Sunday, October 21, 2007

Treatment of IS

Infantile Spasms are the nastiest seizures. They steal your child from you. I dreaded putting Grace to sleep because I knew that shortly after awakening, she would begin seizing. I would hold her as she had her cluster of seizures and hug her and talk her through them. Sometimes we were in public and if I held her close no one could tell that anything was wrong. We just happened to be scheduled for an MRI a few days after Grace began having IS seizures-we had made the appointment at our first neuro visit a couple of months earlier. We met the doctor prior to the MRI and he agreed to do a spinal tap on Grace while she was under anesthesia to check for a certain metabolic disorder we had read about recently. He was the most awesome doctor- he sat on the board at the children's hospital and we caught him right in the middle of a week of inspections and meetings. We were walking with him to the room where they would put Grace under anesthesia and we happened upon a pack of suited hospital big-wigs on their way to another meeting. They inquired of our doc if he was on his way to the meeting as well. He replied that no, he was being a doctor right now. I loved that. I held Grace as they gave her the sedation and in only a few seconds she was as limp as a noodle. She had the spinal tap and MRI and all went well. When we received the results, they were normal except that her brain was a bit small for her age. One thing that IS does is show that development in the brain has stopped and this is evidenced by the head stopping growing. During the couple of weeks Grace was having her seizures, her head circumference was measured several times and it wasn't until the seizures stopped that it grew at all. Pretty scary when you think about it.

Grace's spinal tap test came back normal as well as several other metabolic and genetic tests for abnormalities. It seemed as though we had a mystery as to what caused these seizures. IS very rarely occurs in typical children. Most often it is a secondary condition brought on by some other health or developmental issue. I knew that Grace had a developmental issue of some kind, but we were in the dark as to whether it was genetic, metabolic, or just something that happened in utero. I read a statistic in my researching that said that only 50% of children with developmental delay ever get a diagnosis as to what caused it. When visiting with a metabolic/genetic specialist once he told me that we have only scraped the top of the myriad of genetic and metabolic disorders out there. There are thousands more we haven't even identified yet. I was floored by that. To some parents of these mystery children it is a constant battle and search to find what their child has-many will not solve that mystery. Jumping forward in time to now when she is five, I am content with accepting Grace as she is and I am not burdened with the unsolved diagnosis issue. If I hear of other tests, we will get them. Until then, Grace is just Grace.

Grace began steroid shots ten days after she began having IS. The steroid of choice in this country at least is called ACTH. When I picked it up at the pharmacy, I looked at the receipt. I paid 40.00 for two vials-the price before insurance: more than 2500.00. That might have even been per vial. I can't remember now. I was just forwarded an email recently stating that the price of ACTH is going up to more than 5 times that amount. Thank heaven for insurance. Grace began her shots on a Monday. It was the first day of kindergarten for my oldest child McKay. We took her to school and I left to the hospital. McKay lost her first tooth while this was going on as well. I should have been enjoying these momentous firsts with her, but I was steeped in grief. The nurse at the neuros office gave Grace her first shot. I would be getting a home health care nurse to my home for either two or three more injections (again I can't remember which) to teach me how to administer them and then it would be up to me. Our insurance didn't cover any more than that. I was scared to death. I hate needles. The nurse taught me how to give an intramuscular injection in her leg. She taught me how to put some numbing cream on first for about 15 minutes per my request. I felt much better about poking her if her skin was numb. I actually did a pretty good job for an amateur, but one time (I hate to even share this) I accidentally went too far to an angle and the needle came out the side of her leg. I about died-and I never did that again.

