Sunday, January 22, 2017

fireworks and sunshine

We met with Rainbow's new(ish) immunologist a few weeks ago. I've been processing the news we got since then (it's not bad).

Rainbow is immune deficient, specifically over the past roughly three years his immunogobulin (antibody) levels have steadily declined. Immunoglobulins are sort of like your soldiers. When you hear about your immune system having a memory these are a large part of that, they do A LOT of important things. You can find some info about immunoglobulins here or here in case you'd like to learn more.

A year ago the three immunogobulins we monitor IgG, IgM, and IgA all declined below normal ranges (A & M were really really low). As a result we switched from prophylactic antibiotics to manage his deficiency to IgG infusions. Thus, while we do monitor his IgG levels they aren't really telling us anything about his native immunity. His IgM and IgA might give us a little bit of insight, however.

Fast forward to two weeks ago.

For the first time in a year his IgM and IgA have both inched up above the lowest "normal" cut off (by about a millimeter). This could be pointing to something positive. The thing is these numbers fluctuate like crazy and if we just caught him on an upswing and they are normally low this doesn't help him. Continue to monitor...

Also, our immuno points out: Just because they are present doesn't mean they are functional.


We hadn't even thought to confront that possibility... ugh.

But, what I thought was a really scary indication that his immune system (including his supplemented IgGs) isn't functioning well turns out to be the opposite.

Lately every three weeks or so little bit has woken in the middle of the night with a fever (102ish). We fight it and have terror stomach aches all night. Each time (except for the most recent when he had very obvious symptoms) he wakes the next morning with absolutely no symptoms and no signs of what went on the night before.

My fear was that his body was only intermittently detecting some pathogen then sort of "losing" it because one of the primary functions of Igs is to mark pathogens for destruction. Turns out his immunologist thinks this is actually a sign that Rainbow is picking something up then his (+ his supplemented Igs) system is kicking its ass. Wait, what? Really? This is one of the big things I've been processing. I really like the new immuno and he knows his stuff (bonus points because he has a PhD ;)) but this is still something I'm working to accept.

So, the take home from our most recent visit is: Dr is cautiously optimistic and wants to allow Rainbow to "grow out of" his current IgG supplement. Typically the dose is titrated up as a child gains weight. By allowing him to grow without titrating up we will get a slow and cautious clue as to whether or not he is producing Igs on his own. Dr also wants to vaccinate Rainbow against the specific pathogen that killed his brother and see if his body mounts a specific defense (this will again be a vague hint because of his supplemented IgGs). Dr cautions that there are roughly 485028496 things that could go wrong with the immune system and anytime Rainbow gets a fever he has to be seen by a doctor, every time. I'm down with that and now I have a mandate. So, slow and steady, keep testing, keep watching, and keep hoping.

Ok, finally to the big news... the thing I'm constantly turning over and over and over in my head.

Rainbow's immunologist thinks him starting Tuesday/Thursday school next year is a good idea. He's not pushy about it and is completely on board with us never putting Rainbow in school if that's what determine is best for him but... a good idea.

I'm a part of a few support groups for parents of kids with immunedeficiencies and the most common comment/complaint I see is: my kid is always sick/my kid was in the hospital again. These are statements from parents whose zebras are in school.

We've really never even considered him being in a group childcare situation because daycare is a petridish for every single childhood illness (one of my twitter acquaintances refers to it as "daycare pestilence). I used to be all about the "it makes their immune systems stronger." Then my child died of an infection and my mind changed to oh hell no!

But here's the thing. Rainbow is one of the most social human beings I've ever met. He aches for friends. You'd think, because he's been so guarded his whole life, that he would be introverted but he's not. He is fireworks and sunshine. He loves people.

He has spent so much of his life with adults I feel like we are keeping something beautiful from him. Children are magic. He deserves that magic.

So, we have a choice to make. A choice that keeps me awake at night with longing and with fear. What is best for him?

Hubby isn't even in a place where he can talk about it yet. If he doesn't get there by pre-school sign up time it will be a moo point (friends reference). If we aren't both ready to consider it then it doesn't get considered. So we will have to see...

