Catching up…

Been a while since I posted – and there have been a few important developments to mention.

I have been totally out of it for a week and a half due to a nasty kidney stone. Apparently that 7 1/2 mm stone that I have been fighting for 5 months decided to rear its ugly little head and needed an attitude adjustment. I ended up in the ER, had a shockwave procedure (where they use ultrasound to break up the stone) and then had a stent placed. Both procedures were minor surgical procedures and I was under general anaethesia  for both. Spent a few days in the hospital and have been pretty well out of it since I came home. Not fun at all. The only good things about this adventure is that my mom was able to come and stay with the kids (and me) and that Em has improved so much with the mast cell treatment that I didn’t have to worry much about her. I am slowly recovering and hopefully this ordeal will be over soon.

So, on to the updates…

First, the mast cell treatment (zyrtec and zantac) has been more successful that I ever dreamed. Em is eating without pain and is up and around through out the day. She went to our homeschool Co-op classes yesterday without her wheelchair (!!!) and I attribute that to the mast cell treatment.

Second, and also very important, the amitriptyline has finally started working well. We had to bump up her dose since we only had a few 10 mgs and a new script of 25 mgs. The 45 mgs seemed to be working pretty well. In fact, she has been falling asleep before midnight and waking up, on her own, by 11 am. She finally told me that, on this dose, she was falling asleep quickly, sleeping well and waking up rested. RESTED!!! – she hasn’t had restful sleep for months and least and maybe not for years. We are bumping up the dose to 50 mg, just for simplicity in dosing and if she tolerates it well, we will stick with it. The pain relief aspect from the ami is still in question – but she may be getting a little benefit from it. Hard to tell since she has been way overdoing while I have been ill.

Third, an update on the diamox saga. My husband had to take Em to her appointment with the pain doc at Cinci without me. I didn’t really think this doc would agree to prescribe the diamox but I thought he would at least listen. He sort of listened , a bit dismissively, and refused to help. That was expected but still discouraging. He wants to wait 2 weeks to see how she tolerates the 50 mgs of ami before he prescribes nuerotin for her pain. That is frustrating but I understand. All in all, it wasn’t a great visit and I really wish I could have been there. Probably wouldn’t have changed anything but I would have been able to explain the mast cell and Driscoll theories perhaps better than my husband. Although, I think he did a great job – most likely nothing would have changed even if I was there. There were a number of concerning things about that appointment but maybe I will write a separate post to detail them all. Would take too much space here… Suffice it to say, we are getting more and more annoyed with doctors in general!

We still have our plan d for getting diamox – an appointment on Tuesday with the local neurologist for a second opinion. Praying hard that it is a good visit and we are listened to. I still believe that if I can get a doc to listen to me for 5 minutes with an open mind, we will leave with a script for diamox in hand.

So, a lot has happened in the past couple weeks and we are still trying to move forward. Can’t wait to feel better so I can get on with taking care of Em and focus on her health instead of mine!

Meds…

We have started the mast cell treatment yesterday – Zyrtec and Zantac. On our own, of course, since I have yet to find a doctor who will even let me ask to try it. So, I figured my best option was to present them with a done deal  – it is easier to ask forgiveness, than to get permission! And, since this is over-the-counter, I feel perfectly safe to try it without medical supervision. If it helps, that will be the ‘proof’ we need to stay on it and it, theoretically, should help bolster our request for Diamox -since they are each part of the theory and treatment. If it doesn’t help then, obviously, she shouldn’t stay on it.

Yesterday, she took both medications when she got up. By 3 o’clock, she said she wasn’t itchy at all and she thought the weird rashy/hivey spots that she gets regularly were gone. Her dizzyness was less and her headache even seemed better. Her stomach was hurting much less and the nausea was less as well. She actually asked for a meal – turkey, mashed potatoes & gravy, macaroni and cheese. Oh, and brussel sprouts. Before all of the neurological symptoms, she was an excellent eater and loved things like broccoli and brussel sprouts. (Yes, she is strange!)

Anyway, she ate and ate some more. Not only was this the first time in months that she requested an actual meal, but it was the first time in a very long time that she had seconds – usually, she pushes her plate away before her food is half gone – everything she eats hurts her stomach. So, we felt that the mast cell treatment was going swimmingly. Unfortunately, her stomach wasn’t quite ready for all of that food and she felt awful after eating so well. Two steps forward and one step back.

