It’s been a while since I’ve posted an update on Noah and his battle with Scleroderma. You can find my previous posts/updates here. It’s starting to feel like we go through spells of very little to report … and then get slammed with waves of challenges. At the moment, it feels like a bit of a wave.

We’re currently easing Noah down to a much smaller dose of steroids. This is good as the side effects are frustrating for Noah. He gets teased for being chubby by other kids even though it’s really just a reaction from his boy to the steroids. Unfortunately, over the last few years, the steroids have been the only truly effective response to the Scleroderma.

At the same time, we’re increasing his dosage of Cellcept. The hope is that this will work in holding back the progression of the disease, but it will take time to find out.

Last Friday we spent the day at the hospital getting an MRI for Noah. Because of his age, this meant he had to be knocked out for the duration, so it wasn’t the most enjoyable experience for him – but he’s a trooper! The picture to the right is him playing with my phone and taking selfies after he woke up! Originally, the purpose of the MRI was to get a closer look at what’s happening under the skin at his face and brain, mostly as a precaution to see just how deep the disease is impacting him below the skin, but also to see if there is any connection at all with headaches he’s been complaining of the last few months. But then …

In either a demonstration of great timing … or just the pattern of things surging at the same time, the week of the MRI Noah had an appointment with the dentist where they discovered the tissue in his gums had deteriorated along the path of the Scleroderma to the point of causing one tooth to recede dangerously, as well as impact a couple other teeth as well. No matter what he’s going to have to have oral surgery at some point in the next month or so to correct it. The timing of the MRI was really good because they were able to really take a closer look at that region of his face to see if it’s a coincidence that he has something going on there (highly doubtful), or if it’s the Scleroderma, and if so, examine just how deep the impact is.

On top of everything else, Noah has to start meeting with the plastic surgeon annually so he can track Noah’s growth and the development of his face. As he grows, the scarring around his nose and eyes will look more and more pronounced. By starting the tracking now, it will give the plastic surgeon more to work with once Noah is old enough (probably around 16) .

Pray for Noah! The next couple months will probably be difficult with him; the oral surgery will be a painful experience for him to recover from, and the increased doctor’s visits are not on his list of favorite things to do!

It has been a LONG time since I’ve given any updates on Noah and his scleroderma, mostly because there hasn’t been much to report. Towards the beginning of the year we had to make the switch from methotrexate to celcept to treat it. It’s frustrating, because neither drug is actually designed for his disease – they’re for organ transplant recipients to protect against rejecting new organs. However, the limited success they’ve found over the years in holding off the progression (there’s no cure) of scleroderma has been in large doses of these drugs. Unfortunately, the size of the dose carries with it potentially severe side effects, which Noah began suffering from with the methotrexate (the preferred treatment for the time being). Massive headaches, constant nausea and ultimately, a liver in the red zone forced the doctors to pull Noah off of it. It was disappointing especially given that he was only at a fraction of the dose he needed to be at.

He was switched to cepcept and steroids, with the hope that the steroids would jumpstart the process and undo the amount of inflammation he was experiencing. Unfortunately, while the steroid did seem to succeed in that, the side effect was a lot of swelling, especially in the face, which meant he started getting teased about being fat and chubby. Over the last few months we’ve been dialing back the steroid dose which has been good since the puffiness has also largely gone away.

We’re also about to start increasing the dose of the celcept. The doctors are glad to see the inflammation reduced, but it’s still not to the point where we would want it. Heather and I had been concerned because we were noticing a lot of blueness/darkness appearing around his nose and eye and feared the scleroderma, but according to the doctor it’s due to the thinning of his skin (related to the disease, but not as scary as the inflammation which is the first step in serious degradation and scarring) and being able to see the blood vessels below the skin.

Ultimately, he hopes to get Noah back on the methotrexate injections. The real goal is to get him through the next 5-6 years without much progression and it will hopefully have gone away by then. He won’t be cured, and it could/will pop up in other places on his body, but it will have hopefully have moved on from his face by that point. Noah is generally in good spirits, although lately he’s been complaining more and more about having to take the medicine. He’s a trooper, but after several years now, he’s getting tired of it and wants to be done. The last couple weeks we’ve caught him fibbing to Heather and I when it’s time for his medicine – he’ll tell me that Heather already gave it to him, or tell her I already did.

