Gene Therapy in LA: Days 1-3

A few weeks ago I explained gene therapy. Now we’re here in LA! Here’s what we’ve been through so far. . .

Flying to LA

Our adventure began with flying out to LA from Toronto. Air Canada and Aeroplan deserve a HUGE thanks for putting us in first class.

Let’s pause for a moment to talk about that.

feeding first class

First class is THE SHIZNIT, you guys. UN-FREAKING-BELIEVABLE. If you thought it was way better than economy, you had no idea. It is better TIMES INFINITY.

I felt like a zillionaire in first class. The second you sit down they come ask if they can get you something, like you’re the Queen of England. You get to sit in these individual pods that can turn into full beds — you can stretch out and sleep through the whole flight if you want. You get to choose a gourmet meal from a menu. They bring you a warm washcloth before you eat. The stewardesses are basically your servants, constantly offering to bring you things. They bring you warm mixed nuts and Lindt chocolate bars, even after you say you’re fine. You get your own TV where you can choose from a selection of new movies (I chose Cinderella. I bawled like a baby.)

first class baby(Felix got a whole pod to himself.)

This must be what it feels like to be wealthy beyond measure. (Remember that we are a couple of Mennonites — a woodworker and a stay-at-home mom. This is luxury beyond our wildest imaginations.)

Our favourite part was how they close the curtain at the back to hide the unsightly riffraff in coach.curtain first class

Out of our sight, peasants! We don’t even want to see you in our peripheral vision! (<– nod to Brian Regan) (“Bring me the head of a pig! And a goblet of something cool and refreshing!”)

They’ve ruined air travel for us, because economy will forever feel like a prison sentence compared to first class.

Anyway. We spent our first night in a hotel, and on Monday morning headed over to UCLA to have Felix admitted.

night in hotel

Day 1: Consents

Monday was our first day in the hospital. Ronald Reagan UCLA Medical Center is a huge, gorgeous hospital. The grand front atrium feels like the entrance of an art museum. The cafeteria is super-classy with lots of delicious, healthy options. Felix was admitted in the Mattel Children’s Hospital, which is brightly and handsomely decorated. His room is spacious and attractive. The staff is all super-nice.

children's hospital

The first day was just going over consent forms (dozens of them!), for the study. The doctor went over exactly what was going to happen every day that we’re here (given everything goes as planned). It was exhilarating and terrifying at the same time. We’ve wanted this for so long and it’s finally here!! But oh, what scary risks we are taking. It’s our best choice, but it’s full of risk.

happy baby

Felix continued to be a happy, energetic baby all day. He had to stop eating at midnight for surgery the next day.

Day 2: Bone Marrow Harvest

Early Tuesday morning, Felix was taken down to the operating room. He was still in a good mood despite not having eaten in seven hours. Crazy baby. I suddenly wept as I said goodbye. What an emotionally-charged thing it is to let strangers wheel your baby away to put him to sleep and take out his bone marrow.

ready for surgeryReady for surgery.

We had breakfast while we waited, and even hopped into Whole Foods down the street.

The procedure took about two hours. When we came back to find him in the recovery room Felix was just starting to wake up. The doctors said everything went really well and they got lots of bone marrow.

I held him for the next two hours and he continued to mostly sleep. He got a blood transfusion to make up for all the blood cells they took. We were allowed to give him some sugar water to drink, which he eagerly took.

The rest of the day was very, very long and hard. Felix was in a lot of pain. He spent the next five hours straight screaming. We took turns holding and comforting him though nothing helped. He was starving, but the breathing tube they’d inserted during the surgery had hurt his throat — he screamed every time he swallowed. Finally they gave him some morphine for the pain and he slept.

sleepFinally in a morphine-induced sleep. This is not how our baby usually looks.

We tried periodically to feed him throughout the day and night but he refused to take anything. He wouldn’t even close his mouth to breathe. When we gave him his oral medicine in his sleep he cried out every time he had to swallow.

It’s torture to watch your children suffer.

Day 3: Chemo

It’s Wednesday morning now. Last night at midnight Felix got his chemo (busulfan). (It’s just an IV drug that went through his central line.) It was dosed over three hours.

chemo

Every time Felix would start to wake up he would cry in pain (still from the surgery, we assume), he would get another dose of morphine, and he would fall back asleep. He still hasn’t eaten in over 30 hours. We still don’t know if the chemo has had any negative side affects so far, or if so, what they are.

He just woke up smacking his lips and looking more like himself so we’re feeling hopeful. Ben just offered him the bottle and he took it!

So it’s time to run. Hopefully today is better than yesterday.

In the meantime, Felix’s bone marrow cells are in the lab, being treated with the corrected gene and being encouraged to grow.

Prayer Requests

  • Pray that the side effects from chemo are minimal and that Felix doesn’t suffer too much more.
  • Pray that all goes well in the lab, and that Felix can get his corrected cells tomorrow (Thursday).
  • Continue to pray that there are no complications and that gene therapy cures Felix, so he can live a long and healthy life!

