Hello my wonderful people.
Recently I have been getting a lot of questions as to what exactly is 'wrong' with me, and I realize that not everyone who reads this blog knows me personally the way my friends do, who know everything there possibly is to know about someone b/c they just love me that much. At least that's what I tell myself.
So let's begin. You may want to grab a snack and go to the bathroom before beginning this b/c it's looooooooooooong.
In the wee hours of August 23, 1985 (3:23am, don't you forget it!) the bestest baby to ever grace this planet with their exceptional presence, moi, entered this world. I don't know how much i weight or how long I was, but none the less, there I was. 2 days after I came home, my nana noticed I wasn't breathing correctly. Eventually, I was taken to the hospital where it was discovered I had pneumonia. As time went on they discovered it was a bit more than that, and I was air lifted to McMaster University Hospital in Hamilton where I'm assuming they had better facilities to treat me. There, they discovered I had an immune system problem, and I was whisked away in the dark of night to Sick Kids Hospital where I remained until February 1986.
Turns out, little baby moi was born with an extremely rare condition called ADA SCID - Adenosine Deaminase Severe Combined Immunodeficiency. Yes, that's exactly what it's called. Don't feel bad if you can't pronounce the unabreviated name of ADA b/c even I can't. It's so rare and dangerous (apparently) that I sit here, at almost 22 years old, the oldest living person in Canada (so i've been told, and to anyone who wants to snatch the title away from me risks losing a hand - or both) with this disease. In October 1985 I recieved a bone marrow transplant (tx) from my mum, who was only a partial match. I've been told that had I never gotten that bone marror tx, even with the treatment I get today, I wouldn't be able to go outside, so, THANX MUM! To this day my mum bares that 7 inch scar across the back of her ass. Her battle scar to save my life:)
So my mum was a partial match? What on earth could that mean? Well, it means that our blood types aren't the same, but close enough to try a tx. It partially worked; enough so that after agonizing months on my parents behalf, they could finally take me home in Feb '86. I have the homecoming pic and will try to post it if i figure out how.
I didn't really go to kindergarten b/c if I wasn't sick with something, someone else was. My sister was generous enough to bring home the Chicken Pox once and gave it to me, and when i went into the hospital with it (where no one else had it) i was generous enough to give it to the entire floor:) People with SCID are told that if you get the Chicken Pox that's it - you're done, see you later. I survived! For some time when people came to visit our house, they had to gown up b/c they could infect me with any little germ they had.
But alas, around 1990-91 ( that's what it felt like to me) a breakthrough was made ( I guess) and I began getting infusions of something called Gamma which is also known as IVIG. This, people, is the immune system part of the blood. So when people donate blood, I get the immune system part. GENIUS! Since then I have been getting Gamma every month and am doing wonderful! I rarely get sick, I am so lucky!!!! But you ask, if you're not sick, why in the hell have you been told that you need a lung tx?
B/c I have a low immunity, I am more suseptable to weird infections than others. The immune system and your lungs go hand in hand, much like your liver and pancreas, much like pop and chips, much like soap in a shower. They work together. If one is lacking, the other will most likely be affected too. If you recall, I had pneumonia at 2 days old. Pneumonia can wreck havoc to the lungs, especially in a little SCIDlet like me. I had a lot of chest infections as a kid, and that's when scarring in my lungs began. I always had to go in for PFTs (Pulmonary Functions Tests) to measure my lung functions and all that fun stuff. I had asthma and took medication for it, but it wasn't horrible asthma or anything.
Then, when I was 17, an uninvited guest by the name of H-Flu took up residence inside of my lungs and made me very sick, very fast. This was in 2002. I got sick right at the beginning of grade 11 and looking back, I went downhill very fast. Suddenly, little things like walking up the stairs left me breathless, and I developed a wet cough and would cough up green little gems. It happened so slowly but also so fast, (over a course of 6 months) that i didn't take notice until right before I got admitted into the hospital for it. I remember I sat with my mum in a little room when I found out they were admitting me. I hung my head and began to cry to my mum. "I'm sorry" i said. "Oh hunny, why?" and i cried and said, "I never meant for this to happen." I felt like it was my fault for not noticing sooner.
