Wednesday, March 30, 2011

Ileana Peralta's Story

“It’s the worst skin disease that I know of,” he said. “This disease just cries out for help.” - Dr. Alfred T. Lane, a pediatric dermatologist


“I just try to get through it, the best you can, no matter how much it hurts...” - Ileana Peralta






It wasn’t until Ileana Peralta was in junior high school that she summoned the courage to Google her own disease. The teenager from Livermore knew almost everything about her inherited condition, Epidermolysis bullosa, a tongue twister even doctors call just EB. The disease is caused by the lack of the collagen gene that makes the glue that tethers layers of skin together. It makes her skin seem as fragile as a butterfly’s wing.

Ileana and other children with EB go through life with much of their bodies wrapped in gauze, like a burn patient. Their skin, at the slightest trauma, is prone to form blisters, which are vulnerable to infection. Scarring eventually curls fingers and toes, fusing them. To varying degrees, these children are condemned to a life of pain and disfigurement.

EB is rare. Ileana’s severe form of it, called recessive dystrophic, affects between one and four per one million in the United States, said Dr. Alfred T. Lane, a pediatric dermatologist. Dr. Lane was recruited to Stanford University Medical School 19 years ago to search for treatments for EB, and has been working on it ever since.

“It’s the worst skin disease that I know of,” he said. “This disease just cries out for help.”

The handful of doctors in the United States who deal with EB are experimenting with some of the most advanced technologies in medicine — gene therapy, stem cell therapy and bone marrow transplantation — as a means of eventual treatment.

Last fall, the California Institute of Regenerative Medicine, the state agency that runs the voter-approved $3 billion stem cell research program, allotted $11.7 million to Stanford to find a way to harness the newest stem cell technologies against EB. The goal is a clinical trial by 2014.

Under Dr. Lane’s direction, the California stem cell money will pay for an approach using new techniques that transform ordinary skin cells into all-purpose stem cells. In theory, these stem cells can be engineered to carry the missing collagen gene, and can then be coaxed to grow large amounts of healthy skin grafts, immunologically compatible with each patient, like Ileana.

When Ileana trolled the Web for information about her disease, she discovered that children who have it may not survive to adulthood. “The part that scares me is that some kids died from it,” she said. “I’m glad I’m still here.”

For most of Ileana’s life, Dr. Lane’s team has focused on developing gene therapy. The idea is to implant the missing gene — for a protein called collagen VII — in skin grafts. The chainlike collagen proteins, absent or damaged in people with EB, anchor the top layer of skin to the underlying dermis.

Engineered viruses would ferry the missing gene to laboratory cultures of the patient’s skin, to be grown in sheets and later grafted. A clinical trial among adult patients is pending.

The latest technique, sometimes called reprogramming, holds the promise of sidestepping the troublesome issues surrounding the use of embryonic stem cells — controversial because they are derived from human embryos that are destroyed a few days after fertilization.

Reprogramming seemed like science fiction until 2006, when Dr. Shinya Yamanaka proved it could be done in mice. In 2007, Dr. Yamanaka and other researchers showed it could be done with human cells.

Reprogrammed cells are currently too dangerous for use in human studies, and a variety of hurdles must be cleared before such studies can happen. One study recently found, for example, that reprogrammed cells have a more limited lifespan than stem cells derived from embryos.

On the other hand, researchers are rapidly fine-tuning the mechanisms of cellular reprogramming, making it simpler, more efficient and better understood. The field is now among the hottest in molecular biology.

More controversial is an entirely different approach using bone marrow transplants, which swap a patient’s own marrow with those of a healthy donor. The idea is to treat EB as a disease of the whole body, rather than one that can be repaired with skin grafts. EB affects not only the skin, but also weakens mucosal tissues in the mouth and gut, which cannot be grafted.

Dr. John E. Wagner Jr., a pediatric cancer researcher at the University of Minnesota Medical School, has done bone marrow transplants on seven EB children after studies on mice that showed that a select group of bone marrow cells could migrate to the skin and, for reasons not fully understood, replace missing collagen.

Four of those patients “are having improved quality of life,” said Dr. Wagner, who has yet to publish his pediatric results. But two of the children died — one from infections five months after an initial transplant, and the other during chemotherapy, before healthy bone marrow from a donor could be transplanted.

Dr. Wagner agonizes over those deaths, but is convinced that the disease was so severe that it warranted the risk.

