CT/Endoscopy/Urodynamics/Surgeon

Ethan's had some tests over the past few months so we can move forward with the right decisions for his care/future. Most of these surround his needs to protect his lungs from continued aspirations.

May 10th: Urodynamics/Renal Ultrasound
Ethan was scheduled for urodynamics and left the office in an ambulance due to respiratory distress. We spent a number of hours in the ER to monitor his O2 levels and we were able to go home. Prior to the versed making him stop breathing, Ethan had a renal ultrasound. Results below.

ULTRASOUND OF RETROPERITONEUM/URINARY TRACT, 5/11/2017 1:43 PM  INDICATION: Q90.9 Down syndrome R32 Enuresis ADDITIONAL HISTORY: None.COMPARISON: No recent comparison, ultrasound July 2004  TECHNIQUE: Multiplanar real-time ultrasonography of the retroperitoneum and urinary tract using grayscale imaging, supplemented by color and spectral Doppler as needed.Study was technically challenging given patient's limited ability to cooperateFINDINGS:  . Right kidney: Normal size, contour, and echogenicity. No hydronephrosis or perinephric fluid. No shadowing stone or focal mass is identified. Length = 10.4 cm.. Left kidney: Normal size, contour, and echogenicity. No hydronephrosis or perinephric fluid. No shadowing stone or focal mass is identified. Length = 10.3 cm.. Bladder: Generous in volume with no bladder wall thickening. Post voiding imaging was not obtained. Vascular: Perfusion to both kidneys is documented with color Doppler.    CONCLUSION: Technically challenging exam shows normal renal echogenicity and size with no hydronephrosis.Generous volume bladder

May 11th: Endoscopy/pH probe

The following day, he went in for an endoscopy and pH probe. We were then told that his esophagus was covered in a white substance later to be proven to be candida. Due to the candida, we couldn't do the probe. It was also shown that Ethan's esophageal motility was poor and the plan for a nissen and G-tube was now off the table as having the nissen would potentially come with more complications than help since his esophagus has to be strong enough to push his own saliva through and if it's not, he could "pool" and aspirate. No bueno as that's why we are doing all of these tests.

Ethan was treated for the candida but also acquired another aspiration pneumonia that had to be treated with another double round of abx. I have a theory that Ethan is starting to build a resistance to the abx that treats his pneumonias. :(

July 19th: Sedated CT of the lungs
The Friday before this procedure was scheduled to take place I received a certified letter saying that the 3rd party authorization company had denied the approval for the CT and could file an appeal. Understandably angry, I made calls and got the approval.

Any time Ethan is sedated, I get nervous because of his history, but this was the easiest sedation ever. Maybe because it was such a fast procedure? He went to sleep well, woke up well, and was fully active within the hour.  Results below.

CT CHEST WO CONTRAST, 7/19/2017 10:24 AM

  

INDICATION: BRONCHIECTASIS ASPIRATION, KNOWN OR SUSPECTEDJ69.0 Aspiration pneumonia of left lower lobe due to regurgitated food (HCC) J69.0 Aspiration pneumonia, unspecified aspiration pneumonia type, unspecified laterality, unspecified part of lung

COMPARISON: Chest radiograph from 6/22/2017. CT of the chest with contrast from 2/20/2006.

  

TECHNIQUE: Multislice axial images were obtained through the chest without administration of iodinated intravenous contrast material. Multi-planar reformatted images were generated for additional analysis. Nongated technique limits cardiac detail.

  

Wake Forest Baptist Health Radiology and its affiliates are committed to minimizing radiation dose to patients while maintaining necessary diagnostic image quality. All CT scans are therefore performed using "As Low As Reasonably Achievable (ALARA)" protocols with either manual or automated exposure controls calibrated to the age and size of each patient.

  

FINDINGS:

  

Thoracic inlet/central airways: The thyroid gland is unremarkable. There are punctate calcific densities (series 3, images 15 and 27) in the right supraclavicular region, favored to be vascular in etiology and could relate to the patient's prior right IJ Port-A-Cath. There is mass effect on the posterior trachea and esophagus, related to aberrant right subclavian artery, which is better demonstrated on the prior contrast enhanced CT of the chest from February 2006. Soft tissue density in the right mainstem bronchus, compatible with mucus secretions or debris.There is cylindrical bronchiectasis in the right middle lobe. There is mild, less pronounced, bronchiectasis in the lower lobes bilaterally.

  

Mediastinum/hila/axilla: No discrete mediastinal lymphadenopathy, although evaluation is limited in the absence of IV contrast. Patulous appearance of the esophagus.

  

Heart/vessels: Normal heart size. No pericardial effusion. Three-vessel left aortic arch with aberrant right subclavian origin, better delineated on prior contrasted CT.

  

Lungs/pleura: No pleural effusion or pneumothorax. Minimal dependent atelectasis. There are tiny subpleural cysts located along the lung periphery and extending along the fissures, which measure less than 5 mm in size, and are upper lobe predominant. Thickening of the interlobular septa, predominantly within the upper lobes in the region of the most pronounced subpleural cysts. There is diffuse bronchial wall thickening. Focal solid opacification within the right middle lobe, and this area also demonstrates bronchiectasis and architectural distortion.

