RARE DISEASE DAY

ARE YOU RARE? 

I'm rare! I have a rare disease living inside of me called Pulmonary Hypertension.

What is Rare Disease day?  It is a day where we, those with rare diseases, come together and fight to bring awareness to our diseases. Whether by word of mouth to people like you in our lives, or through letters to legislation to help bring the disease to light. 

Did you know that 1 out of 20 people live with a rare disease at some point in their life? A majority of Rare diseases, like Pulmonary Hypertension, have no cures.  

*What is a Rare Disease?

A disease or disorder is defined as rare in the USA when it affects fewer than 200,000 Americans at any given time.

*Characteristics of rare diseases

Over 6000 rare diseases are characterised by a broad diversity of disorders and symptoms that vary not only from disease to disease but also from patient to patient suffering from the same disease.

Relatively common symptoms can hide underlying rare diseases leading to misdiagnosis and delaying treatment. Quintessentially disabling, the patients quality of life is affected by the lack or loss of autonomy due to the chronic, progressive, degenerative, and frequently life-threatening aspects of the disease.

The fact that there are often no existing effective cures adds to the high level of pain and suffering endured by patients and their families

*Common problems faced

The lack of scientific knowledge and quality information on the disease often results in a delay in diagnosis. Also the need for appropriate quality health care and difficulties in access to treatment and care. This often results in heavy social and financial burdens on patients.

As mentioned, due to the broad diversity of disorders and relatively common symptoms which can hide underlying rare diseases, initial misdiagnosis is common. In addition, symptoms differ not only from disease to disease, but also from patient to patient suffering from the same disease.

*How to get involved with Rare Disease day

It’s simple! Post an image or selfie on any social media channel with your face painted and include the hashtags #ShowYourRare, #MyRare or #RareDiseaseDay. Share an image on your own or with your friends, family or colleagues!

For more information

go here to learn more:  https://www.rarediseaseday.org/

Thank you to Rarediseaseday.org for bringing awareness and allowing the use of your logo.  Information with * next to it was taken from rarediseaseday.org and was not my writing. All credit is to go to them for that information.  

Join me for my next blog: Encouragement living with a disease 😊💜

Living with Pulmonary Hypertension

The most difficult part about getting diagnosed with ANY type of disease I would say is, hearing you actually have it. Especially if you never even heard of it before today's date of being diagnosed.

I mean, how do you deal with learning about something that barely anyone knows about let alone this doctor who just said you have it but he can't treat you because;  "he doesn't know enough about it."  WHAT!? But you just told me I have it! How can you sit there and tell me I have something but you have no idea how to treat me for it.   Here comes the downward spiral.

So you were just diagnosed with Pulmonary Hypertension, what do I do now?  I know what I did, I went into denial. Nope, not me, I can't have it. I'm healthy, just short of breath when walking stairs. I don't have this, you are crazy.  I know what this disease is, I've seen it first hand, live it first hand as a caregiver. This IS NOT ME!  So I go to the Specialist, get the Right Heart Cath. done and I still am in denial. It doesn't matter what he says, what the test says, why oh why are you insisting that I have this.  Reality sets in when I am told; "No traveling, so exercise, no lifting/pushing over 5-10 pounds, no more WORK…WHAT? I am only 40 you can not be serious. He said, “I am very serious. Your pressures are very high and you are in what we consider to be severe PH status. Without getting it under control you are at risk of dying.”   WHAT?  Seriously you are killing me here, don't tell me I can't work, and I can't travel, what the hell kind of life is that? Grrr, who is he to tell me to put my life on hold.

Yes,  you guessed it, here comes the anger. Not just any anger. I am talking full blown, circuit frying anger.  

So now I have decisions to make as to what medications to take. I AM NOT and WILL NOT go on any IV or SUB Q medications. If it is not oral to begin with, I am not doing it, bottom line. He loved how adamant I was in my decision making and asked my viewpoints as to why. I travel, I hike, I swim, and I do not want to hinder my abilities as our summers are spent swimming when the weather is nice. He said understandable. I said, I do not want the pain and side effects my mother goes through, I’m a chicken shit to poke myself….But you have tattoos…those are different than pinching yourself and having the pain to go with it, as well as side effects. A tattoo you get inked it stops hurting you move on. Anyway…He wanted to put me on the study drug program for Adcirca and Letairis, I denied immediately. Not that I didn’t want to be a part of a study, but because I already knew the medicines are BOTH approved from the FDA and this was to see if they were compatible with one another together. Still not guaranteeing me that I would get both at the time or one and a placebo, I denied and said…start me on something that is already approved and we know I am getting the drug and not a placebo, I want under control NOW not months from now. So, June 24 we did the 6 MWT and I failed miserably, only got to 12 laps, 256 meters or 841 feet, because they stopped me for my heart rate going to 243, but hey my sats (saturation) stayed at 95%. July 17, 2012 I did another RHC to see if the CCB and HCTZ was working, pressures came down some but not the PA Pressures. So August 17, 2012 I began my first pathway of Adcirca. I had major headaches from it, so I started to take the pill at night. Of course you start out with 1 ~ 20 mg tablet for the first week, then week two you start 2 ~ 20 mg tablets for treatment. 

