Tuesday, October 20, 2009

A Divine Plan

The lumbar puncture I had last week seems to have left a permanent reminder of the event behind. Since the procedure, I have pain in my lower back and at times down my left leg. There are moments when the pain stops me dead in my tracks. I have called my doctor again, and still no return phone call. It was my hope that I could create this blog as a light of sorts, to those newly diagnosed with PTC; an informative guide with information about the disease and ways to deal with it. The problem is....I can't see the light anymore.

The methods by which they try to cure me seem to cause more pain. Today we celebrated my daughter's 9th birthday. I made her a cake, did some household chores and excitedly watched as she opened her gift. We bought her a Wii. Historically, I would jump right in and play along with my kids....instead, I lay on the couch watching from a distance until I eventually fell asleep in pain. Many times I hear those with chronic illness or cancer declare, "I will not let this disease define me." In my case, PTC has defined me. It defines everything I do. Whether or not I follow my plan for the day depends upon if I wake up in pain or not. I have few friendships because I can't do things that my friends do. I don't go shopping for fun, or hang out at the park with my friends. When I do have a good day...it doesn't last long. Within several hours after an outing, I'm left exhausted and in pain. Work for me is a distant dream, and I find myself jealous of my husband as he dresses up to go out to a meeting. Most days I am found in my sweatsuit or "stretchy pants" so I can rest comfortably. I am far from the woman I used to be.

My disappointment in myself carries over to my family. Often times I snap at them for not doing chores or for fighting with each other or for be disappointed in me. I do these things not because I am truly upset with my family, but rather because I can't do all the chores anymore, because I know that if I were more active with them they wouldn't fight with each other so much, and if I weren't ill they wouldn't be so disappointed by my many broken promises. My kids miss their mommy and my husband misses his wife. Moreover, I miss myself.

I keep reminding myself that "all things have a purpose under God". I'm certain there is a purpose for my current condition, I just hope I can figure it out. In the meantime, I recall the words of a song I used to sing often...

"When the weight of all my dreams is resting heavy on my head, and the thoughtful words of health and hope have all been nicely said...

Well, I'm still hurtin', wonderin' if I'll ever be the one I think I am....I think I am.

Then You gently re-remind me, that You made me from the first. And the more I try to be the best, the more I get the worst. And I realize the good in me, is only there because of who You are....Who You are.

And all I ever have to be is what you made me...Any more or less would be a step out of your plan, as You daily re-create me help me always keep in mind...that I only have to do what I can find...

And all I ever have to be....All I have to be.....All I ever have to be, Is what You made me."

So...for now....I just am. I do not understand my current state of pain and suffering, but am grateful for my family, grateful it's not worse....and still hopeful for God's plan for me.

Friday, October 16, 2009

Back at it...

As a blog that I started to keep track of my own battle against a chronic illness and as a way to bring awareness to Intracranial Hypertension, some have asked why I wrote my last post about the things my sister taught me. There were two reasons actually. One of those reasons is because she will never be forgotten and I felt it was important to honor her life by writing about her here for all to see. The most important reason, however, was in response to a post that another chronically ill patient suffering from Intracranial Hypertension had posted. This person was feeling rather down about her day to day battles against this illness, and in her desperation felt that she had nothing left to live for. She could not imagine anything worse than the daily pain she was experiencing, the things in life she had given up, the burden she was placing on her family and the agony of the treatments she had to endure in an effort to just feel normal. After reading her post, I knew I had to do something. Intracranial Hypertension is horrible...but there are things that are worse. Losing someone to cancer is worse. Battling cancer is worse. Having a life that is only half of the life you were born to live is better than having no life at all. So, I wrote my sister's story as an inspiration to those battling a chronic illness to remember to count the blessings you do have. To thank God that you have arms to hold your children and words to tell them you love them. To thank God that while you may be bedridden in pain...you are still here and will still be here tomorrow.

Lately, I have been battling my own inner demons. Since I last wrote, my doctors placed me back on Diamox. I can't really say whether the medication ever truly helped. I did lose some weight on the drug, but continued to suffer from fatigue and low back pain and pressure. My doctor had given me Dilaudid (a narcotic) to help with the daily pain, but without it I couldn't function. When I had last seen her in late August she had given me a prescription for the Dilaudid, but I wouldn't be able to get back in to see her until May of 2010 due to overbooking. I was placed on a cancellation list. By late September, I had run out of the pain medicine and was suffering from insomnia on top of the back pain. My doctor had gone to Scotland and would be out of town for the next week. I finally called my PCP to get a script. I was told often to go to the ER, but the copay for each ER visit is $100 and we still owe on previous visits. Over the last couple of weeks, the low back pain and pressure turned into head pain and pressure. My jaw in particular became inflamed and I could barely talk let alone eat. After fighting with my doctors office I finally got in for a lumbar puncture this past Monday. Typically, my husband is allowed in to hold my hand during these procedures but, this time he was not.

I laid nervously on my side in a tight fetal position, with my butt exposed for a period of 15 minutes of so, while the nurse and doctor prepared all of the equipment. They spoke to each other about things going on in their lives as if I weren't even there. For them this was just another day and another procedure, for me, this was the 6th or 7th LP I'd had done since March and I was terrified. I knew what was to come, and it never got any easier. First she rubbed my back down with the brown Iodine solution to keep the area clean and palpitated the area to find the "right spot". Then she said, "Your going to feel a little sting and some pressure as I put in the anesthetic." And, I did. The pain from the needle going into my spine was painful and the pressure intense. My body began to shake uncontrollably. Then...there was another. It is rare that you only have one shot of anesthetic. Sometimes it takes two or three. Now, it was time for the puncture. As the needle went in I felt an incredible amount of pressure. Lying there you think, "If she hits the wrong way, I could be paralyzed for life. If she does this wrong, I could end up with a severe infection. Or, if she goes in at the wrong angle this could be very pain...."; Just then she hit a nerve and pain radiated from my spine clear down my left leg. I moaned out in pain and she stopped. She tried again and I got the same result. Finally, she stopped and just let the fluid drip out....slowly. She left the needle in for nearly 25 minutes, while my body trembled before she finally decided to try to move it once more to get a faster drip. It worked. My opening pressure was 30 and after taking off fluid my closing pressure was 16. Normal readings are between 8 and 15 so, my pressure was definitely high. When she was finished I asked her if we now should consider a shunt as the medications weren't working. She said she didn't think I was a good candidate for a shunt and instead was referring me to a pain psychologist to discuss my feelings about my "loss of life" due to this chronic illness. In that instant, it became clear to me that my doctor had given up on me.

