At a recent visit to Ezra's neurologist, we ran into one of the neurology fellows that had been quite involved in one of Ezra's first hospitalizations on an epilepsy unit last summer. So many emotions suddenly hit me when I saw her. As you could imagine, it was bittersweet to see her. She is physician that you could only hope to help take care of your child. She is sweet, very kind, caring, compassionate, has a soft spoken voice, and had words of comfort and support during a bitter, hard and fearful time...
When you start researching "Infantile Spasms"or "West Syndrome", as it's also known by, you'll come across some worrisome and frightening words such as: extremely rare, very serious, catastrophic...
This is what Ezra was diagnosed with last summer, in June 2018.
One morning in early May 2018, I had put Ezra into his feeding chair for breakfast. As I was feeding him, I noticed a subtle quick nod his head. Now, as a Mom of a special needs child, I admit I have hypervigilance about everything when it comes to Ezra. Anything strange or unusual I will pause, watch him, and make a mental note. Because he's non-verbal I try to be as in-tuned to him as much as I can. He can't tell me what he's feeling or how he's feeling so I try my best to read his body language cues and decode his sounds.
Over the next week I started noticing this subtle head nod happening a little more often when he was in his feeding chair, ready to eat. The week after that, this movement was starting to be more obvious, more pronounced. This head bob would occur suddenly, without warning. His head would drop down towards his chest as if it was too heavy for him to hold up anymore. Ezra has macrocephaly (large head), but he had never done this type of movement before. I started noticing this movement occurring sometimes in the morning, sometimes in the evening. I began wondering if maybe he was developing a new kind of seizure. One day, I put Ezra down on the ground to play. He had just finally started sitting up on his own unassisted, a month prior, in April 2018. That particular day, Ezra just got up into his sitting position when I saw his head drop downwards and he fell forwards onto his head. At first I thought he lost his balance as he seemed to be shifting his weight. He had been trying to get into all-fours during that time.
By the end of May, this sudden head dropping was becoming more frequent, and seeming a little more aggressive in its nature. When Ezra would be sitting up on the floor his head seemed to drop and catapult downwards towards the floor. On occasion, you would hear a thud when his head hit the floor. We started putting sofa cushions around him to soften his fall. There were other behaviors, or lack thereof, that we noticed too in early June 2018. Ezra seemed to not want to sit up very much anymore, and would just lay there not really doing anything. He stopped engaging in the few activities that he enjoyed. His participation during therapies also declined. Ezra was scheduled for an ambulatory EEG (an EEG that you go home on for a designated time) mid-June with a follow up to the neurologist at the end of that month. All the while, I was making notes of these head bobs, and different behaviors. I was eager to address them with Ezra's neurologist.
Five days after Ezra's 24 hour ambulatory EEG, we got an urgent call for us to come in to see his neurologist the next day. I called Eric at work and told him he needed to get the day off and explained the urgency to him. At the appointment, before I could even inform Ezra's neurologist about the strange head bobs I had been noticing, I was asked if I saw any jerking movements, like head bobs, his body lurching forward, or backwards... I remember feeling a heaviness in my stomach. I told him yes I did, and started explaining the movements and telling him that I had planned to tell him about them at our appointment... Ezra's neurologist started talking about the EEG findings. He said the entire EEG showed a brain wave pattern called Hypsarrhythmia. He explained that the pattern looks chaotic and unorganized. He had even printed out a page of Ezra's ambulatory EEG and an EEG from three months prior to show us the difference. When they see hypsarrythmia, they immediately are concerned about "Infantile Spasms" or "West Syndrome". Which caught late or left untreated, can cause permanent brain damage... As an Registered Nurse, I am aware of how physicians present a delicate situation. The next words out of our son's neurologist's mouth hit me hard. It bought me back to the first time a medical specialist brought forth concerns about Ezra's development. Ezra's neurologist told us that he wanted to admit him immediately to the Epilepsy Unit to "confirm" the diagnosis of West Syndrome. Not rule out. Confirm.
