Updates on Nicky and What-Nots

IMG_4356skI wanted to share this picture of Nicky’s right hand a year post surgery. It’s doing fantastic! We do wrap the hand religiously and use the splint every night to keep the opening between the thumb and index finger intact, and while we lost a bit on the pinky, it is still ‘straight’, which means it does not pull the wrist inside, which is really great regardless. Even if it gets webbed again, as long as it stays straight Nicky does not mind, it’s the thumb which is most important for us to keep open.

I wanted to share this pic of the same hand 13 years ago (below), 2002 to be exact, just before the surgery he had back then. He was almost 6 when he had that surgery, and it was the last time he had surgery on that hand until last year. Without the wrapping and surgeries, as you can tell, he would have no hand. I know for some patients that is not a big deal, but it is with Nicky, so, as always, I oblige to his wants and needs. They are his hands after-all.MVC-044F_sk

Nicky’s throat has completely healed and with that behind us we can now concentrate on his school and our mundane things such as infusions etc. I do hope he can graduate high school by next June, we’ll see. I did mention earlier that Nicky was accepted into the trials at Stanford, which he will participate once he turns 18 in late November. I have decided not to divulge any details as not to hurt the trials in any way. They did not forbid me to talk about it per se, and they can’t, but they did mention it could damage the research, so if you hear me talk about it, I will try to remain extremely vague on the subject until the official research papers with the results are published. I will keep a detailed diary on my own so I won’t forget anything. Fingers crossed  it will all work out for Nicky!

Thank you as always for the kind words, God knows we need them!

Love & Light,

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New CaringBridge Blog & EB:Disease or Disorder?

logo-caringbridge-stacked-facebookA quick note!  I just updated Nicky’s blog at CaringBridge… I talk about his throat surgery, and lots of good news! Here’s the link! http://www.caringbridge.org/visit/nickyz/journal/

On another note, I’ve been wanting to repost this BLOG I wrote for the EB Info World website about a year ago because it’s still and always will be relevant.

Many hugs and Love <3

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My son Nicky was merely a week old when a good friend, researching the condition, told me emphatically that EB was definitely a Disorder, not a Disease. It may come to a surprise to many when my inner response to that was like ‘whatever’. “Thank You” I replied to her, and never thought about it again.
It truly made zero difference to me if EB was a Disorder, a Disease or, for that matter, a Syndrome, an Illness, a Condition, an Ailment… the only thing that mattered to me was to make sure people at least spelled “Epidermolysis Bullosa” correctly, since I cringe every time I see the folder with EB spelled out wrong by the Doctor that diagnosed Nicky. Can you believe that? Ugh.

There are people however, whose classification matters a great deal. I remember clearly a patient being really upset when the late Princess Diana referred to EB as a disease, or another one crying telling me her life was hard enough without people thinking she was diseased.

Popular belief is that you cannot catch a disorder but you can catch a disease. A disorder is inherited, a disease can be caught by anyone who is not immune to it.
The truth is that, according to my extensive research, contrary to this popular belief, neither disorder nor disease imply particular underlying causes. One term is not more medically accepted than the other. In particular, disease does not require infection (e.g. cardiovascular disease) and disorders can be caused by infection (epilepsy), and doctors use both freely.

Very much like EB, Krabbe Disease, Tay-Sachs Disease, Wilson’s Disease, Huntinton’s Disease and even some forms of Parkinson’s Disease among others are ALL genetically inherited conditions, inherited the same way as well, either recessevely or dominantly.

Confused yet? Let me confuse you even more. Wikipedia describes Epidermolysis Bullosa as a connective tissue disease, WebMD calls it a genetic skin disorder, NIH calls it simply a disease, Medscape a group of inherited bullous disorders, the EBMRF calls it a group of diseases, Stanford calls it a genetic skin disorder, while Cincinnati calls it an inherited disease. Debra (US) calls it a genetic skin disorder, yet has this really great campaign (get this shirt!!!) where it calls it a disease. Many other websites, including other Debra websites like Debra UK & International call it simply a ‘condition’.

