"Suffering the Slings and Arrows of Outrageous Fortune"

Posts tagged ‘pain management’

Rinse and Repeat: Low Pressure Headache

So, Em is struggling with a low pressure headache again. Can’t sit up without excruciating pain, nauseated, her ears feel full, her neck is stiff, the Lumbar Puncture site is sore again. Hopefully, it is just low pressure and not menengitis also. The symptoms has been going on for a couple weeks now and they are not improving on their own. We have been pushing fluids and she is spending a lot of time laying flat. She sneezed a couple times a few days ago and it has gotten worse since then. I don’t know if the leak is in her ear(s) or at the LP site or both, but she is having text book symptoms so it is obvious something is going on. She has no quality of life right now – every few weeks, she ends up flat on her back for days until it heals up. Sadly, no one seems to know what to do to break this cycle or even cares enough to try.

We have tried to wait this out, but I started suspecting another blood patch is needed and, indeed, the doctor wants her to go to the ER to get one. She is, um, less than thrilled about this. The blood patch in January was a horrifically traumatic experience. They used latex gloves, even though she has a severe latex allergy, and she did not ever get numbed sufficiently so the procedure was excruciating. She is not eager to go through that again. And when I say not eager, I mean “you shall not pass”, “over my dead body”, “you’ll have to tie me up and drag me there, ’cause I ain’t going willingly” not eager.

The doctor can’t even see her in the office until July 8th and that is squeezing her in. If they see her at the ER, they will tell us to follow up with the neurologist. When I pointed that out, I was told that she can be seen sooner by one of the other neurologists, so that is maybe a silver lining? And I am hoping that, if I can convince her to go to the ER, since we have already been informed that a blood patch is not an emergency procedure, maybe we can arrange for her Pain Management doc who is an anesthesiologist, to do the procedure. He knows her, knows what she has been through, and hopefully can manage her pain both during and after.

I know we need to document this as an ongoing problem. At some point, one of the scans is going to show SOMETHING a knowledgeable doc is going to recognize as a leak and be able to do SOMETHING about it. We have to do SOMETHING to address this round of low pressure because she has no quality of life right now. We have to keep pushing to get this addressed. Now I just have to convince her to go to the ER again and to trust that it won’t be like it was last time. I have no idea why she would believe me if I promise her that, but that is what I have to do. As a side note, it is times like this that I would love to have every doctor who has treated her badly or hurt her or trivialized her suffering to see what their ignorance has wrought. Anyway…

A while back, I found a video that I meant to share here and just didn’t get around to it. It is a really useful video and it has helped me to gain a little more knowledge and fortitude to keep fighting this particular fight. It is about an hour and a half long but, if you are struggling with these symptoms, know someone who is, or has EDS which makes you more prone to developing these symptoms,  it is well worth the time to watch this video. And, he mentions EDS as one of the frequent causes of CSF leaks so it is great in the respect that it is spreading the word about EDS at least. If I was in California, I would be pounding on this doctor’s door and begging him to help my daughter…

The Mystery Headache: Migraine, Postural Headache, Spinal Fluid Leak

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Everything else that we are dealing with…

So much has happened in such a short time, I can hardly remember what I have posted about and what I put off for later. In addition to the lumbar puncture, the blood patch, the ongoing high pressure issues and the likely CSF leaks, Em has been dealing with a concussion and that injured rotator cuff and, of course, what we presume to be CRPS.

Shoulder Injury

PT dismissed her because she was struggling intensely with the most basic exercises she was given. At the time, we were suspecting that her neck had become unstable and that was a big concern – for some reason people get a little jumpy when you mention her neck is unstable! Fortunately, a lot of the issues that we assumed were down to instability eased up when they did the lumbar puncture, easing the high pressure.

So, the plan was, talk to the specialists at Cinci and wait and see. In the meantime, her neck is better and  she has been able to gain a lot more use of her shoulder and is out of the sling. At this point, we will just carry on and hope the shoulder doesn’t get reinjured. In a perfect world, we would be able to focus our energy on her shoulder but, as we are far from a perfect world, there are too many other things to worry about. Sadly, since it seems to be improving, her shoulder is low on the list of priorities at the moment. I am just glad she has use and motion back and has less pain.

Concussion

She is doing better, but still not well. I think I mentioned that she fell and hit her head (again) on the 9th, which worsened the memory loss and balance problems. That was a little scary, to be honest but the doctor felt like the second injury should not have been enough to cause any damage and waiting it out was the thing to do. We have just made it a priority to protect her head as much as possible and keep her safe until she is steady again. Some of our precautions she doesn’t particularly appreciate, but protecting her brain is non-negotiable.

Her balance has improved from what it was after bumping her head that second time, but it is still not great. She is using her wheelchair when we are out in public. That is frustrating for her, but, I can tell you that I am so grateful we have that wheelchair in times like this.

She has started speech therapy to help rehab her brain. She had her second visit yesterday and will go again tomorrow. Her memory loss is better – she is able to recall details much better than before – but she is still incredibly frustrated when she is searching for a word and just can’t pull it up. And she is bemused at what her brain does recall and at how odd it feels to not know she knows something until she suddenly remembers.

