Never a dull moment…

So… another installment of Em and Beth’s excellent adventures. (Doesn’t quite have the same ring as “Bill and Ted’s” but whatever…)

New Meds

Since I last posted, Em has had to go off the Cymbalta. She has been on several meds that, combined, can cause Serotonin Syndrome, and Cymbalta was one more to add to that list. When she displayed confusion, lethargy, and worsening muscle twitching, we got concerned. The doctor wasn’t – but I was and so I called the pharmacy. The guy I talked to spent a fair amount of time on the phone with me and finally said if it was his kid, she would not take the new med anymore. Which sealed the deal for me. Nucynta is another one that can be a problem, but in a much lesser way than the Cymbalta, so we are hopeful that she can perhaps try it once the Cymbalta is out of her system and maybe it will help. Again, it is a thing that it was helping her pain (although she didn’t actually notice much relief when she had taken it, but rather when it wore off) but she just can’t tolerate it. That gets heartbreaking, to be honest.

I actually haven’t talked to the pain doc about stopping the med – we made an “executive decision” to stop it. He wasn’t a bit concerned when I talked to him about the worrisome symptoms, which is rather concerning to me, if I am honest. I know he is just trying his best to help and sometimes we have to take calculated risks. This just is not one that worked out.

Because he sort of blew off my concerns, I am hesitant to call back and tell him that we decided it best to take her off the med. I don’t think it will be a problem but I just haven’t had the time or inclination to jump back into that fight, for reasons that will be apparent in a moment.

Another Trip to the ER

We are thinking that Em has developed a CSF leak and made a trip to the ER yesterday at the advice of the neurologist. Or, more accurately, the nurses at the neurology office because they didn’t get around to returning my second call until the end of the day when they couldn’t get a hold of the doctors.

It was Dr. Diana that suggested the possibility of a leak – that perhaps the weirdness with her ear was due to a leak. (Thank God for Dr. D, I don’t know where we would be without her.) Now, all of these new and worrisome symptoms were sort of mashed up together, but after she stopped the Cymbalta on Tuesday, she started complaining of a headache that went away when she laid down and quickly got horrible when she is upright. So, she had already been in bed for a few days because the Cymbalta knocked her out. But, starting Monday or Tuesday, every time she sat up, the headache was awful and when she lays down it goes away. So, that seems like a low pressure headache and/ or a leak. Who knows how long it would have taken me to figure it out without a helpful word from Dr. D?

Diamox made it worse, so we pulled back on it and let her miss a dose to see what happened. Her head got a little better, but still, she couldn’t be upright. And, there was the concern of meningitis. So, I called the office late on Wednesday, called back on Thursday when I didn’t hear back and they finally got back to me and they said to go on to the ER.

You can imagine that this was not well received after our last trip to the ER. Understandably, we were both anxious about how it would go.

Fortunately, this trip was much better. They took her pain and symptoms seriously and treated her well. They started an IV, ran some fluids and gave her some pain medication. It took the edge off of her headache and let her rest a little. They did a CT scan, which found no big leaks, but could not rule out a small leak. They could not do the test to check for a leak at that time of night. I am not sure what he was referring to there, but we will follow up with the neurologist and pursue it further.

He said there was no indication that she has meningitis but the only way to tell for sure would be to do a spinal tap. He did not recommend it and we all agreed. Wholeheartedly. And, he clearly didn’t feel a lumbar puncture was necessary which I was also in wholehearted agreement with.

He felt comfortable sending her home to sleep in her own bed and just follow up with the doctor, saying we can come back if it gets worse. I had packed to stay a few days if necessary (the last time we had a real emergency I didn’t and we ended up staying several days at Riley Children’s after a swift ambulance ride in the middle of the night and an appendectomy) and I was pretty darn glad it was unnecessary! If packing to stay is all it takes to get sent on home, I will do it every time from now on out!

This morning, her head is no better when she is upright. So, I think we are dealing with a low pressure headache. She took a small dose of Diamox yesterday afternoon before we went to the ER, but that is all she has had since Wednesday. She can make the call as to whether she needs it or not. And, we will play it by ear until we can get in to see neurology. I am also hoping that when we see the ENT, he might be able to help us figure out if her ear has anything at all to do with her headache. If it is a leak, with any luck it will heal itself and need no further treatment.

It is more than a little frustrating to have to work so hard to get answers – we spent hours in the ER and came away with no answers. And, yet, I am thankful that it was a positive experience, that they took her pain seriously and treated it, that they were caring and compassionate, and that we were able to rule the big stuff out. Of course, that leaves all the little stuff up for grabs, but that is our life.