By the time Grace got her second shot, her spasms had disappeared completely. One other thing I read in my research was that stopping IS within the first month was crucial to getting the best outcome. I was thrilled that I didn't have to see those nasty seizures anymore. Unfortunately, not all parents get these results from ACTH or even other treatments, and they have to have their child on the shots longer or try several things before the seizures stop. I felt so thankful that we didn't have to go through that. ACTH has terrible side effects. Long term use can lead to serious problems. The worst side effect to me was the fact that it causes one to withdraw. No eye contact, no smiles, just a moaning blob. I missed Grace while she was in the fog of ACTH. I missed her smile and her laugh. She wouldn't look me in the eye. She also gained weight in her face and got what is called "moon face" from the swelling caused by the steroid. She would sit in her car seat on the floor and moan all day. I hated it. One time, we were having dinner at Dallas' cousin's house with a lot of family. She was on the floor in her car seat moaning away. Someone said something funny and we all laughed. Suddenly, Grace laughed too. Just a sweet giggle. Now that she is older, she does that a lot. She likes the sound of laughter. We were amazed to hear it that day though, and it seemed like a sign that our girl was on her way back.

Our neuro gave us great news after a follow-up eeg. We had just gotten the best case scenario for IS. Her eeg showed no more hyppsarythmia and her head began to grow again. We were so relieved. We were now able to wean her off of the shots. The day of her last shot I felt like celebrating. She stayed on topomax for a little while longer, but we were soon able to take her off of that as well.

Slowly, Grace began to come back to us. Her smiles were so welcome. This was a nightmare I will never forget and 2003 was one of the worst years of my life. By the time December came, I was ready for a new beginning and a road to healing and acceptance.

Next time....the years of being stagnant.

Wednesday, October 10, 2007

About Grace....the beginning

On Oct. 1, 2002, Grace was born. I was induced at 38 1/2 wks and being that she was our third child, I felt so prepared and knowledgable. I only had a couple of hours after the pitocin was started until she was born. She was as perfect and as sweet as can be. She nursed well, slept well, and was a good natured baby. I remember enjoying every moment of my 2 days in the hospital with her. We brought her home and she did very well for the first few months. She smiled at 6 weeks, and even laughed for the first time @ two months. I remember thinking that she was very advanced for doing that! She pushed up on her arms on time and was very aware and engaged. Because of this, however, I failed to notice the things she didn't do on time. I threw away the month-by-month developmental guide they give you at the hospital because I figured I knew it all by now.

At her four month appt., she passed her exam quite well. Now though, when I look back, I realize that she was already showing her delays by not rolling over or grasping at objects. At her 6 month check-up, it suddenly dawned on me during the exam that she was not even close to sitting up unassisted yet. The doctor didn't seem worried though, and told me just to take her home and work on it with her. I began trying to get her to grasp and hold objects and to work on her sitting. She kept her hands up in fists by her shoulders and arched her back while stiffening out straight-making it almost impossible to help her to sit.

One day when Grace was 7 1/2 months old, I got a phone call from a friend from church. We talked about some other things and then almost in passing, she asked me if Grace was sitting up yet. I told her that I was working with her on it. She said that she didn't want to worry me, but she had noticed in church that Grace was clenching her fists up high and acting stiff. She suggested that I get Grace into an early intervention therapy program. She worked in early intervention with kids with hearing impairments and she gave me a phone number to call. She said that we could get therapy to help Grace catch up. Then I noticed a hint of something behind the casual suggestion and I asked her point blank what she thought might be wrong with Grace. What she said next changed our lives forever. She said that she didn't want to worry me, but with the stiffness she was displaying she could have mild cerebral palsy. She said that she might only need special shoes or some therapy for a while until she catches up.

I really took the news quite well initially. My mother in law commented later that when I told her I seemed so matter of fact about it and without any hint of emotion. I think I was in shock. Suddenly, one day within a week or two, I just lost it. My good friend from high school called me at that very moment. I remember sobbing into the phone: "We think that Grace might have cerebral palsy!". My husband and one of our friends from church gave Grace a priesthood blessing. A few days later at church our friend commented to me that he felt very peaceful that we would be ok while he was giving Grace her blessing with Dallas. I recall feeling a sense of peace also. I received comfort from reading the scriptures as well. As I thumbed through them, I found passage after passage about peace and being thankful. I realized that we were in for a journey, and that we needed to count our blessings along the way in order to receive the peace we sought. This has proven true again and again.

