Welcome New Members- Spring Edition 2024!

[jen-cueva]

Another new member, Jenna, is a caregiver for her husband with PH. She is also new to our forums and wants to share some of her PH journey. Please help me welcome Jenna and her family.

@jsexton shares, “Ok, a little about our whole journey. Dustin my husband was 31 when we found out he had Idiopathic Pulmonary Hypertension. I still remember the day he tried to push mow the yard as he had done a million times over. After 4 doctor visits to different doctors, b/c they kept telling him he just had allergies, I called my mom (RN at clinic) and told her I was sending him to her and the Nurse Practitioner she worked for. That day, they found a second heart sound, and we quickly got him into the Heart hospital for a CT. He actually coded in the CT room as we found out he was allergic to the dye. I was 27 weeks pregnant and lost that baby(stress). They got him back and took him to Baptist Hospital, where they diagnosed him 7 days later. They sent us home to take some random meds that stated if we wanted to get pregnant for me not to touch the bottle. They gave him 3 years to live, and my insurance assigned us an end-of-life specialist through BCBS.

The crazy thing is she had worked with my mom at a hospital about 45 minutes from my home. I started the research and realized that Baptist was not a PH Care Center. So, after a few days, I told her I wanted to go to a specialist for a second opinion. A week later, we were at Barnes Jewish and seeing Dr. Murali Chakinala. We have been with him ever since.

As of June 2024, Dustin is currently taking Adcirca, Opsumit, Amlodipine, 2 Lasix pills, Breo Elllipta, Remodulin SQ Infusion, and just got our first shot of WinRevair.

He went for the first 4 years on Amlodipine only. He is a very, very rare case. I have been told by our doctor that his story is shared with several PH Care Centers. The first few years were pretty easy and we had Twin Boys in 2014. By then, they had already added Adcirca b/c his heart was showing signs of needing more help. 8 years ago, he started going downhill pretty badly. He put him on Remodulin immediately as he had declined so much that he was seriously in trouble. I had to do CPR in the house over 20 times in a year or so until he got to a dose that was keeping him stable, and actually, then he was back to his normal self. He has syncope with his PH, but his heart was so bad that it shut everything down.

When I say normal (why they say he is different), Dustin runs, plays softball, walks miles to hunt, does p90x, and has been very active. Which is how we know when his heart isn’t functioning well. He has ”handled” the pain for those first few years until roughly 2 years ago when I started telling them that this wasn’t going to work. He was having site failure after site failure. Where in previous years, he was getting 6-8 weeks from a site, and the scar tissue was building too badly. He would get a week to 2 weeks at max before infection. They had a hard time listening b/c he was doing so well. We went back in May of 2024, and they finally heard me. So now we are on the Winrevair journey. My insurance doesn’t/ nor would they cover it as it is new. Our doctor’s goal is for us to be admitted to the hospital in the next few months to come off the Remodulin and either go to Orenitram or Tyvaso DPI.

We got approved for Merck Patient assistance this week, and a nurse came and trained us on the shots on Friday, June 21st, 2024. All in all, in his 12 years, he has done well, to say the least. We are truly thankful to God that he has watched over, protected, and guided us through this disease. Dustin will tell you this disease saved him spiritually. He turned his life over to God a few years into his journey. We are very blessed, to say the least! I hope to keep up with more here and maybe help others.”

[jsexton]

More than likely, they will do a right heart catheterization. However, our doctor no longer goes through the groin but goes through the neck.

[Colleen]

@jsexton a right heart cath is not only the best way to diagnosis PH, but also helps determine it’s severity and what treatment might be most effective for the patient. My son has had a cath both through the groin and the neck. I’ll have to ask him which he preferred and why.

[jen-cueva]

Hi @Bren, yes, the RHC is often done through the neck or groin. I’ve also heard some have it done now through their wrist, which many prefer. I can’t count anymore how many caths I’ve had because I was diagnosed almost 20 years ago. But the neck is my preference for me. Although I have a small frame, the groin is so hard not to have it clot as fast, and there is a chance you will bleed some longer and have to stay in the holding/recovery area longer.

But the last time, my team shared that I have so much scar tissue now in the neck area that we may need to try to rotate the entry of the cath.

Thanks, @jsexton, for sharing that info and your experience as you take care of your husband. It’s also often what the center’s protocols are and what other health concerns or health histories each patient has.

[jen-cueva]

Hey, y’all, please help me welcome a few more of our newer members to the forums. Let’s welcome, @Rosa , @BethD , @CBear , @Jeannie , @Mike , @jholland1964 , @Jille , @Buglady , @Saloni , and @Aimee . We are thrilled to have you join us and look forward to learning more about you and your PH journeys.

You can share as much or as little as you wish; below are a few ideas to get you started.

** Are you a patient or a caregiver?

**How long have you or your loved ones been diagnosed with PH?

**What are your hobbies?

**Do you have questions for our members?

**Which PH treatments are you currently on?

Let’s get to know each other and see how we can best help each other along this PH journey.

[jen-cueva]

Our newer member, @Nonie, shared a bit of her PH journey with us, and I want to post it below so it’s easier to find. Please help me welcome Nonie to the forums.

“Hello Jen, Thank you for the warm welcome!

As you know I have been dealing with PH for about 7 months, I was diagnosed in November 2023 after a scare and almost going into heart failure.

I was misdiagnosed for about a year as we thought I was suffering from asthma. I’m a teacher and was starting to have trouble walking across the hall or even my classroom without getting out of breath as well as along from my car to the building. We also thought having covid 3x could have resulted in scarring of my lungs, and then I got RSV in December 2022 and January 2023 and I was really struggling to recover from the cough, so we amped up the asthma treatments with a nebulizer and inhalers. Then in November 2923, I got sick with another respiratory infection and was over-using the inhaler and nebulizer, meantime my oxygen was tanking which resulted in a trip to the ER and tests which indicated enlargement of my heart and possible PH. I went on oxygen therapy and within a week my heart had shrunk and my labs were normal.

I followed up with a cardiologist who cleared me and referred me to a pulmonologist who did a PFT, CT angiogram and a nuclear medicine scan, all indicated an enlarged pulmonary artery. My pulmonologist suspected sleep apnea and after a referral to a sleep doctor and a sleep study, we found that I have severe sleep apnea (I stop breathing 116 per hour). I got my CPAP machine on June 1st and so far so good. I average o.4-1.0 apneas an hour as compared to 116! have more energy and have been able to do things I hadn’t been able to before (cook, clean my house, enjoy my garden, etc.). I still use oxygen daily because lengthly walking or any over exertion causes me to be breathless and my oxygen to go down.

Our hope that that the CPAP therapy will start to reduce the pressure and the size of the pulmonary artery. I have high blood pressure, so I already take Enalapril and HCTZ.

I have a wonderful support system both at home and my school which I am grateful for everyday. I’m doing my best to stay positive and take it one day at a time, however I am encouraged with the progress I’ve made with the therapies so far.

I look forward to connecting to other here.

Thanks!”