When I was in grade school (150 years ago) there was this friend of mine…a foreign exchange student…who could fold both of his legs behind his head in a very “impressive” nature. I remember how “freaky” he looked all folded up like that. There he was standing at 4 ½ feet and could twist himself down to about 2 ¼ feet. Alex was wicked smart too. We never needed our Dungeons and Dragons books (yes, I was a huge nerd); Alex knew all the rules and our games were as easy as a piece of loose-leaf paper, some dice and our imagination. Yes, Alex was normal in every way with the exception of his crazy double-joined-ness…
But there is no such thing as being Double Jointed
No. Indeed, there is no such thing (that I’ve heard of anyway). Imagine a child with two elbows on the right arm. That term: “double-jointed” actually refers to those persons who are especially flexible and not that they have two of the same joint. The “double-jointed” person is actually demonstrating hypermobility of their joints as they extend their range of motion beyond (sometimes well beyond) that which is normal (and sometimes freaky!).
The Anterior Cruciate Ligament (ACL), for example, is responsible for limiting the knee to a safe and effective range. An ACL injury may require a brace or a wrap in order to “hold things together” providing the added support during the person’s recovery process. I am using the ACL as an example because it’s an area that is often injured during activity when our knee may be “pushed” beyond its limitation in regards to its normal range of motion. When the ACL is injured, the pain is usually a motivator to take it easy until the healing process is finished. But, sometimes we don’t want to wait (I know I don’t). So we wear a brace to “support” the joint while the ACL is “taking a break.”
However, in a way, the ACL is the brace…for the joint. It limits the range of motion of the knee to a point where injury would occur to the knee if the range was extended too far. The “human anterior cruciate ligament (ACL) is made up of wavy bundles of collagen fibres arrayed in various directions.” This works like a “brace” for the knee. And this is just an example; all of our joints are supported by collagen-based ligaments and connective tissues. Very tough stuff! As a matter of fact, collagen molecules are five to ten times stronger than steel! MOVE OVER IRON MAN!
You’re Supported by the Triple Helix of Collagen
About one quarter to a third of all of the protein in your body is collagen. Collagen is tough stuff that your body creates from other proteins (like your chicken sandwich lunch), then breaking it down into simple parts (amino acids) and then forming these tough, molecular, cord-like structures that give strength to tendons, skin, muscles, internal organs, blood vessel walls and other stuff. Kinda like rebar in concrete. We’d be mighty unhappy with a lot of our “parts” if they weren’t supported by collagen. These supportive ropes of protein (collagen molecule) measure 1.6nm wide by 300nm long (that’s about 0.0003mm long). They are made up of about 1400 amino acids (giving the molecule length) and are made from the twisting and repeating sequence of only three different amino acids that form a sequential pattern (about 1400 aminos long). Think of the molecular “fiber” like a wire-woven cable.
Collagen is very tough stuff…normally…and that is where we come to Ehlers-Danlos Syndrome.
What Is Ehlers-Danlos Syndrome
Before I get started with my blabbering, I want to share with you a paradigm that will help you take care of the child with Ehlers-Danlos Syndrome. The mamma or daddy of a child with EDS is an expert; I call them the “expert” patients. Unless EDS is your specialty, the parents will have the equivalent of a PhD in how their child responds and the needs of their child specifically related to Ehlers-Danlos Syndrome. That parent will be a much better historian and health needs information-provider than the doctor’s H&P will ever be. Yes, that H&P is very important and the doctor’s direction is important, but the parents’ information will be vital to create an individualized plan of care and an awesome school year. This is Nurse Kevin’s opinion and is the same opinion I have for all illnesses…especially the rare illnesses you see once or twice in your nursing career. That mamma and daddy see it every day. They are indeed the definition of an “expert” patient.
The most common findings with EDS are (Classical EDS):
There are 13 subtypes of EDS each with its own specifics. However, the cardinal signs of EDS are:
Blood Vessel Fragility
Characteristic Subcutaneous Nodules
Smooth, velvety skin that is stretchy and bruises easily
Abnormal wound healing that may result in wide, atrophic scars (flat and/or depressed scars)
Joint hypermobility that leads to frequent dislocations and subluxations (partial dislocations)
Calcified hematomas over pressure points such as the elbow
Fat-containing cysts that are often found on the forearms and/or shins
Decreased or low muscle tone. This can cause the person to seem “floppy” and not be able to move as well as expected.