I came home feeling defeated. For the next two nights I would be up crying in both emotional and physical pain. My back was hurting so much I couldn't sleep and I felt as though my doctor was telling me that I had to face the fact that I would live like this for the rest of my life. My husband slept on the couch because he didn't want to disturb me....but, I needed him. I needed a shoulder to cry on. The puncture was on Monday and today is Friday. I have been in bed all week. When I am upright for any length of time, my head is in pain as well as my back. I have no doubt that some damage was done to my back during the puncture, but only time will tell. As for my head....well, I now have a spinal headache. I am trying to give that time too, as I have had a blood patch before and I don't want to ever have another one. For those of you who don't know what a blood patch is, it is when they take blood from another part of your body....i.e. your arm, and place that blood in the spinal puncture site by doing another lumbar puncture. That blood is used to clot up any cerebral spinal fluid leak. The problem is that they are putting the blood into a space where no space exists....so the area is stretched and it is very painful. So, I'm trying to wait this out.

As for pressure lowering medications....I'm not currently on any. My doctor wants to see how I do in the next few months. My pressures will go back up, undoubtedly...but, there will be nothing other than more lumbar punctures to treat my condition. I could feel hopless right now. I could throw in the towel. But, I have learned the lessons my sister taught me. In many ways I am alone, misunderstood, and may not have a bright future ahead....but, I have many blessings and I'm not willing to give up.

I have scheduled an appointment with two outstanding doctors in Columbus, OH to discuss possibly getting a shunt. Hopefully, they won't give up on me as my doctor here has. I hope.

Sunday, September 6, 2009

What My Sister Taught Me...

She wasn't my sister by birth. In fact, for the first 17 years of my life...we had never met. Once we did Dawn exhibited the shy, quiet mannerisms of her father...who would later become my father as well. When Dawn was small her mother had developed breast cancer. That cancer had spread into the bone and she later died. Subsequently, Dawn watched her aunts and cousins lose their lives to the same fate. Dawn missed her mother, but was raised by a loving dad who tried to fill the void.

I had my first child when I was just 19. Eight months later my mom would marry Dawn's father. We had become sisters, but we weren't very close. Dawn had a close circle of friends, but I was not yet one of them. We lived different lives and while I was very outgoing, Dawn still kept to herself when I was around. Then the unexpected happened. Dawn had met the love of her life. Before long she would marry him and have a child of her own....and then it happened. As mothers with young sons, it took little time before Dawn and I found our common bond. I began babysitting for her and we would take our children out for pizza, or a movie, or the zoo, the fair, or our favorite...the pumpkin patch. Then I married and moved away from home. We didn't see each other as much or speak as often, but she had become my sister and I loved her. Of course, we would still see each other for birthdays and holidays, and we even kept up our annual pumpkin patch visit. We also took our annual vacation with our entire family to Cedar Point. She and I would ride the best roller coasters together and chat about the "awesomeness" of it all. We shared many laughs together.... and tears.

We cried together when Dawn found out, in her early 30's that she had breast cancer. That day she filled us all in on a secret. She said, "I always knew this day would come, I just didn't know when. I have prepared myself for this day my entire life." Amazingly, she was showing us her strength even in those early days, when the news of her cancer was shaking the rest of us to our core. Dawn continued to show that stregnth as she had a double mastectomy, radiation and chemotherapy and eventually had her uterus removed. Her son was a miracle for she was never supposed to be able to have children, but now she knew she would never have the chance to have another child and she wanted a baby so bad. She was sad but, she gave thanks to God for her life and the son she had. (Lesson 1: Thank God for the blessings you have, because your life could always be much worse than you ever imagined.) (Lesson 2: Everyday that you wake up and see your loved ones is a miracle and a gift.)There was good news, however....the chemo and radiation had worked. The cancer was gone. Dawn went into remission. But, Dawn had another secret that I wouldn't know about for another couple of years.

After her battle with cancer, Dawn tackled each day with a renewed spirit. She, her husband and her little man went on trips to Disney World, the beach, amusement parks, concerts and anything else she could think of. She worked hard and she played hard....but, she loved even harder. Eventually, Dawn started to drop little hints regarding her secret...but, I was too naieve to catch on. One night we went to dinner with our parents and our children. I was leaving town the next morning. When it came time to say our goodbyes I gave Dawn a hug like I always do and told her that I loved her....but Dawn hung on a little longer this time, and the hug ran a little deeper. (Lesson 3: When you give a hug and you tell someone you love them, really mean it and make it last. You never know if you'll ever truly see them again.) She kept telling me that she really wanted to come up to see my new home, but I was still in the midst of painting and trying to get our home set up. I wanted her to come up when I had things more in order. (Lesson 4: Don't let vanity stand in the way of spending time with family. The cobwebs will still be around to remove tomorrow...but your loved ones may not.) Anytime we came into town she insisted we stop by to see her. No matter what time of day, she would make the time to spend with us. (Lesson 5: Make time for your loved ones, even if your not feeling well and even if you have to make sacrifices.) That year during our annual Cedar Point trip she wanted me to wait in an hour and a half line to ride our favorite coaster. However, my husband couldn't ride that year and I felt guilty leaving him behind. After several minutes in line with her and our brothers, I told her I felt horrible about it and got out of line. Unusual for her character, she begged me to stay and share this experience with her...but, I got out of line anyhow. (Lesson 6: Sometimes it's okay to live in the moment....because that moment may be all you get.) During that same trip, she talked me into going way up above the ground on the "Rip Cord" ride. So, we tied ourselves together, were anchored high above our family and on the count of 3 one of us had to pull the cord that would send us sailing straight towards the ground and flying through the air. Of course, I'm scared of heights and couldn't find the courage to pull the cord, so she did it for me. We screamed, we laughed and yes, we soared. I was sooooo glad she pulled that cord. (Lesson 7: Strength can often be drawn from your closest friends when fear binds your spirit.) When we got off the "Rip Cord" ride we had the option to purchase the video of our moment together soaring through the air. The video was $25. After a two day trip to the amusement park, a hotel stay and a 7 hour drive from and to home...as a parent of three children this seemed like an unnecessary purchase. Although she begged me to get the video...I passed. (Lesson 8: Every chance you get record your special moments with photos or a video. While we have our memories, there is no better way to capture a moment than with a picture or film of the event.)

