My Wonderful Walls Blog — Rare Diseases
Ella Lives Each Day to the Fullest in Her Garden Themed Bedroom 13
For most of our customers when planning a room mural for their children or grandchildren, their thoughts are of how wonderful it will be watching the child grow with the room. For many they pick out the design they did for the simple fact it can last through babyhood into toddler years and beyond. But for one family, raising a medically fragile child means just taking each day as it comes and making the most of it. Recently, we learned of the struggle one of our young customers in Massachusetts has been dealing with and how her grandparents wanted to give her an over the top room that would give her a special space to play and rest.
Penny and her husband Chris raised five children together including two that they adopted from Russia. When one of their daughters was unable to raise her own daughter, Ella, Penny and Chris gained guardianship and later adopted Ella. Unfortunately, the road for Ella has been a rough one since birth. When Ella was born and still in the hospital, she was misdiagnosed with an eye condition called Aniridia later in 2015 they found out it was actually Congenital Mydriasis. In this condition the iris muscle doesn’t contract so the eye stays fully dilated at all times. At three months old after a period of being very irritable and not eating much Ella underwent an endoscopy but during the procedure went into total heart failure. The next day she was moved to Children’s Hospital in Boston. They found a giant hole in her heart called an AP Window. A few days later, once they stabilized her and had a plan, the doctors performed open heart surgery.
About two years later Ella was also diagnosed with severe sleep apnea. But after surgery to remove her adenoids and shave down her tonsils, she seemed to be doing much better and began to thrive. That all changed on Saturday September 12, 2015 at 3:30 in the morning. The night before, Ella had a small fever and Penny treated it with alternating Tylenol and Ibuprofen. Ella seemed to be in good spirits when she awoke, still feverish during the early hours Saturday morning. She was excited by the thought of being up with a snack, juice and a movie in the middle of the night. A few minutes later, Ella told Penny she was going to be sick and as Penny brought her into the bathroom, Ella collapsed. Penny quickly realized Ella was paralyzed on her right side and couldn’t speak. She knew Ella had had a stroke.
After treatment both at their local hospital and Children’s Hospital, it was both confirmed that Ella had a stroke and suspected that she had a genetic mutation causing Multisystemic Smooth Muscle Dysfunction Syndrome. Later a blood test would prove that was the case. Unfortunately, Ella’s condition has been progressing. Her right coronary artery is blocked 100% and her left is partially blocked making it necessary to have brain bypass surgery. Penny and Chris have been told if the disease continues to progress like this, Ella could start to stroke again. They know that this could happen as soon as tomorrow or maybe in a couple of years. With so little known about Multisystemic Smooth Muscle Dysfunction Syndrome, the doctors simply do not have a lot of answers. Penny and Chris and their entire family hold out hope but are also realistic. Ella will be having a brain angiogram before the first of the year to give her doctors better imaging and more answers hopefully.
Even in the midst of all this, Ella is a beautiful, happy little girl. Penny says that Ella has deep understanding and empathy for others. She makes friends quickly, is patient and accepting of children who find it hard to fit in. She loves to play dress up, princess and tea party. Penny and Ella spend a lot of time doing artwork and hanging out in the garden or the kitchen. Pink is Ella’s favorite color and she loves anything covered in glitter or that sparkles. She recently started her second year of Pre-K and is overall doing well.
Penny wanted to create a space that allows Ella’s imagination to run free and that was inviting for others to join in and play. Since Ella has a lot of restrictions on her activity because of her condition and being on blood thinners, she misses out on some things a healthy little girl usually gets to take part in. Penny wanted her to have a space that would let her be a princess one minute and a mom feeding a baby the next. She wanted Ella to have room to read and rest or sit to do crafts and a theme to make it cohesive. When Penny searched for flower stencils on Google, she found My Wonderful Walls and was happily surprised to find out we also sold wall stickers. The fact that she wouldn’t have to paint a flower garden for Ella won Penny over. There are lots of special touches around the room like the loft bed with a play place for Ella underneath and a My Wonderful Walls personalized pillow that matches Ella’s garden decals. Penny even purchased some extra stickers to really go a bit over the top when decorating.
The only trouble Penny had with using the decals was when she attempted to apply the large tree decals to the walls alone. She told us, “I was advised when I purchased the set, to get help for the trees. But me being me couldn’t resist attempting it on my own. At which point I created a big mess of branches sticking to one another. But great news if you should too decide to become an idiot like myself, it was easy to unstick the branches from themselves and onto the wall.” Penny reports that the decals look like they’re painted on and that you can’t really tell they are decals. She has had nothing but compliments.
Just talking to Penny briefly it is easy to see how much she loves Ella and how educated she has become on a very rare disease. There are less than 40 people worldwide with the genetic mutation that causes Multisystemic Smooth Muscle Dysfunction Syndrome. This particular syndrome effects all of the smooth muscle. The bowel, bladder, lungs, eyes, heart, brain and vascular structure. Every patient is different and only some of these patients have the brain complication such as Ella.
Penny said it best when she told us, “We all are predisposed to believing we will have a long healthy life in front of us and live in that illusion. Some of us dream that we will get married, have children, watch those children grow into beautiful beings and start their own families. Yet there are a million sayings about tomorrow not being promised. My husband Chris and I, have put aside our expectations of what the future will look like and live free in the moment. For we are truly blessed for TODAY!”
For more information about this syndrome and in particular the risk for Aortic Dissection and Aneurysms, check out The John Ritter Foundation page.