Delayed motor development
With skin hyperextensibility, you will usually see the child with very unusually stretchy skin that seems to be able to “stretch” farther away from the body than normal. Kinda like a shar pei puppy (but the person will not look all wrinkly and cute like the dog…though they may be cute…but…I digress). Another example will be like your elbow skin when your arm is stretched out straight. The person with EDS and the symptom of skin hyperextensibility will appear to have skin that will stretch far away from the body but is not all wrinkled and may not even be loose on the body.
Stretchy skin may not be a bad thing in and of itself, but the condition that allows for the hyperextensibility of the skin comes with some caveats such as bruising easily and poor ability to heal.
Cigarette paper scars? What’s that? What is seen after a skin injury in people with the classical form of Ehlers-Danlos syndrome is like most everyone else: a scab forms. But, the skin has poor healing ability. A scab may form and subsequent budding and initial healing. And, due to the life we live in our skin, the wound may split a bit when pulled upon due to the lack of forming sufficient connective tissue. Now there’s a wider wound bed. Eventually the skin will heal (hopefully) and the scar will be wide and the skin will be paper-like…creating the characteristic “cigarette paper” scars. You will likely see a “cigarette paper” scar on a non-EDS person on the elbow where the wound may split during healing due to the normal stretch-then-relax nature of the skin over the elbow area.
Abnormal Wound Healing
We’ve already talked a bit about the person’s skin and the lacking ability to effectively heal. What about more significant wounds? It’s one thing to get a skin scuff or a skin cut, but what about a more significant wound from things like trauma or even surgery? We will not dive too far into this topic beyond what we’ve discussed already. But, we will discuss some issues to consider specifically for the awesome school nurse (See Below).
Like my friend Alex, there are many children who appear to be “double-jointed.” This is likely due to the young, fresh, bouncy, and bendy flesh of a young person (trust me…they’ll be stoved up like poor ol’ Nurse Kevin soon enough (I can pull a muscle just putting on my underpants (TMI…sorry))). Then there are our kiddos with Ehlers-Danlos syndrome.
Bleeding…we’ll discuss that during the “In Walks The School Nurse” section.
There’s not a better way to report the frequency than to ditto what they said over at National Library of Medicine (NLM):
“The combined prevalence of all types of Ehlers-Danlos syndrome appears to be at least 1 in 5,000 individuals worldwide. The hypermobile and classical forms are most common; the hypermobile type may affect as many as 1 in 5,000 to 20,000 people, while the classical type probably occurs in 1 in 20,000 to 40,000 people. Other forms of Ehlers-Danlos syndrome are rare, often with only a few cases or affected families described in the medical literature.” – https://ghr.nlm.nih.gov/condition/ehlers-danlos-syndrome#statistics
**NOTE: As of October 1, 2020, the National Library of Medicine (NLM) will no longer offer Genetics Home Reference as a stand-alone website. Instead, much of the content from Genetics Home Reference has been transferred to MedlinePlus, the NLM’s flagship website for health information for patients, families, and the general public.
Please visit MedlinePlus Genetics (https://medlineplus.gov/genetics) to access genetics information and learn more about this change.**
Considering a school with 500 children (y’all in the big cities will have more I am sure) you may find a child with EDS every 10-15 years (statically speaking). Or you may have two this year and never see another one again during your career.
