Our Hero

Our hero, Cully Charles Kuhl, passed away peacefully in the presence of God on the afternoon of Saturday, December 28.

After battling for 63 days the little man’s body was no longer able to sustain. It was a blessing to have been given eight-weeks to spend with Cully and in his final hours loving family surrounded him. 

One might say the odds were stacked against Cully from the beginning and for him to have survived as long as he did is nothing short of a miracle.   We feel blessed to have had the time we did with him.  During Cully’s short life he taught us so much about life, love, friendship, strength, faith and family.

The team at Children’s Hospitals and Clinics of Minnesota is nothing short of amazing. We made friends, we will not soon forget, who cared for our son with such compassion and professionalism words cannot express our gratitude.  The CVCC Unit is filled with an astonishing team of doctors and nurses, whom we are forever in debt to for the time they were able to provide us with Cully.  We ask that all gifts or donations be directed to the Children's Hospitals and Clinics Cardiovascular Critical Care Unit in Memory of Cully Charles Kuhl (http://givemn.razoo.com/story/Cully-Charles-Kuhl).  

We can’t say enough about the unbelievable support network we have.  Each and everyday you all have amazed us with your thoughts, prayers and love.  Words cannot express the feelings of appreciation and love we have.  Thank you!

We will be celebrating Cully’s life at 10:00 am, Tuesday, December 31, at Saint Mary's Episcopal Church (1895 Laurel Avenue, Saint Paul, MN) with a reception to follow at Eagle Street Grille (174 West 7th Street, Saint Paul, MN 55102) at 11:30am.  We invite all to join us in our celebration of life.

There Were Highs and Lows

Cully is now nearly a week post chest closure!

The past week hasn’t been without its struggles, but he has been remarkably stable and very gradually improving. His edema (swelling) continues to be his main obstacle, as it affects the space his lungs have to expand and limits his ability to move his limbs.

The PD catheter continues pull fluid from his body, as his kidney function remains dormant.  While we seem to have found the sweet spot for him over the past weekend, last Thursday was yet another adventures day.  The day following surgery usually entails quite the swing of fluid intake versus output day over day and Cully is very volume dependent to keep his blood pressure up.  Early Thursday morning his pressure gradually fell into dangerous levels, resulting in a worrisome few hours as he was given blood to increase his overall volume. While his pressures were low his care team ordered an echo and an ultra-sound of his head, both uncovered troubling news.

The echo revealed a good size clot in the right atrium of his heart.  Clots are very common in children with hypoplastic hearts as their blood flow is not as brisk and requires the use of blood thinners (heparin or later in life aspirin) to reduce the risk.  The placement of Cully’s clot on the ventricle wall was not impeding blood flow meaning it could be treated with increased heparin.

This is when we received news that his brain scan uncovered legions or cloudiness in his left frontal lob. A CT scan was ordered to determine what was creating the issue - a bleed would require weaning, instead of raising heparin levels to treat the clot.  The news of these two new issues was tough to handle.  Cully had already overcome so many hurdles and appeared to be improving ever so gradually each day. We anxiously awaited the results of the CT scan to determine the course of treatment for the blood clot and to gain a better understanding of any neurological issues that may be the result of whatever had caused the legions.

The CT scan revealed relatively positive news and there was no bleed, allowing the treatment of the clot to begin.  However, neurology determined the legions in his left frontal lob were caused by a stroke. The injuries suffered were considered “unimpressive,” meaning no major damage had occurred. Cully has continued to move his right arm, hand and leg, all good signs as the left hemisphere of the brain controls the right side of the body. In addition, Cully continues to visually track the doctors and us when awake - great signs that little neurological damaged had occurred.

Since Thursday, Cully has continued to maintain solid blood pressures (with no hiccups volume or otherwise related) while having his medications weaned very slightly, good oxygen saturation levels, constant positive PD runs (bringing fluid intake/output marginally negative each day) and no signs of major neurological damage.  The goal for the coming days is more of the same - quite consistent good days, with slightly negative fluid intake/output to decrease the edema and hopefully continue to wean his medications and ultimately his ventilator. 

