Wednesday, April 28, 2010



This morning...ohhhhh this morning. I finally got to bed about 1am, and then Jackson was up from 2 to 3, and again at 4:15 and I've yet to get back to bed. Emily let her alarm go off for 15 minutes without getting out of bed. She piddled and stalled and played until both Mike and I were up, in her room, lecturing, and prompting her every move, and it STILL took her a half an hour to put on the clothes she was supposed to have laid out the night before. The very state of her room is a nauseating war zone of sparkly hot pink crap and horses of varying sizes- many of whom have been lamed from losing legs or ears under our feet. There are pen markings on the walls and I found half of my brand new pack of bobby pins with the tips bitten off in the carpet. Lecturing the child who moves like molasses in the mornings usually sends my blood pressure a-climbin', but today I managed to keep my cool while informing her in no uncertain terms what the consequences of all this are. Now the day ahead consists of a massive confiscation and donation mission because the glittering Hiroshima down the hall is simply too small to contain it's contents any longer. When I was a kid my mom had to do the same thing. My room was so bad at one point I she had to take away everything but the mattress and alarm clock. I'm not sure it taught me anything, but it did make cleaning my room a hell of a lot easier. I digress! It took an hour of lecturing, prompting, threatening, and general growling from both of us to get her butt out the door and off to school today. In my efforts to explain to her why her mess was so disrespectful when we have to work so hard to give her the things she is treating so poorly, I realized I had let too much slide. IN the interest of keeping the peace with my ex husband and his girlfriend, I have become seriously lax on consequences with her. They're convinced with all that's going on she's being emotionally neglected (phooey) and in turn I've ended up coddling her and letting her get by with things I normally never would. Until now!




All of this before 7:30 in the morning!! I had to manage it between poopy diapers, feedings, meds, teaching my husband how to make coffee, finally losing my voice from my allergies and realizing the mascara I was trying to wipe off my face was actually the dark circles under my eyes.




So WHY, oh why am I in such a good mood??? Because this is what families do. Bitty is too loud and flighty and can NEVER be hurried to do anything. Jackson is a seven week old bundle that still wakes up in the night and occasionally is awake more than asleep when I'd like to be resting. Mike is still not a morning person and I'll have to show him how to make coffee at least three more times. I'm the one in the middle of it all, croaking out directives and hoping we all get enough accomplished to consider the day a success. Our already harried lives have a whole lot more going on now; breathing treatments, CPT, enzymes, clinic visits on top of pediatrician visits...and occasionally some counseling...but I'm seeing more and more that we're all still 'us'. We're still a family, and no diagnosis is going to change the fact that NONE of us are morning people and we will always be cranky and fussy and slow off the mark when the alarm goes off. And today, that makes me happy.














(No, I don't always have this good of an attitude about things, but I try. )




Monday, April 26, 2010

New Drug May Treat Cystic Fibrosis, Other Diseases Caused by 'Nonsense Mutations'

New Drug May Treat Cystic Fibrosis, Other Diseases Caused by 'Nonsense Mutations' (click link for article)

This is why we Hope. This is what we are fighting for. Sometimes it seems too much to hope for, but there is no giving up! We are so close.

Friday, April 23, 2010

Saying Grace



In our home we have what can best be described as a universalist blessing before dinner each night. Formal prayers are waived in favor of sharing with each other what we were thankful for that day. I believe it's crucial to acknowledge even the tiniest of triumphs, celebrate the most minute of blessings, and when we come together at the table and share them, it sheds a light of positivity on our lives and strengthens us as a family. As we often now find ourselves crawling through the darkness, it's more important than ever to share our little pieces of light. In my sharing of medical information and stages of grief, I have yet to do that here.

Jackson is doing so well now. He is steadily gaining weight, which is hugely important because body mass is directly related to improved lung function- not to mention gives him the reserves he needs to fight off any illness he may encounter. The more he rounds out, the more I discover his resemblance to Emily at the same age- as evidenced in the side by side photo of both at 6 weeks.

