called a ‘risk assessment’. She said it’s a way to pull all my test results together into a single ‘risk status’. Risk status helps give her a more complete view of my treatment progress so far and my life expectancy - specifically, my chance of survival over the next 5 years. She did the assessment, and I found out my risk status is Intermediate Risk.”

[On screen text: Tap to look at Pat’s heart and lungs.]

[A side-by-side comparison view of Patricia’s heart and lungs compared to those of a person without PAH appears. Patricia’s heart is beating slightly faster than a person without PAH, which has a slow steady beat. She has less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are narrower and the blood moves through the vessels more slowly due to increased resistance.]

Michelle: ”Intermediate? Is that bad?”

Patricia: “Well there are three levels of “risk status”, Low risk, Intermediate Risk, and High Risk. When I heard that I was Intermediate I was a little scared, but my doctor said while it’s best to get to low risk that any improvement is helpful. And I feel better knowing where I stand so I can make a plan with my doctor to improve.”

Michelle: “Did she say what Low Risk means?”

Patricia: “She said as your risk status improves and moves toward Low-risk, you may feel better day-to-day and your chances of survival go up. I’m not gonna lie, that was a little scary to hear. But I’d rather know than not know. And now I can make a plan and adjust if I’m not getting where I need to be. I want to be around and do everything I can to try to get to Low Risk.”

Michelle: “How do you know if you’re getting better?”

Patricia: “Well, she said current guidelines recommend she assesses my risk status and PAH treatment every 3-6 months to see where I am and where I’m heading. And then depending on whether I improve and by how much we’ll adjust the plan to find the right path for me.

Patricia: “Thanks Michelle. [the dog whines] Ruffles needs to go, and I think I’ve talked your ear off today. I’ll see you soon. Thanks for chatting.”

[Autumn scene continues as Michelle is walking home along a suburban street and reflecting on her conversation with Patricia.]

Michelle: [Inner thoughts] “Risk status. I had never heard of that before! I wonder what risk status I am? Maybe I should look into seeing a PAH expert as well...”

[Chapter Ends]

-

[Michelle is standing in her kitchen on the left side of the screen, Donna stands on the right. It is winter and snow can be seen outside the kitchen windows.

[On screen text: Chapter 2 Catching Up With an Old Friend Over Dinner]

[One screen text: Meet Donna

Donna, Age: 54. Functional Class III. Has been on one medication for her PAH for a little more than a year.]

Donna: “Thanks for having me over tonight. This kitchen smells wonderful!”

Michelle: “Let’s hope it turns out well. I’m trying a new recipe - always a bad idea to experiment on your guests. But if it turns out terribly, please don’t tell anyone else, haha!”

Donna: “My lips will be sealed! Sorry I was late - I ran into traffic coming back from my appointment.”

Michelle: “No problem. Believe me, I understand! How did your appointment go?”

Donna: “It went well. The doc says I’m stable. Honestly, I’m just grateful to be feeling better since the doc finally figured out what was happening and put me on this medication.”

Michelle: “That’s great to hear. I’m so glad.”

Donna: “Thanks. Oh! Right before I left, my doc gave me this brochure on how PAH works and the different substances in my lungs that may be out of balance, causing PAH symptoms. Unfortunately, there isn’t a test currently that can tell my doctor which ones are out of balance. It’s very interesting though. Do you want to see?”

Michelle: “Sure.”

[On screen text: Read Brochure]

[The brochure unfolds. The brochure is the Your PAH Guide from the PAH Initiative opened to the left page of the PAH Treatment Options section.]

[On screen text: Understanding the balance. The small blood vessels in the lungs produce 3 natural substances that are important for healthy blood vessels.

[Nitric oxide: Helps keep blood vessels open]

[Endothelin: Makes blood vessels contract, or become more narrow]

[Prostacyclin: Helps keep blood vessels open, prevents clotting, and slow the thickening of blood vessels]

[The right amount of each of the 3 substances keeps the blood vessels healthy. This balance allows red blood cells to flow freely through the lungs and carry oxygen to the rest of the body. When you have PAH, the amount of the 3 substances changes and they become imbalanced.]

[The brochure shifts to the right page of the PAH Treatment Options section.

[On screen text: Finding the right treatment plan. You are your healthcare provider will decide which PAH medicines will work best for you.

[Nitric oxide: Phosphodiesterase-5 inhibitor (PDE-5i) Soluble guanylate cyclase stimulator (sGCS) Oral]

[Endothelin: Endothelin receptor antagonist (ERA) Oral]

[Prostacyclin: Prostacyclin-class therapy (PCY) Oral Infused (Pump therapy) Inhaled

[Treating PAH through only one pathway may not be enough for many patients.]

“Michelle: “This is interesting. Can I borrow it to read later tonight?”

Donna: “Sure thing!”

Michelle: “Thanks! Hey, can you grab some red wine from the basement? I’m going to throw a splash in this recipe.”

Donna: “Can do, I’ll be right back.””

[Donna walks to the basement, she walks offscreen and the scene fades out.]

