Tag Archives: Ehlers-Danlos Syndrome

Distraction 2/2

Walking into the entertainment center I see the jumpy house with it’s bright yellow entrance which matches the yellow of my gurney. Sitting on a bench is a woman holding  a small girl who’s bleeding from the foot. The firefighters walked in maybe 30 seconds before we did. I’m in the more affluent neighborhood of my mostly urban county. The fire crews are more pleasant here as they don’t get run as hard as the inner-city crews. They are quick to break out the bandages and stop the bleeding.

Fortunately I’m able to get a look at the wound before it’s completely covered. It’s a one inch evulsion (skin torn away from underlying tissue) to the top of the small girl’s right foot; muscle and adipose tissue are visible where the skin should be. It looks as though someone just carved away the skin with a knife. The girl, whom I come to find out is named Kimberly, is crying. It’s more of a sad constant cry than a hysterical outburst. She must be quite a mature little lady not to be flipping out right now. Her mother, on the other hand is very distraught; brow furled, talking quietly in her daughter’s ear, she’s holding on by a thread. While inspecting the foot I notice that Kimberly’s pants have a thick quilted lining sewed into them; obviously a modification. I start to ask the mother questions; I want to take her mind off of her daughter as much as I need to understand what happened here.

“I really don’t know, I mean I only turned my head for a second.” Her brow is furled, voice cracking under stress, trying very hard to hold back the tears; very emotional. “One of the other children must have landed on her. She has Ehlers-Danlos Syndrome so she’s susceptible to skin tears.”

There are so many syndromes out there, I may run into the same one every few years or never see one in an entire career. I have to be honest with her. “I’m not familiar with that syndrome. How does it effect the skin?”

“It’s genetic, I have it also and passed it down to her. We’re missing the gene that creates collagen in the skin so the skin and connective tissue have no elasticity; even a simple bump can create a tear.”

Now that she’s focusing on me and not the compounded levels of guilt about her daughter I see that she’s a well spoken, educated woman. She has lived with this syndrome for all of her thirty something years and is fully versed in all of it’s intricacies.

I’m not going to be able to do much for Kimberly other than understand the nature of the syndrome a little better then treat the symptoms. Genetic repair hasn’t made it to the pre-hospital bag of tricks yet. “Are there any bleeding, coagulation, or clotting issues associated with the disorder?”

“No, it’s strictly collagen, it doesn’t effect the blood at all. That’s why I made the padded cloths and protect her as much as I can but they said she had to be bear foot in the jumpy house. I knew I shouldn’t have let her go in but it’s a special day.” She’s doing her best not to loose it in front of her daughter. The firefighters have finished wrapping the foot and Kimberly has stopped crying; just sporadic whimpers as she’s held in her mother’s arms.

Their both on the edge of cracking, I need to keep them focused on me to avoid a negative emotional feedback loop. “Okay, so Kimberly’s foot is protected now that it’s wrapped, how about you let me take you to the hospital? Their going to need to close the wound, either with sutures, or possibly a glue, to protect against infection.”

“No, I’ll drive her myself, God knows, I’ve done it before.”

“So, here’s the thing. She’s perfectly stable right now and I have no problem letting you drive her. But you’re very emotional right now. It would be much better if you had someone else drive so you can take care of her on the way. The last thing I want is to get called to a traffic accident and find the two of you there. Is your husband around or can you call him?”

“No, he’s out of the picture, I’m here alone.” Her non-emotional response tells the whole story. Paramedic opens mouth and inserts foot; news at eleven…

“Then it’s definitely a good idea to let me take you, it will keep the both of you safer and I can give Kimberly some medicine to help with the pain a little.” She agrees with a bit of a relieved look on her face. With all of the issues that she’s dealing with right now at least this is one less thing to worry about and she can focus on her daughter.

Once in the rig I tell my partner that we can start moving; all of the necessary treatment was done on scene by the firefighters. Kimberly is laying on the gurney in the back of the rig with mom sitting next to me on the bench seat. Kimberley is still letting out a soft stream of whimpers.

While I’m explaining to mom that I can give Kimberly some morphine for the pain I reach up to the front of the rig and grab my iPad. I don’t like to dope kids when their parents are present without a good explanation. I’m planning on using the iPad to keep Kimberly busy while I draw up the morphine and explain the effects to mom. I launch an app and hand it to Kimberly, after surreptitiously checking for blood on the hands.

