PH Plus Awareness: Samantha-Anne's Story

This is a long post. If just one person reads this, though, and it helps them to not have to wait 3 years to be diagnosed with Pulmonary Hypertension, then it has been worth it.

The first time I heard about Pulmonary Hypertension was on August 18th during my first appointment with the pulmonologist. It was included in a list of other things I hadn't heard of that might be causing my illness (interstitial lung disease, cardiac asthma, sarcoidosis and PH; I had heard of pulmonary embolism because that is what killed my brother in 1990 at the age of 47.) I knew that all of these things were serious, so I starting learning about them. I'm not sure why it took so long for my doctor to send me to a "lung doctor" when my main complaint for the past 2 years had been shortness of breath (SOB), but I was glad to finally be there.

PH seemed to fit with what was going on with me. However, a lot of other diagnoses had fit before and I honestly didn't want to be disappointed yet again when the doctor told me that I didn't have x,y or z and that he didn't know why my life had changed so drastically since November of 2011. After doing pulmonary function tests, an exam and having me walk in the office while my oxygen saturation level was monitored, the doctor said I would need to have a CT scan to rule out blood clots in my lungs. That honestly was not scary to me because I've been checked for that so many times due to my family history. He said that if there were no blood clots, then I would have to have a right and left heart catheterization procedure to see if I had PH. I had a left heart cath a year ago so I wasn't nervous about that, and since I had no idea what PH was, I didn't worry about it either.

My pulmonary function tests the year before showed that I have a mild restrictive process in my lungs but no obstructive process, so no COPD (which my mom died of in 2000, so I was glad that was ruled out.) I do have mild Alpha-1 Antitrypsin Deficiency, which can affect the lungs, so I knew that lung problems could happen to me. The PFTs he did on 8/18 showed that my lungs were not moving the oxygen in the air I breathe back into my blood (that's poor diffusion capacity). Mine was about 1/3 of normal. An MRI had already shown that my pulmonary artery is slightly enlarged, so I knew that something was going on and all signs were pointing to PH.

Because I really push to get things scheduled quickly and stay on top of my care, I was able to get the CT scans scheduled for 8/21, just 3 days after seeing the pulmonologist. The CT scans showed some mild lung scarring that I already knew about and thankfully no blood clots.

During these weeks I was dealing with a lot of body wide pain (unrelated to the PH) and swelling in my feet, ankles and legs. They basically were just huge stumps, from my toes to my stomach, with no delineation between the three. They hurt and the stinging in my feet was unbearable at times. I was also having to keep an eye on my kidney status because I have Stage 3 Chronic Kidney Disease. Thankfully, there are 5 stages and my disease is mild.

Let me take a second here to talk about the word "mild". I hate that word now. Everything I had going on with me was "mild". Mild means that the doctors don't do much for you and they basically wait until it's not "mild" anymore before they get serious about talking to you or treating you. At least, that was my experience. So even though "mild" is good, it sucks. My cardiologist told me that my heart issues were "mild" and didn't need to be treated and that I should see if the pulmonologist could help me figure things out. That just didn't seem right to me. I started asking myself a lot of questions and realized that I had a lot of questions for the doctors. My life had changed drastically in the past 2 1/2 years and something was causing that. There is no way I could go from being able to hike 2-4 hours at 7000-8000 feet altitude to being SOB and having a racing heart just walking room to room in my house.

I started believing that PH was the illness that was causing my problems so I made an appointment with the PH Specialist in Denver. Neither my cardiologist nor my pulmonologist told me to do that. I did it because I was tired of not knowing why my life had been turned upside down and I wasn't going to wait on them to tell me what to do to get better (since they hadn't done that anyway.)

At the beginning of September, I went back and looked at the sleep study I had done the previous November. I knew I had sleep apnea and was supposed to wear a CPAP, but it made me feel claustrophobic and like I was suffocating so I didn't wear it. I was diagnosed with obstructive sleep apnea in 2006. I got my first CPAP that same year. I had never used my CPAP consistently in all that time. When asked by my doctors if I used it, I told them how it made me feel and that I was not using it very often. No one followed up, no one did any education, no one did any counseling and no one referred me to a sleep disorders specialist. When I looked at the November sleep study (that I had requested be done,) I noticed that my oxygen level was <90% for >99% of the night with and without the CPAP. That didn't seem right to me. I thought that oxygen levels were supposed to be above 90% all the time. The pulmonologist had mentioned getting me compliant with the CPAP so I thought I should look into that. I called the center where I had the study and no one, to this day, has ever called me back. I just kind of figured that it must not be that big of a deal if no one called me back. I was very wrong about that. 

One of the most fortunate things that happened to me was getting sick around September 7th. I ended up in urgent care for bronchitis and an upper respiratory infection. They gave me oxygen and a breathing treatment and sent me home with a ton of meds. I called the pulmonologists office to let them know about my sudden illness. I spoke with Linda, the doctor's nurse, for 41 minutes on the phone. She was the first person who seemed concerned about the results of the sleep study and that I was so sick for so long without a diagnosis. She asked me to come into the office that afternoon so she could see me. She did a very thorough exam and asked a lot of questions. I left the office that day with supplemental oxygen. I could tell right away that adequate oxygen intake is a good thing. It felt so good to breathe. She took copies of my sleep study and my other paperwork to discuss with the doctor before my appointment the following week. Linda really cared, and her taking the time to evaluate me has made all of the difference in the diagnosis of my illness. Just days later, I was set up with 24/7 oxygen at home, including using it with my CPAP at night so I could get adequate oxygenation. On 9/12, when I had my PFTs done again, my diffusion capacity was 2/3 of normal. That was an improvement. My lung function wasn't as good, but I had bronchitis and the URI so that kind of made sense.

