Sunday, January 22, 2012

The way I have fun on a Friday night

Hey all.  The last time I left you I mentioned my little trip to the ER.  I won't keep you in suspense any longer.  I know you haven't been able to sleep or work or eat since you read my last post because you couldn't think about anything else.  I'm so sorry to do that to you.

Now... back to reality... I'm sure all 3 of the people who read my blog are doing just fine.  Most of my family and friends are since most of them heard through the grapevine or facebook (my husband posted that I was in the ER) and they haven't called to see why I was in the ER or how I've been doing since then.  So if they aren't too worried then I'm sure my blog reading friends who've never met me aren't losing any sleep over it.

Last Wednesday I had an EGD to make sure the severe reflux I have from my gastroparesis isn't damaging my esophagus.  The doctor talked to my dad after the procedure and let him know everything looked fine.  I slept most of the rest of the day because of the drugs they used to sedate me for the procedure.  The next morning I woke up with a horrible headache and nausea.  Nothing I did helped either problem.  My headache remained at a 5-8 out of 10 on the pain scale all day and the nausea and vomiting persisted right along with it. I tried 4 different nausea medications, my migraine med, and my pain meds.  None of it helped.  I was not able to keep much down in the way of food and fluids.  I went to bed that night figuring that I would feel better when I woke up the next morning.  Unfortunately, I was wrong. (I know... that doesn't happen very often.  It came as a shock to me as well.)  I woke up with the same excruciating headache.  The nausea and vomiting followed shortly there after.

The problem is this... With my mitochondrial disease, I can sometimes get stuck in a vicious circle.  Many times, the cause of my bad headaches, nausea and vomiting is too much activity and dehydration.  However, when I have a headache that bad along with nausea and vomiting I am unable to drink many fluids.  That leads me to further dehydration.  Becoming more dehydrated makes my headache and nausea worse.  Worsening nausea keeps me from getting any fluids.  Do I need to keep going or do you get where this is going?

The problem, then, is that I need to get re-hydrated enough to push me out of the downward cycle.  The only way to do that is by getting IV fluids.  I also, by that time, needed to make sure that my body was not entering into a catabolic state.  You can read more about catabolism and mitochondrial disease here.  Luckily, I was not to that point.  I was able to receive IV fluids and go home after my labs came back normal.

As I discussed in my previous post, my palliative care nurse and I are working on getting setting up for home IV infusions.  My nurse, primary care doctor, and I need to discuss the best way for me to receive them (i.e. PICC line, peripheral IV, or port).  There are pros and cons to each.  So I will have to weigh all of my options.  I am really hoping that we can speed the process along.  I would like my last visit to the ER to be just that.. my last visit to the ER.

Saturday, January 21, 2012

Planes, trains, and automobiles

Well... I guess there's no trains.   Sorry to you train lovers.  I hope you didn't get too excited.

The last 2 weeks have been a bit eventful.  Last week my mom and I flew to Atlanta to see one of the best mitochondrial disease specialists there are.  Everyone I've talked to has had nothing but good things to say about Dr. Kendall.  I first met her at the UMDF symposium in Chicago.  She gave multiple speeches on different aspects of mito.  If you think you might have mitochondrial disease, or you have been diagnosed with mitochondrial disease but you're still looking for a good doctor to take care of your mito needs, you should definitely call Dr. Fran Kendall.

I was originally diagnosed by another mito/metabolic specialist in Atlanta, Dr. John Shoffner.  I loved Dr. Shoffner when I saw him.  He is very intelligent and definitely knows what he's talking about.  However, trying to get anything out of him post diagnosis was like pulling teeth. I won't go into detail.  I've already used up enough of my spoons on being frustrated with him and his office.  So, you'll just have to take my word for it.

The trip to and from Atlanta was, as expected, very tough on my body.  The last time I flew was last March when my husband and I flew there and back in one day.  That was difficult.  There's no way I could do that now.  We flew down on Tuesday morning, had the doctor's appointment at 1 o'clock, stayed the night, and flew back to KC on Wednesday afternoon.  The flights both times made me pretty ill.  I have never had trouble flying (motion sickness) until my flight home from Atlanta last week.  During take off and landing I became very nauseous and dizzy if I moved my head at all.