So, what does it all mean? Perhaps Wikipedia answered this question for me. According to them, it seems as if the word ‘Disease‘ is more of a broad, inclusive, generic term, while disorder is more specific and newer (and when you get to the Disorder page there, it links right back to disease): “In humans, “disease” is often used more broadly to refer to any condition that causes pain, dysfunction, distress, social problems or death to the person afflicted, or similar problems for those in contact with the person. In this broader sense, it sometimes includes injuries, disabilities, disorders, syndromes, infections, isolated symptoms, deviant behaviors and atypical variations of structure and function, while in other contexts and for other purposes these may be considered distinguishable categories. Diseases usually affect people not only physically, but also emotionally, as contracting and living with many diseases can alter one’s perspective on life, and their personality. There are four main types of disease: pathogenic disease, deficiency disease, hereditary disease, and physiological disease. Diseases can also be classified as communicable and non-communicable disease.

You say disorder, I say disease. Tomato, tomahto. Whatever you call it, at least one thing’s for sure—they both share the same goal, which is to prevent it, manage it, treat it and/or find a cure.

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Legoland Love & Sad Reflections…

IMG_3897_skThis past week we were fortunate enough to visit Legoland-a friend who did a fundraiser for us bought us the tickets, of which I will forever be grateful. I wish I could convey in words how much fun Nicky had in this trip and how nice everyone was to us everywhere we went.

For starters, I must say that Nicky has gotten quite hurt in the past when doing rides so he always refused to do the ‘crazy’ ones (as he called them) at Disneyland & Universal Studios. By crazy ones I must specifically mention ‘Space Mountain’ & ‘Matterhorn’ at Disneyland and the ‘Mummy’ ride at Universal Studios. He wants and tries all he can, but sometimes the hardest part is not the ride itself but getting INTO the car/boat or whatever that may be. This time, however, I saw a different Nicky. The rides at Legoland are milder since the park itself is more geared toward young children and not adults (as, say, Magic Mountain is) so he flat out told me he didn’t care if he was going to get hurt, he was going to have FUN!

So… I brought a soft blanket for him to sit on and told him I would hold him ‘good’ so he would not bump too much here and there. A couple of rides were pretty crazy, the ‘Dragon’ and the ‘Project X’, but all the attendants let us through without waiting and held up the ride for us so Nicky could get in. It takes a little while for Nicky to get in and out and I was thankful the attendants did not rush Nicky and told us to take our time. They let Nicky take the power chair up to the ride itself and then had the chair ready for us when we got out.

We also had quite a few people approach us saying nice things to us, such as ‘I’ll be praying for you’ or hold the door open and such. One kid, about 12 or 13, walked up to Nicky with his little sister and asked him how he got his injuries. He and Nicky had a nice conversation, Nicky told him about EB and the kid told him he felt bad. Greg and I were impressed at this young man. Most people just stared, he had the courage of coming up to him and ask. Mind blown.

My husband walks slow with his brace on his leg, so I am sure we looked pretty noticeable out there. Many times he would put his hand over Nicky’s chair to get some help. It was hard for my husband to get in many of the rides as well but he was a trooper and never complained once. He told me many times that if Nicky does not complain, he has no reason to. My husband is a man of few words anyway, I normally have to ask him how his foot is feeling, or if he’s any pain, as he’s not one to volunteer information. After the first day he showed me his foot and when I asked him if he was in any pain he said ‘atrocious’. I would have never known. I hugged and kissed him. After one slow ride Nicky got a nasty blister on one of his feet (which we later looked at the hotel and it was a nasty blood blister which covered a third of the bottom of his foot) and told me he just needed his morphine and wanted to keep having FUN. So, later when we did the water splash ride and was laughing of how much he loved it I just couldn’t help but kissing him all over. I mean, this kid is an angel. All of that he endures on a daily basis and he’s the sweetest, gentlest, kindest guy everyone hopes to meet. Later in the Gift Shop he followed me around everywhere I went. It’s like I have a little buddy I can talk to at anytime because he’s always ‘there’. He’s my angel, plain and simple.