So, at 54 years of age, I was on supplemental oxygen 24/7 and taking diuretics to reduce the fluid overload in my body (hypervolemia). I was feeling older than I am. 

On 9/18, one month after first hearing about PH, I was in the cath lab getting my pulmonary artery pressure looked at. The cardiologist told me that I had PH (the pressure in my pulmonary artery was higher than it should be), that my body was overloaded with fluid and that I probably have hypoventilation syndrome (I breathe too shallow) due to being overweight. It was a bit overwhelming. The pulmonologist confirmed the diagnosis on 10/6. The PH Specialist confirmed the diagnosis on 10/7. They both told me that I needed to treat my sleep apnea, continue using oxygen 24/7 indefinitely, lose a significant amount of weight, consider moving to a lower altitude (I live at 6,700 feet) and definitely not travel to higher altitudes and keep my sodium intake to <2000mg/day and my fluid intake to <1.5L/day to combat the fluid overload. That was VERY overwhelming.

Thankfully, I was seeing a counselor to help me deal with being chronically ill even before I got the diagnosis of PH. She has been wonderful and extremely helpful. I also was dealing with the possibility of having lymphedema (causing the swelling in my legs) and had to work through the process of getting that diagnosis ruled out. I had to have a nuclear scan done where they inject the radioactive dye between your toes! Thankfully, it sounds a lot worse than it is. No lymphedema, so that's one less thing to worry about.

I have had a lot of questions for all of my doctors since being diagnosed with PH. There are 5 different groups of PH. It looks like I am in groups 2 (PH due to heart issues) & 3 (PH due to lung issues, which are sleep apnea and hypoventilation syndrome). Group 1 is the only type of PH that has specific medications to treat it. It is also the most rare and dangerous type of PH. Every single patient's experience with PH is different than every other patient's experience with PH. There are similarities, but everyone has something unique to their illness. That makes it difficult to treat PH. In my case, since my PH is due to underlying conditions, those underlying conditions have to be treated to make the PH get better. I have to treat my sleep apnea, my obesity, my fluid overload and my oxygenation issues. I'm hoping that if I treat those things, that the PH will get better and/or go away completely. I may have to deal with PH the rest of my life. To what degree, I don't know.

I have spent a lot of time on the phone with the PH Specialist's nurse. I wasn't supposed to see him again until April, but they want to follow up with me. So, I am going back up to Denver next week. The difficulty with having PH due to other illnesses is that no one is quite sure who is supposed to treat and follow up with the patient. It is a heart disease and it is a lung disease. It is complicated. Thankfully, the specialist is going to make sure that I get my questions answered and that I understand my PH as best as I can.

David and I are feeling overwhelmed right now. We are considering moving to a lower altitude if it would be the best thing to do. We still have so many questions. I am hoping that my appointment with the specialist next week sorts a lot of this out. I am looking at doing a Pulmonary Rehab Program 3x/week for 3 months to get me active again. I have basically been sedentary for the last 3 years. I'm hoping to get a portable oxygen concentrator (a machine that makes its own oxygen) so I don't have to bring oxygen cylinders with me everywhere and have to constantly be refilling them. I want to be mobile and get out again. I want to understand what my flavor of PH means to me. What can I do to feel better? What can I do, if anything, to halt/reverse/eliminate PH in my life?

I am going to see the ENT/Allergy doctor this afternoon to help with the constant nasal congestion I have. That keeps me from wanting to wear my CPAP mask at night. Wearing oxygen full time affects your nose and sinuses. What can I do to make sure I comply with wearing oxygen 24/7? I am going to see the sleep disorders specialist tomorrow. I know that being compliant with my CPAP, treating the sleep apnea and reducing the amount of time that my oxygen level is below 91% is crucial. I need to know if I do have the hypoventilation syndrome. I'm not sure if he would diagnose that or not but I'll ask him. And I definitely need to sleep better. Being rested is important, too.

I have a lot of huge lifestyle changes to make. I'm working on making those changes every day. I am looking at every option from surgical weight loss to moving from my beloved Colorado back to sea level (or as close as I can get). Being diagnosed with PH has changed my life. It has changed David's life, too. I am sad knowing that I cannot go back to Waldo Canyon to hike. My goal since I got sick 3 years ago was to get back to Waldo Canyon to hike again. It's at 7000 feet, though, so that is not an option right now. I can't go up to the summit of Pikes Peak, 14,114 feet, and get a "high altitude" donut (they just taste different up there!). Life is different now. I'm relieved to finally have a diagnosis so that I can move forward with my life, but it's slow going right now. I know that, quite honestly, one of my options is to do nothing and just get worse or at least, no better. I don't think I will do that, though. My pulmonary rehab program evaluation is next Thursday. I may be able to start the program on Friday, Monday at the latest. It will feel good to be active again.

The PHA website has been an invaluable resource since hearing about PH. Not only do they provide lots of information but they also provide a lot of support through online chats, telephone support groups, one on one email and telephone support and educational resources. I can't thank the people and other patients on the PHA website enough for being there for me and helping me to navigate through all this since being diagnosed with PH.

Thank you for reading this very lengthy post. I hope it helps you to understand the journey to a PH diagnosis and the affect it has had on my life. I hope it helps someone who needs to be diagnosed or has been recently diagnosed with PH. 

Written by Samantha-Anne Wagoner