Thanks again for all your support!

*Read Part Two here*

What is Gene Therapy?

Felix 7 mos

As regular readers know, we’ve enrolled Felix in a research trial for something called gene therapy, to treat the life-threatening disease called ADA-SCID. Gene therapy is a cutting-edge medical therapy still in the experimental phase, though outcomes are looking really good so far. Although it’s terrifying to enroll your child in an experimental therapy, it’s by far his best chance at survival.

We are leaving for California in ten days (!!!), so I thought I’d give a better explanation of gene therapy, from my understanding. (Obviously I am not a doctor; this is just the way I understand it, from the explanations I’ve received.)

To begin, you might need a bit of a better understanding about Felix’s condition.

Felix’s Condition

Felix was born with something called adenosine deanimase (ADA) deficiency. It is a genetic disorder of the immune system caused by abnormalities of a specific gene in the body.

The ADA gene is responsible for producing an enzyme called adenosine deanimase. The absence of this enzyme causes the body to accumulate certain toxic waste products, which kill off certain cells of the immune system called T lymphocytes. (T lymphocytes are a type of white blood cell which are necessary to fight infections caused by bacteria, viruses, and fungi.) Without T lymphocytes, Felix has no immune system, which is called severe combined immune deficiency (SCID). (There are many different kinds of SCID: ADA-deficiency is just one of them.)

If untreated, SCID results in death from infection, usually within the first year of life. There are also various other symptoms associated with ADA deficiency which vary from patient to patient. In Felix’s case, his breathing was impaired so that he needed oxygen support, and his liver was slightly inflamed. Other symptoms may have manifested over time.

Ways to Treat ADA Deficiency

In order to treat ADA deficiency, doctors have been trying to find the best way to replace that missing enzyme.

One way to treat ADA deficiency is to simply inject the patient with the missing enzyme (which is harvested from cows). Felix has been receiving this treatment since he was a month old – he gets two injections a week. It very quickly and dramatically improved his health, so that he was able to come off of oxygen, his liver enzymes went down, and he was eventually able to fight off a virus (CMV) that he acquired in his first weeks of life (and that nearly killed him). He has been fairly well for the last few months, though we’ve had to keep him in strict isolation to prevent any new infections.

The trouble with ADA injections is that its effectiveness slowly wears down over the first few years. Moreover, it is incredibly expensive (we’re talking $200,000 a year, for the rest of his life). It’s also not a perfect solution since it’s not human ADA and it’s not being made by the body. So we are still looking for a long-term solution.

Another way doctors have attempted to cure SCID (including ADA-SCID) is through a bone marrow transplant.

Bone marrow contains “stem cells” which make all of the blood cells in the body, including T lymphocytes. So if the patient had his bone marrow replaced with someone else’s that didn’t have the abnormal gene, he should be able to rebuild an immune system.

This has worked with varying success among many SCID patients. The best results come from a matched sibling donor, but you may remember that Lydia turned out not to be a match.

There are many problems related to bone marrow transplants, though, all of which tend to be more severe in patients with ADA deficiency. The major problem is something called graft-versus-host disease, wherein the donor cells attack the patient’s body.

So researchers came up with an idea that could get around the graft-versus-host problem: use the patient’s own bone marrow!

felix playing

Gene Therapy

That’s the idea behind gene therapy: a bone marrow transplant using the patient’s own bone marrow stem cells that have been corrected.

If we could insert the normal gene for the missing ADA enzyme into the stem cells which make T lymphocytes, then theoretically these T lymphocytes would no longer be destroyed by the toxic waste products, and the immune system could be restored. That’s gene therapy in a nutshell.

Different forms of gene therapy have been tried in a few different places in the world – Milan, Italy; London, England; and Los Angeles. Felix is enrolled in the most recent trial in Los Angeles. Here’s how it works.

How It Works

First you harvest the patient’s bone marrow.

Felix will be placed asleep under general anesthesia for the bone marrow harvest. Two needles go into the hip bones to remove about half a cupful of bone marrow, some for backup in case anything goes wrong.

The bone marrow cells then get taken to a lab where they will be treated with the normal ADA gene.

To get the ADA gene into the stem cells, they use an altered virus called a vector. In this particular trial, they are using an altered HIV virus, also called a lentivirus. Yes, that’s right: we will be purposely exposing Felix’s bone marrow cells to an altered HIV virus, because they’re so effective at getting into cells. (The virus has been altered so that it cannot reproduce itself and all the HIV genes have been removed.) We will essentially be “infecting” his cells with the corrected gene! (Yes, I get freaked out every time I think about it.)

The bone marrow cells will be grown in the lab for two days. Once the ADA gene is in a cell, it is permanent, and the cell will pass the ADA gene on to all blood cells made from that cell, including the T lymphocytes that need to gene to function properly.

