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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1/2,381,000

I got hit with a big blow yesterday, one I’ve known all along nonetheless. As the DNA results came back that Nicky did in late April at Stanford to find out if he’s eligible for the skin graft trials, I’ve been trying to decipher them myself. 467516_10151222382997174_1352643367_oI will talk to the Doctor this week (hopefully) to find out if he’s a candidate, in the meantime through my googling I found out that Nicky does have the “Hallopeau Siemens” subtype, which is, sadly, the worse form of Recessive Dystrophic EB. Yes, he has a nucleotide substitution in exon 109, and the mutation is denoted R2685X at the protein level, which points to RDEB-HS and ONLY to RDEB-HS. Ugh. I found this out on THIS LINK, which speaks of this particular strand being of Italian origin. Upon further research I found a link of all the different RDEB-HS mutations in Italian families in THIS LINK. My heart sank. Of course the term ‘Hallopeau Siemens’ is not used anymore, it’s been changed to “severe generalized” in more recent texts, but a lot of information “out there” still refer to it as HS, so I will use them interchangeably.

When I say that my kid is one in a million, I am not exaggerating at all, in fact, he’s 1 in 2,381,000 millions. That’s how rare RDEB-HS is. Considering the population in the US as of right now is about 318 millions, this means that there are about 134 patients with this diagnosis in the entire country and one of them is my son. It’s insane.

I was surprised to read some of the comments below my findings… some parents thinking that there were no ‘subtypes’ of RDEB or that it was either HS or non-HS. That is not correct. I’ve always felt it was my quest to spread EB awareness and knowledge (which is why I created EB Info World back in the day when Nicky was an infant) and one of the reasons why every EB patient is different is because they can all have different subtypes, which can vary widely. There are MANY subtypes of EB for all forms. This is why what works for one patient does not work for another and we cannot lump them all together. It has been very hard for me over the years to explain to the various Doctors, Nurses or other patients why whatever product or course of action did not work for Nicky, it just didn’t. We’re all dealing with our own thing here, and Nicky being one in 134 patients nationwide, I can’t expect to find an expert to tell me what would unequivocally ‘work’ for my son, only Nicky and I, through trial and error can be the judge of that.

So, in an effort to be as thorough as possible, here’s the different forms of RDEB according to Dr. Fine. Complete table for ALL forms available HERE:

Recessive dystrophic EB (RDEB)

RDEB, severe generalized (RDEB-sev gen)

type VII collagen

RDEB, generalized other (RDEB, generalized mitis (RDEB-O)

type VII collagen

RDEB, inversa (RDEB-I)

type VII collagen

RDEB, pretibial (RDEB-Pt)

type VII collagen

RDEB, pruriginosa (RDEB-Pr)

type VII collagen

RDEB, centripetalis (RDEB-Ce)

type VII collagen

RDEB, bullous dermolysis of newborn (RDEB-BDN)

type VII collagen

Here’s a website with a really good explanation of the form Nicky has, severe generalized, also known as Hallopeau Siemens. it. The last sentence was particularly hard to read, even though I’ve known this all along. When they mention SCC they refer to Squamous Cell Carcinoma, a form of skin cancer that is somehow fed by the absence of Collagen Type VII. What I mean is… normally the Collagen Type VII prevents this type of cancer from spreading, so this is why it spreads so fast in RDEB patients. They have none. My biggest nightmare is SCC. It only appears in non-healing wounds, which is why I wrap Nicky to protect him from getting wounds in the first place and make sure his wounds heal properly. Anytime anything looks strange I send a picture to the Doctors. Prevention is our only cure right now.

Of course, at the end of the day, nothing’s changed. We still have hope, especially in the trials. At the moment we’re waiting to hear from the Doctor’s office about a referral to Stanford to get a throat dilatation. Nicky is having a hard time swallowing his own saliva so hopefully once we get the green light the Doctor can take him in ASAP. He’s in a lot of pain right now.

Thinking positive, pushing through every day… one day at a time, that’s all we can do.

Love and Light,

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Have You Read A Good Book Lately?

Click on the image to be taken to Amazon to read more about it…

I don’t often recommend books, and I must admit, I am an avid reader, so I should do it more often. The type of book I look for, however, is not your novel or anything that is fictional. I feel as if there is plenty of time during the day to take my mind off my daily struggles with my son. There is TV, there are movies, there is Facebook, there are trashy magazines, there are hugs and kisses and so much more. But because I have such little time to read, and because I feel as if I have an hour to myself I want to read something that helps me in my life, I go for biographies. I want to know how people survive incredible odds. I want to know how anyone survives when life hands them lemons. It could be how they handled a particularly tough childhood, divorce, death of their child, disability of any kind… but when I came across this book called “I Shall Live” by a holocaust survivor I felt, YES, I hit the proverbial motherlode.

To be perfectly honest, I never read anything so riveting. Here’s a very intelligent young Polish guy, living his life with his family when WWII hits. I don’t know why this book was never made into a movie. This guy did it all. He survived everything and in the book he tells us how, what he thought, the fears and the atrocities he witnessed. He should have been killed so many times and somehow he made it through by ingenuity, by luck, by how fast he ran… the stories in this book make my life feel like child play. It’s always nice to have prospective, and this book gave me a ton.

