5 months Post-op

I had my 2nd total cranio-cervical fusion reconstruction exactly 5 months ago today.  It is a very difficult surgery.  Since I have dysautonomia and suspected mast cell problems, recovery is very prolonged.  It's not fair to judge the success of a surgery based on symptoms at 5 months.  It's better to check at 12 months, maybe even later.  But I'll try to look objectively and see what may be better.

The last month has been challenging.  I was sick with an upper respiratory virus, causing a sinus infection and bronchitis.  For those two weeks, my POTS symptoms were out of control: high tachycardia, lots of shortness of breath, some spells of hypotension, lowered level of consciousness.  Then, I got one of my 4 day migraines.  Luckily, the virus and migraine were gone by August 2, the day we picked up our new puppy!  His name is Charlie.  He is a sweet little Cockapoo.  I'm not allergic to him!  I completely adore this little guy.  Unfortunately, after 4 nights of poor sleep due to the puppy's cries, my whole system shut down.  I couldn't stay conscious, my digestion went crazy, spells of very low blood pressure, tachycardia, shortness of breath, couldn't take care of myself, much less my beloved little Charlie.  So, after 5 days with us, Charlie moved in with my mom.  She and my stepdad are willing to take care of him until he grows up a bit and until I am a bit recovered.  But I really miss him!  I tear up just thinking about him.

The thing is, with all of these complications, my progress has definitely had a setback.  So, I'm rebuilding again...starting with sleeping as much as I can at night, resting as needed during the day, and exercising every single day, as much as I safely can.  My well-being depends on a very delicate balance of rest, exercise, diet, fluids, medications, stress, and most importantly, sleep.  98% of my focus is currently devoted to getting all of these things right, so that I can devote the other 2% to actually living.  Hopefully, this proportion will improve, and I'll be able to participate more in life and work again in time.

The symptoms I'm dealing with the most this week are:
-migraines
-tension headaches
-fatigue
-lowered consciousness
-loss of consciousness/passing out
-tachycardia
-spells of hypotension (not orthostatic)
-shortness of breath
-temperature dysregulation
-exercise intolerance
-body pain
-digestion (usually C, now more D--(friends with IBS will understand)

When things are so hard, it's difficult to see the improvements.  But they are there!  I'll do my best to think of some, as it is important to see the big picture in these difficult times.
-As of today, I do not need to wear the collar at all times.  Admittedly, I've been weaning for a few weeks.  I'll still be wearing the rigid collar in the car and a soft collar as needed for comfort.
-Excluding the muscle tension/tension headaches, I feel capable of going without the collar for reasonable periods of time.  This is a good sign that I have a solid fusion.
-My swallowing has actually gotten better.  I still have good days and bad days with swallowing, but I'm not getting extremely bloated nearly as often!
-I can sit up for longer periods of time.  For less pain, I generally like to sit on the couch so I can rest my head.  But it's still upright, which is better than before surgery!!!
-Before my setback, I think my overall level of functioning was improved from pre-surgery.  I just have to rebuild to get back to that.  Before the setback, I was able to: get dressed most days, leave the house for short trips most days, socialize for about an hour at a time, and do a fair amount of walking (I had reached 1.25 miles.)

My POTS is not better yet.  I'm not to the point where I was before surgery yet.  That will probably take the rest of this year, or more.  This means that I use the wheelchair at times, and I can't stand for long periods of time.  My aerobic endurance is still very low, but I'm working on it every day.

My headaches are not really better.  I either have a tension headache or a migraine (or both!) every day. Either one can be debilitating, although the migraine is most always debilitating.

I am very lucky to have a supportive family.  I also feel very grateful today for a wonderful primary care doctor back in Illinois, who is helping me get what I need through this transition to living in a new area.

Thanks for checking in with me!  Little by little, I'm coming back.  Sorry this post was a bit scattered.  I'm feeling a bit scattered today.  The migraine doesn't help either.

