A Wave and a Smile
December 14, 2009 8:00 PM

Since my last update Juliana has had several surgical procedures, and will have another one in a month or so. Each procedure is but a step in the direction of Dr. Wolf's goal, and our goal, of providing Juliana with a functional face that doesn't hinder her in presenting the wonderful person she is to the world.

Actually, Juliana's level of self-esteem and self-confidence is so high, she has never held herself back from anything. She has refused to be hindered despite ongoing challenges. She greets the world with a wave of her hand and an exuberant friendliness, expecting a similar response.

She's been through so much physical pain, I couldn't bear for her also to be hurt by human meanness. There's an incredible amount of it out there directed at her, but so far it hasn't touched her. She's very special and very loved, and she feels special and loved.

Here's some great news: Juliana now has "normal" hearing thanks to the wonderful Cochlear Baha Sound Conduction System. She actually has a small titanium implant in her skull above each ear with an abutment into which the "sound conductor" (hearing aid) snaps. The titanium implant integrates with her skull (through a process called osseointegration) which allows sound to be conducted via her skull directly to her cochleas. This allows her to hear normally. Absolutely wonderful! The sound conductors are about the size of the tip of your thumb, and are unnoticeable under her hair. They are snapped off at night and back on in the morning. Her previous hearing aids were held against her head by a head band. Sometimes they made a good connection with her head and sometimes they didn't. With these, the connection is always good. She loves them.

Juliana is almost 7 years old now—a great age for enjoying Christmas. She loves the Christmas tree and the lights on the house. She doesn't have a big list of things she wants. She's more interested in doing the giving part. She continues to be just a nice little girl.

—Jeanne ("Party")

Just Another Kid
February 16, 2009 8:00 PM

My apologies again, for not making timely entries in this journal. It gets more and more difficult to write from the viewpoint of Juliana's nurse. For long periods of time the nursing is quite routine. For most of my shift Juliana is asleep. While she's sleeping I give her eye care, mouth care, and trach care. She gets a tube feeding throughout the night, and is suctioned as needed. She may require a respiratory treatment. I bring her to the bathroom and back without even waking her up. I give a couple medications. And I'm there.

If she's recently had a surgery or procedure, there are more things to do. She often has incisions to care for, which is especially difficult when the suture lines are near her mouth. In between her surgeries she's basically a well child. Yet she still has a nurse at night, especially at night, because of her trach. We don't know how long she'll continue to need it.

I've received a few e-mails wondering why we haven't posted recent pictures (a new picture has now been posted). Well, you know, Juliana's facial reconstruction is not finished. It's a work in progress. Juliana has had over 30 surgeries, some of them complicated, some kind of minor. At first I expected, each time, to see a major improvement, but came to realize it's not like that. It's a long, slow process. Sometimes it's "two steps forward, one step back." Occasionally it has even been "one step forward, two steps back"! It's disappointing when it seems as if little progress is being made, but when Dr. Wolf explains his plans and strategy it's easier to bear.

Juliana doesn't have any cheekbones, and the skin over where her cheekbones should be is taut. In order for bone to be transplanted to that area, there needs to be enough skin, or cheek, to cover the bone. First the "cheek" must be formed, then there will be a place to put the cheekbone. It's being done one side at a time, which gives her a kind of lopsided look. She has a new cheek which looks too big and puffy. The next procedure will "debulk" it—as Tami wrote, "smooth it out." She's in the middle of having her left cheek formed, then the process will start over to form her right cheek, then the work can progress to her cheekbones. At each stage in the process there are possible complications, e.g., dehiscence, keloids, infection. Through it all Juliana is a loving, friendly, happy, creative, and playful little girl.

So far she has accepted the way she looks without making a problem of it. She has remarkable self-esteem and self-confidence. Once Juliana and Kendra were somewhere with other children around. One child was calling attention to Juliana and making rude comments. Kendra remarked to the group, "She's just another kid." She is that. And more.

—Jeanne ("Party")

Safe at Home
November 28, 2008 11:00 AM

Still no pictures for you! Juliana returned from Miami yesterday (Thanksgiving Day) with the black surgical dressing removed from around her mouth. However, it was replaced by a sutured-on white dressing, which looks like a pile of cotton balls stuck to her mouth. This will come off in about a week—then we'll see what her new skin graft looks like. Dr. Wolf has been working on the left side of her mouth. He'll start working on the right side on December 17. It seems like a slow process, but she has come a very long way from where she started.

