Sunday, April 15, 2012

The long, hard road with EB

Many of the children we care for have tragic stories. They were born with birth defects or illness that will affect them for life, separated from their families, and endure more surgeries and treatments than you or I could imagine. But perhaps the most heartbreaking of all are the the children who were born with epidermolysis bullosa, usually abbreviated "EB".

You may never have heard of EB. Many doctors and nurses can work an entire lifetime and never see a case of EB. EB is a relatively rare genetic skin problem, meaning that some children are born with it, and there is no cure. Children born with EB are lacking a critical protein in the skin that anchors the layers of skin to the body and to each other. Without this vital protein, the lightest touch causes skin layers to blister and peel off. The wounds of EB can be compared to second and third degree burns, and they can affect any part of the body, including the mouth, throat and internal organs.

As you can imagine, children suffering from constant wounds of this type are in unending pain. They are also susceptible to dangerous infections. Normal things, like the tag on a shirt, the wrinkle on a crib sheet or blanket, learning to crawl or walk, can cause serious wounds to babies with EB. Bath time is not a fun time for bubbles, rubber duckies and splashing; it's an important part of cleaning and dressing wounds, but can be torture for these children.

The only things you can do for a child with EB is try to help them manage the pain, keep the wounds clean, and change the dressings every day. Even then, most children suffer scarring and disfigurement; many children lose fingers or toes or the use of important joints.

As you can imagine, children with EB require incredible amounts of medical supplies, dressings and bandages to cover existing wounds and try to prevent new ones from forming. We are grateful to all of our donors, especially Medwish International for helping us with these!

We'd like to introduce you to two very special little girls, Wen and Jie.

Jie just arrived at Little Flower last week. She celebrated her first birthday last month, but she's anything like a typical one year old. She's the same size (6 lbs) and developmental level of a newborn.

Little Jie has never known happiness or satisfaction; her short life has been nothing but discomfort. Her mouth is full of blisters, so feeding and sucking are not associated with pleasure, as they are for most babies.

She associates her caregivers with painful wound treatments and because of this is very withdrawn and upset by human contact. Even a soft word spoken quietly near her bed is enough to make cover her face and cry.

Every calorie and nutrient she takes in has gone to healing her serious wounds, so there hasn't been any left over to help her grow. Because her previous caregivers were not taught the latest techniques in wound treatment, how to bandage and do physical therapy, she's already lost most function in her legs and feet.

When it's time for her dressing changes she cries in terror and beats her head to distract herself from the pain. It's truly heartbreaking to care for her. What is most distressing is that there is not much we can do to make it better. She won't get better; this is the life she has to look forward to. Her nannies break down and cry too; it's one of the hardest things in the world to care for a baby with EB.

Little Wen is 18 months old, but she was lucky enough to receive very good care before she came to us. She's growing and developing more normally, starting to walk and talk like any other toddler.

Wen has a hard time with her wound treatments too, but she responds to her nannies and they are often able to distract her with songs and games and silly faces.

She also loves to play with cell phones, so that is a great distraction when doing painful dressing changes.

Even so, it's not always fun and games; there are days when her caregivers shed as many tears as she does.

It's never easy to watch little ones suffer, but fortunately, most of the time we know that it's temporary. We are able to help most of the babies we care for, and they do recover from their illnesses and surgeries. We have that hope, that future to look forward to; it carries us through the hard times. But it's a long, hard road for our sweet babies with EB. Unless a cure is found, there is no bright future ahead. And that makes this one of the hardest jobs on earth. Please pray that researchers will find a treatment or cure for EB. And pray for the nannies who care for these children. They need as much strength and support as possible to continue caring for these precious little ones, sharing happiness and hope with those who have none.

Just five days...

Little Zhi is 9 months old and was born with a severe heart defect. He's been in the hospital recently, but when he was ready to discharge, his orphanage asked if we might be able to help nurse him back to health. If he can grow bigger and stronger he would be a candidate for surgery to repair his heart defect.

When he arrived on Monday, we were very discourage to see how thin and frail he was. At 9 months he weighed just 7 lbs. We started to worry that he might not live long enough to qualify for surgery. Unfortunately, that is a reality for many babies we accept for care.

On Friday, just 5 days later, he did look better. His little face was starting to fill out and when we put him on the scale, we were amazed to find that he'd gained 490 grams! (That's over a pound, for you metrically challenged Americans!) His photos on Friday speak volumes:

We don't know what the future holds for little Zhi, and whether he'll ever be chubby, happy and able to have surgery. Right now he's still very unstable and we are taking it just one day at a time. That's all we can do. But even in five short days, we can see the difference made by good nutrition, a high standard of nursing care, and lots of kisses and cuddles. Small successes like this are what keep us going -the very best motivation and encouragement anyone could ask for. Whether this little guy lives another 5 days, 5 weeks, or 50 years, we know we've made a difference. What more could we ask for?

UPDATE:  Zhi passed away on April 22, just two weeks after he arrived.