Anyway, overall he’s doing good at the moment, but still not where we want him to be. Thanks for all the prayers!

A great video on what life with Scleroderma is like. I think she does a powerful job of describing the disease in an understandable way, what it’s like going to doctors and them not knowing or recognizing it, and the realities of vital drugs with side effects that are brutal.

The drug she mentions, methotrexate, is one that Noah was on for a little over a year. He’s our six year old son who has localized scleroderma on his face; it started showing up when he was three, but doctors were unable to diagnose it until he was four and a half. It’s the preferred treatment (even though not designed for this use), but ended up being too rough on Noah’s liver and body so we had to stop a couple months ago before we could ever get him to the dosage levels he needed.

June is Scleroderma awareness month. Share the video to help build understanding!

I was just talking with someone the other day about the subtle expectation we tend to have that given enough resources, anything can be fixed, including our health. He mentioned how we always think doctors know everything, but one his friends (a doctor) admitted he was faking it half the time. I laughed and said that’s my own number one rule in first aid emergencies – looking confident, authoritative and in control. The last thing I need is an injured teen panicking, that will only hurt them worse. So I act like I know what I’m doing and hide any fear that I’m feeling (I am first aid certified, so I’m not faking my response).

But it is a strange thing when you realize the people you assume can help might not be able to, when you start to lose some of that confidence and naive trust. Realizing there are some things doctors just can’t do much about is a disconcerting and scary prospect.

When Noah was finally diagnosed with localized scleroderma, it was a relief at the time. We had spent a year and a half trying different doctors, treatments and tests trying to figure out what was going on with the skin on his face with no luck. Having a diagnosis was something we could act on, and of course, being close to experts meant we were golden. Especially when he responded well initially to treatments.

Then the treatments stopped working and started hurting him. So now we’re trying something else, but it really doesn’t seem to be working. It’s a frustrating reality to have sinking in more and more; this disease does not have a cure. Doctors don’t know everything about it. The experts and top brains in the scleroderma field are still experimenting with treatments. The best luck they have had are with medicines designed for other conditions and diseases that just happen to slow down or stop the progression. Unfortunately, it’s a rare disease that takes years and years to run trials on, which puts it low on the priority list for funding research.

It’s hard to be patient. When the best you can hope for is something to halt the scarring and tissue deterioration, without hope of the damage healing, time lost on ineffective drugs just results in accumulating scars. I made the mistake of Googling ‘localized scleroderma’ the other week to try and find a catchy image for a blog post. It was a terrifying and not at all what I had intended to find. It was the first time I had seen graphic images of Noah’s condition in full force.

June is Scleroderma Awareness Month. I would encourage everyone to visit and like the Scleroderma Foundation’s Facebook page to learn more about this disease. You can find my other blog posts on my son Noah and scleroderma here.

So everything got changed up on our six year old, Noah last week! Sheesh …

You can read some of my previous posts about Noah and his health challenges (Scleroderma and Ptosis) by clicking here.

But back to the point. Basically, we got called into the doctor’s office because Noah’s latest round of blood work came back bad – we were told to stop giving him the medicine and come in for a new plan. For the last year and a half or so Noah has been on one drug; originally orally, but then through weekly injections, to try and stop the spread of the Scleroderma. At first it worked. Then it stopped working. And then it became toxic to his body. For the last several weeks he’s been nauseous and on the verge of throwing up, and then the blood work came back revealing the medicine was at damaging levels in his liver. This was frustrating because he was on less than a third of the dose the doctor eventually wanted to get him to, and with the Scleroderma noticeably spreading and damaging both his skin and the tissue underneath, we’re all very concerned.

So now that the preferred treatment is no longer effective (and I believe it’s the only one they’ve actually completed a successful trial on), we’re on to a new drug. Noah was happy because other than the monthly blood work, this one is oral so no more home injections. Unfortunately, it tastes awful, so it took him a few days to get used to the twice a day dose. He’s also on a steroid for the next six weeks to jump start the drug, so at the moment he’s getting various medicines three times a day (we can’t do them at the same time because of the different food/no food stipulations), which has him thinking about it a lot more than when he was on a once a week injection.