Meanwhile, Felix will get treated with chemo to kill some of the stem cells in his bone marrow, to “make space” for the gene-modified cells he will get back. Fortunately, the type of chemo he will get is some of the least harmful kind, and he will get a much lower dose than most transplant patients. He shouldn’t suffer too many side-effects (no hair-loss, minimal nausea, etc.). It takes a week or two for  the chemo to really wipe out his cells.

If everything goes well, two days later he will have the corrected stem cells infused back into his body. This just works like a normal IV. After that, it’s a matter of waiting for the corrected cells to multiply and take over.

Watching and Waiting After Gene Therapy

We will be in LA for  the span of about a week, if all goes well. Then we hop onto a plan (yes, a regular commercial flight — remember, it takes a week or two for the chemo to take effect) and head back for Ontario, where we will be admitted in the hospital. Felix needs to be closely monitored in hospital for at least a month to watch for infection, since the chemo will have wiped out much of his little immune system.

Once he has some of his white blood cells back, he can hopefully go home. We will then remain in isolation at home for many months (anywhere from 4-12) as we wait for his new immune system to build up.

Our biggest fear in all of this comes from his CMV (cytomegalo virus). It is currently undetectable in his blood stream, but will probably come back at some point after the chemo has wiped out his immune system. It is a very serious virus for immune-suppressed people and could pose a threat to his life. Felix is one of the first patients with CMV to be treated with gene therapy, so no one knows exactly what to expect. The hope is that his immune system will be able to reconstitute itself fast enough and strong enough to beat it, as it has before.

Felix sleeping - batbaby

Prayer Request

  • Please pray that Felix continues to be in good health until we make it to L.A. (He’s made it this far thanks to your prayers!!). We are traveling by commercial flight, after all!
  • Pray that there are no complications in gene therapy, and that it works!
  • Pray that through the right medications and a quickly-reconstituted immune system, his CMV stays down and doesn’t damage his organs or put his life in danger

Thanks so much for your support!

Update on Gene Therapy Plans (And the Year Ahead)

Last week we had a teleconference meeting with Dr. Kohn from UCLA, the head of the gene therapy trials.

It was an incredibly encouraging meeting.

Felix is number fourteen out of fifteen children in the trial. Of the twelve who have already gone through it, eleven are doing really well. (They’re still trying to figure out what’s behind the one child’s persisting problems.) It is so heartening to hear the doctors confidently talk about how they’ll continue to follow Felix into adulthood. Adulthood! My baby who wasn’t supposed to survive past infancy, alive and well in twenty years! What a thrilling thought.

Everyone feels very confident that this will work, that Felix will be cured.

The only disappointing news we got is that our gene therapy date is going to be pushed back a week. This is only to give the lab more time between patients. We’re now scheduled for July 6. In the grand scheme of things, a week is nothing; but we’re so eager to move on to the next phase! We were quite bummed out to hear it.

Dr. Kohn also gave us a bit of a timeline for the upcoming year. If all goes well, it should go something like this:

July 6-13: In Los Angeles for Gene Therapy

I plan on explaining gene therapy in depth yet, but for now: it’s essentially a bone marrow transplant using Felix’s own bone marrow, that has been modified to include the missing enzyme. (Update: gene therapy explained here.)

While at UCLA he will have his bone marrow harvested, will undergo chemo, and then have the corrected bone marrow cells infused back into his bloodstream. He’ll be monitored for a couple of days before returning to Canada. The whole thing takes about a week. (We’re taking a commercial flight both ways. We’ll be asking for your prayers that it all happens safely!)

Mid-July to Mid-August: In Hospital in Canada

Following gene therapy, Felix needs to be closely monitored for about a month to see what his cells do and to watch for infection. This can be done close to home — the results just need to be sent to UCLA. We’re not sure yet whether that will happen in London, where we already spent five months, or in Toronto, which is four hours away from our home.

It takes about two weeks for his platelets and blood cells to drop from the chemo, at which point he will be extremely susceptible to infection again — even more so than now. Which is why we’ll be staying in the hospital. It takes about two more weeks for his cells to go back up again and begin to stabilize. During this time he will continue to get the enzyme injections that he gets now.

Mid-August to Christmas: Strict Isolation at Home

If all goes well, we will return home after a month in the hospital and continue living in isolation as we are now. During these 4-6 months, we are waiting for the corrected cells to multiply and take over, enabling him to rebuild his immune system. We will stop the enzyme injections, with the expectation that his corrected cells will be able to make the enzyme on their own.

We might continue to have Lydia live with my parents since Felix will still be extremely immune-deficient for the first few months. So it’s likely that she will be living away from home for almost a full year. (This also breaks my heart.)

By Christmas/New Year’s, hopefully Felix can begin the process of being released from isolation. At that point we should know whether treatment was effective. We’d still have to avoid crowds or daycare (not that we would send him anyway), but perhaps he could take the occasional outing or see a few visitors.

And KISSES!! I’m counting on Christmas kisses!!!

* * *

So if all goes well, that’s the year ahead of us.

