Making Decisions: Neuro-Immuno Follow up 8-23
It took me a long time to come up with a title for this post. Do I call it "The Answer?" Or "The End of the Road?" Or even "Have we found the Magic Wand?"
But then as I reviewed my notes from all of our discussions today, I realized that we are not at the end of the road. We are only at the beginning of a major intersection.
We arrived at our appointment and were greeted by our son's in-home therapist and her supervisor, as well as our IOP therapist, who has been sending weekly reports to Dr. G, and has been seeing Hunter since January, and then Dr. G, his nurse, and his two residents. He wanted us to explain with detail some of the changes we had seen. After listing several of the positive behaviors, as well as our ability to "normalize" certain activities that we had earlier crossed off our lists, like even going to the grocery store. We have had so many less fits, less aggression, less property destruction, and overall better compliance. We also told him that after the steroids, he seemed less "foggy," as he would respond to conversations and comment on activities going on around him, which had seemed to disappear over the past couple of years.
We reviewed the IOP's graph, as well as our in-home therapists. Here are a few of the changes...and again, the therapists were completed blind as to the date and type of treatment done this summer.
Both at home and in the IOP, there were no changes in the physical movements with the steroid. I'll explain this significance below...
After reviewing the graphs, Dr. G explained the scientific findings of the steroid burst. This picture he drew was a good illustration to really help us understand:
SO Hunter's brain is the circle with the "H." When he had the first illness, it spilled over to the parts of the brain it wasn't supposed to- maybe as one doctor had explained, crossing the blood brain barrier. Once this occurred, his body started to create antibodies. This chain of reactions can be separated into two categories, as shown in the picture above. The first theory is that the infections, antibodies, etc. have formed a series of connections that have changed the brain forever. That is the side we target with our ABA therapy, constant behavioral training, and exhausting compliance to maintain consistency. The other side represents the medical theory, where something within the body is causing a chain reaction. Some type of immunodeficiency has occurred, or is presently occurring. PANDAS, or PANS would fall here.
When Dr. G gave the steroids, they were supposed to reduce inflammation and temporarily stop the production of the antibodies. Just to be clear, our bodies are constantly making antibodies, since we live in a world of germs and are exposed to many types of things daily. For Hunter, his immune system is over-reactive, and all of those IgG levels (how they measure past infections)are elevated b/c his body is constantly creating antibodies to "fight" different things...which in turn is wreaking havoc on the body. With a positive result to the steroids, as Hunter demonstrated, it confirms that there is a medical side to his disorder. However, with the consistent hand flapping and without a decrease in the movements, Dr. G stated that unfortunately, Hunter's issues stem from both sides. Some of those crucial connections have already been formed, and no matter the type of medical intervention, we will still need to continuously work with him for his learned behaviors for the past three years.
He said there were two ways to look at immunodeficiency therapy:
1) Suppress the immune system and it doesn't go back to creating those bad antibodies
2) Some of the normal parts of the immune system will need to be reset; you will have to treat the confused side of the brain.
Hunter's confused side of the brain contributes to all of his issues with filtering information, inattention, engaging with peers, putting up barriers, and mismanaging his aggression, OCD, and anxiety.
He said he still wouldn't label Hunter with PANDAS- he doesn't believe for him it is solely related to strep. Even though strep is a definite and documented trigger, he thinks other viral infections and illnesses could trigger a similar reaction. He has an over-reactive on-switch; whenever he is exposed, it turns on his immune system (the confused portion) and we get the reaction.
I asked him if he had heard of Moleculera Labs, which was developed by Dr. Cunningham to give a more definite picture of PANDAS/PANS. They were established to help families deal with autoimmune movement, behavioral and neuropsychiatric diseases. The test measures the antibody titers against four neuronal antigens present in the brain, including lysoganglioside, tubulin, protein kinase, and two dopamine receptors. He hadn't heard of the testing, but he stopped me mid-sentence and said,
"I don't need any more testing to determine if further treatment is needed. I have no doubt that there is an immune mediated process going on." He said today he would add the diagnosis of Autoimmune Disorder (either unspecified or suspicion of encephalopathy). So the steroid burst did it's diagnostic job...he told us when he met with his team (didn't know he had one!) regarding Hunter, one of the colleagues joked that he was going to get graphs that were all over the place after the burst. Not for Hunter...
Another point he made was that the steroids could have given a temporary result if there wasn't an immune process occurring, but it would have only lasted about a week. We are still seeing results 10 weeks later, although slowly behaviors are creeping back up.
After a lengthy, lengthy discussion with all providers and Peter and I, he presented our options:
1) Watch and wait. Observer another 4-8 weeks and see if he maintains his gains. Define what re occurrence will mean and then at that point do another treatment when necessary.
