With so many things going on at once with Kayli, I thought is best to break it all down into paragraphs for easier reading.
Heart failure: Just weeks ago, Kayli's heart rate was elevated, an Echo (heart ultrasound) was performed, and we were told she is in heart failure. To clarify, Kayli has technically always been in heart failure. She has CHD, or congenital heart disease, because her heart was malformed (she had many holes in her heart, and blood flow was not typical) when she was born (a common characteristic of all Trisomy Syndromes). Although she was in heart failure since birth, it was considered to be a compensated heart failure, meaning her heart was not stressed and was adapting to the heart function God granted her. Weeks ago, Kayli was evaluated to be in De-compensated heart failure. This means that her heart was not able to compensate for the poor function. She was declining and we were unsure as to how she would fair with a heart function so poor. Kayli's medical team rushed to put a care plan together to support her heart (a hard task with the Ketogenic diet to complicate fluid and carb intake). Once Kayli's casts were taken off, her heart rate started to come back down (little stinker REALLY does not like casts on! lol). She was not out of the woods yet, but this was a good sign that she may pull away from the slippery slope of de-compensated heart failure. To further explain the de-compensated heart failure...I need to move on to the next complication she is dealing with...her abdomen.
Bulge in Abdomen: When Kayli was about 7 months old, at our request, a surgeon attempted to put a G-tube (feeding tube) in Kayli's stomach. Instead, the surgeon perforated Kayli's bowel and placed the G-tube in her colon instead of her stomach. Many corrective surgeries later, Kayli is at high risk for bowel obstructions because there is much scarring in her abdomen now. Fast forward to about a year ago. Kayli developed a slight bulge in her right side abdomen. And as she grew in size, her abdomen girth grew with distension. In the last couple of months, her abdominal bulge has grown greatly. Last week, she was given an X-ray (with no answers) and then an ultrasound, to figure out why the bulge and abdominal distension was increasing in size. The radiologist was called into the ultrasound room (never a good sign) and determined Kayli appeared to have a rather large mass in her abdomen. Upon further contemplation, he recognized that he did not see Kayli's bladder and thought there was a minimal chance that the large mass was actually an obtrusively large and greatly filled bladder. Rather than seek immediate medical attention for the mass, the radiologist asked if he could call the pediatric team to the ultrasound room and attempt a catheterization to see if any blood or urine would come out. If so, and the mass shrunk, then it would be determined the mass was actually a grossly exaggerated bladder. Kayli was cathed, and 750 cc's of urine was taken out! A typical full bladder in a child her size, is 125 cc's! The ultrasound was done again, and the mass was no longer there!
Once the urine was out of her bladder, the radiologist noted that Kayli's organs were back in typical placement. Kayli's heart rate went back down to her baseline (normal), and it was determined by her specialists, that she was no long in de-compensated heart failure! The grossly excessive fluid intake (for the Ketogenic diet) has exemplified the poor bladder function. It is assumed that Kayli was not actually voiding (peeing), but rather had a continuously full bladder that overflowed. The overflow was mistakened for voiding. While we are currently testing to figure out why Kayli's bladder is not emptying, the specialists have concluded the following:
1. Kayli's bladder was pushing organs out of placement, and sitting on her arteries. This caused stress on her heart, and pushed her into de-compensated heart failure.
2. Now that her bladder is back to normal size, and her organs are not compromised, she is officially back into compensated heart failure! (This is great news!!!)
3. Kayli's kidneys are appropriately formed, and continue to function well!
4. The ultrasound shows an echogenic focus (spot) on her liver, thought to be a hemangioma. The area of concern needs follow up testing.
Current global medical challenges and plan of care:
1. Heart function: while in a good state right now, we as a team, have decided to support Kayli's heart with a medication, called Captapril. The heart med. will not harm her, but may offer supplemental support. Kayli's heart is now functioning as expected, and she is no longer in any danger. :D
2. Bladder: Kayli is to get a Urodynamics test to determine if she is able to push urine out (contract her muscles). If she is not appropriately contracting her bladder muscles, we may consider one of the following treatments:
a. Continue straight cathing via urethra
b. Surgically place bladder under her skin
c. Surgically place button in abdomen for catheterization (we would only need one person to cathe, whereas via the urethra, we need 2 persons).
d. Medication to enhance bladder contractions
3. Spine: Kayli has significant Kyphosis, Lordosis, and Scoliosis. A reputable surgeon has highly recommended that Kayli has surgery to correct her spinal deformities within the next 3 months. Although surgery at such a young age is not common, his recommendations come from Kayli's concerningly rapid abnormal growth, crowding of her chest organs, and her current pliable (easier fixable) bone structures. Kayli has an appt with an Endocrinologist to test for Osteomalacia (poor bone density). Once the results of that come in, and the opinions of other Pediatric Spine Specialists conjoin, we will likely give Kayli the much needed surgery (with consideration of when she is at her best health). The surgeon has recommended a definitive spinal fusion but since this would reduce her longevity of life (her spine would stop growing, while her organs would continue to grow but now have space to function), we are opting for the growing rods surgery. The growing rods surgery is a major preliminary surgery, with less invasive bi-annual surgeries to adjust the rods so they grow with her spine. The risk of infection is greater, as she will need continuous surgeries vs. one surgery with the definitive fusion surgery.
4. Chest port: With Kayli's Trisomy 18 diagnosis, comes narrow ear canals, airway, and veins. Because her veins are so small (the size of a hair), and because she has had many surgeries (many of her veins are collapsed), it is in Kayli's best interest to have a chest port. A chest port is commonly used with patients getting frequent chemo treatments, and will benefit Kayli, because it is a "port" that is already in a vein. A tech simply needs to put the needle (to draw blood, or infuse medications) into the port to access a vein. This means less needle sticks, secured access, and less pain for Kayli. She currently has a chest port in, but does not work properly (we cannot draw back from it/get blood). Kayli will be going in for surgery this Monday, for an outpatient surgery to take out the ineffective chest port, and implant a more effective chest port (it will not only draw back blood, but also support pressure for the contrast used in CT testing!).
Please wish Kayli well, and pray for the surgeon, his supportive staff, and Kayli's comfort.
Thank you for your love, thoughts, support, and prayers! Hugs from Kayli and Family! <3