PAH Infusion Therapy: Hints, Tips, and Tricks

Part of therapy for Pulmonary Hypertension consists of medications that dilate the blood vessels. While this process doesn’t cure PAH, it does help with alleviating some of the damage it causes. Think of a water hose that is about 1.5 inches in diameter. Now, apply 100 pounds of water pressure to that hose. Eventually, the narrow hose will cause the pressure to damage the plumbing that is connected to it. However, increase the diameter of that hose to 4 inches. This allows more water to run through it thereby alleviating the strain on other piping.

Keep in mind that there was no therapy for PAH until 1995. The first medicine that was released was called Flolan. It was a miracle, but not without its issues. First, it can only be administered via Hickman line, or central venous catheter, to the artery of the heart. A Hickman line is typically used for many cancer patients to avoid numerous I.V.s and punctures the skin. It’s administered 24 hours a day by a medium-sized CADD Legacy Infusion Pump that is worn by the patient. The drug, it was very unstable and has to be kept at a cold temperature with ice packs around the pump. The medication has to be mixed and added to the pump each day. The most important thing is that the medication only has a 5 minute half-life, or the time the medication remains active and in the patient’s system. So, if the pump stops, the patient has five minutes to get the pump restarted or severe, possibly fatal consequences may occur.

So…no…not a whole lot of fun.

Years, later, a drug called Remodulin would be developed that provided all the benefit of Flolan, but not as much hassle. It, too, is administered by infusion pump, but a smaller, pocket-sized CADD MS3 pump. It doesn’t have to be kept on ice, it can be administered by infusion site instead of direct Hickman line, and it has a 4 hour half-life, which give a great deal more time to the patient should an event occur. It was approved by the FDA in the early 2000s. This is the medication that I am on. The only bad thing about Remodulin is that it causes reaction at the infusion side, sometimes severe. Yep…that’s a pain. I’ve been doing this Remodulin thing for about a 2.5 years now, and during that time I’ve found some nifty tricks that have certainly made my life easier. I hope that in sharing them with you maybe I can help make your life easier as well.

When I first began treatment I was lost. I had no idea how I was going to manage my day-to-day. Getting up at 6 AM and getting ready for work was going to be challenge. I wasn’t going to have a great deal of prep time without getting up at 5 AM, and I’ll tell ya…I’m NOT a morning person…at all.

The goal was simple: find a regime that would keep my site and pump dry and sanitary while cutting time spent with bandages and tape.

When the nurse first came and trained me on the medication, she gave me a few pieces of advice and things to use, and I tried each one of them.

• Silhouette Infusion Kit: This infusion pump kit enters the skin at an angle and is meant to offer more comfort.
  • Outcome? I didn’t find it any more comfortable than the standard Sof-set kit. Additionally, the cotton backing is comfortable, but presented the potential to retain moisture from showers and skin. Not ideal when you try to hold a site as long as you can.
• Pump Shower Bag: A bag with a hanger that you can put your pump in and seal.
  • Outcome? The bag was cumbersome to use. I didn’t like the fact they weren’t that reusable-friendly.
• Aquaguard: A “water-proof” bandage lined with adhesive that is meant to keep your site dry.
  • Outcome? This thing was useless. The adhesive wouldn’t hold in any manner. I only used it twice.
• IV3000 Dressing: A moisture responsive, transparent film dressing, specifically designed to meet the needs of catheter fixation.
  • Outcome? I did like this. However, it wasn’t really waterproof. It would guard against a small amount of water, but not the amount of water I would encounter during a typical shower.
• Glad® Press & Seal Wrap: Well…you know what this is. The nurse suggested that I try this to see if it would work as some patients had a good experience with it.
  • Outcome? It worked better for luncheon meat.
• Sleeping: I knew it was going to challenging sleeping with something strapped to you 24 hours a day. The advice given was to place the pump in a pajama pocket, or under the pillow.
  • Outcome? Through the night, the pump easily slips out of a pocket or becomes wrapped around your arm while under a pillow. Not too comfortable.

These things didn’t work for me. By the time I would get enough tape wrapped around my site to manage, I had spent twenty minutes prepping for a shower. I thought there had to be a better way. So, I set out on a mission to find things, modify bandages, and tweak processes in order to find something that would work the best. And I am glad to say I think I found it.

Now, the following things work for me. I receive my medication subcutaneously, so I can afford a certain bit of whoops. Worst case, if I got a slight infection, I would just move my site. If you have an IV site, or Hickman line, please follow your doctor’s orders. I wouldn’t recommend getting an infection in your line. I’ve heard those are not fun at all.

