Catching Up and Hanging On: Part 3 Shoulder Woes

To recap: a lot going on… concussion and post concussion symptoms, possible CSF leak, recurrent ear infections. Lots of doctors. Blah. Blah. Blah. Here are Part 1 and Part 2 if you want the longer versions. 

As I have mentioned the brain/ neurological stuff has us concerned but there are always other issues to make things even more interesting. In November, Em screwed up her shoulder. It had been an ongoing issue, one injury after the other and one day it just quit. Got an MRI and it showed tendinopathy, which is a fancy way of saying tendinitis. Basically she has no movement in that shoulder that does not cause pain and has been in a sling day and night since. External rotation is excruciating. There was no evident/major tear on the MRI but we got her in to the Ortho to find out how to proceed.

If you have read for a while, you might recall that Em is rather needle averse. She will do pretty much whatever she needs to do to avoid any and all sharp objects. But, she wasn’t surprised and didn’t object when they offered her a cortisone shot. Well, she did tell the PA who did the shot that he was rude as he inserted the needle. She had been told it wouldn’t really hurt, just a little pressure, and she found that to be not precisely true. He laughed and told her that he was almost done and she could say whatever she needed to say to get through. Which was not a wise offer in my opinion, considering I never know what might come out of her mouth at any given time – and she was suffering from a concussion so he got off lucky, I think! Later she asked how far the needle went in and I told her she really didn’t want to know. She said it felt like he shoved it into her joint and I told her that is sort of the point of the shot. She was not impressed!

Anyway, he didn’t hold out much hope that the shot would do much on its own but he sent her to PT and was hopeful that the shot would help PT be a little less painful and more effective. In all honesty, I don’t think the shot did anything – she has had no noticeable pain relief, but then again who knows what it would be like if she didn’t have the shot. It was definitely worth a try and I was so proud of her for braving it. She pushes through an awful lot on a daily basis but this was kind of a big deal.

On to therapy… She had her eval on the 31st. She is seeing “her” therapist, who has pretty much been with her from the beginning. So, while he is admittedly not an EDS expert, he knows Emily. And we trust him. Her eval revealed nothing we didn’t already know. Her shoulders are extremely unstable. They slip in and out with pretty much every movement. That makes rehabbing her shoulder challenging. She will not tolerate normal rehab; for now, she has very simple isometric exercises that are done with me stabilizing her shoulder.

Her exercises:

1. arm stabilized and in ‘neutral’ position by her side so she can do a few reps of pushing up, down, in and out. These are isometric exercises so we are talking tiny movements. Doing 5 leaves her winded. Doing 10 exhausts her as if she had just lifted her own body weight. We have pushed up to doing 10 but it is more important for her to do 5 good ones than 10 sloppy ones.
2. With her arm/ shoulder stabilized, she does a few reps of external rotation. This is very painful. The goal is tiny movements to stay below that pain threshold as much as possible.
3. with arm/ shoulder stabilized, she squeezes her shoulder blades together. Sounds much easier than it is.
4. We added to the first exercise at her last appointment – we are going to bring her arm out of neutral, to maybe 20 degrees and try the isometric movements. That one is going to be rough.
5. When her arm is out of the sling, which he does want her to do as much as possible when feasible, he wants her to stretch her arm out straight, gently move her wrist and fingers, and bend it a few times. That last part is incredibly taxing. She shakes as if she was lifting 100 pounds. Clearly, her muscles are weak.

Unfortunately, her other shoulder is nearly as bad. There is no injury but it is weak and it is suffering from compensating for her left shoulder. We are doing exercises on both sides and hoping for the best.

Everybody we have seen while she has been in the sling has made a big deal of her getting out of it because her shoulder will freeze up. We kept telling the doctors that the problem is her arm falls out of the joint if it is not supported. It is unstable enough that freezing up starts to sound good at this point. (I exaggerate there a bit, freezing up is all too possible and we don’t want that either, but you probably get what I mean.) We felt pretty vindicated when the PT was so concerned about her instability and told her to keep it in the sling. She should, and does, take it out but it has to be supported when not in the sling. So, she either holds it in place with her other hand or props it up with pillows.

The Ortho PA did say if PT doesn’t help, the doctor might need to go in and tighten things up. I didn’t go into it then, knowing we will cross that bridge when we come to it, but surgery is not a good option. Seriously. I mean, it could absolutely become necessary and we may be facing that choice down the road but we are going to do everything humanly possible to avoid it, because once we go down that road, I fear we will never stop. And that doesn’t even bring the issue of CRPS into the mix and how bad an idea surgery would be in that regard. I am only talking about the reality that tightening an EDS joint invariably will fail in a year or two or five and she would be considered lucky to get that much time out of a repair. And yet, we could find ourselves in a position where possibly gaining a year or two of function might start looking mighty good.