Anyway, this isn't about me, its about Living with PH and how to deal with it. So let's move on. 

Here starts the “NEW” Normal

Learning how to live a "New" Normal and leaving your old life behind. It isn't easy but it is doable. 

The main thing is to have a positive attitude, no matter what the circumstance is that you are facing.  The biggest thing to overcome was eating out as often as I use to do. I don't mean a regular restaurant either, I am talking Fast food.  Never really looked at the labeling because food was food, oh how wrong of a mindset was that. You learn fast, sometimes, not fast enough, but you learn. I'm almost 6 years into this darn disease and I'm still learning. 

We have to learn to lower our salt intake, our fluid intake, what veggies are high in water, or some have to worry about how rich in vitamin K the produce is.  Watch, watch, watch. Hello, this isn't as easy as you think it is.    The water content in some fruits and veggies are higher that the item itself. For instance, who doesn't love them some good old asparagus? Did you know that 1 spear, just 1 is almost 2 Tablespoons of water! Also, it is a natural Diuretic, so if you are on diuretics watch how much you eat so you don't get leg cramps!!! But its great for the 💜.   1 Cup of cucumbers sliced is a 1/2 cup of water! 1 medium tomato is a 1/2 cup of water. 1 cup of watermelon is 1/2 cup of water. A FULL watermelon that is about 15" long is 17.5 cups of water! GO FIGURE, the things that are suppose to be good for a persons health actually hinders mine because I have to watch what I eat and how much of it so I am not causing myself to swell with excess water weight. UGH. Oh yes, because too much salt and too much fluid can make you retain fluid, and can cause Heart failure. Did you know that?

Mood swings ~ Do not get me started. We have our good days and we have our bad days and then we have our really bad days to where we want nothing to do with anyone and lock ourselves away in our own little world. Why? who knows! It could be the medications, it could be depression, it could be just about anything but it is there.  Forgive us when we lash out, for it isn't easy being sick and trying to be our normal selves when we don't even know what normal is anymore.   

Lets walk through a BAD PH DAY

Wake up and you are swelled from too much water retention. Heart is racing, shallow breathing because its really hard to catch your breath, and you just woke up. Chest feels like you have an elephant sitting in the middle of it and you are being squeezed by a vice in the middle of your chest. So here goes a double dose of diuretics to get the water off today. The bathroom is going to be our best friend. Many of us have an IV medication, which not only has you going to the bathroom more times than necessary, and not just for the normal bathroom run, but it also comes with sleepiness and a rash. So no matter how much sleep you get, they are still tired.  Doesn't sound pleasant does it? Try living with it. Everyone thinks that oh, you just have high blood pressure, that is so controllable you only have to watch what you eat, nothing more serious than that, why are you making such a big deal about it. NO, it isn't the same. The high blood pressure we have affects our lungs and heart and is totally different than regular hypertension of blood vessels. Regular Hypertension can not be felt, Pulmonary Hypertension is felt in more ways than one. 

The worst part of this disease, I feel, is not knowing when I am going to be sick. I mean, one minute I am fine, getting ready to go out and enjoy time with family and all of a sudden I start getting hot flashes, I'm dizzy, nauseated and my breathing is horrible.  I don't want to cancel, but I don't want to spend my night in the hospital either in the event I pass out on you all and you have no clue what to do to help me.   I remember I was at my nieces graduation party. I showed up before it started and helped to decorate. Once everyone started showing up and we ate the fun was suppose to begin. Not for me, I started breaking out in red splotches. Now mind you I wasn't in the sun, I tried to stay in the pavilion as much as I could.  I broke out in this rash and everyone was like Jan, are you ok you are all red and splotchy? I said yes its just my medication, I am fine.  No, I wasn't fine. I then began getting hot flashes and heart palpitations and I was nauseated.  Needless to say, I had to cut my fun short because my PH decided to flare its ugly head at the most inopportune time, when I was having fun.  So please, if we cancel last minute, forgive us as we have no control as to when symptoms will arise, we don't do it on purpose. 

