Mattias is a truly happy 3 ½ year old boy and our only child. He has an infectious smile and a passion for life. Trains, cars and cats are some of the many things that interest him.
September 22nd, 2012 Mattias was taken to the emergency room at the Children’s hospital of Philadelphia (CHOP) with a fever. The ER doctors insisted on a CT scan. As a result, he was diagnosed with a 4 cm HH at 8 weeks of age. He had a “big episode” every 3 to 5 days, which lasted 7 to 20 hrs. Between big episodes he had few seizures the first couple days and then gradually built up to 20 to 100 seizures a day until he reached his “big episode.” We were thankful for the staff at Children’s hospital of Philadelphia for diagnosing him, thoroughly, as fast as they did. Time was on his side, but like so many other parents, the questions about what to do and what could be expected were unclear. Those cluster seizures miraculously went down from approximately 100-300 a day after 4 months, which gave us a little break.
The fine line between providing constructive treatment while at the same time causing as little damage as possible was the guiding force for Mattias’s eventual treatment. There is no correct answer to this as we learned how unique every HH patient is. The first step, however, was getting control of something that could possibly be managed, his endocrine issue. That remains a work in progress but Mattias is doing well. That wasn’t to say his seizures were not a priority. It was at this time we learned of Dr. Daniel Curry and Dr. Angus Wilfong’s MRI-Guided Laser Ablation Surgery at Texas Childrens Hospital in Houston Texas. This was an intriguing option but not one that was unanimous agreed up by different medical facilities. Still, the feeling of anxiety began to go away as we learned about more and more options.
It was in December of 2012 when we began traveling to different medical locations –BNI , Dr. Rekate in Long Island, and then TCH. We were overwhelmed with making the decision for either an open procedure or the newer laser ablation. At that time TCH had no experience with operating on a patient as young as Mattias (7 Months) or a lesion as large. BNI and Dr. Rekate recommended an open procedure. BNI specifically recommended the open procedure over the laser method because the technology was still new and they weren’t confident Mattias’s lesion could be effectively treated due, again, to the size. In the meantime, Wanshu contacted the HH medical center in Japan. We learned that they had over 15 years of experience specializing in MRI-guided radiofrequency thermocoagulation treatment. Dr. Kameyama at Nishi-Niigata Chuo National Hospital wrote us several comprehensive e-mails. Dr. Kameyama strongly suggested that we opt away from the open procedure(s) and supported us in believing that giant lesions in the range of 5 cm to 8 cm could be treated with a non-invasive procedure. Two of his patients are seizure free today. He believes less invasive methods actually have a chance to stop seizures for the giant HH patients.
Rather than go to Japan, we opted to stay in country to at least begin Mattias’s medical journey. The staff at TCH was incredible and they performed 3 surgeries with 5 burr holes from February 2014 through April of 2015 at Texas Children Hospital. As caring and professional as the staff was, the seizures came back soon after Mattias began to wean off the steroids. By June 2015 we learned that there was little matter left that could be ablated. The staff at TCH recommended an endoscopic approach to reach an area on the right side of the brain that would have been too dangerous with the heat of the laser. We had full confidence in the recommendation of the TCH staff but we wanted to revisit Dr. Kameyama. We didn’t expect much.
Wanshu wrote Dr. Kameyama again after three years and mailed him Mattias MRI. As before, he was generous enough to share his time and thoughts. Wanshu also made contact with the HH Russian community online, most of who were operated in Japan. It cannot be stressed enough that every HH lesion is different. However, to our shock, Dr. Kameyama and Dr. Hiroshi Shirozu felt Mattias was an excellent candidate for the SRT treatment, even after three laser ablations. This was both wonderful and frightening news to here. To secure the spot for the surgery required payment in full month in advance as well as a full month stay in the hospital for the surgery. Our thoughts ranged from hope, to “will this actually help?” to “are we making a mistake?” The Japanese staff was very respectful and patient; they took time to learn more. We also talked with the Russian families who had experience with the procedure. In the end it was up to us. We made our arrangements and jumped forward. Again, it was another hard decision because not everyone agreed with the idea to travel so far away for the operation that wasn’t guaranteed to help.