 |
| Ezra hooked up for his 24 hour ambulatory EEG. |
 |
| Ready for a long night. |
Ezra was admitted to the hospital and was quickly hooked up to an EEG with video monitoring. After three very difficult and emotional days, the diagnosis was confirmed. Ezra had West Syndrome. Ezra was immediately started on ACTH therapy (Adrenocorticotropic hormone) while we he was in the hospital. He would not be discharged home unless the hospital had confirmation that his at home medication was received at our home. Eric's parents had been thankfully visiting during this time and his Dad, literally set up camp waiting for the UPS delivery of ACTH. It was that big of a deal. This treatment was going to be intense I was told. Ezra's neurologist and his team explained to me that it would be a very, very hard time. The side effects of high dose steroids were going to be brutal. Those were their words. I was familiar with steroids and had an understanding of the side effects, but never in this capacity. I tried to put my nurse hat on and be "ready", as they did assure me that Ezra had a high chance of overcoming West Syndrome because we caught it early. I started to mentally prepare myself for the next several weeks. But they were right. It was one of the worst times of my family's life. I can't even begin to imagine what it was like for my son.
 |
| On the epilepsy unit. |
 |
| He was hooked up to everything. |
 |
| Ezra had a hard time sleeping at times. We cuddled a lot! |
For the next 6 weeks, Ezra received high dose steroid injections, twice a day. By me. Every single one that I gave him, broke my heart. I was no stranger to intramuscular injections. I gave them to my patients all of the time in L&D, however, I never liked to give them to the newborns. I felt horrible doing it. But now here I was. Having to inflict this pain to my own child. I tried to remind myself that I had to do it. It would help him... But when you have a child that is developmentally behind, non-verbal and is unable to communicate and you sometimes are unsure if he is understanding you... It makes you shed tears. It makes you position yourself in a certain way so that while you give him the injections you can't see his face and he can't see yours. Because you are his mother and you are the one that is suppose to comfort him and not cause him to hurt.
The side effects were worse than they described. Ezra had to have his blood sugar and his blood pressure checked twice a week initially at his pediatrician's clinic. His blood sugars, thankfully were always within the range which was deemed acceptable. His blood pressures on the other hand were not. Ezra was put on antihypertensive medication once a day at first but then when that wasn't helping his dosage was increased and he subsequently needed to take it twice day. He ballooned. The steroids caused Ezra to retain fluid and become swollen and puffy. His poor belly had become so big and swollen that he couldn't get comfortable to sleep (he always slept on his belly). At one point he was so irritable that he cried all of the time. ALL OF THE TIME. I remember a particular night when I was putting him down for bed. He was so tired, but was restless. He started to cry. It was around 8pm. I tried everything to soothe him and comfort him so he would sleep. Nothing worked. He cried until 5am. He finally fell asleep in my arms. I didn't dare move for the next couple of hours. Needless to say neither Ezra or myself were getting any kind of sleep for several weeks.
 |
| Ezra got swollen and puffy very fast. He was not a happy camper. |
Ezra would have to have several EEG's during the course of the ACTH therapy to evaluate if the therapy was working and if the hypsarrythmia was still present. About two and half weeks after the therapy was started, we got the news we were praying for. There were no signs of hypsarrythmia! Ezra's weaning process off of the steroids was started. The side effects lingered long after the ACTH therapy was completed. Ezra was not Ezra. His temperment and personality were so different. I knew it was related to the high dose steroids, but it was so hard to see him like that. He stopped smiling for the longest time, let alone laugh (and he has the BEST laugh, ever!). Eric once mentioned to me that he was concerned Ezra was depressed. I remember at his neurology visits, the nurses always would have words of encouragement for me. Reminding me that he will be back to his normal self in time. And again, they were right. It took weeks after the therapy was over, before Ezra gave a little smile again. I remember crying tears of joy and relief when he did.
 |
| No more hypsarrythmia! |
 |
| Ezra's first smile since starting ACTH! |
 |
| Ezra with his sister, Zara. |
 |
| Cuddle time with Daddy. |
 |
| Still not a happy camper. One of the last few EEG's during his treatment. |
 |
| Our little man, a year later. |
West Syndrome is an extremely rate form of epilepsy. It's even more rare for it to come back. You could imagine with all the unknowns about Ezra, it's always on my mind. I am aware that the probability of Ezra having a reoccurrence is very low. But it's still there. Not a day goes by that I still don't analyze every strange movement that Ezra makes. I always worry that it will come back. I just wish my sweet little boy can just catch a break.
remplacer implants mammaires
ReplyDelete