Speaking of which- SIX WEEKS! It seems a little triumph in and of itself. We have now been dealing with his diagnosis over half of his little life. I don't know why that's a comfort, but it is.

Emily is doing better. She still worries about her baby brother, but the therapist says her emotional state is very normal and healthy for her situation. She also said the things we are doing to help her cope (limit the information about his illness to an age appropriate level and amount, let her help with his care and treatments if she wants to) are the best things for her. Whew! She is sweet and tender with him, naturally maternal. Although I wish I could spare her the worry, I am so grateful for her sweetness and special care of her baby brother. Her readjustment to life in general after a new baby and the news of his illness is pretty astounding. Every day I am so incredibly thankful for her. Monday we should have the definitive results of her sweat test, which she told me it was okay to post pictures of so people could know what it looked like.


When you're crawling through the dark, feeling your way through, don't forget the little pieces of light. Put them all together in one place for enough light and warmth to survive. I don't mean to wax metaphorical...but apparently I did.

How could I forget? Jackson started smiling this week! I am finding that capturing his smile at it's very peak is hard to do, but rest assured it's not gas or a reflex(I may have to fight you if you say otherwise) It may seem a small reward for all we've been through already, but it's more than enough.

Thursday, April 22, 2010

The Sort of Thing...


...that happens to other people. I remember when a dear friend of mine learned her infant son would have to have a highly invasive craniofacial surgery. My heart broke for her, and I wished deeply that there was something someone could do so that she didn't have to go through such an ordeal. Tears stung my eyes as I thought about what she must be feeling and how unfair her situation was. However, we all know that gut-twisting sense of empathy doesn't come without it's own sense of relief. A secret, guilty, "thank God that's not me" kind of feeling that we are loathe to admit. It's normal. It's natural. If nothing else it makes you appreciate your own good fortune in a way you may not have before. But what do you do when it's not the sort of thing that happens to someone else?

The Importance of Being Earnest

I've been thinking a a lot about what I'm sharing here in this little corner of cyberspace. I feel like it's critically important to be honest about the emotions we're going through. I know my family is not the first or only to stand in this spot and for those to come and those who have gone before I feel like I should be completely honest. Sometimes that means saying or feeling something not completely rational or acceptable. I hope you as my beloved reader (and I do love you, I can't believe you get enough out of my words to keep coming back, hearing that you do makes me feel justified somehow, bolstered) can understand I am relaying the knee-jerk reactions of grief and a journey to understanding my own feelings. I never know when something is going to strike a nerve or bring me to tears. Or as the man who so unfortunately cut me off and curb-checked his powder blue Lexus wagon in his desperation to claim my parking spot discovered, make me angry. On that occasion I leapt from my perch next to Jackson's car seat and accosted the man in the parking lot, asking him loudly if he got a good spot, if it felt good to cut me off and almost hit my car. Frankly the guy was an asshole, but I'm not usually one to leap out of a (slowly) moving vehicle and verbally assault another driver.

I'm also not the type to spurn the support and kind words I have been overwhelmed with since Jackson's diagnosis, so it caught me off guard when a particular turn of phrase began to touch a very raw nerve. Why would such a complementary consolation make me simultaneously angry and sad?? Why would I have such a reaction to well intentioned words? It took me a while to figure it out- why "you're such a strong person/amazing mother/etc" made me want to climb the walls and scream. And I figured it out when I discovered that what I wanted to scream is "I don't WANT to be strong! I don't want to do this!" I'm angry that I HAVE to be strong all the time. I'm angry that this isn't just something I have to get through to see better times, this is just how it's going to be. This sadness and worry and fear is not something I can put down, and it's not something my son can shake off. There isn't and end in sight to look forward to. The only hope is diligent preventive maintenace, agressive treatment of infection, and a relentless search for a cure. Somehow I began to feel as if 'you're strong' meant someone thought I was handling this easily...and I'm not. I have a very strong habit of not showing my struggles, not sharing any weakness or admitting to strife until after the fact, when everything's kosher again and I can talk about it like it's no big deal. It took nearly two months for me to tell my own mother that I'd left my first husband. I can't do that now, the news, good or bad, won't wait. I am glad though, that I figured it out. I'm not ungrateful for the words of comfort offered by people who care, I'm simply bumping into my own psyche in a dark room and being forced to feel it out.