Lets walk through daily living with chores

Take your time! Time is all you have and it doesn't have to be breathless.  When doing dishes, keep a stool that is sink level or stove level in there with you. If you don't have one, buy one!  Sit while you do dishes or preparing your foods for cooking. 

Bathing and showering ~ Why reach when you can sit and relax while cleaning yourself. Get a shower chair and a removable shower head. 

Laundry ~ use smaller baskets that are lighter like the fishnet baskets. Put enough clothing in there that won't cause you to be so short of breath carrying it. I have three sizes I use and the larger one's I have my husband to thank, as he carries those and does that round of clothing, where I will do the small baskets. 

Reaching, stretching, bending. Get a reacher rod or a step stool. Not only does it help you prevent yourself from bending over too much but it helps keep arm strength 😊

How to keep your activity without being discouraged

One step at a time. Every person is different one from another. The best advice you will hear from anyone dealing with this disease, walk. The more you walk, the better you feel, no matter how far you walk. Today you could walk from the couch to the kitchen and back, you walked.  Tomorrow you walk from the couch to the kitchen to the sun-porch, back to the couch, you walked. The next day, go outside, go off your porch, back up your porch back to your couch. You see what you are doing? You are building stamina and strength. The more you walk the further you go. The further you go, the better you feel. Just because we are sick with this disease doesn't mean we have to stop doing. Yes, it is hard, but again, we can do it. We cannot be afraid to continue living.

How can we be happy

Life is what we make of it! You can choose to let this disease eat you up inside OR you can choose to control the disease and not let it define you, as a person.  We only live once. NO-ONE knows the day or time in which our life will end, so why not live it to the best of our ability, the best that we possibly can? To our fullest and happiest. No, I'm not saying party hardy. I'm saying smell the roses, not the coffee. Unless you are a coffee drinker 😀Put your hand over your heart, do you feel that? Its still beating! We are blessed to be alive another day, sure we are sick, but we are still here to phight another day. 

If you have a topic you would like addressed, please comment and let me know. I will gladly take the time to write a blog about it. 

Thanks for reading today's episode. Stay tuned for tomorrow when we talk RARE DISEASE DAY 😊💜

Side Effects from Medications

On Monday, February 19 I did a blog on the different medications for Pulmonary Hypertension.

As will all medications, many of them have adverse side effects and every patient is affected differently when dealing with these medications. 

One of my group members asked if I would touch base on what each medications side effects are, so here we go Stacie, thanks for the recommending this Topic :-)

Flolan side effects: 

nausea,
vomiting,
diarrhea,
dizziness,
headache,
flushing (redness, warmth, or tingly feeling),
sweating,
stomach pain,
jaw pain,
muscle or joint pain,
injection site reactions (pain, redness, swelling),
anxiety,
nervousness, or
agitation.

Contact your doctor if you have severe side effects of Flolan including:
fever
trouble breathing
fainting
chest pain
irregular heart rate
coughing up blood

Tracleer side effects: 

Less serious side effects may include:
headache
flushing
swelling of the feet, ankles, or legs
dizziness
upset stomach
fatigue
itching

Call your doctor at once if you have any of these serious side effects:
nausea, stomach pain, loss of appetite;
dark urine, clay-colored stools; or
jaundice (yellowing of the skin or eyes)

Remodulin side effects: (Both IV and Sub Q)

Less serious side effects may include

pain, swelling, redness, bleeding, or a hard lump where your catheter is placed
dizziness
mild skin rash
headache or jaw pain
flushing (warmth, redness or tingling)
diarrhea or nausea

Call your doctor at once if you have any of these serious side effects:

new or worsening PAH symptoms such as feeling short of breath (even with mild exertion), tiredness, chest pain, and pale skin
swelling in your hands or feet
feeling like you might pass out

Ventavis side effects: 

Less serious side effects may include:

flushing (warmth, redness, or tingly feeling)
increased cough
nausea, vomiting, diarrhea
headache, dizziness
muscle cramps, back pain
sleep problems (insomnia)
tongue pain, jaw tightness or pain, trouble chewing or speaking
altered sense of taste

Call your doctor at once if you have a serious side effect such as:

feeling like you might pass out
pounding heartbeats or fluttering in your chest
coughing up blood
unusual bleeding (nosebleeds, bleeding gums)
fever, chills, cough with yellow or green mucus
chest tightness, stabbing chest pain, wheezing, feeling short of breath
anxiety, sweating, pale skin, severe shortness of breath, wheezing, gasping for breath, cough with foamy mucus, chest pain, fast or uneven heart rate

