Do You Have A Success Story After Starting Remodulin Or Similar Therapies?

[Dori Herrick]

@ripple76
I have been on IV Remodulin since my diagnosis in December 2015. I was diagnosed at Stage 4, and admitted to the ICU that day. I was so hypoxic (low on oxygen) that I had to take a deep breath between each word I spoke, even when sitting still. In 3 years I have removed 90 lbs of fluid by following a strict, low-sodium diet and I have worked with Physical Therapy to restrengthen the muscles used in breathing enough to function with no shortness-of-breath sitting and minor breathlessness with general activity. I did use supplemental oxygen 24/7 until recently. (An unrelated diagnosis and treatment for bone marrow cancer has caused my body to process oxygen better, which has allowed me to take a break from oxygen; however, that is not common with PAH, and I do not recommend getting cancer. *wink*)
My doctor has said that he hadn’t expected me to live through my initial hospitalization, nor through two hospitalizations for pneumonia. Statistically, I shouldn’t be alive; but I have a strong will and have learned that we can survive and create enough miracles that they can never give you a solid prognosis.
My advice is to educate yourself about the medication, always do your medication mix the same way, be strict about infection-prevention when changing your bandage, follow the low-sodium diet, and stay positive. Also, keep a support system, find people you can talk to and don’t be afraid to ask questions.
It is scary at first, but you will develop a system, and it will become a routine. Feel free to private message me if you have questions you don’t feel comfortable sharing. YOU CAN DO THIS!

[Brittany Foster]

Dori,
Thank you so much for all the great feedback on this post. You have been through so much and to keep the attitude that you have is truly amazing and shows how strong of a person you are! Therapies in combination with each other is the best approach in my opinion. For me that includes an exercise routine, eating well, taking medications, doing treatments, using medical equipment when needed, seeing my doctors as I should and also taking care of my mental health and seeing a therapist once a week (it’s hard to always keep the upbeat and positive attitude).

Keep educating others about this condition ! Your words really could help a lot of people push through difficulties. Have a great holiday season 🙂

[Dori Herrick]

@brittany-foster Thank you for your encouragement. I believe that if I can help others get through this, my disease is worth it. You are right on combining multiple therapies lead to a better outcome. The doctors give us their medicine and we give outselves the other things which together best keep it under control. Not one thing alone will make you feel better, but the combination of medicine, diet, exercise, rest, etc. will make your condition improve.
Stay positive and strong

[Brittany Foster]

Thank you Dori,
What combination of therapy like meds, treatment, holistic therapies, therapy etc have you found helps YOU the most?

[Dori Herrick]

@brittany-foster
The combination is so complicated, I would best say what helps me most is being mindful of my body and trying to increase my life-expectancy by preventing excess stress to the heart because most of us will pass away when our heart can no longer push enough oxygen to sustain our body.
My best advice to all:
*Take your medications and follow treatments as directed.
*Exercise how you can and if you can; in my case, a workout is stretching and small chores or a walk through a store pushing a cart.
*Follow the a low-sodium diet and water restrictions: Fluid around the heart and lungs really makes them have to work much harder.
*Use your oxygen as prescribed, even if it feels uncomfortable. Additional oxygen is prescribed when your heart needs help getting oxygen to the body. You may feel like you look awkward carrying a tank, but it is much more embarrassing and scary to imagine fainting in public which can happen suddenly without enough oxygen.
*Get treatment for depression and anxiety, and connect with other patients. You are facing a condition that is very stressful and daunting. You will need new tools to cope with the emotions.
*Listen to your body: when you feel out of breath, stop activity until you feel more oxygenated; if you need to stay in bed, do so; and If you feel new, unusual, or emergent symptoms, contact your doctor or emergency services. It is better to be safe and it is their job to help you.
*Learn as much as you can about your conditions and medications. This also helps reduce anxiety by giving you some control.
*Ask questions about everything you don’t understand. With my complex number of illnesses, I have caught near-fatal medical errors by being willing to ask questions about things on tests and medication details. Doctors and nurses do make errors, as they are human.
*Seek out new ways to help your symptoms. Ask about eastern medicine and holistic treatments, try yoga and/or meditation, or escape for awhile through reading or a hobby.

Your health and longevity is a TEAM effort. The medical professionals do their part, but you must also do yours.
And, yes, checklists to help with the foggy memory are priceless.

[Brittany Foster]

Dori
Your advice is spot on and I find myself needing those reminders too! Especially making sure to listen to my body. It’s especially difficult when I find myself comparing myself to others but the reality is that I can’t compare myself because it will just rob me of my happiness. Setting small goals for the day, week, and month has been helpful for me and gives me something to work towards. Without that motivation, I lose a huge piece of who I am. Thank you for that much needed inspiration and words of wisdom. I knoe I’m not the only one who needs to be reminded of all that.