“I normally take care of patients with leukemia,” he said, “but then I think there are some things worse than leukemia.”

Dr. Wagner’s work is backed by DebRA, the Dystrophic Epidermolysis Bullosa Research Association of America, as well as the Epidermolysis Bullosa Medical Research Foundation, two charitable organizations founded by parents.

Dr. Lane of Stanford has reservations about the bone marrow procedure. “For a child with large wounds,” he said, “you are really setting them up for infection.”

Dr. Arthur Caplan, a bioethicist at the University of Pennsylvania, is also uneasy about conducting risky procedures on children.

“The sicker the children become, the harder it is to secure good, informed consent,” Dr. Caplan said. “In my experience, parents become desperate. They want to have hope, and often researchers want to offer hope.”

For her part, Ileana said she was in no hurry to be a guinea pig. “I probably want to wait until they know what they are doing,” she said.

Now 15 and a freshman at Grenada High in Livermore, Ileana confronts her disease with a combination of pluck and a straightforward attitude befitting someone considerably older. She is as matter-of-fact about everyday life as she is about her disease. She confesses to struggling in math at school and far prefers drawing. Her sketchbook is full of cartoon bats and characters like Jack Skellington from “The Nightmare Before Christmas” and SpongeBob SquarePants.

Ileana knows the sting of EB every day when her mother, Stephanie Peralta, bathes her and wraps her in gauze. It can take 45 minutes to whirl the yards of white cotton bandage around her arms, her legs and her stomach. Some days the sores hurt more than others. Some days they hardly bother her at all.

Bandaging Ileana is a labor of love for Ms. Peralta, a 31-year-old single mother, who lost her job at a freight forwarding company when the recession hit. She was 16 when she became pregnant with Ileana. Doctors knew when Ileana was born that something was wrong: on her right leg, there was no skin from the knee down.

“I was a teenage mom with a sick child,” Ms. Peralta said. “I grew up in a hurry, and just dealt with it.”

Ileana appears to have inherited her mother’s strength. “I just try to get through it, the best you can, no matter how much it hurts,” she said.

When she goes outside, she knows people sometimes stare. “If they’re curious, they’ll ask,” she said. “It’s O.K. to ask.”

Recently she attended a party, and met a girl whose mother has cancer. “I asked her about it,” she said. “Pretty soon we were swapping stories.”

Source: http://www.nytimes.com/2010/03/14/health/14sfblister.html?_r=1&emc=eta1

Monday, March 28, 2011

2nd Cannell Auction: Couture Bridal Rosette Hair Comb Fascinator




Our second auction item will be a Couture Bridal Rosette Hair Comb Fascinator.

A handmade rosette is arranged in this gorgeous bridal comb. A pearl peaks out from the "petals," and wispy feathers, lace, and netting accent the piece beautifully.

Everything is securely attached to a comb. The dimensions are about 4.5"x4.5" including the netting. Perfect for a special evening out or for attaching to any type of veil.

This item was donated by Tricia at Hope, Wishes & Butterfly Kisses Boutique HWBKBoutique.etsy.com

The auction begins Monday (3/28) at 8am PST and runs through Wednesday (3/30) at 8pm PST.

This auction is to benefit the Cannell Family in their adopting Carson and John. Find their story here: http://bringingcarsonhome.blogspot.com/

•Bidding
There is a photo album on facebook set up with the pictures of the item.
You must comment on the PHOTO ALBUM to place a bid on the item. Please make sure that you look at all bids on an item before you enter yours and make sure that it is HIGHER than the highest current bid.
•Payment
If you win an item, payment will be made through paypal. At the end of the auction, I will send the winner an email with further instructions on how to pay. After the email is sent, payment is due within 48 hours. If your payment is not received in that time frame, the item will be offered to the next highest bidder. At that point, any payment made by the original high bidder will be considered a donation and they will not receive an item in return.
•Other donations
If you would like to make a donation please visit http://reecesrainbow.org/sponsorcannell
•Shipping
We do not require that you pay for your own shipping.
•Other questions
If you have any other questions, please feel free to post them here or email them to me at plbushnell@gmail.com.