I am still very encouraged by what small improvements we have seen after even one day and fully expect that after a couple weeks, there will be substantial improvement. I had printed off a list of her symptoms – both mast cell and neurological – and asked her to note any changes each day. 19 symptoms had reduced from her normal daily level and 16 had stayed the same, after one treatment. I am very optimistic.

On the amitriptyline front… Since the evil neurologist told us to gradually increase it to 62.5 mgs and we ended up having to fill that prescription, we have decided to use this opportunity to increase her dose beyond what the pain clinic has prescribed. If we get to a ‘good’ dose, we can halt there and talk to the pain clinic about it on the 14th. Honestly, we are kind of stuck since the new 25 mg prescription makes the old 10 mg prescription obsolete. It is better to use what we have to gradually increase the dose than to be stuck suddenly taking an increased dose when the old script runs out. We have increased it to 35 from 30 and may bump up to 45 next week. Better that than 30 mgs to 50 mgs, over night.

I am feeling that we are approaching a good dosage, but that is muddled by the fact that she is taking benedryl at night and that definitely helps her sleep. Once we really get going with the mast cell treatment, I hope she can not take the benedryl a few nights to allow us to determine which med is actually helping her sleep.

Her sleep schedule has definitely improved over the last couple weeks but I don’t know if that is due to the ami or the benedryl or a complete coincidence. She is falling asleep around midnight, sleeping through the night and waking up on her own around noon – and sometimes even earlier. This is a huge  improvement. Of course, last night, she was awake until 4 am but hopefully that was an anomaly and not an end of a good run.  It could be the Zyrtec or it could be just one of those nights. Will have to keep an eye on her. Hopefully, she tolerates the Zyrtec well and it doesn’t create more side effects!

Med update

The amitriptyline is still not working – not optimally, at least. Em has had a couple nights of falling asleep early since she started taking it. ‘Early’ as in 1 am. Otherwise it is somewhere between 3 and 5 am before she falls asleep, which is somewhat better than 7 or 8 am, but still…

So, this dose of ami is certainly not doing the trick, yet. We probably need to up the dose again – 20 mgs is still a bit low – but she has only been on the increased dose for a week, so we should probably wait another week before requesting to increase it again. It is one of those situations where patience is called for – and neither Em or I have much patience in this area at the moment. But, we are in this for the long haul and it is very much trial and error. Frustrating because until we figure out the ami, we can’t start the next layer of pain medication. She has gone back to taking the flexeril at night. It wasn’t helping her sleep, but it was preventing the muscle spasms that are so painful.

Otherwise, not much news. Still looking into the Driscoll theory regarding intracranial pressure and into mast cell activation disorders. There is a lot to figure out regarding the mast cell stuff and very little info to rely on so it is a bit confusing but I do think it is a real possibility.  An intriguing puzzle to put together and just one more ridiculously rare condition to manage. Such is our life!

Tired of waiting on insurance; really, really wanting some resolution regarding the neurological symptoms. Tired of seeing my baby sick and hurting and unable to live. Tired of the whole thing.

And yet, we push on and have hope. Crazy thing, that! 😉

Amitriptyline Update

Em has been on Ami for a week. Started at the lowest dose and it really was doing nothing for her sleep. I called the pain clinic yesterday and they gave us the go ahead to up the dose to 20 mgs. Again, I figured she would be knocked out and, again,  it didn’t happen.

She was somewhat tired but couldn’t sleep. I think she finally fell asleep around 3 and was restless for the rest of the night.

I am getting more and more convinced that her sleep issues are related to adrenaline – literally too tired to sleep. An article that describes this in EDS patients can be found at http://www.dynakids.org/Documents/hypermobility.pdf – you can scroll down to where it talks about sleep issues. Interesting theory. And I wonder if the [theoretical]  high intracranial pressure could be putting pressure on the pituitary gland and straining her  endocrine system, causing adrenaline fatigue. Also, the amount of pain she is in on a daily basis is enough to stress the adrenal system so she might be getting it from a number of directions. Just my musings on a subject that never goes away…

Anyway, we will try this dose for a while and may bump it up again if it doesn’t help. She seems to be very resistant to the effect of meds – which is typical of EDS patients. Either resistant or terribly sensitive – or both! Which makes coming up with a pain management strategy very challenging. Truth is, we might not find a real answer until we address the neurological symptoms in a meaningful way.