On the home front, we are trying to get back to a schedule that allows us to be there for Cully, keeps ourselves in a good place and gives Tillie the attention she deserves.  This weekend we’ve both enjoyed some down time at home, even shared a quiet dinner for two of sushi last night (thanks to generous bite squad gift certificates)! In addition, the down time allowed us to get the house and Tillie into the Christmas Spirit!

As always thank you all for the generous support.  Each of you has made it possible to weather the last six weeks.  We hope you will continue to join us in praying for a week of healing and routine.

All our love,

Carl, Jessica, Tillie and Cully

Giving Thanks

We have MUCH to be thankful for:

We are incredibly blessed to have the toughest 5-week-old to ever grace this planet, Cully Charles.

We are blessed to have an incredible 18-month-old who continues to amaze us each and every day, Tillie Ann. 

We have a support network of family and friends that are second to none; a network, that has made this process bearable by providing love, support, food and just about everything else we could possibly need, You.

And, we have each other. 

Since our last post Cully has strung together eight consecutive days of relative stability.  They have continued to wean his blood pressure medications, and by and large his pressures have remained well within acceptable ranges.  Over the holiday weekend Cully began receiving breast milk via a feeding tube! Cully’s thanksgiving meal. And, he has tolerated the feedings better than any of us has expected. 

Swelling, or edema, and kidney function continue to be the main concerns, neither is considered life threatening at this point, nor is there an expectation of any long term effects.  He has not made urine constantly since being on ECMO and is continuing PD (perinatal dialyses) treatment.  PD treatments do not completely replace kidney function and are not as exact the dialyses provided by ECMO.  This has meant Cully has seen fluctuations in acids cleared from the blood stream by the kidneys and his overall fluid level. 

Changes made to the PD treatments have stabilized and lowered the acid levels, but finding the right balance of fluid off each day has been, and will continue to be, an ongoing battle.  When too much fluid comes off, the volume of fluid in his circulatory system decreases resulting in lower blood pressures.  When his blood pressures decrease due to low volume, the doctors give it back in the form of red blood cells, platelets or saline and his blood pressure quickly returns to acceptable levels.  This is quite the roller coaster, but a necessary evil, as getting fluid off his body has been extremely important in order to close his chest, which has been open since November 15^th when he went on ECMO.

Which brings us to today’s big news...Cully’s chest is closed!  He held is blood pressure throughout the procedure and is back in his room resting peacefully.

Closing Cully's chest is a significant step in his recovery.  Closure reduces his risk of infection and returns his body to a more natural state.  However, it brings with it some risks: first, is the likelihood of swelling; and second, is the pressure that swelling can put on his organs, particularly his lungs. 

As always thank you all for your thoughts and prayers.  Your support means the world to us as we forge through this tumultuous process.

Love, Carl, Jessica, Tillie and Cully

Cully's Wave

This has truly been a journey. Cully is now 28 days old, 23 days post surgery and 3 days off ECMO; of which, the last 6 days have been the most hair raising of all. As things stand today he has had about 24 hours of solid vitals and very few issues, but the past week has been dramatically different.

Last Monday, Cully's care team decided to schedule him for a heart catheterization "heart cath" and ECMO circuit change for that afternoon. ECMO used a series of plastic tubing to circulate his blood and over time fibers build up creating clots, so it is very normal for it to be changed. Everything went great with both procedures; the heart cath revealed no major issues with Cully’s heart function or blood flow. It did detect some concerns with his lungs ability to oxygenate blood, overall this is a concern but something that his body can correct or live with. We all expected and Cully lived up to his reputation of not particularly caring for being moved or major changes and softened his vitals slightly Monday night and into Tuesday morning.

Late Tuesday morning, the left side of Cully’s face, particularly around his mouth began to tick rhythmically. This continued for a few hours raising concerns of seizure activity. Neurology was called in and an EEG was installed on his head to measure brain wave activity. After four tense hours of waiting for results, neurology’s review of the EEG data revealed no sign of seizure activity. The ticking subsided Tuesday evening after he received a dose of muscle relaxant and has not returned. The running theory on this episode is drug withdrawal….along with the fibers that build up, the ECMO tubing also becomes coated with different pain and sedative medications he receives, slightly raising the dose. When the new ECMO tubing was installed it was free of fibers but also the coating of medications.