As you may have guessed by now, it wouldn't be long before Dawn would become ill again. She was fatigued, running a fever and felt like she had asthma. She was told by her doctor that she had the Epstein-Barr virus. (The virus that causes mono.) She would soon find out that her cancer had returned and spread to her lymph nodes, her lungs, her bone and eventually her liver and her brain. When she found out that the cancer had returned she knew this time would be different. We cried together. I visited her at the hospital as much as I could and offered to wipe her forehead, give her the medication she needed...anything I could to make her better. In a matter of months she quickly deteriorated. She was in pain, weak and sad...but, she asked me all the time to call her...no matter what time. She would beg me to stay on the phone with her, as she didn't want to miss a moment. She was so sick...but, we would talk for an hour on the phone until she couldn't talk anymore. I hated being away from her, but, I knew her husband was taking very good care of her and I had children at home who needed me.

And then, I got the call. Dawn had asked for all of her family to come to her side. She was at home and wanted us all there...she knew her days were coming to an end. I rushed from NY to Ohio in what seemed an instant. In her final days, I kept vigil over her. I again, held her hand, wiped her forehead, gave her the medicine she needed and anything else I could...to bring a miracle. I thought that if I worked hard enough, prayed hard enough and gave enough of myself that she would just get better. Hospice was there. Her family was there. But, somehow I thought we could still beat this. Occasionally, Dawn would speak to us about how she had seen her mother and spoken with her. She would make jokes and make us laugh, and she would pray with us. (Lesson 9: Don't lose your faith or your sense of humor...in a crises it may be all you have to hold on to.)In a matter of a week, she eventually lost her battle and at the age of 36 my sister passed away. I felt horrible. I felt like I had failed her. But, at that moment, I learned of her secret....she always knew the cancer would come back and take her life. (Lesson 10: It's best to face life's adversities head on. Denial only keeps us from enjoying the other many blessings we have.) It also came to me in that moment, all of the lessons that she had taught me. Those I've already mentioned including the final two. (Lesson 11: No matter how hard we pray or how much work we put into avoiding our fate...Ultimately, God is in control. Continue to pray and make sound decisions, but know that in many ways our lives our in His hands.) and finally, (Lesson 12: There is life after death.)

If you've ever seen the broadway hit "Wicked", you'd know that it is a musical about the two witch sisters from the "Wizard of Oz". The good witch...and the evil witch. In this musical, the two sisters sing the song "For Good" to each other. The words to this song are as follows:

(Elphaba): I'm limited Just look at me - I'm limited

And just look at you You can do all I couldn't do, Glinda

So now it's up to you

For both of us - now it's up to you...

(Glinda): 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'm who I am today Because I knew you...

Like a comet pulled from orbit As it passes a sun

Like a stream that meets a boulder Halfway through the wood

Who can say if I've been changed for the better?

But because I knew you

I have been changed for good

(Elphaba): It well may be

That we will never meet again

In this lifetime

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 handprint on my heart

And now whatever way our stories end

I know you have re-written mine By being my friend...

Like a ship blown from its mooring

By a wind off the sea

Like a seed dropped by a skybird

In a distant wood

Who can say if I've been changed for the better?

But because I knew you

(Glinda): Because I knew you

(Both): I have been changed for good

(Elphaba): And just to clear the air

I ask forgiveness For the things I've done you blame me for

(Glinda): But then, I guess we know There's blame to share

(Both): And none of it seems to matter anymore

(Glinda): Like a comet pulled from orbit As it passes a sun

Like a stream that meets a boulder Halfway through the wood

(Elphaba): Like a ship blown from its mooring By a wind off the sea

Like a seed dropped by a bird in the wood

(Both): Who can say if I've been Changed for the better?

I do believe I have been Changed for the better

(Glinda): And because I knew you...

(Elphaba): Because I knew you...

(Both): Because I knew you...

I have been changed for good...

Of course, my sister and I aren't witches...but, in many ways we were very different. Given all she has taught me I have been changed for the better for having known her. I wanted to sing this song at her funeral, but in my sorrow didn't feel that I could do this song justice. Therefore, I relinquished....

But, you may be wondering how I know that last lesson to be true...you know, the one about life after death? Besides the promise that Jesus Christ made. And, besides the fact that Dawn mentioned speaking to her mother and grandmother who had already passed while she was on her death bed...Dawn made her presence known in the afterlife.

You see...this year my 18 year old son Josh graduated from high school. Josh was my first born and therefore, out of my three children Dawn had known him the longest. No doubt she wished that she would have lived to see the day. On the day of Josh's graduation the ceremonies were held in an outdoor arena at sunset. It was a beautiful day. Keeping in mind that my sister loved birds...there were birds sweetly singing in the arena on that day. But, what caught my breath was the song played for the ceremony. The choir sang, "For Good"....and I sobbed. My son, who was sitting amongst the graduates could not see me. But, after the ceremony he came to me and said..."Dawn was here today wasn't she?" And, I know she was.

She will be with us forever until the day comes that I can meet her again. She has left a "handprint on my heart", and now after hearing of her lessons....I hope she has left a handprint on yours.

Dawn's Life Lessons in Review:

1: Thank God for the blessings you have, because your life could always be much worse than you could possibly imagine.

2: Everyday that you wake up and see your loved ones is a miracle and a gift.

3: When you give a hug and you tell someone you love them, really mean it and make it last. You never know if you'll ever truly see them again.

4: Don't let vanity stand in the way of spending time with family. The cobwebs will still be around to remove tomorrow...but your loved ones may not.

5: Make time for your loved ones, even when your not feeling well, and even if you have to make sacrifices.

6: Sometimes it's okay to live in the moment....because that moment may be all you get.

7: Strength can often be drawn from your closest friends when fear binds your spirit.

8: Every chance you get record your special moments with photos or a video. While we have our memories, there is no better way to capture a moment than with a picture or film of the event.

9: Don't lose your faith or your sense of humor...in a crises it may be all you have to hold on to.

10: It's best to face life's adversities head on. Denial only keeps us from enjoying the other many blessings we have.