Right now, we are still being guided by her symptoms – she can do the speech exercises in varying chunks of time before she starts hurting. Sometimes she can go straight through for a considerable length of time before her head starts hurting and other times she hits the wall sooner. This week she is going to write answers on a worksheet with various questions and we will see how that goes. She had a disconcerting time a couple days after the second bump when she realized that her brain was sort of disconnected to the actual process of writing. She said it felt like a reflex but that her brain wasn’t really doing it.

We go back to the concussion doctor on the 5th and she will have neuropsych testing done. I think she is definitely better  and continuing to improve: we are all happy with the progress she is making, but this is all new ground for us and it will be good to have knowledgeable eyes on her.

She did sit in her room and play her guitar a couple days ago and that was a wonderful sound. It has been a while and she is just now getting to the point of being able to remember the cords and lyrics that once came so easily to her. Music is such a part of her life – being able to play again is a huge morale boost.

CRPS

Way back at the beginning of December, we saw the pain doctor and emphatically expressed our need for help. (Sort of a comin’ to Jesus type event.) Bless him, he spent an hour with us on a day we weren’t even scheduled to see him and his waiting room was packed.

Anyway, I had taken in a referral form for a doctor I wanted him to send us to – a neurologist specializing in neuromuscular diseases at OSU medical center. I had filled the form out as fully as I could (and told them what to put in the lines they needed to fill out) in the hopes that there would be no excuse to not promptly refer us. It did take a polite reminder but they did eventually get us referred. Honestly, I wasn’t even sure if this doctor would see Em since she is 17 so it was a shot in the dark. His office called to ask some questions and clarify the situation and to inform us that he normally wouldn’t see anyone under 18 but that he would consider it.

Thankfully, he is willing to see her and we see him on the 15th of March. I am cautiously optimistic that he will be helpful in giving her a diagnosis and figuring all of this out. Recently, her lab results got a little more complicated and it is doubly good that we are going to him – I will get to that issue in a just a minute. I hear he is very smart and very kind so I believe we are on the right track and I am beyond grateful he is willing to see her.

New Diagnoses

Em has had a ton of labs done – between the CSF testing from the lumbar puncture and the blood work that has been done – we have at least been able to look at a lot of things that can be ruled out or addressed. We saw the neurologist last week and I was not surprised to hear him say Em is beyond him and needs someone with more knowledge than he has. He has absolutely done right by her for 4 years, so that was a little emotional. He will be available if we need him, but we need to get somewhere with more knowledge.

He was concerned – not overly but said it needs to be looked at – with one of her labs. The one test shows some markers for MS. She has three bands and four indicate MS. Now, he doesn’t think she has MS and I don’t think she has MS. I just think her entire nervous system is on fire and there is nothing at all conclusive about that test in regards to an MS diagnosis. However, she needs to be evaluated by someone who can handle all of her complexities. Fortunately, we already have an appointment with a doctor specializing in neuromuscular disease. That is exactly where we need to be and I doubt we would be able to get a sooner appointment anywhere. So, it all seems a bit Providential when I look at it.

Also, the doctor ordered an additional blood test which has come back elevated, indicating Sarcadosis. Am I surprised that at this point she is showing signs of an autoimmune issue? Not at all. Truly, I am just surprised she hasn’t shown any autoimmune signs previously. Again, and almost as always, she has enough symptoms that sort of tick the boxes for one thing but that also overlap with about a dozen other things. So, who knows. She also had a lung x-ray and we are being referred to a pulmonologist. Again,  I am not getting too worked up about this – if it is sarcadosis, it is treatable. If it isn’t, the symptoms (which, if they exist, are being masked by everything else that is going on) are certainly explained by everything else she is dealing with.

ER Complaints

We have taken our complaints about the whole lumbar puncture/ blood patch/ ER/ latex exposure debacle to administration and we are, fortunately, being taken seriously. So far, profuse apologies and some ideas on how to make sure this never happens again – not to us or anyone else – have been offered. I am not going into details right now, because this is, after all, my husband’s employer, and I want to tread lightly but suffice it to say they are doing right by us and I think we will be satisfied by the time it is all done. I am very hopeful that this will be an opportunity to share awareness about Emily’s rare conditions and help foster an atmosphere that better understands how to treat complex, chronic illness. Which, in the end, is what matters to us.

One Last Thing

Finally, I would like to ask for your prayers for my mom. She has been diagnosed with breast cancer and will be having a mastectomy in the near future. But, she has great doctors and her prognosis is good, so, even though this is a blow, we have faith that this is all going to be ok.

 

So those are the highlights from our crazy life right now. If it all sounds exhausting, it is! But, we are hanging in there and trying to keeping our sanity intact.

 

The Absolutely Predictable Trip to the ER

I really wish our lives were boring enough to be able to cover events in a single post, but, alas, that is not the case. Have been needing to split posts into multiple parts a lot lately and probably will for some time to come…

When I left off last, Em had had a lumbar puncture and, although I asked for it and the doctor ordered it, the concurrent blood patch was not done. I asked for the blood patch to prevent the very likely problems that were going to occur after the LP – a blood patch simply helps stop a CSF leak from the LP site.

Em did fine after the LP, in fact, her head was pressure free for the first time in a long time. But, unsurprisingly, she started having problems by that evening. Her back was extremely painful and she was enduring a spinal headache – low pressure. She also appeared to have developed another leak in that same ear where the first presumed leak was and that probably added to the low pressure issue. Sitting up was painful, so she laid flat for most of the weekend. We dutifully waited out that time (as ordered) to get through the window until she got to the point where she was either willing to go to the ER or until the doctors would take her seriously.

