Aaron's Ohtahara (www.ohtahara.org) was founded in 2010 by Daniel and Brianne McDonald. After their twin boys were born, one of them, Aaron, was diagnosed with Ohtahara Syndrome (OS). It was not an easy diagnosis to hear let alone find. After hearing OS as Aaron's diagnosis, Brianne found an online Yahoo! Email group and joined immediately. Her welcome was heartwarming during such a terrible period of time, she found so much support among the members. Outside of the email group, not a lot of information was to be found on OS. What was found was very dark and although that is one aspect of OS, Brianne and Dan felt there were many more aspects. That was the beginning of Aaron's Ohtahara. Today, Aaron's Ohtahara is reaching out to these families, offering support, guiding any new families to the online support groups if not already found, as well as offering care packages to the families. The objectives of our organization are to help support the families with children diagnosed with OS, to bring awareness throughout the world, and to help support and fund any research actively being completed regarding Ohtahara Syndrome. The following is an essay by co-founder Brianne McDonald on her experience with Aaron’s diagnosis of Ohtahara syndrome
Initially, Aaron seemed so healthy. He had a great color and a healthy cry, nothing abnormal. My husband was the first to notice that something was not normal. He described to me with a note of worry in his voice, a very rhythmic movement that Aaron was doing. These movements did not last long and when they did occur no one was in the room with us to witness. My husband began recording these movements on our cellphone and camera to show the doctors. We were told it was a newborn reflex, commonly called the startle reflex. Aaron's head, arms, and legs would come together, as if he were doing a full body crunch. This only lasted a minute or two in the beginning but as we would soon learn, it would progress to a much more serious matter. My lactation consultant was the first to see these movements. I told the nurse, "these are the movements we have been telling you about." My eyes are filled with tears again, this time with such a sadness and a worry for my son. Aaron is taken from me and brought to the NICU at the hospital where he is being observed for any more signs of this activity. At this time, we are told that Aaron is having seizures. About twenty minutes later, my husband, who had been with Aaron in the NICU, came back to my room, now with tears of his own. I can not see my husband cry, without tearing up myself. I am already wiping tears from my eyes when he tells me that Aaron had a seizure and stopped breathing. He describes for me the seizure and then the heroic actions of the nurse practitioner, who began lifesaving measures for Aaron. He tells me Aaron is breathing now on his own and his heart did not stop. As he is telling me, our nurse comes into the room. Such a sweet woman, with kind eyes, and a huge heart. My husband later tells me, that she was even crying. She sits down on the bed with me, pats my leg, tells me Aaron is okay, and asks me if I feel up to going to the NICU to see him. I can say, nothing would keep me away from that room, nothing. She gave me a hug as I slowly and painfully got up from the bed and into the wheelchair.
My first sight of Aaron is him laying in the bassinet, with an IV now placed in his arm, the size of a doll's arm, and a small hat on his tiny, two day old head. He looks calm, as he sleeps, so innocent and young. I hold his little hand with my fingers and talk to him. For the next week Aaron is in the NICU , placed on several medications, and has every test imaginable completed on him. Still through all this, we do not know what is wrong. Yes, Aaron is having seizures, but why and what has caused them remain a mystery. With discharge papers in hand, we leave for home, still wondering what will the future bring our little boy.
We were home for a few hours and Aaron began to seize again. From this point on, I began a journal, to relay to the doctors, the frequency and duration of Aaron's seizures. His seizures were now lasting five minutes or more and we had nothing to stop them. Not knowing what was wrong and continuing to see seizures, my husband and I decided we were not going to wait any longer for another test or another medication. I called the neurologist and explained in detail the amount of seizures that Aaron was having on a daily basis, at this moment, six to eight clusters of seizures a day, each lasting almost 10 minutes in duration. We were then told to come to Children's Hospital, where Aaron would be admitted and seen by more specialists. Although it was another admission to a hospital and more tests to be done, this was the path that lead to our answers.