And here is a video of our sweet Felix, just as a reminder that he’s a real person and not just a medical diagnosis. (Sometimes I need that reminder when I hear about other kids going through medical procedures.)

This is how he wakes up every day — singing and celebrating life. He is SUCH A HAPPY GUY.

Thanks again for your continued prayers and support!

Life At Home in Isolation

felix and sophie

For those who are interested, I thought I’d talk about what our days, weeks and months look like, now that we’re in protective isolation with Felix at home. (For anyone who’s new around here, Felix has severe combined immunodeficiency, and we are awaiting gene therapy in the hopes that he will someday have a functioning immune system.)

The Home

entrance(Entrance to Felix’s room. On the shelf: disinfectant wipes, masks, gloves, calculator for calculating his formula intake. On the wall: whiteboard for tracking his feeds, hand sanitizer dispenser. Notice tape on the floor.)

Felix lives in our main floor living room — what used to be Lydia’s place space. He doesn’t leave that room. The bedrooms are upstairs, so we moved Lydia’s double bed down to Felix’s room so we can take turns sleeping near him. (We may not be able to co-sleep as we wanted, but we’re not going to make him sleep alone!)

We moved our couch to create a barricade, separating Felix’s room from the kitchen and allowing a path to the bathroom. We also put down a strip of tape on the floor as a marker. Nothing goes past that couch/tape without being sanitized first. This is Felix’s space, and everything inside it must be kept as sterile as possible. The tape also reminds us to sanitize our hands before entering.

Felix's isolation room

play areaWe also have hand sanitizers installed around the room, so we can sanitize before we touch him. When possible, I prefer to wash my hands in the bathroom, where we have a paper towel dispenser installed (since towels can harbour bacteria). (We are composting the paper towels to make it a little less terrible for the environment.)

Daily Routine

me and felix 1

Most of our day is pretty typical for a family with a new baby. You know — feeding, diaper changes, cuddles  (but no kisses). The biggest oddity is simply the fact that we never take him anywhere.

Then there’s the medical part of every day, which we have quickly gotten accustomed to. Every day we:

  • give him his oral meds — a preventative antibiotic. (I know. Antibiotics. But after everything else we’ve done to him this is the least of my concerns.) Since he still has the NG tube, we almost always give it to him through that so he doesn’t have to taste it.
  • take his temperature. A fever is one of the earliest, most obvious signs of infection.
  • change his IV bag (This is Ben’s job)
  • sanitize the room, including the floors. We have disinfectant wipes we use to wipe down his play mat, light switches, toys, door knobs, etc. We use a steam mop to sanitize the floors.
  • make his formula. I make a 24-hour batch every day and keep it in the mini-fridge that’s used exclusively for his formula and medication. His formula is this nasty, processed, powdery, amino-acid-based crap that I have to mix with sterilized water. Making the formula involves first sterilizing ALL the equipment in boiling water (whisk, spoon, measuring cups, scoops, etc) and the bottles in a steam sterilizer. I don’t do as much cooking anymore because making his formula every day is as much work as cooking a gourmet meal. And I need to keep the counters perfectly sanitary.
  • feed him. Lately, he takes almost all of his feeds orally, which is so awesome and exciting. It takes about an hour, though, every 3-4 hours. We just top up with the NG tube every once in a while to make sure he’s getting enough calories.

As I mentioned above, every night Ben and I switch off spending the night in his room. Back when he was exclusively tube-fed, he often slept through the night (the nurses would just come and get the feed started without waking him). But since we’ve transitioned to oral feeding, Ben and I have actually trained him to wake up at night to feed. (He doesn’t take in enough calories during the day alone). He usually wakes once or twice to feed, which we consider positively dreamy (after Lydia, who woke up 3-5 times a night for the first two and a half years).

schedule

Weekly Routine

  • Twice a week (Tuesdays and Fridays), I give Felix his injection in his thigh. Ben holds him. I hate it, but it’s not as bad as I expected. All of us have forgotten it ever happened within 90 seconds of the shot. And it’s the number one thing keeping him alive and well.
  • We usually have to put in his NG tube at least once a week because he somehow manages to pull it out. This, by contrast, is absolutely horrific every time and goes against every instinct I have. For better or worse, though, I’m getting increasingly numb to his screams.
  • Twice a week, Ben changes Felix’s IV tubing.
  • Once a week, we take the two-hour-long trek to the hospital for his clinic visit.

Weekly Clinic Visits

Clinic visits are the absolute worst.

The drive sucks because we’re not supposed to turn on the fan or A/C or open the windows. He’s supposed to stay under a plastic cover. We always break some of the rules because these conditions are unbearable. Somehow, his IV pump always acts up and we end up scrambling to try to fix it, terrified that his line will clog up and he will need to get a new one surgically implanted. Feeding in a moving vehicle is difficult to manage in a sanitary fashion, especially if we end up needing to use the NG tube.

