Mito Mighty Kaden

06/20/2025

Kaden had a relatively good night and appears to be improving. One of his blood cultures returned a gram-positive result, but both the medical team and we believe this is likely due to contamination. As a precaution, the doctors have started him on vancomycin, a broad-spectrum antibiotic effective in treating sepsis.
To be thorough, they are repeating the blood cultures using samples from both his port and a peripheral line. Since cultures require a 48-hour observation period, we expect to remain in the hospital for at least a few more days.

Kaden is still on oxygen, and we are working to wean him off again. Once he is stable on room air, a follow-up chest X-ray will be done to confirm that things are continuing in the right direction before discharge.

06/19/2025

Quick update on Kaden:

Kaden spiked another fever last night. Although all of his viral panels came back clear, we had agreed that if his fever returned, he would get another chest X-ray to rule out any concerns. The latest X-ray shows a “possible PNA,” which could indicate pneumonia. The left lung appears slightly hazy, showing some opacity. There’s also a possibility of atelectasis, which could mean a partial lung collapse.

Based on this, the care team has started him on antibiotics in hopes that we’ll see improvement soon—ideally, right away. This morning we were able to wean him down to half a liter of oxygen, but unfortunately, he’s back up to 2 liters now. So we’re monitoring closely to see how things progress.

He currently has quite a few doctors assigned to this hospital stay: pulmonary, neurology, a keto specialist, his outside clinic nutritionist, and his regular rounding doctors. It’s a team effort right now.

They’ve also increased one of his anti-seizure medications due to a recent uptick in seizure activity.

We’re in the process of transitioning him to a Modified Atkins/Keto diet. He’s not yet in full ketosis, but the goal is to help reduce seizure activity as he enters that state. We’re working closely with the nutritionist to increase his caloric intake while still aiming for ketosis. Since starting this transition, he’s lost nearly 8 pounds, which is definitely not the goal.

We’re hoping tonight and tomorrow will be better—hospital life is tough, to say the least.

04/08/2025

Kaden had two seizures this morning—one lasted around 15 seconds and the other about 45 seconds. While they weren’t long, they’re still seizures nonetheless. And that’s just what we happened to catch. It’s hard not to wonder how many more might be happening that we don’t see or hear. Thankfully, he already had a Teledoc appointment scheduled with his neurologist today. I sent her the videos and a log of his seizure activity since we increased his Keppra.

After reviewing everything, she expressed some concerns. Increasing any of his four anti-seizure medications could overly sedate him and potentially worsen his muscle weakness. Since he’s already struggling with swallowing, she’s hesitant to increase doses or introduce anything new.

She believes his frequent coughing and the ongoing struggle to manage saliva or mucus might be contributing to the seizures. So we talked through some alternative seizure management options.

The first is the VNS (Vagus Nerve Stimulation) procedure, which involves implanting a small device under the skin of the chest that sends electrical pulses to the vagus nerve. This cranial nerve helps regulate brain activity, and the stimulation can reduce the frequency and intensity of seizures. If we go this route, Kaden would need testing to determine if he’s a candidate.

The second, less invasive option is dietary therapy. She recommended starting with a Modified Atkins Diet (MAD), with the possibility of transitioning to a full Ketogenic diet later on. Both have been shown to help reduce seizure frequency, particularly in drug-resistant epilepsy. She’s already put in a referral for us to meet with a dietitian, so that’ll be our next step.

We’ll follow up with her again in two months and, in the meantime, continue focusing on getting his respiratory issues under control.

04/01/2025

Here’s my rant for the day… Insurance companies are the worst. I get that we need medical insurance, but do they have to make everything such a nightmare?

So, Kaden has been dealing with a persistent cough and raspy breathing. Some days are better than others, and increasing his steroid inhaler has helped, but that’s not a long-term fix. Months ago, he had an esophageal X-ray, and the results came back the same day, showing motility issues—basically, his saliva isn’t moving down properly because his esophagus muscles are weak. His GI doctor recommended trying a medication called Montegrity, which could help. The only concern is that Montegrity can speed up digestion, which is something we need to monitor because of Kaden’s ulcerative colitis.

The doctor prescribed it. And guess what? Insurance denied it. Why? Because Kaden isn’t 18. Even though Montegrity has been successfully used in pediatric cases, insurance still refused. It might not even work for him because of his UC, but we won’t know unless we try!