Once seizure activity was ruled out, weaning of the ECMO flow rates began again. Throughout Wednesday flow rates were continuously reduced and the decision was made to remove the ECMO support on Thursday. This was a major step forward, but a perilous one. We had given his body a week and half to recover on ECMO and all signs were pointing towards his body being able to manage the additional duties. ECMO had run its course, once removed putting him back on ECMO was not likely to help him recovery, only prolong his life. 

On Thursday afternoon Cully was scheduled to go back to the OR to have the ECMO cannulas (tubing) removed, so late that morning the flow from the machine to his body was discontinued and stented off. This was the first major test - could he maintain solid vitals that would allow for a trip to the OR? Three hours past while the surgery before Cully continued and Cully had held all his vital with in ranges, in fact slightly higher than we had expected. He had taken over for the ECMO machine and was showing positive signs, next stop the OR; and knowing Cully’s history we all expected the hours after the visit to be a bit rocky, but felt fairly confident he could handle it.

The OR visit went smoothly. Cully had managed the cannula and adjustment to his external pacemaker wiring. Post Op, as usual, was a rollercoaster as vitals softened, lab results did not look great, and we spent an excruciating 30 minutes waiting for blood. However, his body began to adjust late Thursday evening and the labs began to normalize faster than they had on Monday following the cath. All signs were pointing in the right direction with his vitals were still registering on the low end of his ranges. 

Treatments with the PD catheter began Friday afternoon, as fluid began to build up again following the OR visit and removal of ECMO. Cully handled the PD treatments well and kept his vital at the low end of his ranges. Things were progressing, however so slowly. 

Early Saturday morning things began to turn in the wrong direction, Cully’s vitals began to weaken over a 5-hour period. At rounds that morning the care team was not at all optimistic about his prognosis. We made the decision that it was time for Cully to meet his sister. Dad and Tillie arrived at the hospital around 10am, she spent an hour entertaining the care team and meeting here brother.

Our first family photo, Saturday, Nov. 23, 2013.

We then proceeded to have some very difficult conversations with the care team about the options if Cully continued to deteriorate. After a long walk and some lunch, we settle on a game plan to do all we can for him that will support his healing process, but would not prolong his life with measures that he would not likely rebound from. 

Apparently, these conversations motivated Cully! Over the last 24 hours his vitals have increased to the top end of the ranges – when awake his blood pressure is in the mid 80s and when sleeping the mid to low 70s (low 70s were his highs on Friday). He is keeping his oxygen saturation in mid-70s to low-80s (about 10 points higher then expected). The strength of his vitals have allowed for the gradual reduction of some of his blood pressure medications and at rounds this morning the care team was much more positive. 

We enter a new week on a positive swing, with a cautiously bullish outlook on his recovery. We have a long way to go, hopefully less eventful than the past, but each and everyday we make progress is a success.

The goal for this week will be to get fluid off his body, decreasing the swelling allowing for his chest to be closed again. Which will hopefully be his last OR visit for a little while.

As always thank you all for your thoughts, prayers, food and assistance through this process. You all have made it possible for us to focus on Cully, Tillie, and each other, for that we are eternally grateful. 

Perspective

The past week has been about finding perspective and allowing Cully time to heal.  We have now had a week to process Cully going on ECMO and learning more about his condition.

As you may remember prior to Cully’s surgery, he received a CT scan and echocardiogram revealing an additional anomaly with his pulmonary veins.  Prior to birth it was believed Cully had a classic HLHS defect, but the addition of the pulmonary vein anomaly has significantly altered Cully’s prognosis.  These two defects together produce a significant number of late term miscarriages as the body alters the normal course of blood flow.  In Cully’s the case the pulmonary issue was not that significant, however it greatly reduces the overall survival rate.  Coming to terms will this has been difficult for us, but we feel blessed that we have had this time with him and there is more to come, Cully has a lot of fight left.

Enough with the reality check.  While Cully has a long and tenuous road ahead of him, he has begun to make progress; however, slightly.  The majority of the excess fluid that built up in his body has come off over the week and he has been holding his own heart rate without the assistance of a pacemaker for about 48 hours. 