My lung function was down to the 30's (%) and they couldn't figure out why. I was in for 2 weeks and I think i fought with them every single day to let me out. I had a bronchoscopy (where they stick a tube that's got a camera on it) into my lungs to see how bad they were. A Bronch also allows them to take tissue samples and sample of the infection as well. I remember my doctor telling me it was "completely disgusting" inside of my lungs, and that I was growing a lot of other infections too. This is also when the biggest pain in the ass entered my life: the PEP Mask, aka PHYSIOTHERAPY.
The PEP Mask is a device that much resembles a bizarre sex toy to be perfectly honest. It's got a mask at one end, and a pressure valve on the other. You breathe into it and it creates 'positive' pressure in your lungs and allows you to cough up everything that's in your lungs. I remember one nurse trying to sympathize with me by telling me she had pneumonia once and had to do physio too, but only for a year. I wanted to strangle her and say, "That's nice, but I'll be doing this forever so shut up."
After much pleading they released me back into the world on March 9th, 2003. Ironically, this day the first case of SARS was reported. Luckily i fled the city and couldn't be considered a culprit, what with my fancy schmanzy new lung problem and all.
So what is it you've got, you ask? Well, many people have asked me, and still ask me if it's CF (Cystic Fibrosis); sorry to burst your bubble but it's not. HOWEVER, it is something so similar to CF that my current chest doctor (who is a CF specialist) told me you can line up every single similarity with CF and what I have and you would think they were the same thing. The difference between CF and what i have is that CF is genetic and affects more than the lungs (it affects the lungs, sinuses, digestive, and reproductive). CF causes mucus to excrete from almost any organ and clogs them up. My chest condition is called Bronchiectasis - yup, that's what it's called, and go ahead and laugh at the name b/c i'm pretty sure i laughed the first time I heard it too. In my case it is not genetic but caused as a result of years of infections. Bronchiectasis is the chronic inflammation of the bronchi and airways in your lungs, making it, the same way as CF, produce mucus all the time. This mucus is thick and sticky and extremely hard to evacuate from ur lungs. What happens when you can't get it out? Well, you get sick, and you develop bizarre infections that are hard to treat. These infections don't just come and then leave and never come back. They come back, and come back, and sometimes they never go away. The combination of the constant production of mucus as well as the constant infections damages the lungs in the form of scar tissue, which if you don't know, cannot be reversed. The scar tissue then blocks your airways and air sacs in your lungs and you lose lung function and will never get it back. In CF however, I must clarify, it is possibly to regain function. In bronchiectasis, you can't.
When they sent me home I got sick again a month later (i was told to expect this. I went on antibiotics and all was well, until Jan-Feb 2004 I got sick again, and idiot me, took the medication the wrong way and ended up resistant to the drug Biaxin. I was scheduled to go on a grad trip out east and guess who again, got sick? ME! ME! ME!. My doctor decided to put me on prednisone and a new antibiotic(abx) called Avalox. Avalox, i need to formally thank you and tell you I love you. If it was possibly to hug you, I would. Avalox took care of the infection for me, and prednisone got rid of the swelling in my airways and I felt back to my old self! YAY! I was able to go on my trip! The prednisone made me gain 10lbs on that 6 day trip and gave me terrible moon face. Needless to say, I don't look at any trip pictures after the first couple of days.
Since 2004 I have remained infection free. YAY! However, bronchiectasis is a progressive disease and unfortunately for me, the damage was done long ago. My current lung funtion is 27% (the highest it got after i got diagnosed with this was in the 40's (%) i believe). The mucus production is constant and I need a lung transplant.
Do not, for a second, feel bad for me! I did not write this for sympathy, or for pity, or for anything along those lines. I wrote this to share my story with others who are going through something similar (be it SCID, Bronchiectasis, CF, what have you), and to let them know that they are not the only one going through shit. Yes, it sucks, yes it's not fun, yes I do cry over it, but i could have it way worse than I do. You need to find humour in these things. I consider myself extremely fortunate to have the option of recieving a lung tx, b/c not many people are. Most people with bronchiectasis never get as bad as I am, and they have to live their life feeling really crappy. Would i want that? No. Do i want the oppourtunity to start anew and give back to those who have given to me? YES! More than anything.
I think my first start was beginning this blog and I am very happy that i did:) I hope you have enjoyed it thus far and aren't completely bored. Leave comments b/c i love them!!