I heard a really great quote yesterday, so I'll post it here:
"Life is mostly froth and bubble; two things stand like stone: Kindness in another's trouble, courage in your own."
--Adam Lindsay Gordon

*I almost forgot: photos of Charlie!  Charlie is my reason to get better.  My motivation to exercise every day.  I want to be healthy enough to bring him home!

Sick and complaining

First, our new insurance plan started on July 1, but we haven't received the new cards yet, so I can't get any prescriptions or see any doctors right now.

So, on Thursday, I woke up with pink eye in my right eye.  Luckily, I had leftover and not too expired drops from my last case of pink eye, so I started using those, and sanitized everything I could find in the house.

Then, Friday, I woke up with a really sore throat...which is always the first sign of me getting sick.  I came down with a cold, but it wasn't too bad yet.

Friday night, I couldn't sleep too well, due to the sore throat.  Lots of salt-water gargling, lozenges, and Tylenol to get me through the night.

By Sunday, after more sleepless nights, I had a deep chest cough and mostly lost my voice.

Last night, I couldn't sleep again, from the coughing and the painful throat.

I slept a lot today, just trying to recuperate.  Feeling the same, so far.

Of course, since my body is busy fighting off this bug, everything else is worse.  My POTS symptoms are flaring, as well as potential mast cell symptoms. 

Then, of course, I have a sinus headache.  This is, of course, in addition to my regular tension headache.  And, just for fun, I've been fighting off a migraine for 8 days.  Plus, my massage therapist had to cancel, so I've gone a few weeks without a massage, making the headaches all worse.

Now, my hubby contracted my pink eye.  This is all overwhelming.  So sick.  Hoping for some relief soon.  And hoping that my hubby doesn't get my bronchitis/laryngitis thing.  It's hard living out in the middle of nowhere, far from family.  Especially when things are hard like this.

Hopefully, I'll be recovered soon, cause we're getting our new puppy in less than two weeks!

But, every illness is a setback, so I will try not to expect too much too soon from my delicate body.  This was a pretty big hit, so there will be some rebuilding to do.

Avoidance Gets You Nowhere

I have so much to write about.  I keep thinking of new things I need to blog about, but I never get it done.

So, I guess I'll start with a subject I've been trying to avoid for at least a year now.  It's only now that it seems I can't completely avoid it anymore.  I may have another condition.  No, I'm not diagnosis-hunting.  It's just that there is another condition that is now being linked to the whole EDS/POTS/Chiari/CCI group.  And I absolutely would not care for a second unless I were actually having symptoms right now, which unfortunately, I am.  I also would not care unless there was a chance something could be done about this that would improve my level of functioning and quality of life.

That being said, the name of the condition is "Mast Cell Activation Disorder" or MCAD.  I will add some informational links here soon.  In the meantime, if you are looking for information, the disease functions very much like Mastocytosis.  There is not much information on MCAD (also called MCAS for "Syndrome".)  But it is still being treated.  The current understanding is that MCAD is caused by over-reactivity of the mast cells to many benign triggers.  If you've known me for a while, you know that I've had quite a few odd and even dangerous reactions to medications over the years.  You may also know that my throat closes up whenever I'm around cigarette smoke.  These could potentially be mast cell reactions.  You know how I have had a variety of food intolerances over the years?  These could potentially be mast cell reactions.

A seemingly insignificant part of my medical history is my allergies.  As a child, I tested positive for every major allergen.  During college, my allergy symptoms worsened, so I was retested, but found to have no true allergies.  This would be an important part of my mast cell history.

If it were just these things, I still probably wouldn't look into this diagnosis, because who needs another diagnosis?  But, mast cell reactions can also include: persistent fatigue, brain fog, headaches, tachycardia, sudden drops in blood pressure, fainting, cognitive impairment, vision problems, nausea, problems with anesthesia, decreased bone density, shortness of breath, temperature sensitivity, gastrointestinal pain, and bloating, If these symptoms are being caused by inappropriate degranulation of mast cells, treating these overactive mast cells could be an important part of my treatment.

There are a few easy treatments that doctors often try as a way to see if MCAD should be considered.  If you respond positively to these treatments, you probably have some form of mast cell disorder.