The actual visit is always extremely traumatizing. It always involves me pinning down my baby while they do something horrible to him. He screams and flails with abject terror in his eyes until he can hardly breathe. It’s a nightmare. The last time, the sheets ended up spattered all over with blood as they tried to get blood from his heel because his central line clogged up. (They managed to unclog it later.)

It happens in a dim isolation room with no windows, which doesn’t help. It’s a cold, stark, medical-looking room where strangers in masks walk in and out and hurt him.

Then we drive home, picking up fast food on the way because he can’t leave the vehicle.

BUT it’s only one day a week. Once we’re home and comfy again Felix is back to his smiley, goofy self.

Lydia

Lydia watermelon

Lydia visits once a week. We even have her sleep over. It’s difficult because she can’t be in Felix’s room — she can only peek over at him. And Ben and I can’t ever be with Lydia together — we have to take turns. BUT: it’s infinitely better than in the hospital when we had to be in separate buildings.

Her visits are the highlight of our week, though. We walk to the park, read books, swing in the back yard, and play downstairs where we’ve set up a new play area. We do science experiments, play with play-dough, and practice the phonetic alphabet. She’s gotten so independent, she will happily play by herself downstairs for long stretches of time. It’s heavenly, just like old days.

We also visit her throughout the week at my parents’ house, too, and usually have Sunday lunch together.

She’s still having a great time living at Grandma and Grandpa’s. She spends her days helping in the garden, terrorizing the kittens, jumping on the trampoline, dancing with her aunties, and playing hide-and-seek with her uncle. And generally just wearing everyone out, like she used to do with us. :)

Next, I’m hoping to give you an update on our gene therapy plans. Thanks for sticking with us!

How Far We’ve Come

Felix 6 months

In the days and weeks after Felix’s diagnosis, I lost my desire to eat. Food just had no appeal. I remember thinking, Why bother eating. My life is over.

It’s been almost six months. We’ve come a long way.

To make note of our progress, Ben and I often remind each other of what horrors we’ve been through.

Remember when Felix used to projectile vomit after almost every feed? [Now he hasn’t thrown up a single time in months.]

Remember when he was hooked up to oxygen, an IV pump, and the monitor, so we could hardly hold him? How he used to lay in his hospital bed for hours and hours? [Now he’s just hooked up to a portable IV pump, which we can carry all over the room with us. He can roll around on the floor and sit in his Exersaucer . . . he can do all the things normal babies can do. Except leave the room.]

Remember when we tried tirelessly to feed him every three hours, day after day, and we could hardly get him to drink 1mL? And how we celebrated when he finally drank 20mL in one sitting? [Now he routinely drinks 100-120mL at every feed, and can go days without the feeding tube. He eagerly takes the bottle when he’s hungry.]

OLYMPUS DIGITAL CAMERA

Remember when we were so worried because he wouldn’t look us in the eyes and still hadn’t smiled at almost four months? [Now he smiles almost every time we look at him. He’s a very smiley, interactive baby. He smiles the most at Lydia.]

Remember when we found out he had CMV and we thought he was going to die in the next couple of days? [Now his CMV levels are at 1% of what they were, and he is very much alive.]

We’ve come so very, very far. He’s growing and developing and bringing us so much joy.

We have a long way to go, but we do so much better when we glance backward at the awful place we’ve come from.

Turns out I still have lots to live for.

(Including this beauty:)

Lydia playing

On Being Home

On Being Home

We’ve been home for a week now.

We are SO, SO HAPPY.

It is amazing. We love everything. The first morning back, I almost cried with joy over being able to use my chef’s knife again. And my cast iron pan! And when I didn’t have any onions for my scrambled eggs (Eggs! Farm-fresh eggs!) I ran outside into the sunshine and grabbed a handful of fresh chives.

After five months of living in a sixth-floor hospital room and eating cafeteria food, it was heaven.

For days, Ben and I would just rattle off all the different ways life was wonderful. A whole fridge of food all to ourselves! Coffee, any time we wanted! No elevators! No key cards! Comforters on our beds! It was like we’d just come home from a third-world country.

It was also a ton of work, though — getting everything set up and learning what we needed to know to care for our special-needs baby. We got home at 5pm on Monday night and didn’t stop working until midnight. We got up the next morning and repeated the same thing for the next three days, hardly stopping to eat and/or relax. (It’s a good thing Felix is such a happy camper. We pretty much ignored him for the first several days as we got everything ready.)

We had to set up a room for him on the main floor — crib, change table, etc — and set everything up for constant cleaning and sanitizing. We installed hand sanitizer dispensers and paper towel dispensers. We organized all his medical supplies. We set out gloves, gowns and masks, for ourselves and the nurses. We had to learn how to use and change the bags on his IV pump, how to sterilize his bottles, and how to steam-mop the floors. We had to find clothes for him, now that the hospital isn’t supplying sleepers. Nurses came and we had to sign paperwork.

And we had to move back into our home. Unload the van and unpack all our suitcases after almost half a year of being away. We still had Christmas presents to find places for.