When the doctor first examined her, he said he was going to go read her records and see if they could give him more insight into what was going on. When he came back in, a couple hours later, after the pain meds and CT scan, he sat down and said that he had been reading about Em and that it was enough to keep him busy for awhile.

How right he is! Having been kept busy for the last 6 years, I can tell you that truer words were never spoken.

 

I spoke too soon…

Ya know how I previously said Em didn’t break a leg at opening night? Well, I probably shouldn’t have said such a stupid thing and asked for trouble! I should know better…

It was not really unexpected but Em wrenched her ankle badly before the show Saturday night – dislocated, subluxed, sprained, whatever you want to call it. Whatever you want to call it, she hurt it. I walked into the dressing room and found her almost in tears, limping. She was very quiet (always a bad sign) and downplayed how bad it was, but I understood it was bad.

I called her Dad, who was on his way to the theatre after getting off work, and had him stop and get a brace and some ice. So, she took a pain pill, propped her ankle up and rested as much as she could;  we iced it and wrapped it up and prayed it was enough! (She wears black tights with her costumes so the black brace blended right in; we just grabbed some black tape from back stage and covered the little orange label that is sew into the front.) The other girls were great – M offered to switch shoes if it would help and they all helped her walk outside to the front so she wouldn’t slip on the ice when the nuns go out and they helped her up and down the stairs. It was a comfort to know she had people watching out for her and helping take care of her.

She made it through the performance, but, by the end of the evening, she was in pretty bad shape and then had to be up early the next day for the Sunday matinee at 2 pm. She took her crutch with her, just to remind herself to take it easy and to try to spare her ankle a bit when she wasn’t on stage. She got through and now has a couple days to rest, thank goodness! We perform again Wednesday but hopefully she is better by then.

Now, you probably also know that the whole EDS thing was never to be spoken of at the theatre – not openly anyway – she didn’t want anyone to know so she wouldn’t have to put up with whatever comes after she tells someone. But, the end result of this injury is that we shared about her medical issues – you cannot go around telling people you dislocated your ankle and not make some additional comments. So, I shared a bit with some of the cast when they asked about her injury and she shared with others when it came up. Essentially, her secret (kept so carefully for the past 2 1/2 months) was out of the bag. And, guess what? Everybody was cool. Sympathetic? Yes. Concerned? Yes. Helpful? Yes.  A bit curious? Yes. Mean? No. Condescending? No.  Do they treat her any differently? Nope. Do they like her just as much as they did before? Yup.

So, I think this has been a great experience for her. Nothing bad happened when she came clean and we are sharing now information about EDS with people who are very interested. Frankly, we have seen suspicious symptoms in several cast members and have been searching for a way to reach out. Saturday, we were given the opportunity to reach out to them. The flood gate is open, but I will write about that in another post – way too long to add to this one!

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!

Surgery Tips Part 2

So, Part 1 described the things that went well for us during Em’s emergency appendectomy. Thank God, all went well and was much easier than I would have thought (if I had had time to actually think!). With EDS, it is so unusual to have everything go smoothly that I was pleasantly surprised (shocked, actually) at how great the whole episode went. However, there were a few things that weren’t exactly ideal and that I will be able to learn from. I will be focusing on this in Part 2 – we can learn from both the good and the bad.

What didn’t go so perfectly:

