December 28, 2015
Hi Everyone,
We apologize that it has been awhile since our last update. We have been busy with doctor appointments and spending quality time with Ms. Kaylee. She has been doing well and has made some nice progress in several areas.
Kaylee has been going to speech therapy weekly and physical therapy bi-weekly for the past few months. She's now sitting up on her own, tolerating tummy time for longer periods and recognizes her name when we call her. She is not crawling yet, but is definitely close. She's become highly skilled at picking up and throwing her toys around, which gives us more exercise, but it's a good sign her motor skills are developing.
We've been working hard with speech therapy. Kaylee does not take anything by bottle and probably will end up bypassing it entirely. The biggest change we made was gradually speeding up her feed rate from 1.5 hours to just under one hour. This has led to her being more hungry and taking more pureed food by mouth. Her GI doctor also approved us cutting out her midnight and 3am feed as well as increasing her Zantac since she had grown out of her old dose.
Kaylee also had the Ear, Nose and Throat (ENT) doctor perform a scope in November. It showed her vocal chords are intact and not paralyzed as we had initially feared. During this same scope, Kaylee's speech therapist attended and had her swallow 5-10 mL of green colored milk as part of a Fiber optic Endoscopic Evaluation of Swallowing (FEES) study. The study showed Kaylee is not aspirating (fluid going into lungs), which was great news to hear.
There has been discussion over a Gastric tube (G-tube) for Kaylee, but it's more for convenience than medical necessity. Since it involves another surgery, we are opposed to it and have let Kaylee's doctors know. What we have learned is there is a wonderful community on Facebook for parents in weaning the tube. In order to get Kaylee to take more by mouth, we need to get her hungrier. We plan on cutting out more feeds in the future after we meet with the GI doctor in a few weeks. One expert who has helped many families said a 50% cut from the NG feeds is necessary to induce enough hunger. That said, we anticipate some minor weight loss, but have been told that is normal.
Speaking of weight gain, Kaylee has been doing great. She's over 15 lbs. and into the 20th percentile! That is pretty remarkable considering she wasn't even on the growth curve a few months ago. Kaylee's blood pressure has remained higher than normal, so the kidney doctor increased her dose from 0.1 to 0.2 mL in November. Her Sildenafil for her pulmonary hypertension remains at 0.2 mL four times per day. Our hope is that she will not need it anymore after her follow up with the Pulmonary Hypertension team in February.
It has been really nice to watch Kaylee grow and develop these past few months. She is such a joy and such a happy baby. We are optimistic that she will be off the Sildenafil and most importantly, get the NG tube out within the next few months. She has become an expert in pulling it out and we have to watch her like a hawk.
Keith's parents have been a huge help as well giving us breaks, cooking meals and going to appointments with Kaylee and I. As well as my parents and aunts who visit weekly to help out and enjoy extra snuggles with Kaylee. Additionally, we wanted to thank you all for all the support, thoughts, prayers, and kind gifts. We do not know what we would do without family and friends like you.
We send our warmest wishes and hope the holidays were special. As we continue to celebrate with the new year, please keep those in mind that have passed and their families. You are always in our thoughts and prayers.
With Love,
Keith, Julianne and Kaylee
Here are a few videos of Kaylee. Enjoy!
Kaylee balancing at physical therapy
https://youtu.be/wryp9Ly7FZo
Kaylee the jumping bean
https://youtu.be/KqX8VOKq7W8
Kaylee laughing with Nana
https://youtu.be/E0xvdHr2Vvs
October 3, 2015
Greetings Family and Friends,
We hope you were able to enjoy the nice September weather, wherever you are. Kaylee has been doing well and has kept us very busy. Sorry this update long -- we have a lot to cover!
She gave us a bit of a scare a week and a half ago. On late Thursday / early Friday (Sept. 17/18) she had four bloody stools of bright red blood. It is rare for her to have poopy diapers at all at night, so we took her to the ER at Central DuPage Hospital (CDH) in nearby Winfield. It's only a 20 minute ride for us, and since they are affiliated with Lurie's, they also have Lurie's doctors on staff.
In short, the serious/life threatening possibilities such as pneumatosis ("heart attack" of the gut) and Meckel's Diverticulum (birth defect of small intestine) were ruled out via x-ray, blood test and ultrasound. The Gastroenterologist (GI) did not observe any anal tear/fissure, so he presumed it was a milk allergy. Although Lurie's had previously ruled out a milk allergy the last time Kaylee had bloody stools, the GI doctor said as babies grow, their immune systems can "kick on" and start attacking the milk proteins in their body. As a result, he had Kaylee start a hypoallergenic formula called Neocate mixed with Enfamil water.