Last night Juliana had a new DVD to watch as she went to sleep, a Barney Christmas DVD. She couldn't go to sleep, though, until she had danced along to all the little dances. Barney and the characters in the video use a lot of arm movements in their dances, and she quickily learns all the moves, which she does while sitting up in her bed! Not only that, but she wants me to do them too. She'll be watching the screen while at the same time keeping an eye on me to make sure I'm doing it right.

Usually on her first night back from the hospital it takes her quite a while to relax, and then she'll sleep lightly, pushing away my hands when I try to work on her. Last night she watched Barney and two Signing Time DVD's before she fell asleep, but she slept well and I was able to clean her suture lines without waking her up. When she sleeps well like that I know she's feeling safe, back in her home.

—Jeanne ("Party")

"Party" All the Time
November 25, 2008 4:30 PM

You may notice that my name is now signed "Jeanne (Party)" in this column. I have written about the name "Party" before, but I looked back and the last time it was mentioned was September 26, 2007.

It's Juliana's name for me. She's always referred to me as her party. She signs "my party," emphasizing the "my." It started when she was about a year old. She'd be fighting sleep and I'd lay her down again and again, singing an old Eddie Murphy song—"My girl wants to party all the time, party all the time." Sometimes Tami would stick her head in and say, "How's the party going?" When I'd get there in the evening Tami would say, "Juliana, your party's here." So that became my name.

One of the things that Juliana is able to say quite clearly is "Party, look!" I love to hear that. You might notice some of the letters in the Guest Book mention "Party," referring to me. I love that, too. I play with the spelling and sometimes spell it "Partie," but that's who I am, Juliana's party.

—Jeanne ("Party")

Brave Little Angel
November 25, 2008 7:30 AM

Sorry, no pictures yet. Juliana has a "surgical dressing" sutured around the left side of her mouth, which is black and looks like a handlebar mustache. It's temporary and will be removed today, as she goes back to Miami for another skin graft.

When I first saw it, before I realized what it was, I was shocked. I thought it was necrotic tissue, and that the skin flap had failed. But thankfully, that's not the case at all. The skin flap "took" very well, and her suture lines are healing. The area is sensitive and she doesn't allow much touching or cleansing of it, so a lot of that is done while she's asleep, but she's in a lot less discomfort than we were anticipating.

The main challenge has been securing her trach. Usually it's secured by a velcro tie around her neck, but because of the skin flap and sutures, we couldn't put anything around her neck that would constrict that area. The flanges of the trach were being taped—held down with duoderm and waterproof tape. Because of increased oral secretions and drooling, the tape often became loose.

Juliana needed constant supervision to make sure she didn't cough out her trach! Also, changing the loose tape was an ordeal, because her neck was becoming iritated. Finally her sutures had healed enough that we decided to try loosely applying a trach tie, just enough to secure the trach without putting pressure on the skin flap. Thank God, it works! Anyone who's had a dressing removed knows it pulls the little hairs on the skin and it hurts.

Juliana was breaking my heart: trying to be so good and let us remove the tape from her sensitive neck area, but unable to resist trying to get us to stop. I'm so glad we don't have to do that anymore. Next, when she returns from Miami, I'll let you know what's different.

—Jeanne ("Party")

Work and Rework
November 12, 2008 11:30 AM

Juliana had an extensive surgery on November 5, during which Dr. Wolf utilized tissue and skin produced by the "tissue expander" to work on her upper gum and lip area. This had to be revised yesterday, November 11. I was concerned that she had to go back to the OR so soon, but Tami reports that the revision is an improvement and that Juliana feels much better. If there are no further complications they should be home Friday. I can't wait to see her. I've really missed her. We'll post pictures when she gets home and settled.