  

Upper abdomen: Visualized portions of the upper abdomen are unremarkable.

  

Chest wall/MSK: No acute osseus abnormality. No aggressive lytic or sclerotic osseous lesions are identified.

  

CONCLUSION:

 

1. Focal solid opacification within the right middle lobe with associated bronchiectasis and architectural distortion, compatible with sequela of prior pneumonia or other insult.

2. Upper lobe predominant subpleural cysts with associated interlobular septal thickening, favored to represent Down's syndrome-related interstitial lung disease.

3. Diffuse bronchial wall thickening is a nonspecific marker of inflammation, possibly due to chronic aspiration.

These results kind of made me have what we will call a "moment". I cried, a lot. Like, ugly cry tears. I even called our ENT on his personal cell phone at 9pm while he was out of town to go over it (he's the BEST!). Layman's terms on these results are: Ethan has lung damage and we need to keep it from progressing. The feeding tube is no longer a question, it's happening.

August 3rd: General Surgery
We met with Dr. Zeller again to discuss the feeding tube surgery. The only saving grace to keeping the G-tube on the table is if the upcoming pH probe shows that Ethan is NOT refluxing stomach contents (the acid is under control with Prevacid). High probability is that E will have at the very least a GJ-tube, but likely a J-tube. Dr. Zeller also brings up that we don't have to intervene at all. This was another "quality vs. quantity of life" discussion. NO. There is no way I could ever not intervene to keep his lungs safe. Nope. I hate the fact that we will be taking a love of his (food) away, but I love HIM and his functioning lungs way more than his love of pancakes. I want to have the surgery done ASAP before school starts. I don't want him to be dealing with the transition to high school only to be pulled soon after for the transition to a feeding tube, so I'd like it done before school starts if possible.

August 10th: Endoscopy/pH probe/Urodynamics
Because of what happened on May 10th, I requested that all sedated procedures now be done at the hospital with anesthesiologists directly overseeing care. Because Ethan is a complex case and needs to be sedated for things most wouldn't, we arranged to have GI and Urology coordinate schedules to see him during the same sedated event. We arrived to the hospital at 6am. We were going to be admitted for 24 hours for the pH probe.

Dr. Safta (GI) came out and said that Ethan still has candida on his esophagus. (Grrrrrrr....) We can't do the pH probe, AGAIN. He also may be developing Celiac disease (yay! ::sarcasm::). We discuss options and I've decided that we will move forward with a GJ-tube. Dr. Safta says that the candida and Celiac may be affecting the motility of the esophagus. She will discuss with Dr. Glock (GI) and Dr. Zeller (surgeon). I want it scheduled ASAP.

Next, Dr. Atala (Urology) comes out and says that they placed the catheters for the urodynamics study without any problems. Then, the information I had no idea about...the only thing they did was place the catheters- the actual urodynamics study had to be done at the off-site clinic. Ummmm, ok.

So, I've got to get Ethan over to the clinic while he has two catheters placed in him (urethra & rectum). I call ahead to request someone meet me outside with a wheelchair- what a freaking ordeal. Get in to the office, am told to go across the hall to check in. Go across hall to the adult lobby that is handling peds as well only to wait in a line that is going out the door. (Pick a number- 37, and they were only on 20) Ethan is showing signs of discomfort (hello, TWO catheters!) so I go to another window and ask how to get the process expedited. I'm told that the computers are slow and I just have to wait. That doesn't go over so well for me and I tell them that we just came from the OR and things are already in place so we need to be seen right away. They look him up and then tell me that our appointment was at 8am. Ummmmmm, who the freaking frack did that?!!?? We were still in the hospital then. The assistant nurse manager proceeds to tell me that there were other patient appointments for 9/10/11am. I'm sorry someone screwed up, but Ethan's next. She said it wasn't fair to bump the other patients. But it's ok to bump the kid with a disability with cath's already in place? Nope. Not happening. Ethan's next. End of discussion.

I was PISSED. There isn't any way to sugarcoat it. I dropped many "f-bombs" and an entire lobby of people were introduced to this mama bear.

The test was torture. They had to fill Ethan's bladder with fluid to check capacity and pressures. He had over 500 mL added and was obviously hurting, but he hadn't peed yet. They took the cath's out and still waited another 5 minutes or so to pee. I could tell that even after peeing, it sure wasn't 500 mL worth. After a small ultrasound that can measure the bladder's contents, it showed that he still had 350 mL left in his bladder. That's not good news. They need him to be at least below 200 mL so they had to re-cath him to empty his bladder down to 1mL.

It took 4 of us adults, including one man, to hold Ethan down for this test. It was torture. And, I don't know if these results are going to end in Dr. Atala saying we have to cath him regularly now. I have no idea. Results on Tuesday, August 15th.

Someone really messed up with the urology side of these tests/appointment and I let Patient Relations know it.

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So, I've really been struggling with all of this, like bad. I've cried so many tears, I can't understand how I have any left. I've not reached out to talk about it because I just cry and I don't want to cry anymore.  My heart hurts for my baby. I just want him to be able to enjoy life, you know? No more doctors/tests. Enough already. Just let my boy be happy.


{totally not proofreading this tonight, maybe another day}