A simple echocardiogram started it all on December 18, 2004. After abnormalities were picked up and my O2 sats were 72 at rest.I was hospitalised in NE Victoria immediately to be stabilised with O2 therapy and medications. I'm on oxygen 24/7. December 24, 2004 and I'm at the Alfred Hospital Melbourne being given the diagnosis of PPH (now IPAH or IPH). Great Christmas present!!! I was told to go and enjoy Christmas and New Year and be back at the hospital on January 4. Terrific when the family are flying to America on the 6th.
Ten days rest and a barrage of tests confirmed moderate to severe PPH. After further tests and a RHC in March I was started on Bosentan. What a difference one little tablet made. I had energy and felt better than I had for ages after only 8 days. I was also diagnosed with sleep apnea and prescribed a CPAP machine for use when sleeping.
Sadly in late 2007 I was one of the few that develop liver problems with Bosentan and had to stop it after 2 years. Sildenafil replaced it and, whilst I was stablised, I didn't have the energy levels I did with Bosentan. One thing I have proved possible in 2008 is that with planning and good surgical staff and back up staff it is possible to have surgery with PH. 2 operations this year with no problems thanks to the expertise of medical staff at the Alfred just shows that it is achievable.
In hindsight we are sure that I have had this beast for at least ten years prior to being diagnosed. What initially was diagnosed as asthma probably wasn't, particularly around the time I started telling doctors my asthma medications weren't working. We can all be wiser with hindsight and do not need to dwell on the past but look to the brighter future.
Roll on to now 2013.
In 2009 the sildenafil didn't seem to be doing the job so in for a RHC to see if the pressures had changed, yes they had so new meds were looked at. I was one of the first in our state to be given ambrisentan (Volibris) and I noticed the difference within 2 weeks. I had more stability and les shortness of breath. 2011 was a horror year after May when I was in and out of hospital with lung infections then hit with full blown H3N2 flu in October, 10 days in hospitals but with side affects of "looping" low blood pressure that is still with me today. I stayed stable with pulmonary pressures and blood flow until 2012 when I started to feel more breathless doing the simple things. Medications were tweeked but things got worse so after failing my 6mwt test (and a repeat weeks later) a RHC was ordered and my pressures had jumped dramatically. Our government subsidised medication scheme will not provide the medication we are on when we fail so an application was made for compassionate supply from the Drug company which was granted and I'm eternally grateful. We had added tadalafil (Adcirca) and it will be interesting to see how dual medications perform.
It is now 2015.
I've been on the dual medication of Ambrisentan and Tadalafil now for over 2 years and I can say they are working. I can now do things I haven't been able to for years and my pressures have dropped. At my last specialist appointment in Hune it looked like my heart had reduced in size, a surprising development. My oxygen saturation levels are higher as well. I will be interested see if I get my ususal 2 to 3 year change in this disease, hopefully nothing occurs.