Connie

Name: Connie Smith

Birthday: August 28, 1960

Location: Frankfort, Kentucky, USA

Family members with EB: Catherine and Samantha, Identical twins, 2 years old, RDEB

Biography... in her own words:
When I was 8 weeks pregnant I had my first ultrasound to satisfy my doctor’s concerns about an accurate due date (she suspected something because I was so large already). When I finally figured out that what I was seeing was two babies’ heads, and not one head and a tumor, I literally laughed and cried at the same time. I had never had such an experience in my entire life. I laughed because I couldn’t believe that I was having twins and I cried because I had a 4-month-old at home already, not to mention the other 4 children that my husband and I brought together from our previous marriages. Let me total them up for you—7. Their current ages are: Russell (13), Lauren (12), Parker (8), Hayden (5), Keaton (3), and Catherine and Samantha (2). My sister and friends all laugh at me since I was the “career woman” who only wanted one child. At 35 however, I had a heart-to-heart talk with myself and wondered if that was what it was all about. I just couldn’t see a job/career being more important than family and spending your time with the ones you love. I had an 8-year-old and he and my job were my life. Don’t get me wrong I liked my job and had put a lot of effort into it. But I was just up in the air about marriage and kids and careers. Then my decision was made and it was a very easy one when I met my Prince Charming, Tony. When we met, we both knew that was it -- we were meant for each other…period. Little did we know, however, how fertile we both were.

Now let me tell you how I felt when I had the girls and found out that something was not quite right with them. The pregnancy went as most twin pregnancies do. Best rest at 32 weeks and deliver 4 weeks early—not unusual. Delivery went well. I joke that I have had children every way you possibly can: my 14-year-old was a cesarean section, my 3-year-old was with a lot of pain medicine (I couldn’t even feel my legs), and my twins were natural—not by choice, the epidural did not work. Their weights were good—Samantha 7 lbs. and 6 ozs. and Catherine 6 lbs. and 7 ozs. (I like to think of myself as ---or at least having been---a petite woman, but you wouldn’t know it from the size of the children I have had). Catherine Leeanne, the first born, had a piece of skin about the size of a quarter torn/missing on her ankle. Samantha Rose, born 6 minutes later had a smaller sized area on her index finger. The doctor also thought she had swallowed some blood and suctioned her mouth out—later as we found out it was from the blistering. I got to hold Catherine and brushed my finger on her face and noticed how she cringed when I did that. I knew something was wrong, but the areas were small so I didn’t really think anything THAT major was wrong. I mean, how bad can a condition be that JUST involves the skin, right? I had never heard of anyone being impaired by a skin disorder, much less dying from one. The girls were sent to a larger hospital in Louisville(50 miles away) shortly thereafter. I knew the doctors didn’t really know what they had when one of them asked me if I had ever had herpes, because that’s what she thought it looked like. I just looked at her like she had stepped off a spaceship. I got out of the hospital the next day, went home to pack my bags, threw up from still be nauseated from the delivery, and went to Kosair Children’s Hospital in Louisville. I knew the routine you see, because my 3-year-old went through the same thing when he was born. The day we were to be discharged from the hospital the doctor (a wonderful small town pediatrician who has recently moved to California) found a “mass” around his kidney/liver area. We went to Kosair’s, they did tests for three days and on October 31 (how ironic, it was Halloween) diagnosed him with neuroblastoma (a rare and almost fatal cancer in children). They operated on him the next day and the surgeon came out flabbergasted. He said he would have bet his medical license that that was what he had. But, during the surgery they discovered that it was just a cyst on his adrenal gland. They removed the cyst/adrenal gland, biopsied his liver (it was enlarged from the cyst), and we went home two days later. He has been fine ever since. I still say the cyst was caused during the long, arduous labor because he was 10 lbs. and 13 ounces. (My first child was just shy of 10 pounds.) Back to the girls. So we go to Kosair’s. Find out that their skin had been “removed” from the heart monitor that was put on them at the hospital when they were born. The staff at Kosair’s was pretty sure of what the girls had when they came to pick them up in the ambulance. They said they got about one case every year to year-and-a-half.

So we spent the next several days trying to understand this strange condition called Epidermolysis Bullosa (EB). I couldn’t spell it, I couldn’t pronounce it, and I just couldn’t fathom how a skin condition could be that bad. But I was in for a rude awakening. Family and friends got on the Internet and found the DEBRA site pretty quickly and we slowly just kept learning more and more.

Now I will make my plug for Silvia’s web site and the EB Mommas’ Web site. I can’t imagine how people made it through before having these. It must have been excruciating not knowing probably another person around with this disease. With all the wonderful information, wealth of resources and truly special people being brought together via these web sites, I have no doubt that the care of my girls has been dramatically improved and my sanity saved.