But we are just so happy to be home. Especially now that we’re settled in.

And thank goodness, Ben’s mom and sister cleaned our house floor to ceiling before we arrived, and our friends filled our freezer with frozen meals. (We are blessed beyond measure in the friends and family department.)

welcome(This is how awesome our friends are. They threw us a “sanitary welcome home party.”)

The changes to Felix’s care have been so great for all of us. Instead of an IV pole, his IV meds are contained in a little bag we can wear like a purse. We can take him everywhere in the room without dragging a heavy wheeled machine around.

A nurse comes once a week. Not every couple of hours.

We still use the gowns/gloves/masks, but only on an as-needed basis. If we’re just talking to him, we’ll keep our faces bare; but we’ll put on a mask to pick him up. (I cannot be trusted not to kiss him otherwise.) We only put on gowns if we’ve just come in from outside or a public place. We only wear gloves if he’s likely to suck on our fingers or if our hands are getting too dry and cracked from all the washing/sanitizing.

It feels amazing to touch his skin with our bare hands again. The squishy little nugget!

We can make his bottles ourselves and take them out whenever we want. It’s fabulous. No rigid schedule anymore. If he’s not hungry at 4pm we can wait an hour. Or we can take it out early. No force-feeding or waiting unnecessarily, just to stick to their arbitrary schedule.

The only downsides to making his formula ourselves are (1) it’s a LOT of work. We have to sterilize everything that comes into contact with his feeding, and (2) I HATE it. It forces me to actively face, every day, the fact that I can’t breastfeed.

(Tangent: I HATE, HATE, HATE making his formula. HATE IT. I realize they did it for Felix’s own good and that my breast milk almost killed him, but I will always and forever subconsciously hate the medical community for forcing me to end one of the most meaningful, satisfying, and enjoyable experiences of my life. It was just awful, what they did to me. I HATE THEM.)

(Moving on…)

Being close to Lydia is so, so wonderful. We’ve seen her almost every day since we’ve been home. She gets funnier and smarter every day. Playing in our back yard together for an hour feels like a vacation in paradise.

Felix loves being home, too. He spent he first couple of days in awe of his surroundings. He was fascinating by everything. The light fixtures! The animals on his bedding! The mirrors on the wall! He is a marvelously happy little boy.

Again: it just feels SO GOOD to be in our cozy home. We’ve always been homebodies and appreciated what we had, but this experience has intensified our love. Which is a good thing, considering we will likely be living in isolation here over the next year. Which brings me to . . .

What’s In Store for the Future?

Our journey with Felix and SCID is not nearly over. We’re just home temporarily until we can take the next big step.

The goal is still gene therapy L.A. in July.

Closer to that time, Felix will have to undergo an intense series of tests to make sure he is eligible. As I’ve mentioned, it’s an experimental therapy and Felix is one of fifteen patients tentatively enrolled in the clinical trial.  (You can learn more about gene therapy here.)

It’s a terrifying prospect — bone marrow harvest, chemo, genetic modification of stem cells, transplant — but still our best hope for him, as far as we understand it.

Our time in L.A. should only be about 7-10 days. After that, we expect to spend another month or so in hospital back in Ontario to have Felix closely monitored.

After that, it’s hopefully back home in isolation for another 6-12 months.

If all goes well, Felix will hopefully be able to go out into the world some time in 2016.

And hopefully I can kiss him again for the first time since December 4, 2014. I’ll be counting down the days.

I want to thank you all again for your prayers and support. We couldn’t have done this without you.

We’re Going Home!

LHSC(Our home for the last five months)

Yesterday we sat down with our team — infectious disease doctor, nurse practitioner, occupational therapist, nutritionist, and at-home care coordinator — to talk about going home.

We’ve been in the hospital for almost five months with Felix (since December 4th). We’ve been through torment, we’ve been through tedium, we’ve been through renewed hope and joy. And for the last month or so, Felix has been doing really well. We’ve become more and more hopeful for his future.

We talked about visiting nurses. We talked about his injections and IV meds. We talked about feeding and infection control and emergency protocols. We talked about the long-term plan.

We have a discharge date and time: this Monday, April 27th, at noon. We will be walking out of the hospital room that has been Felix’s home for the last four months. We will leave behind the gowns and the masks and the gloves. We will touch him with our bare (though frequently-washed) hands for the first time since he was a skinny little newborn.

We will still be living in isolation when we get home. No visitors except for the nurses and grandmas. No Lydia. But we will be close to our friends and family. Only eight minutes away from our little girl rather than two hours away.

I am SO EXCITED. I am SO SCARED.

I can hardly imagine life with Felix outside this hospital room. What does he even look like outside these four white walls? I can hardly imagine life without nurses coming in and out to check on him every couple of hours. We’re going to be in charge of his bottles and medication. We’re going to be his full-time caregivers. That’s a scary prospect with a normal baby; it’s a terrifying prospect with an immune-deficient baby.

