Noah’s Good News


This past week we had another check-in with Noah’s primary doctor regarding his Localized Scleroderma. You can read my previous updates on his battle with it here; essentially it’s a disease that attacks his skin, tissue and bone structures near the skin. There hasn’t been a cure discovered yet, and while it’s a lifelong condition it typically attacks a region of the body for about eight years.

When Noah was two years old he first started showing symptoms. It took two years for doctors to finally figure out what was going on; it’s an incredibly rare condition that most don’t know to look for. The medications are powerful, and have the potential for serious side effects, which required him to have regular blood work done to keep track of how he was responding. I’ll never forget holding him down for that first round of blood work, him sobbing and struggling, and me being overwhelmed with the thought that this was going to be our routine for who knew how many years.

He’s tough, and over the months and years, through all the tests, surgeries, doctor’s appointments, procedures, and whatever else, he took it all in stride. Often times he saw the bright side of things when we would be upset. And just like the doctor predicted, after almost eight years the disease seemed to stopped progressing. Months ago we stopped giving him the medication to see if it was just the drugs keeping it at bay or if it really had run its course.

And last week the doctor told us it’s dormant! We’ll go in to see him again in a year, but more for Heather and my peace of mind than an actual need. I have to be honest, after so many years, it doesn’t totally feel real! Yes, we do still have to keep our radar up for its possible reappearance, but in the mean time, after spending the majority of his life on some seriously powerful medicines, Noah is off everything and thrilled about it!

Quite the answer to prayer!

The Latest on Noah


It’s been a while since I have posted an update on Noah and his battle with Localized Scleroderma. You can read some of the past updates here. The short recap is that he originally began showing symptoms of Scleroderma attacking the skin on his face around two years old, but doctors were unable to discover the diagnosis until he was four years old. He is nine years old now, has had numerous surgical procedures, powerful medications, regular blood work, multiple biopsies, and more. He is a TOUGH kid who has gone through a lot!

This past week we had his latest appointment with his primary doctor. It was good news. Essentially, Localized Scleroderma tends to attack a region of the body for around eight years. He will have it for life – there is no cure, but it’s attacks, or flare-ups, will be unpredictable and sporadic. The one we have been fighting has been on his face, and the goal of the medications was simply to halt the progress and hold it at bay until it had run its course, which the doctor is hopeful is this year. It appears to have stopped its activity; perhaps because the medicine is doing really well, perhaps because the attack is wrapping up. Either way, around Christmas the goal is to begin easing him off the medication with the hope that it will continue to be inactive! This is GOOD news.

It doesn’t mean it’s over, however. Noah will continue to have regular doctor’s appointments – both to monitor where it has been active to make sure it is truly done attacking his face, as well as to monitor potential flare-ups elsewhere on his body, at which point another eight year struggle of medications, blood work, biopsies would begin again. When he is older, as well, he will be able to have plastic surgery to undo the scarring Scleroderma has left on his face.

Ultimately, though, this is exciting. When we were told so many years ago that this was going to be an eight year battle most likely, that felt so far off in the future. Pinning him down for his first round of blood work was a nightmare and I remember thinking with horror that this was going to be our lives every few weeks for endless years. Noah quickly learned to handle the challenges, often times doing far better than kids much older than him at the hospital. He is resilient! And like us, he is excited at the thought that we are only months away from perhaps ending the medication and blood work!

Noah and Scleroderma

Noah's selfieIt’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!

An update on Noah

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!


Living with Scleroderma

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!

Doctor’s aren’t magicians?

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.

Changing it up with Noah

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.

Noah’s street cred

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 sees the good

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.

Life Tattoos

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!