We got Grace into early intervention therapy, and I took Grace to see our family practice doctor again. I began explaining to him what had happened over the past two months and just broke down again. He is a wonderful doctor and calmly referred me to an excellent pediatric neurologist at the local childrens' hospital. I remember wanting to go to this neuro and have him reassure me that everything would be ok. Dallas and I both went to the appt., and after a brief exam, he told us that he thought on a scale of 1 to 10, Grace was probably a 2 or 3 and that she would probably be able to catch up with the proper therapy. We left that appt. on cloud nine. We went out to celebrate on the way home at our favorite restaurant.

I began taking Grace to a friend who was a chiropractor. He heard our story and thought that regular adjustments would help her. I remember at this time, the thing that bothered me the most is that she would tip over even when sitting in her stroller or highchair. It was so depressing to me that my 8 month old needed to be propped up. I had hope at this time though, and was looking forward to getting Grace caught up. Then one night in August of 2003, everything changed dramatically again. This time it was much worse.

We had gone out to dinner with just Grace one Friday night. She was so sweet sitting there in her carseat while we ate, and I felt so happy and hopeful that evening. We got our kids into bed and went to sleep. When Grace awoke in the middle of the night, I picked her up and began to nurse her back to sleep. Suddenly, she began making movements as if she were startling every minute or two. She would put out her arms in front of her and jump as if someone had slammed a door. I suddenly remembered something the neuro had said to us in his office: sometimes startling can be a seizure. At that moment I knew that she was having seizures. I really wasn't terribly worried, I figured I would call the doc on Monday and that we would get them taken care of.

She only had the seizures right after she woke up and for only a few minutes. They weren't even violent or strong. I remember calling my chiro friend to ask him what he knew about seizures and he just said that adjustments do help to stop seizures in many cases. I called the nurse at the neuros office on Monday at 8 am and left a message. I got a return call rather quickly. She asked for a more detailed description of what was happening, and said she would ask the doc and call me back. Within another short period the doctor himself called me. He asked for another detailed description. When I finished I will never forget what he said. He simply said "Oh nuts" and I knew from the tone in his voice that it wasn't what he wanted to hear.

He informed me that it sounded like Grace was having a type of seizure called infantile spasms. He needed to do and eeg to confirm what he suspected. At first he could only get me in later in the week, but he sounded worried and called back with an appt. on Wednesday. Dallas and I took Grace in for the eeg. It was so sad to see her head all wrapped up with those wires coming out the top of the bandage. It was an hour for the eeg and we were taken directly in to see the doctor as soon as we were finished. Little did I know that that rarely happens-especially on such short notice. She didn't have any seizures during the eeg, but while they were taking her vitals in the nurses station after the procedure, she began having a cluster. I told the nurse to call the doc so he could see them. He came running and when he saw them, he just nodded and said that they were most definitely seizures. When we met with him in the office, he confirmed his suspicion of infantile spasms by showing us a particular wave pattern on her eeg called a hyppsarythmia (sp?). Hers was faint, and not as noticable as other kids he had seen with IS, but it was definitely there.

He told us that the gold standard for treating IS was a steroid called ACTH. It had to be special ordered and administered by intramuscular injection. He also started her on a sprinkle seizure med called topomax. We had to wait until Monday for the ACTH to be in and picked up so we could start it. It was so somber in that room. The doctor said that his hopes for her future had suddenly become much more bleak. He told us that he unfortunately could not tell us what to expect for her future, he just didn't have a crystal ball. I cried while holding her and saying-"but she's just so smart"...over and over. The nurse very quietly told us that she was sorry and that this type of seizure is the worst kind to have. I remember coming back to my in laws house where our older two were and breaking the news.

Our doc told us to go on the internet and read about IS. That was both the best and the worst idea. The best because I learned what we were really up against, but the worst because it was the most depressing internet search of my life. Sunday was my birthday. It was also the most depressing birthday of my life. I got up the courage to search on the internet that morning. When I read that IS has a very poor outcome and that 75-80% of children who get it end up moderately to severely mentally retarded, I began shaking and crying. I just wept and wept. I also learned that if the seizures are not stopped within the first month, that you can almost guarantee the worst outcome. Basically, these seizures are a sign that development in the brain has completely stopped. I couldn't believe that this was happening to us. We went over to my husband's parents for a birthday dinner and presents. I couldn't even eat a piece of cake and barely touched my dinner. Seriously, it was the saddest birthday ever.


