...amongst the frustration

My hands are committing treason today and doing their own thing, but it has been so long since I have been able to share.  I placed my laptop on a table at a local coffee shop this morning.  I ordered a scone and large coffee, looking forward to a relaxing start to my day.  Within seconds, I tipped my cup with a rogue finger, but luckily, it just wobbled and corrected itself.  I took a deep breath and opened my scone.  After a few bites, I looked on the floor and realized that it looked like a child had been eating in my spot, then realized the mess was all my own. 

I began this blog two and a half years ago as a method to keep my friends and family updated.  It soon became a tool to express my feelings about my disease and current methods of treatment.  One blessing, I had hoped for and was given, was that I would reach other patients or family members with Hashimoto's Encephalopathy.  I recently received a comment on my last blog from a daughter of a HE patient.  My heart aches for the pain she experienced, but I also rejoice in and am comforted by her sweet words.  After all, there aren't swarms of people wearing support t-shirts or wearing ribbons for HE.  Lisa shared her father's experiences with various treatments.  That is why I do this. 

As many of you know, I first experienced mild symptoms (headaches, anxiety, difficulty with speech, hand tremors, and thyroid dysfunction).  I was hospitalized after starting to experience what was initially referred to as myoclonic jerks.  High dose steroids stopped the movements.  I had mild stroke-like symptoms for a few weeks, and then my only symptoms were from medications.  Complications from the steroids sent us to the Mayo Clinic searching for other possible medications.  After a relapse of myoclonus, I began Cell-Cept.  This allowed me to taper off the steroids, stopped symptoms, and within a year, I was able to discontinue the insulin from the steroid induced diabetes.  Nearly two years later, I began to experience tremors, poor motor skills, memory problems, and headaches despite the addition of Clonozapam.  My doctors and I agreed that the benefits from the Cell-Cept no longer out weighed the risk factors.

My immune system was fully suppressed for nearly 3 years.  Now that it is awake, it has started to attack the lining of my stomach and intestines again.  The tremors and muscle spasms now include my heart, creating arrhythmia's.  I  had originally been told that a pacemaker would be in my future, but I was told at my last visit that this may not be an option.  My Cardiologist is concerned about the complications my auto-immune disease would create.  He prescribed a new medication for the rapid heart beat I experience occasionally, but I have not been able to take this due to periods of time when my heart is slowing down too much. 

My Neurologist increased my medication to slow my tremors and aid with pain they are treating as fibromyalgia, but is common with HE.  However, I have been having trouble with it interfering with my work and need to drive.

I have begun to have dizzy spells after standing for an extended period of time.  This is most likely related to the low heart rate, but it could also be a result of my disease, blood sugars, or my medication. 

On a positive note, I was picked up by my husband's insurance during open enrollment.  This will mean we will be paying more than last year, but the benefits will be much better.  It is also less than what we would be paying if I had stayed with my own private insurance which was going to increase this month. 

Other than the dizzy spells, chronic fatigue, muscle and joint pain, arrhythmia's, and roller coastering blood sugars, all is well on this end!  I am taking my kids camping this weekend and I turn 34 in two weeks.  The holidays are approaching and I still feel blessed amongst my frustration.  

He prepared us for this...

Two weeks ago, I was in full mom mode.  I was taking my fifth and seventh graders to school, making sure home work was done, running Logan to Baseball practices and games, hauling Bradie to tennis practice, while figuring out how to fix dinner and keep up with household chores in the process.  This is not much different from what many American mothers are doing these days.  However, having a progressive neurological disease made this a little more challenging. 

We have been reducing the immune suppressants that were slowing the progression of the disease, but since I have had recent relapses and increased symptoms while on them, we decided that the complications and risk factors from the medicine no longer out-weighed the benefits.  I had begun to feel pressure building in my head.  After a couple of days, I began to feel my insides spasming.  Then the tremors started in my hands.  Random muscle twitching consumed my whole body.  Luckily, I had an appointment with my Immunologist scheduled. 