The first treatment is with OTC Histamine blockers.  H1 blockers include typical allergy medications, like Zyrtec, Claritin, and Allegra.  H2 blockers also block histamine, but in the stomach.  These include typical antacid medications, like Zantac and Pepcid.  So, to treat MCAD, you start with daily use of H1 and H2 blockers.

The thing is, I've been taking these for a while (since I've had an inkling that this might be part of my medical puzzle), and I was still seeing an increase in symptoms.

So, I often have to take Benadryl, an anti-histamine, to resolve a reaction I am having to a known or unknown trigger.  Since this is happening more often, I decided that I need to start working on figuring out my specific triggers, and avoiding them when possible.

Every mast cell patient has different triggers, so it's important to try to find your individual triggers.  Sometimes, it's obvious what has triggered me, like medication reactions.  So, I need to avoid medications that have caused reactions.  I have known reactions to medications like Morphine, Compazine, and a few others.

A huge mast cell trigger is fragrance.  I have known of my sensitivity to strong fragrances, like air fresheners, perfumes, and hair sprays, for a long time.  But I need to take these more seriously now, and avoid fragrances whenever I can.  So, we're trying to switch over to fragrance free products.

An easy trigger for me to avoid is alcohol, since I've always known I've reacted negatively to alcohol, so I never drink.

The trickier triggers to figure out are food triggers.  The most common triggers seem to be foods that are high in histamine, high in salicylates, or high in tyramine.  This includes a lot of foods.  I only need to identify which ones are my personal triggers, not actually eliminate every possible food.  It's still a bit tricky.  A strict elimination diet would be the most effective way to identify my triggers.  I already eat a very regular and simple diet, so I should be able to figure out some of my triggers.  One obvious group of foods is fermented foods.  So, I have to give up my Greek yogurt.  A couple other very high histamine foods are tomatoes, strawberries, and shellfish.  I can avoid those pretty easily.  The rest of it will be trial and error, which I'm working on now.  Most trigger foods cause me to get an itchy nose and throat, so I am keeping a food diary and taking note of symptoms.  I recently had a pretty dangerous reaction to a protein bar, with my throat getting all itchy and swollen, my airway constricting.  Had to take Benadryl for that one.

Hopefully, this will be all it takes to reduce the frequency of mast cell degranulation, which actually increases your ability to tolerate small exposures.

If these measures don't work, there are plenty of other things to try.  I'm am using NasalCrom, an OTC mast cell stabilizing nose spray, and Ketotifen opthalmic, an OTC mast cell stabilizing eye drop.  If I need further help, I will have to see one of the few doctors willing to treat this disorder.  Other potential treatments include: Singulair, GastroCrom, and Doxepin.  A commonly used medication that is most effective at stabilizing mast cells in the whole body is Ketotifen oral, but it is not yet available in the US.

Okay, I know that's a ton of information.  I've got a terrible headache.  So, that's all for today.  I know this is a ton of information.  This is what I'm working on right now.  I'll let you know how it works for me.

3 Months Post-op

I know: I'm late.  Surgery was March 14, so it's a little more than three months post-op.  This past week, my husband and I moved to a new apartment.  It's a little smaller, so we've been sorting through everything and donating what we don't need.  It already feels pretty homey here, but it is hard being even further from our families.

So, I've missed a lot of updates along the way.  The biggest was our trip to Toledo to see Dr. Grubb's APNP, Beverly Karabin (specialist in dysautonomia).  It was a 6 hour drive each way, but it was worth it.  She validated my post-surgical experiences being most likely related to my dysautonomia.  The surgery, the medications, the bed-rest, the lack of sleep, and the lack of medication management in the hospital all contributed to a horrible crash of my autonomic nervous system.  She said to expect recovery to take nine to twelve months.  Until then, I need to be patient with my body, recognize that it is healing very slowly, and gradually increase activity as I am able.  To help with that, we are experimenting with some new meds.  I'm taking a very low dose of a beta blocker (Propranolol 5 mg) at bedtime.  This is to reduce the heart rate spikes that happen in my sleep, which should improve the restorative quality of sleep.  I think I am sleeping better on it, but I can only handle a very low dose before it starts affecting my blood pressure.  The other change is that I am taking Nuvigil to help with my daytime alertness.  It actually really does help!