RISKS: a crazy, anxious mom
2) 2nd round of steroids. If you repeat it at the regular levels, you can get the same response. The goal is that it wipes out the inflammation and you have to repeat it less frequently. There are a couple of theories that show that there is a critical period, in which you want to administer the second dose quickly following the first, so the antibodies don't have a chance to come back; otherwise it can make it difficult to restore the progress you had made.
RISKS: Arterior Necrosis (hardening of bones after a part dies from a reaction to steroids) risk is the same- doesn't increase with repeated exposure; Bone Density- we wouldn't know this immediately. Repeated use of steroids increases the risk for brittle bones in the future. :(
3) Rituximab: It helps the immune system destroy cells. It is actually a therapy for lymphomas and other autoimmune diagnoses. He explained that the immune system has a lot of soldiers in the army...by taking out the B Cells (which are important for antibody production), it would stop this misfiring. The B cells are like people, so the therapy would knock out the adolescent-type cells. This coordination makes the confused cells go away. This treatment would be considered aggressive in that the B Cells would be gone for 6 months to a year.
RISKS: One serious side effect is called PML, which is a serious brain infection, sometimes fatal. Patients can also have skin and heart reactions. Although these are rare, reading about the potential things that could happen to your child is extremely scary.
4) IVIG. In the past, Dr. G had said plasmapharesis might be the better route. After the steroid burst, he realized that without losing the physical movements, we were not dealing with just an acute infection. The other side has already been altered. IVIG would be a better route in this case, but Dr. G also said he has had the most failures with this treatment as well. It doesn't suppress the immune system, but replaces all of the red blood cells to stop the confusion (of making the bad antibodies). With Hunter's recent Vitiligo diagnosis, it was just another indication of the immune mediated process that is occurring. He felt IVIG would be one of the safest routes we could go.
RISKS: Aseptic Meningitis is one of the most severe, also most rare. Typical risks associated with blood products
So in reflecting over today's visit and notes, I have very mixed feelings. I think the hardest part of today was hearing something that I have really known all along but haven't wanted to admit.
The damage has been done.
At every point in our life as a child, we are going through critical stages of development. When you hear of regressive Autism, you typically see a child losing physical skills and speech declines. Sometimes, the child goes from verbal to non-verbal overnight. The common time frame is around 18 months. Some of those parents claim vaccinations stole their child from them. For Hunter, that time period of his brain development coincided with an autoimmune reaction that spiraled throughout his body. In order to recreate those synapses, remap the brain so to speak, we need intensive therapy. But would you ever tell a stroke victim not to start therapy to regain their skills? If a 60 year old can talk again, or learn to walk from square one, my five year old can hopefully learn to handle social situations and anxiety with proper training. There is something to be said about the plasticity of the brain.
I honestly didn't think we would be needing ABA therapy for years to come, but I guess I presumed too much. Even after the proposed medical interventions, we still need to recover that brain. We would be able to hopefully make quicker progress if it wasn't confused or foggy, though. And that's where the medical intervention would help.
The positive light from today was hearing Dr. G say he had "no doubt that this was an immune mediated process." It was seeing graphs and charts that, in a controlled data collection, demonstrated a favorable response to steroids. It was looking at Hunter and knowing that the sleepless nights and endless research has all been worth it.
We were sitting in a room with six professionals justifying our suspicions. If you would have asked me three years ago if we would be here today, I would have never been able to predict the paths that we have taken on this journey. But I always maintained hope.
So do we take the risk and medically intervene? Would it truly make a difference?
Last night, I was up way too late prepping and realized that I hadn't watched the videos we found of our sweet boy prior to initial insult. As I lay in bed, I smiled through tears at this happy little boy with bright blue eyes that I once knew. The little boy who giggled while pretending to drive a boat, the little boy who made "stew" on the kitchen floor with Paige, stirring and pouring in my pots and pans. It was a little boy who pointed at things, directed me to show something of interest, answered my questions, and engaged with all around him.
I also heard something else while listening to our commentary in the videos. I heard parents who had laughter in their voices, who had pride over the "firsts" documented in little snapshots of activities, parents whose biggest worries were if he was going to choke on his first "puff" or trip while walking for the first time.
Never in my mind did I ever think we would be making decisions as big as these, to improve something that should just come naturally: living and loving life as a care-free child.
Contemplating and reflecting this weekend, but also celebrating a fool-proof, justifiable, documented diagnosis that we have been searching for over the past three years.
But then as I reviewed my notes from all of our discussions today, I realized that we are not at the end of the road. We are only at the beginning of a major intersection.
