Living with PH Plus... HHT

Christine Fini, PH and HHT Patient

My name is Christine, and I have pulmonary arterial hypertension (PAH) and hereditary hemorrhagic telangiectasia (HHT). My PAH was diagnosed in November 2006. I knew months before that there was something wrong; I could not physically keep up with co-workers, family or friends. I just assumed that my age (49) and weight (well beyond pleasingly plump) had finally caught up with me. I currently take sildenafil (RevatioTM), bosentan (TracleerTM), and as of October 21, 2009, I began taking treprostinil (TyvasoTM) which replaced my iloprost (VentavisTM) and seems to be really working well for me.

HHT is something that has always been a part of my life. Ever since I can remember, my father has had nosebleeds; it was just a part of my family members’ lives. I have had AVMs (arterio-venous malformations) coiled in my lungs and have had to have nasal cauterization to help manage my nosebleeds. My father and one of my sisters have been diagnosed with HHT; three of my four other siblings have been screened and do not have HHT. We also suspect that my four-yearold niece has it since she has an unusual amount of nosebleeds for such a young child, and we plan to have her screened when she is a little older.

Living with these two diseases is a challenge. I am still employed full time. I enjoy my job very much though it has evolved in the past three years to accommodate my PAH and HHT. At times my fatigue, which I believe is a combination of anemia from the HHT and just a part of PAH, can set me back. I’m still learning to accept my ‘bad days’ and to enjoy my ‘good days.’ ‘Bad days’ are the days when I can’t seem to muster the energy to get out of bed to go to work or when just getting ready to go to work is so tiring that I consider my 45-minute commute a chance to rest! ‘Bad days’ are also the days when I have the opportunity to babysit my nieces and nephews and have to decline because I just do not have the energy. ‘Good days’ are those days when I feel like my ‘old’ self. I can do normal, simple things like grocery shopping, house cleaning and putting in a good day of work without exhausting myself. With the love of my family, all my days are truly blessed.


What information do you want to share with other patients living with associated PH? Both HHT and PH are diseases without cures, but there are many wonderful researchers working to change that. It can be difficult to manage having both PH and another serious associated disease, but with the help of physicians who specialize in both, it can be done, and we patients with associated diseases can live long and productive lives.