He introduced me to a new Physician's Assistant that had joined his practice.  He reviewed my case with her while I sat feeling a bit like a dancing chicken at the circus...and probably resembling one as well.  Lacking grace, I climbed upon the exam table.  I tried with all my might to not fall.  My balance has been hampered for several months.  We discussed my current symptoms and a previous relapse I had after my last appointment.  That relapse was the most intense one I had experienced since coming off of the steroid treatment.  It left me with stroke-like symptoms on the left side of my body.  I wasn't paralyzed, but had severe weakness.  Like trying to walk through water, I was capable, but all movements required concentration and more effort.  Sitting on the exam table, I was asked to perform a few tests similar to a field sobriety test.  I failed...miserably. 

My doctor and the PA agreed that I needed a new medication to calm this episode, but their first choices in medication would have been affected by my heart medication.  The PA suggested a drug she had used with patients who have chronic pain.  It would calm the nerves and slow the spasms and convulsions.  I was sent home for the day as they did not know if I would be capable of working or driving.  Remember the long list of responsibilities I mentioned before?  They also include working a part-time job.  I picked up my prescription and climbed into bed.

An hour into my first dose, I felt intense pain in my arms and legs.  Shortly after, I felt like I was boiling in my skin.  "These are just side-effects that will go away!" I assured myself.  Nearly four hours after taking the drug, my phone rang.  It was a nurse from my doctor's office.  "Whatever you do, do not take the prescribed medication!" I was ordered.  Uhhhh...too late!  I informed the panicked nurse that I had already taken a dose.  He then explained that my neurologist had been consulted and the new medication would increase seizure activity and cause a stroke in patients like me with a seizure disorder.  "Fantastic!" I said, not even trying to hide my sarcasm.  I was then instructed to increase my Gabapentin to 5 times my current dose.  "Hmmm...two makes me stoned," I stated.  "Well, this is what the neurologist would like you to try," he said. 

So, I have been taking a magic carpet ride for the last week.  I have not made it to the recommended dose yet, but I am getting there, and my body is slowly adjusting.  The doctors have done all they know, are grasping at things to try, but it is ultimately in God's hands.  This incident could have made me mad or scare me, but I am at a different place with my disease.  You see, I was prepared in a way for this time in my life.  Nine years ago, I layed in a hospital bed holding my infant son.  He had been diagnosed with failure to thrive.  He had stopped eating, drinking, and growing.  He had lost too much weight and a feeding tube was the only thing sustaining him.  We laid in a hospital while every available specialist tried but failed to figure out why.  It was obvious that we were losing him, and becoming clearer that we were running out of time.  His hair had fallen out, his skin was translucent, and the feeding tube had given him an infection in his lungs and sinuses.  We felt helpless and all we knew to do was to lift him  up to God and beg for protection.  I knew we had not lived lives that deserved his grace, but prayed for mercy.  Strangers entered our quiet hospital room in the darkest hour while we prepared to say goodbye to our baby.  They sang Amazing Grace, and prayed over him.  Thirty minutes later, my baby ripped out his feeding tube and screamed bloody murder.  The tool keeping him alive was gone.  I lost my mind for a moment, then noticed his screams had turned into a hunger cry.  He drank two bottles and ate three jars of baby food.  It all stayed down and he was released 12 hours later without a doctor's explanations. 

God had us then, and he has us now.        

Time reveals all

My last post was some what melancholy, but we now know that I was in the middle of an "episode."   I had begun to taper off my CellCept, but it had only been a week.  There is not any reason to think that this episode was triggered by anything other than stress and fatigue.  We began tapering off of the CellCept because we did not have any recent evidence that it was slowing the progression or that it was helping more than it was hurting.  For those who are walking a similar journey, please know that I fully support the use of CellCept.  Despite all of its downsides, it was my superhero that gave me my life back...spandex and all!  It allowed me to come off steroids and stay off of them. 

Normally, I am able to stop an episode by hydrating and going to bed.  I just wake up with what feels like a hangover...or like my brain was put in a Magic Bullet!  I woke up after this last episode with left side weakness...the typical stroke-like symptoms that are seen with people who first present with HE and are not being treated.  I have been on thyroid replacement for years, my immune system was still fully suppressed, and I was taking Clonazepam to help with the convulsions, and Gabapentin to calm the nerves.  So, we can say that this episode is just the natural progression.