As a parent, the last six months have been disappointing to say the least. It seems like each visit has been bad news in some way. The disease continues to spread. We can’t give him enough medicine. The medicine goes from being helpful to toxic. We’re taking aggressive steps with potentially bad side effects. And all this is just for the hope of halting the spread of the disease. There is no cure. And he won’t heal where it has already done damage – the only prospect there is plastic surgery when he’s an adult. Even the medicines we try aren’t actually designed for Scleroderma – for the most part they are actually intended for organ transplant recipients to help their bodies not reject the new organ. They’ve found by significantly upping the doses in patients like Noah they can slow down or stop the spread of the localized Scleroderma. It’s even strange going to the pharmacy; because these drugs are so rarely used, they always have to special order for us. It’s disconcerting to be recognized at all these places (the pharmacy, the blood work lab, the doctor’s office). We’re there a little too frequently!

All that to say, keep praying for our son. We’re only a week into this new treatment plan and we’re hoping when we return to the doctor in a month that we’ll hear something good.

Today was Noah’s monthly trip to the hospital for his blood work. Because the medication he’s on for his localized scleroderma is powerful and has the potential for side effects, his blood has to be monitored. The hope when we began a year and a half ago was that there would be enough margin of safety in the results that he could eventually dial back the frequency to every few months, but unfortunately his results have been consistently borderline (on the safe side of the line, thankfully) that we’ve had to keep the frequency high.

Anyway, we’ve got it down to a routine. This keeps it predictable for him and gives him an air of control, and even somewhat special (he’s the only one of our kids allowed to play with my phone, and only when at the hospital; he gets a milkshake, stickers, etc., and his brothers are jealous – little things that are a big deal for a six year old). So today we walk in to the needle room and an older teen was on one of the couches getting prepped to have blood work done. He saw Noah and said something to the effect, ‘oh man, that little guy is going to be crying.’

Three of the four nurses immediately responded with comments like, ‘Him? No way, he’s the best at this.’ ‘Noah’s a tough guy, he never cries.’ ‘He always does better than everyone.’ I’m not sure, but I thought I saw Noah get a bit of a swagger on his way to his couch.

I was grateful for how they all pumped Noah up.

I was also sad that he’s there so often that in a hospital that treats people from the world over, and in the blood work lab where there is always a massive line of people and I can’t fathom how many they see in just one day, that most of the nurses know him and remember him.

Noah, our six year old, is a trooper. He gave me a lesson in perspective today. For the last year and a half or so he has had to go to the hospital every month for bloodwork. Since the fall, he has also had to have a weekly injection at home.

So today was his bloodwork day. And it coincided with a shot at home, and he knew he was going from one to the other. On top of that, they’re running some extra tests so they took more blood than normal. I was thinking about how unfair it was for him.

So I was pretty shocked when we were in the car leaving the hospital, on our way home to another shot, and he said, ‘daddy, this is my luckiest day!’ I asked him why it was lucky.

He gave me a list. He was going to get a Shamrock Shake which normally isn’t at McDonalds and it’s his favorite (he gets a milkshake after bloodwork). Not only did he get four stickers from the nurses like always, but he also got a silly band. He got to play a new game on my iPhone (Puss ‘n Boots Fruit Ninja). And the emergency helicopter took off from the hospital while we were waiting on the curb to leave which meant he got to see it fly. ‘It’s a lucky day, daddy.’

I was pretty quiet while I drove to McDonald’s. I was all choked up and my vision was blurry. I had a list of all the injustices he was suffering and he had a list of all the special things he got to do.

The other weekend, when Heather and I were at the National Youth Worker Conference in Atlanta, there was a rapper who opened some of the main sessions named Propaganda. He really stood out to Heather and I, for a variety of reasons. We loved his style, his message, his presence – hopefully one of these days he’ll wander into our region.

One of the sessions he performed ‘Beautiful Pain’, which is also on his album. In it he raps about different people, both from his life and in the Bible who suffered – but he spoke of the beauty in their pain. He called the scars ‘life tattoos’ that were marks from God about their strength, courage, faith. He transformed tragedy, hardship and pain into something that truly was beautiful. It really, really spoke to me.