Monday morning (the 18th) Radiology called to check on her and were concerned with her symptoms and told us to call the neurologist. I did and played phone tag with them for two days. When I finally talked to them, they said to either go to the ER or call the pain doctor. I called the pain doctor and was told he was not in and they had no answers for us.

So, I informed a very reluctant and miserable Emily that we had to go to the ER. Have you ever felt too bad to go to the ER?? (I know many of you know how that is.) Well, that is where Emily was Tuesday afternoon.

When we got to the ER, they took her seriously but likely because we were able to say the neurologist sent us. Which is why I waited until we had their word to go: it can get ugly when we don’t jump through that hoop. Anyway, they took her seriously and quickly got her into a room and got an IV and pain meds started.

Here, it is probably worth mentioning the thought process of the other parental unit in this situation.  You know I was angry and frustrated. My hubby was livid that they did not do the blood patch along with the LP and forced us to the ER resulting in yet another bill. So, he was already angry that we had to drag Emily out to the ER, in the bitter cold, on snowy roads, when it likely could have been prevented.

The ER doc walked into this minefield when she came in and said Em needed a blood patch but…

Well, it isn’t considered an emergency procedure so if there were no anesthesiologists available, we would have to just go home and schedule it for the next day. You can imagine how this went over with us.

Fortunately, the anesthesiologist on call was willing to come back in even though she had just gone home. I appreciated that – giving credit where credit is due.

When she got around to doing the procedure, she snapped on the gloves that were in the epidural kit that had been sent up. Unfortunately, those gloves were latex. You may remember that Emily is severely allergic to latex, to the point of if one glove was laying in the corner or if latex had been used in that room for the previous patient, she will have a reaction. Air born latex particles will cause a serious reaction very quickly. I mean, no, she hasn’t died yet from it, but she has taken Benedryl many times and there have been times it has been scary. I would really prefer we not have to prove how bad it is through her death.

So, Em is sitting on the edge of the bed, in pain, half naked, prepped for the procedure, scared out of her mind because she hates needles and vividly remembers how much the LP hurt and has been told this is going to be the same. I am not paying attention to anything but Emily at that point. I am in front of her holding her up, because I know she cannot hold herself up. She looks at me and asks if those gloves are latex. I look over her shoulder and ask, mostly to humor her, because they won’t make that kind of mistake, right?

When I ask about the gloves, the doctor stops in horror and says, oh crap. (Or something like that. My mind was racing at that point but that was the general idea.) And rips them off, apologizing. I look back at Emily and ask if she is ok. She says, “Not really.” So I tell the doctor she needs Benedryl. She assures me she didn’t touch her with the gloves and was horrified to find out that just taking them out of the package was enough to causes a reaction. So, she wraps everything up in the now unusable kit and rushes it out the door and gets the nurse, who brings in Benedryl. (Funny story, she brings it in with a syringe and Emily, in the midst of an allergic reaction, is like, hey, I have my own Benedryl in my bag, no need to give me a shot. I am good. Needless to say she was relieved to find it was going to go directly into her IV, although she was understandably suspicious until it was done.)

At this point, I am past livid. Especially when I realize they hadn’t put an allergy bracelet  on her when they checked her in. Would it have made the difference? Honestly, I doubt it. But that isn’t the point, is it? And why does this ill child have to save her own life?

So, they have to order a new kit and leave her sitting, miserable, freezing, scared, and feeling like they had just tried to kill her, for far too long. Finally, they get the new kit and the doctor and the phlebotomist both come in. The phlebotomist sort of joked that the hospital is a latex free facility and they must have found the only pair of latex gloves in the place. I call BS on that because I know better. Their system failed at every step of the way and could have killed her. This is being addressed.

The procedure was, well, not fun.  The doctor was trying to get her numb enough to do the procedure but never really succeeded. They took a huge amount of blood (20 mls) pretty quickly, too quickly for her. If I had not been holding her up, she would have been on the floor. You can’t take that amount of blood from a POTS patient without some effect. I thought she needed more fluids afterwards but she just wanted to go home so I didn’t push that issue, although I wish I had.

They gave her time to rest after the procedure and  then basically kicked us out. I asked for them to do something for her pain, which was now as bad or worse than it was when we arrived, because we had a 45 minute ride home and she was in agony. They had given her morphine, a couple hours before and were not inclined to give her anything else. They offered her a pain pill, half of what she already takes. The doctor came in and explained since she didn’t know exactly what was going on, she didn’t want to throw narcotics at Emily, which she ironically did anyway. She also implied that we would be violating our contract with the pain doctor if we pushed the issue.

In the end, we had a child who was in more pain than she was to begin with, making a liar out of me. I promised her they would help, that they would treat her pain. Once again, they have  bolstered the wall between this ill child and the care she needs by showing me to be a liar and refusing to take her seriously.

These experiences leave us battered and bruised, especially when they should have been prevented. There will be more to this story: we are not going to accept this kind of treatment without making some waves. The latex exposure is huge. Huge. That needs to be addressed. Because my husband works at this hospital, our insurance forces us to do everything possible there. If her specialists at Cincy order blood work or an MRI, can’t do it there, have to go to our local hospital. If she needs to go to the ER, we have to go there. But, they clearly are not providing a safe place for her to be treated. So, yes, this one is a hill worth dying on. They either need to get their act together or allow us to take her somewhere where she will be safe.