[Donna returns from the basement, the scene fades back in as Donna is at the door and walks over to Michelle. She’s holding a bottle of wine and is moving to sit down, catching her breath.]

Michelle: “Are you okay?”

Donna: “I’ll be fine. I need to sit down.”

[On screen text: Tap to look at Donna’s heart and lungs.]

[A side-by-side comparison view of Donna’s heart and lungs compared to those of a person without PAH appears. Donna’s heart is beating significantly faster than a person without PAH, which has a slow steady beat. She has less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are much narrower and the blood moves through the vessels more slowly due to increased resistance.]

[Scene returns to Donna sitting in the chair, holding the bottle of wine.]

Michelle: “I know that feeling. I’m still struggling with stairs as well. I’m sorry. I shouldn’t have asked you to do that.”

Donna: “It’s okay. This happens all the time.”

Michelle: [Concerned look, and pauses] “...Your doc said you’re stable?

Donna: “He says I’m doing well. But sometimes I wonder.”

Michelle: “Does your doctor see a lot of PAH patients?”

Donna: “Um... I’m not really sure? I may be the only one.”

Michelle: “Has he mentioned anything about your risk status?”

Donna: “I don’t think so, no... Why?”

Michelle: “I’ve been looking into this more. You know, I’ve been on treatment for less a year ago and only recently did I learn about this stuff. If your doc isn’t bringing it up, it might be worth looking into it more and bringing it up with him.”

Donna: “But I’ve gotten better - doesn’t that mean my risk status is good?”

Michelle: “Not necessarily. Risk status is a different way of looking at your PAH. Stability looks more at how you’re doing today compared to the past, while risk status is more about your future. You might be feeling better than when you were first diagnosed, but are you still improving?

Michelle: “I feel better than when I was diagnosed, but I’m not where I want to be either. I recently heard medical guidelines recommend most patients should be on at least two medications.”

Donna: “I’d never heard that. Do you think it really makes a difference?”

Michelle: “I think so. I know guidelines are built on clinical trial data from various PAH studies and the collective wisdom of PAH docs across the world, so there must be good data behind it. It sounded like using at least two medications is important to getting symptoms under control.”

Donna: “I never knew that. I will ask my doctor about that next time.”

Michelle: “If you’re still having symptoms, talk with your doc about your options. There may be more you can do!”

Michelle: “Anyway, dinner’s ready. Let’s eat!”

[Scene fades to black]

[On screen text: After Dinner]

[Michelle is sitting at the table by herself reading the Your PAH Guide from the PAH Initiative brochure that Donna let her borrow.]

Michelle: “This is what the information from the PAH Initiative that Donna gave me says about these 3 pathways...”

[On screen text: Read Brochure]

The brochure unfolds. The brochure is the Your PAH Guide from the PAH Initiative opened to the left page of the PAH Treatment Options section.]

[On screen text: Understanding the balance. The small blood vessels in the lungs produce 3 natural substances that are important for healthy blood vessels.

[Nitric oxide: Helps keep blood vessels open]

[Endothelin: Makes blood vessels contract, or become more narrow]

[Prostacyclin: Helps keep blood vessels open, prevents clotting, and slow the thickening of blood vessels]

[The right amount of each of the 3 substances keeps the blood vessels healthy. This balance allows red blood cells to flow freely through the lungs and carry oxygen to the rest of the body. When you have PAH, the amount of the 3 substances changes and they become imbalanced.]

[The brochure shifts to the right page of the PAH Treatment Options section.

[On screen text: Finding the right treatment plan. You are your healthcare provider will decide which PAH medicines will work best for you.

[Nitric oxide: Phosphodiesterase-5 inhibitor (PDE-5i) Soluble guanylate cyclase stimulator (sGCS) Oral]

[Endothelin: Endothelin receptor antagonist (ERA) Oral]

[Prostacyclin: Prostacyclin-class therapy (PCY) Oral Infused (Pump therapy) Inhaled

[Treating PAH through only one pathway may not be enough for many patients.]

[Michelle reading from the PAHI material]

Michelle: [Inner thoughts] “Hmm...It says here... ‘Today, PAH patients are typically treated with more than one medication. Because patients with PAH generally respond better when more than one substance pathway is treated, it’s suspected that many patients have more than 1 substance out of balance. Adding a PAH medication could lead to additional symptom control, so you feel better and you’re able to do more. If you are still experiencing symptoms, talk with your healthcare provider about your options.’”

“Hmm. Maybe I should talk to my doctor about this.”

[Chapter Ends]

-

[Michelle is standing in a neighborhood next to Linda, who is gardening. It’s a beautiful spring day.]

[On screen text: Chapter 3Springtime]

[On screen text: Meet Linda

Linda, Age: 47, Functional Class II. Takes two medications (a PDE5 and oral prostacyclin-class medication). Overcame initial challenges with side effects. She is in the same support group as you.]

Michelle: “Linda! So great to see you. I’m sorry I had to miss the last patient support group so I didn’t see you last week.”