11: No matter how hard we pray or how much work we put into avoiding our fate...Ultimately, God is in control. Continue to pray and make sound decisions, but know that in many ways our lives our in His hands.

12: There is life after death.

Wednesday, September 2, 2009


Diamox is a fluid reducing medication originally created to treat Mountain Sickness, certain seizure disorders and glaucoma. As mentioned previously, there are no medications currently on the market specifically developed to treat IIH. Therefore, somewhere along the way doctors thought ...."Hmmmm, what medications are available today that reduce fluid levels in the body." To which several responded, "Diamox!"

I was first introduced to Diamox (Acetazolamide) shortly after I was diagnosed with PTC/IIH. Typically, high dosages of Diamox are reserved for those whose opening pressures are above 40. (An opening pressure, is determined when a lumbar puncture is performed. That pressure is than guaged as the cerebral spinal fluid rushes out into the vial.) My opening pressure was 28. While stil high, my pressures were not high enough to justify a starting dose of 1500 mgs. of Diamox taken in 250 mg. tablets....two in the morning, one in the afternoon, and two more before bed.

Those first days were a nightmare. I couldn't get out of bed. I was literally dying of thirst. I felt like my body was shutting down. You see, Diamox, lowers fluids which can be a good thing if you have IIH....but, this also means that your Potassium levels and Electrolytes get all screwy. In turn, you feel like you are dying. I remember crying in the shower because I had to sit down and didn't have enough energy to bring the wash cloth to my face. I was like this was for 4 weeks. I suppose had my headaches gone away, I may have felt it was all worthwhile. But, they didn't. On top of being weak, nauseous, and feeling very dry....I also had a tingling sensation in my feet and hands. (Kind of like when a body part "falls asleep") Food and drinks tasted funny. Sodas especially were out of the question. A Coke tasted like battery acid. Occasionally I would get a rash...but, overall....I was just miserable.

Because I was still having headaches my neurologist at the time decided to up my dose another 1000 mgs. per day. Once I was up to 2500 mgs. per day, I was really miserable. My headaches actually worsened. I was taking narcotics everyday to control the pain and nausea. After a week, I decided to stop all medication.

I was then connected with a specialist and eventually placed on Topamax. This medication was primarily developed to treat migraines and seizures, but, doctors again, found the drug somewhat helpful in treating PTC/IIH. Initially, my starting dose was 25 mgs., then up to 50 mgs. then up to 75. However, getting up to 75 was tough. For some reason at that level, side effects would kick in. The first time I had eye pain, the second time I had extreme vertigo and the last and final time, my hair started falling out. While I felt that this medication helped somewhat...it could not work alone.

Then I saw a specialist at OSU who suggested I take 20 mgs. of Lasix twice a day with a Potassium supplement. Lasix, like Diamox is a diuretic used to treat fluid retention in Congestive Heart Failure, Liver Dysfunction, and Kidney Disease....also used to treat High Blood Pressure. Again, although this medication was not created to treat IIH/PTC...some genious felt it was a good try. Unlike Diamox, Lasix is much milder and is typically used in combination with Diamox or Topamax to properly treat the symptoms of IIH. Once I added the Lasix and Potassium to my regimen, I began feeling a little better. However, I was still going to the ER approximately every 2 weeks or so for severe uncontrollable pain.

Therefore, my specialist recently decided that it was best for me to go back on Diamox, albeit a much smaller dose. Vertigo, losing hair and continual pain only pointed out that the Topamax wasn't the best idea for me. So a week ago I started taking 500 mgs. of Diamox at bedtime while sticking with my Lasix/Potassium regimen. Since that time, I have been in bed almost daily. Blocking out the light and taking narcotics for pain and nausea. I can't imagine that the medication is helping greatly because the "whooshing" sound in my ear has returned, indication that my pressures are high. I have neck muscle pain and low back pressure as well. The scary part about this is that my next step is shunt preparation. If there is anything worse than Diamox....it's shunt surgery.

So...just for the record....I'm considering becoming a habitual liar. Eternally taking a medication that doesn't work, only to claim that it does. Managing my daily pain with narcotics and gritting through my teeth....all in an effort to avoid the shunt. It's all about staying away from the shunt. Diamox...Shiamox....Can't live with it....Can't seem to avoid it!

Monday, August 24, 2009

Ego Over A Miracle....What Would Jesus Do???

So, I got to thinking. If Jesus were alive today and He had the opportunity to heal those with IIH ....BUT...the catch was He had to do it secretly and couldn't take credit for it....would He still do it?

Well, of course He would. That was what was so spectacular about Jesus Christ. He came to us minus an ego.

Doctors are healers. They go to school for a long time to learn how to heal people. I currently see an "IIH Specialist" here in Rochester, NY whose job it is to know how to properly treat those with IIH....albeit, current treatments are insufficient and much research is needed.

As I have mentioned in my earlier post; I cannot idly sit by in pain, with friends in pain, and do nothing. Therefore, I have decided to try and organize a fundraiser for IIH research. So, imagine my enthusiasm when I went to see my specialist this afternoon for my two month checkup.

After my appointment, I told her of how deeply it pains me that the medications don't seem to be working for me and many others. How I realize that a surgical shunt may not be too far off in my future and the idea frightens me terribly because I know that the procedure is not sound. I expressed my deep concern for the way IIH patients are treated so poorly in emergency rooms and hospitals across the country by medical personnel who know little about our pain and this illness. I told her that "the treatments being used today are the same treatments used 100 years ago and much research is needed." And, I told her that "it is not in my nature to sit by and take this lying down...I have to fight. God gave me this ailment for a reason. I want to make a difference, no matter how small that may be."

I said, "I want to start a fundraiser here in Rochester, NY for IIH research."

She said, "And who would these funds go to? Who would be doing the research?"

I said, "The Intracranial Hypertension Research Foundation."

She said, "Well, you can count me out. I won't participate in a fundraiser for someone else's research!"

Me: (Taking a moment to absorb the shock)

I said, "Um, okay....why not? This is not someone else's research. This is research for IIH. My disease. Your work. Why would you not fund that?"

....Now, let me interject here and say that there can only be one reason. EGO! This is a simple case of a "Big Fish in a Small Pond" syndrome. My doctor is the same one that told me not to go to the ER at OSU hospital while visiting family in Columbus, OH. She said that "it's the worst place you could go for this." Nonetheless....I went to OSU....and I was so glad I did!!! Unlike many other hospitals across the country they knew what IIH was and moreover had protocols in place for the staff on how to treat it!!!! It was like heaven for people with IIH. I suppose my "specialist's" ego has gotten the best of her.....and unfortunately....it may be getting the best of me.

