Paul's Story: Parent's Letter Project Neurological conditions- Paul's dad

My son Paul was born with a rare neurological disorder called Septo-Optic-Displasia (SOD). Although he appeared healthy and normal at birth, it became apparent at the age of 3 months that something was not right. He was not tracking visually and so we took him to our pediatrician who then ordered some tests, including an MRI. The results of the MRI showed SOD. We had never even heard of this disorder; much less knew what it was. We learned that SOD is a mid-line brain disorder that has many associated problems, some severe, and some not so problematic. This disorder is a result of the brain not completely forming at a certain time in gestation, usually in the first five to 10 weeks and has no known cause. The most serious problems with SOD are blindness, mental retardation, growth and endocrine disorders (hypo/hyper-pituitaryism, diabetes insipidus, etc.). Paul was very lucky in that he seemed to have only a variant of this very serious disorder. His vision improved, or developed tremendously in the next 3 months or so, he was growing appropriately and we felt very lucky that he seemed to be doing pretty well, despite this serious diagnosis.

All was going just fine until a week after his first birthday when we awoke one morning to find him covered in vomit, unresponsive, and obviously having a Grand Mal (Status Epilepticus) seizure. He was rushed to the hospital and we were told that if he had another one, he would be prescribed medication for seizures. Well, he did have another one about a month later and was as severe as the first, so he was put on anti-epileptic medication (AEDs). This was the beginning of a very long and extremely dark nightmare of seizures, medications and a host of associated behavioral problems.

This was to be the pattern for the next 11 years… severe seizures, many medications, and many hospital visits. Paul has been rushed to the emergency room 43 times for Status Epilepticus, some from home, some from school, daycare, and relative’s homes and from out-of-state vacations, etc. Some of these episodes would go on for over two hours at a time and would not stop until he was put under intravenous “loading.” All this time, Paul has been taking many different kinds of AEDs, all with little or no effect whatsoever. In fact, the seizures only seemed to get worse. Paul has had close to 100 of these severe seizures through the years.

Paul has been seen by just about every specialist you could imagine, neurologists, neuro-opthalmologists, gastrointestinal doctors, ENTs, allergists, endocrinologists…too many to name. In 2004 we consulted a Baltimore hospital hoping to get a second opinion or another approach to his treatment. We consulted with a well known surgeon who looked at Paul's films and said that he was not a candidate for surgery, period. We even looked into and considered the Ketogenic Diet, but were told that it probably wouldn't help Paul and to just continue with medications. Later in 2005 we took him back to the Baltimore hospital to consult with the Pediatric Diagnostic Clinic, and after reviewing Paul's charts again, they just told us to learn to accept his disorder, and to go home and just love him. We already did that.

The toll that this situation took on us was very difficult and traumatic. Our marriage disintegrated before our eyes, nothing seemed to help and we started arguing about the best way to treat Paul. We tried therapists, counselors, for us as well as for Paul himself. He was becoming more and more depressed and his self-esteem was at an alarmingly low level. To make matters worse, most of Paul's seizures were during sleep (nocturnal seizures) and it was nearly impossible to sleep every night worrying about the next seizure, and eventually becoming so sleep deprived that every facet of life was affected.

Even his doctors said that they did not know what else to do besides prescribe medications that even they admitted were doing no good. This was very frustrating for me, watching my son suffer from a severe seizure disorder and on top of that, given handfuls of drugs that made it impossible for him to function. I felt as if Paul's childhood was stolen from him and I was getting very distraught with anger, depression, bitterness, and hopelessness. I was not at all satisfied with the direction his doctors were taking with Paul's treatment and this led to several confrontations with them.

So, after 11 years of this horrible nightmare, it was an incredible shock for me to hear that Paul's mom was considering surgery for Paul. I was not present at most of the neurologists visits in the last couple of years, mainly because I had become so unhappy with the way things were going and did not want to continue seeing this neurologist, but had no choice in the matter, so I thought it best not to go and cause friction. Evidently Paul's neurologist had suggested to Paul's mom that we possibly visit Children's National in Washington, DC, for a surgery consultation because they had been making significant progress in epilepsy surgery and maybe it would be worth looking into.

I would be the first one to say that I was definitely not thinking that this would be a possibility, or even could be a possibility. Especially after what we were told by the other hospital years earlier. I thought again, that they were just grasping for straws and had no other options or ideas and this was just going to be a way to appease the parents, to give them some false hope or something… I was very skeptical. For one thing, I never thought that the surgery itself would stop Paul's seizures. I was convinced that his seizures were more hormonally driven and that the seizures would just happen in another part of his brain, had they decided to remove any brain tissue. I also never thought that Paul could handle the surgery, physically as well as emotionally. I read quite a bit about this type of surgery and knew that it was a very difficult procedure for anyone, much less a kid with all the issues as Paul… turns out I was very wrong!

When we first met the surgical team at Children's National, I was immediately impressed with their confidence and knowledge of epilepsy physiology. Amanda Yaun, MD, was the neurosurgeon and William Gaillard, MD, was the team neurologist. Both are fairly young doctors and seemed easy to talk to. I'll never forget the first day we checked in for Paul's two week neurosurgical assessment at Children's National. Dr. Yaun and Dr. Gaillard were reviewing an MRI film and I couldn't help noticing that it was Paul's MRI. I told them that I was Paul's father. They immediately turned around, put me at ease and invited me in on their conversation. I was very grateful that they included me and didn't try to dumb down for my sake. That meant a great deal to me and it made me trust them more. It was then and there that they showed me an area in Paul's right temporal lobe that was abnormal appearing, a definite area of suspicion. They explained to me in detail what this area should look like and that this could be the reason for all of his seizures! This was the first time anything was ever said about an area of abnormality and they were showing me right there on the film where it was. This was to be the target area for the next step in the surgery process…placement of the EEG grid.