• Hard Collar  This is my biggest complaint and still has me pretty ticked off, almost 2 weeks later. When I spoke to the anesthesiologist, I expressed how concerned I was about Em’s neck (instability, etc) and how it was recommended that she wear a hard collar during the surgery. [Ok, no doctor actually recommended this, I had read how other EDS patients who had been through surgery wished they had been in a hard collar because their neck was totally messed up afterwards.  Like Em doesn’t have enough neurological crap to deal with already. And it was the middle of the night and they couldn’t exactly call Dr. Tinkle to ask – and actually I have no idea if he would have agreed with me. My personal opinion is that it was a precaution that was wise.] The anesthesiologist I spoke to, said he saw no reason she couldn’t wear a hard collar and they even tried one on her before taking her up to the OR and took it with them. According to Em, and I will be speaking to the surgeon when we go to our follow up appointment in a couple weeks, someone (not the surgeon and maybe not the anesthesiologist I spoke with) made a snap decision that the hard collar was unnecessary because ‘ her fingers don’t tingle when she moves her neck’. I have a real issue with that, because I was assured that she could wear it and trusted she would. Fortunately, her neck seems fine but that was a risk I was not willing to take and for someone with zero knowledge about hypermobility to make that decision is not acceptable. Whoever made this decision needs to understand that you don’t mess with the neck of an EDSer. I don’t know what else I could have done to change the outcome, but I would somehow get more agreement with the whole surgical team  so more people were in on the decision and one person couldn’t simply undo it. This was very frustrating and I want to find out who made the decision and make sure this doesn’t happen to another EDSer in the future.
• Pain Control They were pretty diligent about keeping her pain under control, but I suspect they could have done a little more. I felt like the fact that she was already in a great deal of pain prior to appendicitis, meant they needed to be a little more conscientious about controlling her pain. Also, we had some mixed messages about pain control. One nurse would imply Em needed to be weaning off pain meds and another would say she could ask for additional pain meds if necessary. So, I am not sure if we should have asked for more or if what she had was good enough. Pain is always such an insidious part of her life and it is so difficult to manage. The reality is, there will be pain after surgery – that is unavoidable so I think this aspect of our experience was ‘ok’.  I just have a nagging suspicion it could have been better than ok. Definitely something to address with your surgery team so you have the best assurances of  pain control.
• Nausea Control  They did a pretty good job controlling her nausea, but my biggest complaint was that we had to ask about getting something for the trip home. A two hour trip home with a child who is recovering from surgery, who fights nausea everyday and who gets car sick on a good day obviously needs some nausea meds for the trip home. I suggested a scopolamine patch – it is used for motion sickness as well as post surgical nausea – because I thought it would be a good option. (I was given one after my kidney stone procedure and I will say that the patch is AMAZING. No side effects like with oral nausea meds and one patch lasts 3 days. If you have motion sickness or are prone to nausea, seriously, check into it.) Anyway, they looked at me like I was nuts and finally got a chewable Reglan pill for her to take on the way home. This complaint can probably just be chalked up to my know-it-all attitude, but, still, it would have been nice to have them at least consider my suggestion, particularly since she has been battling nausea ever since. Personally, I find nausea even worse than the pain after surgery, so I take this really seriously for my kid. FYI, they can use the scope patch PRIOR to surgery to minimize post surgical nausea. Definitely something to discuss with your surgical team, if nausea is a problem for you.
• Restrictions/ Dismissal Instructions  I had concerns about what our discharge instructions were – Em is supposed to start water therapy in the next couple weeks and I just couldn’t see that was a good idea until she is well healed. The doctor on the floor came and talked to us and said she just couldn’t be immersed in water for 7 to 10 days, after that, swimming was no problem. But therapy is not the same as going swimming and I think he was pretty clueless about how EDS was going to affect her healing. The surgeon originally said she wouldn’t even guess how long it would take Em to heal and bounce back, so I felt we really got some mixed messages. Rule of thumb, for EDS healing is twice as long as a normal person. I don’t think anyone beyond the surgeon took that into consideration. This might have been one point when the team forgot she was actually a ‘zebra‘ and not a ‘horse’. My solution: just hold off on scheduling therapy for a couple weeks, probably until after we see the surgeon for our follow up appointment just to be safe. And just encourage Em to be careful about lifting anything heavier than the cat (because there is no way I am going to be able to prevent her from lifting that cat and the cat isn’t very heavy anyway). It strikes me as a little ridiculous that I have to make this stuff up as I go, but, honestly, the doctors probably guess as much as I do when it comes to EDS and might be less accurate, since I know this child better than them.
• Hydration/POTS  When Em got to the ER, she was already dehydrated and getting more so by the minute. By the time they told us she needed surgery, she was so dehydrated she had no tears to cry. Her mouth was so dry the skin was actually tearing. She couldn’t even have ice chips, but we finally got a swap to dip in water so we could moisten her mouth a little. ASK FOR THIS IF YOU NEED IT! It is a small thing but something that can make a real difference in your comfort level. I really wished I had some Biotene mouth spray in my purse for her. More importantly, as we got ready to get on the ambulance, I told them they needed to at least give her some IV fluids or they were asking for trouble – as in, she would have increasing problems with her heart rate, etc. They said she couldn’t have much because of surgery but they would give her a little. I think they gave her very little – just enough to shut me up. Now, I know you shouldn’t have food and liquid before surgery but they can make sure you are hydrated even before surgery and that is what is recommended for POTS patients. It all worked out and she did well without hydration, but this is definitely an area I would have addressed in a stronger way if I could do it over again.
• EDS Weirdness  There is a lot of weird stuff that comes along with EDS: odd metabolism of meds – EDSers can be either too sensitive to small doses or quickly metabolize huge doses; temperature regulation – another part of dysautonomia; poor reaction to local anesthetic and so on. I warned them about all of this and they listened – I just don’t know if anything I said sunk in. So, I have to put this on the unsatisfactory list, because I just don’t know. These are definitely things to share with the anesthesiologist and surgeon when preparing for surgery – you don’t want them caught off guard if EDS weirdness occurs during surgery!