Friday, March 11, 2011

{Wise Words on Parenting}



"Cleaning and scrubbing will wait ’till tomorrow, but children grow up, as I’ve learned to my sorrow. So quiet down cobwebs! Dust go to sleep! I’m rocking my baby and babies don’t keep."
~ Ruth Hamilton


“You don’t really understand human nature unless you know why a child on a merry-go-round will wave at his parents every time around – and why his parents will always wave back.”
- William D. Tammeus


"Don't worry that children never listen to you; worry that they are always watching you." ~Robert Fulghum


“To be in your children’s memories tomorrow, you have to be in their lives today”
- Anonymous


“Too often we give children answers to remember rather than problems to solve.”
- Roger Lewin


"The quickest way for a parent to get a child's attention is to sit down and look comfortable." ~Lane Olinghouse


"You will always be your child's favorite toy."
~Vicki Lansky, Trouble-Free Travel with Children, 1991


“What a child doesn’t receive he can seldom later give.”
- P.D. James


"A child enters your home and for the next twenty years makes so much noise you can hardly stand it. The child departs, leaving the house so silent you think you are going mad."
~John Andrew Holmes


"The trouble with being a parent is that by the time you are experienced, you are unemployed."
~Author Unknown

Tuesday, March 8, 2011

Cannell Auction Item #1: Handmade Butterfly Necklace









For our first auction we will be auctioning a one-of-a-kind handmade butterfly necklace.

Beautiful hand blown glass butterfly in deep garnet red & cobalt blue float on three strands of glass beads & Swarovski crystals in black, garnet & sapphire. Necklace measures approximately 18" with a 1" extension chain.

This necklace was donated by Tamara at JewelryByTam.com

The auction begins Monday (3/14) at 8pm PST and runs through Sunday (3/20) at 8pm PST.

This auction is to benefit the Cannell Family in their adopting Carson. Find their story here: http://bringingcarsonhome.blogspot.com/

•Bidding
There is a photo album on facebook set up with the pictures of the necklace.
You must comment on the PHOTO ALBUM to place a bid on the item. Please make sure that you look at all bids on an item before you enter yours and make sure that it is HIGHER than the highest current bid.
•Payment
If you win an item, payment will be made through paypal. At the end of the auction, I will send the winner an email with further instructions on how to pay. After the email is sent, payment is due within 48 hours. If your payment is not received in that time frame, the item will be offered to the next highest bidder. At that point, any payment made by the original high bidder will be considered a donation and they will not receive an item in return.
•Other donations
If you would like to make a donation please visit http://reecesrainbow.org/sponsorcannell
•Shipping
We do not require that you pay for your own shipping.
•Other questions
If you have any other questions, please feel free to post them here or email them to me at plbushnell@gmail.com.

Monday, March 7, 2011

We will be helping to bring Carson home!!

Hope Wishes and Butterfly Kisses is going to help bring Carson home! We will be doing a number of auctions over the coming weeks. Our first one will be announced soon!

Follow Hope Wishes and Butterfly Kisses on facebook for all of the details!!

Hope Wishes and Butterfly Kisses - FB

Creating a Peacful Home: Our Routine



My routine makes me feel satisfied and organized. Daddy's feel that their home is peaceful. And children thrive on it. We need a good stucture so we can maintain our lives and relationships.

This is not about perfectionism.

But, this can be one of the hardest things for a family to figure out. So, I am here to give you some tips and show you my own personal routine.

I keep some leeway in my plan to stay flexible, and adjust for spontaneous outings, unexpected guests, etc, while still maintaining the basics.

Some projects are done daily, as maintenance, while others are done just once during the week, to save me time later.


Morning/Evening Routine

I use a morning/evening routine. This kind of structuring provides simplicity and flexibility. Your morning tasks can be completed sometime between 8:00 and noon and your afternoon/evening tasks before you go to bed.
Here is what an average day looks like at our home:

Our Daily Morning Routine

I complete these before Ashton wakes up and before the daycare kids show up. I don't have laundry to do every day, so some days that step does not apply.

  • Make Beds

  • Put load of laundry in washer

  • Unload dishwasher (this makes it to where you can put dishes in the dishwasher throughout the day)

  • Switch laundry to dryer

  • Fold and put away laundry


  • I do have variations from day to day. Each day of the week I focus on a specific area of our home.

    Monday:
    Stove top meal prep for the week

    Tuesday:
    Deep clean bathrooms

    Wednesday:
    Deep clean kitchen
    Wipe down bathroom and change hand towels
    Coupon clipping and start weekly meal plan

    Thursday:
    Baking Day

    Friday:
    Dusting, vacuuming, mopping, etc.