Saturday, April 17, 2010

A Return to Normalcy...or at Least a Visit




This week we managed to return to our schedule (sort of). I attended Emily's ballet lessons and met with her teacher, went to softball games, wrangled homework and cranky mornings, and we even all went out for crawfish Friday night with friends. It felt so.....NORMAL. We were just a family, a bit frazzled by the lack of sleep that comes with a newborn, but resuming life with a new family member. Then there were the moments that remind me that things are different now. A sense of panic when a stranger reaches to coo over the baby. I remind myself that it would not be ok to yell at them to go wash their hands! ( a little rubber sign does dangle from his car seat carrier stating "Please wash your hands before touching mine", but some people get so excited over a tiny squishy bundle they simply don't see it.) And those moments are nothing compared to pushing the stroller around the elementary school grounds. In those moments I empathize with anyone who has germ phobias.

See, I've never been that person. I've always happily believed that the interactions and time spent with people are far more important than appearances. That's a flattering way of admitting I'm a happy-go-lucky slob. I've never believed in hand sanitizer or avoiding the dirt, dodging contagion never particularly worried me, and I've never boiled a bottle to clean it in my life. I liked that the fear of those things had zero impact on me. Now...well, I'm making some monumental adjustments and trying to maintain some sanity at the same time. I can not, and have no desire to keep my son in a bubble. I know he will pluck a lollipop from the dirt and direct it to his taste buds someday, and I know he'll attend a low key booger tasting at least once in his young life. I am the type to relish a good mud puddle, I mortify the 'proper lady' set with my hearty encouragement of hands on environmental exploration. *sigh* The pendulum had to swing a little as I now have to sanitize nebulizer cups daily to discourage any bacterial growth. Hand washing has also become a necessary habit, and I have invested in antibacterial soap and paper towels for every available sink. Bacteria is the bane of a CF lung's existence. Striking the balance between keeping my child safe and not keeping him isolated is going to be tricky. I've started by observing the behavior of the human male offspring and it appears I will be spending half of my life biting my lip and clapping a hand over my eyes...


Second Semester Cystic Fibrosis:

While we're on the subject of 'normal', we are adjusting to our new routine. I am trying to space out the dosing of Jackson's medicines so his little body doesn't have to process them all at once. A rundown of his daily routine, and what each of the medicines does;
6am-
Enzymes and feeding
Jackson's pancreas is clogged with the notorious thick and sticky mucous of CFers, so digestive enzymes aren't able to pass to his intestines and break down the nutrients in his food. This is called Pancreatic Insufficient Cystic Fibrosis, and 85 to 90% of CF patients fall into this category. Each time we feed him, we start by sprinkling a capsule of enzymes on a tiny spoonful of applesauce. The granules have a special coating that hangs on through the acidic stomach environment and only opens them up in the alkalinity of his intestinal tract to do their business.
Zantac-
Yep, the anti reflux meds. And they changed his life. One quarter of one milliliter twice a day helped Jackson's intestines to revert to the alkaline environment they need to be ( the acidity of his stomach had extended itself to the intestines previously, causing him a significant amount of pain and gas), also helping his enzymes function at peak capacity and enabling him to get the most out of his food. Were he one of the many CF babies who struggle with reflux, it would have nipped that in the bud, too.

9am-

Enzymes and Feeding
Salt Supplement
Because of the faulty transfer of salt and water in Jackson's cells, he loses much more salt in his sweat than you or I, and we replace it for him. Right now he takes 1/8 of a teaspoon each day, and thanks to the kind folks at Whataburger, we have a drawer full of pre-measured doses. I mix it with about 2mL of water and squirt it in his mouth. I've perfected doing it so he doesn't spit it out, much to his chagrin. Of all the things ha has to do and take in a day, this is his least favorite.