Sildenafil side effects: 

warmth or redness in the face, neck, or chest
stuffy nose
headache
stomach pain
upset stomach
nausea
diarrhea
memory problems
back pain
an inability to differentiate between the colors green and blue
loss of hearing
ringing in the ears
and dizziness

Stop using sildenafil and call your doctor at once if you have a serious side effect such as:

sudden vision loss
ringing in your ears, or sudden hearing loss
chest pain or heavy feeling, pain spreading to the arm or shoulder, nausea, sweating, general ill feeling
irregular heartbeat
swelling in your hands, ankles, or feet
shortness of breath
vision changes
feeling light-headed, fainting

Letairis side effects:

headache
stomach pain, vomiting, constipation
stuffy nose, sinus pain, sore throat
warmth, redness, or tingly feeling under your skin.

Call your doctor at once if you have a serious side effect such as:

anxiety, sweating, pale skin, severe shortness of breath, wheezing, gasping for breath, cough with foamy mucus, chest pain, fast or uneven heart rate
swelling of the feet, ankles, or legs
pounding heartbeats or fluttering in your chest
nausea, upper stomach pain, itching, loss of appetite, dark urine, clay-colored stools, jaundice (yellowing of the skin or eyes).

Adcirca side effects:

redness or warmth in your face, neck, or chest;
cold symptoms such as stuffy nose, sneezing, or sore throat;
headache;
memory problems;
diarrhea, upset stomach; or
muscle pain, back pain

Stop using tadalafil and call your doctor at once if you have any of these serious side effects:

changes in vision or sudden vision loss
ringing in your ears, or sudden hearing loss
chest pain or heavy feeling, pain spreading to the arm or shoulder, nausea, sweating, general ill feeling
irregular heartbeat
shortness of breath, swelling in your hands or feet
seizure (convulsions)
feeling light-headed, fainting

Tyvaso side effects: 

cough, sore throat
pain or irritation in your throat after use
dizziness
nausea, diarrhea
headache
flushing (warmth, redness or tingling)

Call your doctor at once if you have:

a light-headed feeling, like you might pass out
easy bruising or bleeding (nosebleeds, bleeding gums), or any bleeding that will not stop
unexpected vaginal bleeding
coughing up blood or vomit that looks like coffee grounds
blood in your urine or stools, black or tarry stools

Adempas side effects: 

headache
dizziness
indigestion
upset stomach or stomach pain
swelling of the legs and feet
nausea
diarrhea
nausea
vomiting
dizziness
low blood pressure (hypotension)
low levels of iron in the blood (anemia)
gastroesophageal reflux disease (GERD)
constipation
palpitations
stuffy nose
nosebleed
difficulty swallowing
abdominal bloating
swelling of the extremities

The following serious adverse reactions are discussed elsewhere in the labeling:

Embryo-Fetal Toxicity [see WARNINGS AND PRECAUTIONS]
Hypotension [see WARNINGS AND PRECAUTIONS]
Bleeding [see WARNINGS AND PRECAUTIONS]

Opsumit side effects: 

low red blood cell count (anemia)
common cold-like symptoms (stuffy nose or sore throat)
bronchitis
headache
flu
urinary tract infection

Clinically significant adverse reactions that appear in other sections of the labeling include:

Embryo-fetal Toxicity [see WARNINGS AND PRECAUTIONS]
Hepatotoxicity [see WARNINGS AND PRECAUTIONS]
Fluid Retention [see WARNINGS AND PRECAUTIONS]
Decrease in Hemoglobin [see WARNINGS AND PRECAUTIONS]

Orenitram side effects: 

headache
nausea
diarrhea
flushing
jaw pain
pain in the extremities
low blood potassium (hypokalemia)
abdominal discomfort

Please read your FDA insert for other side effects, as this is the only information that I found available without having to post a long explanation.  More information can be found here: https://orenitram.com/

Uptravi side effects: 

Headache

Diarrhea
Jaw pain
Nausea
Myalgia
Vomiting
Pain in extremity
Flushing
Arthralgia
Anemia
Decreased appetite
Rash

Please read your FDA insert for other side effects, as this is the only information that I found available without having to post a long explanation.  More information can be found here: https://www.uptravi.com/

Veletri side effects: 

flushing (warmth, redness, or tingly feeling)
nausea, vomiting, diarrhea, stomach pain
headache or jaw pain
joint or muscle pain
dizziness, sweating
feeling anxious, nervous, or agitated