Night 2

Last night wasn’t so good – Em was awake until 4 am. Not really surprising, on a number of levels. First, she tends to have this kind of experience with meds frequently – great the first time, then nothing. Strange reactions to meds is pretty typical of EDS. She was totally knocked out with 5 mg of flexeril the first time she took it, then nothing, then 10 mgs did nothing when it would have knocked out a grown man. So, I kind of expected this. Secondly, she is currently on the lowest dose of amitriptyline and we will be gradually bumping her up until we find the right dose.  They said try this dose for 5 nights – so I will call Monday morning and see if they want to bump her up. Most people take 30-40 mgs for this purpose so there is some wiggle room. It takes some doing to find the perfect dose – one that helps her sleep but doesn’t make her groggy all day. Thirdly, her sleep schedule has been horribly messed up for months so it won’t get perfect over night. I still maintain that it is the pain that keeps her awake, but I also know that her body has become accustomed to staying awake at night so there is a behavioral component in play. She is going to have to learn to sleep at the right time and the ami, at the right dose, will help her do that.

She was in a lot of pain yesterday. Not sure if it was the weather (a serious possibility) or that she had to stop taking her tramadol. It didn’t seem to be helping when she was taking it and she didn’t bother taking it during the day anyway, but maybe it helped more than she realized? The headache was worse than normal and she hurt all over. Nothing new there but it was  just worse than usual and it must have been significant because she actually complained about hurting. It was terribly hot here – upper 90’s – and that always makes her worse. Of course, she stayed inside all day – if she had even stepped outside in that heat, I would have had to scrape her off the ground. Her body does not deal with heat well at all.  And we had a late afternoon thunder storm and that always makes her head hurt worse. She is my human barometer at times!

I really cannot wait until we can add the next layer of pain relief. The ami can have some pain relief effect, but only after several weeks and it will likely be a mild effect. She really needs a med like Lyrica and maybe something for breakthrough pain. But, we have to go slowly to make sure whatever she ends up taking will be effective.

I just hate seeing her suffer and am impatient to help her feel better. I want her to have a life again and getting her better before fall so she could actually do some school work in the coming year would be awesome. But, with the cranial instability, will anything but surgery actually help? I just don’t know but I can hope!

EKG and Amitriptyline

We saw the pain doctor on Thursday. He wants to try Em on amitriptyline – to help with her sleep and pain. He wanted her to have an EKG before he prescribed it. So, since her appt with the pain clinic was in the morning we drove to Reid Hospital from Cincinnati so we could get the EKG asap. It was a very long day for Em – she had had her joints bent and twisted during the exam, not to mention getting up early after very little sleep and dealing with the ever present headache. But it had to be done and we figured we may as well do it right away rather than wait.

So, we got the EKG and I called down the next day and gave the nurse the number to call to get the results [note: it is rather inconvenient to deal with 2 separate hospitals and to be the go between!] Called again late Monday afternoon after not hearing anything. Playing a bit of phone tag Tuesday morning, but finally got word that the EKG was normal and she could start the ami.

She got to take it last night for the first time and eliminate the flexeril and tramadol from her list of meds. Started with 10 mgs but may need to titrate up until we find the optimal dose.

Em was not optimistic at all – it made her dizzy, but that is nothing new. She was convinced that she was going to lay awake all night. She kept saying she wasn’t sleepy, so at 12:30 I said we needed to turn off the light. (We need to use this opportunity to adjust her sleep cycle so we may have to bite the bullet here in the beginning.) She laid there and tossed and turned and huffed and puffed, complaining that she was never going to get to sleep. Then by 1:00, she was snoring!

I am not going to get too hopeful because she has typically reacted well the first couple nights to anything to help her sleep, then it quickly stops working. BUT, I am hopeful that we can work with the dosage to make this a real option for her. This is a very standard medication used with EDSers – it is an anti depressant but it is most helpful for helping with sleep issues. It also can have a slight effect of helping with pain levels, especially as it builds up in the system. A side benefit can be a lifting of mood since it is an anti depressant but at such low levels it probably won’t do much in that area. However, any help it gives will be gratefully accepted!

We will try this dose for a few days and see what happens. If it helps, we will continue at 10 mgs. If not, I can call and they will bump up her dose. This is one of those meds that can take up to 4 weeks to really see the full benefit, so we have to be a bit patient. But, she got a decent night of sleep so that is definitely a step in the right direction! I will wake her up in a little while [working towards that better sleep schedule] and see how she feels. She very possibly will be sleepy and groggy through the day until she gets used to it.

So, so far so good and we will just wait and see. I am very excited about the prospect of real pain and sleep relief – this is what hope feels like! The pain relief is ‘only’ a year late but today, I am just grateful that it finally is within reach.