12 Noon
Enzymes and Feeding
Albuterol and CPT
Albuterol is a bronchodilator, meaning it opens up his airways to allow more airflow in the lungs. It is administered via nebulizer, popularly known today as a 'breathing machine'. He has a special attachment to his that's a pacifier, so he om-noms on a paci while the meds go straight up his little nose, forming tiny beads of condensation on his nose and eyebrows. We follow up the treatment with CPT, or Chest Physiotherapy. I use a little rubber cup called a chest percussor and manually tap on his chest over his lungs to loosen and dislodge the mucous. When we piggyback the albuterol with the CPT, Jackson usually coughs up some of the junk, and feel pretty good afterwards.

3pm
Enzymes and feeding
Vitamins

Jackson has prescription vitamins. His body has a hard time absorbing some fat soluble vitamins, so he takes a vitamin supplement. I think these vitamins are pretty cool because they've managed to molecularly wrap them in water, so that the body thinks they're water soluble and digests them more easily. Ta Da! The only problem is that they're super concentrated and stain everything they touch a carroty orange...ultimately including his diapers. The diaper service hasn't cut us off though, so I guess there's no downside!


At this point we're done with the first med cycle of the day. After that, Jackson still eats and takes enzymes about every 3 hours, and gets another dose of Zantac and another Albuterol and CPT session. When you add this to the normal eat, sleep, poop routine of any five week old it takes up most of the day. Sometimes I realize we haven't left the bed yet and it's noon, but not because we're watching Wendy Williams and eating bon bons. It is getting easier, and I'm working on the logistics of travel and outings, and before long it will all just be second nature. I also journal all of Jackson's feedings, meds and diapers so that if something changes, the doctors will have a perfect record of how it all went down.


Okay, if you've read along this far, you get a cookie! I want this blog to be educational as much as an emotional outlet. I delude myself that someday it will help someone in a big important way, so I put a lot of effort into information and accuracy. Now in addition to having DONE all these things before I cleaned the house and made dinner today, I've won myself out in the relaying of it all. I'll try to reward your loyalty by sprinkling in oh so cute baby pictures.

Monday, April 12, 2010

On Positivity and Hope


Upon reading over my words of the past two weeks I realize how resounding the sadness is in my posts. While I believe it's understandable and the blog has given me an outlet for a lot of the things I can't quite say out loud, it leaves something to be desired. I have spent the last couple of years of my life working very hard to be a positive person. Being of the stubborn sort I like to fancy that nothing can get me down. You can throw bad days and bad luck at me, but I'll be damned if I don't just bounce back. Not only that but I believe in positivity on a molecular level. I hold to this belief like a life raft of science in the sea of spirituality, and desperately, feverishly apply it to my son's future. I will never let him sink into his disease and let it take hold of him, I will battle the damn thing with sweetness and light and beat it into submission.

Needless to say my self-perceived invincible optimism has taken quite a beating. I'm surprised at the depth of grieving one can go through without actually suffering a death. And I'm surprised at the outpouring of empathy the world seems to offer us in this moment.

While I freely admit I've stumbled upon real, enduring sadness, I refuse to allow it to gain any kind of real hold on my outlook. I may worry and cry and fret sleepless hours away, but I will not succumb to it. If my son and my family didn't bring me such abiding joy, I wouldn't feel so intensely the threat of their loss. And that underlying joy will continue to be my great motivator, through all of the things I anticipate and all of the things I fear.