I will continue her story next time. My fingers and eyes are tired. This is a story that may take a few posts to tell. Next time....treatment of IS.

Sunday, October 7, 2007

About our family

I thought I would take this post to tell you about our family. Next time I will tell the story of our journey with Grace so far.

Dallas and I were married in 1994. We met at church shortly after we both returned from serving volunteer missions for our church. He was in Australia for two years and I was on the other side of the world in Finland for a year and a half. We both arrived home early in 1993. Dal's family had just been transplanted here from northern Virginia. His mother worked for Mobile Oil and had a job transfer here to Dallas, Texas. Dallas and I met in a singles congregation-both very new back to life after a mission. I was preparing to go to Utah and live with my sister and attend the University of Utah. So much for that idea. I met Dallas and knew that I wouldn't be leaving after all.
We dated for a year and got married in the Dallas Temple. A few days after returning from our honeymoon, we packed up his dad's truck and moved to Austin so Dallas could finish up his bachelor's degree at the University of Texas (hook 'em horns!). We lived there for a year and a half. I worked for NationsBank and Dallas went to school full time. We were the typical young married couple: complete with cheap furniture, trips home to mooch off our parents, and scraping change together to go to Taco Bell for cheap food. We made great friends down in Austin and fell in love with the city and the university.

In December of 1995, Dallas graduated with his degree in Political Science. A couple of weeks before that, my father died of a heart attack at the age of 73-the day after Thanksgiving. My parents are truly amazing people and I plan to write a book about them soon. My mother and father raised 6 children on their own--not so amazing you say, but when you find out that they were both blind, it does become quite amazing. I was the youngest of the 6. My father was a great man. I only wish Dallas could have known him better. My mother still lives with one of my brothers and his family here in the Dallas/Fort Worth area and we visit her weekly. She is about to be 80 next week.

After graduation, Dallas and I moved back to DFW and lived with his parents for a year. Dallas got a job at an investment company here locally thanks to the contacts of some friends. We decided to begin trying to have a child. In August of 1996 I found out that I was pregnant. We were so excited and began telling everyone our great news. At my 12 week appt., I was having a sonogram when the doctor began to look worried. He could not find a heartbeat. Apparently at about 101/2 weeks the fetus had stopped growing and the heart had stopped. We had the fetus tested for genetic abnormalities, but there were none. She was a normal little girl. We do not know why the pregnancy terminated, but miscarriage is fairly common. This was a difficult time. I had to be scheduled for a D&C. I felt so devastated. We had to un-tell all of the people we had told- not a fun task. After that, we vowed never to break the news until I was safely into my second trimester.

I was still working for NationsBank, but the miscarriage prompted me to want to go part time. I took a position closer to home and with less hours. Within the first couple of months of 1997 I was pregnant again. McKay was born in the fall of 1997. She was a fun, roly poly little girl, with lots of energy. She was a fairly easy baby with only a brief brush with jaundice and mild colic.
We decided to move out of the apartment we had moved into in the fall of 96 and build a home. We moved into a little 3 bedroom house that we rented from Dal's parents when McKay was almost 1 year old. I began getting baby hungry again, and we found out we were pregnant again in the spring of 1999. Alexa was born the day before Thanksgiving that same year. She was what we called "our little peanut" weighing in at 6lbs 8 oz. A little ball of fire with endless energy. Both of our girls were right on time with their milestones, and even a little early with the talking and cognitive ones. I was just like any other mom, comparing my girls to other peoples' kids and patting myself on the back for all of their great accomplishments. Life was carefree and simple. I had no idea what our third child would bring.

When Alexa was almost 2 and McKay was almost 4, we moved into a new four bedroom home that we built a little closer to Dal's work. We qualified for a relocation package and shortened Dal's commute from almost an hour to about 10 minutes. Again, I began getting that baby itch.....



Saturday, October 6, 2007

First Post, Short Post

I did it! I finally have a blog! WOO HOO!