Suffice it to say, her shoulders are not good. We feel we are in good hands, both with the Ortho and the PT. We follow up with the Ortho (last time she just saw his PA) on the 18th and hopefully she will be making some real progress in therapy by then. I think it is safe to assume this will be a long, hard, grueling process. There is no easy fix and she is willing to do what she needs to do to get better.

We are concerned about her neck. Of course, wearing a sling is hard on her neck. Her muscles are already tight and it is just getting worse. As I mentioned in Part 2, the Concussion Expert wants her to do stretches to loosen up her neck and  hopefully ease her headache. She can’t really do any of the stretches he suggested, so I have been massaging her trigger points hoping to ease the tightness. Unfortunately, that has led to her neck being rather scarily unstable. When those trigger points are released, she ends up a bobble head. And that is not good. It is a matter of constantly juggling one issue with another and trying to figure out how to manage them both; often, they are competing problems with solutions that cause direct complications for another issue. It feels like we just go round and round.

But, we have a plan and know where we need to be headed. Everything else is just part of the journey. We have been down a similar road before and made it through; there is every reason to believe this time will be no different!

A year later…

A year ago, Emily had spent a very unpleasant few hours in the ER, endured a very bumpy, rapid trip to Indianapolis in an ambulance in the middle of the night and was recovering from an emergency appendectomy that was done in the wee hours of the morning. It was a frightening experience but she endured like the trooper she is and came out of it better than we expected.

It is hard to believe a year has passed – in some ways Em has made real progress in fighting EDS & Company, in other ways, we are still fighting the same battles. Certainly, some of the issues have worsened and we go round and round trying to find answers. And, it has been a long recovery period. I don’t know that she has really fully recovered from the surgery even now but, she has made some strides in other areas.

The surgery revealed some problems that desperately needed addressed – a little like peeling back the layers of an onion. We are still peeling away and trying to figure out what those layers reveal. One of those layers will be peeled back on Monday – she will finally have a gastric emptying study. Who knows what it will reveal, but it is long overdue.

This business of living – day in, day out, year in, year out – with EDS is exhausting and frustrating. The problems never seem to stop, never really go away – they seem almost eternal. You address one issue and another pops up. Like running on a treadmill, you feel like you never really get anywhere.

But, a year ago, we weren’t thinking about any of that – we were just feeling incredibly blessed to have our daughter safe and sound and still with us. Not a bad thing to remember when dealing with the phone calls, appointments, tests – all of the minutia and uncertainty of today.

All’s well that ends well

My hubby’s knee surgery was yesterday and it went very well – he is doing great and is home complaining about being bored.

Apparently, the long list of problems listed on the MRI report was indeed just irritation from the meniscus tear that he has hobbling around with for years. You might recall that I was worried that his surgery would reveal all kinds of serious problems – he clearly thought I was overreacting. (Pretty much the story of our lives!)

If it wouldn’t embarrass him, I might mention that telling him the doctor said his ligaments were all fine got him to stick his tongue out at me while he was feeling a little loopy in the recovery room. But, it might embarrass him, so I won’t mention it!

Anyway, the doctor said the surgery went great – took less than an hour, he cleaned up the tear/tears and chunks of cartilage floating around. His ACL (the one he blew out in high school) is intact and, while a little loose, is fine. His LCL (the ligament he was really concerned about) looks ‘rock solid’. Repeating that comment is what earned me an emphatic ‘I told you so!’ from my husband.

We definitely are thankful that we ended up with the best possible outcome and hopefully this surgery will last him for another 10 or 15 years.

He probably will be able to return to work next Monday and hopefully will have a lot less pain to deal with. He is already able to walk better than he could before the surgery, so that is a hopeful thing. He will probably spend the days off work reminding me how he was right and I was wrong! I think I am so relieved that nothing serious was found that I don’t even care!

The EDS he doesn’t have…

I haven’t mentioned this but my husband, who isn’t diagnosed with EDS but clearly has it, has a bad knee. If you have been reading for a while, you know that we joke about ‘the EDS he doesn’t have’, because he is in a wee bit of denial about it. However, he may be getting an official diagnosis in the near future…

In high school, he blew out his ACL playing a pick up game of basketball. He had surgery to reconstruct it, but his knee has been a problem ever since. The EDS explains why that is and why he blew it out in the first place, of course, but for most of his life, we were clueless as to the explanation. 14 years ago, when Em was a baby, he had another surgery to clean up the meniscus. It helped, but that knee was still just not right and after a few years, he was back where he began.