Call your doctor at once if you have a serious side effect such as:

fever, flu symptoms, mouth and throat ulcers, rapid heart rate, rapid and shallow breathing, fainting
chest pain, trouble breathing
fast, slow, or uneven heart rate
pale skin, easy bruising, unusual bleeding (nose, mouth, vagina, or rectum), purple or red pinpoint spots under your skin
blood in your urine or stools
coughing up blood
feeling like you might pass out
numbness or increased sensitivity anywhere in your body

DISCLAIMER:  These are not a complete lists of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088

I hope this information helped you to get an understanding of the different types of side effects we as Patients deal with on a daily basis. Next blog: Daily living with PH 😊💜

Dealing with Illness and Pulmonary Hypertension

The worst thing in the world is being sick with a cold/flu or pneumonia when dealing with PH.  We already have a hard time with our lungs and breathing, let alone adding a cold to our condition.

When you get a cold you need to watch and make sure it doesn't turn into a Respiratory Infection, or worse yet, Pneumonia.   Make sure you are getting vaccinated and wear a mask when out in public during the flu season, especially at the Doctors office or Hospital.

If you are sick, stay out of my house. If you were exposed to someone with the flu or pneumonia, stay out of my house.

I do not want to end up in the hospital due to your negligence, my health is already compromised, I don't need your added germs coming in contact with me. It is much harder for us to recover from being sick than it is to prevent sickness. So please, respect our wishes when we say, stay home! 

Great boosters to help your immune system that are safe to take

Zinc

Vitamin C

Elderberry Syrup (any drug store has it) 

Over the Counter Medications that are SAFE

ANY Coricidin HBP product as it is specifically made for people with high blood pressure and heart conditions. 

• Dylsum Cough syrup
• Robitussin Regular cough syrup
• Regular Mucinex
• Zyrtec
• Benedryl 
• Claritin

Nasal Sprays

• Saline Solution
• Neti Pot
• Afrin that has Oxymetazoline ~ PH Pharmacist fine to use for short period of time
• Flonase

Medications you want to Avoid like the plague

ANYTHING that says it has:  

• phenylephrine
• pseudoephedrine
• ephedrine
• naphazoline

Dislaimer:  I AM NOT a doctor. This is NOT Medical advice. When in doubt and in need of medical attention, please consult with your doctor before taking any medication, as every PH Patient is different and is on different medications and react differently.  Always seek medical advice from a licensed practitioner before adding any medication or supplement to your current medication list. 

Thanks for joining me today. Keep on the lookout for the next blog: Side effects of medications 😊💜

You're not alone

When diagnosed with ANY disease, not just Pulmonary Hypertension, that is the biggest pill to swallow, and the hardest! How can you possibly get through this disease when you have no idea how to personally deal with it?  Family won't know what to do, how to act!  So how in the world am I going to get through this and deal with this?

SUPPORT SUPPORT SUPPORT

I can't say that I didn't know where to turn, because as many of you know, I have been dealing with this disease for many many years with other family members, but when it hit me oh boy was I a rats nest of a mess. NO LIE!   I denied it, I got mad, I got scared, I got frustrated and I had a support system behind me, my mom, then she died a little over a year after my diagnosis, then I felt totally alone.

There is a huge difference between support of a person who understands and the support from a family member looking in from the outside, just not the same.  So I did something about it, so I wouldn't feel so alone.

I looked up support groups on Facebook, got frustrated with the one's I joined and started my own with a very good PHriend, Tiffany Gunderman, may she continue to breathe easy.  I already knew about the disease, how to support others and comfort them, I just needed the interaction to help me cope better.  What I didn't need was the confrontations, and drama. So my group was created.  Anyway, back on track here.

I then joined PHAssociation and started doing emails with mentors and was put in touch with a local support group here in Pittsburgh, two actually.   One is for young adults from the age of 20-late 40's and then our regular support group for all ages.  Being around other's dealing with the same thing you are going through is a lot different than being alone and trying to do it on your own.  They understand and can show you empathy and sympathize with what you are dealing with, whereas a family member or friend can only show sympathy!  So again I say to you:

SUPPORT SUPPORT SUPPORT

And when I say support, it isn't just for patients, its for Caregivers, and children alike.  We all need support when it comes to dealing with a disease. No matter what it is.  Help your family help you but connecting them to support groups too!