We also are going to fight the policy that prevented the blood patch being done along with the LP. I am not stupid – I get that the two procedures are done by different departments and it is complicated, but are you telling me it is impossible to schedule this? Seriously.

At best, this is a situation where the right hand doesn’t know what the left hand is doing. At worst, well, I hate to speculate about profit, but there it is. Then there is the fact that her doctor ordered the blood patch. Ordered it. I was not aware that a doctor’s order could be viewed as a suggestion. If they couldn’t do it, it was on them to contact him so he was aware. They did not call him, neither before or after, I have been told by him personally. Again, their system failed this child at every point.

So, we are beyond angry. There is absolutely no reason any of this needed to happen. To be told they can’t do the blood patch along with the LP and that we just have to go to the ER if there is a problem, only to find out the blood patch is not an emergency procedure, is mind boggling. To be told by the pain doctor’s office they have no answers for us. The whole thing is a nightmare, to be honest.

On the upside, I think the blood patch did help. Her pressure is normalizing (read: getting high again) and she is trying to manage the increasing pressure with her Diamox but not taking too much so it dips down into low pressure. That is a juggling act to be sure. The procedure did not help her back pain, but that is not too surprising. She is still in enormous pain – every movement is painful, but at least the blood patch wasn’t pointless.

We were able to get in to see the neurologist on Friday. That was a minor miracle. And there are other things going on as well, so I will be posting about all of that, trying to catch up. And, with any luck, there will be more to report on how our complaints are received. I don’t want anybody fired, although Emily and I have thought it would be useful to make everyone who had a part in the whole latex portion wear a scarlet “A”. I am thinking it would be an effective deterrent, but I suppose I won’t push that since HR might object.

Honestly, I just want to educate the people providing my daughter’s care. I want to help them be better. I want them to take her problems seriously. I want them to take chronic illness and chronic pain patients seriously.  I want policy to change – how many people have suffered and had no recourse like we do? It is not ok. I want to help them offer the care my kid needs. But, there are things that need to change and we are going to do our best to see that happen. In the meantime, Emily is slowly recovering – her physical wounds will heal but her emotional ones will take longer, I think.

 

A Win: Finally

Picking up the tale where I left off in yesterday’s post, we were in desperate need of someone to help. Em’s pain was out of control, the Rheumatologist was clearly not interested in helping, and we had wasted 3 months seeking help from doctors who had no interest in helping.

I was done, with the whole situation. Done waiting. Done playing nice. And, I knew that our best chance of getting some help in the near future was from the Pain Management doctor. Yes, he had rather foolishly and unhelpfully stated “if it isn’t broke, don’t fix it” at our last appointment but we have always liked him and he has had Em’s pain under control for 3 years now. So, if it wasn’t him, we were going to have a problem.

I have been researching, talking to others living with CRPS, and formulating a plan of sorts. I will be the first to admit that it may not be a perfect plan, but it is far and away better than anything anyone else has come up with to date. So, I prepared for our appointment and was ready to fight for my plan, while being open to any constructive ideas.

My plan involves two parallel tracks. The first track involves getting to a neurologist who actually knows CRPS. If he has an opinion about Small Fiber Neuropathy, so much the better. We need someone who can advise and guide us, from a position of actual, current knowledge not a position of vaguely remembering learning about CRPS 20 years ago. In a CRPS facebook group,  I heard a name of a doc at OSU whose interest is in CRPS and autonomic dysfunction. I have no idea how good he actually is but it is a name and that is the best I have right now. So, I wanted a referral.

The second track is Ketamine Infusions. This is a very big gun but we are facing a very big enemy. A big gun is absolutely necessary, in my opinion. We know how serious a treatment this is but everything I am seeing says this is our best hope of getting Em into remission. There is no guarantee that it will but I believe that it is our best shot. Because her CRPS (assuming that is what she has) is full body, some of the other treatments are just not options. Furthermore, she is already on many of the meds that would be the first choices to treat it, so our pharmaceutical options are a little limited. Narcotics are not the answer – we need to stop the pain cycle, not drug her. So, Ketamine. (And, may I say, this is the hardest thing about parenting a medically complex kid. The doctors can’t or won’t make these decisions so the parent is left to push for it. All I can do is pray that my instincts are correct.)

At our last visit, I mentioned Ketamine and he was not receptive to it. But, this time I was determined to have a serious conversation. This time, I also had a name of a doctor in Indianapolis who does Ketamine Infusions for CRPS and I was asking for a referral to him. However, I decided that it would be better all around for us to have Dr. V do this treatment at our own hospital – it would be easier on Em to travel less and it would benefit others with CRPS in the area if he can offer this treatment. It seemed wise to push for Dr. V to investigate/ learn how to do the infusions but have the referral request as a back up plan.

So, I had printed off both referral forms for both the neurologist and the other Pain Doctor – I had them filled out as much as I could and had all the paperwork requested attached. All they had to do was fill out their part and fax them. I must admit, this was a stroke of brilliance on my part. 🙂

I also had numerous articles about Ketamine and how this is the treatment of choice, how to actually do the infusions, billing codes (inpatient, not outpatient as we would be hoping for but it proved my point that this is a legit treatment), and a page of contact info of several docs who do these infusion who Dr. V could consult with.

I was ready to fight. To throw a fit. To get hysterical. Whatever it took, this was the day.