Saturday, April 10, 2010

What Goes Up

Eleven days. We are eleven days into this now, and somehow I still expect it to suddenly make sense. Like in the next moment, I will be adjusted to the reality, and I will understand the scope of its affect on our lives, and I will know how things are going to play out. Or at least that I will feel something other than an earth swallowing sadness or lighthearted denial. I have figured out that I can go two or three days without crying. With the fundraising efforts and keeping the unbelievable quantity of Jackson's and our supporters informed, I can stay busy for that long, and as long as it feels like I am DOING something to save him, it can't swallow me up. But on Friday we went back to the hospital for his first fallow up. Just pulling into the garage of the castle-shaped home of joy and sorrow put me back to diagnosis day. The grief welled up in me with an unexpected force as we walked down the hall, past Radiology, and up the elevators to the CF clinic. The waiting room wasn't empty as is was last week- instead crowded with children and parents in varying degrees of illness. We found a place to sit that carefully kept Jackson above the recommended minimum 3 feet from all the other patients. We held our breath as we undressed him at the Vitals Check as he laid on the scale and the nurse converted his weight from grams to pounds. We cheered when he clocked in at 8lb, 3 oz, a 12 oz gain, and again when he measured half an inch longer than his last appointment. I literally wanted to go up to the roof and shout. The appointment was brief and informative after that, we discussed which chest sounds should be alarming, got some medicine to help alleviate the gas pains that were dominating his hours and preventing any sleep for any of us. We were thrilled as we left, the news was all good at this juncture.

It wasn't until I began to plan out his daily medicine schedule that I began to choke on my tears again. (I'll explain all his meds and what each one does again later, as I want the blog to be as educational as possible.) Breathing treatments twice a day, Zantac twice a day, Chest PT as needed if breathing treatments don't alleviate coughing, enzyme capsule at every feeding, vitamins and a salt supplement each once a day. It just seems like so much for a little guy who hasn't even finished a month of life yet. Too much. I tried to get angry, just to have an outlet for some of the emotion. Angry at the pregnant women in the store who had experienced none of this, angry at irresponsible teenage mothers with perfectly healthy babies, angry at all the other shaded carriers in grocery carts. I tried to get angry at my genes, angry at God, or angry at my statistical fate, all to no avail. I can't bring myself to blame the blameless.


My worry is compounded by the apparent affect Jackson's illness may have on my daughter. We have explained to Emily that her brother has a sickness, that he will a=always have it, and it will mean he has to do some things differently. We will have to be more careful about washing our hands and things like that, and he will take lots of medicines to stay healthy. I thought any more than that might be too much for her to process. I don't know if someone at her father's house has been discussing it or someone at school said something- I've certainly not uttered or entertained thoughts of this nature myself- but somehow she has gotten the idea that her brother is going to die, ultimately leading her to have a panic attack at school. Anyone who knows Emily will vouch for my saying she is best summed up as a bouncy ball of sweetness and light. She is tender and maternal and a born care taker, and she is very worried about her brother. Her counselor at school is putting her in contact with another student who has a sibling with CF, and I think and hope that helps her grasp what it may be like to live with. We are also talking with the Child Life specialist at the CF clinic about some play therapy to help her cope. I still worry that having Jackson's medical schedule dominate so much of the day she will feel left at the wayside. I've managed to pencil in Mom and Bitty only time at least weekly to try to maintain my bond with her.

There is so much more to share...I consciously decided to share this journey, no matter how painful. There have been moments I second guessed that and executive decisions about privacy since I began, but I think it's important enough to share. Most of us let slide details of our personal lives for the frivolity of facebook, or twitter, or the like- certainly this merits a bit more than that. Someday someone might find themselves in my shoes and eek out some comfort from my words. Maybe you have learned by reading this about Cystic Fibrosis. Inform and educate, certainly that is worth something.

Sunday, April 4, 2010

Worth His What?? (Cystic Fibrosis 101)

Since I've spent the last several days poring over books and websites (reputable ones ONLY, the CFF website, the Mayo Clinic, WebMD... NEVER EVER EVER go to wikipedia or just a random Google search)I have a lot of knowledge now, and am pretty sure I can give you the breakdown of what Cystic Fibrosis is in laymans terms.