This first step was probably the most difficult part of the whole surgery process. It was an eight hour surgery that involved a craniotomy (removal of a portion of the skull). This was a fairly large area and I was obviously very worried about the possible complications of this surgery. Paul did well for the surgery, but looked awful afterwards. He was heavily medicated with morphine for pain and was badly swollen and had a complete head cast with wire leads sticking out of his skull. They didn't wait too long to get him connected to the EEG monitor, it was actually the next day.

Being connected to the EEG monitor was very hard for Paul, it was not a very good design, as far as being able to move around and he was pretty much confined to the bed for the next 11 days. There were constant problems with the connections, IV lines, blood pressure monitor, heart monitor leads, it was very hard for Paul to sleep due to the alarms constantly going off because of a bad connection, etc. He did not do very well at all for the first five to six days, very depressed and despondent. He kept looking at us like “what in the heck did you do to me!” He did not understand why we had to do all of this. I think if it were up to him, he would have been fine continuing on the same path, as opposed to having surgery. But, slowly he started feeling better and was even laughing a little bit when we were playing with some of the gifts he got for his birthday (yes, he spent his birthday in the hospital, and Thanksgiving).

All the time Paul was connected to the EEG monitor, he was being recorded for seizure activity and at first, there wasn't much activity at all. Even when they completely withdrew all of his medications, he did not have any significant seizures, which was what they needed to analyze the data for the area in question. Eventually he did start having some, but in my opinion, it didn't seem like it was enough or not as well defined as you might think it would have to be to make a decision on what area was the problem area, but they decided after 11 days that they thought they had enough data to make a decision and the next surgery was planned for the day after Thanksgiving.

The one bright spot in the last couple of weeks was that they thought that Paul's head dressing could come off on Thanksgiving Day and that made Paul very happy to get that thing off. It had been falling apart and was hanging in his face for weeks. He still had the wire leads sticking out of his skull, but at least he could get that smelly, nasty thing off his head. Also his mom's family had all gotten together and came down to the hospital for a couple hours to celebrate Thanksgiving, even though there was a strict no-visit policy because of the H1N1 flu going on at that time. This picked up his spirits quite a bit, but he was getting very worried about the next surgery. For the last week or so, the doctors came in every day discussing the “next surgery” and Paul was not happy at all with the thought of going through that again. He knew what the first one was like and he was extremely depressed about the next one.

So on the morning of November 27, the nursing team came in bright and early, about 6:00 am and Paul just cried. He did not want to go and it was very hard for me to keep it together, but I knew that I had to encourage him as much as possible and not show my fear. It was another hour or so before the actual surgery, so he calmed down quite a bit and it seemed like he just got to the point where he accepted this was what he had to do and he quietly laid back while they wheeled him back to surgery. I cried to myself, he was so darn brave and trusting. Of course I was scared to death. They kept telling us that the second surgery was easier than the first and I just could not believe that…. but they were right. He did much better with this one, even though it was a nine hour surgery, they took a portion of his right temporal lobe out and after all the trauma of the first surgery…I expected him to be out of it for at least a day, but he came right out of surgery asking questions, asking for something to drink, asking for his mom and dad. I couldn't believe how well he did for this one. He looked very good immediately after the surgery, but after a day or two, he really started to swell badly and he looked very rough.

The doctors thought Paul did outstanding for this last surgery and they thought he could go home just two days later. I was very nervous about going home so soon but it was the best thing they could have done. Paul was really anxious to get out of there, as you could imagine. The doctors did explain to us what they had removed from Paul's brain and said it was an area of malformation that was grayish in color and a hard rubber-like texture and was obviously not doing him any good. They said it was there from the beginning and was probably the reason for this awful nightmare. I believe Dr. Yaun said that this was the hippocampus area of the brain and she removed it completely, along with a smaller area of adjoining tissue.

It's been 66 days now since his last surgery and he hasn't had a seizure since then! For the first couple of weeks I was very hopeful but quiet, just waiting. I still can't believe it! After so many years of hopelessness and despair, and to all of the sudden have this opportunity come out of nowhere is an incredible miracle! Each day that passes gets me more and more excited; this is surely an answer to our many prayers.

I can't say enough about Children's National and especially Dr. Yaun. Everything that she said was true. Her confidence and boldness was inspiring and I felt like Paul was in good hands the entire time he was under her care. After so many years of being told there was nothing more they could do, and then to have Dr. Yaun come out of nowhere saying that she could make him better, and then actually do it, has left me stunned and changed forever. I never believed in miracles, that this could never happen to us…well I do now!

It's still too early to say that Paul has been cured of seizures, but it's obvious that he's much better now and there doesn't seem to be any loss of any neurological functions that he previously had, which is what I was very worried about.

Life was very dark during those 11 years of seizures and the sense of despair and hopelessness was so deep, I never thought life could change for the better, much less so fast!

I wish that we had read a story like this, because there was no real support for families going through this kind of turmoil… friends and families empathize, but don't really understand. My hope is that maybe someone in a similar situation may read this and realize that there is hope, that there is someone out there who can help, no matter how much it seems that there isn't. I would also hope that if there were any doubt over what to do when faced with an option as drastic as brain surgery, that someone would consider this type of operation and not be so skeptical. Modern medicine has made some incredible advances just in the last couple of years. We are perfect examples of what can happen when you trust in talented people, when you have faith in a higher power and to not give up, no matter how hopeless things seem.

Paul has become my hero for all his strength and bravery. His attitude during all of this was nothing short of inspiring and I want the whole world to know what he's been through. He still has many challenges to deal with and I believe that he will rise above all the hard times to become a productive, independent, and happy young man.

Sincerely,

Paul's dad

Treatment Team

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