I did some research on Henry Orenstein… not only he survived the holocaust by running, hiding and the lack of food in concentration camps, but after having been rescued by Soviet troops, he emigrated to the United States and became the toymaker who convinced Hasbro to start producing the Transformers. After his retirement he discovered the world of poker and invented the hole card camera, a table level camera which allows television audiences and announcers to view players’ hands and hidden cards. This innovation led to a boom in poker tournaments and television shows, some of which were launched by Orenstein himself.

Why isn’t there a movie on the life of this incredible man? Common Hollywood. In the meantime, if you want some prospective in your life, read this book. I promise, you will never forget it.

 

Hugs and Blessings…

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A Grateful EB Mom

The word of the day might as well be ‘awareness’. Not only Nicky’s book was featured on a story on FoxPhilly, but a good friend told me how anytime he buys EB eggs he thinks of me! That was too funny.

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Nicky and Connor at Camp

At any rate…  we got back from Camp Wonder  this past Saturday afternoon and the boys can’t stop talking about it. It was magical as always. To have a Camp sponsored by companies such as Cetaphil that enhances the lives of children in great pain every day, it’s beyond humbling. The Children Skin Disease foundation is doing amazing things and I will forever be grateful to the Tenconis, especially Francesca, who make it happen every year. THANK YOU!

As a mother, to have a place for me to leave Nicky and know that not only he’s going to be taken care of, but have fun, when I can’t leave him in anyone else’s care during the year, it means a lot. This is why the other moms and I are able to take little excursions during the week and have the ‘me’ time we rarely if ever have. We talk, sightsee, take our time… I can never really “take my time” when I go anywhere unless I know my husband is at home to make sure Nicky is OK. I can’t really leave Nicky alone more than a few hours at a time. He can’t walk or even do simple things like open the fridge or go to the bathroom. Yet, his sense of humor always leaves me chuckling. He told me on the car on the way home that another camper told him that EBers don’t live longer than 18 years. Nicky is going to be 18 in November. Do you want to know what he told him? “You’re wrong, Bit$#”. Bah ha ha. Nicky does not ever cuss, so this was just hilarious!

Now that we’re home all of a sudden his throat is 100% worse so he told me he really needs a throat dilatation… I started working on getting the referral, the last one was almost 3 years ago so that’s awesome that the last one lasted this long. I will write a more detailed blog on Caringbridge soon with all the details. We also received the DNA results which will tell us if he’s eligible for the skin graft trials at Stanford once he turns 18, I will wait to talk to Dr. Lane to figure it all out before I post that update.

Since the story aired a lot have commented how Nicky does not look 17 and looks maybe 12. When Gina asked me why I had to do a double take because I knew, yet I didn’t know how to explain it. The bottom line is that his body requires a LOT of calories. Even though he’s tube-fed and gets about 2000 calories or more every night, that’s not enough. The nutritionist wants me to give him 5 cans a night at 500 calories a piece, but as it is I have a hard time getting 4 in at 100ml per hour. Recently we also sadly figured that the reason why Nicky was having all the stomach problems that he has was because the Nutren 2.0 had High Fructose Corn Syrup rather than sugar. When we briefly switched back to the Nutren 1.5 his problems disappeared. That’s 500 calories LESS calories per day though. Ugh. What are we to do? What is the less of two evils? We might start combine or switch back and forth. He has a big tummy and being heavy makes it worse for him to move around, but the reason why he still looks 12 is mostly because he hasn’t gone through puberty yet. He has no hair anywhere, his voice hasn’t really changed. Is that a bad thing? It’s all relative I guess. I was bombarded when I mentioned the fact that he would most likely not get married the way he is now, without a cure. EB patients that are not nearly as severe as he is called me all kinds of vile names. I was not talking about them. I was talking about my son. My son knows what his limitations are, he has accepted the fact that as it is, many things are out of reach. I asked him what he would do if he was EB free for 1 single day. His #1 wish? That he could EAT. Eating is a big wish in his book. If that does not explain what we’re dealing with here, I don’t know what does.

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Vintage Silvia… I must get this shirt.

Finally… oy. It’s a big birthday for me in a couple of days. I will hit the half-century mark. It’s weird how I felt much worse turning 30 than I do turning 50. I think part of it is the fact that two of my closest friends died in their 30s. My childhood friend Paola died of a brain tumor when she was 38, leaving her 2.5 year old little girl behind which took her 10 years and 3 IVFs to conceive. Life is just cruel sometimes. My former boss and amazing friend Darren died from complications of his HIV diagnose also at 38. That old saying that goes… “Don’t regret growing older, it is a privilege denied to many” comes to mind. It is what it is. Aging is out of our hands, I will never hide my age. I am good with turning 50. Hear me roar! I am still a work in progress and I have work to do. I want to see Nicky be cured because I refuse to give up hope. I had a long conversation with Nicky a few months ago and asked him how he felt about his life. I made sure he knew I was in his corner no matter what. I wanted him to know that if he “hung in there” I would as well, but if one day he decided that ‘enough is enough’, as heartbreaking as that would be, I would respect his wishes. He told me flat out that as long as I was there to take care of him, he would have hope.

So, if you have a minute, please say a silent prayer not only for Nicky, but for all EB kids and adults out there who live daily with pain and please spread the word if you can.

Much love and blessings,

smalldolly1

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