We don’t really know how we’ll do things with Lydia. We kind of don’t even want her to know we’re going to be living at home, because how crappy would that feel, knowing your whole family was living at home without you? But how can we get around it? We hope to spend time with her outside the home — at the grandparents’ houses, at the park, at the library.

I’ve seen my house once in the last five months. I don’t even remember how my bed feels.

I’m so excited to be the master of my own kitchen again. That might be the thing I’m most excited about: cooking. Being in charge of what we eat. I can’t wait to not eat cafeteria food again. And although the food and generosity of the Ronald McDonald House will leave me eternally grateful, I’m happy if I never eat mass-produced pasta and caesar salad again in my life.

I wanted to let you know ahead of time, because I imagine we’ll be really busy for the first little while, getting settled back home. This is not a life we ever expected when we got pregnant with our second child.

We’re so grateful to be taking our precious baby home. Thanks so much for your ongoing prayers and support.

Turns out, I want/need more Jesus. Who knew?

Turns out, I need Jesus

These last few months, as you know, have been hard, hard, hard. It’s a special kind of hell, watching your child fight for his life. Worrying about his survival. Helplessly witnessing his suffering.

It’s been more than my body and soul can bear some days.

I ache for peace. I long for something to give me strength during this trying time. But what?

As a lifelong Christian, I understand that the answer is supposed to be “Jesus,” or something along those lines.

But here’s the thing. And it’s embarrassing. Jesus has been largely absent from my life for several years. (And I write a blog about participating in Jesus’ radical Kingdom. Huh.)

I’ve retained the name of Christian, and in principle I’ve tried to live according to Christian teachings. Being kind to others, caring for the earth, talking about God, reading the occasional spiritual book.

But I’ve also spent the last few years feeling very cynical about the Church. I’ve lost interest in the Bible, in listening to spiritual music, in praying, in attending church services. Those things all felt kind of . . . lame. I was sick of pastors guilt-tripping us to do these things. I had no real internal drive to do them. So I let them go, living essentially as a functional atheist who admires Jesus from afar.

So in this time of extreme anxiety and grief I’ve been unable to find any real source of comfort. I mean, I take comfort in my husband’s presence, the love of my friends and family, etc. But I’ve longed for something a little more . . . transcendent. I want reassurance that this agony is something small and temporary within something else that is eminently good and eternal. Because it sure as hell doesn’t feel like it.

I’ve been finding myself . . . wanting to listen to music with spiritual lyrics. (I don’t think I’ve really done that since I got married nine years ago.) It feels weird, to be honest. Goofy, even. Me? Christian music? Aren’t I too hip for that now?

I’m starting to crave the wise words of spiritual men and women. I even want to read the Bible, the most boring and confusing of books, convinced it must have some words of comfort in there somewhere. Doesn’t Jesus talk about a peace that passes understanding and stuff like that? Eternal life? I could desperately use some eternal life right now.

I’m finding myself in an awkward place these days. I really, really want Jesus. For realz. I want  the Son of God. I want Eternal Life. I need the great I Am, the one who promises joy to those who mourn, strength for the weak. The one who loves the saddest, most pathetic creatures best of all.

For the first time in my life I am so weak. So stupid. I have so much grief. For once in my life, I actually NEED the things that Jesus promises.

I know that Jesus’ favourite people are the most pitiful ones, the most wretched ones. For once, I’m in that camp. That’s me.

I’m desperate. I acknowledge that I have been so full of pride. I have been so ignorant. I need help. I can’t do this on my own. (And all those other cliches from Christian songs I’ve never connected to.)

Turns out, I need Jesus. Not only that, I want him.

All this time as a Christian and I’ve never been here before.

I’ve never been desperate for Jesus before. Why would I have been? I was perfectly happy and healthy and capable of taking care of myself.  I’ve never really connected to hymns and psalms for that reason.

It took suffering for me to realize how badly I need Him. So I guess some good things always come from bad ones.

(*Note: I wrote the draft of this post several weeks ago when things were really bad. Things have been much, much better in the last few weeks. We are so full of hope for Felix’s future. Just wanted you to know that.*)

Image courtesy of frankenschulz via Flickr.

An Excellent Couple of Weeks

Felix almost five months

Hi friends. Sorry I haven’t been updating you much on Felix lately. The thing is, things have been really uneventful lately . . . in the best possible way.

For the first four months or so, we were going from crisis to crisis. We were constantly getting bad news, and Felix was constantly manifesting some scary and confusing new symptom. But in the last few weeks, things have finally settled down. He hasn’t vomited, had seizures, or spiked a temperature in weeks. He’s taking more and more of his food orally, too — he consistently drinks about half of his total volume. That’s a dramatic improvement, considering just a month ago we were struggling to get him to drink anything at all, and rejoiced if he drank a quarter.