Linda: “Michelle! Yes, I was looking for you. It was a great session.”

Michelle: “How’s the garden coming along?”

Linda: “It’s growing nicely... I have some beautiful petunia seeds that I want to plant. Hopefully the rabbits won’t get them this year!”

Michelle: “It’s so great to see that you can still garden. You must be doing well.”

Linda: “Overall I would say I’m doing well. I still have to plan my activities and make sure I make time to rest, but I can do more now than I could before, and I can do what I need to do each day a little more easily.”

[On screen text: Tap to look at Linda’s heart and lungs]

[A side-by-side comparison view of Linda’s heart and lungs compared to those of a person without PAH appears. Linda’s heart is beating at a slightly faster rate than a person without PAH, which has a slow steady beat. She has somewhat less vascular branching in the blood vessels in her lungs that the person without PAH. Her blood vessels are only slightly narrower and the blood moves through the vessels at a slightly slower rate due to resistance.]

Michelle: “What was the biggest change that got you to this point?”

Linda: “Honestly, it was working with my doctor to improve my risk status. We looked at my risk assessment results and we set a goal to help me improve my risk status by adding another medication, a prostacyclin-class medication. I didn’t love the idea of adding another medication, but I wanted to reduce my symptoms so I was open to it after she explained it to me more. And once she explained the impact that it could have if I get to Low Risk - I was ready to give it a try. I want to stick around as long as I can. I’ve got too much left I want to do.”

Michelle: ”What kind of medicine is that? Oh wait, isn’t that the pump for very sick people?”

Linda: “They actually have it in pill and inhaled forms too now. I’m on the pill and it’s working well. It’s not just for sicker people - I’m using it before my symptoms get worse.”

Michelle: “Don’t prostacyclin-class meds have side effects though?”

Linda: “Sure. The side effects vary some depending on how you take it, but common side effects can include headache, diarrhea, nausea, jaw pain, and flushing.”

Michelle: “Does everyone get these?”

Linda: “No, but they are pretty common. My docs were familiar with these though, and they helped prepare me.”

Michelle: “Interesting. I’ve been talking a lot with my doctor lately about my risk status as well. It’s great to hear you got to low-risk! Way to go!”

Linda: “Thank you so much Michelle. It’s been a hard journey sometimes but I feel it is worth it in the end. My doctor put it this way, the goal is to have fewer symptoms and to continue with my daily activities for as long as I can. My doctor told me people who get to low risk have a better prognosis. These flowers need me! They aren’t going to water themselves!”

Michelle: “That’s a good way to think about it.”

Linda: “At our meeting you said you are feeling better than you did, but still wish your meds did more to help your symptoms. You should ask your doctor about your risk status and about adding a therapy. There may be more you can do and it could make a difference for your future.”

Michelle: “Yeah, I know I should. I just figured she’d let me know if there’s something else out there, you know?”

Linda: “I hear you. Like we were talking about at the last meeting though, it’s up to us to be our own health advocates. Sometimes we’re the ones who have to start that conversation.”

Michelle: “Yeah, that’s true. I appreciate the encouragement.”

Michelle: “Well, I’m going to head to the store. I’ll see you at the next meeting!”

Linda: See you, later, Michelle. Thanks for stopping by.”

[Chapter Ends]

-

[Michelle standing back in her kitchen, reflected on the past year when she has learned so much.]

[On screen text: Chapter 4: Your Story]

Michelle: [Inner thoughts] “What a year - I’ve learned a lot.”

Michelle: “Takeaway #1: Find a PAH Specialist”

Michelle: “I wonder if I should look into finding a PAH specialist. I really like my doctor, but Pat said she was able to see a PAH expert and still keep seeing her existing doctor.”-

Michelle: “Takeaway #2: Ask about risk status”

Michelle: “I still have a lot of challenges with everyday activities; I think it might be time to ask more seriously about my risk status. I’m glad Pat mentioned risk status to me and it was really helpful talking things through with Donna, but now it’s my move. My doctor is helping me in this but ultimately, this is my life, my PAH, and my opportunity to get to Low Risk status.”

Michelle: “Takeaway #3: Maybe there’s more I can do”

Michelle: “It was so great running into Linda the other day! And our conversation really got me thinking. Since I’m still having symptoms, there may be more I can do to improve. I’ll talk to my doctor about my goals and what we can change. Adjusting my dose or adding a medication may help better control my symptoms.”

Michelle: “Takeaway #4: Ask about prostacyclin”

Michelle: “I think I am going to ask my doctor about prostacyclin-class medicine. Linda said it’s available in pill or inhaled forms, as well as a pump. She wasn’t waiting for her symptoms to get any worse!”

Michelle: “If prostacyclin-class medicines have been shown to help control symptoms so I can do my everyday activities and improve my risk status, I should ask my doctor about them.”

[Chapter Ends]

-

[On screen text: Your Story: What will your story be?

Learn more about PAH, Risk Status, and available treatments.

Find a PAH specialist.

Learn more about prostacyclin-class therapies and what you can do to fight PAH.]