    Saturday:
    Quick "wipe down" of the bathroom and put out fresh hand towels
    Finish weekly meal plan
    Clean out fridge: throw away old food, wipe down shelves
    Go shopping either today or Sunday

    Sunday:
    File receipts (for personal and business)
    Catch up day for anything that was not finished Saturday

    Afternoon/Evening Routine

  • Finish cleaning up from dinner and loading dishes in dishwasher while Daddy plays with Ashton (make sure to grab dishes from any other rooms in the house)

  • Run dishwasher

  • Wipe down counters

  • Tidy up living room (have children put all toys away and put all dirty clothes in laundry basket)

  • Read children Bible story before bed

  • One on One time with Derik



  • I hope all of this is as helpful for you as it has been for me.

    Enjoy!

    Friday, March 4, 2011

    The Bushnell Home: February

    What a great month! Our anniversary was on the 3rd and then Valentine's Day on the 14th. Also, all of the things I have been implementing into our daily lives has really been working out beautifully! I am so excited to share it all with you.

    For our anniversary we went to the coast. We rented a nice little cabin near a creek. The ocean was close by, so we were able to take a short little hike to the beach. On our hike we went through a tunnel. It was 780 ft long!! It was pretty crazy, but way fun! It was such a nice relaxing weekend. I look forward to when we go again. Next time we go will be nicer weather so we'll take Ashton and let him play on the beach. Here are some pictures of our weekend...








    On Valentine's Day Ashton and I made Daddy a card and some cookies. It was fun. I love homemade gifts. Here's Ashton and a couple friends making their cards...



    So, since the beginning of the year I have been following a daily/weekly/monthly routine. It has been going so well!! I love it! Our home has been clean and tidy and we have time to spend with eachother. I don't feel overwhelmed because I have a kitchen full of dirty dishes, 10 loads of laundry, and can't see my floors anymore! It is so freeing to have everything done. Are you interested in knowing what my routine looks like? Stay tuned because I will be posting it in detail.

    Do you follow a routine in your home?

    Thursday, March 3, 2011

    Help Payton Thornton: Extreme Home Makeover



    It’s already been a year of miracles for Payton Thornton.

    Against all odds, the 5-year-old White Plains boy was allowed to come home in late December, just six months after receiving a stem cell/bone marrow transplant at the University of Minnesota Children’s Hospital.

    Payton suffers from a rare genetic skin disorder called Recessive Dystrophic Epidermolysis Bullosa (RDEB). Doctors believe the transplant could well save his life, and all indications are that Payton is continuing to improve.

    But after returning home, the family discovered a potentially dangerous mold in their household air, being circulated by the air-conditioning unit. Payton’s immune system was weakened by the transplant, and the fungus, aspergillus, could cause a serious infection. Continued...



    Read more: Anniston Star - White Plains miracle kid faces new obstacle - a toxic house

    ***Extreme Home Makeover***

    To vote for the Thornton family:

    1. Go to www.abc.go.com

    2. Scroll to the bottom of the page and click “Contact ABC”

    3. (1st Section) Enter Your Personal Information

    4. (2nd Section) Beside “Select a Show or Category,” choose “Extreme Makeover: Home Edition”

    5. Beside “Subject,” choose “Comment”

    6. Beside “Message,” type “The Thornton Family, White Plains, AL” and state the reason you feel that they are deserving.

    7. Click “Send”

    Baby Butterfly: Jameson Setsma



    Remembering Baby Jameson today. It has been 2 years since he got his Butterfly Wings.

    Read his story here:

    http://jtsetsma.blogspot.com/

    Wednesday, March 2, 2011

    Cannell Family: Bumps in the Road

    The Cannell Family has hit some bumps in the road on their journey to bringing Carson home. Luckily they have God on their side and are moving forward with faith and strength. This is such a beautiful family that has such a loving heart and an open home to this precious little boy. Please keep them all in your prayers and consider helping them bring Carson home with a donation or by purchasing one of the lovely items that has been donated for fundraising.
    More info here: Bringing Carson Home: Waiting...
    Online Fundraiser Info: Carson Fundraiser

    Update on Charlie Knuth: After Stem Cell Transplant




    Charlie is doing great and is starting to be able to do more and more things. He is feeling much better, his skin is clearing up, and his spirits are high. Pretty soon he'll be tossing around his football with his dad and brothers. His Mother is so happy to see him doing so well. He is very thankful and you can just see all of the joy in him and everyone surrounding this sweet little boy.
    We are continuing prayer for Charlie and his family as they are still in the recovery phase. Much love goes out to them all.