I try to plan something ‘fun’ every year, and it’s true I’ve had help financially from my ex-husband (Nicky’s dad) and various charities and organizations, but to be honest, I don’t know what else to do. His life is so limited. He can’t do anything. I have to help him with the most basic things that Connor has been able to do himself since he was 3 or younger. There are days that I honestly feel I can’t see him suffer as he does one more day. But what is the alternative? Nicky, despite it all, loves living. As long as he wants to hang in there, so will I. The form of EB he has is progressive and degenerative. That means he gets weaker, day by day. Each day, he needs more and more help, more support. So many children with the same form of EB like Nicky have already passed away. Soooo many! I don’t know how much longer we’ll have him for. He could die tomorrow, or, if a cure/treatment is found he could hang on for decades. The future is a scary, unknown place, so I try to plan ‘fun’ for the future so I don’t dread time clicking away but instead I can look forward to something.

Daily life for Nicky is daunting. It’s hard for me to put it into words. Not many people understands how serious it will be if he falls and breaks a bone, or that a simple infection could result in death for him. Thank goodness for Connor and a couple of online friends, because he has no friends that come over and play, he never had them. Have you ever thought about what it would feel like to see your child losing all abilities, and start relying on you for everything? Does your teenager need you to wipe his bum or put a diaper on him at night? Probably not.   Imagine hearing your child talking about what sort of car they will buy when they grow up, knowing they will most likely never get their drivers license. While we were waiting at the restaurant for our table Nicky was curious about how to drive, so I taught him and told him everything he needed to know. And it killed me inside. I wish and hope he can someday, but reality is staring at me smack in the face. Without a cure, he will die, sooner rather than later, but first I have to watch his body completely waste away. It makes my heart hurt just thinking about it. This is why I try as hard as I can to pack an entire lifetime into the time he has left. I can’t just let him sit around while I go to work to ensure financial security in the future. Not when there is possibly no future. I have to do it now. I ask Nicky where he wants to go next year so I can try to start saving money for it, so that he will have lived an entire lifetime by the time he dies. Everyone should be able to die with more memories than dreams. That’s what I am trying to do.

Here’s the crazy thing – some people, even family, have actually been jealous of those few things we have experienced. I’ll be the first to tell you I would give it all up, every single thing, for a cure. Most people don’t realize how blessed they are. They look to their problems instead of their luck. In our life, everything is a challenge to overcome, everything has to have a silver lining. I am lucky Nicky was not born with an even more severe form of EB, or other conditions that kill babies. I have to be thankful and hopeful and steer away from drama while tackling life’s challenges. Everywhere we go has to be wheelchair friendly or we can’t do it. I opted to get a power-chair that breaks down in pieces and fits in the trunk of my Prius because a wheelchair-accessible vehicle is prohibitively expensive. And I drive a Prius because I use a lot of gasoline taking Nicky around to various Doctors and Hospitals. Visiting friends and family or going to parties and other outings is extremely hard. Nicky can’t climb stairs and places need to be wheelchair friendly. Unless family come to us, we won’t see much of them, and we haven’t. We have to fight with ignorant people who think disabled parking is for anyone. Toileting in public is also an issue. Usually there is only one disabled toilet, and it’s often taken, so we have to get Nicky to squeeze in those normal size ones, a challenge in itself since then the door does not close. I would happily give up all the conveniences if it meant my son no longer had EB. But he does. And that isn’t going to change.

We do have another outing planned for next month-since Connor is such a universe fan, we plan to go to the Griffith Observatory and the California Science Center to see the Space Shuttle, both are local so it won’t be too much trouble, hopefully. I finally heard there ‘may’ be a class reunion happening in October and, well, we’ll see. It’s in New Mexico, two states away and a two day drive. When the dates are set in stone I will check and see if I can manage it. Not sure if I can.

So, at the moment my plan for next year is a 3-4 day cruise to Vancouver from San Francisco. Nicky wants to do another Alaska cruise, but since that’s just out of my price range, I told him Vancouver is as north as I can afford, 3-4 days is the most I can also afford and departing from SF means we can drive there and don’t need to fly nor need a hotel. It’s the cheapest way for me to make sure he gets his wish. Connor loved the cruise, so he’s all for it. If anyone has any ‘insights’ on deals for the Princess Cruise line that does this sort of trip, please let me know!! I know I need to book ASAP, I will have the money for it in a few months (by Xmas at the latest).

Then for 2016 I promised him Italy for graduation. I promised him a trip to see his grandparents for graduation a LONG time ago, so I must deliver. I mean, both Nicky and Connor want to go to Italy now, but I just cannot afford it. I need these 2 years to save enough, and hopefully I can save enough because that ain’t cheap, ha ha. I am always looking for ways to skimp here and there so I will keep clipping my coupons and shop at thrift stores in the meantime!

