Understanding Pulmonary Arterial Hypertension (PAH)

Pulmonary Arterial Hypertension (PAH) is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and may become life threatening.

In the advanced stage of the disease, the blood vessels in the lungs are narrowed, thickened and stiff, causing it difficult for the heart to pump blood through, causing the heart muscle to weaken over time. Clots can form and block part of the vessel's lumen as well.

PAH is commonly associated with connective tissue diseases (CTDs) including systemic sclerosis and systemic lupus erythematosus (SLE).

If you are diagnosed with Pulmonary Hypertension, you can still lead a fulfilling and meaningful life. Take appropriate measure to take care of yourself and avoid factors that can worsen your condition.

This video is brought to you by National Heart Centre Singapore, in partnership with Singapore General Hospital. Supported by Actelion

[martinaskupin]

When I went to hospital to be hospitalized in April 2018 for not being able to breathe for several months, doctors behaved like I was lying and pretending my problems.

I was listening from 6 doctors for months, since June 2017, that my problems were caused by psychosomatical reasons, that I should just find psychologist and I will be fine. Cardiologist, pulmonary specialist, vessels specialist, alergologist, psychiatrist, practical doctor etc. Pulmonologist suggested even yoga, so I can "learn to breathe properly". Psychiatrist wanted to hospitalize me on psychiatry, so I can go on psychotherapy and that will solve my inner problems and I will be then able to breathe normally.

Every one of them dismissed my symptoms, which started last June. It was clear to me, that my problems with breathing must be caused by problems in heart or by lungs. The first thing I did was, that I went to cardiologist. She did echocardiogram. And she didn´t find anything. Symptoms were getting worse and worse. In August I visited alergologist. Nothing. In September I visited pulmonologist. Nothing.

In September I started to vomit, had diarrhea, coughed so much, because I was suffocating, that I vomited. Nausea 24/7. In december 2017 I lost consciousnes when I got coughing attack and my brain didn´t have enough oxygen. When I fell, I injured my legs, I fell in the bathroom and hit my head with the wall.

I sometime lost consciousness sitting in the chair.

At work I had to eat only in the morning, because I went home at 4pm and it took me one hour to get home. I didn´t eat after morning, because I tried to avoid possibility of having diarrhea or having to vomit in the bus or at the bus station. If I ate then when I got home, I vomited that meal too. I vomited once few meters from bus station, minute after I got out of the bust. Once I got home in the last second, I had to hurry, even when I couldn´t breathe, because I got diarrhea at the bus and I held it as I could.

Since January my legs were getting swollen and then they were swollen even to half of thighs. I couldn´t walk.

I had nervous breakdown during visit to pulmonary doctor in January 2018 (I have also asthma), I just couldn´t take it anymore physically and psychologically.

No doctor cared to find out why I couldn´t breathe, why I was able to walk max.3 steps and then stop.

In April 2018 it was so bad I went to hospital. Doctor touched my horribly swollen legs with two fingers and said, that they aren´t swollen. They sent me to CT. Nothing.

After like three hours there they sent me to cardiologist, I had to carry my heavy packages I had prepared for hospitalization, and this doctor did echocardiogram and suddenly found, that I have very severe pulmonary hypertension and ordered me to immediately sit down, because “you can´t walk around in such state”.

After two days they sent me to centre for pulmonary hypertension and PH specialist did right heart catheterisation and confirmed, that I have Pulmonary arterial idiopathic hypertension, in the worst phase and said, that it was developing in me for 2 years already! And that without immediate medications I would be dead within few months!

But according to those 6 doctors I was perfectly OK and I couldn´t breathe due to my depression and anxiety, while whole time I was dying. And that doctor, according to whom I didn´t have swollen legs? I lost 11 kilograms in two weeks in the hospital. All of it stored water. Lazy doctors. Lazy cardiologist last July. Lazy pulmonologist. If only that cardiologist last July really did her work, I would be diagnosed in so much better state and now I don´t know, if I will be alive in 5 years. Without diagnosis and medications I would be dead by now already.

Per the last study among patients with Pulmonary arterial hypertension, we are usually diagnosed within 4 years since first symptoms. I didn´t have 4 years. I would be dead within 3/4 year since first symptoms.

After doctor told me diagnosis, I had nervous breakdown and developed PTSD.

I used to go to theatre, cinema, library, fitness - nothing of that is possible now. I used to ride on bicycle around my town - impossible now.

I barely walk. Since spring 2019 I walk with cane, but I am able to walk only few meters till I have to stop and rest.

I have arythmia, palpitations, sometimes syncopes from coughing. I am on full disability.

My skin is horribly dry, my hands and arms itch.

I live in the chair in the living room with laptop - youtube, TV shows, pinterest.

I can´t read much anymore, I can´t focus on longer texts.

Since my dad died, I can´t read fanfiction.

I had to leave my job, where I was best in my team.

I was diagnosed late, very late. My PH specialist said directly, that without immediately getting on meds, I would die in two months. Meds are maybe keeping me alive, but I don´t feel better. My health state is getting worse and worse.

I loved my independency. Now I have nothing. Only depression, anxiety and PTSD, huge problems with memory and concentration and I can´t even go on massage, so my back will hurt from no movement till I finally die.

I was on Veletri for 8 months. It was horror. I was getting worse. So they took me off Veletri, put me on Revatio - Sildenafil with beautiful side effects of the worst anxiety attacks I have never experienced in my whole life and with huge problems with sleeping. After two months they put me yet on Uptravi - amazing side effects of days lasting diarrhea. Thanks to Sildenafil I didn´t sleep properly for 3/4 of year, now I am on Trittico and Rivotril for sleeping and trying Cipralex to help with depressions and Xanax. Plus I am on Volibris. I don´t feel better.

Since this year 2020, prices of basic food in Czech republic went rocket high, vegetables, fruit, meat, even potatoes. Some food is even 100 % higher.

I get to my bed on 1st floor step after step with horrible arythmia and hoping, that I won´t faint on stairs. I go upstairs only once a day for sleep. The rest of day I spent in the living room, only going to toilet and kitchen. I take 250 mg of diuretics every day. Omeprazol for heart burn. B-complex. Contraception. And I have heart burn from drinking water, so I can´t drink it. I have also allergy on most of fruits. I am exhausted 24/7. Everything physical is unbelievable exhausting. Also, oxygen doesn´t help me, they tried it at the hospital, no effect.

[katrinejacinto]

What do ix the medical term of pulmonary enlargement of the heart?