[Chris LaRose Mev]

i was on IV Remodulin from 9/2011 to 10/2017, then transitioned to Orenitram (oral version) while in hospital 4 days. i was extremely sick at diagnosis (very high pulm pressures). Currently, on stable dose with fairly normal pressures, having an echo next yr to see how doing. Still plenty of side effects with meds and unable to do much even on good days.

chris

[Brittany Foster]

Chris,
That’s the tricky thing about the medications. We need them to live our life the best way and longest possible, but they don’t come without side effects. I hope you have found ways to manage the side effects or at least make them less exhausting !

[Brittany Foster]

I’m sorry the side effects of the medication is so terrible for your quality of life. Have your doctors made any other suggestions for you or suggestions on modifications to help you participate more in what you enjoy? That must be so isolatong and overwhelming for you. I know you are doing all you can to try to fight through this and I know some days it can feel hard. What medication do you think contributes most to your side effects?

[Randolph Reynolds]

Thanks Dori and Chris. Perseverance and trust in God. The variations of PAH and the different methods used to manage it indicate to me that the more a Physician understands about this disease the more reasonable the treatment.

[Brittany Foster]

Randolph,
it’s so important that doctors try to understand the cause of the PAH as well. For me, mine can’t be treated with medications because it is stemming from another condition ( a breathing disorder) that can only be helped with oxygen and ventilation treatments. I am also on medications for my heart. It’s amazing to me how different each person’s story is and how individualized our treatments have to be ! The doctors certainly have their work cut out for them with this disease!

[Randolph Reynolds]

These comments are valuable. When I first started with the pump I was emotionally depressed. The nurses that taught me how to manage the pump and all that is involved were adamant that everything be in a sterile environment. I have had some problems with the pump but got help from the pharmacists and nurses at Acceredo. It is time for me to ramp up to changing out the pump every two days and eventually go to a stronger dosage. I can see that it is helping my breathing but I have a long way to go. One problem is that as the titration moves up the supplier is working off a monthly schedule and if my dosage was constant that would work. But I have to order Remodulin early.

My hope is to make this treatment less invasive in my life and Dori is correct. I hope this day is a good day for all who are dealing with they various issues. Blessings this Christmas season

[Dori Herrick]

@ripple76 The titration up is the hardest part. You have to try to remember your new routine and dose, while dealing with some awful side-effects. It is ok to take your time with this, too. I pushed too hard and had way too many side-effects at first. It got better when I let my body get back to “normal” between titrations. I spent about 36 hours in bed after each increase due to side-effects, so yes, it is a disease so serious, we must endure a lot to get it controlled. Unlike people 15 years ago, we can live because of this.
Once you reach the point where you have a steady dose, it gets easier. Accredo should be there for every question, don’t be afraid to call them with any doubts.
Whatever makes your mixing easier, do it. My husband made a check-off list in the beginning, and we mixed together while I was titrating. One mistake can mean life or death. And, if you are unsure of anything you have done while mixing, it is better to toss a batch than to risk accidentally putting the wrong dose on your line.
Again, pm me if you have questions. I try to check at least once daily.
You have got this!

[Brittany Foster]

Blessings to you too , Randolph. I hope you were able to enjoy your holiday and Christmas. I know that many of us on here have dealt with or are still managing depression. Are you taling depression or anti anxiety meds if you don’t mind me asking? I do for sleep. You are right when you make the suggestion to call with any doubts or concerns . Its so important for our health and our mental health to make sure that we have some type of control back.

[Brittany Foster]

Dori,
Thank you for being so active in the forums and for providing myself and others with such useful information. I’m usually on here as much as I can too. I took Christmad Eve and day off (which is a holiday break for many of us) but I’m glad to connect with you all even on my off days. A check list has been super helpful for me. That and even storing medicstions in different colored bags for those that can’t fit it all on a organizer. I know this has been helpful for me since I am a visual person. I forget things way too easily without doing this visual reminders . I’m glad somewhere is there to support and help you. It can be overwhelming trying to manage it on our own

[Randolph Reynolds]

Dori,
I am probably better off on working with Remodulin as I only have to fill a cartridge and the pump pulls it in. I don’t know about the mixing business. I hope that when I move to the next level of concentration I will have fewer problems breathing while being active. Maybe I can get to the point where I don’t need additional oxygen from my concentrator.

Hope your New Year starts well.