We got there and I was already a little discouraged – they were clearly swamped and running late and I worried that we would be brushed off. The NP, who we have normally been seeing for the past year or so, came into the room, her normal cheery self. She took one look at us and knew something was wrong. She sat down and listened intently. As soon as I mentioned Ketamine infusions, she said, “you are going to have to talk to the doctor. I can’t help you.” I expected this, but I had anticipated that we would have to make another appointment. Instead, she fetched him immediately and he sat down with us ready to talk about Ketamine. He expressed his concerns but was open to discussing it. I handed him articles about this treatment and he skimmed through them right in front of me, and made comments that indicated he was actually absorbing the info. He thanked me for doing so much leg work for him.

He stepped out to make a phone call, saying he was getting his billing people to look up billing codes. We discussed it further and we agreed that there were some obstacles to over come – insurance being one. He suggested oral Ketamine as a more immediate option. He stepped out again. I think he talked to someone about finding a compounding pharmacy to prepare it. I am hoping he figures out that Ketamine lozenges are a real option – I know a couple folks who are getting real relief from them.

So, at this point, he is willing to look into infusions. He knows the doc in Indianapolis who I was going to ask to be referred to and can talk to him about how to do the infusions and billing stuff and hopefully make it happen. Not tomorrow, but hopefully soon. And that was as much as I could hope for.

In the meantime, he wanted to try a couple new meds. Cymbalta and Nucynta. And he upped one of her current meds to try and get her some immediate relief. I am not thrilled with adding two more meds but I am very thankful he understood that she needed something NOW. We will just cross our fingers that she tolerates these meds and they don’t cause more problems. Serotonin syndrome is a big concern but we are watching her closely and it is a calculated risk.

He agreed to refer to the neurologist at OSU – the sooner the better because there is always a waiting list. We will fight to get her in asap but we have to get the referral first. He seemed surprised that I had a name, because he has had no one to refer patients to. This could be positive for other patients as well.

He spent an hour with us, on a day when he was not scheduled to see us and when he had patients already backed up in his waiting room. We didn’t get around to talking about what to do about her shoulder and we didn’t get around to talking about a plan on how to handle future ER visits if her pain is out of control. And, we left the whole Ketamine issue a little more open ended than I wanted. He is going to look into it – hopefully he will call with answers, but I can call the office and ask if I don’t hear from him. All things considered, I was so grateful for the time he spent with us and for him taking the issue seriously, that I would not complain at all.

I feel bad that the other patients were waiting so long, especially the gentleman who was already mad about his billing situation and entertainingly vocal about it, but, honestly, Em has been juggled and passed along and ignored for 3 months while living with the most painful condition known. It was our turn to be focused on, to be cared for, and it was, quite frankly, overdue. After the awful ER visit, after being dumped by the Rheumatologist, after months of everyone just saying, “Good Luck”, we needed a win. And this was a win.

In reality, we are no further ahead than we were before Friday – although two new meds to try is progress. We still have no answers, no diagnosis, no obvious path to treatment. But, we have a sketchy plan, a name of someone who might help, a treatment option that wasn’t an option before, and we have a doc who cares. And we have hope. Which we were pretty short on before this visit. So, while we technically aren’t further ahead, it feels like we traveled miles on this journey in one afternoon. Because one doctor went out of his way to help us.

That shouldn’t be amazing. It shouldn’t be a novelty. But it is. And we are very, very thankful.

The Latest in Our Ongoing Adventures

Things have improved, slightly, since our awful visit to the ER. Not her pain, that is still uncontrolled and terrible, but we are a few steps closer to getting answers or at least some real treatment.

MRI Results

Em’s MRI was last Wednesday and it actually showed a problem. Turns out, she has “Tendinopathy of the Supraspinatus Tendon” which means that tendon is either irritated or torn. Putting it in simpler terms, she has a rotator cuff injury. Personally, I think it is a tear, since her description is that she had it resting behind her head in bed and all of the sudden something popped, it went numb, fell down and she couldn’t lift it. Unfortunately, it keeps getting re-injured in her sleep and the slightest movement away from her body causes awful pain and makes it go numb again. We are figuring out what to do about it. The doctor who ordered the MRI probably will not be the doctor who manages the problem, so we are going to have to get her to the ortho ourselves.

And, we have resisted the urge to pop into the ER and see if our “favorite” NP is there and do a we-told-you-so dance at her. Bottom line here, is that Em needed an MRI. We were right, the NP was wrong. X-rays would not have shown a thing and would have only hurt her more and possibly made it worse. (Em is emphatic that she knows that the would have made her move her shoulder in ways that would have hurt and maybe damaged it more. At this point, I am inclined to believe her) And, if she had just gone ahead and ordered an MRI while we were there, we would be a week ahead of figuring out how to treat it.

So, we are feeling pretty validated after finding out the results. That validation comes at a pretty high cost though. However, it does confirm that, generally, we know what we are talking about and that will make facing down rude doctors (or NPs) a little easier. Confidence is  good thing.

Bloodwork

The Rheumatologist ordered some bloodwork – mostly to check for Vit B deficiencies – when we were there in November. We had to do it at our local hospital and not Children’s for insurance reasons, and I think the Rheumatologist still doesn’t have the results. I am not quite sure if it is their fault or the hospital’s fault but it is ridiculous. I am not happy with any of them, to be honest. My husband finally just called and got the results. All but one is fine – it looks like her B6 is low – which could contribute to neuropathy. But, again, the doctor who ordered these doesn’t seem to want to manage this, so we are pretty much on our own with dealing with this.