I want to thank everyone for the kind words-they mean a lot to me. And also a BIG thanks to Juan Lewis for the tickets to Legoland!

Love and Light

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A Day In The Life

The other night it took me over one hour just to put Nicky to sleep. Not that it was a stretch, as it usually takes me at least half an hour to 45 minutes. There was the oral care, the g-tube preparation, the bed preparation (bed needs to be cleaned daily) the undressing, the changing of the gauze around his underwear area, the splint for his hand, the medications, the ‘getting the room ready’, which include the various fans, TV, water, tissue, throw-up towels and finally the kiss goodnight, my favorite by far. What took longer that night was the changing of the gauze per se, it was particularly time consuming.

I am not complaining, mind you, just describing a day in our life. Nicky’s daily care starts when he wakes up in the morning, I usually give him his medications, unhook him from the g-tube machine, dress him, help him out of bed, and wheel him to his computer, at which point I get him ice water and make sure he has tissue and a throw-up towel nearby. He has an urinal that I have to clean at least once a day, and during the day he will need refills of water, pain meds etc.

IMG_9831skHe does not always need a ‘throw-up towel’, this is just a recent thing due to the pre and post throat dilatation. Before surgery he could not swallow his own saliva, now post he has a lot of loose skin in his throat that needs to come out. Not a pretty sight, I tell you, especially when he insists I look at it. I mean, gross. Ugh.

During the day he needs help on and off. To go to the bathroom, for water, popsicles, anything really. He spends his day playing Minecraft, or the WiiU, watching videos on YouTube, there is really nothing else he can do.

At around 7:30pm we start with our bandage change routine, which it has changed over the years. We used to do a whole bandage change every third day, but it was too taxing, too much. I was reminded of how overwhelming a complete bandage change truly is just recently when I took my son to camp. Because they have so many activities and I wanted him to miss as little as possible, we picked two mornings during the week where they didn’t have much going on and we scheduled a bed in the ‘med shed’ for 8am. On the second morning we were there 5 hours non-stop. It took so long that my roommate did not believe me when I told her we did the bandages ‘the whole time’ and asked me where I went. I was so exhausted all I could do is grab a milkshake on my way back with my friend May and we went in to put our feet in the hot tub. I still wanted to make this day ‘fun’, hence I went with the girls to various downtown shops, but the rest of the day I was basically a zombie. I sat outside one of the stores while the girls were inside and I almost blanked out.
So… if I was this exhausted and drained, what about Nicky? He was OK. He’s far stronger than me without a single doubt.
At any rate, usually the routine is, he comes over to the living room couch at about 7:30pm and I give him some pain meds. We sit on the couch, which has three big bins of various gauze stuff under the coffee table and we do one third of the body while watching TV. We have certain shows we like to watch together, such as American Idol, Hot in Cleveland, Two Broke Girls, America’s got Talent and we like Kim Russo, a medium that does this show called ‘The Hunting of…’, a spinoff of one of our favorite shows, called ‘Celebrity Ghost Stories’. It’s just fascinating. Yes, we do believe in the paranormal and supernatural, mostly because we have our own ‘encounters’ with it. If you’re interested, you can read one of my encounters here–> http://blog.silviaskingdom.com/?p=172

But, I digress. We divided his body into ‘thirds’ and we do 1/3 per night. One night we do legs, one night an arm, and the following night another arm. The arms take a long time mostly because of the work involved in wrapping the hands. To me his hands are extremely important to wrap, I know that is not the case for many other RDEB patients and that’s fine, you know, it’s the proverbial ‘whatever floats your boat’, but Nicky loves and cherishes his hands, and wrapping them is as important to wrap for us as any other part of his body. We cover the hand with vaseline gauze, including the fingers, to keep them soft and supple, since the dried up scar tissue is what is responsible for the webbing, and then I wrap them following the instructions given to me by both Nana Sheri (http://blog.ebinfoworld.com/?p=258) & my friend Lorraine (http://blog.ebinfoworld.com/?p=269).
This whole thing takes about 2 hours, it all depends on how eager or picky Nicky is that night. He likes to clean his own wounds, pop his own blisters etc, and he takes a long time doing that. We do this at night because we are both “spent” afterwards and all we want to do is sleep, ha ha, and we do this every single night, so there is never a bandage-free night, no matter what. No matter what we did that day, if we went somewhere, like school, or if we’re at a hotel, none of that matters, we have to do it every single night.