In Walks The School Nurse
We school nurses will not (usually) be participating in surgery for these children. And a post-op child with EDS will have a nice, new plan of care from the hospital when they return to school. “Children will be children.” And, we do have children with EDS wanting to be “normal” children…and children do what children do (job security baby!!). So, let me make a list of things to consider when the EDS child (forgive the labeling) walks into your office. This list is not exhaustive. If you have a thing or two you’d like to add, let me know
High Risk of Altered Skin Integrity. Consider the band-aid. The wonderful band-aid is the friend of all school nurses and has the ability to help the child with the teeny-tiniest boo-boo feel all better and go back to learning mode in their classroom. But, the adhesive bandage may not be the dressing of choice for the child with EDS. If you have the OK from the parents / guardian (and if your nursing judgement indicates) to use sticky bandages on the EDS child, cut the ends of the band-aid off on one side and make a “handle” on the other side. I’ll make a video to show this method. Actually, I use this often for most all my children. You know what they say about Band-Aids and taking them off…”Like ripping off a Band-Aid”…1-2- (HEY! What happened to “3”?).
And though you are the nurse, try to avoid dressing changes at school. As a matter of fact, dressings that are left in place (though not dirty or wet and within a reasonable amount of time) will actually result in better healing for the EDS child (and for most folks as well). But, if you have to change a dressing (no matter how small the “dressing” may be), change it but with caution as not to cause additional trauma. Techniques such as soaking the dressing as well as planning on spending a bit more time with the child should be considered.
High Risk of Trauma. Consider the weak surrounding tissues of the child’s joints. Physically assisting the conscious child from here-to-there could very well result in a dislocation of a joint. Goodness forbid you have to care for any unconscious child. Even more so if that unconscious child has EDS. As an example: teams in operating rooms have to pay special attention to alignment and positioning to avoid joint trauma with EDS patients. My suggestion with any unconscious child is: get some help if you need to reposition or relocate.
And, consider the pain that will result from a dislocated joint. What is actually hurting? Maybe the pain with you or I is secondary to our overstretched connective tissues. The connective tissues are weak and “flexible” in the child with EDS. He or she may not behave like any other child with a dislocated joint. It may not hurt. If you suspect a dislocation, have them seek medical care now. Trust me, mamma or daddy will understand your plea to “come now.”
And…while we are on the topic of dislocations vs pain…don’t use ice packs. They may not “feel” the cold and the ice may damage their already-at-risk skin integrity (consider the effect being similar to Raynaud’s Phenomenon)
Dislocations most often happen in these joints:
High Risk for Injury. If a child with EDS has had a slight mishap, check the child over for skin injuries (within reason and as privacy allows). A small fall to the ground may result in a skin tear and subsequent bleeding that may not make itself known until later (bleeding may last longer than normal (plan on it)).
But, it’s just not that simple. Just telling a kiddo that he or she can not play soccer, football, or even track will not be as easy as that. They really and truly need to avoid high-impact fun and physical engagement (sorry…”fun” and “engagement” is what they believe they will be missing out on). Consider the child taking part in the activity by being part of the team. For example: is it possible for the child to “bike” while “participating” in track (trying to think outside the box)? It’ll take some doing but is it possible? What about working with the football team instead of actually playing the game?
This may not be a simple issue to deal with. But, with the parent’s, child’s, doctor’s, school’s and the awesome school nurse’s input, I am sure a solution can be found. But, “just being a ‘normal’ child” may not be an option. Afterall, what is normal? The star quarterback…is he ‘normal?’ If the star quarterback was “normal” then all his peers would be star quarterback, right? I know that being a star athlete is a much different paradigm than being a bench-warmer. But, that term “normal” indicates that all children are equal on every level. And that…well…that is a story for another day.
High Risk for Bleeding. No discussion on EDS would be complete without mentioning the risk for bleeding at the “more-than-normal” rate/amount. Let’s look at this for a bit…
Bleeding and prone to bruising is not only associated with clotting and platelet disorders such as Thrombocytopenia (though EDS patients have platelet function abnormalities). In the EDS child, the bleeding disorder has to do with the integrity…or lack thereof…of the perivascular connective tissues that “facilitate the movement, protection and the nutrition of the blood vessels.” And, like the information under the above heading: You’re Supported by the Triple Helix of Collagen, your blood vessels are no exception. “Vascular fragility affecting medium-sized and large arteries and veins is typically observed in the vascular subtype of EDS.”