I was not happy with the flight home.  The airline screwed up and didn't give us assigned seats close to the front of the plane when I called prior to the trip to let them know I would need assistance and that I have a wheelchair.  So, since it was a full flight, we ended up sitting in the second to last row.  My mom talked me into using the aisle wheelchair (I think they called it a jump seat) to get to my seat.  I was embarrassed.  I'm a 33 year old woman; I'm not paralyzed; but I needed a wheelchair because I couldn't walk the distance of an airplane to get to my seats in the back.  I was being stubborn and declined the aisle wheelchair when we were getting off the plane in KC.  After I walked the distance of the plane and got in my wheelchair right outside the plane, I realized why I needed the aisle wheelchair.  The good thing is, my mom got to hear me say "You were right."  That doesn't happen very often.  ;-)

As you can imagine, it took a few days, but I recovered.  Luckily, Dr. Kendall does follow up appointments via webcam.  So the next time I need to see my mito specialist in Atlanta, I will be able to do it from the comfort of my bed.

As for the details of the appointment... Dr. Kendall discussed genetic testing with us.   I have received the mitochondrial DNA test results from Dr. Shoffner.  The geneticist at KUMed told me that the mutation that was found in my mitochondrial DNA is causing my mitochondrial disease.  That specific mutation usually causes completely different symptoms,but a form of mitochondrial disease none the less.  Dr. Kendall, however, stated that she doesn't think it's likely that that mutation is causing my mitochondrial disease because  of the fact that it normally causes a completely different set of symptoms and disease.  She, therefore, recommended that I get my complete nuclear DNA panel tested.  However, it's completely up to me.  I am not going to get it done at this time.  It won't change anything.  She said there wouldn't be any change to my treatment plan.  It would only give me a definitive genetic diagnosis.  I did, however, ask her, with the information she had in front of her, along with my clinical picture (signs/symptoms), if she agrees with the diagnosis of mitochondrial disease.  Her answer was "Yes".  So, there's my second opinion.

She gave me a prescription for a medication that has helped many of her patients with dysautonomia.  If you're a dysautonomia person, and you want more info on the medication, email me.  Dr. K ordered some lab work that she will do at least once a year in order to keep an eye on all of my body systems (kidneys, liver, heart, etc.).  The geneticist at KUMed said the exact same thing.  He also stated that I need to have an echocardiogram once a year to keep an eye on my heart as well.  Last, but not least, she discussed getting IV fluids.  It's about DAMN time!  I was happy that I didn't have to bring it up myself.  She wants me to start getting a liter of IV fluids once a week to help me catch up on hydration.

With dysautonomia, I need to be taking in 2 to 3 liters of fluids a day... closer to 3.  However, because of my GI problems I usually only drink less than a liter.  As you can imagine, that causes problems.  If I have a day in which my nausea is worse than normal and I drink a lot less, it makes it very difficult for my body to catch up.  Getting IV fluids every week will start to help that.  It will at least make it to wear I don't continue down the vicious cycle of nausea, don't drink as much, dehydration which makes my nausea worse, more nausea, drink less....  well, you get the point.

My palliative care nurse and I started the discussion this week about how they will administer the fluids, PICC line or portacath. I won't bore you with the details of what each of them are.  Try wikipedia, my 2nd favorite website.

Unfortunately, we didn't figure it out and get it put in quickly enough to help me in my "mito crisis" this week that landed me in the ER.

And now, like your favorite tv show, I'm going to leave you in suspense. It's 1am and I'm struggling to keep my eyes open.  Soooooooooooooooooooooooo....

To Be Continued.............

Thursday, January 5, 2012

Catching up - Part II

Now that I've recovered from my little nervous breakdown...

As promised, I am posting again to catch everyone up on everything that's gone on in the last month.  I know.  You were all on the edge of your seat just waiting for part 2 of my original post, "Catching up - Part I".   I'm sure it's been very stressful since I left you in such suspense.  You can all relax now.  I'm here to tell you more, tell you more... like does he have a car.

I have had 2 other doctors appointments in the last couple of weeks.  The first of which was with a colorectal surgeon that the GI doc referred me to.  As I'm sure you could guess, there's more poop talk coming.  So if you're one of those people who gets embarrassed by discussions about poop, this is your cue to stop reading.