Rheumatologist

We really liked him – he was a great guy and all – but he seems to be washing his hands of Em. He prescribed a med called Trileptol, in the hopes it would help her pain. As she was titrating up to a full dose, just a couple days into the process, it was helping her pain but it messed with her head in a pretty significant way. She has always been “feisty” but this med made her uncharacteristically angry and unreasonable. And I won’t say she was suicidal, but let’s just say she was talking in a way I had never heard from her before. It was pretty scary. And dealing with it was  complicated because this was all taking place the day before Thanksgiving, the day Em asked to go to the ER. She stopped taking it and the nurse I talked to tried without success to talk to the doc about it. She finally called back, having not been able to talk to him, and said don’t take it anymore and that she would make sure the on call team knew about the issue if we needed more help.

The office eventually called back Monday – after the holiday and the weekend – with his thoughts. He said that med shouldn’t cause mood changes (if that is true, why did the info from the pharamacy say it does and to inform your doc?) and that she should go back on it and titrate up more slowly. Riiiggghhhttt.

So, yeah, Em will not be taking that med anymore. Haven’t heard back from him and the nurse – I don’t know if she was just reading from our appointment summary or if he gave her new notes – informed me during that call that we should just go to the Pain Management doc to get more pain relief and to “find someone to refer us to someone who can investigate a diagnosis of Small Fiber Neuropathy”. That makes me insane: “Oh, I think you don’t have that unusual disease that you think you have, I think it is this one, which is far more rare and nearly impossible to diagnose. And I have no plans whatsoever to help you get that diagnosis.” Hence my belief that he is washing his hands of us. Lovely.

By this point, I was starting to get fairly annoyed. Or possibly livid. Possibly ready to go to war and/ or all Mama Bear on the next medical person we encountered.  The doctor we waited two months to see had nothing to offer and dumped us. That was the final straw. Every single doctor we had seen about the CRPS had played hot potato with this kid. She was tossed to the next guy with a polite smile and a hope that he would do something. And, if that didn’t happen, what did it matter once we were out of their office?

We had a pain management appointment on the 4th and I decided it was going to be our line in the sand. If I had to throw a gigantic fit, he was going to do something constructive to help my child. That appointment deserves its own post, so that will be up tomorrow. It was good and we so needed good right now.

Another Ear Infection

After a very long PM visit on Friday, Em said we needed to get her ear looked at and, since we were right there, we popped into Urgent Care. I don’t think I mentioned here that, while we were at the Rheumatology visit on November 19th, they looked at her ear and said she had a perforated ear drum. At least the doctor doing the exam was pretty sure it was – she looked at it a long time and said she was pretty sure it was perforated and to make sure to keep the ENT visit that is scheduled for the 15th.  So that ear is just an ongoing issue.

Weirdly, which is her norm, during the MRI on Wednesday, the noise made her ear suddenly pop and hurt. Since then, she wasn’t able to hear out of that ear. She knew she wasn’t going to make it another week and a half until the appointment, so we popped into Urgent Care.

Good thing we did. The poor doctor looked in her ear. And looked. And looked some more. Then said – and you just LOVE to hear this as a parent – “I’ve never quite seen anything like this before.” She couldn’t even see the ear drum because there was “white powdery stuff” blocking the view. She grabbed the other doctor to come in and look. Fortunately, this was the same doctor we saw last time and, not surprisingly, she remembered us.

Long story short, it is either a fungal infection (Em asked, “if I google spores, will I be freaked out?” They told her not to google it.) or fluid that has crystallized from the ear canal after the ear drum perforated. It was very fortunate that we knew her ear drum was perforated as of the 19th, because that means ear drops are a no no. Initially, they apologized that they had nothing to offer and said they would tell the ENT to see her asap. They came back and said they had ear drops as a suspension fluid that she could use, so, we have been doing ear drops 4 times a day again. And, I am currently waiting on a call from the ENT. I doubt they will see her today yet, but hopefully they can soon.

We are hanging in there. A lot is happening. We still have few answers but are slowly getting there. I will post about the pain management visit tomorrow.

To the Nurse Practitioner who saw my daughter in the ER Thanksgiving Eve:

To the Nurse Practitioner who saw my daughter in the ER Thanksgiving Eve:

It was the day before Thanksgiving. You probably didn’t want to be there any more than we did. But, I am willing to bet that the time we spent in the ER  left more of an impression on us than it did you. And, I have a few things to say to you.

My daughter – my beautiful, smart, precious daughter who has lived for the last 6 years in such chronic pain that would put you and most other “normals” on the floor, who has had her childhood ripped away by a genetic condition, who has experienced loss and suffering and affliction, the likes of which you can probably only imagine, yet does not view herself as a victim and refuses to be defined by her medical condition – came to you for help. For the first time in 6 years, she asked to go to the ER because she was in such pain.

It was complicated – with her it always is. She is medically complex. A bemusing mash up of chronic and acute issues is what we threw at you that day. That is not what you normally see and it was not clear cut. I get it. I even sympathize, a little. But, this is our daily reality and frankly, I don’t think it is too much to ask for you to do right by a kid who is desperate for help.

