Have you ever had that feeling of being so completely overwhelmed and depressed, yet refusing to give up because, well, you’re not made that way? That’s how I am today. Sometimes I wish I was the kind of person that just ‘gives up’, ‘put a fork in me, I am done’. This whole thing we call life is just depressing.
The thing is… I can accept EB and what has brought into my life, but the sheer amount of pain it has brought to my son on a daily basis, it’s just incomprehensible to me. I don’t get it. Yes, 17.5 years later, things are getting worse, much worse, and I just don’t understand why, how, what the heck? When I say ‘My son is in hell’, it’s not a figure of speech. He is. And yet, he refuses to give up, he has hope, he enjoys life. Faced with such amazing courage, who am I to complain?
15 years ago my now husband started calling me his ‘princess’ and he was, of course, my ‘prince’. This is why our wedding was at the ‘Excalibur’ in Las Vegas (we were dressed up as King & Queen to boot) and this website is called my ‘Kingdom’, but let me tell you something, I don’t feel as if I am the Queen of anything. Yes, Nicky has called me the Queen of bandages, that about sums it up. I am the Queen of medical supplies. That’s where it ends. I overheard someone a few months ago, and I am not sure if they were talking about me or not, but they stated how upset they were about ‘someone’ being the ‘Queen’ of having a child with the worse form of EB, and who gave ‘them’ the crown etcetera etcetera. It was all very sarcastic and void of compassion or care. I was so upset over that remark, wether that was meant for me or not, I am still thinking about it. I don’t remember ever saying that Nicky has the ‘worse’ form of EB, because, well, he does not. I’ve seen worse. I’ve seen babies dying. I’ve seen children dying. I’ve seen adults with cancer, with limbs amputated, in kidney failure, liver failure… the list goes on.
Nonetheless, what Nicky deals on a daily basis, the pain is in, is pretty horrific, and how dare anyone have so little compassion? If someone is in more pain than you, are you mad at them because they get more attention? Of course not. It’s all about being human. Anytime I hear of another EB patient becoming an angel or go through an unexpected medical emergency, I don’t think of myself, I put myself in someone else’s shoes and shed a tear for them. I don’t want a crown, I am not the queen of anything. I would give my life for Nicky to be spared his pain. I speak out because Nicky wants people to know about his disorder. I don’t much words, I don’t exaggerate, I tell it like it is. We need a cure. Awareness is my goal. For better or for worse, EB is what my life revolves around. It is what it is.
And how dare anyone that is not dealing with a medical crisis (as it seems we always are), or has something manageable or painless can tell anyone, let alone me, how “We all have our mountains to climb”? I am sorry, but Nicky’s mountain seems more like twice the size of Everest. We’ve been climbing mountains for nearly two decades, and the climb is getting more and more steep. I don’t want the climb to end, because that means my son’s life is over, I want a cure to ease the pain, ease his life. That is all.
Nicky just before the surgery
So… this past week has been a roller coaster. Nicky could not swallow his own saliva, that’s how bad his throat was closed up. He was in a ton of pain. Amazingly enough (I still can’t believe it!) the insurance approved an emergency throat dilatation at Stanford, and by all accounts the surgery went well, but seeing Nicky throwing up blood from his mouth and nose in excess at the hospital was just too much for me. I felt weak in the knees. It lasted for hours. While I was at the hospital I then went up to see Nicky’s friend Garrett, who also has RDEB-HS and is two months younger. He was in the PICU following oral surgery, and to see him just lying there with his lips caked in blood, I mean, for goodness sakes. His mom later told me his tongue had fused to his lips and he ripped them off with a cotton swab. Good God!!! I gave him a few sweet kisses, because I know kids like these (even though at 17 they are not kids anymore) need so much love and suffer so incredibly, it really makes me mad when anyone diminishes their suffering. You know, when people give me those ‘We all have our crosses to bear’ platitudes. I know I speak for Lorraine (Garrett’s mom) as well in saying if only we could take away 50% of their pain, they would think their life was a vacation. Nicky and Garrett, severity wise, are pretty much par-to-par with a few differences here and there. They are both genetically classified as Hallopeau Siemens (now called severe generalized RDEB) which is sadly the worse form of RDEB. I am not saying this because I want pity or sympathy or a crown, I am just stating a fact is all.
There are many different genetic mutations of RDEB, which is why some RDEB patients, while in pain, don’t need to be wrapped much, can be independent, drive, get married, have a family etc, and some seem to be on the opposite end of the spectrum. Nicky and Garrett are at the end of this spectrum. I don’t stop Nicky from doing anything he wants to do, but his limitations seem to stop him from doing just about anything. I have to dress him, get him water, needs help going to the bathroom, can’t really walk… everything needs to be done for him. He’s also on the self-preservation side, so he won’t do something if he feels it’s going to hurt him a great deal. He’s basically an adult now, I don’t tell him what he can or can’t do, I let him make his own decisions and my job is to encourage him and help him do whatever he wants or needs to do.
Nicky is still in the midst of throwing up now, but not of blood, just mucus and he’s dealing with the pain of it all. To say EB sucks it’s a major understatement. He is trying to drink very cold drinks to numb his throat. I can’t wait until he’s no longer in pain and his throat heals completely so he can at least try to eat something, not sure if that is ever going to happen though, not soon anyway. Please keep him in your prayers!
Wound-wise he’s not doing too bad actually! I pad him excessively so he does not literally scratch himself to death. Itching is a major component and the main reason for all his wounds, so to keep him padded and dosed up with anti-itch medication seem to do the trick. The less wounds the less infections and possibility of cancer etc.
Thank you for your care, compassion and understanding.
Much Love,