Until next time...

Wednesday, November 2, 2016

Oy with the poodles already!

Disclaimer: If you do not understand the title reference then I am sad sad sad that you have not discovered and obsessively watched the best show ever made: Gilmore Girls. My most favoiritist show in all the world. 

So, pain... I have a bit of a knack for metaphorizing (is that a word? Well, it is now) emotional pain. It's central to my life. It's all day, every day, influences and underlies everything I do. Emotional pain I get.

But this post is about physical pain y'all.

Ho-ly CRAP.

Turns out I likely have a fun fun fun connective tissue disorder called Ehlers Danlos. A few months ago someone set fire to the low-lying-I'll-just-ignore-it pan in my back and joints and put sedatives in my drinks. 

Lets back up a bit so we can do that fun pan in thing movies always do.

When I was about 13 I blocked a shot in a basket ball game and ripped all sorts of muscles in my left shoulder. As a result I was immobilized and had to sit out two weeks of basketball games. At this point in my young life that was maybe the worst thing that could have possibly happened so when I was finally allowed back on the court I emphatically insisted that I felt fine, my shoulder was fine, everything was just fine, peachy in fact.

It wasn't. I managed to hide the pain until my shoulder started randomly falling out of "socket" (the quotes are because the shoulder isn't a true ball and socket and I'm a scientist and I just can't) and my mom was like "um, no." Long story short I had to have my rotator cuff surgically repaired then spend 6 weeks completely immobilized in a "gunslinger" brace (imagine the most obvious back brace you can then add and equally bulky arm piece sticking straight out at a 90 degree angle with an oldschool video game stick at the end; yeah I was cool) then three months learning to move my arm again. I started practicing again before I was released and less than a year later had torn the other shoulder (repeat above surgery, super sexy brace, and PT) and then quickly retorn the first. 

All that to say upper back/shoulder pain was pretty much the norm for me. From that first injury on I don't remember my shoulders and back ever not hurting. It was my normal. 

Follow this with bursitis and tendonitis in my knees (which I wrote off to being a runner), a slipped/bulging disc in my lower back, temporomandibular joint disorder (my jaw locked down completely about two years ago), and "flares" when every thing hurt so bad I couldn't open a pickle jar occurring with increasing frequency and I probably should have clued in that maybe I'm not the normalest human physiologically. 

Turns out I'm not. After two solid weeks of hurting so much at the end of the day that I could barely participate in getting my kids into bed I broke down and talked to my doctor. My likely diagnosis (pending testing and consultations with, you guessed it, a battery of more doctors) is Ehlers Danlos and Mast Cell Activation Disease (maybe I'll write another post about how my food sensitivities nearly put me in the hospital a few years ago but I'll spare you now). 

I had never heard of these conditions, like ever. Last week we went on the Rainbow's doctors world tour (we saw 4 doctors in 7 days in 4 different towns and 2 different states) and it came up TWICE! New neurosurgeon (talking to me) "I was looking at your hands and was going to ask if anyone in your family has Ehlers Danlos." After leaving I realized I was too distracted by the on-going discussion about possible cranial surgery on my 3 year old to ask him: "My hands? Whaaa?" because in this context we were discussing the likely possibility that my Rainbow shares this shitty condition. 

Oy. with the mother friggn. poodles. al-ready.

Oh... and the Chiari Malformation... I may have that, too.

You know what that means? That means that these two debilitatingly painful, dangerous conditions came from me. It means that I gave him this and now I know how painful it really is. I can now look back and see so many times that fit perfectly. So many weird dislocations and subluxations, so much damn pain... I gave it to him. There is no cure, only "pain management." 

All I want in all the world is to protect my children an my very DNA poisons them. Sounds so dramatic but, for the love, it's true and it hurts and it sucks and I'll be danmed if he is going to hurt like this his whole life. 

For the eleventy-gazillionth time she thinks "Why didn't I go into medical research!?"

Signing off from my bed where I am propped up on 6 different pillows so I can whine at y'all from the perfect angle.

Until next time...

Saturday, October 15, 2016

Well hello there...