Twice a week I take him to school. I wheel him to the bathroom for his oral care, while I get the car ready. I usually get his water and school stuff in his car, turn the car on and drive it outside to get the passenger’s door closer to the front door as much as possible, turn the A/C on blast and then I go get Nicky. Nicky can’t really walk, so I use his walker with seat and roll him out to the car. When we get to school I put together his power chair which is always in the trunk and make sure he has his fan, tissue box and water for the roughly two hours he’s there to take tests or do homework. Since it would be a nuisance to go home to just be home an hour and have to drive back, I just stay nearby and run errands.

M758At least once a month I have to take him to get labs locally, which is always a horrible experience, and then also to CHLA for a blood/iron infusion, in which case the whole day is basically gone. We leave around 9am or so, takes us about 2 hours to get there and then do the infusion and drive back. Usually because of traffic or waiting around etc, we don’t make it home before 5pm and then I have to take Connor to Karate at 6pm. Of course during the school year I am also Connor’s Taxi, taking him to school in the AM and picking him up in the afternoon. He normally has swimming too on Saturday morning. My aunt made me laugh the other day when she asked me ‘who’ took Connor to Karate and Swimming. ‘What do you mean who?’ I told her… me! “Oh” she said. I don’t know what she’s thinking. Did she really think I had nannies or something? Ha ha. She’s funny. I love her to death. Nop, It’s just me. I am Mom’s Taxi service!

I never had a nanny, which most people don’t have, but what most EB families have that I never had is a nurse. When Nicky was a baby I was so overwhelmed with his care I used to call to BEG the pediatrician to get a Nurse to help me. Heck, when Nicky was a baby, back in the olden days of 1996, I could not even find any other parent online to help me finding ways to care for him at all. It was all trial and error with us from the very start. The internet was not then what it is today. Insurance refused a Nurse over and over year after year, claiming ‘I knew how to do it’. Yes, that was their excuse. Wow. When I moved to California I did finally get approved for a program called IHSS in 2002 when Nicky was 6 years old, which is a program originally designed for the elderly to hire someone to care for them, usually a relative, which saved the state a lot of money preventing the elderly from being put in nursing homes where the state would have to foot the bill. Keeping them in their homes was not only the more humanitarian thing to do, but it saved the state money. They eventually broadened it to the disabled for the same reason. It’s much cheaper to have someone care for someone in their house, rather than pay some “nursing home” to do it. So, I basically get paid minimum wage for a certain amount of hours a month to care of Nicky. The lady comes by every  year to assess Nicky’s needs and see how many hours a day it takes me to do this and that that is above and beyond what any mother would have to do. Many have told me that with this money I should just hire a nurse and go to work. Nice sentiment… just dump my son off to a stranger, not that a trained nurse would work for minimum wage anyway!!! And this nurse that works for minimum wage would have to come in at different times of the day since caring for Nicky is not exactly a 9-5 job, but an hour in the morning, at least 4-5 hours at night and then would be responsible to take him to school, hospital etc. Who is this person going to be exactly? As if this is not a sacrifice of love that I am doing, because I do love my son more than life itself. As if I am selfish or lazy or even a ‘moocher’. As if living without a hefty paycheck for myself to afford nice vacations, new furniture, bigger house, lavish wardrobe, perhaps a maid, is something I don’t wish I could do. Why does our society frown on social services I will never understand. I grew up in Italy, and people there don’t think like that, so I don’t either. I can’t. It’s not part of the fabric of which I am made. I never learned to look down on the poor, the disabled, the disadvantaged, the elderly, and I never will. I don’t judge people I don’t know because I don’t know their story. I don’t know their sacrifices, I don’t know their struggles, I know nothing. I don’t judge, period. It’s not who I am.

