I have been waiting to blog about this, because there are so many unanswered questions, but I am feeling a little better about sharing this with you, in an uplifting way now. :D
Months ago, Kayli had broken her leg (tibia and fibulia). Her heart rate was slightly elevated because (we thought) she did not like the casts. Any time we put casts on her legs (for club foot corrections), she has detested it, and tried (many times succeeded) kicking the casts off. It was easy to belittle her higher heart rates, thinking they were temporary grudges against the castings.
In the third week of January, Kayli started the Ketogenic diet. The diet consists of a lower calorie intake, low sugar, and high fats. It is imperative that Kayli get high amounts of water while on the Keto diet, because a side effect is dehydration and/or kidney stones/damage/failure. Kayli was not given the correct amounts of water when originally put on the diet, and she was slowly dehydrating without our knowledge.
Then in February, Kayli had another bilateral clubbed foot surgery. During the prep for the surgery, she was to hold all food at midnight, then all fluids early in the morning. Looking back, this was a very dangerous thing to do to Kayli, seeing as how she was suppose to be on water, to ward of dehydration. After the surgery, Kayli had complications. She was severely dehydrated, but we did not know this at the time.
After she came home the day of surgery, her Ketone counts were very high. I was told this was ok, but looking back I now understand that what was happening was NOT ok. Kayli was starving, and her body was taking Ketones (fat) from her own body because she was not being provided any through diet while fasting for surgery. With the dehydration and starving, her heart raced to keep up with the stress of lack of nutrients her body was enduring.
It is important to know the reason a person dies from dehydration. When dehydrated, you loose electrolytes. An important electrolyte, is Potassium. If you have too much, or too little potassium in your system, your heart will stop. Therefore, the reason you die from dehydration, is because your body does not have enough of the electrolyte, Potassium, which stops your heart from functioning/pushing blood through your body. If you are a heart patient, your potassium and fluid intake are monitored closely for this reason. When Kayli was dehydrated before, during, and after surgery....she did not have enough Potassium in her system. Her heart was stressed, and she was starving.
Kayli presented with high heart rates after surgery. We thought it was because the casting techs in the OR put her casts very high up her thigh. The right cast was so high, it was over a portion of her genitalia area. No matter the position she was in, it caused pressure on her pelvic, muscles, and bones. Within 24 hours, we had Kayli in the ER so another tech could cut back the cast. Kayli's heart rate continued to be high, but did come down slightly, so we assumed she was (once again) upset with the casts on her legs.
During the following weeks, Kayli's heart rate remained high. The Dietitian for the Ketogenic diet found her mistake in formula calculation and water intake. Kayli's diet and fluid intake increased. Her heart rate declined somewhat, but still remained elevated. The Cardiologist was called, and the Dr. placed Kayli on a heart halter to monitor her heart rate, and check for any signs of heart failure. The halter confirmed her heart was in distress. The dr. then ordered an echo, aka ultrasound, of the heart. The Echo showed slight decrease in Kayli's heart function. Kayli is confirmed in heart failure.
Heart failure means many different things, depending on the person, age, and situation. To Kayli, heart failure means her heart is not functioning, or doing it's work as well as it should be. She is not pumping the blood through her body as easily as she should be able to. She now needs support with her heart function. Kayli needs to be placed on heart medications to support a healthier heart function.
While it might seem to be a simple solution to many, it is a difficult task to come up with an easy medication plan for Kayli. There are many interconnecting factors. For instance,
1. The Robinul (Glycopyrolate) is a medication Kayli is on to help control secretions (produced with the humidification system to keep her trachea moist...a trachea does not have filters like our nose, which we would normally breath through if we don't have a trach). The Robinul can cause/add to heart failure. Although I have refused to increase the dosage of Robinul, the medication itself does not help support healthy heart function...but Kayli needs the Robinul to maintain moisture in her trachea.
2. The Ketogenic diet requires much fluid intake, to maintain electrolytes in order to ward of kidney dysfunctions Potassium, is an electrolyte in water that (if given in proper dosages) maintains healthy heart function. A heart med that Kayli will need, will be Potassium.... the amount of potassium will be a critical factor in that it cannot be too high, nor too low because either miscalculation can cause a heart to stop.
3. A heart can be in failure (or distress) if there is too much fluid on it. The Ketogenic diet requires high amounts of water to keep out of kidney distress. If Kayli has too much fluid on her lungs and heart, this will cause more stress on her heart, and increase the heart failure. Lasix (a diuretic) will need to be given, to take fluids off Kayli's heart. The diuretic will wash out all the extra fluid needed to keep Kayli hydrated while on the Ketogenic diet.
4. The Ketogenic diet is working beautifully in controlling Kayli's seizures, so simply taking her off the diet would not benefit her brain function. She has been on many seizure medications, but the side affects were alarming, and poorly altering her quality of life, while they did nothing to stop her seizure activity. The hope is to get Kayli off ALL seizure medications, and be seizure free! This is now a possibility, with the Ketogenic diet! Seizures can be deadly, so we are with hope in prolonging Kayli's life and quality of life, with the Ketogenic diet!
Kayli will need to be monitored closely, receiving many blood draws to assure her medication levels are within safe ranges. Kayli is a very hard stick (hard to get blood from). Not only does she inherit that from her mother (me), but it is also a common characteristic of Trisomy 18 (to have very thin veins). Kayli does have a chest port (Bard, mediport), but it has not drawn back (blood) since a month after it was placed (3 years ago). Kayli has an appt. with her general pediatric surgeon next week, to discuss taking out and replacing her chest port. The Cardiologist will give approval for this surgery, even while she is in heart failure, but he has stated Kayli will not likely be able to withstand more major, invasive surgeries, like spinal growing rods, hip surgery, etc. now that she is in heart failure.
The Cardiologist has stated that once in heart failure, you do not come out of it....you either maintain the function as it is, or you get worse. But then, this is Kayli we are talking about! Technically, she has been in heart failure since she was born (her heart has never functioned "normally")! :D Kayli has ALWAYS done things her own way, and we follow along, pretending we are able to stay one step ahead of her! lol
With all that being said....Kayli got her casts off, and AFO's on. Since the day the casts came off...her heart rate has decreased and is almost back to her baseline (normal)! Her Daddy said from the beginning...she just doesn't like the casts!
I wouldn't be surprised, nor would I bet against the fact that if given another echo...she would be functioning her little heart as normally as she used to (out of heart failure)! ;-) After all...this is our willful, stubborn, yet lovable Kayli we are talking about! <3
God's Blessings unto you!