I feel incredibly fortunate that this experience went as well as it did and that the complaints I have, for the most part, are fairly minor. Some, like the barely adequate pain control and nausea issues, are understandable in that this was an emergency. I think with planned surgery, at least, those issues could be better planned for. The other issues I had, could also likely be better with time to discuss it with a surgery team. To a certain extent, emergency surgery is just going to be rough and I think we got off very lucky.

Of course, there are some lessons that can be learned from our experience – both for me personally and perhaps even by you. Each EDSer has their own personal issues that would need to be addressed in an emergency situation, so any plan would need to be individualized, but there are surely some generalizations that can be made and it is always good to be thinking about what can or should be addressed.

In Part 3, I am going to attempt to pull all of this together into some dos and don’ts in Part 3 and see if I can eventually formulate some kind of plan that I can have on hand, just in case we ever end up in a similar situation. The final plan might end up being Part 4 – it is all rather lengthy. I will be working on this over the next couple days, but if you have any suggestions or personal experience that you think should be included, feel free to make a comment or email me. I truly believe the fact that we (the entire EDS family) can communicate and share info is our biggest saving grace. I have learned from so many I have come into contact with online and I am happy to share my own experiences!

Surgery Tips Part 1

Since Em’s recent appendectomy was not remotely planned or expected, I felt woefully unprepared as far as a plan for surgery went. (Hmm… I guess that is why is it called an ’emergency’, isn’t it?)  I had given almost zero thought to any emergency procedures in my determination to avoid joint or spine surgery that some ignorant doc might try to foist upon us. So, when faced with an emergency, I had to wing it.

Fortunately, I have a special filing cabinet in my brain designated to store all of the minutia I have gathered over several years while learning everything I could about EDS. I had seen – somewhere, couldn’t tell you where now – suggestions about how to make surgery (and recovery) easiest for EDS patients and ‘filed them away’. Those suggestions, along with ideas gleaned from various conversations on EDS forums and important information about Mast Cell disease that I was made aware of via Dr. Diana’s work (thank you, THANK YOU, Dr. D!), all were pulled out of that overflowing filing cabinet when I needed them. It became very clear to me that every second of the countless hours I spent pouring over endless articles, websites, forums, and blogs was paying off when it mattered most.

I was able to share good information with the surgeon and anesthesiologist but I sure wish I had been a little more prepared. So, I thought I would share what worked for us, what didn’t work so well and make some suggestions of things for you to think about – just in case. I will be using these notes as a way to formulate an emergency plan to have on hand if, God forbid, Em ever needs surgery again. (I will divide this into 3 separate posts, so it is a little easier to read.)

Things that worked:

• Joint Protection  The last thing Em needed was a dislocation on the operating table – being unconscious for a couple hours with a dislocation or even a subluxation could cause long term issues and would certainly cause unnecessary pain. The surgeon took my concerns to heart and allowed Em to place herself on the operating table. (Often, they will start the sedation prior to arriving in the OR, so you need to specifically request to stay awake.) The surgeon (bless her) took it one step further and allowed Em to wake up on the table, so that she could be assessed and assist them in moving her to the bed. If you deal with unstable joints that dislocate or sublux, this is something to talk with your surgeon about. Obviously, all staff need to be warned to be extraordinarily gentle and to never pull on any limb – I had no qualms about telling everyone who came into contact with her about this.
• Comfortable Bed  I remember seeing a reference to requesting an air mattress while in the hospital and probably would have eventually thought to ask about it. But, as I sat beside her in the wee hours after she had been taken to her room ( and the nurses had done their thing and left us to rest before rounds began) I noticed her bed was making noise and had more buttons than I had seen on a hospital bed before. She actually had an air bed – instead of a regular mattress, the mattress was air filled and inflated around her body, gently cradling her with every move. Em said that her bed was very comfortable, something I have never been able to say about a hospital bed! If you are having scheduled surgery, ask about an air mattress ahead of time. In an emergency situation,  it might be more difficult to arrange but you can still ask. We lucked out on this one, but it is definitely something to think about – decent sleep is essential to recovery and is a vital part of controlling pain.
• Careful Surgical Techniques  EDSers need to be treated with extreme care. Again, we lucked out with a surgeon who had experience with EDS patients. I didn’t have to tell her to be ultra gentle – she described to me how gentle she would be. She opted for a traditional incision, which she hoped would put less pressure on Em’s intestines. She did not cut across the abdominal muscle, but cut lengthwise through it. She was careful about internal stitches (presumably using more than usual and closer together) and used steri strips on the incision.  I was very pleased with her knowledge but it is still crucial to make sure a surgeon is up on the special techniques necessary. In a scheduled surgery, you would at least have the opportunity to discuss this or find a surgeon you are comfortable with. Again, in an emergency situation, it is a little more complicated. Which is why I would recommend having all of this information written up in the form of a plan that you can access in an emergency – more about that in part 3.
• Communication For the most part, I felt heard. I spilled everything that could possibly be important to both the surgeon and the anesthesiologist and I felt that they both listened to me. I had no articles or printed info to back up what I was saying, but they listened and encouraged me to share, seeming to take my suggestions and statements in good faith. I was a mess, trying to make sure I covered everything that was important, convinced I was forgetting something important. It would have been so much easier if I had some kind of plan printed up to refer to, but, generally, everything went pretty smoothly.
• A Helpful Phrase In a discussion on Inspire, I saw someone mention the concept of EDSers needing to be treated as gently as a newborn baby – the same level of tenderness and pressure and motion. I said this very thing to the anesthesiologist and it seemed to resonate. I reiterated how careful he needed to be of her jaw and her neck (I will speak more about her neck in part 2) and, this is a bit fuzzy to me now, but he mentioned using a camera during the intubation process so they could see what they were doing.  Her jaw was sore afterwards, but nothing out of the ordinary, so I think that part went well.
• Medication List  Before  we headed to the ER, I took a moment to update and print off a list of Em’s medications – a couple scripts had recently changed so I needed to update it. I am so glad I did that – instead of trying to remember each med and the dosage and the recent changes, I was able to take it out of my binder of medical info and just hand it to each doctor/nurse. Ten different people probably used that list that night and the hospital ended up providing all of Em’s meds during her stay, based on that list. The only thing I wish I had included on that list, was the reason for each med because I was asked as they were prepping her for surgery.  An additional column in the chart would have given me space to add that in. Keeping an updated meds  list is crucial and having it with you at all times is the best plan.
• Medical Binder  Any time we head to the doctor, I take along my huge medical binder and I had the foresight to grab it on the way to the ER.  Not only was the list of medications in it, but I also had Dr. Tinkle’s card with contact info and could have provided contact info for most of Em’s doctors, and a plethora of other info,  if I had to. I did come to the conclusion that my system of organization of medical info desperately needs updated and streamlined, but I was so glad I had my current system with me. It was invaluable, albeit disorganized (and heavy!).
• EDS Knowledge  I think this belongs on this list – because having a base of knowledge was crucial in making sure I could advocate for my daughter. I cannot stress enough how you have to learn everything you possibly can about EDS. You simply cannot rely on your doctors to know anything helpful about it. We were fortunate to have an EDS savvy surgeon, but it could have easily been different. If she hadn’t been quite so knowledgeable, I could have shared with  her about the specific techniques to use with EDS patients and encouraged her to research before surgery. There is no substitute for being your own expert and advocate, whether it is well received or not.

These are the things that went really well for us – although I suspect a good part of it was simply God watching out for us rather than anything I accomplished. Nothing I knew from my reading would have convinced an arrogant, less-than-knowledgeable doc to do things differently, I know that much. But, I was able to advocate for my daughter and it gave me a great deal of peace to know that I had knowledge that could help her. That the staff was willing to listen – well, no amount of prep work can guarantee that outcome and it was the key to a good experience for all of us. It is important to have information that you can insist on for the care of your child or yourself, though and to be willing to make a stand. I learned a whole bunch from this experience, although I sure hope to never need to use what I have learned again!

Part 2 will discuss the things that didn’t go quite perfectly and that I would do differently, if I could go back and do them again.

A Cautionary Tale

I wanted to take a few minutes to talk about one particular aspect of our frightening experience with my daughter’s recent appendicitis and surgery. I am sure that experience is frightening enough for any family who deals with it, but EDS added a layer of complexity and unease that, most likely, other folks don’t have. Beyond all of the general EDS concerns I had, one particular comment that the surgeon made stands out to me, and I think it deserves a frank discussion.

A bit of background first…

My daughter woke up that Monday not feeling well. Her stomach hurt and she was a bit nauseated. Neither of which is out of the norm at all for her. She has had daily (sometimes severe) abdominal pain and nausea for more than a year, thanks to EDS GI issues and Mast Cell disease. This pain seemed a little unusual as the day went on – she couldn’t eat, the pain increased and the nausea got worse and worse. However, it was also time for her period, which is always is accompanied by severe cramps. So, the water was doubly muddied for us in trying to decide if this was run-of-the-mill stuff or if it was serious enough to warrant medical attention.