First of all, it's a genetic condition. In order for a child to inherit the disease, both parents have to be a carrier of the faulty gene. Mike and I both come from large families with no history of the defect, so we didn't even know we should be tested. But as it turns out, we both carry a faulty gene, Delta F-508, the most common of the over 1200 known gene mutations that cause CF. Any child we concieve without medical intervention has a one in four chance of having the disease, and a 50% chance of being a carrier. If we have the means to have another child in the future (which we very much hope to do), IVF can select embryos that are not infected and implant them. Not only am I not ready to think about that, it has brought about some unanticipated moral questions in my mind about discarding fertilized embryos.

The Doctors and specialists use the term 'basic defect' when it comes to the root cause of the troubles CF brings on. This basic defect is in the epithelial cells, a channel used to transport salt and water through the cell walls. (these channels are called CFTRs, (cystic fibrosis transmembrane conductance regulators- five times fast, I dare you) They aren't able to transport salt normally, and as you know, salt dehydrates things, in this case, mucus. The dehydrated mucus becomes thick and sticky, and hard to get rid of. It provides a fertile breeding ground for bacteria in the lungs, leading to frequent lung infections that are hard to clear, again due to the difficulty of removing the thickened mucus. The other primary trouble lies in the digestive system, where the pancreas is unable to transport digestive enzymes to the intestines, leading to malabsorption of nutrients and if untreated, malnutrition. Many CF kids have a hard time growing and gaining weight because in addition to this, they have at least double the caloric needs of another person of the same body size. Body mass has been directly linked to improved lung function, so it's hugely beneficial that Jackson was diagnosed and started treatment so early, before 'failure to thrive' became part of our nightmare.



In the 1930's children with CF rarely made it to their third birthday. I'm happy to tell you that's no longer the case. The projected average age of survival is today in the late 30's. Yes, that scares me beyond words, but I have every reason to believe that 30 years from now CF will be CURED. There are genuinely thrilling things going on in the world of treatment, including the drug VX 809, currently in clinical trials, that has the potential to FIX the basic defect. There arehuge advancements going on in treatments too, drugs that thin the mucus, aid digestion and nutrient absorption- as a matter of fact Jackson's vitamins were just recently made available for prescription, and the technology behind them is amazing. Perhaps you can understand why my new full time job is fundraising for CF research? For more information on the Drug Development Pipeline, go here: http://www.cff.org/research/DrugDevelopmentPipeline

Currently Jackson takes Enzymes before every feeding to help him digest his food and suck up all the good stuff so he can grow. And now we know why he wasn't the behemoth he was predicted to be at birth. He also takes the space-age vitamins, and a salt supplement each day to replace the extra salt he loses in his sweat. For his tiny body, he takes a Whataburger packet of salt a day, no kidding. As he grows, we will do everything we can to get him into clinical trials and to further drug development. More than the desperate mother in me believes we can cure this disease in his lifetime. I think knowing you're likely to outlive your child is just about one of the worst things that a parent can go through, but the misery is tempered by the potential to change that outcome. I want my son to grow old, and I want him to be able to have his own children if he chooses. Without medical intervetion he only has a 2% chance of doing that right now. I can change that, I can spread awareness and raise money and make a real difference in the most important fight of my life. And that's exactly what I'm going to do.

Saturday, April 3, 2010

Introductions I'd Rather Not Make


My name is Amy. I could give you the typical intro; I'm a 30 year old mother of two, twice married, aspiring artist and semi-reformed hell raiser whose family falls delightfully outside the societal norms, etc, etc. But I'd rather get down to brass tacks. I spent most of my life narrowly sidestepping real personal heartache. I consider myself tough, and have had plenty of 'hard times', but usually squeak through on dumb luck and bull-headed, teeth grinding stubbornness. I've never really had to face any heartbreak I didn't bring on myself. I always felt a little guilty for my good fortune, I've frequently told my husband "nobody gets what we have, nobody gets to be this happy". And in spite of everything, I still feel that way.