As I've already discussed before, my entire GI system is slower a snail going uphill on a salted sidewalk.  In addition to that, whenever I have a bowel movement, I have to utilize the pushing techniques I learned in lamaze class despite having very soft, usually easy to pass, stool.  I was informed by the GI doc that the colorectal surgeon would be able to do tests on the muscles used to have a successful bowel movement.  So, since I don't have enough fun in my life, I agreed to see the colorectal surgeon so he could put things in the orifice which is normally labeled as a one way street.

Sure enough, he explained that he believes that I have "pelvic floor dyssynergia" or "anismus".  Everyone has muscles in their pelvic floor surrounding the colon/rectum that stay contracted.  When having a bowel movement, those muscles contract to allow stool to pass.  However, in anismus, those muscles do not relax.  So, in addition to my entire GI system not wanting to move, the muscles  that help the stool pass do not work either.  BUT (no pun intended)... like I said above, he "believes" that that's my problem.  So, lucky me!  I get to endure more tests that will determine for sure what is going on.  I am SO looking forward to more tests that require cold hard objects to travel the wrong way on a normally one way street on my body.  Three of them, in fact!!!  While all of you suckers have to work all the time, I get to do fun stuff like that.  Ha - Ha!!!  (said like Nelson from the Simpsons)

So, I will have to let you know at a later time what comes of all of the poop shoot tests.  To Be Continued...

In 5 days I will be traveling to Atlanta for an appointment with Dr. Fran Kendall.  She is a renowned mitochondrial disease specialist.  I actually met her briefly at the UMDF symposium last June.   She gave a few talks.  I have also talked to numerous people who are or were patients of hers.  They have said nothing but good things about her.  Whereas Dr. Shoffner, also from Atlanta, was able to diagnose me, he was not turning out to be very good at taking care of things  when I am not in his office.  Dr. Kendall will be able to manage the mito aspect of my care from a distance.  For instance, if I come down with a virus, she will be able to talk to my primary care physician about what needs to be done since my body handles illness a lot differently than a normal person.  In addition, follow up appointment will be held via webcam from the comfort of my house.  I am hoping that this will be my last trip to Atlanta.

We are leaving Tuesday at the butt crack of dawn and returning Wednesday late afternoon.  I have to admit, I am very nervous about the amount of activity required for this trip.  In "Catching up - Part III", I will let you know what has been going on with me and how sick I get when I have too much activity.

Until next time...

Wednesday, January 4, 2012

My Life, or lack there of

WHY???  Why me?  Why did this have to happen?

I don't want this shit called mito anymore.  I'm done with it.  It needs to move on to the next person, or ant or rock or something.  I had a fucking life.  Mito stole it from me.  I had hopes... dreams... plans.  Once again, mito took those away from me.

I had a job.  A job that I loved none-the-less.  I had friends.  I had hobbies.  I had fun.  I was beautiful.  I ran.  I LOVED to run.   I loved completing races.  I was a good mom.  I was a good nurse.  I was proud of myself. I was happy with my life.

I had goals.  I had plans.  I had hopes and dreams.  I was going to do something with my life.  I was going to travel, a lot.  I was going to go back to school for my masters.  I was going to adopt an older child who had been in the foster care system most of their life and needed a family to love them.  I was going to save money to send both of my daughters to college.

I want my life back.  I want A life.  I have my family.  Other than that, I have nothing.  I have no life.  My life is mito.  Mito is my life.  Doctors appointments, medical tests, laying in bed, laying on the couch, pain, nausea, fatigue, weakness, feeling really sick, feeling kinda sick, feeling a little bit sick.  That's my life.  Day in.  Day out.

I'm not happy.  I pretend that I'm happy everyday.  If I start to think about something that gets me down, I very quickly think about something else.  I just don't think about the fact that there is no meaning in my life.  I'm not depressed, but I'm not happy.  Far from it.  However,  I fake it well.

I'm 33 years old and my life is over.

Sunday, January 1, 2012

Catching up - Part I

HELLOOOOOOOOO internet world.  In case you were wondering, yes, I am alive.  I could tell you I've been too busy to post. Or I've been too sick to post.  Or I've been too well to post.  Instead, it's "D" All of the above.