The truth is, my children need me. They need me NOW, not at some point in the future when they are adults.While Connor will most likely be self-sufficient soon, Nicky most likely never will be. As he’s getting older, he’s getting harder. He’s bigger, and he can do less and less. It’s the nature of the condition. RDEB (Recessive Dystrophic Epidermolysis Bullosa), unlike some other forms of EB, is a degenerative condition. According to the Merriam Webster dictionary, ‘Degenerative’ means “causing the body or part of the body to become weaker or less able to function as time passes”. It’s hard for me to describe or what it means to watch your own flesh and blood deteriorate in front of your eyes.

We need a cure, sooner rather than later, since time is not exactly his friend. Am I lucky to be able to be home? Of course. I never take it for granted. I know there are people in worse situations than mine, I watch the news. I know about my great-aunt losing her entire family and what it did to her, I know about families that lost children to EB, in some cases more than one. That’s why I want awareness, that’s why I started the ebinfoworld.com website, and everything else. Hell on Earth is what this is, make no mistake.
As per my finances, following not one but two bankruptcies, I make whatever money go far by clipping coupons and shop at thrift stores. I call myself ‘cheap’, but my husband gets mad when I say that, he said I am ‘frugal’ and there is nothing wrong with that. I also realize not every mom can stay home, but all those that do usually have family nearby, which I don’t. And single moms with disabled children? YOU ROCK. I was a single mom for a while there and it was horrid. Even if the children are healthy, it’s an enormous job, one we can never understand unless we’ve been there. To be the sole caregiver for another human being with no help from anyone… I almost went crazy. People go through all kinds of hell, respect is all we ever ask for.

caring-is-being-thereI see photos of children spending a week or longer with their grandparents or other family members and please know that, yes, I am jealous. I try not to be, but alas, I am human. I am jealous of my own sisters (which I absolutely adore), cousins and friends that can just ‘drop off’ their kids with relatives. I can’t drop Nicky off anywhere and I am the one that could use a break the most. My son can really only go to his dad’s for the weekend, and he does go every other weekend for a total of 4 days a month, and even though a couple of years ago I was able to go to see my family in Italy for 3 weeks, that was a huge, gigantic stretch, one I will not do again anytime soon. Not because his dad didn’t do a really great job (I have nothing but respect for his dad, we may no longer be married, but he’s an awesome dad), but because I promised Nicky I wouldn’t. He and I are as close as any two human beings can be. He’s part of me, like a leg or an arm. If he’s not with me I feel as if I am lost in some way. I am planning to go to Italy again sometime in the next couple of years, but the kids are coming with me. Que in the Twilight Zone music on how in the world I am going to pack all the bandages and liquid food without going over in the weight limit! Ugh.

Do I wish I could do other things with my time? Of course. I would love to have a fulfilling career, have the freedom to travel anywhere at anytime… heck, just being able to leave the house which at times I feel is my prison. I sometimes crave time ‘alone’, to think, read, figure things out, sleep. But caring for Nicky is important to me and I do feel my work is important. I honestly feel this society as a whole thinks taking care of children is an unvalued skill. I disagree. Taking care of the next generation is ‘the’ most important skill. Just my opinion, of course. Sometime I wish people would take into consideration a lot of things when it comes to Nicky, EB and his care. I remember when his middle school teacher used to constantly complain about his ‘smell’. I find it quite insulting when anyone’s only reaction to Nicky is his ‘smell’. He is a child in pain, and all you notice is the smell? He truly does not smell that bad, and, frankly, if you want to talk about ‘smell’, you should be the one touching and handling the bandages filled with oozing wound stuff. The smell is the least of my concerns. It’s the state of his wounds that worries me the most.

OK, so, I went off on tangents here and there, but that’s pretty much what our days are like. Sometimes I feel we live in the movie “Groundhog Day”, every day is just the same, so that is why I look forward to little vacations I save for, fundraisers we can attend to, camp, getting together with my friends, getting a massage or facial every now and again. I try to treat myself and carve a little time to read a couple of days a week too. I tell my kids I am going to take a bath for one hour and to not disturb and I read while I soak. It’s my ‘me’ time. I wish I could do it everyday.

Thank you for the kind and supportive words, they lift my spirits more than I can express. Writing has always been my therapy, and the reason for this blog. I am still working on my book of Nicky’s life. It’s been harder and harder to remember some of the things, but I know what a weight off my shoulders it’s going to be once it’s done.

Hugs and Blessings… Love and Light <3

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It Is What It Is

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.

IMG_3673_sk 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.

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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,

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