DIETARY CHANGES
In the meantime, he recommended Julianne cut all dairy and egg out of her diet so it would not be in the breast milk. Kaylee's bloody stools stopped immediately after she switched to the IV and did not re-appear with the Neocate. However, she began vomiting with nearly every feed of the Neocate, which worried us. She also appeared puffy in the face, which alarmed us since we had not seen her like that since months earlier at Lurie's.
After 4 days of no dairy/egg, the GI doctor said it was ok for Julianne to give Kaylee breast milk again through her NG tube. The breast milk was fortified with a few scoops of Neocate too. Kaylee did not have any bloody stools and kept all of her feeds down! Her puffiness (usually in the mornings) also improved, but not completely go away. We asked several doctors about it, although nobody knew what was causing it. Her Nephrologist (Kidney doctor) started Kaylee on a very tiny dose of Captopril, a blood pressure medication, since some of her lab results from Lurie's indicated that kidney function may have been impacted due to all of the diuretics she had been on. We have a feeling that the Captopril may have caused the puffiness, but now she looks like her normal self in the mornings.
DOCTORS IN AND OUT
During Kaylee's stay at CDH, she was seen by many doctors. The Ear, Nose, Throat (ENT) doctor did two bedside scopes of Kaylee's vocal chords. The first time he said he didn't get the best look and seemed a little concerned about her left vocal cord not moving as much as the right. However, during his second scope, he got a better look and said her left vocal chord only had general inflammation, in part from her NG tube. He said we don't need to see him for another month for a routine follow-up. We were also fortunate that Kaylee's Cardiologist was able to visit her. She said Kaylee's heart, lungs and pulses were all very good. She didn't see anything from a Cardiology perspective that would give her concern. After checking with surgery at Lurie's, she said Kaylee could stop taking the Aspirin too. The speech therapist and dietician also stopped by for visits, which were very helpful.
We were discharged on Sept. 23 from CDH's Pediatric Intensive Care Unit (PICU). During Kaylee's stay, we were fortunate enough to stay at a newly built Ronald McDonald House just across the street. Although we live closer to CDH, it was really nice to be within walking distance of the hospital. Usually, Keith would take the first part of the night shift to be with Kaylee and Julianne would take the second half of the night shift. Kaylee won over several of the nurses and other staff members. We think she is trying to build franchises, and now has started up a suburban one to go with Lurie's, haha.
KAYLEE'S FIRST CARDIOLOGY APPOINTMENT
To back up a little bit, Kaylee had her first cardiology check-up on September 11. She did great! She got an electrocardiogram (ECG) and an echocardiogram. We were very happy to learn that Kaylee's arteries show no signs of narrowing, blockages or kinks. Her heart function looks good and the Cardiologist saw no fluid near her heart. She was impressed with the repair work. Her oxygen saturation levels were 100% too.
As a result, she said we could go down to 1x per day on her Aldactone (diuretic) and then stop it altogether after one week. We are glad Kaylee is now off the Aldactone and Aspirin. She also said it looked like Kaylee's pressures in her ventricles have come down slightly, a trend we were pleased to here with her Pulmonary Hypertension.
These are Kaylee's current medications:
1. Sildenafil -- 4x per day for Pulmonary Hypertension, 0.2 mL
2. Captopril -- 3x per day for blood pressure, 0.1 mL
3. Zantac -- 2x per day to help with spitting up, 0.6 mL
4. Lasix -- 1x per day to diarese her, 0.6 mL
5. Multivitamin -- general nutrition, 0.5 mL
Kaylee has her first follow-up with the Pulmonary Hypertension team at Lurie's on Oct. 8. She will also get another echo during this appointment. Depending on how this day goes, her Cardiologist said she will defer to the Pulmonary Hypertension team about whether to stop the Lasix or not. This will be the same team that saw Kaylee throughout her stay at Lurie's, so we're glad to have that familiarity with them.
WEIGHT & EATING
Kaylee has been steadily gaining weight, about an ounce a day! At her last check-up with her Pediatrician, she weighed in at 11 lbs. 8 oz., 23". During her stay at CDH, she actually gained a pound, which we were very surprised with, especially considering some of the vomiting she had. Her GI doctor said leaving the IV running, along with the Neocate, probably contributed to the sudden weight gain. We asked if it was a side effect of the Captopril, but the Kidney doctor didn't believe so. As long as Kaylee continues her positive trend with weight gain, eventually she'll catch back up on her growth trajectory.