So, without further ado, here are my pointers:

• Cleo 90 Infusion Kit: I love, love, love this thing! It is so freaky-easy to use. No message bandages, no tape, no injection gun. Nope, this thing comes all inclusive. The needle is contained in the main unit, so that means no sharps to throw away. Also, the site bonds pretty easy to the skin, so no need to bandage and bind it to the skin. It is 100% better than the old, bulky Silhouette and the Sof-set kits.

• Nexcare® Knee and Elbow Waterproof Bandages:This has been my greatest discovery! Nexcare has a great waterproof bandage that around 4×3 inches. Using a standard IV prep wipe, I can have the bandage last more than four days, sometimes six. It holds great in the shower and I don’t have to worry about it. I leave the bandage on to keep it dry and germ free.

• IV3000 4” Dressing: This actually is a GREAT skin tape. I found actual tape a little irritating to the skin after being worn for a few days. However, the IV3000 texture flexes with the skin very easily. I trim the edges from each end and use them to add extra protection for my site. I use one end, trimmed in half, to line the bottom of my Nexcare bandage right where the tubing exits. The other end, I cut in half and use to tape the tubing to my side.

• Adhesive Removal Wipes: As you may imagine, after having adhesive on your skin for a while, you may get a little dirty and sticky. So, using these adhesive removal wipes help clean and sanitize the skin.

• IV Prep Wipe: These are GREAT! If you have super baby-soft skin that is freaky young for your age (like me), then these will help secure bandaging to your skin and provide a longer life to your tape points.

• Zip-lock Bags with Zipper: I love it. Get the ones with the plastic zipper. That way you just toss the pump in the bag and zip it closed. Easy-peasy.

• Shower Cup/Shelf with Suction Cups: You have to get one of these. Run to your locate department store and pick one up. It suctions to the shower wall. Simply get it, toss the pump in the holder, and relax. Even though you have the zip lock bag, when placing your shelf, put it somewhat higher that your shower water to keep it from getting too wet.

• Exercise Arm Strap: Not only does it keep your pump secure, it prevents it from rolling all over the bed. Just like the straps you get for iPod or mp3 players. It makes sleeping much better.

All and all, these things may not be the magic bullet for you, but I know they have helped me maintain an easy regime. The goal is to make things easy, make them feel normal. Adding this sense of normalcy to your day helps reduce stress and elevate your mood tremendously.

Have you tried other things that work for you? What advice can you offer?

Prayers to you and yours…

My Story: PAH – Fighting My Boogeyman

My name is James Grea. I write stories for children with a magical fountain pen named Solomon J. Inkwell. I spend a great deal of my time writing about Boogey Men and things that go bump in the night. I love spooky tales. They are my favorite. My monsters are harmless and even quite fun because they are imaginary. But there are times when monsters can be real, and sometimes they can even live inside of you. Unfortunately, I discovered this around two of years ago. 

On Thursday, March 29^th, 2012, I arrived to outpatient registration at the hospital at 5:30 a.m. with my sister and my partner. I was surprisingly calm. I swear to you, I don’t know why. Maybe I had convinced myself that I was going to receive good news. The mind has an odd way of masking the unimaginable, especially when it is standing right in front of you. We sat in the lobby waiting to be called. My sister patted my leg and smiled at me. My uneasiness wasn’t apparent to those who didn’t know me, but she knew I was shivering on the inside.

Soon they called my name and my chest tightened at the sound. I took a deep breath and followed the nurse who showed me to my curtain-walled preparation area. Two older gentlemen were on either side of me hidden behind their own curtain walls. They were apparently having the same procedure as I that morning. I tried my best not to listen to the questions the nurses were asking them. “When did you have your first heart attack?” “Do you have a living will?” I stared at the ceiling tiles trying to think of other things, things I would do after I received the good news, movies I would see, food I would eat.

Right heart catheterization — it is a fairly simple and relatively painless procedure where a measuring device is inserted into the right femoral artery of the leg. It travels to the heart where pressures are measured and dyes are injected. It’s a common test that can locate blockages in the arteries. It’s also the only exact method of diagnosing pulmonary arterial hypertension (PAH). There are instances where this disease is inherited. I come from a fairly large family consisting of eight children in total, of which I am the youngest. By the time I was 21 years old, I had lost my oldest brother (diagnosed age 24, passed age 27), my nephew, who was 10 months younger than myself (diagnosed age 11, passed age 12), and my mother (diagnosed age 60, passed age 61) — all of them victims of PAH. You could say that the disease has stalked my family and me all of our lives. We all spend a great deal of time praying it doesn’t catch us. My new adventure had begun with a routine EKG that had shown an abnormality, which led to further testing. They eventually found mild enlargement on the right side of my heart. I heard the word “right” and immediately knew what was happening to me.