Since then, I have recovered quite well.  I am down to a 1/4 of the CellCept without any major complications.  I have had a little more energy.  I still fight headaches and now have mini episodes when I am tired.  I just have twitching and small convulsions that do not last very long.  I feel blessed.  We have had a full summer of spending time with family, celebrating both of my children's birthdays, and time with friends (though not as much as I would like). 

My daughter went to an amazing tennis camp and a mission trip.  She has grown physically and mentally.  My son enjoyed having his parents to himself while she was gone!  I could not ask for more at this time in my life...well, maybe fewer medical bills...but then I am good!  hahaha        

With trembling hands

It is a little after midnight and I am waiting for my insomia meds to go into effect, despite doubling the dose.  I am fighting a migraine and I am struggling through tremors and spasms, because my anti-convulsants are not doing enough. 

Most of you know that a couple of days ago, I met with another doctor to get a second opinion on whether it was time to call it quits.  My miracle drug that allowed me to come off the steroids, kick the insulin habit, and allow my bones to strengthen, is no longer providing me with enough help.  I am taking the most that can be prescribed for someone my size, but the symptoms are continuing to get stronger and more medications are needed to keep them under control.  My miracle drug is very toxic, especially at high doses, so it was determined that the risks no longer out weigh the benefits.  When I began the CellCept, my immunologist said the only other option of suppressing my immune system, if the CellCept did not work, would be IV Chemotherapy.  But immune suppresion is no longer a goal.  I am too symtomatic to put my body through the risk and stress.

I just started back to work a month ago to cover costs for my medical insurance and bills.  My doctors told me that coming off the CellCept while working will be extremely difficult, but I do not have a choice.  While I qualify for disability, as a married person, I would have a two year hold to get medicare coverage.  I cannot work and get disability, but I cannot afford my private insurance without working. 

Anyway, I have to admit that I am a little frustrated that there doesn't seem to be anything else to try to fight my disease.  I am scared about how fast it will progress without the CellCept.  And I am sad that I feel so alone.  I have amazing friends and family who have loved me and supported me along the way.  But there are so few people with my disease, and of those, so few are progressive.  I am alone in the fact that so few of us are walking around with an advanced case because so many have had strokes, gone into comas, or died. 

I am sorry that I am not strong enough tonight to assure everyone that I will be fine or to crack the usual jokes.  I am just too tired.  I thank all of you who have been praying and keeping me in your thoughts.  Call me, text me, email me...and lets do lunch or something.  That will bring me joy!      

new adventure

Two years ago this month, I was diagnosed with Hashimoto's Encephalopathy, however, May was the 3 year anniversary from when I first noticed that I was sick.  Those who were along for the ride two years ago know that I was admitted to the hospital with convulsions, trouble with my speech, memory problems and some mild paralysis.  The fact that I was already on thyroid medication probably is why I did not go into a coma like many advanced patients do.  We also began high dose steroids by IV within hours.  I was blessed to have received the care that I did when I did.  After a few months though, we knew that the steroids were making me very sick, but my every time we tried to come off of them, I would relapse and my condition would become worse.  I would develop more symptoms. 

After a trip to the Mayo clinic and another relapse, I began seeing a Rheumatologist/Immunologist who started me on Cell-Cept.  This drug that is used as anti-rejection medication has similar immune suppressing powers as chemotherapy.  I was placed on the highest dose possible and told that if I made it two years, than we might start discussing whether coming off this medication would be possible.  After another hospitalization and being told that my case is progressive, no one knew if we would see the two year mark, and if I did, whether we would be able to even consider removing the Cell-Cept.