I've heard child death described as an amputation by those trying to find the words to explain how it completely changes your life. I think this is as close as you can get to explaining it in a physical way. Except it's a double amputation performed without anesthesia, you're wide awake and helpless, feeling every single stroke of the blade.

For me, the pain knocked me out, for a year.

I've said before that I was something resembling comatose for a very very long time. I screamed and cried and agonized. I remember very little of that year.

Now, four years later... how has it been four years? I rarely have panic attacks and I can function pretty normally. I laugh and I mean it. My legs are gone forever and the pain never ever recedes but I've learned to function and find moments of happiness.

I find myself hesitant to talk about the good moments because people latch on to them like they are being sucked into a jet engine and refuse to acknowledge the swirling agony that underlies every smile. "I'm glad you're doing so well!" Um.. I'm not doing so well. I'm not all better and I'm so done with a society that glosses over pain and grief and the reality that is death. We will all die y'all. But I digress...

I tend to think I've reached a plateau of learning to live after Damon's death. Just learning to leave the house was a straight up vertical climb. Grounding out panic attacks, having conversations... seriously this was insurmountably hard for a long, long, long time. It was so damn hard to talk to anyone about anything. I could not do normal. I've worked so hard to get to a place where I can converse with other human beings, take my son to the park, laugh at my husband's terrible jokes, lecture a classroom full of undergraduates, present at scientific meetings... all of these took concentrated, purposeful effort to recapture, and still do, every time.

So, it has been a weird, uncomfortable surprise to realize that, without purposely striving for it, I've reaccessed a part of me that I didn't even realize existed. Now that this part of me is growing I remember, of course, but I didn't.

I don't know how to explain what this new/old thing is other than to say I want to do things... with people.

Um... yeah, everybody wants to do things Jodie.

Nope, not everybody. Not me, not before about a month ago. If you saw me doing things it was because I needed to do those things. There was a conscious effort in doing those things. I need to do this for my career, I need to do this for my kids, I need to do this for my husband, I need to do this because I care about this person and they've asked me to participate in the thing. I didn't want to. I wanted to go home, always. I wanted to go home and be safe inside my walls with my little family and dis-a-freaking-ppear.

I first noticed this weird new/old sensation when I was at TJ Maxx a while back. There were a few women there, obviously a group of friends just out spending the day together, and I ached. The moment was weird and disorienting. I didn't understand what I was feeling or why I was feeling it. Things like this kept happening, pictures of friends hanging out on social media (I know, it's the devil but there it is), mentions of going out to dinner, or some event participated in and I ached.

I can remember my close friends (most of them very far away physically) saying things like "I miss you" over the past four and half years and not being able to access those emotions. That part of me just wasn't there for me yet. I honestly didn't think it ever would be. This weekend when texting with a friend, who has undoubtedly considered just throwing her hands up in frustration with all the effort she has put in to staying in the relationship with almost no reciprocation, and I realized that I miss her.

I told Hubby this weekend that it's as if this part of my personality lay dormant longer than my consciousness and only now awoke to realize I've stranded it on  deserted island. Because I did. I withdrew from everyone and everything. There is nothing wrong with what I did. I had to but it has certainly left me very alone and entirely unsure of what to do about it. The fact remains that I'm super-dooper-crazy levels of broken. Even without the broken I'm super-dooper-crazy levels of introverted. Long story short, people freak me out, people in groups horrify me. Still, I want to do things... with people (or maybe person, maybe no more than 3 people at a time, did I mention I'm broken?).

So there it is world. I'm lonely... weird.

Until next time...

Monday, October 10, 2016

Where is that white horse?

You know, I really didn't expect it to go this way. Even after a divorce, ensuing pain and drama, figuring out how to do grad school as a single mom... That was hard but I still believed things were going to be good.

And for a while they were. They were really really good. Then it all came crashing down. I got knocked down so hard I didn't even begin to wake up for a year. Now, I walk through this life with a spear through my chest. I hurt. Always I hurt. There is no moving on after a child dies. There is learning to live around the anguish but it never goes away. It never diminishes. You just get used to it and you learn to function.