....I digress.

So, she said: "Well, I used to be on the board of the Intracranial Research Foundation and I just wouldn't fund research there." (No specific reason given)

I said, "Okay, well my goal here is to make the most progress toward a cure. If you have better ideas of where the funds could better be used I'd be up for any suggestions. Are you suggesting that you are doing research here?"

She said, "Well, we have a grant for research, but we aren't currently doing any research."

(Long Stupid Pause)

Me: "Ummmm.....Okay......Well, when you do start research what will your research be on?"

She said, "We will be doing research on headaches and how they affect daily living."


She suggested that I do a fundraiser for the National Neuro-Opthalmology Board earmarking the money for IIH research, as the N/O Board issues research grants each year. But, I only think she finally made this suggestion after I continually pressed her for an explanation. The bottom line was....She would not participate in helping with a fundraiser to finding a cure for IIH.
And this is my doctor!!!

I guess I better start praying for some miracles!

Sunday, August 23, 2009

My Story

Idiopathic Intracranial Hypertension (IIH) or Pseudotumor Cerebri (PTC)....what is it? Prior to my diagnosis I had never heard of it. Nobody in my family had it. I had no friends who had been diagnosed with it. To me, it was all a foreign language. My family members and friends were equally confused...and I think to some extent still are. In fact, I think it is fair to say that many of the medical professionals I come across are confused as well....some who have never heard of it. So, let me define it for you here....

Intracranial Hypertension is a condition in which the body either produces too much cerebrospinal fluid pressure or doesn't absorb it well, resulting in increased cerebrospinal fluid pressure. It is likely due to high pressure caused by the buildup or poor absorption of cerebrospinal fluid in the subarachnoid space surrounding the brain, but the reason for the buildup or poor absorption are also unknown. The disorder is most common in women between the ages of 20 and 50. And at one time, being overweight seemed to be a contributing factor. "Seemed" being the operative word, as the subject of weight and it's impact on IIH is still being researched. There are many people in the world who are over 500 pounds who do not have IIH and there are very skinny people who do have the disease. There have been patients who have been overweight and dropped thier BMI range down to "normal" levels and still had IIH. Unfortunately, this disease affects men and children as well, of all races, skinny and fat, short and tall. It knows no bounds. IIH affects us each differently. Some of us will have success with medications and some of us will require surgeries. Some of us will go into remission, only to have the disease return months or years later. Others will go into remission and be lucky enough never to see the disease rear it's ugly head again. And, then again, there will be some who will be disabled for a lifetime. No amount of medication, lumbar punctures, surgeries or dietary changes will make a difference. At least, not yet. We hope with more research and funding for a better day.

Symptoms of Intracranial Hypertension are: headache, sometimes daily, sometimes severe, not relieved by medication; hearing loss; impaired vision or eventual blindness; memory problems; nausea; pain behind the eyes; pulsating intracranial noises ("swooshing" sound or heartbeat); shoulder, back and/or neck pain; tinnitus; and vomiting. In addition, patients have complained of swelling behind the ears; swollen lymph nodes; night sweats; muscle spasms; abscence seizures; insomnia; depression; mood swings; and dizzy spells...although not all of these latter complaints have been thoroughly researched enough to be included in the definition of IIH.

Diagnosis: *A lumbar puncture (or spinal tap) is considered the gold standard when it comes to an IH diagnosis. This test measures the pressure of the cerebrospinal fluid that circulates in the sub-arachnoid space, which surrounds the brain and the spinal cord. When intracranial pressure is high, that pressure is transmitted through out the entire sub-arachnoid space. Diagnosis is usually confirmed after a spinal tap reveals a high opening CSF pressure.

*For adults:Normal pressure readings are generally below 200mm/H2O. Borderline high pressure readings are between 200-250mm/H2O.Anything above 250mm/H2O is considered a high pressure reading.

*For young children:Anything above 200 mm/H2O is considered a high pressure reading.

Once the spinal tap is taken and the opening pressure is taken the fluid is checked for abnormalities. Patients with IIH have normal cerebral spinal fluid (CSF) findings. In addition, patients with IIH will also have the following for diagnosis:

  • Normal CAT scans and MRI's
  • No sign of brain injury (No paralysis/muscle damage, etc.)
  • Has signs and symptoms of increased intracranial pressure such as headache and/or papilledema (swelling of the optic nerve behind the eye)
  • Patient is awake and alert
  • Has no other cause for increased intracranial pressure

Treatment: Now that I have given a thorough, and respectfully, a rather boring definition of this disorder let me say here that the incidence of disease for the general population is only 1 in 100, 000. So, by all standards this disease is considered rare. Therefore, we do not have a popular celebrity speaking on our behalf to find a cure. No pharmaceutical companies have stepped up to the plate to find new medications. The same treatments that were used 100 years ago to treat this disease are still be used today. Those treatments consist of medications which were created for other diseases (i.e. glaucoma/mountain sickness) and as such give IIH patients horrible side effects.

Or, for those with severe papilledema...optic nerve fenestration surgery. *During surgery, a small window-like opening is made in the sheath around the optic nerve, which allows cerebrospinal fluid (CSF) to drain behind the eye and relieves optic nerve swelling. This surgery is primarily used as a tool to try and save vision, but does nothing to relieve the pain of daily headaches, neck and back pain caused from increased (CSF) squeezing the brain and spine.

Lastly, and probably most barbaric, are the neurosurgical shunts. Shunt operations are also performed when drug therapy has been unsuccessful. *Shunts may be used to control papilledema and prevent vision loss, as well as to treat headaches that have been unresponsive to any medication. A neurosurgical shunt is a surgically-implanted catheter that is used to drain CSF into another area of the body such as the abdomen. A shunt lowers intracranial pressure by removing CSF to another site, where it can be absorbed. The problem is that these shunts are used on the brain as if we were treating a plumbing problem and not a neurological one. Therefore, there are many complications. *Shunts have a checkered history with an initial 50% success rate and alternately, a high revision rate of 50%. They are the most common pediatric neurosurgical procedure and the second most common neurosurgical procedure for adults.
Unfortunately, there is no “perfect” treatment for chronic IH, especially since every person’s experience is different. While surgery certainly helps some people, it can also lead to repeat operations, which can produce serious, sometimes life-threatening complications. The most common problem with shunts is that the catheter becomes blocked and has to be replaced.

