Saints, Martyrs, and the gift of ALD

This is not a feel-good post.

I’m tired of fear and pity parties. I mean, don’t get me wrong – we’re all entitled to having a pity party sometimes – but after a while, our friends need to grab us by the shoulders, tell us to get over ourselves, and go send us out to help someone who has it worse off. Maybe to go help a LOT of people who are worse off until we get it through our heads for the time being that the present moment is actually just fine and our lives are not so especially disastrous.

I am talking, of course, about ALD.  What else?

I’ve said it before and I’ll say it again: ALD is frightening. Sometimes, it’s like you have arachnophobia and are forced to live in a tarantula house at the zoo. But, then you learn that the spiders really aren’t so bad, they don’t have it out for you (except for that one little bugger with the orange stripes. He’s a douchebag) , and they’re just doing whatever it is that spiders do as part of their life cycle. You’re just there. They don’t really have it out for you personally (just watch out for the orange jerk. He hates you and he’s poisonous). Of course, then one of them lands on you and you freak out for a while. But then you remember that it’s all going to be okay and at least it’s not snakes. Or starving tigers.

Living with ALD hanging over your head is exactly like that. 

Or at least it should be. 

Here’s the tricky part of living with a disease like ALD and AMN: it’s really, really easy to sacrifice yourself on its altar and become a martyr to it.  It’s expected. Neigh – it is applauded by society at large and parts of the disease community itself.  Society likes to memorialize us before our due and the community likes to get in pissing contests about how bad we’ve all got it. This is because in both cases, it makes our egos individually feel a lot better about whatever is going on in our lives. 

 The Buddha is credited with saying “have compassion for all beings, rich and poor alike. Some suffer too much, others too little.” I’ve always thought that the word order of that was an interesting choice because it eliminates the idea that it’s necessarily the well-off suffering too little. Along these same lines, there is a poem that circulated the internet memes to the tune of “I wish you enough” where the speaker wishes one enough rain to appreciate the sun and enough suffering to appreciate small joys. I particularly liked that one. Thomas Merton warns us not to try to avoid suffering because the harder we try, the more small and insignificant things begin to torture us. Suffering in life is important – it is what drives and shapes our journey. It’s what makes us who we are depending on how we choose to respond to it. Whether we stay attached to it and feed ourselves a story about how bad we’ve got it OR use it to be grateful for everything we DO have and be in the present moment is the perpetual choice.  And it’s a damned hard choice sometimes. But here is the cheat code for the video game of life: it’s ALL suffering. Your response to it is what decides whether or how long you actually suffer.

And frankly, I’m tired of hearing how bad we families with ALD have got it. I’m tired of hearing that we’re all facing one terrible fate and that fate is determined by our genes. So, I left my support group after almost two years. It was very helpful at times – I wouldn’t have gotten through the earlier days without them – but at others it was shackles around my ankles because I’m tired.

I’m tired of hearing about the “beast” our family fights every day as if it makes us special. It’s tough. But we’re not special and we’re not saints. Our prospects as parents are no worse than anyone else’s and our suffering is no greater than what’s expected of every human being on the planet. It’s been almost two years and Cutler and I have come to terms what living with ALD means. We’ve come to terms with the knowledge that there is a chance that Gabe could die because of his condition. But there is a greater chance at this point that he – and you or I – will die of heart disease. 25% of Americans die of heart disease versus 8% of males with the ALD gene who get cerebral form after childhood. The risk of cerebral will increase to 20% if he gets a formal diagnosis of AMN one day, but that’s still less likely than heart disease and cancer (we ALL have a nearly 25% chance of dying of cancer in America). Not all boys have this statistical chance, someone - 35% - will be that 35% with CCALD, but the fact that most boys DO have a better chance should give us ALL hope.