I've also been able to discontinue Midodrine and Florinef.  Which means that my POTS is getting more under control.

I'm still having serious problems with muscle spasm in my neck and right shoulder.  Daily trigger point massage is the only thing keeping the tension-induced migraines away.  Unfortunately, we moved away from my physical therapist that was doing dry needling therapy on my trigger points, so we're managing on our own for now (meaning my husband is providing daily massages on all of the rocks in my neck).

I definitely have ups and downs during every day.  If I exert myself, I know I will have to spend some time resting, likely with reduced consciousness.  So, I just have to plan my life around that.  I am trying really hard to keep exercising, which is a challenge since I don't have a gym anymore.  But I do have my recumbent bike, which is the very best cardio exercise for me.  I'm also doing lunges and squats to build up my leg muscles when possible (although this past week, the work of moving has been exercise enough!).  So, every day still has its own challenges.

But, the improvements really are apparent at times.  On Tuesday, the weather was perfect, so Gustavo and I went to explore downtown Madison.  We took my wheelchair, but I didn't have to sit in it the whole time.  We went from the capitol to campus and back.  And we stopped for dinner along the way.  Then, we bought some groceries.  When we got home, I fully expected to crash, but I was actually able to do a little more organizing around the apartment that evening!

So, my endurance really does seem to be improving.  I still have migraine days.  I have times where I'm barely conscious.  But I have plenty of time to keep recovering (although financially, the sooner I can start working, the better).

EDIT: I forgot, I also got my butt kicked by an infection a couple weeks ago.  For a few days, I just couldn't wake up, my bp was really low and non-responsive to meds.  I could barely stand without blacking out.  Thank goodness, we figured out what was wrong (UTI) and it resolved pretty quickly with antibiotics.  Thank goodness we had COBRA insurance this month.  I really needed help to get through it.

Finally, we have put down a deposit on a new puppy, which we're expecting to get by the end of the summer.  I'm so excited!  We're getting a cockapoo.  Training and exercising a new puppy will be a good next step in my recovery, to get me moving again.  After a couple months of that, I hope to be well enough to take on some new piano students.  But I know I need to be patient with my body.  Little by little, I expect to continue to improve.  Long term prognosis is still unknown.  So, I don't know how well I will get.  I don't think I'll ever be very functional in the mornings.  I don't think I'll ever work a full time job.  But I am still getting everything I can out of life.  I am so grateful for these new moments of feeling more alert, more alive, and more capable.  I cherish the good times, and cope with the bad.  My family has been so supportive of me through yet another surgery.  I am so lucky to have them in my life.

Thanks for reading today.  Sorry for the lack of updates.

Great article on living well with chronic illness

http://www.psychologytoday.com/blog/turning-straw-gold/201305/12-tips-12-years-sick

What is Ehlers-Danlos Syndrome

From the Ehlers Danlos National Foundation
Written by Mark C. Martino

More than 1.5 million people around the world have Ehlers-Danlos syndrome (EDS). Someone with EDS is born at least every half hour, and many won’t know what’s wrong with them until it's too late for them to be helped. EDS occurs in between one in 2,500 and one in 5,000 men and women, of every race and ethnicity. EDS was named less than 100 years ago, and it remains largely unknown. Recent evidence indicates it may be one of the most under-diagnosed disorders, because there is no cure and many doctors consider EDS too rare for anyone to have. They fail to see that diagnosis is important, not just in accurately assessing how many have EDS, but in getting help to their patients for the many problems known to be caused by EDS.

Ehlers-Danlos syndrome is a group of disorders. Each type of EDS is defined as a distinct genetic problem in making or using one of the types of collagen. Collagen is used by the body to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched, but not past its limit, and then is safely returned to normal. WIth badly built or processed collagen, some of the soft tissue can be pulled further than is safe, causing damage. Collagen is the body's most abundant protein and can be found almost anywhere, in skin, muscles, tendons and ligaments, blood vessels, organs, gums, eyes, and so on.