Let's fast forward just a little bit—his doctor’s office appealed the decision. But here’s the kicker: insurance requires our permission to process the appeal. And how do they get our permission? By sending a notice via snail mail. So I get the letter, sign it, return it to the doctor’s office for them to sign, then it gets faxed back. And just like that, the clock starts over.

Insurance denied it on February 28th. They received the signed notice on March 15th. Now they have another 30 days to decide. I even called and asked for it to be expedited—ha, what a joke. Apparently, they only expedite cases in scenarios. Here was the example of scenario I was given, a cancer patient needs medication to avoid hospitalization. Since Kaden doesn’t have cancer, they basically shrugged off the fact that he’s at high risk for aspiration pneumonia from choking on his own saliva. Their response? If they expedited it now, it would only “delay their ruling.” Seriously?
The doctor’s office called for an update, and—shocker—it’s still “pending review.” And the cherry on top? If their medical reviewer (who isn’t Kaden’s doctor, by the way) denies the medication, we get to go through another appeal by requesting a fair hearing. Absolute insanity.

I understand the need for insurance, but this process is beyond frustrating. Lord have mercy, this is ridiculous.

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03/01/2025

Today, on Rare Disease Day, I want to celebrate my incredible son, Kaden. He is stronger than words can express, facing each day with courage despite the challenges of PIGN chromosome disorder, and Leigh’s syndrome (mitochondria syndrome). His journey is not easy, but his resilience, joy, and love inspire everyone who knows him. Raising awareness for rare diseases means advocating for those like Kaden, who teach us the true meaning of strength. He is rare, he is extraordinary, and he is so very loved.

02/16/2025

Kaden had his ENT appointment this past Friday to explore possible solutions for his constant coughing and raspy voice. The doctor used a camera to examine his throat and found thick mucus. Unfortunately, there isn’t much that can be done—sinus drainage is normal, but since Kaden isn’t swallowing it, it’s accumulating. The only two solutions she could offer was either a nasal rinse which I have no idea how we are going to do this (but we will) or a tracheotomy. A tracheotomy would not be for breathing support, but to make suctioning out the mucus easier.

On another note, we received his calprotectin results, and his level is 132. While this is technically abnormal (normal is under 50), for Kaden, it’s not bad at all. For kids with ulcerative colitis, this is actually considered a good result. His levels may never be in the normal range again, so we’re taking this as a win.

I’m trying to get in touch with GI to discuss starting a new medication. It carries some risks for UC, but we need to see if it can help with his esophageal motility and allow him to swallow the mucus instead of it sitting in his throat. The last few days have been especially rough, even at night. It’s heartbreaking to hear him struggle constantly. We encourage him by saying, “Good cough, buddy,” or “Swallow Hard” but it’s tough to watch him go through this. Please pray that once he starts the medication, it works without worsening his UC. The thought of what happens if it doesn’t work is overwhelming.

I’m also reaching out to GI or hematology to request an iron infusion, as Kaden’s ferritin levels are low again. It seems like he needs a boost every year, and we can definitely tell—low ferritin affects his sleep, making him really fidgety. Ideally, it would be great if by a miracle I can get this added to his regular infusion appointment on Monday, but we’ll see.

The Bible says that when two or more people pray together in Jesus’ name, God is present with them and will answer their prayers. Please pray for relief for our little man and that we find a non-surgical solution. This struggle is so real and it’s scary.

I think we’ve consulted with all the specialists who could provide insight:
• Ortho – They support spine surgery but can’t guarantee success. Once it’s done, there’s no reversing it.
• Pulmonary – We tried a higher steroid dose, which initially seemed to help, but now it’s not making a difference. His lungs look good overall, with some mild inflammation or asthma. His ferritin is low, so an iron infusion is recommended since low ferritin can contribute to restless legs at night.
• GI – We’re going to try a new motility medication to help with his GI function and esophagus. However, there’s a risk it could trigger a UC flare.
• Cardiology – His heart looks good.
• Neurology – He’s having some seizures, so we’ve increased one of his anti-seizure meds. He’s currently on three different medications, and no one wants to add another. If increasing Keppra doesn’t work, we’ll adjust one of the other two.
• ENT – Discussing possible nasal flushing and the option of a trach.

So, that’s where we stand. Now you know everything we know.

01/29/2025

Last Thursday, Kaden underwent a GI scan, specifically an esophageal barium X-ray. The Interventional Radiology team came down to perform the procedure, inserting a long rubber tube through his nose to administer the barium into his esophagus. Almost immediately, it was evident that he is experiencing motility issues—his esophagus isn’t draining as quickly as it should.