From a feeding perspective, Kaylee has only been taking about 1 mL of milk by mouth. Fortunately, we met a really nice speech therapist at CDH who's been helping kids feed for 32 years. She showed us some new exercises to help stimulate Kaylee's oral senses and get her to swallow. We need Kaylee to swallow at least 5 mL in order to do the swallow study. For now, we do not have a swallow study scheduled, because we need her to make more progress in this area. Once she starts making strides, we will probably schedule it locally at CDH since they can do it and have better availability than Lurie's.
We are very motivated to get the NG tube out of Kaylee and we're sure she is too. She rubs the tape by her nose and has pulled it out several times already. It makes her sneeze sometimes and causes a little inflammation with her vocal cords, so we do the exercises with her every chance we get during the day. Putting the NG tube back in is not a fun experience for Kaylee or for us, but we know it's necessary for her to keep gaining weight. She still gets fed every 3 hours and her feeds still run very slowly at 1.5 hrs, so we are either stopping or starting a feed every 1.5 hrs. We hope to eventually speed up her feed rate, although we tried this temporarily at CDH and that was when she was puking.
PHYSICAL THERAPY
Kaylee has been doing really well with physical therapy. We have had visits from Early Intervention from the county and state, and they do not see any developmental delays with Kaylee! She rolls around from side to side and is close to rolling onto her tummy. The only thing they wanted us to work on with her was turning her head to her left more, since she was favoring the right. She tolerates tummy time longer now, swats at toys and has one powerful kick. We think she's going to be a soccer player someday. She kicked a small box of cereal out of Julianne's hand one day, spraying it all over the floor at CDH :)
Sleep-wise, Kaylee sleeps in large chunks of anywhere from 4-8 hours at a time. She'll wake up periodically for a quick diaper change at night, but usually goes back to sleep pretty quickly. We are hopeful that she's outgrown what our Pediatrician called a "witching hour" where she used to cry for roughly an hour around bedtime for no apparent reason. Despite crying for everyone who tried to calm her, usually Mommy was the only one she would eventually settle for. She seemed to stop doing this during our stay at CDH. So far, it has continued (to our relief!) She usually naps 2-3 times a day at the same times, so we are glad to have her on a schedule.
We thank our families for their constant support and help, as well as all of you who have so generously donated to Help Hope Live. We will post the same update on the webpage there as well. Please enjoy the attached photos of our little heart warrior.
We will try to send our next update after Kaylee's Pulmonary Hypertension follow-up on Oct. 8.
Love,
Keith, Julianne and Kaylee
August 23, 2015
Good afternoon,
Kaylee made good progress in the later part of the week. She did not vomit anymore and only spit up 1-2 times per day. She regained weight, with her final weigh-in at 9.4 lbs. Kaylee had many nurses stop by her room to say goodbye. She has developed quite a fan club! As a result of her progress, we got discharged from the CCU on Friday afternoon.
Rather than go home immediately, the team suggested staying at the Ronald McDonald House for a 24 hour stay. If Kaylee vomited or had any issues, we would be close to the hospital.
Fortunately, Kaylee had a good night. She spit up once on Friday afternoon, but slept the majority of the night. That gave us plenty of time to draw many of Kaylee's medications and fortify her breast milk. We called back to the CCU Saturday morning to let them know how it went and decided we were ready to go home.
Keith's Dad came and helped load up the cars while Keith's Mom organized everything at home. This helped our transition immensely. Despite the Eisenhower expressway being closed, the alternate route only took one hour to get home. To avoid missing any feeds, we had Kaylee's pump run while in the car. Kaylee cried a little, but eventually fell asleep.
We have also been fortunate enough to have Julianne's family's help. We were greeted by "Welcome Home Kaylee" signs that her sister Jamee and the Gerdes family made. Julianne's Aunt and Mom spent the night last night to allow Julianne and I breather and get a little sleep as well. Keith's parents also brought some delicious sandwiches for dinner to keep us re-energized.
Kaylee is definitely not your average baby. Our dining room now looks like a makeshift pharmacy (courtesy of Keith's Mom). This organization is necessary and is extremely helpful.
We have carefully measured, drawn and organized her medications. Kaylee will remain on the Sildenafil for an unknown amount of time for her Pulmonary Hypertension. She got her last dose of Ativan yesterday as part of her wean schedule and will be off of her Methadone in several days too.
To help Kaylee catch up with gaining weight, we fortify her breast milk with 2 teaspoons of Enfamil powder. She still gets fed every three hours with her pump at 85 mL. We give her meds with her feeds as well. We can connect her nasalgastric (NG) tube to an adjustable pole or to a little backpack that's more mobile. So far, Kaylee is tolerating the slower feed rate well. We continue to work with her on taking 10 mL by bottle once in the morning and once in the evening.