We arrived at our appointment and were greeted by our son's in-home therapist and her supervisor, as well as our IOP therapist, who has been sending weekly reports to Dr. G, and has been seeing Hunter since January, and then Dr. G, his nurse, and his two residents. He wanted us to explain with detail some of the changes we had seen. After listing several of the positive behaviors, as well as our ability to "normalize" certain activities that we had earlier crossed off our lists, like even going to the grocery store. We have had so many less fits, less aggression, less property destruction, and overall better compliance. We also told him that after the steroids, he seemed less "foggy," as he would respond to conversations and comment on activities going on around him, which had seemed to disappear over the past couple of years.
We reviewed the IOP's graph, as well as our in-home therapists. Here are a few of the changes...and again, the therapists were completed blind as to the date and type of treatment done this summer.
 |
| IOP total behaviors, with the steroids and strep shown. |
 |
| Aggressive behavior at IOP- kicking, hitting, etc. Declined after steroids. |
 |
| Prompts to stay engaged at IOP- if behaviors are as high as they were, typically you wouldn't see this few prompts to stay involved in the activities. |
 |
| In-Home ABA therapy- definite decline in property destruction and aggression; not in hand flapping or tantrum ( but those include just saying no) |
 |
| Demand- Rate/Minutes per hour- decreased in aggression and tantrums |
 |
| Tic- rate/minutes per hour (blue is hand-flapping) |
 |
| Hand-flapping and perseverative speech; no change or pattern with steroids |
After reviewing the graphs, Dr. G explained the scientific findings of the steroid burst. This picture he drew was a good illustration to really help us understand:
SO Hunter's brain is the circle with the "H." When he had the first illness, it spilled over to the parts of the brain it wasn't supposed to- maybe as one doctor had explained, crossing the blood brain barrier. Once this occurred, his body started to create antibodies. This chain of reactions can be separated into two categories, as shown in the picture above. The first theory is that the infections, antibodies, etc. have formed a series of connections that have changed the brain forever. That is the side we target with our ABA therapy, constant behavioral training, and exhausting compliance to maintain consistency. The other side represents the medical theory, where something within the body is causing a chain reaction. Some type of immunodeficiency has occurred, or is presently occurring. PANDAS, or PANS would fall here.When Dr. G gave the steroids, they were supposed to reduce inflammation and temporarily stop the production of the antibodies. Just to be clear, our bodies are constantly making antibodies, since we live in a world of germs and are exposed to many types of things daily. For Hunter, his immune system is over-reactive, and all of those IgG levels (how they measure past infections)are elevated b/c his body is constantly creating antibodies to "fight" different things...which in turn is wreaking havoc on the body. With a positive result to the steroids, as Hunter demonstrated, it confirms that there is a medical side to his disorder. However, with the consistent hand flapping and without a decrease in the movements, Dr. G stated that unfortunately, Hunter's issues stem from both sides. Some of those crucial connections have already been formed, and no matter the type of medical intervention, we will still need to continuously work with him for his learned behaviors for the past three years.
He said there were two ways to look at immunodeficiency therapy:
1) Suppress the immune system and it doesn't go back to creating those bad antibodies
2) Some of the normal parts of the immune system will need to be reset; you will have to treat the confused side of the brain.
Hunter's confused side of the brain contributes to all of his issues with filtering information, inattention, engaging with peers, putting up barriers, and mismanaging his aggression, OCD, and anxiety.
He said he still wouldn't label Hunter with PANDAS- he doesn't believe for him it is solely related to strep. Even though strep is a definite and documented trigger, he thinks other viral infections and illnesses could trigger a similar reaction. He has an over-reactive on-switch; whenever he is exposed, it turns on his immune system (the confused portion) and we get the reaction.
I asked him if he had heard of Moleculera Labs, which was developed by Dr. Cunningham to give a more definite picture of PANDAS/PANS. They were established to help families deal with autoimmune movement, behavioral and neuropsychiatric diseases. The test measures the antibody titers against four neuronal antigens present in the brain, including lysoganglioside, tubulin, protein kinase, and two dopamine receptors. He hadn't heard of the testing, but he stopped me mid-sentence and said,
"I don't need any more testing to determine if further treatment is needed. I have no doubt that there is an immune mediated process going on." He said today he would add the diagnosis of Autoimmune Disorder (either unspecified or suspicion of encephalopathy). So the steroid burst did it's diagnostic job...he told us when he met with his team (didn't know he had one!) regarding Hunter, one of the colleagues joked that he was going to get graphs that were all over the place after the burst. Not for Hunter...
Another point he made was that the steroids could have given a temporary result if there wasn't an immune process occurring, but it would have only lasted about a week. We are still seeing results 10 weeks later, although slowly behaviors are creeping back up.