We started our journey in Japan at November 2nd. What we found in the Japanese was a completely unique experience compared to what we went through the past couple years. Not better, not worse. We were comfortable in the hospital and there was no added stress by those who cared for our three years old HH child. Mattias had tests almost every day the two weeks prior to the surgery (11/17/15). The ratio of nurses to patients was excellent. Mattias’s main neurosurgeon was Dr. Shirozu. He is a talented and disciplined Doctor with warm and gentle heart. He personally did most tests for Mattias, which is different from USA health care system. They also offered a special teacher who often set up playground for Mattias and other children over there. The day before the surgery we had a rehearsal with the teacher and she tried to arrange a less stressful environment to send Mattias into operation room.
On the financial side, our sacrifice included a one month stay with three beds and three meals a day for all three of us. Wanshu was able to relax not worrying about Mattias nutrition and caloric intake because they offered three delicious and balanced meals for Mattias and us. Mattias’s most happy time was standing outside the room and watching the meal car driving down the hall stopping at every room on the way to us. There was an open comfortable living room area where all the patients conjured. Then there was the surgery.
This hospital didn’t use the high doses of steroids, nor for the extended length of time, which we were accustomed to in prior surgeries. This did seem to help with Mattias’s behavior, pre and post-surgery as well as less weight gain. He was a little wobbly the day after the surgery and he slept his fair share, but he recovered just fine. He had about 5 seizures over the next two weeks and they only lasted approximately one to two seconds. He’s gone a stretch of days without a seizure, and a stretch where he has one a day. We understand that we have to wait at least 3 months up to one year to conclude his seizure condition. We are happy with the current result because we didn’t bring back his seizure medications after surgery. Finally, he is able to sleep well every night. Mattias didn’t experience major side effects. He had a low fever a couple days and low sodium. His sodium was resolved in three days by adding salt into his meals.
Horner syndrome was another, minor, effect that resulted from the surgery. It takes time to resolve but it doesn’t affect his daily life. The big advantage for this hospital is that we are able to see a neurosurgeon at least once a day. During the two-week evaluation we have time to know the neurosurgeon and build a trust and good relationship. Therefore, when the surgery date came we felt comfortable to hand Mattias hand to him. Mattias was their 150th patient. They do have experience for many HH shapes, locations and sizes. Hence, they had enough data to provide an accurate probability of side effect and complications that Mattias might experience. We felt everything was under control.
What we didn’t expect was meeting 5 HH families over there, three from Russia, and one from Korea and one from Japan. We were pleased that we were able to share our experience and get courage from each other. We’re as prepared as we can be for the long haul with Mattias and we know things can change. For now, we’re happy to say he’s recovered well from the surgery and better for having gone through it as he’s showing noticeable improvements both with the Gelastic seizures and cognitive development.
The big challenge in our friends and family’s mind has been “how did we deal with the language and culture issue?” Most of the staff and some of the doctors don’t speak English. Dr. Shirozu speaks English well, so he is the main person we communicated with medically. There is a full time Russian translator. She works there for all Russian family but she speaks English as well. She helped us in translating daily tasks and explained the policies of the hospital. Often, we used a translation App on our IPad and IPhone to communicate with the staff, nurses and even family over there. Most staff have a lot of patience to communicate with us. It didn’t hurt that Wanshu’s native language is Chinese, so she shared the written text with the Japanese. Also, she understands Japanese culture enough to be comfortable for us to make the decision.
We were very thankful for the warm and supportive emails from Lisa Soeby, Roza Wu, and other Hope for HH members. It was never easy to make such a big decision for your loved one. We want to say Dr. Curry and Dr. Wilfong are awesome doctors and a good choice for HH treatment. Most of us are able to get a thorough account of an experience at TCH. We spent most of our time with this introduction to describe our experience in Japan because we were the first American family to have an operation there, and it was a very positive experience. Also, we would like to reiterate that Japan is a viable option for SOME patients with HH. They have their limitations like any other procedure. They are a competent and courteous staff that is highly skilled in a very delicate procedure. With so few options and whole planet with only sprinkles of people enduring this condition, we hope what we’ve done is give families another option to… Hope