"Suffering the Slings and Arrows of Outrageous Fortune"

What a week…

Or two or three or…. whatever. As bad as the last 6 months have been, this past week or so has been especially harrowing.

Em hit her head last weekend. Again. Not bad but bad enough to bring on more memory loss and very poor balance. And she had been having this weird thing where she felt like her throat was collapsing, which was more than a little freaky. Along with the latest head injury came worsening headaches and a very unstable neck. Just like 5 years ago, she was having all kinds of problems and I started assuming many of them were from cervical instability. And that was not a pleasant thought.

The concussion doc was willing to see her, but we agreed that there was nothing he was going to be able to do – time to heal and protecting her head from further injury is what was needed. So, we will see him at her follow up February 5th and wait and see. He is a phone call away.

We decided that some blood work – particularly to check her CO2 level but also a couple other things – would be a good idea. So I called the Neurologist to ask for that. I was not surprised when the answer I got back was that they were ordering blood work and a lumbar puncture.

For more than 4 years, we have avoided lumbar punctures. Not only was I aware that they might not be completely conclusive regarding high pressure but EDS folks are delicate enough that even this relatively easy procedure should not be done lightly. Once I found the Driscoll Theory, I was 100% comfortable doing a trial of Diamox instead of an LP. If Em responded to Diamox, then it would logically follow that her headache was the result of high pressure. We found one doc who was willing to try and the rest is history. The headache she had had for 2 years was gone in a few hours after that first dose. For almost 4 and a half years, we have managed her headache with Diamox, eventually  having to increase her dose and taking it 3 times a day rather than once, and we have managed her CO2 level (which allows the Diamox to work) with baking soda tablets. We had tried to skate around the issue with new docs, endured some eye rolling when we admitted she was being treated for high pressure but had never been actually tested for it and just generally crossed our fingers hoping no one would push the issue. It was all fine for 4 years.

But now, clearly something had changed. My suspicion was it was the head injuries but she probably had a CSF leak (in her ear) in December before the concussion, so maybe the headache is coincidental or cumulative or something, but the bottom line is what we had been doing was no longer working.

In my mind, there were 3 possibilities: she needed more Diamox to control the high pressure, her CO2 level was low which would mean she needed more baking soda tablets to bring it up so the Diamox would work, or there was something else entirely going on, like cervical instability. At any rate, I knew in my heart the time for an LP had come. So, I agreed but asked for a couple things to hopefully make the procedure easier and more successful. I wanted them to use an atraumatic needle, to minimize damage and leaking. And I asked that they schedule a blood patch to be done immediately after the LP, instead of waiting until she had problems. Because, let’s be real, there was a nearly 100% chance she was going to have problems.

The doctor’s office ordered both without issue, so I was happy.

A nurse from Radiology called to pre-register her for the procedure and asked a ton of questions about EDS and her medical issues. She was awesome. She promised to check into the special needle – she had never heard of such a thing before – and did say she wasn’t sure about doing the blood patch because they normally don’t do them but that she would ask the doctor and if she could do it, she would because Em is clearly a special case. So I was content that I had done everything I could to make the LP as safe and easy as possible. When I heard nothing back, I assumed the blood patch was a go.

Em was not thrilled – anything involving a needle is not her idea of fun – but as we waited for Friday I started thinking it couldn’t come quickly enough. Her headache was a 12 (on a scale of 1-10) when her Diamox wore off and a 9.5 when it was working. Clearly there was a problem: she was miserable. We got the blood work that I asked for done and were able to slide in blood work they needed before the LP at the same time, so we minimized the needles for her. When even a simple blood draw is challenging, is it any wonder we were nervous about a lumbar puncture?

We get there on Friday morning and once she is in the room and has been given some Zanax to help relax her, I find out that they will not be doing the blood patch. LPs are done by Radiology and blood patches are done by Anesthesiology.

“Sorry, I don’t know why you were ever told it was possible. It is our policy to not do them here”,” the nurse said. “If she has problems, you will need to call your Pain Management doctor or go to the ER.”

Now, at this point, I am starting to fume.  No one has called me to tell me this and I was given the impression it was happening. So, when she said that, I just gave her a look. The Look. Which she correctly interpreted. She sheepishly acknowledged that, since it would be over the weekend, we would, in fact, not be able to get a hold of the pain doc and that we would just need to head to the ER. I told her I understood this was not her fault, but that we had specifically asked for the blood patch to avoid almost certain problems that would arise due to EDS, avoid a trip to the ER, avoid being charged for the trip to the ER, avoid causing Emily more pain and suffering, etc.

So then I, huffily, I am sure, asked about the needle. She told me that they didn’t have the atraumatic type and the doctor didn’t think it would make a difference anyway. By this time I am livid…. Halfway tempted to tell them to stop and refer us to somewhere else that can meet Emily’s needs and I told the nurse so. And, that with all due respect, I disagreed with the doctor’s opinion and that I did indeed believe that the atraumatic needle is necessary. I was very unhappy and I wanted to walk away, but, in my heart, I knew she needed this procedure done and that it could wait another week or two to reschedule it.