So, through the evening she got worse and I kept asking if it was her normal period pain. She kept saying she thought so. By the next morning, I knew she had to go to the ER. She hadn’t been able to eat since Monday morning and was having trouble even drinking water. But, she was resistant and leery of ER docs – that it was a 45 minute trip to get to the ER was also something she dreaded, being in such pain. So, it was afternoon by the time I was able to get her out the door, not knowing that her life was in more danger with every passing minute. When all was said and done, at 1 am Wednesday morning she was taken into surgery to remove her appendix.

When the surgeon came to speak to us after the surgery, she told us the appendix was large and gangrenous. Then she made the comment that I think is vital to share… She told us that the inflammation and appendix pain was not new – that it had likely been developing for a while and that Em probably didn’t even notice because of her chronic pain.

That was a sobering thought, to say the least. The pain she lives with every day, even though it is being managed, masked a very serious, life-threatening condition. What do I even do with that information? It was hard enough before – making the decision whether or not to go to the doctor or the ER is always an excruciating one. EDSers typically do their best to avoid doctors, and try to manage on their own. Turns out, this time, that could have been fatal.

We moms of EDS kids have a difficult task and a huge responsibility when making these decisions – I know I often feel totally out of my league. I have no medical degree – how am I supposed to make these decisions? Especially when I have been made to feel stupid for taking her to the ER for what I thought was good reason but the doctor saw nothing of concern. We are between a rock and a hard place for certain and our lives would be so much easier if the medical community knew a little (a lot) more about EDS and didn’t leave us to make all of these tough decision on our own. To me, this is the absolute hardest part of dealing with EDS on a daily basis – to be responsible for making medical decisions that I am not remotely qualified to make is absolutely terrifying.

Another point that should be made in this conversation -I never assumed her pain was EDS related, but that would have been a pretty easy mistake to make (at least in the beginning), when EDS can (and does) cause pain and nausea. I did, however, jump to the conclusion that it could be a kidney stone, because she is on medication that can cause them. So, in a way, my thinking was following the EDS trail – if I had simply Googled her symptoms I would have seen that she was really a text book case of appendicitis. We tend to link every symptom and problem back to EDS, which, frankly, is probably true most of the time – certainly, in some situations it is even more difficult to make a distinction. But, sadly, EDS does not make one immune to all of the other normal stuff that can happen to anyone. That bears real consideration when we have these types of decisions to make. I am always accused of overreacting when my daughter is having a new set of symptoms, but this time, I underreacted and that is sobering – and a bit confusing, to be honest.

So, the moral of this story is, to be very careful making the decision to stay home when something out of the ordinary is happening – whether an adult or a child. Pain is part and parcel of EDS and being in high levels of pain is the norm for many EDS patients. But, when something is a little odd, or seems really abnormal, you need to really try to parse whether it is something that needs medical attention or can be dealt with at home. My advice is to err on the side of caution.

Looking back, I don’t know what I could have done differently with the information I had at the time – the pain and nausea were definitely out of the norm, but that she was starting her period, really obscured the true picture for me and delayed us getting medical attention for about 18 hours. Fortunately, everything worked out for us – we had great doctors and an EDS savvy surgeon, but no one can say how long it would have been until the appendix burst, making the whole situation that much more serious. That haunts me, to be honest, although I am just trying to focus on how well everything turned out.

The last point I want to make is that we need to be particularly careful of our daughters (or ourselves, for that matter). We can ascribe so many issues to the menstrual cycle and, often, rightfully so. But, if there symptoms that are unusual or seem much worse than normal, do not discount it – get it checked out. Follow your gut and don’t make assumptions.

This has been a sobering lesson for us and we are going to have to try to assimilate this experience into our decision making process. It won’t make it easier, but it very well may make it more effective in the long run. I think this was a very close call for my daughter and I think she is very fortunate to be on the mend and doing well. I always tell my daughter that she knows her body best…except when she doesn’t. There are times she assumes symptoms are normal, so she can’t always be relied on to know what is serious and I think this was one of those times. She was so accustomed to abdominal pain and nausea that serious pain didn’t signify anything to her except more pain.

Hopefully, our experience can help another family if they ever find themselves in a similar situation.

 

A horse instead of a zebra…

The floor my daughter stayed on during our recent sojourn at Riley Children’s Hospital, was, of course, beautifully decorated with pictures of animals. Each room had a name plate with a different animal and the child’s name.