“Kimberly, this is Talking Carl, say hello to him.” She looks at the screen to see an animated character waving at her. He looks like a short fat sibling of Gumby.

In a tentative voice, “Hi Carl.” Two seconds later in a slightly higher pitched voice Carl’s mouth moves and he mimics Kimberly, “Hi Carl.” bobbing back and fourth he waves at Kimberly. She starts giggling, “Mommy, he talked to me!” two seconds later Carl starts giggling and moving his mouth and in his exaggerated high pitch voice, “Mommy, he talked to me!

This goes on for ten minutes of giggles and laughter as we drive to the hospital. I show her how to tickle Carl on the screen and Carl starts laughing then she laughs and Carl mimics it and it starts all over again. It looks like we just created a positive emotional feedback loop. As Kimberly is now busy and mom is actually starting to smile for the first time since I met them I have a minute to look up the syndrome on my iPhone. It’s exactly what mom described.

With Kimberly fully engrossed in playing with Carl I see a relieved look on mom’s face and we have a few minutes to talk. Quietly so as not to distract her daughter mom confides in me. “I didn’t want to take off her shoes but they said she had to be bear foot in the jumpy house. I just wish I could wrap her in bubble wrap for the next ten years..” She’s conflicted with emotion; she wants to protect her daughter but at the same time she wants to let her explore, have fun, and be a normal child. She remembers her own childhood all too well and wants to do good by her daughter.

I’m pulling out my iPhone again, “Have you ever seen the shoes that martial artists wear to protect their feet when they spar? It covers the top of the foot with a half inch of foam dipped in a rubber coating. It’s bare foot on the bottom so it might be okay for the jumpy house.” I pull up a picture on my phone and mom sees how it may be applicable to her little girl. It’s not bubble wrap but it’s a good idea. We spend a few minutes browsing the site and looking at the different protective padding options that may be applicable.

As we arrive at the ED they are both a little more relaxed. Walking into the room, pushing the gurney, to get Kimberly settled in the bed I realize that I actually had two patients on this call; Kimberly with her foot and her mother with her anxiety. I didn’t lay healing hands on either of them nor did I open up the drug cabinet or perform any paramedic skills. Rather I treated with compassion, conversation, laughter, and a little distraction.

Distraction 1/2

dis·trac·tion

1 : a thing that prevents someone from concentrating on something else

2 : the act of distraction or the condition of being distracted

3 : something, especially an amusement, that distracts

4 : extreme mental or emotional disturbance; obsession

Kimberley always new she was different, ever since she was young. Now that she’s older she’s starting to understand it better. Mommy says that she can get hurt easier than the other kids so she has to be extra careful. But this is a special occasion; today Kimberly turns six years old.

Kimberly has her padded pants, her thick long sleeve shirt, the squishy headband around her head, and a big smile on her face as she enters the jumpy house. All of her friends from school are here; brightly lit faces and laughter as the primary colors of the inflatable castle illuminate everyone in a surreal light. The smell of kettle corn and pizza speak to the treats that await them when play time is over.

Outside the jumpy house Kimberly’s mother nervously talks to the other parents as the inflatable castle deforms from the constant jumping of the kids inside. She had reservations about bringing Kimberly to this place today but she wants to let her beloved and sheltered little girl have a special day. She paces outside the jumpy house, holding her breath at every fall.

She carries the weight of the world on her shoulders. Being a single mother of a fragile little girl she is on constant vigilance for Kimberly’s safety. She carries the guilt for her contribution to Kimberly’s condition and the roll that it may have played in her father taking himself out of her life.

One of the other parents brings her a soda as they all know she wants to stay close to the jumpy house. As she turns to accept it the smile of gratitude turns to alarm as she hears the sound of Kimberly cry out in pain. It’s a primal instinct that all mothers are programed to respond to; the sound of their child in distress. Spilling the drink on her shirt as she rushes to the clear observation panel of the jumpy house she knows what what to expect from her own experiences.

Laying in the corner of the jumpy house is Kimberly. Blood smears the bright yellow inflatable fabric, children scatter away from her daughter as they don’t understand what’s happening. How can such a happy experience go bad so fast? Kimberly’s mother knows exactly how it can happen; from personal experience and from the lengthy explanations from the geneticist.