We were admitted to Children's Hospital and within 24 hours we had our diagnosis, Ohtahara Syndrome. I was sitting on the bench, in the room, with my mom, Conor, and Aaron. Aaron's neurologist walked into the room and immediately I knew that this conversation would not be what I had hoped and prayed for. Our neurologist had a somber look to him, a look of one who is about to tell you some bad news. The resident could not meet my gaze. This time, I held the tears at bay, until I began to speak. With my paper and pen, I wrote down what the doctor said, what he described Ohtahara syndrome as, and the prognosis. I had to write everything down because I knew that without it, I would not be able to repeat it to my husband. Through a shield of tears, I wrote, Ohtahara syndrome, developmentally delayed, with severe retardation, and progressive seizures. Ohtahara syndrome is described as a very rare, progressive epileptic encephalopathy, involving intractable seizures with severe mental retardation. I could not take my eyes off of Aaron, laying in his hospital bed, still looking so peaceful and innocent. My mind ran a continuous course of what I could do and would do, to take this away from my child. My voice was so shaky and I could barely see through my tears when I asked if there was anything that could be done for him. We were given a small light in a dark tunnel. With further testing, Aaron could be a candidate for a surgery that could stop the seizures.
That light became our hope to grasp onto. Our neurologist referred us to a specialist at the Minnesota Epilepsy Center. In Minnesota, Aaron underwent more testing and met several other specialists. It was found that the left side of Aaron's brain had a malformation that was doing little if anything, to help Aaron function. My husband and I were told that Aaron was having 200-300 seizures per day and with the way he was progressing could have maybe two years to live. We were also told some of the best news we had heard in six weeks, Aaron was a candidate for surgery. The surgery involved removing a portion of Aaron's left side of his brain and disconnecting the left hemisphere from the right hemisphere. This surgery was not only the best option for Aaron, but my husband and I viewed it as our only option. The thought of not having Aaron with us, of not watching him grow up along side his twin brother Conor, was unacceptable. In this situation, we had to take a leap of faith and pray to God that this was our answer.
In preparation for surgery we were told some of the possible outcomes for Aaron. Aaron may not be able to use his right hand to grasp a toy or pen, Aaron may walk with a limp, and Aaron may still have seizures. Despite these warnings, our doctors had complete confidence in their work and reassured us to the best of their ability. Aaron's surgeon has such a way about her that shows confidence and a love for what she does. Her eyes light up when she holds either Aaron or Conor and to watch her with them is very calming. She exudes confidence. With all this in mind, the day of surgery was fast approaching.
I carried Aaron down the hallway, holding him so tightly and walking so carefully, as if I were in a race, holding a fragile egg on a small spoon. We were on our way to the operating room. Handing my child over to the arms of the waiting nurse was one of the most difficult things to do. I handed my son over to the nurse, but all my fears stayed with me. Surgery lasted eleven hours, but when our surgeon walked into the waiting room with tired eyes and a smile on her face, I knew it was worth it. That smile did more to reassure me than anything anyone could have said after the surgery.
We were told he may not grasp a toy with his right hand, now, almost a year after surgery, Aaron not only holds a toy, but moves the toy with his right hand. We were told he would have difficulty eating, he takes every meal with a smile and is now grabbing the spoon and eating table food. We were told there may still be some seizure activity after the surgery, to this day, two years later, we have not seen a single seizure. Aaron is now two years old and starting to take steps with assistance, he is pulling himself to kneeling and will stand up with assistance. The light in the dark tunnel, turned into a ray of sunshine. The name Aaron means mountain of strength. Through this little boy, I have grown so much and have found a strength that only God can give. I have learned that although I am not in control of everything, God is, and those seemingly large problems and complaints I had before, are not so large anymore. I am taking advantage of every moment with my children and every time I hear them laugh, I can not help but think it is my favorite sound in the entire world.
Aaron was also diagnosed with Hemimegalencephaly which manifested as Ohtahara Syndrome. This lead to Aaron being a surgery candidate. This is not the case for the majority of children diagnosed with OS. These children face seizures daily along with other medical problems such as respiratory issues. These children may not see seizure free days or ever experience walking, let alone crawling, however, they share the same strength. A strength that perseveres through every seizure, that shines through every bad day, and gives a smile that can bring you to tears. Having the honor of meeting a few of the children diagnosed with OS has been such a great joy. They have all touched our lives and we thank them for being such a wonderful light in this world.