Vascular Type EDS is particularly destructive because of potential arterial or organ rupture; life expectancy is shortened with a majority of individuals living only into their forties. Arterial or intestinal rupture commonly presents as acute abdominal or flank pain that can be diffuse or localized. Spontaneous arterial rupture is most likely to occur in a person’s twenties or thirties, but can occur at any point in life. Cerebral arterial rupture may present with altered mental status and be mistaken for drug overdose. Mid-size arteries are commonly involved. Arterial, intestinal, or uterine fragility or rupture usually arise in EDS Vascular type, but should be investigated for any EDS type.

There is a very wide range of symptoms because of how widespread in the body collagen is, and it is still unclear why one collagen defect can be expressed so differently in people, even in the same family. EDS can cause systemic weakness and instability throughout the body, resulting in many and unexpected effects. Some can be seen, but not all; EDS is largely invisible. Pain, for instance, can be widespread—collagen makes up the fascia, the tissue sheet that holds everything together and wraps around your entire body—but the pain will not show up in MRIs or X-rays, and will be probably be out-of-proportion to any findings. While EDS is not curable, many symptoms can be prevented or treated as they arise: joint hypermobility (loose or unstable joints that slip or dislocate); early osteoarthritis; tissue fragility, skin that tears and bruises easily, blood vessels and internal organs such as intestines that can rupture easily; eye problems and gum disease; spine problems that may also involve the cerebellum and cerebrospinal fluid pressure.

Collagen is found throughout the body. Ehlers-Danlos syndromes come down to a structural problem. An analogy. If you build a house with bad materials, say weak wood or cheap nails, you know you'll have problems. Some problems are more likely to show up than others, but because the bad materials were used everywhere and aren't necessarily visible, you can be surprised by a problem. So the problems resulting from being built out of a protein that doesn't behave the way it should can be widespread, in a wide range of severities, and maybe even show up in places you wouldn't think are related until it occurs to you that collagen is used there, too.

Each doctor you see is going to look at things differently. Endocrinologists see hormones as the problem, or CFS. Rheumatologists in the US think fibromyalgia's the problem. Surgeons see weaknesses that need surgery. And if none of them are remembering that EDS is the root cause, and maybe their solution won't work in that environment of your body, they may be inappropriately treating you. There are some medical specialists, and more with each generation of doctors, but the best suggestion for the longterm health of someone with EDS is to learn everything you can about EDS and what/how it affects you, so you can educate those doctors and nurses who are willing to admit they don't know everything and are willing to learn.

More than a quarter century of experience, free information and resources from Ehlers-Danlos National Foundation can be found at www.ednf.org or reaching the foundation on Facebook. "So You Think You May Have EDS?" is an introduction to EDS written by EDNF members at http://bit.ly/Olw4L, where you can also discover many links to professional and patient publications and web pages through the "EDS Guides" and "EDS Knowledge" menus on the left side, throughout the web site. EDNF holds a national conference, usually each year.

Recovery and Setbacks

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!! 

Every recovery has its setbacks, and this one is unfortunately no different.  After a few weeks of relative peace, with my condition gradually improving, this week has been a setback.

I've had a change in my POTS symptoms.  My blood pressure is no longer low.  I don't need Florinef or Midodrine to keep it in the normal range.  This is great news!  Unfortunately, I'm not feeling any better, because I've developed constant tachycardia.  Not just orthostatic tachycardia.  Constant tachycardia.  Perhaps the related autonomic condition called Inappropriate Sinus Tachycardia.  This makes me feel very short of breath all the time.  It really feels like I'm running, even when I'm laying down or sitting.

I don't want this condition to prevent me from working hard on my recovery.  I still need to exercise on the recumbent bike twice a day.  Unfortunately, I'm not able to walk around much right now, because my heart gets out of control.

So, we've booked an appointment with Dr. Grubb's PA, Beverly.  We'll be driving out to Toledo for an appointment on May 20th.  I've just got to survive until then.  This is so freaking exhausting.  It's one thing after the next with this recovery, as usual.