The animal on Emily’s name plate was…wait for it…a horse. Down the way, another child was a zebra, but Em was just a plain old horse for 2 whole days. And that is a perfect illustration of her stay in the hospital after her emergency appendectomy.

For once, she was a horse. Yes, a horse with some hidden stripes that made life a little more complicated, but a horse nonetheless. She had a condition the doctors and nurses were familiar with,  something they knew how to treat with one hand tied behind their backs  and which was totally routine – even boring – to them. When a new nurse came on duty, she knew what an appendectomy was and what she needed to do to help my daughter get better. The doctors knew what it was and didn’t have to google it. I didn’t have to spell it or teach them how to pronounce it.

Pretty refreshing, to be honest. Normally, she is a puzzle hidden in a mystery wrapped in an enigma, so it was strangely comforting to not have to instruct the medical staff on what to do. (Ok, so I gave lots of instructions, endless instructions, as they were prepping for surgery, but once she was out of surgery, it was just pretty run-of-the-mill recovery that I could just let them handle. Being able to concentrate on comforting my child, instead of educating the staff every single second, was lovely beyond words.)

Of course, now that we are home, we are getting back into the zebra routine. I am pretty sure most appendectomy patients don’t have to reduce a hip dislocation during the first week of recovery. Or worry about the sutures tearing fragile internal tissue. Or have damaged skin from the tape that secured her IV. Then there is the issue of exactly how long it will take to heal – probably twice as long as normals. We never were given firm limits on how long to restrict her, the surgeon wouldn’t hazard a guess as to how long it would take her to bounce back.

So, we are back to business as usual. But, I gotta say, it sure was nice to take a little break from the Zebra Reality Show and just be a horse for a few days!

Feeling so blessed today

I mentioned previously that I would tell all about how we had such a great experience during Em’s recent appendectomy. For someone who has tried very hard to keep my kid away from the surgeons and who has some serious trust issues with doctors, this was a very scary episode. Of course, it is never fun to let your kid be wheeled into surgery, but that feeling is multiplied when you are dealing with a rare and serious medical condition.

We are home now – got home last night about 7 – and she is doing really well. So, I have some time to sit and tell how right things went for us and how blessed I feel. It is a rare thing when every single thing goes right, but in our case, it did and I am immeasurably grateful.

I was apprehensive about heading to the ER in the first place, but it was apparent that we couldn’t avoid the ER this time. But, both doctors we saw were great (we were there for shift change). The first one, was very quiet but listened at lot. He didn’t argue when I said we needed to rule out an abdominal rupture. He said he was ordering a CT and we would get to the bottom of what was going on. The second ER doc, once we knew it was her appendix, asked questions about what type of EDS Em has. He said he remembered there were several types and that some were dangerous. When they asked where we wanted to go, I said I would prefer Cincy but insurance wouldn’t pay. He told me he would ask at Riley and if they couldn’t do it, they would see about getting us to Cincy. They took all of my concerns seriously and put me at ease.

The radiology nurses at Reid were great, and listened to me at every step. They had a heck of a time getting the IV started but they tried to be so careful and felt terrible that they were hurting her.

The paramedics who transported us to Riley were great. They asked questions, let her transfer herself to the gurney and told the ER staff at Riley to let me fill them in, since I knew more about it than they did.

The nurses at Children’s were great, wonderful, awesome – what nurses should be. Riley attracts the best of the best. (Having been a patient  elsewhere, I am thinking it is not quite fair that Children’s Hospital get all the best nurses – it would be nice if they could be spread around a little… But, I guess the kids deserve them, so I won’t complain!) One of the nurses who took care of Em, even has a best friend who has EDS, so we really felt like we were in the right place.

Best of all, the surgeon was awesome and actually had experience with EDS kids. Her first words to me were ‘I have operated on enough EDS kids to know how very special they are.’ She never claimed to be an expert, but her actions spoke for her and she consistently demonstrated she knew a lot about EDS. She spoke about the fragile intestinal tissue (she said, “I like to say, it cannot take a joke”) and she debated about which method to use – laproscopy or traditional. She was concerned that laproscopy would cause problems for the intestines and opted for the traditional method, although she admitted neither option was perfect and she did not presume to think she had the answer.

She told us she would make a small incision, very carefully split the muscle instead of cutting through it, and very carefully search through the intestines to find the appendix. That she would stitch inside but not do any stitches outside. I agreed that the traditional method was the best way to go – it is  a very small incision – perhaps 2 inches, maybe only 1 1/2 inches. If Em had been known to have real issues with wound healing, we probably would have gone the other way, but the traditional way seemed like the right choice.