If you read the last blog I posted, then you know that I will be looking at a pacemaker in the near future and another surgery around my birthday to remove abnormal cells caused by the Cell-Cept.  While my health situation is not getting better, I was able to return to work after being "retired" for a year.  I met with my Rheumatologist today and I was completely shocked by the visit.  We discussed all of the health problems that have shown up since I was there last.  We agreed to meet again in September.  He asked me, "Do you know what September will mean for you?"  I knew that September was the month I first saw him.  He said, "In September, you will have been on Cell-Cept for two years."  I had not realized that.  "Do you think the medicine is still helping you significantly?" he asked.  I fumbled for words.  "Well, I know it is the only way I was able to come off of the steroids," I answered.  "Yes, but do you think that it is continuing to help you?" he asked.  I have had relapses while on the medication without significant side effects, but we are pretty sure that it is not slowing the progression of my illness.  So...drum roll please...we are going to try and taper down the Cell-Cept to just a fourth of what I am taking now!  This will come with risks.  If I relapse on a lower dose, I will run the risk of speeding the progression or being left with lasting side effects, but if it goes well, then I should have much more energy, have lower risk of infection, lower risk for cancer, and over all feel better!

 I have been determined that if my illness defined me, it would be because of my strength and the new ability to appreciate what and who I have in my life.  The doctor told me how impressed he is with how I have handled all that has been thrown at me.  I assured him that I do not have a choice.  No one asked me if I wanted to be sick.  I also have two amazing children that need me to be strong.  "Oh Kim, that is not true," he said.  "I have patients with far less debilitating illnesses that crawl in a fetal position and feel sorry for themselves," he explained.  I told him that I have had my fair share of pity parties, but two years has made a great deal of difference.  He laughed.  As he walked out the door, he smiled and said, "I have had two shoulder surgeries this year and I am not where I want to be, but I look at you and strive to be as strong."  Luckily he had closed the door before I started to cry.  Wow...we have come a long way friends!
  

Take up your cross daily!

 Most of you know that when I was diagnosed with Hashimoto's Encephalopathy, two years ago, I thought the time of suffering without any answers had come to an end.  However, being diagnosed with a rare auto-immune neurological disease meant that there were a few opinions about my illness, but very little knowledge.  The Mayo Clinic was clear about the fact that there would not be a cure, but that there were a few treatments available, and my body would decide if any of them were going to be right.  Two years, 10 specialists, and 22 medications later, we know that my condition is progressive, and is effecting almost every organ in my body.  A month ago, my kidneys were added to the list.  I have outlived the studies done on progressive cases, so I am treading in uncharted waters...and by the way, I don't swim!  I do doggy paddle and float on a sun raft with grace.

My point is that in the beginning, I was brave.  Truth is that it was probably a combination of being stubborn and in denial.  If it were a burning building, I probably would not choose to run inside.  I did have peace though.  I had faith that I would be saved.  We tried many different medications and they would give hope and short term relief, but none of them were magical.  I began to accept that the doctors were not going to save me.  I remember attending a funeral for one of the most amazing women I have ever met.  She had battled cancer multiple times while raising her two children, teaching at a school, and playing the piano and organ at church on Sundays.  Cynthia's children sat on the front pew, while people stood...one by one, to share how she had touched their lives by sharing her heart or showing compassion.  I was a new mom then and instantly knew that when my time came, I wanted to have lived a life that would make people want to stand and tell my children how I had made a difference.  But in reality, nearly a year into my illness, an anger grew. 

I was angry that I might not see my children grow and get married.  I was angry that my life had already seen so much pain and grief, and I wasn't getting the break I thought I deserved.  I was angry that people in my life continued with theirs.  I was angry that some who stood by me in the beginning grew weary and disappeared.  I wasn't living the life of a woman giving a testimony of how great my God is.    We are never promised that following God will lead to an easy or fair life.  In fact, Jesus says in Luke 9:23  "If people want to follow me, they must give up the things they want.  They must be willing to give up their lives and pick up their cross daily."   Over the last two weeks, I have learned that my heart isn't working correctly and that I will need a pacemaker and medication.  Last Tuesday, I received news that my six month cancer screening showed regrowth.  But I am not going to live my life in fear.  We have decided to stick solely with medication for my heart for as long as I can.  And since the re-growth of cells is due to the medication I am taking, and will take for the rest of my life, we have decided to wait six months and re-evaluate my status, rather than having another surgery.  I also returned to work part time on Tuesday after being home for the last year per my doctor's instructions.  Life is not a piece of cake, but I will pick up my cross daily and take one step at a time, trying to be an example the best way I know how.