His blood tests have all been really great, too. His CMV counts (i.e. the virus in his blood that we feared would take his life a few months ago) is down to a tenth of what it was a month ago. (It started out around 8 million when we first discovered it; hovered around a million for a few months; and is now down to about a hundred thousand.) And the reason? His T-cell counts (i.e. the antigen-fighting cells his body can’t make without enzyme therapy) have skyrocketed — from .22 to .7 in a matter of weeks. Thanks to his bi-weely injections, he’s building an immune system, and it’s fighting the virus!

Felix’s development is coming along nicely, too. (He’s almost five months old.) I suspect he’s still behind, though no one has told me officially and I’ve avoided looking it up. (It’s just not something I want to add to my worry list.) Whereas a few weeks ago he almost never smiled, he’s getting to be a real charmer now, grinning when we walk into the room, and even occasionally laughing if we tickle him and he’s in the right mood. He coos often and beautifully. He’s starting to grab onto toys and put them in his mouth.

His weight gain has been steady and great, too. He now weighs over 17 lbs!

His doctors are over the moon about his recent lab results, and we’re getting serious about going home. It’s mostly a matter of getting everything organized now: arranging to have a visiting nurse come to our home; getting the appropriate equipment and supplies rented/set up; figuring out the medicine portion, now that his needs have changed; etc. We’re talking about a little more than a week from now. (EEK!)

Ben and I are learning to insert his NG tube ourselves, which is positively dreadful. I’m serious, it’s one one of the most horrible things I’ve ever had to do — Felix screams and gags and flails, and we just keep shoving that tube down his nose without mercy until the job is done. It often ends up coming out his mouth, which means we have to pull it out and start all over again, sometimes up to five times in a row. As an enormously sensitive attachment mom, this makes my heart throb. Next, they want me to learn how to give him his injections. At least it can’t be any worse than the NG tube.

Felix continues to be a very happy boy. He forgives us quickly when we have to do something mean. He loves cuddles but is happy to lay on his back and watch his miraculous hands and talk to himself, too. He is an absolutely joy.

Thank you SO MUCH for your continued prayers and support. We are anxious to get home and start the next chapter of our lives.

 

Dear Felix: You Have No Idea How Many People Love You

Dear Felix

And most of them have never even met you.

Literally hundreds of people around the world are praying for your well-being. Crying out to God on your behalf. I get messages every day from people saying they’re praying for you — other moms of SCID babies; people who have been reading my blog for years; distant relatives I’ve never met; people I went to high school with; etc. They tell me that you’re beautiful and they want you to live.

A friend of mine whom I only know online enrolled you in a prayer membership with an order of nuns in Missouri. The Holy Spirit Adoration Sisters offer monthly masses to you, so that you may share in the benefits of all their prayers, sacrifices, and good works. (Neither she nor I are Catholic.) This friend checks in on you almost every day via email because she cares so much.

The hospital staff here? They love you. The nurses adore you and take good care of you. (Especially Tammy. She signed up to be your primary nurse because she wanted to care for you. She cuddles with you like you belong to her.) Dr. Salvadori cries when your test results come back negative and dances when they come back positive. She fights for you every day. Dr. Barton-Forbes prayed over with tears streaming down her face when you had those seizures, and was overjoyed when returned to your old self. The cleaning lady gets excited when she gets to see you awake because you’re so friggin’ cute. Kerry, the occupational therapist, lovingly plays with you and brings you toys to help your development. She says it’s a lot of work for you, growing and getting better all while “looking this handsome every day.” Lisa, the nurse practitioner, enjoys flirting with you and making you smile. She says making you smile for the first time was a highlight in her career.

Oh, and I almost forgot that a bunch of our friends and relatives organized a huge fundraiser for you within days of hearing your diagnosis. Thousands of dollars poured in within weeks. They organized a dinner and silent auction. Dozens of people donated items. They provided music, art, time and energy to raise money for you, so that we could stay near you. The whole town soon knew your name.

You, Felix. All for little  you. When there was only about eleven pounds of you.

Then there’s family, of course. Yours grandmothers fawn over you and miss you. Your aunts and uncles try to sneak in to see you even though they’re not allowed. They also cry when you’re not doing well and dance when you are.

And Lydia. She adored you from the moment she met you. It was hard to keep her off you, she wanted to smother you with hugs and kisses. She didn’t get to see you very long before you had to go to the hospital. Even when she hadn’t been able to see you in weeks — and she’s only three years old — she cried and said, “I miss Felix! I want to pet him because I like him!” And when we took her to the museum, she said, “We forgot something! We forgot Felix!” She had hardly even seen you and she considered you a crucial part of our family.

Your Daddy loves you so much. He cares for you so tenderly. He never complains. He doesn’t care how you turn out, he just wants to love you.

And, well, there’s me. I loved you and wanted you before you were even conceived. I worry about you night and day because that’s what moms do. It’s agony for me, not being able to kiss your face. I can’t wait for the day I can cuddle you again, skin-to-skin, and kiss you and smell you. You’re my baby, and I will love you to eternity.

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