A little back story...a near tragedy in the family led my husband and I to realize that nothing is guaranteed, and we weren't going to wait any longer to have children. "Life is too short!" After a perfectly normal pregnancy with all the normal annoyances, we had a shockingly beautiful baby boy on March 11, 2010. We were beside ourselves with joy, and set out toward a life as a family of four. I asked my husband (who I knew wanted more kids) "so, whenever I say I want another one, we'll just do this again??" We knew we'd at least have to wait until he found a job. A recent grad of Fire Academy and Paramedic school. That meant that against our fiscally conservative (fiscally only) views, this little guy had been born into the medicaid system.

We hate going to the Medicaid Pediatrician office. I mean don't get me wrong we're appreciative of the care, but there are a lot of people there who aren't so much.
Unkempt runny nosed children fill the waiting room while we hover in the corner sheltering our newborn son behind layers of blankets and a well-guarded car seat carrier. Jackson had a little more than normal jaundice and so we were making weekly trips there to monitor his bilirubin levels. Two days before his last appointment I got a phone call from the office- not the automated appointment confirmation system, a "it's critical that you return our call today" call. Determined not to panic at the unusual message, I called back. I was patched through directly to the Doctor herself. She informed me in her thick Latin accent that Jackson had tested positive for one of his screenings at the hospital and we needed to see a specialist to rule out a problem and they would be calling me shortly. I pushed down the lump in my throat and assured myself it was a false result. I mean, when I went to planned parenthood I once tested positive for Cervical CANCER, because they left my pap slide exposed to the air too long and it damaged the cells. So surely this was some sort of mistake in the over-taxed public health care system...right?

It didn't take long for the Pulmonologists office to call. About 15 minutes if I recall correctly. I tried to hold on to my 'office voice' (poised, professional, and polite) and not let it crack as she informed me that after the initial test had shown elevated IRT levels- the initial indicator of CF, that they had done DNA testing and discovered two gene mutations indicative of Cystic Fibrosis. In my heart pounding panic and attempts to stay calm I didn't hear that as a definite diagnosis. But I did get that it was serious enough that we would be sitting in the Pulmonology clinic at Cook Children's in the next three hours. I barely had time to wash my hair before we had to get out the door. I had to call my ex husband to pick up my 7 year old at school and take her to softball practice. My brain went into serious denial mode and began to catalogue every reason this couldn't be possible. Cystic Fibrosis is genetic- you can't get it unless both of your parents are carriers. Mike and I have no family history, and we both come from big families- someone would have turned up with it by now. It just has to be a mistake, and when they figure it out we'll go home and make a drink and toast to the scariest misunderstanding of our lives and be thankful for a healthy baby. Yeah, I would be making time for a cocktail after this.

The thing about children's hospitals is this: they're awful and wonderful all at once. Candy colored puzzle pattern hallways can't disguise the fact that they are hospital hallways, and that the parents who have walked these halls before me shared the same feeling of being sucker punched with razor edged brass knuckles. the elevators are a rising and falling collection of tragedies, families rolling their babies in and out, some with problems I can't even stomach thinking about. A child of maybe four is rolled by in her wheelchair by her father, and for that family it's business as usual as her head lolls off to the side and a greeting nurse dabs the drool off of her cheek as we pass by. "Stop whining and feeling sorry for yourself, look what they have to deal with"

Before I can get through the first two pages of the thick patient info packet in the waiting room we are ushered downstairs to radiology, where nobody second guesses my tear smacked, red face and the nurses greet my tiny son by name as we prep him for chest x rays. Other than his distinct objection to being stripped naked and cold, he isn't phased by the process. Two nurses hold his arms and legs and position him as Mike and I are ushered out the door to avoid unnecessary exposure. The x rays are over quickly and we thank everyone on our way back up to Pulmonology. There's no waiting there either and a little pang enters my heart in the CF clinic as I feel pain for the parents whose lot it is to bring their children here regularly. We make a pit stop for the vital signs check just past the front desk. Jackson is just too tiny still to get a reading from the infant BP cuff on his leg, so me move on to weight and I'm suddenly more afraid and confused as the nurse reports that he weighs only 7 lb, 7 oz. He's lost 1/2 lb in the last week, and is now back below his birth weight. Somewhere in the dark places in the back of my head there's a siren and a red light flashing that I'd REALLY like to run from. My husband, Mother in law, and I file into a patient room and are told that the Doctor will be in shortly, and then the dietician, then the social worker, then the CF coordinator and the nurses...I can't help thinking they sure are going to a lot of wasted effort just to rule out a problem we don't have. Is it OK if a Med student sits in on this one? Sure. Another body in the room. My father in law arrives from Dallas, another body in the room. Doctor, Nurse, student, Mom, Dad, Mike, myself, and a tech doing a sweat test on my sleepy newborn in his white fleece blanket, all in a clown car patient room.