Where do you find support

These links will give you the schedules on when you can meet up via telephone and talk with others

Local support groups:   https://phassociation.org/localsupportgroups/

Patient to Patient Telephone support:  https://phassociation.org/support/support-line/

Telephone support: https://phassociation.org/telephonesupport/patient-telephone-support-group/

Caregiver support:  https://phassociation.org/caregivers/support/

MyPHA support forums:  https://phassociation.org/mypha/

Young Adults support: https://phassociation.org/telephonesupport/young-adult-support/

Facebook support groups

My group that I run:  https://www.facebook.com/groups/304235876377617/  this is for Family, Friends, Patients and caregivers to learn about PH and support one another.  Age limit is 18 and older.  

All Mens group for men over the age of 18 who have PH or are caregivers but it is MEN ONLY:  https://www.facebook.com/groups/921528987974047/

All Women's group for ladies dealing with PH who are over 18 WOMEN ONLY: 

https://www.facebook.com/groups/1473142016347780/

Thanks for tuning in today. Join me tomorrow when I talk about: Dealing with sickness and PH 😊💜

Clinical Trials and Studies

What is a Clinical Trial?

A clinical trial is a study that involves a set number of people to test the effectiveness of a certain medication.

What is a Study?

This is normally done in  your clinic by various pharmaceuticals or researches gathering information where there is no medication involved. Usually based on survey's or blood draws to study the dynamics of  

Why are Clinical Trials important?

They play a very important role in the development of new medications to treat any number of diseases. Without trials, there would be no new treatments developed to help live a better life than what we are!

Why participate in a Clinical trial or a study?

Speaking from a Patient point of view living with Pulmonary Hypertension, a rare and debilitating disease, there aren't enough medications out there compared to other well known diseases like that of cancer, diabetes etc. So why not help find better medications and give your time to answer questions regarding your overall health?

To be a part of a clinical trial you have to take into consideration all of the pro's and cons. I remember when my mom was diagnosed in 1997 with PH, reminder, mine is hereditary.  She came home and said she only had the option of taking one type of drug, flolan. She was a full time employee (Manager) and a mother of 3 and grandmother of 2 small grandchildren with one on the way.   She came home from one of her appointments and said, we need to have a family meeting because I have a decision to make.  So we all sat down and she said, I can go on this drug which will hinder a lot of things I can currently do, or I was given an option to go on a blind clinical trial to test a drug for a new medication they are testing called Subcutaneous Remodulin.  I won't know until after a year if the medication is the real thing or a placebo. I actually still have all that paperwork 😊 Some of the family was against it, but the more her and I spoke the more she was leaning to going for the trial.  Finally she made her decision and went with the trial.  She had to go to the hospital for a few days to learn how to administer the medication, had a visiting nurse come to the house to show us how to help her in the event she couldn't do this for herself.  It was a long process going every few weeks to get monitored. But the thing with this was, we knew right off the bat she had the real thing, it stunk to high heaven! Her site became inflamed and she was gaining more energy. She also was  breathing easier.  She mentioned it to her doctor and he said he couldn't tell her that information, as he didn't know either, it was a blind study.  Anyway, without her partaking in this trial, she wouldn't have lived as long as she did, and it may have taken longer for it to be developed. 

Participating in a clinical trial can be a very satisfying and a worthwhile experience.

• Taking an active role in managing your care
• Gaining access to new treatments before they are more widely available
• Helping advance our knowledge of PH
• Receiving a treatment that works for you 

I have and still am in studies.  One was with medication that was already FDA approved. It was called the Symphony Program. You had to be willing to add Macitentan (Opsumit) to your current pathway, and they would monitor you for a total of 5 1/2 months with going back and forth every 6 weeks to the doctors office and answering questionnaires on a table and sending the information to the study company on how we were feeling while taking the medication.   Within a few weeks of me starting this, I was able to increase my 6 MWT by a total of 6 laps. You can find that trial and the results here: https://clinicaltrials.gov/ct2/show/NCT01841762

The other study I participated in was the Genetic code testing. Where they they took two bottles, not test tubes, of your blood and they study it for deficiencies and other things to see how they can possibly come up with more medication/cure for the disease.  The current study I am in is a sister study to the one I just mentioned. It is to do a case study on women, since we are most prone to getting this horrid disease.  It is basically an environmental case study that only involves questions. I will do these questionnaires every 6 months. 

There are also risks when considering a clinical trial:

• Side effects of the medications or treatments being studied
• Unwanted events during the trial that may or may not be related to the study drug
• Failure of a treatment to work

When doing a clinical trial, there has to be so many patients willing to partake in order for it to be a successful study, we'll get into that now. 

