Blog · December 4, 2013

No, I am not Latina ;-) & misc. EB stuff

Good Morning to all my friends. I feel a little chatty this morning, so please indulge me!

One of the most interesting assumptions people have about me because I live in the Southwest part of the United States with my name spelled with a ‘i’ instead of the English-ized version of Silvia with a y (Sylvia) is how I must be either Latina or at the very least I must be able to speak Spanish. When I tell them I am Italian I am invariably told that Silvia is a Spanish name.
Actually… that is not true. Not only I am Italian, my name is also. Wikipedia lists my name originally as Italian, and it’s also a very old name. In Roman Mythology, Rhea Silvia was the mother of twins Romulus and Remus, who founded Rome 2766 years ago (753 BC). Saint Silvia was the mother of Pope Gregory I (also a Saint). I am not sure my name can be more Italian than that! OK, enough about that.
About speaking Spanish… I understand it, but I don’t speak it per se. Italian is a very close cousin to Spanish so I can converse quite comfortably with a Spanish speaking person by responding in Italian. I never felt the need to learn it, as to me it sounds like a weird version of Italian, ha ha. I feel the same about the Venetian Italian Dialect (which is what my Nonna spoke) and the Piedmontese Italian Dialect (which most of the older generation speak in my hometown in Italy), I understand both of them perfectly, I just can’t answer back in it. I also speak fluent English and I speak some French, so I feel I can travel anywhere and be understood. I am good with that.

So, since these famous “Silvia’s” are mostly remembered as mothers, I find it interesting that I also mostly think of myself as a mother first and foremost. My children are my life.

I wrote a blog post last year about Nicky, answering some questions about his condition and misc. things, but I feel I must delve a little deeper on a couple of questions I’ve gotten recently, hoping to shed more light on the condition and my feelings on things.

Life Expectancy with RDEB

On my other blog post I wrote the following: “These children’s lives can be taken by infections, Anemia and Skin Cancer. RDEB patients live a painful life that can range from 0-30+ years in most cases, although there have been patients that have lived longer. Some with the very mild version live normal lives and have a normal life span.”

The 'EB Angel' on our Christmas tree a friend sent me years ago.

The ‘EB Angel’ on our Christmas tree a friend sent me years ago.

The reason why, in my humble opinion, most Doctors are reluctant to tell parents ‘how long’ their child with the more severe forms of RDEB will live, is because these patients don’t die from the condition as, say, a patient might die of Cancer with the different stages. RDEB patients die of complications related to the condition, the lack of collagen type 7 does not cause death in itself, it’s the collateral damage that does, which is extremely different with each patient. These complications could hit them as babies or in their 30s. I was told by Dr. McGuire when Nicky was little that the #1 RDEB enemy was infections. Infection and/or lack of proper nutrition due to inability to eat because of scarring in the mouth/esophagus may cause heart/kidney/lung failure. Losing blood through the wounds causes severe Anemia, and Skin Cancer is as real as it gets, and scary as hell. According to a study done by UNC Medical School, RDEB patients have a 6% chance of getting SCC by 20 years of age, 21% chance by 25 years of age, nearly 40% by 30 and 53% by 35. SCC (Squamous Cell Carcinoma) is usually a slow moving cancer in normal skin people, the Collagen 7 fights it. But with RDEB patients, their lack of this protein means it runs rampant and it travels fast. It also only appears in wounds.

Ever since Nicky was little, making sure he HEALS, that he gets the proper nutrition, that his wounds are kept infection free, hence keeping him wrapped to prevent new wounds and allow proper healing of existing wounds has been my #1 priority. Doing whatever it takes to get those wounds to heal to prevent infections and skin cancer means giving my son a higher quality of life and a longer life expectancy. We are so close to a cure, I want… need him to make it.

I know some people may think I am crazy by sharing the following anecdote, but I feel the need to share today. Who knows who it might help.

Alex is always with us

Alex is always with us

When Nicky was about 10 years old (give or take) his health spiraled out of control. I was beside myself. My first baby, Alex, which is Nicky’s big brother, was stillborn at full term, and I’ve always felt his presence in many ways. You may roll your eyes at this, but I’ve had Alex visit in my dreams, saying the identical things a psychic told me he was telling her. “Things” have happened on his birthday, such as his photo falling from the wall, toys going off by themselves and more… When I ask Alex to chose the next song on my iPod, a song about Angels or about losing a loved one/loss ALWAYS comes on. It has happened way too many times for me to think this is just ‘coincidence’.

There is no doubt in my mind he’s always around, taking care of Nicky. I also know that sometimes we have to ‘ask’ for help from the ‘other side’. So, out of pure desperation, one day I started talking to him. I told him to please get Nicky better, to take care of him, to be by his side and to heal him. That he needed to be alive when this ‘cure’ or ‘treatment’ came, and that his mom could not, simply could not bury another child. That if he wasn’t going to do it for his brother because he wanted him with him in heaven, to please do it for me, to think of what Nicky’s death would do to me. PLEASE HELP ME! I begged.

Within the next few days, Nicky drastically improved. Not a little, it was major. Not only his wounds started to heal a little bit, his mood changed from the morose to the sweet and happy and I was grateful. I still talk to Alex and ask for his help.

Another thing I wanted to touch base with today was about Nicky’s wound pictures that I post every now and again. Some parents that also post pictures have been called out and ridiculed and told they were ‘exploiting their child’. I hate, HATE that this is happening.
All the photos of wound pics from this blog where posted after Nicky himself asked me to, in an effort to make people understand. That’s all we want, to make people understand. Of course I used to post photos way before Nicky could approve nor ask, but I can’t tell you how many people thought Nicky got rashes until they saw the photos.

Knowledge is power, we can’t explain the condition when we cover the evidence.

And that’s just my 2c.

Thank you for reading and Happy Holidays!