She also listened to all of my concerns – from MCAS and high intracranial pressure, to vagal nerve compression, to my concerns about Em dislocating on the table . She allowed Em to position herself on the table and then, when the surgery was over, she allowed her to wake up and help move herself. And she conversed directly before with Em and answered her questions – the end result being, Em actually liked her a whole lot. She had a very quiet, gentle, unassuming manner that very much put us at ease but she was also quietly confident and trustworthy. You can tell, I liked her a whole bunch too!

So, we ended up with the best possible situation and felt at ease the whole time (as much at ease as was possible in that situation). I believe we were led to exactly where we needed to be to get the best care. At every step of the path, we were surrounded by the very people who could help us and were exactly who we needed. When the local surgeon refused to do the surgery, we were a little disappointed – my husband knows the surgeon and was pleased that he would be her surgeon.

When we were told he wouldn’t touch her – wouldn’t even consider it, we sort of thought he was over reacting, that he could do it but was being scared away by the EDS diagnosis. We didn’t object to going to Riley, but we just quietly thought we could stay at Reid and be fine. The ER doc told my husband the surgeon was not even on the fence about the decision – he was definite about not wanting to do the surgery. By the time we got done talking to the surgeon after surgery, we knew we were exactly where we were supposed to be. And that the surgeon at Reid Hospital had done us a huge favor by sending us to Children’s. Andrew plans on thanking him personally next time he sees him.

So, that is our story. There is probably more but it was such a whirlwind and I am so tired that I can hardly think straight. I just know that I feel very lucky to still have Em with us and to have had such a positive experience during such a scary time.

An appendectomy was not in my plans this week…

So, we have had an exciting couple of days. I think it is only a few days but it feels like a month.

Monday, Em started complaining about a stomach ache. As the day progressed, so did her pain and nausea. By evening she was miserable but, lucky, lucky girl, she was starting her period and we initially thought it was just bad cramps. She was miserable all night and by morning I knew she needed to go to the ER. I was actually pretty well convinced it was a kidney stone (there is a family history, after all AND she is on a diuretic, that is known to cause kidney stones!). I saw myself in her suffering as I witnessed her pain Tuesday morning – she said it felt like she swallowed scissors, so I thought I knew the score.

We made our way to the ER at Reid, Tuesday afternoon and settled in for, what was to be, a very, very long night. I took her meds and a pillow and blanket for her, a comb (still not sure why??) and my phone and that was about it. Again, I thought we were going to be sent home to pass a stone.

They did a CT scan – which was a horrible ordeal, although the nurses were as gentle and kind as they could be. She hadn’t had anything to eat since Monday afternoon and very, very little to drink either day because she was so nauseated. So she was really dehydrated and is a hard stick on a good day. They had to start an IV so they could do the scan with contrast – unfortunately, they had to stick her three times and do a lot of digging before they got it. It took about 35 minutes and she was just a mess. But a trooper the whole way.

By this time it is 8:30 pm and we are all exhausted and Em just wants to go home – she is dying of thirst and they wouldn’t let her drink anything. In retrospect, that should have stood out as a bad sign.

The doctor comes in and tells us it is her appendix and they are setting up a surgeon to take it out. Then, he comes back to tell us the Reid surgeon won’t do the surgery because of the EDS and wants us to go to Children’s. We sort of understood that but also sort of thought he was overreacting a little. Things started moving quickly at that point and we were shortly on our way to Riley Children’s Hospital in a very bumpy ambulance.

She was taken in to surgery at 1 am Wednesday morning and the surgeon came out to talk to us at 2:30. She said the appendix was 4 inches long, growing behind the bladder and gangrenous. It wasn’t perforated but I think she was a very lucky little girl. We ended up understanding why the Reid surgeon bowed out and have a new respect for him.

At the moment, we are having a’lovely, very unplanned, very expensive ‘vacation’ at Riley Children’s Hospital in Indianapolis. We are on day one and have no idea when we will be heading home. Hopefully, we will be able to go home Friday, if she is going well enough. She is doing pretty well – she finally got to drink after the surgery, has been eating a few bites here and there, and has been up to the bathroom and walked down the hall. Her pain is relatively under control and she even spent a few minutes on the laptop checking out all the nice things our friends are saying about her on Facebook.

She just asked how many days she has been here and was shocked to find out we are still on day one. Poor kiddo is pretty disoriented but very focused on going home! An hour after surgery, she was asking what she needed to do to go home.

We have actually had a very good experience with this unexpected crisis – been surrounded by a lot of good people who are taking very good care of us and have been very, very blessed. I will share about our extreme GOOD fortune in another post coming soon.

So, yeah, my week took a major detour. How’s your week going? 😉