—Jeanne ("Party")

Best-Laid Plans
September 02, 2008 8:00 AM

It's been a couple weeks since Juliana went to Miami Children's Hospital for a major surgical procedure which we were looking forward to and dreading at the same time. To augment what Tami talked about in her last post:

What happened was that Dr. Wolf did a different procedure than the one we were expecting. He found that there wasn't enough tissue to do what he wanted (reconstruct her upper gum and lip), so he inserted a "tissue expander" into her left scapula area (left upper back). Once this incision has healed we will start the process of injecting a small amount of the prescribed fluid into the port, which will gradually fill the reservoir that Dr. Wolf placed under her skin. This will cause the area to gradually expand and stretch the skin, similar to how the skin stretches when we gain weight. When this process is completed there will be extra skin and tissue for Dr. Wolf to use, and he'll do the next surgery.

What Juliana has been through with this sequence of events so far has been quite painful for her. She resists having the bandage changed, etc. But she remains resilient and forgiving, ready to hug and play.

Just before the trip to Miami the film crew was here taping, and they also went to Miami to tape there. They may be able to return to include the next step of the surgical reconstruction in the documentary, but they definitely plan to be here filming when Thom returns from deployment in December. Knowing that, don't expect the finished documentary any time soon. But at least it's begun, and is in the works! Juliana loves being filmed, and is ready to pose at any time.

—Jeanne ("Party")

Networking at Myrtle Beach
July 11, 2008 11:00 AM

In 1985 there was a movie named Mask, starring Cher, based on a real-life story of a facially disfigured teenager. Since that time, Cher has remained involved with disfigured kids and is the National Chairperson of the Craniofacial Children's Association (CCAKids.org).

Along with other events, this association holds a retreat each year for children with any number of disfiguring syndromes. They just held their 18th Annual CCA Retreat and Juliana was able to attend. It was held at a convention center in Myrtle Beach and was attended by about 85 families. There were group activities, such as a visit to the Ripley's Aquarium on Friday and a trip to the beach on Saturday. In the evenings dinner was in a huge ballroom (three ballrooms with the dividers removed), followed by a talent show on Friday night and a dance on Saturday night. Most of these families have been coming to the retreats for years and have come to know each other already, but it didn't take long for Juliana to make a lot of new friends.

It was the first time Juliana had actually seen and played with other children with trachs, feeding tubes, hearing aides, etc. She quickly befriended a little girl a couple of years younger than herself, pushed her around in her stroller, and even tried to suction her. The girl has her hearing aides on a headband like Juliana does, but her headband is red (Juliana's is black), so Juliana refers to her as "my friend with red ears."

The favorite activity of the two girls was to run—the three ballrooms opened up to a space comparable to three football fields, and they loved to just run from one end to the other. Juliana was watchful though. If she thought her friend was venturing off too far she would come and get her friend's dad, taking him by the hand and pulling him out of his chair. Watching them run reminded me of how my son loved to run at the beach when he was that age. Kids like these don't often get to show their exuberance like that, as they learn not to draw attention to themselves. They love going to these functions where there are so many children like themselves.

On Saturday night the dance floor was full. There was a disc jockey playing a variety of music, including line dances. Juliana would watch the dance for a few minutes, then join right in. She loves to dance.

I was so proud of her at her dance recital a few weeks ago. It was probably the first time she had been more than a few feet away from someone she knew and trusted with the suction apparatus. She had to stand with her group, in the wings, in the dark, waiting for the curtain to go up. Tami was close by, with the suction, but Juliana couldn't see her. She did fine, just what she was supposed to do, followed her little group onto the stage and did the dance steps she had learned. Amazing!

Juliana also loves to play dress up. She has several "Disney" style princess gowns. Sometimes she goes upstairs to get her pajamas on, and comes down with a princess gown over her pajamas. She also loves boots. Once she came down with a princess gown over her pajamas and Kendra's fireman boots on. She's quite imaginative!

Finally, it looks like there actually will be a follow-up documentary. It's in the works! Filming hasn't started yet, though. We'll let you know.

—Jeanne ("Party")

Bags and Baskets
March 25, 2008 5:00 PM

Juliana had her 5th birthday this month. She had a "birthday party" with family and a few friends. She put her presents into one of those large pretty bags which are used for gifts and carried the bag around for days. She loves bags. When I get there in the evening she gathers up some toys and books, puts them into a bag, and brings it upstairs to her room. She wants the bag full of toys in the bed with her. Once she gets in the bed she wants to "multitask." She wants the DVD to be playing, and while she's watching it she also wants to be coloring or reading a book. She'd want me to get into the bed with her but I don't really fit! I kind of lean on the edge of her bed and she lays against me with a hug. She likes to "read" storybooks to me, holding the book and turning the pages for me to see the pictures. After a while she'll settle back against the pillow and soon be asleep.