 I have made plenty of mistakes and made wrong choices, but ultimately learned that my illness gave me a gift.  Several in fact.  This may sound weird, but my illness has been much like many friends I have had in the past. Sure, it has caused me pain.  It seems to come and go from my life in ways and at times that are hard.   But it has also caused me to grow in my faith.  It has brought people into my life that give unending love and support.  It has introduced me to people across the world that are walking the same path.  It has given me the opportunity to grow my patience. It has forced me to realize that God is the only true constant in my life.  And it has given me the chance to teach my children how to face adversity knowing that the Lord is on our side.  If you can imagine this disease as a bazaar relationship with me for a minute, then you can see how my favorite song from the musical Wicked says it best:
                     I've heard it said, that people come into our lives for a reason
                     bringing something we must learn.
                     And we are led, to those who help us most to grow
                     if we let them, and we help them in return.
                     Well, I don't know if I believe that's true,
                     but I know I am who I am today because I knew you.

                    It well may be, that we will never meet again, in this life time
                    so let me say before we part
                    So much of me is made of what I learned from you
                    You'll be with me, like a hand print on my heart.
                    And now whatever way our stories end
                    I know you have re-written mine by being my friend.

                    Like a comet pulled from orbit as it passes the sun,
                    like a stream that meets a boulder halfway through the woods,
                    who can say if I've been changed for the better,
                    but because I knew you, I have been changed for good!                        

heart broken...

I expect a crazy week at our house, but after all...it is May!  Logan has 3 baseball games in 5 days and we are throwing karate into the mix.  John, my husband, will be in Houston all week for a certification program on solar energy, (yes, he is a nerd!)  And I have two important doctors' appointments to attend.

This morning, I met with my cardiologist who gave me some results from tests that I had run last week.  They showed that I have supraventricular arrhythmias occuring.  In very basic terminology, my heart has an electrical problem in the upper chambers of my heart.  Sometimes, it beats too fast.  Sometimes, it beats too slow, and the timing between beats is often irregular.  As I have Hashimoto's Encephalopathy, an auto-immune disease where antibodies made by my immune system are attacking neurons in the brain, creating erratic neurological impulses...it is fair to believe the two are related, but no one will commit to that just yet.  No matter what, we will treat it the same way regardless.  The blessing is that we are going to try to treat this.  I feared that they would say my disease created too many obstacles to treat aggressively.

It appears that we will treat this with a combination of a high-tech pacemaker and medications.  The fact that my heart changes speeds so often, they expect that the battery will need to be replaced every six years, rather than the 10 that many people are able to wait.  The fact that I am on immune suppressants has perks and complications.  This medication is used as an anti-rejection medication, but it increases my risk of infection.  They found mitral valve prolapse as well, but this only increases my chance for a bacterial infection in the heart.  They are not too concerned over this.  Initially, we will start medication and monitor its effects for two weeks.  If I have luck with it, we will wait as long as possible for the pacemaker.  If the medication slows the heart too much, then we will walk into the process of getting the pacemaker.  We are waiting on the results of one more test.  The computers  crashed while I was in the office.  Nice to know I am not the only one with technical issues!  If it shows that my heart is going into a block (getting confused and pausing for at least 3 seconds), then we will do the pacemaker immediately.  Driving will be a game time decision.  As my disease and status changes from minute to minute, the doctor wants me to be the one making the call.  He knows how life changing it would be for me to stop driving all together, so it will depend on how I am feeling and how I react to the medication. 

Lastly, if my prayer warriors would join me in praying about my appointment tomorrow.  As many of you know, the immune suppressant I take makes me 900x more likely to develop about 6 different kinds of cancers.  Those are not good statistics for long term use.  We found abnormal cells six months ago and had them removed.  I go in tomorrow for screening to see if they missed cells or if they have grown back.  It will take a couple of weeks for those results, but just going in for the screening has me a little worked up.

Thank you friends!  I also want to share that I have had other patients with HE read my blog, as well as physicians and they have contacted me.  God has created a network of patients for a really rare disease and I have been blessed to walk some of them through my journey.  I love it!