As a nurse, it's been my experience that people in general tend to overestimate how long they will live. Here in the U.S. average lifespan for women is about 80 and for men 76. That means midlife for women is around 40 and for men 38. People don't tend to feel "older" when they're in their 30s and 40s though! However, in the hospital I take care of people in their 50s and 60s every day who are experiencing life- altering disease and disability. In fact, most people in this country don't make it all the way to retirement age of 65, and the leading cause of early retirement is medical issues. (I always tell people, if you want to do something don't wait...just go for it!) That's an overall view of the general population, but people living with a chronic illness tend to have reduced lifespan. Elevated phe levels over a lifetime have a cumulative negative effect, unfortunately. Here in NY we started testing for PKU in 1965, so that would mean if they stayed continously on treatment, our oldest PKU patients would be coming into their 60s now. There isn't any tracking or comprehensive data on these patients though, so we don't know how they are faring health-wise. We also don't know if they are able to access meds like Kuvan or Saphience. I have been appealing to my insurance for over a year now and so far have not been able to trial either drug, and I have good insurance. Saphience appears to have an additional method of action that Kuvan lacks, and so I hope it will become more widely available in the future.
Thank you for your insight and dedication. Sephience did not work for me so I went back to my LNAA that I have been in for 20 odd years. This allows me more protein per day and gives me the benefits you talk about, but still, pkuer’ers in old age and the effects of diet, levels and medicines ( formulas and pharmaceuticals) still remains a mystery for healthcare workers and patients alike. I guided my dad through a diet for kidney disease ( since I understood nutrition better than him) and saw how incompatible the pku diet is with the kidney diet. So yes, there is a lot of work to be done still- just in terms of co-morbidities that can be more harmful than pku itself. I appriciate your writings and discussion of these because being an “older pku’er” is often left aside.
This post is right on time. I'll actually be speaking on a panel about the topic of PKU and aging in July at the NPKUA Conference in Chicago. I've begun my own, slightly more audacious, experiments with managing the effects of PKU. I've written about it on LinkedIn and plan on sharing it here very soon.
As a nurse, it's been my experience that people in general tend to overestimate how long they will live. Here in the U.S. average lifespan for women is about 80 and for men 76. That means midlife for women is around 40 and for men 38. People don't tend to feel "older" when they're in their 30s and 40s though! However, in the hospital I take care of people in their 50s and 60s every day who are experiencing life- altering disease and disability. In fact, most people in this country don't make it all the way to retirement age of 65, and the leading cause of early retirement is medical issues. (I always tell people, if you want to do something don't wait...just go for it!) That's an overall view of the general population, but people living with a chronic illness tend to have reduced lifespan. Elevated phe levels over a lifetime have a cumulative negative effect, unfortunately. Here in NY we started testing for PKU in 1965, so that would mean if they stayed continously on treatment, our oldest PKU patients would be coming into their 60s now. There isn't any tracking or comprehensive data on these patients though, so we don't know how they are faring health-wise. We also don't know if they are able to access meds like Kuvan or Saphience. I have been appealing to my insurance for over a year now and so far have not been able to trial either drug, and I have good insurance. Saphience appears to have an additional method of action that Kuvan lacks, and so I hope it will become more widely available in the future.
Thank you so much for sharing this. This shows how critical it is to make these drugs more accessible.
Thank you for your insight and dedication. Sephience did not work for me so I went back to my LNAA that I have been in for 20 odd years. This allows me more protein per day and gives me the benefits you talk about, but still, pkuer’ers in old age and the effects of diet, levels and medicines ( formulas and pharmaceuticals) still remains a mystery for healthcare workers and patients alike. I guided my dad through a diet for kidney disease ( since I understood nutrition better than him) and saw how incompatible the pku diet is with the kidney diet. So yes, there is a lot of work to be done still- just in terms of co-morbidities that can be more harmful than pku itself. I appriciate your writings and discussion of these because being an “older pku’er” is often left aside.
Thank you very much for sharing this. I’m glad you were still able to use a treatment that helps you even if Sephience did not work.
I really hope the global conversation shifts a bit more towards these issues for older PKUers.
This post is right on time. I'll actually be speaking on a panel about the topic of PKU and aging in July at the NPKUA Conference in Chicago. I've begun my own, slightly more audacious, experiments with managing the effects of PKU. I've written about it on LinkedIn and plan on sharing it here very soon.
Thank you once again for an insightful read.
Wow, it’s great! I’ll be very curious to learn more. 😀