One of my struggles seeing my son Noah go through his various surgeries on his face for ptosis, the condition that affects his eye, and the scarring that the scleroderma is also causing on his face, has been worry about how he’ll handle all of the scars that are accumulating. If I could take them I would. For me that phrase ‘life tattoos’ jumped out at me big time. I would love for those marks to become tattoos from God that speak to his courage, strength, faith and patience. He’s a tough little dude – he’s been through a lot for a five year old. Finding a way to communicate it all in a positive, hope filled way is a challenge.

Propaganda is an artist with the label Humble Beast. His album, Art Ambidextrous, is actually available as a free download on their site. You should download it immediately!

I’ve blogged in the past about our son Noah’s challenges. He has to have surgery every few years on his right eyelid because of a condition called Ptosis, for a while he had heart arrhythmia that required a lot of monitoring, and for the last few years he has been battling Scleroderma, a condition that affects his skin and can cause a lot of discomfort, tissue damage and scarring.

About a month ago we went to see a specialist in Philadelphia who had equipment that could more accurately assess how active his affected areas are with Scleroderma. I blogged about it here. We had been suspicious that it was starting to flare up more severely, which was confirmed. Our Doctor here in Wilmington (who Noah thinks is awesome), upped his dosage on the medicine he has been taking for the last year or so, which unfortunately ended up being too much for his liver – his bloodwork came back at unhealthy levels. Another setback.

After a couple weeks of being off the medicine completely to clear out his system, we have resumed, but with weekly injections instead of him taking it orally. The benefit is that he will actually get more of the medicine, it will absorb directly, and bypass the liver all together. The negative … weekly injections at home. Yesterday was the first day, which involved a lot of screaming and crying, me holding him still and Heather popping him with the needle. He’s actually pretty good with his monthly bloodwork now, so once he gets used to this is will hopefully go better than it did yesterday.

Either way, it stinks. He’ll actually have to have the bloodwork even more frequently for a while to monitor what happens internally with the injections vs. drinking it, and over the next couple months we’ll hopefully be able to easy the dosage up to almost double what he has been taking – we’re all very concerned about it flaring up and spreading in spite of being on the medication for the last year.

On the positive side, it’s awesome that God landed us in a church and town with one of the best children’s hospitals in the country, and half an hour from some of the top specialists in the nation (Philly)!

It’s been a while since I’ve blogged about our son, Noah, our five year old son. He’s had a few extra challenges that our other kids don’t have; Ptosis, Scleroderma, and for a while a heart arrhythmia.

We had actually thought we were having a pretty good year. Over the summer of 2010 he had surgery on the Ptosis (it affects the muscle in his right eyelid), and started an experimental treatment on the scleroderma that seemed to be working. In fact, about half a year ago we we found out that the trials ended and the experimental treatment had become the official treatment.

But in recent months we started to notice that it looked like the redness on his face was starting to grow instead of recede, indicating a return of the scleroderma. Our doctor agreed, increased his dosage of the medicine (which does has risks for his liver, hence the monthly bloodwork he has been undergoing for the last year or more), and referred us to one of the national specialists located in NJ who has more experience and has ultrasound equipment that can detect just exactly what was going on under the surface.

Seeing the specialist was a mixed blessing. She really knows her stuff, and shed a lot more light on Noah’s condition. Unfortunately, she noticed another patch on Noah’s face we hadn’t even seen, and shocked us with the news that they’re realizing scleroderma is a lifelong condition that Noah will have to deal with for the foreseeable future.

Up until recently, so little has been known about the condition that they thought it generally went away in around eight years. I hadn’t realize how much I was just counting down the days and telling myself, ‘at least he’ll be done with this by the time he’s in middle school.’ Hearing that it actually continues to crop up for years and decades after was like a punch to the gut.

On top of that, the increased dosage did end up putting his liver levels in the red zone, so he’s off the drug for a couple weeks while they let his system clean out and then we’ll start again, probably with a lower dose. Ug.

In the grand scheme of things, there are many parents out there with children fighting much more severe conditions. So there’s a part of me grateful that the worst we worry about at the moment is the degree of scarring that will happen to his face. And hey, for a boy, that can even be cool – Harrison Ford’s scar on his face is awesome. But it would be nice to not have that worry, either.