After a lengthy, lengthy discussion with all providers and Peter and I, he presented our options:
1) Watch and wait. Observer another 4-8 weeks and see if he maintains his gains. Define what re occurrence will mean and then at that point do another treatment when necessary.
RISKS: a crazy, anxious mom
2) 2nd round of steroids. If you repeat it at the regular levels, you can get the same response. The goal is that it wipes out the inflammation and you have to repeat it less frequently. There are a couple of theories that show that there is a critical period, in which you want to administer the second dose quickly following the first, so the antibodies don't have a chance to come back; otherwise it can make it difficult to restore the progress you had made.
RISKS: Arterior Necrosis (hardening of bones after a part dies from a reaction to steroids) risk is the same- doesn't increase with repeated exposure; Bone Density- we wouldn't know this immediately. Repeated use of steroids increases the risk for brittle bones in the future. :(
3) Rituximab: It helps the immune system destroy cells. It is actually a therapy for lymphomas and other autoimmune diagnoses. He explained that the immune system has a lot of soldiers in the army...by taking out the B Cells (which are important for antibody production), it would stop this misfiring. The B cells are like people, so the therapy would knock out the adolescent-type cells. This coordination makes the confused cells go away. This treatment would be considered aggressive in that the B Cells would be gone for 6 months to a year.
RISKS: One serious side effect is called PML, which is a serious brain infection, sometimes fatal. Patients can also have skin and heart reactions. Although these are rare, reading about the potential things that could happen to your child is extremely scary.
4) IVIG. In the past, Dr. G had said plasmapharesis might be the better route. After the steroid burst, he realized that without losing the physical movements, we were not dealing with just an acute infection. The other side has already been altered. IVIG would be a better route in this case, but Dr. G also said he has had the most failures with this treatment as well. It doesn't suppress the immune system, but replaces all of the red blood cells to stop the confusion (of making the bad antibodies). With Hunter's recent Vitiligo diagnosis, it was just another indication of the immune mediated process that is occurring. He felt IVIG would be one of the safest routes we could go.
RISKS: Aseptic Meningitis is one of the most severe, also most rare. Typical risks associated with blood products
So in reflecting over today's visit and notes, I have very mixed feelings. I think the hardest part of today was hearing something that I have really known all along but haven't wanted to admit.
The damage has been done.
At every point in our life as a child, we are going through critical stages of development. When you hear of regressive Autism, you typically see a child losing physical skills and speech declines. Sometimes, the child goes from verbal to non-verbal overnight. The common time frame is around 18 months. Some of those parents claim vaccinations stole their child from them. For Hunter, that time period of his brain development coincided with an autoimmune reaction that spiraled throughout his body. In order to recreate those synapses, remap the brain so to speak, we need intensive therapy. But would you ever tell a stroke victim not to start therapy to regain their skills? If a 60 year old can talk again, or learn to walk from square one, my five year old can hopefully learn to handle social situations and anxiety with proper training. There is something to be said about the plasticity of the brain.
I honestly didn't think we would be needing ABA therapy for years to come, but I guess I presumed too much. Even after the proposed medical interventions, we still need to recover that brain. We would be able to hopefully make quicker progress if it wasn't confused or foggy, though. And that's where the medical intervention would help.
The positive light from today was hearing Dr. G say he had "no doubt that this was an immune mediated process." It was seeing graphs and charts that, in a controlled data collection, demonstrated a favorable response to steroids. It was looking at Hunter and knowing that the sleepless nights and endless research has all been worth it.
We were sitting in a room with six professionals justifying our suspicions. If you would have asked me three years ago if we would be here today, I would have never been able to predict the paths that we have taken on this journey. But I always maintained hope.
So do we take the risk and medically intervene? Would it truly make a difference?
Last night, I was up way too late prepping and realized that I hadn't watched the videos we found of our sweet boy prior to initial insult. As I lay in bed, I smiled through tears at this happy little boy with bright blue eyes that I once knew. The little boy who giggled while pretending to drive a boat, the little boy who made "stew" on the kitchen floor with Paige, stirring and pouring in my pots and pans. It was a little boy who pointed at things, directed me to show something of interest, answered my questions, and engaged with all around him.
I also heard something else while listening to our commentary in the videos. I heard parents who had laughter in their voices, who had pride over the "firsts" documented in little snapshots of activities, parents whose biggest worries were if he was going to choke on his first "puff" or trip while walking for the first time.
Never in my mind did I ever think we would be making decisions as big as these, to improve something that should just come naturally: living and loving life as a care-free child.
Contemplating and reflecting this weekend, but also celebrating a fool-proof, justifiable, documented diagnosis that we have been searching for over the past three years.
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