Deterioration and New Medication

Dear Readers,

Since the beginning of March, I have noticed a decline in my PH symptoms, the worse being the shortness of breath. For me, PH is usually an invisible illness, but as time has gone on, it's become more and more visible, and I have hated it.


At the beginning of the deterioration, we blamed it on the cold weather, and kept saying, once it's warmer, I'll start to feel a lot better. Unfortunately this didn't happen, and I began to struggle more and more. I am a very active PH'er and really enjoyed going for a cycle on my e-bike with Terry, however this had to come to an end. I do have an exercise bike at home and I go for a very short cycle with my oxygen on, every other day, to keep my fitness up.

During the summer, I went on a mission to lose weight, as I put on over a stone during lockdown. With slow and steady exercise, I managed to shift this weight thanks to my exercise bike, but I find it difficult that I can't continue to exercise the way I did. 

Any type of exercise, I am now having to use oxygen, whether it's cycling, walking or just moving around the house. It's been awful for me. I do have prescribed oxygen at home, but I only take it when I need it, eg, after a busy day out with friends, I go on it as soon as I get home. Now, it's first thing in the morning, before I go to work, when I get back, before I go to bed. It seems never ending. I have also had to be pushed around in my wheelchair when we go on walks or go out for the day. Though Terry is amazing and is happy to push me around, I hate being so reliant on him. I am usually a very independent person, so this has taken quite a hit. PH is taking over my life and making a big impact, so I knew I had to try and get something sorted!


I have been very fortunate that I have my new job, well I've been in it for 5 months now, but it has kept me going. It's only a few hours a week, but it is local and I am loving every minute. It has kept me going and gotten me out the house. It helps me to keep my mind off of my health and concentrate on something I love.

Due to lockdown, hospitals have been very busy seeing many patients, but I was fortunate enough to go to a day clinic on 16th August. I had the usual tests; ECHO, ECG, bloods and we were really pleased to hear that my heart is still looking strong and stable, with no change! But, what was causing the severe shortness of breath?! I went home and waited for the PH team to have a MDT to discuss my case.

A few weeks later, they had arranged a sleep study for me. This is because, during the night, I have been having trouble sleeping and have woken myself up choking and gasping for air. The results of this came up as not having sleep apnea, but this could be related to the deterioration of my PH symptoms. They feel, once I get this sorted, I hopefully shouldn't get the sleep disruptions.


I hadn't gotten any better since I went on 16th August, so it was arranged for me to go as an urgent case back to the Brompton yesterday. Terry and I went to the PH clinic and we saw my PH consultant. I repeated the ECG and bloods to see if there was any change from a month ago, but everything was still looking good. After a long discussion, we have decided to change one of my medications. We are still not sure what is causing the severe shortness of breath, but are hoping that this new medication may be the answer. I have been on Sildenafil for 13 years now, since I was diagnosed, and sometimes the body can get used to the same medication after such a long time. I am now on Tadalifil, which is better, as I only need to take this once a day, instead of 3 times a day and I have also kept on the Macitentan. 

We are keeping everything crossed that this works, as the last time I felt this poorly, we added Macitentan to Sildenafil in 2017 and I was back to my normal full health very quickly. As I only have one lung, we are being very careful not to miss anything with the respiratory side of things, so I will be returning to the Brompton for a lung function, MRI and a Cardiopulmonary Exercise Test (CPET) as soon as I have received a date for these tests.


I know changing to Tadilifil won't make an instant change to my PH symptoms and I hope I don't suffer from too many side effects, if any at all, but let's keep positive that it's onwards and upwards again! Also, don't forget to join myself and Terry every Wednesday for a new episode of our podcast, PHighter Tess Podcast.

Thanks for reading!