It has been awhile since I updated and that has been because there has just not been any time. Maddie continues to have seizures every day.
I did not know much about Infantile Spasms (IS) when she was diagnosed and so I did not share much about it in the last post. We still are not completely sure of the cause, though we suspect chemo. If we knew the cause, it would give us a little bit of guidance as to how to treat it. No matter the cause, Maddie is a very atypical case of IS because it showed up much later than in most kids. That makes it even harder to know the best course of action to treat it. She is such a strange case that our neurologists are publishing her case, because they feel she is the first they have ever seen. Not very encouraging…..
I will try to give a little more info on IS and what it means for us. The reason Maddie’s seizures are characterized as IS is that the spasms themselves look exactly like typical “Salaam spasms” and her EEG shows an abnormal brain wave pattern that is almost hypsarrhythmia. Her development has also stopped since these seizures started, and that is one of the identifying factors for IS. IS is considered one of the “catastrophic” epilepsies because the seizures associated with it sometimes do not respond to treatment. Kids with IS often develop more types of seizures, even if the IS seizures can be stopped, and they are very likely to have developmental delays, and sometimes severe mental retardation.
As I have said before, Maddie is a very unusual case and we hope and pray that we can stop her seizures and that she will begin to develop again. At this time, we would not say she has regressed significantly since the seizures started, but rather that her development has stopped.
After the Infantile Spasms diagnosis, we had one clear first choice for treatment which gets rid of the seizures (at least temporarily) in almost 70% of patients. Unfortunately, Maddie did not get seizure free in response to steroids. And she has been on a very high dose which has made her feel absolutely horrible ever since we started them on Feb 19th. She does not sleep at night, but we sometimes get 4 hours from 5 am til 9 am. Otherwise, she naps from time to time and feels awful when she is awake. This has been pure torture to watch. It would have been worth it if it got rid of the seizures, but it did not. Because of how high the dose is, we have to wean them slowly, and while we have started, we won’t be done for 14 more days. For you other cancer parents out there, her dose was 3 times the induction dose of steroids for the first 2 weeks. It has truly been insane.
In addition to feeling anxious, irritable, and unable to sleep, she also has major muscle weakness from the steroids and it makes walking difficult for her. Steroids incease appetite hugely, and she has already gained four pounds since starting them 3 weeks ago. That is more than she has gained in the last 16 months of chemo, and it definitely is excessive. The skin on her little face and tummy looks stretched taut. I am counting the days until we can stop the terrible steroids.
So the next drug of choice for IS is called Vigabatrin and it is not yet approved by the FDA because of a very bad side effect. There is a risk of permanent vision damage with this drug. We plan to start this drug tonight, as it just arrived by mail this afternoon. The FDA plans to approve it later this year because the possible benefit (stopping seizures which cause brain damage) outweighs the risk of vision loss. Giving Maddie this drug is not a decision we have made lightly. It is clear to us that the toxicity of chemotherapy drugs we gave her to kill leukemia has played a role in causing these seizures. We know all too well the risk of side effects of medicines. But we cannot watch our baby suffer with these seizures if there is a cure out there. The seizures have taken our lively, energetic, curious child away from us and we want her back.
In other news, we have decided not to resume chemo when she is done with the steroids because we feel there is no evidence yet that the extra 8 months of maintenance chemo improves longterm survival of her type of leukemia. We also hope that giving her immune system a chance to heal may improve her chances in beating these seizures. Unfortunately, letting her immune system heal means giving the leukemia a more “fertile” environment to reappear if it is still lingering in her somewhere. So we ask for your prayers again that she remains in remission as we enter the scary and uncertain world without chemotherapy. It will take her immune system a long time to get back to normal, so we are not going to be out and about anytime soon, especially as the seizures have us basically housebound except for doctor visits.
On that note, I want to especially thank my mom and Lance for keeping us fed and picking up all our necessities, like prescriptions and diapers. They also help out a ton with Maddie during our frequent sleepless nights and they never complain that they can hear her screaming like a banshee all night long either. They truly had no idea what they were getting into letting us live here, and they have truly been saints.
I also want to thank my fabulous husband for eight wonderful years of marriage. I could noy imagine my life without you, honey! Things have not always gone as planned but we are truly in this as together as we could possibly be. Despite living 350 miles away, he is here every weekend, and has been for 16 months. He basically lives in his car and gets no sleep when he is here for two nights before turning around to head back for another week of work.
At this point, we will not be moving to Quantico because Brandon now has orders to move back to Camp Lejeune in Jacksonville, NC this May. We hope to visit him up in Quantico soon as we are no longer as tied to the Oncology clinic for blood draws all the time. We also plan to try to move to Jacksonville with him in May or June. We hope to move our Oncology follow-up care to Chapel Hill now that Maddie’s neurologist is at UNC. That is only a little more than two hours from Jacksonville. We would do just about anything to get to live together again as long as it does not compromise Maddie’s care. It looks like Brandon will most likely deploy again outside the US for awhile starting in October 2009. He will also have quite a bit of away-from-home training to prepare for the deployment, so we need to take advantage of the little quality family time left before he has to leave.
Thanks also to those of you who remembered our anniversary and sent us cards and gifts. We have truly been blessed with such a thoughtful family and feel so lucky for it.
From here, we hope to see positive results (no more seizures) when we start the new medicine today. We will travel to Chapel Hill next Tuesday for a visit with her Neurologist and to have a lumbar puncture procedure under anesthesia to see if we can shed more light on what might have caused Maddie’s Infantile Spasms.
If you made it this far, thanks so much for your concern. We cannot tell you how much we appreciate everyone’s support on this long journey. Those of you who know me know how controlling I am. I am a planner, and things must go according to my plan. When cancer found us, I was able to adjust my plans and make beating cancer my goal for the next two years. IS is a whole new animal for me: no clear prognosis, no endpoint to treatment. It has taken me a little bit to wrap my head around it. It is much harder for me to face the challenge head on when we don’t quite know what we are facing or if it will ever end. What I have come to terms with is that I have never had more determination to do the very best I can to advocate for and take care of my extremely precious daughter.
Love to everyone,
Liz, Brandon, and Maddie