Today was an incredibly long day. We met with Kaden’s GI doctor, who recommended trying a new medication called prucalopride, which is designed to improve GI motility. While it’s generally well-tolerated with minimal side effects, there is a risk that it could trigger a flare of his ulcerative colitis. We decided to move forward with the plan but will first check his calprotectin levels. His levels haven’t been normal for a while, though they haven’t been dangerously high either unless he was actively having a flare up. If they are elevated, we may reconsider this route.

After that, we went to the infusion wing for a port flush, followed by a GJ tube exchange with Interventional Radiology. Kaden went in wide awake and handled the procedure on his own for over an hour—his third time staying awake for it. Though I haven’t witnessed it myself, the process involves placing him under a large X-ray machine, threading a wire through his existing tube, and replacing it. This time, the doctor opted for a longer tube in hopes of preventing it from coiling back up, as his last two GJ tubes have.

We didn’t make it home until 4 p.m. poor baby was exhausted. Him and Kaden’s nurse were busted napping on the way home. We came back with a special gift—a calendar featuring some of the hospital’s amazing working dogs: Dexter, Declan, and Pilot. Dexter and Kaden have a special bond, so this was a thoughtful gift from the child life team.

And so, the journey continues.

01/12/2025

On Thursday, Kaden had the second appointment in a five-part series with his doctors. He saw his pediatrician, who also serves as his palliative care doctor. Many important topics were discussed, including the possibility of back surgery and carefully weighing the risks versus rewards. None of us want this for Kaden, and the risks are understandably daunting.

During the appointment, his pediatrician was able to see and hear firsthand the coughing and breathing struggles I’ve been sharing through videos. While his lungs sounded clear, there’s a persistent gurgling, raspy noise that seems to be coming from somewhere. We agreed to update Kaden’s pneumonia vaccine. While this vaccine won’t prevent all pneumonia, it protects against 23 known strains. He’s had it before and was due for it again. However, we understand this won’t prevent aspiration, which remains a concern. A chest X-ray was also ordered and showed improvement compared to his last one. She wants to revisit everything after the pulmonary appointment.

On Friday, we had Kaden’s pulmonary appointment, the third in the series. We spent an hour discussing potential strategies. His chest X-ray showed findings consistent with “possible viral or small airway disease.” While there’s no evidence of pneumonia, his lungs showed cloudy areas that could reflect asthma or inflammation. The pulmonologist pointed out specific areas on the X-ray that are minor concerns, but overall, the lungs look good, with healthy black areas indicating good airflow.

The pulmonologist outlined several steps to try and help with Kaden’s symptoms:
1. Increase inhaler dosage with a new, stronger steroid prescription to see if it helps with the coughing and raspiness. If there’s no improvement in a month, we’ll taper off the steroids. She also emphasized the importance of oral hygiene to prevent thrush, a common side effect of inhaled steroids.
2. Increase atropine drops to two drops twice daily to help dry up secretions. However, we will not increase the steroid inhaler and the atropine drops simultaneously. This will allow us to identify which intervention, if any, is effective.
3. Resume daily chest CPT therapy—exhausting but necessary to help loosen and move mucus.
4. Try the cough assist device to clear secretions. The device forces air into and out of his lungs to induce a strong cough, helping expel mucus. The process involves combining breathing treatments to open the lungs, chest CPT to loosen mucus, the cough assist to force it out, and, if needed, deep suctioning through his nose or throat. Suctioning is painful and distressing for Kaden—and for us—but sometimes necessary.

The pulmonologist is also advocating for a palliative care meeting involving herself, Kaden’s pediatrician, and his GI doctor. She also raised the possibility of additional imaging for his esophagus and lungs, considering Kaden’s pectus excavatum (a chest wall deformity). While this condition has improved over the years, it can push organs inward and potentially affect the esophagus or other structures.

We also discussed revisiting a CT scan or EGD to evaluate reflux. Kaden’s last pH probe confirmed reflux, but it’s unclear if it’s causing his current symptoms. A pH probe measures how long stomach acid remains in the esophagus, while an NG probe is more precise, measuring both acid and saliva and identifying the specific location of reflux.

Surgical interventions, such as spine surgery or an esophageal disconnect (rerouting the esophagus to the small intestine), remain a last resort due to the significant risks involved. The focus is on exploring non-surgical options collaboratively.