He was probably due for another surgery 4 or 5 years ago, but he has put it off and put it off. Over the last six months, maybe even the last year, his knee has been rapidly worsening. He diagnosed himself with a likely meniscus tear and figured that wasn’t the end of the world. Finally, when he could barely walk on it, he reluctantly made an appointment with an ortho he trusts. (In fairness, this doc did his second surgery years ago and then was no longer affiliated with the hospital where my husband works. He was waiting for him to come back, which he recently did – thank God!)

The initial x-ray didn’t really show anything, so he had to have an MRI. Understandably, he was worried that it wouldn’t show anything wrong and it would be a waste of time. (I was pretty sure it would show something, since he was in serious pain and could hardly put weight on it.) He grumped and grumbled before, (probably during), and after the MRI.

Then, a day or so after the MRI, he got his report back.  There is a list a mile long of things wrong with his knee. He still has cartilage, so that is good, but it is torn – probably the source of much of his pain. Beyond that, pretty much every ligament in his knee is either partially or completely torn. They are hoping that it is just irritation, but I have my doubts. Plus a handful of other medical terminology that is above my pay grade.

His scope is planned for Feb 26th – they will clean up the meniscus tear and check out the ligaments. If they are as messed up as they appear on MRI, we will have to figure out where to go from there, but that would likely be an additional, much more complex surgery. Because all/most of the ligaments in his knee are involved, any reconstruction will be more complicated and require a ‘special’ specialist. Of course, the EDS complicates this all the more and will need to be taken very,very seriously. (My darling, normally intelligent hubby didn’t feel any need to share the fact that he almost certainly has EDS with the doc. Fortunately, I was there and made sure the doctor was told. He later told me that he was planning on mentioning at some point before surgery…What would he do without me?)

So, we have another surgery on the calendar – none of us are really looking forward to it, but hopefully it helps him have less pain and more function. I may lose whatever is left of my sanity after having him home as an invalid for a couple weeks – he is not the best or most cooperative patient in the world. You probably already figured that out, though!

It isn’t often that I talk about Andrew and not Em, but, he gets to be front of the line right now. Prayers would be appreciated, if you are willing. It is going to be an interesting few weeks…

Summer Plans. Or the lack thereof…

It is hard having a child with a chronic illness.

Unless you experience it yourself, you really cannot grasp how thoroughly it permeates every facet of your life. How that illness turns everything you knew on its head and changes your life completely. It is especially hard when you have to interact with people who really don’t understand what you are going through, because they haven’t ‘fallen down the rabbit hole‘ like you have. Those people are almost always well meaning and simply making small talk, but sometimes that small talk is like a knife in the heart.

Case in point: Now that summer is here, from time to time, someone will casually ask something like, ‘so, what are you doing this summer?’ And because my choice of answers are rather limited…

1. Nothing at all, because my kid is too ill to travel
2. Therapy
3. Pushing calories to make sure she doesn’t loose any more weight and hoping she continues to recover from her recent surgery with no further complications
4. Oh yeah, we are pretty excited to be going the the EDNF Learning Conference. What’s that, you ask? Well, it is this medical conference where we will get to hang out with other EDS families and learn from the best EDS experts in the world. What exactly is EDS, you ask? Oh, never mind…

I have about 2 seconds to ponder what to say that won’t come out sounding like a pity party (and if Em is with me, I really don’t want to say anything that will make her feel sad or guilty). And, then, because I was busy thinking all of that and didn’t actually give an answer other than rueful shake of my head, I usually get treated to an enthusiastic rundown of their plans.

Which are nothing much, of course. Just camping and canoeing and swimming and amusement parks and traveling and such. None of which my kid can do, even on her best day.

And all I can think is during this type of conversation is, ‘you have no idea how lucky you are’. Lucky to be clueless about chronic illness. Lucky to be “normal”. Lucky to have no limitations to consider when making plans. Lucky to be planning summer fun rather than doctor’s appointments.

It is so hard not to feel bitter and envious when confronted with conversations like this. But, how can I be truly upset when that person really is clueless – they just live in a different world than I do.  The same world I used to live in. Before chronic illness saunters into your life, you don’t even have a clue that it exists, let alone what an upheaval its arrival will cause. Ignorance is bliss, right? And being genuinely ignorant of living with chronic illness is not something I can penalize anyone for.