Then the doctor comes in and introduces herself and tells me she will be using the special needle. I immediately, and understandably, assume that I am being lied to because I have just been told otherwise. But again, I feel trapped – not because it would be an inconvenience to stop the procedure, but by sheer need. And I did not believe I was going to get a straight answer even if I made an issue of being lied to. Then I had to leave my medically complex child on the table with people who I believed were lying to me. This may be the way things are done, but this did not help my mental state.

My hubby popped down (he works at the hospital) to check on us and he found a very unhappy mama fuming in the waiting room. He had to get back to work before she was done so I was alone when the doctor came out to talk to me.

Turns out she wasn’t lying after all –  she did actually use the atraumatic needle. She said she didn’t like it, it felt weird going in, although it worked fine. I don’t know if the nurse had told her I was mad about it and she decided to use it or if she was planning on using it all along, but… whatever. The nurse later apologized for telling me wrong, implying there was a lack of communication somewhere along the line. I tend to think the doctor changed her plan in light of my anger but I suppose I will never know.

She had to lie flat in recovery for an hour, but, and this is the third EDS specific request I made, I told them she was going to need to be in recovery for longer than normal. No matter how many times I said this, they still started pushing her out the door. At about an hour, they had her sit up – and she found that her head actually felt normal for the first time in a long time. Her head felt better after the LP than it did before. Her back, not so much. In the end, I could not fight both the nurse and Emily who said emphatically, “I am starving. Get me out of here and feed me.”

So we left, I fed her and she went home to lay flat for the rest of the day.

Anyway, after all was said and done, we did fortunately get some valuable information from the LP. You may have already guessed that by her headache being relieved by the LP that she did indeed have some high pressure going on. Her opening pressure was 20.2. The doctor told Emily that normal should be between 10 and 15; I read the range is 7 – 18. Either way, Em had high pressure, although she is on Diamox three times a day. She had taken her morning dose 2 hours before the LP, which means it basically should have been reaching its peak effectiveness when the LP was done.

We have our proof as far as I am concerned. I would be fascinated to know what her pressure would have been without Diamox – not that I am willing to find out, mind you. But, my guess is it would be quite high. In fact, the symptoms that had been plaguing her: headache, unstable neck, blurred vision, light/ sound sensitivity, even that issue with her airway, all improved dramatically when the pressure was relieved. Exactly like it did September 19, 2012 when she took her first dose of Diamox.

Once we knew that there absolutely was high pressure causing the headache, the burning question became: is her pressure high because her dose needs increased or because her CO2 is low, thus making the Diamox ineffective? Hubby was able to call that afternoon and get her CO2 level – 26. It needs to be 21 or higher. This clearly is not the issue. We don’t have all of her bloodwork back yet, but that was the important one at the time.

Oddly, she had what we think might have been another CSF leak Saturday night, in her bad ear. She was laying on the side with the ear that has been problematic for months now and suddenly said that her ear was wet. I declined sticking my finger in her ear like she asked me to but her hair was damp where she had been laying. And she got a tissue and, after laying on it for a while, it was damp. But, it cleared up quickly, we think, and we saw no more signs of a leak.

However, this leak may have added to the problems that were to follow. If that sounds ominous to you, you are on the right track.

Part 2, in which we unsurprisingly and very predictably end up in the ER and have LOADS of fun,  is coming up soon…

 

 

 

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Comments on: "What a week…" (5)

  1. Cathy said:

    Does your daughter have Vocal Cord Dysfunction?? I only ask, because you thought her throat was collapsing. I’m EDS3 & have had Vocal Cord Dysfunction, since I was 12 yrs old. I found out what it was when I was 55…, at least what it is called. What I use for the attacks are cough drops with something to deaden my throat. I also stay away, as much as possible from triggers, such as getting to warm, stress, church, cramped spaces, although sometimes it happens with no known cause for me. It’s no fun for the person or anyone helplessly watching.

    • She hasn’t been diagnosed with it but she has definitely had some symptoms all along. Right now she is having some episodes we suspect to be seizures and after them she seems to have a spasm that makes it hard to move air. So, I think we will probably be looking into this more in the near future!

      Thanks for commenting!

  2. So frustrating! So sorry you’re going through all this. My EDS 17 year old and I are sitting ER as I write this….trying to figure out what is wrong with her right arm which has been an ongoing issue since July! She felt a “pop” brushing her teeth. It blew up (swollen and bruised). She has had off and on numbness and pain with repeat swelling and bruising for 6 months! She has received Physical Therapy all year let ear after 2 knee surgeries. The PT worked on the arm a little. Now she has OT for the arm. Today, when in Occupational Therapy, the OT watched my daughter “feel a bruise coming” and then it swelled and bruised all over arm and hand, yet again. They sent us to ER to check for blood clot. Probably won’t find anything again. It is always this way. She just wants it fixed!!! I do too! But just wanted you to know there are others like you and your daughter out there who have all this frustrating, long lasting issues with no answers!! Go Momma! You remind me to keep pushing for answers!

    • Sorry it took me so long to reply to your comment – hopefully your daughter is better by now!

      It is comforting to know that there are others who really do “get it” and to know we aren’t alone.

      You hang in there and we will both keep pushing for answers! Thanks for the comment and the encouragement!
      Beth

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