!/images/22.jpg (Tiffany House)!
I’m proud to bring you the second interview for Fighters. Tiffany is also one of the Pompe patients that I consider a close friend. Even though we haven’t met in person I feel a closesness with her. It’s probably because of all the patients I know she’s one patients close to my age. So our state of mind is closer.
She’s a strong person who’s been at the forefront of the Fight against Pompe. She and her family have done so much for Pompe. They started the “AMDA”:http://www.amda-pompe.org which provided a lot of support for Pompe patients.
So please read on and enjoy our conversation.
*Juan:* Please tell us a little something about yourself, a brief background.
_Tiffany:_ Well, I am the oldest of three children at 23. I was diagnosed with Pompe the week before my 12th birthday, and as of right now I have been receiving ERT for over 7 years. My parents also formed the AMDA in the year after I was diagnosed to aid in finding a treatment for Pompe disease.
*Juan:* How did you feel when you were diagnosed?
_Tiffany:_ To be completely honest, I don’t really remember a lot about being told I have Pompe. I remember traveling to Minnesota with my mother, having a lot of tests done at the Mayo Clinic (including a very painful muscle biopsy), and then having my life be different. I had to cut carbs out of my diet, eat a lot more protein. I started using a bi-pap. At first, I felt the same, just, all of a sudden I was told I had this disease. I don’t ever remember being in denial, but I do know that it was hard to adjust. For instance, I never told any of the kids I went to school with that I had been diagnosed with a rare disease, and when I first started using a bi-pap I didn’t even want my brother and sister to see me with it on. I didn’t want people to suddenly look at me differently-I was still the same person. As time has passed, I have become a lot more comfortable talking about the disease, and my limitations, and letting people see me with the bi-pap, but at first it was a very private thing that I didn’t know how to talk about.
*Juan:* How was it like growing up with Pompe? Having to gradually lose your physical abilities?
_Tiffany:_ It was hard. I declined very fast in the year following my diagnosis, and by Feb-March 1996 I was no longer able to attend school. At that point, my life “shrunk” to include only my family, some close family friends, working out with a personal trainer, and receiving schooling at home by two home school teachers.
Looking on the bright side, I made two lifelong friends during this time (my math tutor, Tiffany, and my trainer, Jordan) but it was also a very hard time. I was starting to feel weaker and weaker, I was developing very severe scoliosis and the activities outside of the home that I could attend were getting fewer.
By the time I left to go the Netherlands to partake in the clinical trials, I was barely able to walk, my breathing was down around 15-20% (I think), and I was only able to be sitting up for about 4-5 hours a day.
*Juan:* So when you started with the clinical trials, how did you feel? What was going through your mind?
_Tiffany:_ Well, of course I was hoping that it would work. I think I had some ridiculous idea like “I am going to have so much energy at first because I will finally be using all of those stored up carbs.” That didn’t happen, and at first I didn’t notice any change.
See, I was on a low dose at first, and it was not until about May/June 2000 (my first infusion was in June 1999) that I started to notice that I was feeling a little better. I had a little more stamina. However, at the same time my scoliosis was getting a lot worse too, and on June 30, 2000 I had the first of two operations to correct it.
But back to how I felt about starting ERT, I guess I never really thought about what would happen if it didn’t work. See, despite the fact that I knew my chances of living past the age of 20 prior to treatment weren’t good, I never really thought I was going to die. I knew I could but I never thought I would, if that makes sense. I think a lot of these feelings can be traced to how hard my parents worked to find a treatment for me.
*Juan:* That makes sense. The dying part I mean. I feel the same way.
_Tiffany:_ When my parents were told that there was nothing out there (treatment wise), they researched until they had the names of several researchers who were working on Pompe, then they started calling them up and asking them what they knew. Then they organized the first scientific conference for researchers working in the field of Pompe.
I have been told by some of those individuals that it was the first time they had actually met each other, and I know that they started collaborating after that.
Anyway, my point is, my parents were working so hard to try and “save” me, that I didn’t really feel like I had to worry. They hoped and believed that ERT would work, so I started the trial without really being scared that anything would go wrong.
*Juan:* How many years have you been on infusion?
_Tiffany:_ 7 years. But in that time I have been on 4 different forms. I started on transgenic, then one of the Duke forms, then there was one called the B/I form, and then Myozyme.
*Juan:* After 7 years and 4 different forms of ERTs, how do you feel? What’s your outlook like?
_Tiffany:_ I feel good. Over the last couple of years I have noticed significant improvements in my health and strength, and I am continuing to improve all the time. For me, these improvements mainly come in the form of an increase in my endurance level, less stomach issues, and the ability to do more for myself on a day-to-day basis.
As for my outlook on life, it is positive. I received a BA in English in May 2005, and I am currently working on my masters. My plan is to complete the graduate program by May 2007, and begin Law School in August 07.
_Tiffany:_ As for other aspects of my life, I have great friends and a truly awesome family.
For me, Pompe is really just one facet of my life, and I am not going to let it dictate what I do with the rest of my life. I will always have to make some accommodations in my plans because of it (i.e. bi-weekly infusions will have to be worked around), but I have always refused to be defined simply by the fact that I have this disease. Especially now that ERT is a reality, we don’t have to be limited in our dreams for the future.
*Juan:* What’s the best and biggest lesson you’ve learned dealing with Pompe?
_Tiffany:_ That spending a lot of time worrying/being upset about “what might have been” is pointless, and it is unproductive. Yes, I have Pompe, and yes it has changed the course my life would have taken if I didn’t but all I can work with is what is, not what could have been. What is, is that I am alive, I am doing well, and I have a future in front of me that is waiting for me to take it.
*Juan:* Is there anything you’d like to tell other Pompe patients, family or people in general? Parting words, etc..
_Tiffany:_ I guess I would reiterate that Pompe does not need to define who we are. It is important that we accept that we have it, accept the reality of life with Pompe, but that we also need to remember that we are not only Pompe patients and that we have a brighter future in front of us now that ERT is available. I would also like to say that, from my own experiences, reaching out to friends and family, and finding a way to have open discussions about Pompe makes life a lot better.
Earlier I stated that when I was first diagnosed I never really talked about having Pompe. Over the last two years one of the people I have known my entire life has become a great friend because she reached out to me, asked me some questions about Pompe, and made it easier for me to talk about what I am dealing with. Together with her boyfriend, and some of their other friends, they have helped me to be upfront with people about the help that I need, and she has made me feel comfortable in talking about the health/help issues that I would have previously only discussed with my immediate family.
In short, they have made Pompe an even smaller part of my life because it is something that they want to understand, something that they help me with, not something that is like an elephant in the room: seen by all, but talked about by none.
Maybe smaller is the wrong word; maybe I should say that they have made it less alienating.
Ok, I think that is all I want to say. Oh wait.
I also want to say that talking to you, and other Pompe patients has also been extremely helpful to me because I feel that through my discussions I learn new ways to cope with the disease, as well as they leave me feeling like some one else knows exactly how I am feeling about the trials that can arise in day to day life when you have Pompe.