Instead of dwelling on what we can’t do and what we are missing, a little counting our blessings goes a long way:

1. We get to go to the EDNF Conference in August and hang out with people who ‘get it’ and learn from the best minds in the field – last year we couldn’t.
2. We have access to therapy, good therapists and doctors who care and can help Em recover from surgery and get better and stronger than she was to begin with.
3. Em is still alive and kicking, when the appendicitis could have killed her.
4. She is getting better everyday and therapy is helping her get stronger.
5. She has decent pain management, which she did not have last summer.
6. She has proper and fairly effective treatment for her headache, thanks to Dr. Diana and the Driscoll Theory, which she did not have last year.
7. We know so much more about the cause of most of her symptoms and have real hope for treatment in the coming months.

What are the typical pursuits of summer when held up next to these? Nothing at all, when you stop and think about it.

A few updates

Been awhile since I posted, so I thought I would do a general update:

Em is continuing to recover after her appendectomy. It still is up and down for her, but generally she is doing well. She still has a lot of stomach pain, particularly when she over does and her pain medication wears off. The Miralax is working very well and has addressed a lot of her issues and she truly wishes she had tried it sooner. [We recommend trying the Miralax in cold apple juice (not too sweet, not too sour) and letting it dissolve thoroughly!] Her appetite is still very poor and she still gets full after a few bites but I think we are seeing some small signs of improvement. Could just be wishful thinking though… The weight loss has slowed and she is hopefully going to start at least maintaining where she is.

Em started water therapy last week and did really well – except that we stupidly scheduled therapy two days in a row for the first week. Seemed like a good idea at the time, but it totally kicked her butt. However, she managed better than I expected and it was a pretty big accomplishment considering all she has been through. She did have a big increase in pain though after the second visit, so we will need to let them know so they don’t add in more in this next week. The therapists are ones she has worked with for the last few years, so they know her and she knows them. And, they are my husband’s co-workers/ friends, which helps my comfort level. At any rate, I think Em is going to really benefit from a few weeks of therapy -hopefully it gets her jumpstarted and feeling better and stronger. She  is scheduled twice a week for the next 3 weeks and then we will see where to go from there.

We had a family event to go to  this weekend and that is always a challenge for Em, especially after her back to back therapy sessions . It involved a 2 hour car trip each way and going to an outdoor party where it ended up raining and cold. But, it was for a very good cause and Em wouldn’t have stayed away for  anything. Seeing friends and family (and having a couple slobbery dogs to love on) is almost always worth the expenditure of energy it takes and I think she enjoyed herself. She will pay for it for a few days but that is better than never doing anything fun.

We got registered for the EDNF Learning Conference, thanks to a ‘donation’ from my parents. With the conference being in Cincinnati, I hated to miss it but just couldn’t see how to afford it. My parents stepped in so we could go and we really appreciate it. Even Em wants to go – previously she has rejected any suggestion that she meet other kids like her or anything of the sort. But she is starting to talk about it and asked to go to the Conference with us. I just don’t see how she would be able to do the full weekend, but I am hoping she will be able to come down for one day/afternoon and maybe participate a little in the kids program. I know it would be good for her so I am hopeful that we can get her there for at least a day. I know I am looking forward to it now that I know for sure I am going!

I think that is all my updates for now – BTW, I am still working on my Emergency Plan and will be posting soon about some ideas I have about making it work.  (See Surgery/ Emergency Tips Parts 1, 2, 3 and 4) I haven’t forgotten and I am working on it! It is a big task but I am anxious to try out a couple ideas and share them with you, if they work!

 

1 month post surgery

Yesterday we saw the wonderful surgeon who did Em’s appendectomy for a follow up appointment. And she was still wonderful.

First, we saw a young doctor – probably a surgical fellow – who works with Dr. West. Emphasis on ‘young’ – he didn’t look much older than my 16 yo son, so that was kind of funny to me. However, he was very nice and gentle with Em, so he got points from me. But, it was when I brought up mast cell activation that I decided I really liked him. When I told him about Em’s suspected mast cell disease and that, because surgery is a known trigger, I thought it had really stirred her mast cells up causing a lot of her current problems, he totally agreed and said that surgery was a major activator of mast cells. No rolling of the eyes, no snorting, no perplexed look while trying to remember exactly what mast cells are. Very refreshing.

Then, Dr. West came in and talked to us. Em’s incision is looking good and healing well – better than any of us really expected, I think.

But the ongoing GI issues and the fact that she continues to lose weight are concerning. She has lost at least 10 pounds, maybe more like 11 or 12 and most of that in just the last couple weeks. Dr. West thinks a lot of her pain and poor appetite is from the constipation so we are going to work on that. Miralax twice a day for a week and see if it helps, if it does, we can bump down to once a day. Regardless, I am to call Dr. West in a week and let her know how things are going and we will decide from there. She hopes to not have to see Em again, but will if necessary.