What is the protocol for a clinical trial?

How many patients will participate
Who is eligible
What tests patients will get and how often
What type of data will be collected
Detailed information about the treatment plan

Every clinical trial is different and lasts for a different amount of time.  If they do not get enough participants, the trial ends without any results.  There are a lot of factors to consider, the biggest is who is eligible. 

How can you get involved with a clinical trial or study?

Speak with your PH Team and see if they have any trials or studies taking place. If not, ask them to refer you to another clinic that has one that is open and registering patients. Remember though, they will discuss with you your condition and whether the trial would be a match for your condition.  Not all trials are able to be done by all patients. 

Where can you look up clinical trials and studies?

You can go here:  www.clinicaltrials.gov 

You will find currently recruiting, past studies/trials, completed, and terminated trials/studies.  I hope you found this information helpful, and thank you for reading. Stay tuned for my next blog:  Not being alone in  your PHight 😊💜

Pulmonary Hypertension Treatments

Treatment history

Prior to 1995 there were no FDA approved drugs to treat Pulmonary Hypertension. The only way to help a patient before that time was placing them on a transplant list for a Heart and Double lungs.

Today we have a total of 14 FDA approved drugs to treat, NOT CURE, Pulmonary Hypertension.  Of those 14 drugs we only have ONE FDA approved medication to treat Pediatric patients from the age of 3 to adult and that is Bosentan.

Medications in the same class as one another CANNOT be used together.  You are able to use 3 pathways for the treatment and control of PH, three is the most aggressive process in getting the disease under control.

You can see the average time span from one medication to the next for FDA approval of treatments for PH.








Our first approved medication was Flolan and Veletri, they were approved in 1995.  This is an Intravenous medication that uses a pump and hickman line directly to the heart for distribution of medication. Both medications are administered the same way, however; they are different.  Flolan has a different additive glycine-mannitol excipients and Veletri arginine-mannitol excipients.  Excipient means: Inactive substance to make the workings of the medicine. 

6 years between approval

Bosentan (Tracleer®) is an Endothelin Antagonist Receptor (ERA) oral medication that helps prevent the blood vessels from narrowing.  This medication was FDA approved for Patients in 2001 and recently the First FDA approved therapy for Children under the age of 18 for use. There is currently three different milligram dosages for a patient to take. There are a total of three ERA medications, which we will discuss later on. Bosentan was FDA approved in 2001 for the treatment of PH.

1 year between approval

 Treprostinil (RemodulinⓇ) Treprostinil is a synthetic analogue of prostacyclin, a naturally occurring substance in the body, which has effects on dilating blood vessels.  Subcutaneous infusion was FDA approved in 2002, picture to the left.  Intravenous with a hickman line direct to heart was FDA approved in 2004, picture on the right. This is the same type of intravenous administration as flolan which is pictured above.



Iloprost (Ventavis®) Iloprost is a synthetic analogue of prostacyclin, a naturally occurring substance in the body, which has effects on dilating blood vessels.  FDA approved in 2004.  This is like a mini nebulizer which is an inhaled treatment.

Revatio (Sildenafil™) is a Phosphodiesterase Inhibitor or PDE 5 which helps the lungs produce their own natural vasodilators. This medication was
FDA approved in 2005. There are two forms of this medication Pill form for adults and Liquid for pediatrics. However; it is not FDA approved for children but it can still be administered. There are two PDE5 medications the other will be discussed later.

1 year between approval

2 years between approval

Ambrisentan (LetairisⓇ) is also an ERA like that of Tracleer, that is orally administered.  This drug was FDA approved in 2007. 

Tadalafil (AdcircaⓇ) Another PDE5 oral medication (one of my therapies) was FDA approved in 2007. The same type of drug as Revatio.

2 years between approval

Treprostinil INHALED (Tyvaso®) is the inhaled version of Remdoulin and was FDA approved in 2009

4 years between approval

Riociguat (Adempas®) Soluble Guanylate Cyclase (sGC) Stimulators increase the interaction of sGC with another chemical (nitric oxide) to help the blood vessels in the lungs relax. FDA approved in 2013.

Macitentan (Opsumit^®) is another ERA that falls in the same category as Letairis and Tracleer. It was FDA approved in 2013. (also my drug)






Oral Treprostinil (Orenitram^®) oral version of Remodulin. FDA approved late 2013.

2 years between approval

Selexipag (Uptravi®) Selective IP Receptor Agonist targets and activates a prostacyclin receptor helping the blood vessels in the lungs relax. FDA approved in 2015.