First and foremost, I want to wish everyone a Merry Christmas and a Happy New Year!  I hope everyone had a wonderful holiday and, for my mito and chronic illness friends, you didn't use up too many of your spoons and make yourself sick.

But, now it's been almost a month since I've posted and I have a lot to catch you up on.  As I stated in a previous post, I started palliative care shortly before Thanksgiving.  They have helped me tremendously.  They made some changes with my medications and put me on Scopolamine.  Some of you may be familiar with Scopolamine.  It is a patch that you put behind your ear that is supposed to help with motion sickness.  The last time I used it was when I went on a cruise with my mom after I graduated from nursing school.   However, it isn't just used for motion sickness.  It's also used for nausea due to illness as well and it has worked wonders.  As I stated in my post on Nov. 20th, I was not tolerating much activity at all.  The primary problem I had when I was up and around for even a little while, was that I would be sick for the next 24 hours.  In addition to that, I was nauseous all day everyday.  Not to the point of vomiting, but enough to prevent me from eating or drinking much of anything on a daily basis.

This Scopolamine patch has decreased my nausea ten fold.  Where I was nauseous more often than not prior to the patch, I am now nauseous much less frequently.  I have been able to eat and drink enough each day.  Well, enough to get me by.  I can still tell that I am somewhat dehydrated (by mito standards that is, not healthy person standards).

Palliative care has also helped in other ways.  There is a wonderful lady who is a volunteer with KC palliative care coming about once a week to help with stuff around the house or whatever I need help with.  A social worker has visited a couple of times to help me with a few things.  I have also had in home physical therapy and a dietitian visit.  The PT was just okay.  I only got 3 visits per my insurance.  The first visit was just an evaluation, the second was a therapist teaching me some exercises, and the third was to reevaluate me to see if anything had changed.  Here's a big surprise... I didn't get stronger after only one visit by a therapist!  I know, you're surprised like I was.  However, I now have exercises I can do, primarily with my legs, while I'm laying in bed or in my recliner.  Therefore, I don't have to worry about autonomic dysfunction while doing my exercises.

I had a good holiday.  I felt decent enough to get through it and able to enjoy it for the most part.  My girls loved their presents from Chris and I as well as what Santa brought.  Nothing real eventful there.  Which is a very good thing.  Uneventful is great!

I have seen multiple doctors in the past month since I last posted.  I have seen my new GI doc, who seems MUCH more competent than the other one I was seeing.  I have seen the Colorectal surgeon, based on the recommendation of the incompetent GI doc I saw.  However, this was one thing I agreed with her on.  And, just this last week, I saw my geneticist.

One at a time...  I like my new GI doc.  One of the most important things he said to me during my appointment is, "I don't know much about mitochondrial disease, but I can promise you that I will know a lot more by your next appointment."  That is something I've never heard from a doctor before.  Some of them won't even admit that they don't know much, if anything about mito.  Most of them, however, will say that they don't know much, and that's the end of it.  It's quite obvious that they don't take the time to look it up.  My previous GI docs was one of those who made it quite clear that she didn't take a second of her time to learn more about the disease her patient is dealing with.

So, this new GI doc is going to do some research on mito prior to my next appointment.  For now, he has ordered 3 medications to keep my GI system moving and he will be doing an EGD on me to make sure my extreme reflux has not caused any damage to my esophagus.  That wouldn't surprise me.  About half of the time when I eat, I get a burning feeling in my throat.  I don't mean after I eat because it's causing reflux.  I mean, as the food is going down, it burns.  Unfortunately, the medication he ordered to help my GI system move better, has yet to be approved by my insurance.  So, it's been 3 weeks since my appt., and I'm still waiting for insurance and my doctor to come to an agreement.  My doctor doesn't want to back down.  He wants me to take this form of the medication and my insurance says they won't pay for it.  Soooo, they fight like an old married couple and until they kiss and make up, I am not taking anything for my very slow moving stomach.

As I stated before, I have a LOT to catch you up on.  I'm not going to be able to do it in one post.  Nor would you want me to.  I'm sure you have a lot better things to do than to sit and read my blog for hours.  Therefore, for your sake and mine, I'm going to catch you up in segments.  That what I get for not keeping up with my blog when I have so much going on that I want to share with you all.

Until next time...