So how do I do it? How do I cope? What is it like having twins and twins with EB at that? First and foremost, I have a great husband who loves children, is easygoing and would give you the shirt off his back. He is a very dedicated person. Nothing rattles him. He can stay at home all day with 7 kids, clean the house, do laundry, make dinner, and then go to work all night. I am a wreck after 3 hours alone with just 3 kids while accomplishing none of that. He tells me they have my number and know how to work me—do they ever! (He has just recently convinced me that they do better for their bath/bandage changes when I am not around—they cry the whole time for me to hold them and not a whimper when he does them.) Of course, he can’t be Superman 7 days a week and I will say getting them ready in the mornings and into bed at night are some real trying times (he has a rotational shift which changes every three months). Tony and I met in Louisville, were born and raised there, but live in Frankfort since that is where I have lived for the past 13 years and that is where I work. He works in Louisville and commutes one hour each way. That’s the kind of person he is. I also have a wonderful, tireless friend, Martha, who comes over when we do most of the baths. She shops for me, helps me remember things like anniversary gifts, and makes sure that Tony and I get out every now and then. I have parents and an aunt and uncle who have fallen in love with the 3-year-old and would keep him for months—if I let them. I have a great baby-sitter who I have known for 14 years that I can safely leave my kids with and who doesn’t mind the extra burden of having to deal with the girls’ condition. I have in-laws, who I can’t begin to tell you how much they do for the 11, 7 and 5 year olds, and an ex (never thought I would say this) who shares ½ of the rearing of the 14-year-old. I guess what I am saying is that I do it with a lot of help. Sometime I don’t do it especially well. There are many times when I am like the troll under the bridge; my disposition is not very good having only gotten 4 hours of sleep (several days in a row mind you). Not to mention that I haven’t had one uninterrupted nights worth of sleep in over 3 years. I have no life of my own but that doesn’t really seem to matter now (they say that happens with twins anyway). I am like a person trying to quit smoking (which I am trying to do)—I take it one day at a time and do whatever it takes to get through it. My children are my life. I only wish I could spend more time with the older children. Anyway, my husband says that since I work for the state I really don’t have a job. He kids me about government workers and I kid him about making “quality” Ford trucks and cars. I thought about quitting but since I may be 3 years away from retiring and as flexible as the state is with my hours, I am trying to hang in there. I work for the state environmental protection agency enforcing solid and hazardous waste laws. Talk about never a dull moment, that is another article.

What does the future hold? I don’t know. I take each day as it comes. I keep telling myself that Catherine and Samantha will get better, that they are different, that they won’t get the blisters in their mouths, that they won’t throw up gobs of blood, that they won’t be the ones to loose their finger nails (all this has happened). I am now telling myself that they won’t have to have a g-tube, that they won’t lose their hair, that their precious little hands won’t fuse together, that they won’t get squamous cell carcinoma, that they won’t…But, I quietly wonder if my miracle was used up when my 3-year-old’s neuroblastoma turned into a cyst.

I see pictures of other kids that have EB and I want to scream and cry. It is just too unfair to see children suffer and scream from taking a bath. To write in your baby’s book that today was the first day that her bare feet got to touch the floor and she is 2 years old. To wake up throughout the night and know that all you can do is hold your child while she “pats” her sides to stop the itching. We tell them not to scratch but to pat so it won’t tear the skin and they are now always telling us “I pat it Mommie, I pat it.” I see pictures and hear about kids and young adults dying from EB and I think, oh, my girls aren’t really that bad. But…I always have that nagging in the back of my head…that I try to push away…that says…you don’t know that. I see pictures of toddlers with no skin on their entire back and I say, gee, my girls aren’t that bad. Then I see childhood pictures of people who have died from EB and I say, oh, my girls look much worse than that. So, I guess what I am saying is that you don’t know. You just wait for things to progress, for them to happen and then you deal with it. You get on the Internet, you talk to mothers who have kids with it, you talk to doctors who have treated it, you watch every new medical advancement, you do anything in your power to find what the best way is to treat the symptom they are having at the time. And you try different things. My husband is always trying different ways to bandage them and for now I see an improvement. But, will it last? My husband always wanted to be a doctor and he studied genetics in school—now he can put both those skills to use.

Some might say it’s unfair to have one child with EB much less two. But I wouldn’t have it any other way. They are so cute together. They are starting to “help” unwrap each other. When we ask “Who wants to take their bath first,” they point to each other and say “Sissy does.” I listen to them on the room monitor and hear them argue back and forth, “My Mommie”, “No, my Mommie”. I laugh and cry (again) when I am unwrapping them and they stop me and look me straight in the eye and say, in that sweet, soft voice “You be eassee”. The thing that I feared the most about having twins (okay--other than them being Siamese) was that I would just have a duplicate of the other child. How fun could that have been. I am happy to say that they are like night and day. Sammie is the calm, laid back, rarely gets mad, would probably sleep all night child. Callie on the other hand is active, high strung, wait on me, up all night, can’t sit still, child. Don’t’ let any one tell you there isn’t a good twin and a bad twin. Subsequently, I surmise that Catherine’s EB is somewhat worse than Samantha’s because of her disposition. I know this is terrible to say, but, sometimes it is really funny to sit and watch them fight. One will bop the other on top of the head and the other will try to bite her in retaliation. If I didn’t intervene, I have no doubt that blood would be shed. One day Samantha put her finger in Catherine’s mouth and was astonished (and in pain) when she bit it. And with identical twins there is always the story of feeding them as babies and getting confused as to which one you just fed. The first time this happened I actually panicked—it’s not like one was going to starve or the other couldn’t eat again. One thing I find interesting with Catherine and Samantha’s EB is that about 95 percent of the time, they will get blisters or “bad spots” in the very same locations at the same time. I know that sounds crazy, but it’s true. We went from knees, to feet, to elbows, to underarms, to stomachs, and then to necks on both girls simultaneously. I know that twins share a special bond and I am hoping that it is that special bond that will carry them through the hard times. The best advise I heard on how to deal with twins was from a mother who when asked how she did it replied, “I do something once and then I turn right around and do it again”. I am not only looking forward to retiring in a few years to devote more time to all our children, but I want to do what ever I can to personally help raise funds and awareness for this devastating condition. I just can’t imagine children continuing to be born with this condition and people not wanting to help if they knew what EB really was—it isn’t JUST a skin disorder and it really is THAT bad.