I hope this information helped you to get an understanding of the different types of medication used to help treat, not cure, pulmonary hypertension. Please join me with my discussion of Clinical trials 😊💜

Who Treats Pulmonary Hypertension

Finding the Right Doctor

When looking for a Doctor to treat your Pulmonary Hypertension there are many factors that you need to take into consideration. The first being, what is the right doctor to give you the proper care and treatment! Not all Pulmonologists or Cardiologists know enough about the disease to be qualified to treat the disease. You want to find an accredited PH Specialist who has all the correct knowledge and means to treat your current disease of Pulmonary Hypertension. 

Why not a Pulmonologist?

Not all pulmonologists are equipped to handle a Pulmonary Hypertension Patients current diagnosis.  Just because they treat lung diseases, does not mean they are qualified to treat Pulmonary Hypertension. Many have no idea how to even go about treating it, let alone knowing what the disease is.  They mainly specialize in the following diseases:

• Allergies         
• Asthama
• Chronic obstructive pulmonary disease (COPD)
• Cystic fibrosis
• Idiopathic pulmonary fibrosis
• Interstitial lung disease
• Sleep apnea and other sleep disorders
• Vocal cord dysfunction

Why not a Cardiologist

When you think of Cardiologist, you think heart condition! That is the main reason why you would not seek looking for a cardiologist to treat Pulmonary Hypertension. Although the disease does affect the right side of your heart, it mainly affects our lungs, before it affects our heart.  Here are the conditions in which a cardiologist treats:

• Coronary artery disease
• Heart Attack
• Abnormal heart rythms, arrhythmias
• Heart Failure
• Heart valve disease
• Heart muscle disease
• Pericardial disease
• Aorta disease
• Vascular disease

Where do you find the correct doctor

So, I mentioned not to seek out a regular Pulmonologist or Cardiologist, however; there are MANY that are PH Specialists that can assist. Its just a matter of finding the correct one to treat you.  The best place to do that is here: https://phassociation.org/patients/doctorswhotreatph/ although this is not a complete list of all the doctors that are trained to treat PH, this is the most accurate listing you will find.  Another great way is to ask another PH Patient for recommendations on who they see.

Find a Pulmonary Hypertension Care Center

There are two types of Centers that are accredited to helping those with Pulmonary Hypertension. One is Center of Comprehensive Care and the other is Regional Clinical Program.

PHA board of Directors and the Scientific Leadership Council worked very hard at getting centers accredited to get the best care possible for PH Patients around the States.

 To find an Adult PHCC Center near you please visit this link: https://phassociation.org/phcarecenters/accredited-centers/

For Pediatric PHCC please visit this link: https://phassociation.org/phcarecenters/peds/

Thanks for checking in today, stay tuned for tomorrows blog where I talk about treatments 😊💜

Stairs with Exertion

How winded are you walking Stairs?

To a normal person, that isn't a problem for you.  For us, it isn't an easy feat! Walking up a set of stairs is a great accomplishment and feeling!

So what does it feel like when you walk a set of stairs?  Let's do a test shall we.  

1.   Take a straw, open it, insert into mouth

2.   Plug your nose and start breathing through the straw

3.  Walk up the stairs and down again for 1 full minute

HOW DO YOU FEEL?   Did you Actually do this?

For a PH Patient this is how we feel; tight chest, heart rate is racing and we feel faint and dizzy.  It will take many of us about 4 to 5 minutes to fully catch our breath before we can proceed to do anything else.

I don’t know about some pholks with PH, but I have 15 stairs in my house to get me from the first floor to the second floor, and that is the biggest challenge for me.

Someone in one in my support group forum on FB asked how come it makes us so short of breath when we do a flight of stairs. The explanation I was given by my CRNP is this: “You are going in a vertical position when walking up the stairs, carrying not only your weight but using more oxygen (O2) when you are moving upwards.”

So think of it like this: When you are on a flat surface, you have no weight with little restriction to your lungs or body while moving. When going up a flight of stairs you are now putting added weight to yourself because you are in an unwavering position while walking up those stairs. Many lean forward to counter balance the incline, which causes our diaphragm to close off and makes us breathe shallower which gets us out of breath faster than being on a flat level surface. Make sense?  

So what can we do to help ourselves from getting out of breath so easily going up the stairs? Hold onto the Banister and Stand straight up and down, DO NOT lean forward.  Stop in the middle, catch your breath and then continue going up. When you get to the top, stop and once again catch your breath before continuing. 

Thanks for reading, until tomorrow when we talk about doctors. 😊💜