I don't really know what the hell order things happened in in the next few minutes. The bow-tied doctor began to talk to my Mother in law and they were using doctor words...I'm certainly not dumb but I'm also not a trained medical professional. I heard somewhere in the conversation for the first time the term 'deltaf508'. The doctor was looking at me as he talked. I tried desperately to get outside of my own body as the atmosphere was sucked out of the room and every one's faces smooshed into unreal shapes. It took me a while to realize I wasn't yelling. It took me a while to realize everyone was sobbing. It took me a while to realize that I couldn't rationalize the moment and I couldn't look at it from the outside and recount "stages of grief" to assess what I was going through. But I would do anything to get out of that moment, so I kept trying to be a third party. My son, my brand new baby who had only shed his umbilical stump less than a week ago, who had never slept through the night, who had only had two real baths in his lifetime....my son was sick. He has a disease, and it won't go away. People die from it, many as children. My son, who slept through his sweat test as all this happened. My son.

I'm not going to share the remainder of this moment. It's too personal, too raw, and I share it with others who may not be as enthusiastic as I am about letting the public in on it. There were parts of it that I will spend the rest of my life trying to forget. They don't get to go in print.

We moved on to discuss a plan of action, and in turn met with the social worker, dietician, program coordinator, respiratory therapists, and nurses. We learned the ins and outs of the disease, put together a treatment plan, and listened to a LOT of information. I can't say enough for the entire staff at Cooks. They're unbelievable and somehow managed to be the straight man as we cried though the day without seeming like assholes. They were treating our son and making sure we were taken care of too. I'm glad we have them. They made a binder with Jackson's name on it, filled with his treatment plan, a staff directory,and a book outlining his disease for us to get familiar with. We were supplied with multiple sets of fresh nebulizer cups for when he begins to need breathing treatments, and prescriptions. I'll tell you about the prescriptions later, when we get to the breakdown of what Cystic Fibrosis is and how you treat it. I have a lot to say but can only do a little at a time right now.

This was day one. Since then I've oscillated between disbelief, hope, and soul- crushing despair. There have been a million times I tried to grasp "this is your life now". I've gone about the day to day. I've worried about how this will affect my older daughter. I've thrown myself headlong into fundraising for CF research. I've spent quiet moments before dawn singing softly to my baby like any mother new mom. I've fought off fear and desperation and been inspired beyond expression by the empathy and real desire to help that has been heaped on us by our loved ones and strangers alike. I remember the social worker telling me that it was okay to be overwhelmed, that in time, this would become the new 'normal'. I found that strangely comforting and also bizarre. We're not even close to feeling 'normal
yet, but knowing that it won't always hurt so goddamned much is nice.

I'm not sharing any of this because I need a response, or comfort, or pity. As a matter of fact I'd like to ask that pity be left at the doorstep because it's my least favorite emotion. I know that I can handle this, I'm not sharing the story looking for sympathy. I'm sharing the story as a real look at how it was and how it is to live with a chronically ill child. I know people are curious and I don't mind being forthcoming and honest. But I need you to treat it more like National Geographic and less like The Notebook, if you don't mind. I have a lot more to share just to begin with, but somehow putting it into words is physically exhausting. For now I'm going to crawl back in bed with my husband and cuddle my baby and pretend like there's nothing else in the world. I'll be back with CF 101 later.