I remain impressed with this surgeon and would choose her to operate on my kid if it was ever necessary again.

On the Mast Cell front: On the way home, we stopped to eat and Em had a major reaction. She had not taken her Zyrtec and Zantac yet and just started feeling awful after eating a little. She ended up taking her Zyrtec and Zantac and then took a Benedryl when she started feeling like she was going to pass out. After the Benedryl, she eventually perked up but felt pretty rotten the rest of the day. Of course, she had to get up at 7 to get ready to go that morning and had only had about 6 hours of sleep, so feeling rotten was a given.

It is hard to believe that we are a month past surgery and that Em is doing as well as she is. The mast cell stuff and the weight loss is concerning, but hopefully we will be able to get her appetite going again if everything else settles down. Knowing we have a caring, knowledgeable doctor on our side is a great comfort – I feel less alone in dealing with all of this now which is a pretty cool thing!

 

Updates – surgery, headaches, etc.

Em is 3 weeks past surgery and is doing better, finally. Still on pain meds, but generally starting to feel better. Her incision looks good and is healing nicely (a pleasant surprise), although, it is still painful. (To the point of having to wear dresses when she goes out in public, because she can’t bear any pressure on her tummy. A bit of a conflict for my non-girly girl!) She is finally getting up and around more and that will help everything – from morale to her pain in general.

This past weekend, she and a friend actually went to a movie (Hunger Games). We had promised her friend we would all go for her birthday but Em’s surgery interfered. So, we finally got to go and, while it wore her out for a couple days, she had fun and it was a big morale boost to hang out with a friend for a few hours. Yesterday, Em actually went shopping with us. It was just a quick trip to Walmart and she was in her wheelchair, but, still, it was a milestone. It is so nice to see her getting out of the house.

Last night, she actually went down and painted woodwork at my parent’s new house for about half an hour. (They are moving down the street from us and will be moving in in June.) I worried about her overdoing and she did complain of her back hurting, but, still, a few minutes of activity is good for her and she listened to her body and quit when she started hurting. Later, she took the cat out (on the leash) on the back patio for a while and hung out with her dad and me. All in all, she was more active yesterday than she has been since before her surgery.

Next week, we see the surgeon for a follow up appointment and then Em will start water therapy and will hopefully start building up strength that she has lost over the past few months. This surgery has definitely been a setback that she really didn’t need, but hopefully she will be able to build back up relatively quickly.

Her appetite is still very poor and she has lost at least 6 pounds – maybe more like 7 or 8. She isn’t underweight so losing a few pounds isn’t the end of the world, but she also doesn’t have a lot to lose before it becomes a problem and it is starting to concern me. My fear is she will be loosing muscle and she definitely can’t afford that! I think I am going to have to start keeping track of her daily calorie intake again, like last winter. She is back to eating watermelon and not a whole lot else again.

Watermelon is a low histamine food and it seems to be what she gravitates towards when her mast cells are acting up. And they are definitely acting up – surgery is a known trigger for  mast cell activation and she has been flaring big time since surgery. She is not having any flushing episodes that I have noticed, but she has the typical abdominal symptoms and  more dermatological signs than she has had in the past. Her stomach hurts when she eats and particularly after foods that are high histamine. Skin-wise, she has this odd sort of rash around her eyes. It is always there, worse at times than others and when it is bad, like now it is red, itchy and very noticeable. I would bet any amount of money that it is mast cell related. We have a lotion that we make with nasal crom in it that she can put on those itchy places and it helps for a few minutes, then the ‘rash’ comes right back. I think we need to go to a dermatologist but I also am convinced it is just a part of this flare she is in right now. Gastrocrom would help, if I could get anyone to give it to her!

She has another issue that is very common to EDS, but I am not allowed to mention constipation so I won’t.  😉

On the headache front, we think the baking soda tablets are starting to help – her headache isn’t completely gone, but is generally improving. She is now taking 5  650 mg tablets a day and I need to call and get the results from her latest blood test. This is a really rotten time to be trying to manage this, but I think we are making a little headway and we will happily take any improvement!

So, a mixed bag I guess but, generally, she is improving and whenever we are at least moving in the right direction, I am happy. Many of the issues she is having now, after surgery, are the same issues she was dealing with before surgery so there have been no real surprises. I am just relieved that we are seeing improvement and she is able to get out of the house and start living again!

Surgery/Emergency Tips Part 4 – A Plan

Here is the general plan I have pulled together from Parts 1, 2, and 3 – hopefully it will end up being about 2 printed pages, I just couldn’t get it any shorter and I am not really happy with that. Brevity means a doctor might actually read the document, so I need to figure out how to make it easier to use.