So, now you ask....how did I get here? Well, I have been asking that question myself. Sometimes, I think it may have been a lifetime in the making. The term "Idiopathic" essentially means that there is "no underlying cause. Doctors and scientists don't really know what causes IIH. But, they do believe there are some risk factors as listed below. Additionally, they have discovered a second type of IIH called Secondary IIH which unlike primary IIH has a definitive causitive source for the disease. These are listed below as well....

Risk Factors for Developing IIH:

Exogenous Substances:

  • Non-steroidal anti-inflammatory drugs (NSAIDs), tetracycline, nitrofurantoin, isotretinoin, tamoxifen, nalidixic acid, lithium, and the starting or stopping of steroids
  • amiodarone,
  • antibiotics,
  • carbidopa,
  • levodopa,
  • chlordecone,
  • corticosteroids,
  • cyclosporine,
  • danazol,
  • growth hormone,
  • indomethacin,

Systemic Diseases:

  • anemia,
  • chronic respiratory insufficiency,
  • familial Mediterranean fever,
  • hypertension,
  • multiple sclerosis,
  • polyangiitis overlap syndrome,
  • psittacosis,
  • Reye syndrome,
  • sarcoidosis,
  • infectious mononeucleosis
  • systemic lupus erythematosus…
  • Disorders of cerebral venous drainage

Secondary IIH : While pseudotumor cerebri or primary intracranial hypertension is idiopathic, secondary intracranial hypertension always has a clear cause. Possible causes include:

  • dural venous sinus thrombosis
  • kidney failure
  • leukemia
  • lupus
  • excess Vitamin A
  • growth hormone treatments
  • nasal fluticasone propionate

As the story goes my mother says that the doctors thought I had something like Leukemia when I was 3 years old. One day I just didn't wake up. I didn't come rushing out of my room, with giggles and grins. My mother finally came in to check on me and I had a horribly high fever. I was lethargic and had a rash. I believe she said I was like this for weeks.

After this, I guess my health never really was perfect. My childhood was riddled with terrible strep throat infections. One after another. (I believe I read somewhere that the streptoccocus bacteria was linked to IIH as well). I remember there was always a gaggle of nurses holding me down to keep me still in order to get that penicillin shot in my behind. In between the strep infections, I constantly had urinary tract infections and subsequent kidney infections. It seems I was always on an antibiotic for something.

Around the time I was 9 or 10 I got my first cold sore. I suppose, I am forced to admit here that I was a long time, neverending, thumbsucker. It was a habit I never wanted to give up. As such, when I got my first cold sore on my lip, it was eternally exacerbated by my bedtime thumbsucking. By morning, that one cold sore had turned into 3 as it had spread across my lip. Three or four days later I was unrecognizable as from lip to nose I had cold sores. I guess my brother had a bout of impetego when he was a small child. My parents had been informed that the way you treat such a thing was to scrub it with hot soap and water. Without taking me to the doctor, they assumed that this terrible mess I had on my lip was what my brother had when he was small. This is when the torture began. My mother would have to hold me and my father would have to scrub the cold sores until they bled. I would scream. They thought they were doing what was right...I suppose. But, the infection spread horribly and just got worse and worse as the days drug on. Eventually, the sores were so bad and so ugly that they had to take me to the doctor and they were told that I had cold sores or (HSV1). My parents felt horrible, of course. But, due to this initial first experience....other outbreaks would always be worse. I mention this as an importance because, to this day I get these cold sores. Not as often, and certainly not as bad. But, when I do get them....I get them up inside my nose. As the arteries and nerves in the nose and sinus are so closely linked to those in the brain and (CSF), you may understand my concern about this chronic infection in my nervous system? (It's also important to note that it was about this time that my parents first noticed the swelling behind my ears.)

When I was 13 I suffered a severe case of infectious mononeucleosis. I was out of school for 3 months. My liver was swollen, I had blisters on the back of my throat resembling mold growth and I had high fevers. The good news was that after this infection it would be many years before I would ever have strep throat again!

During all of this my mother had been taking care of my maternal grandmother who had suffered multiple strokes. My aunt had been diagnosed with Rheumatoid Arthritis and she was having some health troubles of her own. My mother was suffering from debilitating migraine headaches. It wouldn't be long before I began having migraines of my own....and in fact, by the time I was 17...I was.

Initially, the migraines would only hit once a month. Right around my menstrual cycle. They were pretty predictable. Sometimes stess or lack of sleep could bring them on....but mostly, it was hormonal. I could take a couple Excederin Extra Stregnth tablets and within an hour I would have relief. By my Senior year of high school, however, I started having other problems. There were times when the Excederin wouldn't help with pain...in fact...nothing would. I started having memory problems, and vision problems (seeing spots, blurred vision, etc.) And, there were times when I would have horrible muscle pain in my neck and shoulders. I recall asking my friend Maria for massages and/or sometimes missing school due to a pulled muscle.

When I was 19, I had my first child. Maybe due to hormonal changes, lack of sleep, stress or a combination of all three, the headaches worsened. By the time I was 21, I was seeing a neurologist for the migraines. Over the years the doctors tried muscle relaxers and triptan medications typically used for migraines such as Imitrex, Axert (sp?), and Relpax. Nothing seemed to work. The only thing that ever seemed to give me a little bit of relief was the Excederin or some sort of pain medication.

By my mid 20's to late 20's I was diagnosed with Fibromyalgia. My neck and shoulder muscles hurt often, and just like in my teens I was getting pulled muscles as well. But, the diagnosis never really seemed to fit. I read up on Fibromyalgia. I didn't hurt all over my body. I didn't hurt to the touch. It was just my head, neck and back. He put me on a regimen of muscle relaxers. The thought was that if we could get the muscles to relax that suddenly, the migraines would dissipate. Well, the muscle relaxers made me too drowsy to function. As I was a single mother...I had to function. This wouldn't work. I had to stop the medication.