The next steps for Kaylee include gaining weight and many medical appointments. She will have her initial visit with her Pediatrician this week, a follow up at Lurie's with a CCU staff member, as well as appointments for speech and physical therapy.
Thank you for all your love and support!
Love,
Keith, Julianne and Kaylee
August 23, 2015
Good morning,
Unfortunately we had to take Kaylee to the ER early Tuesday morning. She threw up large amounts three times at home after we fed her. We had never seen anything like it and were pretty scared by it. We were discharged Monday afternoon, but got stuck in traffic. Kaylee missed her regularly scheduled 3pm feed, so we got it going as soon as we got home around 5. We are not exactly sure why she was puking, although it could’ve been the car ride, the big change of going home or her feed rate was a little too fast for her stomach.
We kept the on-call Cardiologist updated. After she had puked a second time, the Cardiologist said if she pukes a third time to bring her to the ER. She did not have a fever and her x-ray was normal. She was re-admitted to the Cardiac Care Unit (CCU). Since we spent 6 hours in the ER, Kaylee did not get some of her scheduled medications, so she went through some withdrawal on Tuesday and was very agitated. She threw up a little during her first feed Tuesday, but one of the Nurse Leaders speculated that her nasalgastric (NG) tube was slightly too high. She said when it’s too high, it can be near a sphincter, allowing food to come back up. As a result, she lowered it 3 centimeters.
We also put Kaylee on a very slow feed rate of an hour and a half. She spit up a few times on Tuesday, but did not puke anymore since these adjustments were made. Our nurse also observed Julianne and I drawing and giving all meds, mixing up the fortified breast milk and using the pump we had used at home.
She got a precautionary echocardiogram and x-ray on Wednesday. Both were normal. They increased her volume from 80 to 85 mL on Wednesday morning as well, since she had lost a little weight. She spit up with every feed on Wednesday during the day, but did not puke. She did not spit up or puke at all over night last night. For today, the team will leave Kaylee on the same volume of 85 mL and see how she does. One of her surgeons said a possible option is a Gastric tube (G tube) which would be another surgery / intubation.
This is a last resort and only if she continues to vomit or spit up with every feed. Hopefully, that can be avoided. If Kaylee does well, her surgeon said we could leave on Monday. He would like to keep her for the next several days to observe her and ensure she shows a positive trend, so we are not back again so soon.
Thanks for the continued thoughts and prayers.
With Love,
Julianne and Keith
August 12, 2015
August 11th
Happy Birthday, 3 Months old today.
Kaylee had a good night last night. Her stools are still slightly bloody, but not nearly as much as before. The doctors now believe it is likely a small tear/fissure from straining. She will continue to get the stool softener. Dr. Porta pointed out that Kaylee has been on aspirin. Since it’s a blood thinner, we could see the blood for several more days while the tear/fissure heals. Otherwise, upon examination, Kaylee’s stomach looks normal and so are her bowel sounds.
Dr. Mazwi said Kaylee had her “best echocardiogram,” since she’s been in the Cardiac Care Unit (CCU), so that was great to hear. Her shunting (blood flow) between her ventricles is all left to right now, which is normal. Her right ventricle looks healthier while her Atrial Septal Defect (ASD) remains the same, as expected. There was no narrowing of her aortic or pulmonary arteries, which was also great to hear, since that can be a side effect of the arterial switch surgery. That will be monitored long-term. They were not able to evaluate her collateral veins since Kaylee got a little agitated towards the end of the echocardiogram, but Dr. Mazwi did not seem concerned about that. Kaylee will have follow-up echocardiograms as well to keep an eye on those.
Her BNP (measure of stress on her heart) was actually up from 410 into the 800’s. Dr. Mazwi said he didn’t know what to make of that, but said that it would continue to be monitored.
Kaylee continues her gradual wean off her respiratory treatment. She’s gone from every 6 hours, to every 8 hours and today will be every 12 hours. The doctors would like to evaluate Kaylee’s condition when she is not getting any respiratory treatment. She has been doing well breathing room air. She has also been slowly weaning off of her Ativan and Methadone (pain medications) and tolerating it well.
Her weight was up 70 grams today to nearly 9 lbs., so that was nice to see as well. Her weight has been up and down for various reasons, so the doctors are hoping to see a positive trend in her weight for the rest of the week. She did not get an x-ray today, but has been breathing comfortably.
Thank you for all your love, prayers and support.
With love,
Keith, Julianne and Kaylee