One commentor stated she has her medical info (med list,  surgical/medical history, tests/procedures) printed on note cards, which can easily be copied by the office/ER staff. Something like that could maybe work for this info – perhaps each section could be on a separate card and just that particular info handed to the doctor/ nurse/ lab tech when needed, instead of overwhelming them with 2 printed pages. Something to think about.

Another stray idea is to somehow color code the info. So, for example,  mast cell info might be highlight yellow on the ‘Plan’ document and any additional printed info could be on yellow paper, Pain management info could be highlighted blue and any additional printed info could be on blue paper, and so on. Not sure how this would work, but it might be doable.

My current plan, which could change, is to have a simple folder with this info in my car and in my medical binder. Theoretically, it should just be a few printed pages and I could just pull out whatever was needed at the moment or hand over the whole thing, if that was called for.

The most important thing, I think, is to come up with a system that you are comfortable and will use. I am partial to both a binder with hard copies of any documents AND a digital system as a backup plan. I love the Care Medical History Bracelet – my daughter has one she wears as a med alert bracelet and I am able to store a ton of info on it. I am going to get one (or their USB card, necklace or keychain) for myself to store all of this info and to be my digital backup. I don’t have to wear it, but I can keep it in my purse.

In addition to the info listed below, a list of current medications (doses, reason for taking), doctor’s/specialist’s names and contact info, medical history, mast cell protocols, printed info from EDNF, and anything else that I can think of, will be in my binder and on a Care Medical History Bracelet so it is available for reference. Obviously, this is information to have on hand, not necessarily something I would just hand en masse to a doctor or nurse. The medical bracelet we have allows you to print off a document with all of this information and I am seriously considering doing that and just having it in my folder – it would keep a lot of this info in one place and easy to access.

On to the plan:

In making this plan personalized, I will plug specific information that applies to my daughter and substitute her name for ‘EDSer’ anywhere that particular info pertains to her. As of now, I am planning to also include that general EDS info as well as personalized info. Obviously, this is a good beginning but you would need to add and subtract and personalize where needed.

Blood Draws/ IV

• EDSers are often hard sticks. Use a butterfly needle and would be best for the most experienced, confident person to do place the IV or do the blood draw.
• Ask the individual if one site is historically better or worse than another. Consider other sites, other than the arm and hand, if placing the IV is challenging.
• IV pressure may need to be at a lower, slower rate when administering fluids.
• EDS blood vessels are fragile and may collapse easily.
• Use Koband tape instead of a bandaid – this may help minimize bruising.

Diagnostic Imaging

• Move the x-ray machine around the patient, do not expect the patient to move to get the imaging. An EDS  patient can dislocate effortlessly, do your best to avoid provoking one.
• If patient presents with severe abdominal or chest pain, CT scan or MRI should be done immediately to rule out rupture.

Surgical Techniques

• Refer to the Emergency Surgical Suggestions listed in the Emergency Physician’s Reference from the EDNF http://www.ednf.org/documents/cdrombooklet.pdf
• Stitches should be more plentiful that you would normally use and closer together, to prevent wound dehiscence. Leaving them in longer than usual is also helpful.
• Use steri strips to close an incision (and lots of them)
• Expect longer healing times – perhaps twice as long or more than would be expected – and take this into account when restricting the patient’s activities post surgery
• Use extreme care with the tissue of EDS patients – pulling and tugging at normal strength, or anything that stresses fragile tissue, is unadvisable. Be as gentle with an EDS patient as you would be with a newborn baby, with every move that is made.
• If you are not familiar with EDS, educate yourself before operating.
• Minimize blood loss during surgery – EDSers can have bleeding issues, so this is a concern to be aware of.
• Plan for likely complications and know that the EDS patient will likely be a challenge. Be prepared to think outside the box.
• Surgery can be done successfully on an EDS patient, it simply requires a bit more care and thought.

Anesthesia

• EDS patients often metabolize meds oddly. Sometimes huge doses of meds will wear off quickly or be ineffective, other times they will be extraordinarily sensitive to tiny doses and it can even vary within one individual.
• Local anesthetics often do not work for EDS patients. If they say it hurts and that they can feel it, take them seriously.
• Mast cell issues  are common with EDS patients. Monitor carefully during surgery, as many of the initial signs of anaphalaxis are obscured during surgery. Refer to emergency protocol info provided*
• Be very careful when intubating. Be mindful of how fragile the tissue is and avoid causing damage.
• If neurological symptoms are present, allow the patient to wear a hard collar during surgery to protect the vulnerable cervical spine. An EDS patient’s neck should be protected at all costs.
• Be very careful of the jaw when intubating.
• Refer to the Emergency Surgical Suggestions listed in the Emergency Physician’s Reference from the EDNF