Yesterday, his care team decided that he has made enough progress that they would begin weaning the flow of the ECMO machine (lowering the speed at which the blood flows) and begin lowering his IV medications. In that past two days, they have pulled the flow 50 points, from 120 to 70, and reduced one of his blood pressure medications.  We are elated to report that Cully has tolerated this very well.  He has held his blood pressure and heart rate well within ranges and has continued to urinate!

This is a first step in the long road toward recovery, but is a step forward and for that we are extremely grateful.  He has been alert much of the day, interacting with his care team, as well as, mom and dad.  We moved a balloon into his line of sight to give him something other than 

Cully looking at a balloon while on ECMO.

Once again, we would like to thank you all for your thoughts and prayers, Cully and the whole family can feel the love of all of you.  We would also like to thank all of you for the offers of assistance, meals and most of all for being there when we need to talk or allowing us the space to process everything.

ECMO

It was a tough weekend for Cully and the Kuhl Family.  On Friday night, Cully’s blood pressure steadily declined and his lactate rose significantly.  The decision was made Saturday morning to put him on ECMO, a bypass machine that does all the work for his major organs – heart, lungs and kidneys.   ECMO has taken the pressure off his body allowing him time to heal. In the past 48 hours he has become more stable which has allowed some of his medication to be decreased.

Going on ECMO was tough for us.  We always knew it was a possibility, however we had hoped that we were past the prospect after we made it through the first 72 hours post surgery.  We’ve now had the opportunity to accept that this is best for Cully and is truly helping him in his recovery process.  The machine and the people who manage it are amazing.  He continues to look more and more like our son, as they are able to pull some of the excess fluid out of his tissue and reduce the swelling. 

Cully’s care team has not yet been able to determine the primary cause of this deterioration. One possibility is infection, which they are testing for and treating with broad-spectrum antibiotics.  Prior to the drop in his BP the care team was preparing for a heart catheter test in an effort to determine why they had been unable to wean him from his medication and ventilator settings, Cully’s progress has been slower than anticipated.  All the usual reasons for the slow progress such as heart function were looking very solid and no issues were revealed during x-rays or echocardiograms.  The heart catheter test has been put on hold until the end of the week at the earliest.

This week will be all about allowing Cully’s body time to heal while being fully supported on ECMO and remove the excess fluid in his body.  He is currently resting comfortably, holding his own heart rate and peeing – all very good signs.

Getting Tillie back on her schedule has allowed her to manage this process with all the grace of an 18 month old.  Her days are as normal as we are able to make them and all she really understands is that her little brother is not home, but mom and dad are around as much as possible.  She is in a great place, which makes mom and dad feel that much better and is a much-needed therapy.

Once again thank you all for your love and support.  The generous outpouring of support has been heartwarming. Thank you all.

A Step Forward

Since we last checked in Cully has continued to pee. He shed fluids so well over the last 72 hours they were able to close his chest this morning! This is a big step forward in a long road to recovery. 

We were reminded just how long when the care team recommended that a PD catheter be implanted during the closing procedure. While Cully has discarded a large quantity of fluid and begun to decrease the swelling throughout his body, his kidneys are not functioning at optimum levels. The PD catheter allows for dialysis to be administered, which will aid his kidneys in cleaning the toxins out of his system and assist in pulling the excess fluid from his body. In addition, the PD will allow him to receive additional nutrition and other fluids to help his body recovery. 

All of Cully's vital signs remain strong (heart rate, blood pressure, etc), he is alert and tracking people as they talk and work on him, but in order to begin weaning him from the life supporting measures we need for his kidneys to recover.  We are hoping for a quiet few days that will allow for his body to begin catching up.

Tillie has had an awesome week, settling right back into her routine at day care with Mary Rose and evenings with Mom and Dad.  Earlier this week she brought a picture of her baby brother to show all her friends.  Today, lead by Annie, Mary Rose’s daughter, they made Cully a sign!

 

A picture for Cully's door from Tillie's daycare family.

We are beginning to find our rhythm as well.  We are settling into a nice schedule that allows for each of us to spend time at Cully’s beside, while having quality time with Tillie each evening, along with time to rest and decompress a little.

As always, we thank you all for the love and support.  We could not do this without each and everyone of your thoughts, prayers and giving.