My rainbow baby brought light back into my life and we have fought for him to live from the first moment he entered the world. He was born unable to breathe, into the world before his lungs had developed the surfactant that would allow oxygen into his bloodstream. His brain wasn't developed enough to keep telling him to breathe and his little heart was so confused about what it was supposed to be doing.

We fought. We slept on the couch in his room, we maneuvered IVs and monitors and tubes so that we could hold him. We just sat and held him.

Every moment of his life that child has been a light.

As he grew it was clear that he was exceptional. He loves everything and everyone. He smiles, he laughs, he runs and plays. He lives. I wish I had words to describe the light he carries. The light that beams from his little heart.

All I want in this life is to protect that child.

Because of the financial strain (impossibility) of paying for the infusions he needs to survive we agreed to join a clinical trial. The trial would supply 6 months of free medication in exchange for data. That data is collected by blood samples.

We knew there would be a lot of blood samples. We had no idea how traumatic they would be for him, how hard it would be to hit his veins, or how much they would scar.

This week, after months of blood draws, he was supposed to go through "PKs." This means his blood was supposed to be drawn twice on infusion day, then the next three days in a row, then again every other day for two more draws.

Long story short, he is too scarred and too traumatized for them to get blood. He becomes hysterical as soon as the tourniquet is put on and kicks and screams through the whole thing. He is scared. After the first attempt on day three (after failure on day 2) he was crying and saying "I want to hold you!" I had had enough. I grabbed him, told everyone we were done and booked it out of the room.

Today, on a conference call with the immunologist I was told that the reason they can't get blood is he is moving too much and "hamming it up" because I am in the room. Not because he is scarred from all of the blood work. He clearly isn't genuinely freaking out because the three year old child has been stuck more times than most pin cushions. No, it's me. Despite the fact that I was holding him the only day they were actually able to get blood.

I was so pissed I almost missed the lecture about how we had gotten thousands of dollars in free medications and we have to follow the rules. We made a commitment, blah, blah, blah... (by the way, from the beginning it was made clear that we could quit the study at any time).

To stay on the study and continue to benefit from the generosity of the multimillion dollar pharmaceutical company Rainbow would have had to start the week of PKs over. That would have been 5 sticks in 4 days, again.

So, we are off. We are off and looking at an uncertain future. We are off and hoping for insurance approval so he doesn't have to go without medication, without protection, and I have a strong feeling his doctor is in no rush to get that done quickly.

I am terrified and despondent and worried and hurt that people I thought were my allies were more interested in using my child for their own gains.

This in addition to the knowledge that one of the people most dear to me in all the world will leave this earth in no more than a year and the memory of the person who loves me more than I could ever fathom will soon be gone has me feeling like there will never be light again.

I asked on Facebook today "Is adulthood just near constant pain and fear for everyone or are we just special?" Today, I feel like laying down and never getting up again. I'm tired of being "such a fighter" and "so strong." Do you know what I would give to have a simple, normal, scariest thing that happened to our child growing up was a broken arm, kind of life? What I would give to be able to believe that the world is safe?

I'm so sad, desperately sad, and there is no one riding to the rescue.

Until next time...

Tuesday, September 20, 2016

Where do I start?

We saw the first neurosurgeon a few weeks ago. He feels strongly that Rainbow's headaches can be managed pharmacologically rather than surgically. This is very desirable because surgery is very very dangerous for Rainbow. We have to see a neurologist to get started figuring out if there is a medication that will work for him. Neurologist isn't scheduling until January. Called around, pretty much everyone who is taking new patients is scheduling roughly that far out. We see the next neurosurgeon Oct 26th. Rainbow is no longer allowed to play on trampolines or in bounce houses because his Chiari Malformation makes those activities dangerous. His trampoline is his favorite thing in our back yard. Suck.

Additionally, Rainbow has a "malformation" of his back (his musculature is very asymmetrical and his spine is slightly "bent"). We showed this to his neurosurgeon and he said "Yeah, that's weird. He needs to see an orthopedist." Is anyone keeping up with the doctor count? Appt with the orthopedist in a few weeks. He also has symptoms the neuro doesn't think are associated with his chiari and may be autoimmune (Pediatric Rheumatologist for that one, add another).