Before We Get To the FREE Care Plan Download (It’s not as awesome as I wanted it to be but…), Let’s Talk About How Someone “Gets” Ehlers-Danios Syndrome.
Ehlers-Danios Syndrome is not something that can be “caught” like COVID-19 or influenza. EDS is the result of mutations in one or more of several different genes. To really dive into all this may be a bit out of the interest of many of us awesome school nurses. However, if you are feeling a tad-bit more awesome today than normal and you want to learn more about how someone “develops” EDS, we’ll cover that now (in brief).
There are 13 types of Ehlers-Danlos syndromes (EDS):
Hypermobile EDS (autosomal dominant)
Classical EDS (autosomal dominant)
Classical-like EDS (clEDS) (autosomal recessive)
Cardiac-valvular EDS (cvEDS) (autosomal recessive)
Vascular EDS (autosomal dominant)
Kyphoscoliosis EDS (autosomal recessive)
Arthrochalasia EDS (autosomal dominant)
Dermatosparaxis EDS (autosomal recessive)
Brittle Cornea Syndrome (BCS) (autosomal recessive)
Spondylodysplastic EDS (spEDS) (autosomal recessive)
Musculocontractural EDS (mcEDS) (autosomal recessive)
Myopathic EDS (mEDS) (autosomal dominant or autosomal recessive)
Periodontal EDS (pEDS) (autosomal dominant)
Though each of the 13 types have similar findings, there are differences between the types of EDS. And, explaining each one’s uniqueness is well beyond the scope of this article (though I’d love to dive into all that…I am sure this read has gone on a bit farther than you like (thanks for sticking in with me)). However, we will review both the autosomal dominant pattern of inheritance or an autosomal recessive pattern of inheritance.
But first, let’s do one thing. Let’s understand better the differences between Classical EDS (autosomal dominant) and Classical-like EDS (clEDS) (autosomal recessive).
CLASSICAL-LIKE EDS (CLEDS)
Skin hyperextensibility with velvety skin texture and absence of atrophic scarring
Generalized joint hypermobility (GJH)
Easily bruised skin or spontaneous ecchymoses (discolorations of the skin resulting from bleeding underneath).
CLASSICAL EDS (CEDS)
Skin hyperextensibility and atrophic scarring
Generalized joint hypermobility (GJH)
Okay, let me see if I can explain the difference between autosomal recessive and dominant disorders.
Autosomal recessive disorders are where mamma and daddy are carriers of the mutation in their genetic makeup but do not have the actual disorder. Maybe the mother and the father have the mutated gene for… let’s say Tay-Sachs disease but have no symptoms of the ailment. They are carriers of the mutation and suffer from none of the issues associated with Tay-Sachs disease. However, depending on the this-that-and-the-other (a long story), there’s a 25% chance that their child will actually develop the disease, a 50% chance that their child will carry the mutated gene, and a 25% chance the mutation will not find its way into their child’s genetic makeup at all.
Autosomal dominant disorders are where one of the parents actually suffers from the genetic issue, and the other does not have the condition or the mutation in their genes. The result will be a 50% chance that their child will actually develop the syndrome and a 50% chance the mutation will not find its way to their child’s genetic makeup. If one parent has the mutated gene and suffers from let’s say Marfan syndrome, then there will be a 50% / 50% chance the child will develop the condition and a 50% chance the child will not have the condition at all.
Now with the FREE Stuff
Here’s a nice template for a care plan that you could use. You’ll be hard-pressed to find a pre-done care plan for EDS (if you find one…let me know…please-and-thank-you). A care plan for EDS would be based on the actuals and the possibles. So, I looked at the symptoms when I created this care-plan-ish care plan. Hope it helps. Here is the care plan here. Yes…you click here.
I also like to make sure I have a letter for substitutes to give the teacher for their sub books / binders / plans (I do this for my diabetics and cancer kiddos too). Here’s a letter that may be something to use as a template for your letter.
And, if you want to know more about Ehlers-Danlos Syndrome, here are a few links (as well as a few embedded links above):
Well. That’s it for now. I have been contemplating articles on polymorphic light eruption, Cerebral palsy and seizures. Let’s see where we end up.
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