When I was 30, I got married. We moved two hours north from my hometown of Columbus, OH to Medina, OH. By the time I was 31, we now had three children and I had come down with the virus that cause mononeucleosis called the Epstein-Barr virus. I was sick for over a year with fevers, swollen lymph nodes, chronic fatigue and migraines. This seemed to be the turning point. Because after this illness, I had daily headaches and back muscle pain. We again saw neurologists. I was placed on preventative medications like Gabapentin and Depakote but hated the side effects (drowsiness, weight gain) so much that I stopped the drugs. They also tried Amitryptiline but that just seemed to make things worse. Often times, after trying Excederin Migraine I would find no relief and would end up in the ER hooked up to an IV with a dose of Demerol and Phenergan to relieve the pain. This would typically give me some breathing room for approximately 3 days or so before the headaches would begin again.

I blamed it on the stress of a new environment, having young children, a new marriage, a snoring husband, lack of sleep, etc. But, honestly....it didn't matter....I was just miserable. The doctors were frustrated and nobody could seem to figure out what was wrong with me.

Then I started having strange symptoms. I would get tingling and numbness in my extremities. Sometimes I thought I was having a stroke. Then, when I was 35 we moved to Rochester, NY. Shortly after moving there, I had an episode that left my entire right side numb and weak.....but some of my left side too, from the knee down. I was admitted to the hospital. The doctors there diagnosed me with Hemiplegic Migraines. "Hemiplegic" meaning one-sided paralysis. So that explained the numbness on my right side. What they couldn't explain was the weakness on my left side as well. After several days in the hospital, I developed SEVERE head pain. MRI's and CAT scans came back normal. I was told that they could give me Tylenol and accused of drug seeking. Of course, I believe that as early as the age of 31 I started having IIH symptoms and this time in the hospital I was having an IIH episode misdiagnosed as Hemiplegia. Like many IIH patients, I may have gone misdiagnosed and mistreated for years. And like many IIH patients, I too, have been accused of "drug seeking." After 8 hours in a state of EXTREME pain, I finally called my husband and checked myself out of the hospital.

I started having lymph node swellings and jaw pain along with the daily headaches. Insomnia and night sweats too. I went to my OB/GYN and had blood work done to check for any hormonal imbalances or thyroid disorders. Everything came back normal. My menstrual cycles became irregular and my hair began falling out. My fatigue worsened. After a couple of years, I woke up one day with a sore throat. When I went to the doctor he said he thought it was just allergies. Only a few hours later, I had developed a lump at the front of my throat the size of a baseball. I went back to the doctor and he immediately sent me to the ENT. After some additional testing, I was diagnosed with a congenital defect called a Thyroglossal Duct Cyst. This is a condition that typically shows up in childhood....but, oddly enough, it showed up when I was 37. The cyst was surgically removed.

The lump was gone. But, my condition didn't improve. I was still having night sweats, occasional lymph node swellings, jaw pain, neck/back pain, daily headaches, memory function trouble, insomnia, chronic fatigue, irregular periods, and yes....occasionally, the front of my throat would still swell up too. I also started gaining weight. Lot's of weight. Like....40 pounds of weight. I hadn't changed my habits, but, my body was changing....quickly. (Other IIH patients have complained of this as well. They had strange symptoms and then suddenly had a large onset of weight, that they could not lose.)

But, one morning I awoke to a new symptom. A "whooshing" noise keeping time with my heartbeat in my ear. It was constant. (This, unbeknownst to me was caused from the CSF putting pressure on my blood vessels.) I started seeing a new neurologist. I would wait at times for up to 3 hours to see him. I recall one time being in such excruciating pain in his office and they forgot about me. They almost closed up the office and left me there. I ended up in the ER many times during those months. Every visit he would check my vision and if I had severe head pain I would have blurred vision. He commented several times that he saw some papilledema. Once he gave me an IV of a cocktail they call "Depakon". I don't know what is in it, but I got severely ill vomiting all night. Then he tried a Calcium Channel Blocker used to treat Hemiplegic Migraines called Verapamil....but I broke out in itchy hives and had to take Benadryl as I was allergic.

Then I started having more cognitive problems. I would smell something that smelled like an "electrical smell". I would ask those around me if something was burning but nothing could be found. Shortly thereafter I would be in the midst of a conversation and just stop midsentence or just "disappear". These were small seizures caused from the immense pressure (still undiagnosed) on my brain.

I also had a constant runny nose....constantly. It was a clear fluid coming out of my nose. I needed a tissue all of the time. It was embarrassing. I would have to get up from meetings to get a tissue, or excuse myself at lunch. Of course, this was a condition called CSF Rhinorrhea caused by high CSF pressure for long periods eventually wearing down the sinonasal bone creating a hole or a CSF leak. Again....I was still undiagnosed....and at this point, probably walking around with very dangerous pressure levels.

My mother, in the meantime, had just been diagnosed with a mild case of Multiple Sclerosis. As some, but, not all, of my symptoms, sounded a lot like MS I asked my neurologist about the possibility. He said that indeed, it could be a possibility and given the fact that my mother had been diagnosed my chances of having it had increased. An MRI and MRV were ordered. The tests came back negative. My doctor called my husband and I into the office and after another gruesome 3 hour wait, went through each of the scans with us. Laughingly he told us that, "You have MS....not." Like it was some kind of joke. He said, "You just have migraines." I knew he was wrong. I knew there was something more wrong with me. I broke down and cried. Sobbed really. He said, "What is wrong? Why are you crying?" I said, "I have been seeing you for months. I have had tests run, I have been poked, forgotten about, poisoned and yet you have done nothing to help me. Everytime we come here we wait over 3 hours to see you and you laugh at me as you tell me that I 'merely' have migraines when I know that there is something more seriously wrong." And do you want to know what he said in response? He said, "I can no longer care for you. I will no longer be your doctor."

So, I resigned. Upset and defeated I went home and looked up Hemiplegic Migraine on the internet. Maybe there is more to it than I know. As I started looking up information and medications used to treat this type of migraine I came across a medication I had never heard of before called Diamox. I clicked on the hyperlink and sure enough this medication was defined as a drug used mainly to treat mountain sickness but also to treat a disease called Pseudotumor Cerebri. Upon reading more about this disease, I jumped up and screamed for my husband in the other room. I said, "This is it!!!! I know it!!!! I'm not crazy!!!! This is what I have!!!! I have had all of these tests run except for the one defining test that counts....A lumbar puncture! I have to find a doctor that specializes in this!" I immediately began looking for a doctor in our area who knew about the disease, and I found her. But, when I called her office I was told that she was on vacation and she wasn't accepting new patients. I left my name and number anyway, and begged the secretary to ask the Dr. to reconsider. I had no idea that fate would step in.....