She enjoyed Easter too. She has an Easter basket which she carries around everywhere. She doesn't eat (she gets her nutrition through her feeding tube), but she likes to taste things. She doesn't really swallow any, but she gets it smeared around her mouth. She tasted an egg and some chocolate. Once she tasted some chocolate cake and signed that she liked "the brown cake."

Juliana's a little "clothes horse." She absolutely loves clothes and shoes. She's going to be in a dance class starting this week and has real tap shoes, which she loves. And she'll love the class! I can't wait! She already loves to dance around. She has a sense of rhythm, even though we don't know how much of the music she can hear. Her hearing is supposed to be close to normal with her hearing aides on, but she's always taken them off for the day by the time I get there. Also, I leave in the morning before she puts them on. It's hard to tell how much she hears of what I say to her. Some things she doesn't seem to hear at all (her monitor alarm, the telephone), but usually if I say her name loudly enough she'll look. We communicate mostly through signs and gestures, but now it's to the point where I need to take a class in signing. I used to be able to bluff her, but that's not good enough anymore.

I do have some good news to report. There was a long time when it seemed like the suture line over her left mandible (jaw) just wasn't going to heal. Dr. Wolf had removed the external screw, but left a piece of metal inside (for a future purpose). In spite of all the care we gave the site, the incision line wouldn't heal. Eventually the metal inside became visible. Then she had an appointment with Dr. Wolf, but came down with a virus, and the appointment had to be postponed. Finally, though, she had the surgery, the metal was removed, and now the incision line is completely healed. She has a scar along her jaw, but that will be revised later. It's just so good to see it healed. It really did have us worried.

—Jeanne ("Party")

Flower Child
January 05, 2008 3:20 PM

I realize people would like more frequent updates. I'm always meaning to send one in, but keep putting it off.

Sometimes my reasoning is "I'll just wait until her swelling goes down" or "I'll wait until she gets over this cold." I don't like to write an update that makes it seem like she isn't doing well. The thing is, there's often something going on, which might be worrisome (such as an infection at her pin site), or might just be a nuisance (such as a cold). It isn't realistic to expect her to be doing great all the time, although most of the time she is. Even when she's fighting off a complication from one of her procedures, she's still doing "great," in that she comports herself exceptionally well. I have written some version of this so many times it seems like I'm repeating myself, about what a good "patient" she is. But it's true.

Juliana has just returned from Miami where she had another procedure done. Dr. Wolf removed the external screw from her jaw and tightened up her mouth a bit. It was done as an "outpatient" procedure—she didn't have to be admitted to the hospital! The family drove down to Miami on Wedneday, the procedure was done on Thursday, and they were home on Friday. You know, even a cut hurts, and anything that's swollen hurts, and stitches hurt, but Juliana just carries on, being herself, playing and being sweet.

I've been wanting to tell you all how sweet she is with her dolls. She has seven or eight Dora dolls, of all sizes. Some of them are little plastic dolls, and she likes to put them into various bags and little purses. At night she gathers them all together and brings them to bed with her. She takes them all out of their containers and has them all kiss each other. Then, if "Dora" is on the DVD screen, she has them all kiss "Dora." Then she settles back on her pillow, ready for sleep.

Christmas morning was fun. This was the first year Juliana actually enjoyed opening her presents. The house was full of relatives, and Juliana loves having a lot of people around. She'd open a gift, then bring it around for everyone to see. She liked handing out presents too. She got a new bike—but still prefers the older one, even though she's getting too big for it! Really, the kids had the most fun with empty wrapping paper rolls, using them for swords. They do like to use their imaginations!

I'm always amazed by how much smarter kids are now than when I was a kid. I know Juliana is smarter than I was at her age, and she loves to learn. Someone once referred to her as "a flower." I'll add "a flower, in the field of possibilities."

—Jeanne ("Party")