A/N not mine, I own nothing.

This chapter will have a little more of the medical aspect as Marie, begins to go over PH with Stephanie a little more. Some of it has been implied already in the story, but there will be a bit more detail.

Slowly Suffocating

Marie broke the ice by asking me about Bobby, and I asked her if she wanted me to put a little bug in his ear about her. She nodded her head, and I grinned ear to ear. I wanted to see my Merry Men happy, and I think Marie is just right for Bobby. I looked up at her and realized that she now had a serious look on her face. I sighed, time to get started.

"Stephanie, I am not going to lie to you. This disease will change your life, and your everyday existence. I want to make sure you understand that. There are going to be many doctors visits in your future. Do you know if anyone else in your family has had PH?"

"I just found out that an uncle on my father's side passed away from it. I think he was really young."

"Ok, so you most likely either inherited it, or another disease that causes it. I am going to suggest you see a geneticist to help determine that. It can help with long term treatments. We can already rule out a few things that you have already been tested for, such as Lupus, sarcoidosis, and pulmonary fibrosis. Have you ever noticed that your joints are more flexible than most people?" I nodded. "Can you take your thumbs and bend them down to touch your wrist for me?" I did as she asked, each time she asked me to move a joint it moved freely. Then she showed me that she could not do any of those things.

"I think you may have Ehlers-Danlos syndrome, or possibly Marfan syndrome. I am leaning more towards EDS, because of your height, although you are tall, you are not well above average, also you are not pigeon chested. EDS can affect the joints and the vascular system. You may want to ask your Dad if your uncle was flexible, or what most people call double jointed. That could give us a clue. EDS can affect the vascular system, and when it does Pulmonary Hypertension can happen. If this is inherited, then if you have any siblings, nieces, nephews, or even cousins, it may be wise to let them know so they can be looked at for EDS. That way if they would have it they can be aware of what to look for if they begin to develop PH as well. It is not likely that they would have it, and if they did, developing PH is very Rare. The potential is there though. I want to help you transition into your new life as easy as I can possibly make it. By the time I am no longer visiting your home you will either be so sick of me, that you'll be glad to see my backside, or we will become friends."

"I would prefer friends, now how often will I need to see Dr. Z?"

"Until you are at a good stability, it may be once a week. I know you will be going home on oxygen, and with the Flolan you may be able to decrease the amount of time you use it, you may always need it, but not 24/7. Once Dr.Z thinks you are on the right track your visits may go down to every three months. I haven't seen a patient go longer than that, but if we keep you on the right track, it is a very achievable goal to work towards. you will also need to see a cardiologist, and a geneticist. I am not sure how often that will happen, but it will happen. You are also going to have to modify you diet, it will help with the heart failure, and to keep the swelling down."

"Crap." That was all I could say. I knew things were going to change, but I really wanted to stay in denial land. How was I supposed to do this? I know that Ella would follow my diet to the letter, and her food was the best, so maybe this wasn't so bad after all.

"When you are released, I will be coming to your home to teach you how to change your dressings, mix your Flolan, set up your cassettes, and run your pump. I will also be showing Bobby and Ranger, so you need to learn to lean on them for help. I will help you keep your independence, while learning to accept the help when you need it. We will take this one day at a time, and it will be ok. You just have to fight for it."

I was unsure of what to say, so we left it there, with her assuring me that she would see me at least one more time before I went home. I think it was time to make a list of questions to ask her and Dr. Z, I need to get a handle on this and fast.

A/N I have Ehlers-Danlos syndrome, and that is what they believe to be the cause of my PH, along with some valve problems with my heart.