I tried to peel back the layers for you – she has Ehlers Danlos Syndrome, she has autonomic dysfunction, and she has something else – probably Complex Regional Pain Syndrome, the most painful condition know to medicine.

It was her shoulder that brought us in to the ER that day. It had dislocated August 1st. That injury started the CRPS, which quickly spread to her whole body and the shoulder has been a worsening problem ever since. We had seen the Rheumatologist a week earlier and he was very concerned about that shoulder, because she couldn’t move it after it simply gave out a couple days earlier. The earliest an MRI could be scheduled was December 2nd. She reinjured it in her sleep and the pain was just too much.

And she asked to go to the ER. Because she needed help. We thought the least you could do was go ahead with an MRI, so we could figure out the source of the pain or if there was any damage. And, I was hoping for something to help get her pain under control so she could participate in our family Thanksgiving.

We weren’t drug seeking. I flat out told you we didn’t want another prescription. She already has pain meds that aren’t working. I don’t want to throw narcotics at her. But, when your child is shaking and pale and nauseous because of her pain and has been for days, and when she has had a bad reaction to the pain medication the Rheumatologist prescribed and for the first time you hear her say ‘death would be easier” and you honestly don’t know if it is the side effects or the pain, you ask for help.

What we got instead was attitude. You were dismissive from the moment you walked into the room. We were probably defensive – living with a rare chronic illness that is poorly understood by the medical community will do that to you. This may come as a surprise to you, but the ER is the last place we wanted to be on Thanksgiving Eve.

You asked, rather snidely, what we expected you to do.  Let me tell you now: I expected you to listen. To show some compassion. To offer ideas. To order an MRI. To show some humanity.

Did my daughter communicate effectively? No, she didn’t. Because she was in profound pain. Agony. She is trying her very best to manage a handful of medical challenges that make adults weep. Of course she didn’t communicate well.

She is still learning to advocate for herself appropriately and effectively. All of the past negative experiences with doctors have built a wall that has to be dismantled if you want to really reach her. Furthermore, her medical conditions cause brain fog and anxiety: she has trouble finding the right word, particularly when stressed and in pain. Complicating all of this is that the new med that was supposed to help, instead turned her brain to an angry mush of aggression and depression and she was not in a good place.

And she is a child,  a scared little girl, a prisoner of her own body,  tortured by her own central nervous system and her genes, who decided to take a risk and ask for help.

You, on the other hand, are the adult. And the professional. The onus of communication is on you. You could have shown some sympathy. You could have listened to her. You could have walked in the room not assuming we were drug seeking. You could have acknowledged her case was complex and confusing and made an effort to understand the challenges that we live with on a daily basis. Instead, you copped an attitude and acted surprised when things went downhill.

You begrudgingly offered an X-ray instead of the MRI that we all knew was necessary. Perhaps if you had been a little more compassionate and actually allowed her to express herself, if you had asked questions and cared about the answers, you would have been included in the conversation that followed. Instead, my daughter, and you would have understood why if you had listened to her, acted like the frightened and angry child she was and refused your x-ray. She may only be 16 but she knows perfectly well that not one of the multitude of x-rays she has had has EVER shown any conclusive results. She knows how much it hurts to have X-rays when you are in pain. She also knew you thought she was drug seeking. And she wanted to leave and never lay eyes on you again. She has zero tolerance for crap on a good day and this was so not a good day.

In the meantime, I was desperately texting an SOS to my husband who was finishing up his work day, three floors up in the hospital. When you walked back in the room, prepared to lay into my child for refusing the x-ray, you were obviously taken aback by a hospital employee that you know, standing in the room and standing up for his daughter. I can only imagine how things would have gone if he hadn’t shown up. I am pretty sure it would have involved my daughter telling you what you could do with both your condescension and your x-ray as she stormed out, with me juggling purse and coats and trying to salvage the situation.

Instead, you perhaps understood for the first time, that we weren’t random people who were crazy and stupid, and you changed your tune a bit. I cannot tell you how sad I am for your other patients who are also not random, crazy, stupid people but don’t know someone who works at the hospital and cannot salvage their visit at all. I mean, you were still pretty snide, but you finally offered a shot of morphine or a pain patch. Emily chose the pain patch just so she could go home and get away from you, vowing to never return, no matter how dire the situation. (That should scare you as much as it scares me.)

Forgive me if I am not overly grateful for the bone you threw us: if you had actually known about EDS like you pretended to, you would have been aware that she has fragile skin and the adhesive on the pain patch could be a serious issue, which it  has. And, I’d probably be more grateful if they had helped, which they haven’t.

This particular day was just another stop on a very long and winding road. We have had some really, really good doctors over the past 6 years. We have had some really bad doctors throughout this journey. The bad ones leave such deep scars that the good ones can barely get near us for our reflexive flinching at a white coat, like a battered and abused child who never knows if the outstretched hand will be gentle or inflict pain.

Unfortunately, you inflicted a wound that will leave a scar. One that will make it harder for her to ever ask for help again. The next time she needs help, she will be more likely to just give up and suffer. And that is potentially deadly. That scar makes my already difficult job parenting a medically complex child that much more difficult. I am angry. I am frustrated. Mostly, I am sad and tired from fighting this fight alone. But, I have sworn a solemn oath to this child that I will never stop fighting for her. I cannot and will not give up.