Last week Rainbow had a random (very low) fever, no other symptoms at all. We consulted with his immuno and it resolved on its own and we thought "huh, weird." Last night he woke up freaking out because his head hurt (not really all that new) but when I went to put my hands on his head he was burning up. His temp was 102. We spent a good amount of time on the phone with his immunologist's office (the on-call doc was new to us and was freaking awesome) and three hours on the ledge of "which is more dangerous taking him to the ER or keeping him home?" It took ibuprofen and acetaminophen to break his fever but then he slept from 3am - 7am. Woke up a little grumpy but ate and had no fever.

Dr D's (his immuno) nurse called at 8:15 "how soon can you get him here?" Um... crap, that's not terrifying.

His immuno did a full updated history, spent an hour with us, did a full exam, and.... nothing. Like, seriously, nothing. He doesn't even have a runny nose. That probably sounds like a good thing but to us it's scary. We don't know what we are dealing with.

She ordered bloodwork. Being the boyscouts that we are we were prepared with this numbing cream (don't leave home without it). So, we slathered it on and waited for it to do its work. We went to our usual blood draw lab (how screwed up is that, these people know us on sight) and Raz screamed and screamed and screamed. The phlebotomist hit a vein twice, I saw it, the blood wouldn't come. The phlebotomist's acted like this was the weirdest thing they had ever seen, awesome.

I slumped down to the floor and called his doctor. She didn't want him poked again. He's too worn out. He needs to hydrate like crazy and we will try again tomorrow. If it doesn't work we will have to go to the hospital where they have ultrasounds to do this...

He is sleeping now.

Wednesday, September 7, 2016

The Neurosurgeon

I do this thing when I'm freaking out. I research. I read and read and read and read and look up terms and read some more. In preparation for this appointment I read every paper this neurosurgeon had published in the last twenty years. I also read a bunch of related papers.

I think I do this because it makes me feel like I have some semblance of control over the situation, and because I'm a scientist. Research is what I do.

The thing is, in all of these papers, cases where Chiari Malformation was successfully managed non-surgically were never mentioned. We went into this appointment certain we were looking at an inevitable neurological surgery, on our immune deficient three year old. On the drive there we were both jumpier than a long tailed cat in a room full of rocking chairs.

So. Much. Fear.

Then, a crazy thing happened. We were given hope. We were given hope that our child may get to live a normal(ish) life without anyone ever slicing his head open...

We spend A LOT of time in doctor's offices. While we've developed strong relationships with Rainbow's regular doctors and feel heard by them anytime we see a new specialist we brace to be rushed and brushed off. It's exhausting and frustrating and infuriating. Dr. Mapstone asked tons of questions, examined Rainbow, then sat with us comfortably in absolutely no rush and talked with us about his evaluation. He took Rainbow's immune deficiency very seriously and factored that into his decision about whether or not to recommend surgery. He answered every question we had honestly. We felt heard and we felt that this doctor was going to make decisions with Rainbow's quality of life and health as his foremost concerns.

The bottom line is that there is a very strong possibility that we will be able to manage Rainbow's Chiari Malformation medically, avoiding surgery, possibly forever. If we can, working with a neurologist, find a medication that manages his pain without unacceptable side-effects then he will have an MRI once a year to evaluate his Chiari and go from there. The doctor made it very clear that if his pain cannot be managed or if he develops other symptoms Rainbow needs to come back to him as soon as possible.

We have a plan.

There are so many unknowns and this neurosurgeon was also very upfront about the controversy in his field regarding the best course of treatment for Chiari. Some of the other neurosurgeons with whom we've consulted are more aggressive in their treatment and we still have an appointment at Dallas Children's next month.

We will be adding three more specialists to his team to evaluate an apparent spinal malformation, to possible remove his "huge" tonsils, and to determine if he has Ehlers Danlos Syndrome or possibly some other condition causing the odd sensations and pain in his limbs.

But... No one is going to be cutting his head open in the foreseeable future and I'll take that.

Until next time...