When a parent gets the diagnosis of ALD, sometimes it’s too late for treatments to be of much help. That’s because we don’t have New Born Screening in most states and children and their families are only diagnosed with they start to exhibit serious symptoms.  About 1/3 (up to 35%) of all males who have the ALD gene will get the worst form – childhood cerebral. Another 8% (20% of 40%) will go on to develop AMN that becomes adult cerebral. But the majority – the remaining 67% - will survive with either AMN of some degree, Addison-only, or no symptoms. The data on the percent of the population who are totally asymptomatic is low but inconclusive because until we get standard NBS, we will continue to identify patient populations based on the exhibition of symptoms.  The fact of the numbers does not seem to be one that is embraced outright by the ALD community because most people involved in the community are the 17% of carriers whose children developed the CCALD form. The statistic that their children are in the minority is not helpful for them when they are in pain. They don’t want to hear that most of us will be okay because they are not ok. (Where did I get 17%? A carrier has a 50/50 chance of having a child with the gene and a 35% chance of the 50% who inherit the gene getting CCALD. That’s 17% of the carrier population who will have a child with CCALD). That 17% percent is a very vocal minority and that’s GOOD because it’s them who are leading the charge for cures and them who really need it. But it skews the view of ALD for everyone else.  THAT is the trouble with support groups.  People who survive ALD and are doing fine generally don’t need support groups, so new terrified parents join seeking answers and are confronted daily by the experience of 17% of carriers and 35% of boys as if this outcome is the inevitability. It’s needlessly terrifying when what we should be doing is reassuring frightened people that ALD is not a death sentence. Not convincing them by the war stories of the minority that it is.

But as I said: I’m tired.  I’m tired of being scared and scaring people who are already frightened. I’m tired of bereaved women accusing carriers who decide to have their own children of child abuse by daring to have babies who have an equal potential to live with ALD as they do to have a baby who eventually dies of either cancer or heart disease. And yet don’t see the irony amid their accusations of child abuse for giving birth of their simultaneously pushing forms of abortion for mutants as compassionate (frankly, as one of the offending mutants, I’ll take living with nerve pain, etc  to the alternative, but that’s just me.) I’m tired of being made to feel like I should be ashamed of having this mutation and that it’s irresponsible for people like my son and I to exist because of community members projecting their own fear and guilt. I’m ready to just join the “normal” population again. Almost half of them will die of either cancer or heart disease. 10.4% will die of accidental death before they are adults. 100% of them and us are suffering. 100% will die at some point and most of the non-mutants don’t spend all their time brooding on this inevitability or convincing everyone else that their mortality is a burden. (except the goth kids, but we let them because they make funny movies with gallows humor).

Here is what I have learned from working in healthcare: being with the terminally ill and dying is – absolutely, without question – a privilege. We’re going to die. ALL of us of SOMETHING. If we’re LUCKY, we get to see it coming, come to terms with it, and say goodbye to the people we love. 10.4% of children and teens die of sudden, accidental deaths. Their parents don’t get to plan and say goodbye. I’m not saying it’s not completely horrible either way, but take your blessings where they fall.  At least we families of ALD are given the very tangible gift of knowing that our time could be limited and to make the best of it. We have an albatross hanging around our necks and it’s a gift. It tells us that life is PRECIOUS. We have been given the opportunity that parents who loose their children suddenly don't have.  Children with ALD and any other chronic or terminal illness are not sentenced to a life of suffering any more than each one of us are. We ALL suffer. That is what life is about – how we deal with life itself and all of its beautiful tragedy. Whether that is ALD, heart disease, cancer, loss of income, or any of the myriad other sufferings that humanity faces. It is not irresponsible to allow people like my son and I to live, it is not shameful that we have a genetic mutation, our genes are not our fate, and it is a privilege, not a burden, to parent a child with ALD because they tell us exactly how beautiful, precious, and precarious life is.

I’ll be afraid the next time Gabe gets an MRI. And then at some point I’ll shake off the spiders and calm down because at least they are not starving tigers. I know I’ll be afraid every time something changes, but I’m not afraid now and I’m tired of living under the proclamation from other parents in the community that I should live in constant fear and shame. This is the antithesis to my philosophy of life and I refuse to be a martyr. To quote that beautiful story The Fault In our Stars, ‘some infinities are bigger than others .’ There is no separation between me, the mutant, and you the non-mutant:  neither of us know if our infinity is contained in a second or an hour. The difference is that I just might be lucky enough to get to count the seconds. ALD gave me that.