[Brittany Foster]

Randolph.
I always hope for that too , that a medication or something will help me get off the oxygen. It’s hard carrying it around all the time (or wheeling it)

[Carol Volckmann]

I am sorry it took so long to respond to the person looking for someone who has had good results with Remodulin. I have. I have been on subcutaneous remodulin for nearly 7 years and it has definitely helped my PAH. My six minute walks continue to be better and my exercise program of treadmill and some weigh exercises have helped. I am also on Adcirca and Letairis – my medical team likes the three handed approach. Is Remodulin without pain and nasty side effects – no – it has all of that, but it is keeping me alive! To go back on how I came on Remodulin – I was diagnosed 10 years ago, two different Pulmonologists told me I had a very expensive and serious illness and I could count on about 2-4 years of life. I was not going to believe in them. I went on line in search and found my team at UCLA, 1000 miles away. My doctor told me that if I were his mother he would want to be very aggressive with this – I asked him “…how much do you like your Mom?” He likes her a lot. I am still here and very stable. Being on Remodulin is no fun but I have learned over the years a few tricks to help. It is especially painful with site changes so I buy over the counter Lidocaine 4% and put 5% Lidocaine which is provided by Accredo Group Health, on the 4% patch and leave that on the new site area for about 1.5 hrs. That really does numb of the area for me, I also take vicodin as needed and make sure for the next week I take it easy. Another thing that helps is using Aquacel Foam to waterproof up – it is totally waterproof, easy to put on and leave on and step in the shower. I leave it on 24hrs and don’t need to change it but for every 3-4 days. I also get this through Accredo Health. Now tearing of the skin or pulling and it protects the area continuously while letting it breath (while being totally waterproof). Wishing you all a very HAPPY, HEALTHY, NEW YEAR!

[Brittany Foster]

Hi Carol,
Thank you for sharing your experience
I’m glad they the combo of the medications are working well for you. I know it’s hard when you experience side effects, but at the same time, the benefits outweigh the risks. Hope you have a great new year too!

[Randolph Reynolds]

Thanks Carol,
I was the fellow who was inquiring about experiences with Remodulin. I had not thought about using a Lidocaine patch. As for site changes I havn’t had to make a change for about three weeks. This insertion is working out ok. Again thanks for your inputs. May we adjust to being a little different from other people.

[Brittany Foster]

Randol
I’m happy that you are getting such great feedback on here! We really can learn so much from each other ! Happy to have this type of communication in my life. It helps a ton!

[Peter G Pierce]

I have WHO type 1 idiopathic PAH. Diagnosed October 2016. I’ve been on Orenitram since August 2017 (titrated up to present dosage over a 4 month period). Experienced no side effects during titration or since. I take it in combination with adcirca and opsumit. Changed from letairis due to terrible nasal congestion it caused. I’m stabilized and have experienced no deterioration. In fact my echo pressure has been cut in half. Six minute walks have all been over 400 meters. since Orenitram I’m unusual in that I’m asymptomatic and operate quite well with O2 levels in the mid- to low 80s. Doctors suspect I’ve had this for some years and just adapted. Use of O2 is a nuisance but have gotten used to it. Use an Inogen One to get around for shopping, dining, ball games, etc. I attribute present wellness to the Orenitram.

[Brittany Foster]

Peter,
It’s so great to have success stories like yours ! I’m glad they were able to get the right combination of meds and that you are doing well managing with the oxygen. How do you like the oxygen that you use? I use a tank so it’s not as easy to get around. My oxygen is also in the 80s and I need it with more exertion. I’m glad they have these new technologies available !

[Peter G Pierce]

I use a Millennium M10 O2 concentrator by Resperonics at home. I also have one for travel that is attached to a cradle with wheels I had made. The M10 in cradle weighs about 60 lbs. I can no longer fly because cabin pressure is at 8,000-10,000 feet and my top altitude is Denver. So we take road trips and plan to try a cruise in September. I use the M10 at destination because it goes to 10 liters continuous. For one night stays en route I have an inogen stationary unit that goes to 5 liters continuous. The 5 liter unit is only about 10 lbs. At night I attach a concentrator to my bipap. In the car and walking around I use the portable Inogen One that goes to 5 liters puls. We travel with a lot of equipment and electronics but it’s better than having to stay put. We did a 10 day trip to New Orleans in November and plan a trip to Texas Hill Country in February. Everything worked great on NOLA trip, so I’m optimistic that we have the formula down. Fortunately, I have never had to use tanks.

[Brittany Foster]

Peter,
I always find it amazing how we can adapt to all of this and make it work for us. It seems like you definitely have a good system down and I’m sure it took some trial and error to get there ! A lot of my learning about how to navigate oxygen and all of my medical equipment was through trial and error. I always learned something new and could improve trips that I took in the future. I must say that one of the best vacations I went on and very disability friendly (I found anyways) was a cruise ! I hope you are able to take that trip 🙂 Let me know if you need any pointers when the time gets closer.