Joint Protection

• Always be mindful of how fragile the joints are.
• Allow the patient to place themselves on the operating table, before being sedated. If possible, allow the patient to awake on the table and assist in transferring themselves to a gurney.
• Never pull on an EDS patient’s limbs or try to move them without their assistance. This is of particular importance when the patient is unconscious.
• Dislocations are very common – allow the patient to reduce one themselves, as they are the expert. If they need help, they will ask.
• Support the joints, manually or with pillows, whenever possible. Be careful when taking the blood pressure cuff off or with any seemingly simple action that stresses joints.

Skin Protection

• EDS skin is very fragile and adhesives can be a problem. Be careful with medical tape removal – avoid adhesives if possible.
• A cosmetic surgeon should be consulted for any (even minor) facial wounds, due to the tendency to scar
• Incisions need to be closely monitored, wound dehiscence is common for EDS patients, as is infection and other complications
• Assist the individual in moving if necessary, so as to avoid pressure sores. Remember to not pull on their limbs as you help them.

Pain Control

• Refer to the EDNF Pain Management Medical  Resource Guide. http://www.ednf.org/documents/MRGPainManagementS.pdf
• Develop a plan prior to surgery that will ensure the highest level of pain control
• An air mattress provides the highest level of comfort and the best chance for recuperative sleep. EDSers routinely have problems with sleep, so this is a vital part of good pain control.
• EDS patients live with high levels of chronic pain and they deserve the best possible pain control. EDS patients often metabolize meds oddly. Sometimes huge doses of meds will wear off quickly or be ineffective, other times they will be extraordinarily sensitive to tiny doses and it can even vary within one individual. Local anesthetics often do not work for EDS patients. If they say it hurts and that they can feel it, take them seriously.
• EDS patients live in chronic pain and may not show ‘normal’ signs of pain. Remember, they have learned to hide their daily pain to spare family and friends; you will need to try to see beneath the mask they wear.
• The pain scale commonly used by medical staff is woefully inadequate for chronic pain patients and EDS patients in particular.
• Nausea is a huge  issue for many EDSers, take aggressive steps to treat it after surgery.

Mast Cell Complications

I haven’t totally finished this section, which is ok because it is probably the most individual of all. Check out http://www.tmsforacure.org/documents/ER_Protocol.pdf  This gives a ton of great info that I will be printing off to keep available to share in an emergency.There is a list of medications that should be avoided and a list of meds that are generally ok. This is a very individualized matter though, so keep a record of any medication reactions you might have had in the past.There is also a section on planning surgery, which would be invaluable when developing a plan for planned or emergency surgery. Also, if you have been diagnosed with masto or MCAS (Mast Cell Activation Syndrome) obviously discuss this with your specialist and get their recommendations.

Medical Team

• Communication is key – listen to the patient, they are an expert on their own body and likely know more about EDS than any doctor who might treat them.
• When there are medical decisions to be made, present the options to the patient and give them information to help them make a decision.
• Never forget how out-of-the-ordinary an EDS body is. It can defy logic – the impossible is often very possible for an EDSer. They can dislocate with no trauma whatsoever. They can dislocate countless times a day. If they say they can feel a procedure when they shouldn’t, believe them.
• Dealing with an EDSer requires out-side-of-the-box thinking. Lateral thinking rather than linear thinking. Search for solutions, include the patient in those discussions.
• The condition of an EDS patient can change rapidly, monitor carefully.
• Every EDSer deserves to be treated with respect and compassion.

Obviously, this is large amount of basic information and needs to be organized into something usable.  As I said, I will be customizing this for my daughter and will have it available at all times. Feel free to copy this and personalize it for yourself, adding and subtracting as needed, so that it works the best for you and your own system of medical information.  As always, feel free to share your thoughts with me!

My sources:

I took some of this info directly or in part from http://arthritisinsight.com/medical/surgery/eds.html  This was part of the material I referred to when I spoke of filing information away when I first started researching EDS. I don’t know where the original article is…

I also used the EDNF ER/Surgery booklet as a reference. I really wish it were printable.

A bunch of conversations on Inspire from various EDSers who have been through surgery.

And I incorporated a few suggestions from my readers – I greatly appreciate the contributions!

Surgery Tips/ Emergency Plan Part 3

I am working to bring all of this EDS ER/Surgery information that has been gathered in bits and pieces and odd moments here and there into something concise, organized and useful. (This isn’t nearly as easy as I initially thought it would be!)

