I told you so!

It did not surprise many in my family when I was diagnosed with a rare disease.  Don't get me wrong; they were horrified at the diagnosis of Hashimoto's Encephalopathy or HE.  It is just that as a kid, I stayed sick.  It became a joke in the family about which illness I would have during the holidays.  There were many Thanksgivings spent on the couch.  One year I had scarlet fever and the year my sister got married, I had Mono, strep throat, an upper respiratory infection, and my liver enzymes were elevated.  They were concerned my spleen would rupture, so I was put on bed rest from Thanksgiving through New Years.  One thing we noticed though, was fatigue was a huge factor.  If I stayed up late, I would wake up with a sore throat the next morning.  To say the least, my teen years were a lot of fun!  lol  As I would walk out the door with my friends or a date, my mother would remind me, "You know what happens when you stay out too late!"  I would wake up the next morning and be handed my orange juice with a big healthy, "I told you so!"  haha

If you have read my last couple of blogs, you saw my frustration with the fact that the Cell-Cept (immuno suppressive drug) was no longer enough to control the symptoms.  Clonazapam was added to stop the convulsions, then Gabapentin added later when the other two were no longer enough.  My symptoms continued to progress, so we made the decision to remove the immuno suppressive drugs.  Our theory was that if it was no longer enough to control the symptoms, then why put myself at high risk for infection and the other side effects. 

I rapidly declined and began to bounce between episodes of convulsions and tremors, then experience episodes of stroke-like numbness.  The only answer was to increase my medication to a higher level and go to bed.  As a mother, that was not a good solution for me.  I wanted to be proactive and not just curl up in the fetal position. 

After a whole community of prayers, I received a call last Tuesday from my Immunologist who had consulted my other specialists and decided to re-start immuno suppressive therapy with a stronger medication.  Of course, I will have to wait until I clear out the sinus infection I came down with after New Years, but it is a start.  My blood count will need to be monitored closely with this medicine, but I feel better knowing that we are taking steps to treat and not just mask the symptoms.       

in limbo

I am sorry that it has been so long since I have sent an update.  Between life, the holidays, and my scrambled brains...coordination has been difficult (pun intended.)  I went to the doctor this morning and finally have enough to share.

A month ago, I experienced a fairly significant increase in my symptoms (tremors, migraines, and convulsions.) Following a day of continuous movement, and I mean the involuntary type, I developed a headache that would not go away.  I awoke the next morning with continued pain.  After being awake a few hours, I suddenly lost the ability to move my left arm.  I began to drag my left leg a little as well.  This improved almost as quickly as it occurred and within a few days, I had regained full use. 

I notified my neurologist the following morning about the new symptom and of my concern.  I was assured that I just needed to increase my medication during those times.  I stressed that the increase made it impossible to stay awake, but my doctor's orders remained the same.  I have had three or four additional episodes that were less in severity but longer in duration.

I met with my immunologist today who agreed with my concern, but does not know of any new options.  He is considering putting me back on the Cell-Cept (immune suppressants) but wants to see my lab results and consult with my neurologist and immunologist first.  I have started taking Celebrex for the muscle and joint pain and I was asked to consider returning to the Mayo Clinic.  I will update when I have the results of the conference.  

...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!