Next week, we’ll see cardiology to assess whether any heart-related issues, like slight enlargement, could be contributing to esophageal pressure. The complexity of Kaden’s pectus excavatum adds another layer to consider, as it may be causing esophageal compression or collapse.

This is a lot to process, and it feels overwhelming. Hearing Kaden cough and rattle all day long is heartbreaking. He must be so uncomfortable and raw from coughing so much. This has been one of the longest stretches of symptoms, and we’re desperate to find a solution to improve his quality of life. Imagine not being able to sleep because of constant coughing or the inability to clear your throat. As his parents, it’s agonizing to watch him struggle like this. We just want to find something—anything—that will help him feel better.

12/05/2024

This post is mainly for us to document and remember the details because well….there is allot to digest.

We visited Kaden's orthopedic doctor yesterday. Kaden has been dealing with a persistent, raspy cough for months, and it’s the strangest thing. At times, he seems fine with no issues, but then it can suddenly become severe. The coughing is often constant, day and night. At night, he mouth-breathes with a crackling sound in the back of his throat.

We’ve tried various approaches, including allergy medications, and even considered whether new teeth might be causing the issue. For a while, things improved—just recently, we had about two months where everything was great. But today, things are not great again.
We’ve tried various treatments, including the CPT vest, chest percussion, breathing treatments, and deep suctioning. Kaden also underwent an endoscopy with a PH probe placement to rule out possible reflux, as he is tube-fed directly into his intestines. Thankfully, the results from that procedure were good. However, during the endoscopy, the GI doctor encountered difficulty placing the PH probe due to the curvature of Kaden's spine, which raised additional concerns about his back.

Right after the endoscopy, Kaden’s primary care doctor, GI specialist, and pulmonologist had been communicating closely with his orthopedic doctor. The orthopedic specialist presented Kaden’s case to a panel of five other orthopedic surgeons as part of an internal review process to ensure consensus on potential surgical outcomes. While most of the surgeons agreed on a possible surgical approach, one was strongly opposed. They acknowledged that surgery might not guarantee a resolution of Kaden's issues, but they will support our decision one way or another.

The options are challenging:
• Do nothing, which risks recurrent pneumonia. (Knock on wood—Kaden has been lucky so far.)
• Do nothing, accepting this as his "new normal" quality of life.
• Try surgery, a highly complex procedure with significant risks and no guarantee it will resolve the issue.

Kaden’s spine has a 40% thoracic scoliosis curve, which varies slightly based on his positioning during X-rays. If surgery is pursued, it would involve correcting his spine with long rods and multiple screws from T2 to T12. The operation could take up to 10 hours if complications are avoided. The surgeon emphasized that while the spine correction itself is manageable, the post-surgery outcomes are unpredictable.

Typical children can recover from spinal surgery relatively quickly, sometimes leaving the hospital in two days. However, due to Kaden’s extensive medical history, his recovery would be more complex. Straightening the spine could allow a deflated lung to expand, but this might require time, support from a BiPAP machine, or other interventions.

Several precautions would be taken during surgery:
• An MRI scans would be performed beforehand to identify any complications, such as cysts or tumors. These are critical to visualize, as their presence could impact proper alignment and the surgical outcome.
• A neurology team would be present in the operating room to monitor nerve function throughout the procedure. Due to Kaden’s neurological complexities, they will ensure that no discs are compromised during the process of screwing them in (at least, this is how I understood it).
• Several CT scans will be performed during the surgery to monitor the positioning of the screws and rods for accurate placement.
• A plastic surgeon would assist by layering the spine to reduce the risk of infection and improve comfort.
• Multiple drains would be placed during surgery and removed as soon as they are no longer needed.

Kaden would likely recover in the ICU to monitor his lungs, pain, and potential infections, especially if BiPAP support is needed or his body temperature fluctuates. If everything goes smoothly, the hospital stay could last about seven days, with ongoing monitoring of blood work, infection, and pain management.

The surgeon recommended waiting until summer for any potential surgery, allowing us to get through cold and flu season and ensure Kaden’s lungs are as healthy as possible. Surgery presents risks like lung complications or the body going into shock.

We haven’t made any decisions yet. Kaden has upcoming appointments with his pulmonologist, cardiologist, and primary care doctor to gather more input. The orthopedic doctor explained that while correcting the spine might not directly fix his esophagus issues, it could improve his condition by elongating the area and allowing gravity to help.