The orderly wheeled me into the catheterization laboratory and began to prep for the procedure. I welcomed the sedative and tried to focus on its feeling. Through my wooziness, I could see the large monitor above me. On the screen was my beating heart, and I watched as the thin cath line traveled into its chambers. I took a deep breath and tried to relax myself. Moments later it was over. The rather abrupt cardiologist I had been working with had no experience with PAH. So, he thought nothing when he rounded the table and said nonchalantly, “Well, it looks like pulmonary arterial hypertension.” Stunned, I asked, “How high was the pressure?” “It’s 110 over 44,” he said. I sank into tears. And when I did, the doctor disappeared. He was so stunned by my reaction; he apparently had to get away. I, too, wanted to get away, but now there was nowhere to go.  

Yes, the Boogey Man under my bed had found me at last. I continued to cry for three solid days. To me, my life was over. I wondered how much time I had left. How long would it be before I couldn’t walk to the bathroom without passing out? I was no longer James Grea. I no longer wrote scary stories. I was now a zombie who wandered around my house sobbing. Finally, I decided to reach out to PHA. I began reading everything I could on PHA’s website, trying to find any sign of hope. I obtained the Envelope of Hope, which contained a wealth of information. I went one step further and called PHA’s support line where I spoke with some great people who were living with PAH. I learned that in today’s world I have great chances, far better chances than others in my family had. In the past, there was nothing at all that could treat PAH. But today I learned it is very different. Then, I began to do things I hadn’t done in a while. I began to talk more with God. I began to see the value in little things in my life that I had taken for granted. I began to realize that not one of us is guaranteed to see tomorrow. At any moment we could receive that test result that could drastically alter our lives forever, or even get hit by a moose.

Today, it is July of 2014. I have been living with PAH for over two years now. I’ve really been living with PAH all of my life. By March of 2015, in my family I will be the longest living PAH survivor post-diagnosis. I am fortunate enough to still be able to get up every morning and go to work. I pay my bills and enjoy time with my partner, friends, and family. I try to live life as normally as I possibly can, with one little exception: I now have a CADD MS3 Infusion Pump connected to me at all times that administers Remodulin therapy. I have found very creative ways to deal with the medication—changing my site, taking a shower, wearing the pump, and so on. Sometimes, it’s like it’s not there at all. Recently, I have also started Adcirca, so I am extremely fortunate to have my specialist and the PHA. I am also fortunate to have the Caring Voice Coalition, for without their assistance I could never, in a million years, afford my medication. I often wonder what the future holds for me. Will my symptoms continue to worsen over time regardless of the medication? Will I eventually have to stop working? If so, how will I survive on Disability? Will they find a cure soon? Yes, those questions sometimes haunt me.  I shake it off, and carry on. I am certain there is a part of my mind that denies what is happening to me. It is a coping mechanism, you see. Regardless, I have a heart cath each year, and the pressures aren’t exactly perfect, but they are getting better. Anything lower is a step in the right direction, right?

I’ve never said, “Why me?” Why not me? What makes me better than anyone else? Absolutely nothing. So, I choose to have hope. For the only other alternative is to lose hope, and that is unacceptable. So, why am I telling you this? I’m really not sure. Maybe I want you to come along with me. Maybe I want us to be like old friends, sitting in a room, drinking coffee, and talking about the frailty of life. Maybe I just want you to listen. I’m not certain. That’s why I think I’ve decided to start this new blog. I only know that I am moved to tell you these things because a writer must write. It could be possible that you, too, are afraid — for yourself or someone you love— and maybe it will make you feel just a tad less lonely to know that somewhere out there I am here, being afraid along with you, living life as best as I can with this disease that no one understands. Who knows what tomorrow holds. Miracles happen every day, and my miracle could be right around the corner…OUR miracle. Ironically, being diagnosed with this disease could very well prove to be my miracle.

So, I will look toward today and value what I have in my present world, my present health, and my present life. For today, I am alive — we are alive. And what could be better than that? And yes, I am finding my identity again. My name is James Grea. I write stories for children with a magical fountain pen named Solomon J. Inkwell. And I just happen to have pulmonary arterial hypertension.