So, I am going to divide my advice into some dos and don’ts in this post as a general outline, which I have used as a springboard to come up with a  relatively brief document that I can keep handy. (The brevity of said document is in serious question at this point and I am trying to think of ways to improve this so as to not have to publish a book.) In Parts 1 and 2, I talked about what worked well and what didn’t work so well during my daughter’s appendectomy. The following is general advice I can give from our recent experiences:

Do:

1. Have a system of organizing your medical records and have access to it at all times. [Consider a medical alert bracelet like the Care Medical History Bracelet or another medical USB device to easily keep all of your info on hand. You need to include current medication list, allergies, doctor’s names and contact info, etc. A binder with the hard copies of this info is a good Plan A – however, I am inclined to have Plan B in the form of a USB device as a solid back up plan.]
2. Create an emergency plan that addresses your individual needs. Keep it on a USB drive AND in your binder AND an extra copy in a folder in your car.  You want this on hand at all times because an emergency will always happen in the most inconvenient way possible!
3. Learn as much as you can about EDS. You have to be the expert because the chances of ending up with a EDS savvy doc is slim and even if you are lucky, you will need to help them customize you or your child’s treatment in a way that takes your specific issues into account.
4.  Remember that you will know your body better than any doctor can. Learn to be your own advocate.
5. Educate everyone you come into contact with at the hospital, in some small way. It is extraordinarily frustrating to have to spell out E-h-l-e-r-s D-a-n-l-o-s for every nurse and doctor you see, but every time you do, that is one more health professional who has at least heard of EDS. We may not have the power to move a mountain, but we can move one grain of sand at a time – and, in the end, that is how the mountain gets moved.
6. Have a family member or friend who can be an advocate when you are unable to do so for yourself. Allies are necessary in this battle- you cannot fully fight for yourself when you are unconscious or in severe pain. If you don’t have this, be diligent about #2 so you have a document to speak for you.
7. Know what works for you. For instance, my daughter is a hard stick – many EDSers are. We have learned that a butterfly needle is the key to turning a hard stick into no big deal. That and being well hydrated. Another issue, lying flat on her back causes problems that medical staff needs to be aware of. Don’t be afraid to say what has worked in the past and firmly request/demand it along with what causes problems for you and thus needs to be avoided.
8. Take advantage of documents created by the ENDF. They have excellent Medical Resource Guides that you can print off and have on hand to share with your doctor. Unfortunately, the ER/Surgery booklet isn’t printable, so I am going to just have to take excerpts from it and make a separate document or something. Not ideal, but I can at least have the link available so it can be viewed online.

Don’t:

1. Assume the doctor will know ANYTHING about EDS. If you get one who does, it will be a lovely surprise, but assume you need to educate them because you really can’t rely on a doc who says they know EDS.
2. Wait until AFTER an  emergency to make your plan – do it today. I really, really wish I had a plan ready when we needed it!
3. Forget to update your medical info as it changes. If your medication has changed, update that list today. If, say, your daughter has had an appendectomy recently, update her list of surgeries. (Note to self: go update Em’s medical history on her med alert bracelet!) Seriously, be diligent about this so your med list or medical history isn’t partially accurate and you aren’t in the ER trying to remember those details. This is why I really love our med alert bracelet – the software is very easy to use and you can easily update the plethora of info stored on the bracelet whenever you need to. Whatever system you use, make sure it is simple enough that you will use it.
4. Assume that a non-EDS emergency can’t happen in your focus on living with EDS day to day. Any normal problem/ emergency can pop up and having EDS will just make it more complicated.
5. Assume that every problem is EDS related and allow new symptoms to go unaddressed.  New symptoms should be investigated, even if you find out it is nothing to worry about.
6. Be put off if there is something you need while in the hospital or that you believe the staff needs to implement. Sounding like a broken record is one way to make sure your needs get met and we all know that the squeaky wheel gets the grease.
7. Leave anything to chance. Ok, that is impossible, but what I mean is that we need to really take time and think through each potential obstacle or stumbling block, each co-morbid condition and symptoms that could be problematic in an emergency situation, and make an effort to share enough info to make sure that issue can be managed.

Most of this is common sense, but unfortunately, we can get so wrapped up in just surviving each day with EDS, that we might fail to think of these things. And, when an emergency happens, we find ourselves in a more challenging situation than we need to be. If anyone benefits at all, even in the smallest way, from this series of posts, I would be very happy to know that my own struggles have helped someone else.

Part 4 will be a generic plan for emergencies and for surgery, divided into various categories of concern. Part 5 will be our own personalized plan – taking the generic plan and plugging Em’s name in and listing specific information that applies to her. Coming soon!