At the end of the day, we have only our own reaction to this experience – it is the only thing we can control. So, we have decided to turn this into a positive: We will use this experience to empower Emily by helping her learn to communicate better in the future.   And, instead of feeling victimized by this experience, we will have a discussion with the head of the ER. Not to get you in trouble or exact a pound of flesh, but to wrangle some good from a bad situation by educating. We have a unique opportunity to educate and advocate for awareness of EDS and CRPS – if we step outside of our comfort zone and reach out to the ER staff in good faith, it will benefit Emily and possibly countless other chronic illness patients.

We are determined to learn and grow and find some measure of good in this bleak situation and I can only hope you might be willing to do the same. Please, next time you encounter someone like my daughter – hurting, scared, scarred and a little angry – do better. Your actions matter and my daughter, and all of the other chronically ill patients who pass through your hands, deserve better.

 

 

 

 

Update on CRPS, Shoulder Injury and Ear Infections

A lot has been going on, so I will will try to catch everyone up…

On November 19th, we saw the Rheumatologist (Dr. Henrickson) at Cincinnati Children’s. On the upside, we liked him. We liked the Rheumatology Fellow who talked to Em and took her history. Dr. H said we were educated on Em’s issues and thus in a good position to beat this. When I mentioned being in an CRPS group on facebook, he didn’t chide – he just said our knowledge was useful. That earned him points, let me tell you!

We felt that they took her pain seriously and listened to what she had to say. He added a med to try and help with her pain, Trileptal. She has to titrate up to a full dose over the next week or so, but she thinks the tiny dose she is currently on is helping. That gives us hope that the full dose will help. Which is good because she is in incredible amounts of pain every moment of every day. I have never seen her in pain like this, not even back in the days before she had any pain management. The situation is getting dire and there are no easy answers. I doubt they have a clue how much pain she is in, because she has gotten very good at putting up a front to hide her real desperation. But, they took her as seriously as they could and I appreciate that.

As far as an actual diagnosis, we still don’t have one. He essentially (my best interpretation of what he said) believes that once Complex Regional Pain Syndrome spreads from one region to the whole body, that it is no longer CRPS, that it becomes “something” else. He suggested that, depending on what some of the testing we are doing, the more likely diagnosis is Small Fiber Sensory Neuropathy. The problem is, Cincinnati Children’s can’t do the testing for that. Only a few hospitals in the country can, so this is another rare diagnosis that would be challenging to pursue.

My problem is, as far as I can see, Small Fiber Neuropathy really doesn’t fit her symptoms, not as a whole, anyway. She would be a very atypical case if that is what we are looking at. Emily said that she feels like for the first time a doctor has ignored the more likely explanation and opted for the LESS likely, MORE rare explanation. And, when it comes to treatment for SFN, it seems that it is a matter of addressing the underlying cause (diabetes, for example) and controlling it with pain meds. If, what she has is actually CRPS instead, treatment for SNF would mean the CRPS would go untreated and that would be a very bad thing. So, I am just not buying this idea of SNF – in my heart, I believe she has CRPS, which has rapidly gone full body, and needs aggressive treatment to break the pain cycle that her central nervous system has developed.

Complicating our visit and his understanding of her pain, was a new shoulder injury. The left shoulder, the original dislocation that started the CRPS spread in the first place, has been a problem off and on since. A couple days before our appointment, her shoulder just sort of gave out. Went numb and floppy. She put it in a sling because that has helped in the past but instead of getting better, it got worse over the next couple days. Usually, this type of injury gets better after a few days. Not this time. By the time we saw the Rheumatologist three days after the injury, she couldn’t move it and it was causing her severe pain.

So, he wants her to have an MRI. We have one scheduled for the 2nd. In the meantime, she is profoundly miserable and I think she may have really messed her shoulder up this time.. The pain, a combination of CRPS pain and a damaged shoulder that hurts with every slight movement, is so severe that she shakes and gets nauseated from it. She doesn’t want to, but we may end up at the ER to try to get her pain under control. With luck, they could do an MRI right there so we don’t have to wait until Dec. 2nd. The situation is getting desperate, to be honest. She should not have to be in this amount of pain.

The MRI will help us know what is going on with the shoulder, and he ordered some bloodwork to check on vitamin deficiencies that could cause neuropathic pain. Baring no useful results there, he wants her to do PT and desensitization. If that doesn’t help, pursue SNF testing.

As much as we liked this doctor, I am not loving his plan. I think she needs to be seen by someone – a neurologist who knows both CRPS and SNF. If this is CRPS it is crucial we start treating it – the longer it takes, the worse her prognosis. I am weighing our options and figuring out a path that will take us where we need to go. The treatments for CRPS will not hamper treatment for SNF if it turns out to be the true diagnosis, but, from what I can see, treatment for SNF would not adequately treat CRPS if that is what she has. It seems prudent to pursue the diagnosis/treatment we can now and wait to investigate SNF down the road.

Basically, everything is still up in the air and we have no more answers than we had to begin with. Except the exam at that appointment revealed that she has a perforated ear drum. They either missed it at Urgent Care or it happened since she was last there. We have an ENT appointment set up for the 15th, but her ears are so painful and she has developed vertigo, so we may end up back at Urgent Care in the meantime.

We are tired. And stretched thin.  And scared. And frustrated that we are not being offered any real help in what we know to be a desperate fight for her future.

But, we are hanging in there and at least have an idea of where we need to go. For now, we are just taking this journey one step at a time.

 

 

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