Interestingly, studies show that correcting physical issues like spinal curvature can sometimes improve neurological conditions, such as seizures. The doctor also noted that most parents report more positive outcomes than negative after major surgeries like this, but it was also mentioned there is different dynamics for everyone so take this with a grain of salt.

Still, we face tough questions: Do we take the risk, knowing the possible complications and uncertain success? Or do we accept his current coughing and raspy breathing as his quality of life?

We’re praying for clarity as we continue consulting with specialists to determine the best path forward for Kaden. No decisions have been made.

09/18/2024

I don’t post as often as I used to, but this boy could really use some extra prayers. He’s been having some health issues. One concern is potential seizures. Here’s a video where you can only see the back of him because he prefers to lie that way, away from the camera. We’ve consulted with a neurologist and are holding off on adjusting his medications until we can determine if these episodes are actually seizures. Neurological issues can be unpredictable; what looks like a seizure might not be one at all. After a long wait, he had a 4-day EEG yesterday. Please pray that we get some answers. He’s currently on three anti-seizure medications, one of which is at maximum dosage, and he often wakes up at night or early in the morning, leaving him exhausted during the day.

The second issue is also quite concerning: he may be experiencing reflux, even though he shouldn’t be, since he isn’t eating through his stomach anymore and has had a Nissen fundoplication. Alternatively, his esophagus might be too weak for him to swallow his saliva properly. He coughs almost non-stop, and you can hear the secretions, which are especially bad at night. I’m really worried about the risk of aspiration, as that could lead to pneumonia, and it’s also affecting his sleep. My heart breaks for him; we’re doing everything we can to be proactive in his care. We’re doing breathing treatments and deep suctioning several times a day. If you’ve never experienced deep suctioning, it’s not pleasant. We use a long red tube that goes through his nose to suction out secretions, and it’s incredibly difficult to watch. I spoke with his doctor yesterday, and she wants us to consult with GI for an endoscopy and pH probe to rule out reflux. There’s a chance that with more teeth coming in, he’s producing extra saliva that he can’t manage, or it could be allergy related.

Today, Kaden’s case coordinator came by for his annual Medicaid benefit renewal. Medical kids are starting to get denied, and even though she doesn’t feel that Kaden should be denied, there is a possibility. So, let’s pray he can maintain his benefits for another year.

12/13/2023

I have not updated on little man in a while.

Last week, Kaden saw a new scoliosis doctor. Kaden did have Xray’s last year and we compared them to this year. Here is a side by side. The report shows several things:

1. There is superimposition of T11 and T12 on the frontal radiograph. – I believe this means that there are disc spaces, but I am not for sure on this.
2. Approximately 54 degrees of dextroscoliosis is noted from the superior endplate of T5 to the inferior endplate of L3. - Dextroscoliosis is type of scoliosis or an abnormal curvature of the spine that bends to the right and away from the heart. The doctor would like to rescan his back in 6 months and see if there have been any changes.
3. Bilateral coxa valga- This means there is a deformity of the hip in which the angle between the femoral shaft and the femoral neck. Although Kaden has his hips labeled “hips at risk”, the hip bone is deep socketed, and the doctor is not concerned whatsoever.

Today, he had a neurology appointment. Kaden has a seizure yesterday. It was less than a minute and he went to sleep after. He has started to have allot more myoclonic jerks. His neurologist would like to increase one of his antiseizure meds. We all think he has grown, and his body just needs more to keep everything balanced. He is no longer little, but he is long, and heavy. If he has any more seizures after the increase, we will regroup.

I am trying to schedule him a dermatology appointment. He has a rash that we can not get rid of. The doctor seems to be concerned that we need to know what type of rash this is. Since Kaden has ulcerative colitis, which is an auto immune disease, there is a risk that his UC can cause skin problems. So, his main pediatrician put in a Hale Mary prescription cream for us to try and if that doesn’t work then they will need to biopsy his rash and try to identify it.

I swear there is never a dull moment in this household. If it is not one thing….it is a complete far spectrum on the other side of the universe other.

10/17/2023

Kaden and I are kicking ass and taking names today. I woke up at 3:45 this morning, got medicines done, food ready, coffee made, got the kid up and on the road. This is a huge achievement!! Because anyone who knows me, knows I usually have to be dragged out of bed kicking and screaming. This handsome fella has a MRI this morning and a port flush. Then we are headed home to be reunited with…. My bed.