I must confess that often I avoid some of the more personal aspects of having Hermansky-Pudlak Syndrome on this blog to protect some of the people around me. But, at the same time, it’s important to tell the story, the whole story.
Over the weekend was one of those experiences that left me dumbfounded, and about which I’m not entirely sure I should blog. But then I thought of my old friend V. who passed away from HPS several years ago, and I decided this is the sort of story that should be shared, albeit carefully.
We are planning a large family get together this summer to honor my grandmother who will turn 90 years old on July 11th. In the course of conversation with a particularly close relative, I relayed some of the upheavals at work and my uncertainty about how they all might turn out.
Most likely in an honest, yet poor, attempt to be interested and caring, my relative said, “Well, the thing I worry about is what this could do to your saving for retirement. I’m worried you won’t have enough money to retire.”
My mouth dropped open and hit the ground. This person has been told repeatedly about my diagnosis, and yet the comment indicated that they clearly don’t get it.
I’m worried about a lot of things right now, but retirement is so far down the list that I don’t think it even scores in the top 20.
The conversation was already starting to get heated, and so I managed not to blurt out what immediately cane to mind for me – that right now the chances I’ll make it to 65 are slim to none. Yes, a handful of people with the pulmonary fibrosis of HPS have managed to cross the 65 line, but their fibrosis started later in life. Mine has already arrived.
I got off the phone feeling intensely angry. I wanted to yell at this person, but I know it would achieve nothing. It isn’t a lack of information, or even a lack of understanding that is the problem – the problem is this person emotionally can’t cope with the reality that is my life. They can’t hack it, so they choose not to deal with it.
I felt angry that this person seems to have no appreciation for how lucky I am right now. This coming August will be five years since my first trip to NIH where I was told I probably had five to seven years. I think I’m going to hit that five-year mark with lung functions in the normal range, or at least close to it. I feel extremely blessed.
I also think there’s a chance I might be around another decade. I’ve seen other HPS’ers with pulmonary fibrosis come off of oxygen on Pirfenidone and be around years longer than could have been expected. If I’m not on placebo, I’ve started the drug with much less fibrosis than they had, and thus I expect my result could easily be as good as theirs.
And who knows. That could buy me enough time to take advantage of the next medical advancement, whatever it might be.
Yes, I might just be riding the cusp of medical advancements that will knock the pulmonary fibrosis of HPS down from a killer to a chronic illness – I might be in that first generation that is a PF ‘survivor.” But, if I do manage to ride that wave, I suspect it will involve a lot of medical intervention, a lot of large bills, interrupt my career several times, and I’ll wash up on the beach of old age pretty much penniless.
It’s a problem I’d like to have one day.
As I stormed around my house, ranting out loud to no one in particular, I thought of V. I remember briefly talking to one of his family members after his death. She told me the family had no idea how sick V. was or that he could die from this disease.
I didn’t tell her what was on my mind. V was already dead. He left behind many open familial open wounds and I didn’t want to throw salt in them. He wouldn’t have wanted that.
But I knew that V. had talked about his situation with his family often. He was seeking a lung transplant and he needed their support to be a good candidate. He was on oxygen 24/7. It wasn’t that the family didn’t know. They didn’t want to know. I can easily envision my story will be pretty much the same.
Over the weekend was one of those experiences that left me dumbfounded, and about which I’m not entirely sure I should blog. But then I thought of my old friend V. who passed away from HPS several years ago, and I decided this is the sort of story that should be shared, albeit carefully.
We are planning a large family get together this summer to honor my grandmother who will turn 90 years old on July 11th. In the course of conversation with a particularly close relative, I relayed some of the upheavals at work and my uncertainty about how they all might turn out.
Most likely in an honest, yet poor, attempt to be interested and caring, my relative said, “Well, the thing I worry about is what this could do to your saving for retirement. I’m worried you won’t have enough money to retire.”
My mouth dropped open and hit the ground. This person has been told repeatedly about my diagnosis, and yet the comment indicated that they clearly don’t get it.
I’m worried about a lot of things right now, but retirement is so far down the list that I don’t think it even scores in the top 20.
The conversation was already starting to get heated, and so I managed not to blurt out what immediately cane to mind for me – that right now the chances I’ll make it to 65 are slim to none. Yes, a handful of people with the pulmonary fibrosis of HPS have managed to cross the 65 line, but their fibrosis started later in life. Mine has already arrived.
I got off the phone feeling intensely angry. I wanted to yell at this person, but I know it would achieve nothing. It isn’t a lack of information, or even a lack of understanding that is the problem – the problem is this person emotionally can’t cope with the reality that is my life. They can’t hack it, so they choose not to deal with it.
I felt angry that this person seems to have no appreciation for how lucky I am right now. This coming August will be five years since my first trip to NIH where I was told I probably had five to seven years. I think I’m going to hit that five-year mark with lung functions in the normal range, or at least close to it. I feel extremely blessed.
I also think there’s a chance I might be around another decade. I’ve seen other HPS’ers with pulmonary fibrosis come off of oxygen on Pirfenidone and be around years longer than could have been expected. If I’m not on placebo, I’ve started the drug with much less fibrosis than they had, and thus I expect my result could easily be as good as theirs.
And who knows. That could buy me enough time to take advantage of the next medical advancement, whatever it might be.
Yes, I might just be riding the cusp of medical advancements that will knock the pulmonary fibrosis of HPS down from a killer to a chronic illness – I might be in that first generation that is a PF ‘survivor.” But, if I do manage to ride that wave, I suspect it will involve a lot of medical intervention, a lot of large bills, interrupt my career several times, and I’ll wash up on the beach of old age pretty much penniless.
It’s a problem I’d like to have one day.
As I stormed around my house, ranting out loud to no one in particular, I thought of V. I remember briefly talking to one of his family members after his death. She told me the family had no idea how sick V. was or that he could die from this disease.
I didn’t tell her what was on my mind. V was already dead. He left behind many open familial open wounds and I didn’t want to throw salt in them. He wouldn’t have wanted that.
But I knew that V. had talked about his situation with his family often. He was seeking a lung transplant and he needed their support to be a good candidate. He was on oxygen 24/7. It wasn’t that the family didn’t know. They didn’t want to know. I can easily envision my story will be pretty much the same.
Comments
As the mother of a 7 year old who had a double lung transplant at 11 weeks of age, I understand your frustration with those who do not see the fragility of your life. It is very unlikely that my daughter will make it to adolescence, but I still have people in my family who want to talk about where she will go to college and her as an adult. All I can say to you is, hope is a human condition, and I've learned to receive all such comments as their expression that they want us to beat the odds. So, try to think positively, even though it is really hard. I'm sure many people care about you, and most of them are not ready to accept their own mortality, much less yours.