We've had some simply wonderful news. Preston's March, a foundation that provides adaptive bikes for children with mitochondrial disease, is having an adaptive bike built for Noah . . . and they are doing his Bike Reveal on June 2!! We never in a million years expected this to happen so quickly and had in fact anticipated waiting closer to a year. Many of you have contacted us asking about donating money for a bike for Noah, but we've not felt comfortable accepting any of those kind offers. A bike is a want - an important want, to be sure - but not a need. I'd like to ask any of you who offered to give toward a bike to Noah to instead consider donating to Preston's March at http://prestonsmarch.org/ . This money will not go toward Noah's bike but will allow them to continue to bless children with mito by giving them the chance to own a bike.
We're planning a big "Bike Bash" to celebrate the big reveal. Preston and his mom Deb are coming from Virginia for the bike reveal and we are working on ways to make the day extra special for Noah. He knows he's getting a bike at some point but has no idea it's coming so quickly so this will be a huge surprise for him. Please pray that all goes well with his health and the weather and that we can make this a totally amazing surprise for him!!
Jeff and I had our conference call with the specialist who suggested we consider the surgery I mentioned in my earlier post, and we also had an excellent meeting with Dr. B and many of the members of Noah's hospice team. The meeting was hard, but we were 100% in agreement across the board. In short, Jeff and I (and our team) recognize that Noah is doing about as well as he's ever done. He's not been seriously sick for months, he's learning to do some walking, he's regaining some lost cognitive/academic skills, he's got a birthday coming up, and he's happy. While we've long held to a standard of "doing things for Noah and not to Noah," we are refining that focus a bit to say, "let's not rock the boat." Unless something has an overwhelming potential to dramatically improve his quality of life, we're not willing to intervene or interfere with the way things are right now.
What does that look like in practical life?
It means that we all were in complete agreement not to do a surgery that has the potential to prolong his life but almost certainly would bring a reduction in quality of life and could potentially reduce his quality of life dramatically. I can't tell you how reassuring it is that not one person at the meeting was even hesitant about this but instead agreed absolutely that this wasn't the way to go right now.
It also means that to improve quality of life, we are willing to take risks or push things just a bit. His bike is a perfect example - it may challenge his body quite a bit to ride his bike, but when we spoke to his pulmonologist today, he agreed that it's totally worth it. He asked us to get some oxygen tanks back in the house and just be prepared to give Noah a couple of liters of 02 while he's riding if it seems like he needs it. As I write that, I know that might sound kind of surreal for parents of healthy children, but given the fact that he'll be riding his bike with IV pumps in the back basket, adding a small bottle of 02 is really not a big deal. Noah is really just soaring right now and it's up to us not to clip his wings if we can possibly help doing so.
We also had a productive discussion about working out how to handle things if Noah were to have a sudden and unexpected life-threatening event. Noah tends to stick to the same playbook most of the time and when things go south, they tend to go south in one of only a few ways, but he is at risk for certain sudden and unpredictable events. It is important to Jeff and I to have a plan that honors Noah's wishes and our wishes while not in any way withholding needed emergency medical care for Noah. Dr. B came up with a perfect plan and we anticipate meeting again with him and some of the staff of our local (not Greenville) hospital to fine-tune the plan and get it in writing. While an event like this is pretty unlikely, it's important to us to have these kinds of decisions made while things are going well rather than in the heat of an emergency.
Dr. B is thrilled about Noah's bike. He didn't know about the Mobo Cruiser I mentioned in an earlier post and he asked if there would be any way for Noah to access something that would allow him to practice a more recumbent forward-and-back pedaling in addition to regular um-and-down bike pedaling. He was pleased when we told him about the Mobo Cruiser and said that it will be important for Noah to use both his bike and the cruiser (which is like a low to the ground pedaled go-cart). We got the cruiser the other day and Matt put it together last night. We were in Greenville today but are hoping to get Noah on it (in the house) tomorrow. We are so thankful to Claire and her mom Karen for making that possible for Noah and are thrilled that we had the perfect piece of equipment to do the exercises his pediatrician is requesting.
Along those lines, Dr. B is working on getting Noah to the Gait Analysis Lab at Shriner's in Greenville. The Greenville Shriner's has a very state of the art lab where a multi-disciplinary team can evaluate Noah and the muscles he uses for his walking. Among other things, they will attach small sensors all over his feet/legs then video tape him while he walks. The sensors will give them the ability to run computer models and get a fantastic sense of how he's using different muscles and joints and how they may (or may not) be able to help Noah get a more functional walk. They can even measure his breathing exhalations somehow and use that to determine how much metabolic energy is being expended by walking. We don't anticipate any real orthopedic interventions but it's important to get this information. For one thing, we want him to walk as much and as safely as possible. If we can fine-tune or help him we want to do so. For another thing, his feet are becoming deformed and Dr. B feels that Noahs dysfunctional walk is contributing to the problem. We won't stop him from walking even if it is causing additional problems (and Dr. B isn't suggesting we do so), but of course would love it if we learned there was a way to avoid this. We're also eager to learn just what all this activity means metabolically. It's a matter of counting the cost so that we are informed even though we have no intention of slowing him down.
Noah does not (and will not have) what is called "community ambulation." This means you aren't going to see him walking into church or in a store or walking out to a vehicle and climbing in. Even so, his walking is very, very key to his quality of life right now. It makes a MASSIVE difference to him to be able to grab his walker, swing out of bed, and get to the family room under his own steam and in an age-appropriate way (instead of crawling or creeping or scooting). He is in absolutely no way independent - he requires the assistance of at least one adult to assist him in getting out of bed and to manage his IV pole and lines, but for him it is a quantum leap of independence. He does struggle with walking and breathing at the same time so it's also important for the supervising adult to be sure he IS breathing be prepared to remind him to breathe or to intervene if he isn't breathing and his 02 sats fall too far. This isn't like an exercise-induced asthma with wheezing -instead, like a preemie who struggles to juggle feeding and breathing, he struggles to "remember" to breathe when walking (hence the 02 on hand for bike riding!) We also need to keep an eagle eye on all of his lines as they can (and have) snag and break or simply work loose from all his moving.
It may seem that this tiny bit of walking isn't a terribly good idea - so what if he can walk several feet to another room, and why would we risk having him stop breathing or risk his pulling/breaking a line or a drain just to allow him to walk a room or two away? The social and emotional benefits cannot be overstated and the benefits to his heart, lungs, and muscles are priceless. To everyone involved in Noah's care, it's an easy decision that these relatively minor risks are by far outweighed by all of the benefits that walking affords to Noah. We anticipate that riding his new bike will only multiply these benefits and we are looking very forward to a busy and active summer for our indomitable boy! This long period of relative medical stability is giving Noah the chance to make wonderful forward strides and I'll admit that it thrill all of us as much as it thrills him - I LOVE being able to share about the gains he's making instead of just the losses he's facing.
I'll leave you with one of my current favorite pictures of Noah. I took it on Easter as he was presenting Jake with his "Easter Basket." Noah was determined that Jake needed something special for Easter but we felt that buying a bunch of Easter treats for a dog was beyond frivolous. He was in need of a few new squeak toys so we got some squeaky "Peeps" on clearance then filled a basket with a bunch of treats he'd gotten as prizes from the pet store. We tossed in some kibble-filled plastic eggs and Noah ended up convinced we'd gone all out for his best buddy.
Noah is getting his supra-pubic catheter changed on Tuesday. I don't have a pre-op time yet but will try to update on FB from my phone. As always, we appreciate your prayers for another uncomplicated and easy procedure.
Blessings,
Kate