A few weeks later I woke up with a pulled muscle in my neck. It was very painful and I couldn't move. The doctor described the muscle as being "infected", as if something were irritating it. I was put on muscle relaxers. But, a few days later I awoke with THE WORST HEAD PAIN OF MY LIFE!!!! I have had migraines at this point for over 20 years, but this was the worst pain of my life! My husband was trying to get my girls off to school but I couldn't wait that long....he had to bring my daughter to the hospital with us. When we arrived, I told them of my symptoms and then I said, "the only test that has never been done is a spinal tap. I think I may have Pseudotumor Cerebri. Please do a tap."

A tap was done.....my opening pressure was 280, I had papilledema behind both eyes, my fluid was normal, my CAT scan and MRI was normal. It finally happened.....I had a diagnosis. I had Intracranial Hypertension. Thank God! Now I can get well.....

.....too bad that's not the way this disease works! I'll leave out all the details about how I was temporarily paralyzed and couldn't urinate or have a bowel movement on my own due to the first lumbar puncture (for almost 7 days). And, how I've had Urinary Tract Infections ever since. I will tell you that I was placed on an abnormally high dose (for my opening pressure), of 1500 mgs. of Diamox to start. Diamox lowers your fluid levels and can mess with your electrolyte levels, kidney function and potassium levels if not monitored properly. Needless to say, I wasn't being monitored. I was also taking 20 mgs. of Lasix every day (another fluid lowering drug). I couldn't move. I was very weak. I had tingling sensations all over. I couldn't take a shower because I couldn't lift a wash rag. I slept all the time. I was thirsty all the time. I remember my husband brought me some orange juice and I thought it was the best drink on the face of the planet. I slurrped it down in about .02 seconds. Those first couple of months were a nightmare. I wasn't feeling much better. I was in and out of the ER. The doctors would occasionally put me on steroids to help with inflammation, but that would just make me gain weight. During this time, I put on 15 pounds. I was miserable.

My doctor decided to up the Diamox to 2500 mgs. each day. But, now I was having horrible headaches. And they were throwing all kinds of narcotics at me....morphine, liquid morphine, toradol, vicodin, dilaudid, etc. I was working with a neurologist who knew very little about IIH. It would seem that they had overshot the therapeutic window and sent me straight into the gates of Hell. It couldn't get any worse. I made an executive decision....I stopped all the medications. I called back to the specialists office I had tried way back when, before I ended up in the hospital.....and I got in.

This brings us to today. I'm still not well, but I am better. I currently take a regimen of 100 mgs. of Wellbutrin every morning for depression. I take 20 mgs. of Lasix once each morning and once every afternoon with a Potassium supplement for therapeutic effect all day. And, I take 75 mgs. of Topamax every night at bedtime. I still have daily headaches, backaches, and neck aches but, the papilledema and "whooshing" is under control. I have had 5 lumbar punctures done in 5 months with opening pressures ranging from 250 to 330. Many days I am still on the couch with fatigue and pain and am still in the ER every couple of weeks or at the very least once a month. Dilaudid is still the only medication to take the edge off the pain. I still have insomnia and nausea. I'm not back to work and far from having a "normal" life. My doctors and I have a ways to go to figure out what will work for me to get me back to the living again. It is my goal to stay away from shunts for as long as I can.

Like many other patients with IIH it may take a very long time to find a regimen that will work to control my levels and still manage the side effects of the medications. Even once we reach a point where medications keep my pain and fluid levels down, eventually these medications will stop working. There may come a day when I have to have a shunt. I hope for a cure before that day comes. It is a relief to have a diagnosis...but, currently the diagnosis brings little relief.

In the meantime, I want to do what I can do. I want to bring awareness to this disease, in hopes that others like me won't have to wait so long for a diagnosis and will be treated with dignity and respect in hospitals and doctors offices across the nation. And, I want to help raise money for research. I can sit on the couch and whine about the limited amount of resources that are available to me to fight my pain and suffering, or I can help raise money to develop a cure. I choose the latter.

So...this is my story. It's a long one, and if you read all of it then I thank you for your commitment but mostly for your support. Please stay tuned to my post for updates on my progress. But, more importantly for updates on the progress of the development of our fundraising event next year. Hopefully, you will jump on board and help donate to the cause.

To make a donation go to: www.IHRFoundation.org

* Excerpts taken from Intracranial Hypertension Research Foundation


I don't like time. I tend to rebel against it. Clocks stress me out. If I had my way we wouldn't have a clock anywhere in the house, and alarms would be banned. People would just come and go as they please and somehow the world would be a better place for it....or at least a more relaxed one. Albeit, I have been disciplined by my parents, teachers and yet again, by many a boss for showing up late for dinner, class or work....but....I was in a better mood when I got there.

Last night, I finally fell asleep at 5:30 in the morning because I was up chatting with a friend on my IIH site, and, yes...creating this...my very first blog. I knew that this wasn't a good thing because I have a chronic illness....I need my sleep, and no doubt, I'll sleep until at least 12:30. My children and husband will be up way before that. And yet...I continue to rebel. I will not be a puppet to the clock! Why is that??? What is in my nature that makes me so angry about time constraints?

I recall living with my high school sweetheart in college. He had early classes and would always set his alarm for 6 a.m. every morning. Without fail, unconciously, in my sleep....each night I would turn his alarm off....and, without fail...each morning he would miss class. No need to tell you how that relationship ended.

Maybe this all stems back to an earlier more dysfunctional time. My mother would always stress about getting places on time. We always had to look perfect and she would rant and rave about the time. Fights would break out and objects would fly. Getting places on time was a rather tumultuous process. If you knew my mother today, you would see that it still is.

And, so it goes....

This is a work in progress. I realize that in order for an alien like me to fit into a world where clocks and meetings and "times" are necessary, I will have to conform. Without time, we would all live in a fit of chaos. This is why I labeled my blog as such...(using the "K" from my first name to be cute). It was my nickname in high school.

I am currently feeling a little stressed because I realize that my carefree timeless days of summer are about to come to an end. My girls are about to start school again. Alarm clocks will be ringing, buses will show up....on time....

.....I wonder how many times I'll be driving them into school this year????