Narrowing Aorta and Feeding

Our family on Mother's Day. Thanks, Devra, for taking our pic! ;)

Caelin socking Daddy in the face!

Such a proud Mommy!

Two months old! Look at how I have grown!

Mommy & Cael

Daddy & Cael

It is so great to see his whole face! What a doll.

We apologize for taking a week to update the blog. Each time we would try throughout the week, the internet would not work at the hospital. We have been having a lot of issues with the internet connection in Caelin's room. Wednesday, Mommy tried for an hour to connect and could not get on. So over the past week a lot has happened.

First, let us say how wonderful it is to be able to see Caelin's whole face! This is the first time he has not had ANYTHING on his face since the day he was born. He is such a handsome baby boy!

Over the weekend Caelin was pretty agitated and irritable yet tired. They restarted his feeds through the J tube (going to the intestines) at 10 mL/hr. They ran at this rate for four hours and then he was bumped to 20 mL/hr. Between the time feeds were restarted on Saturday and Sunday at 6 PM he had 7 emesis of bile. Because of this combined with the discomfort of the tube being placed into his stomach from the outside, Caelin refused his binky and was a very cranky kid. So Sunday at 6 PM they decided to try to feed him through the G portion of the tube (stomach). From then until 10:00 AM Monday morning, Caelin had only 3 emesis. This was an improvement and we were proud of him, but he was still doing it a little too much and that made us nervous. He was also having difficulty pooping. It is common for anesthesia to have this effect. So they tried a suppository up his rear-end but he refused to let it go in and stay in! He turned purple trying to push that thing out as the nurse held it in. This gave Mommy and Daddy a good chuckle. Since the suppository failed, they ordered him a liquid to go through his tube. This ended up helping to get things moving! Sunday was also my first Mother’s Day. It was a beautiful day and I enjoyed every minute spent with my two men. The night technicians made a card with a lovely quote and with Caelin’s footprint (two of the same foot because he had an IV in his left).  Daddy and Caelin worked together to hand make me a special and unique picture frame! It is perfect!

Since the team of doctors change on mondays, the new doctor wanted Cael to go slower on his feeds. His feeds were bumped from 20 to 12 mL/hr. They remained here until Tuesday morning when they were increased to 15 mL/hr. Tuesday morning Mommy asked the doctors what they were doing here that we could not do at home and the intensivist’s response was, “IV Fluids.” And Mommy said, “Really? Because he’s not on any…” So apparently they had the intention of putting him on fluids to prevent dehydration since his body was getting significantly less volume of feeds. But no one wrote for an order for him to get them. The night nurse even asked the night intensivist if he thought she should put him on some and he said no. So at this point, their only counterargument for him staying here is that he needs IV fluids and they did not put him on any. Talk about another frustrating situation.

Through Monday and Tuesday his temperament changed. The tube site still seemed to bother him but he started becoming more lethargic. He was sleeping a lot more and seemed less alert when he was awake. Diaper changes didn’t seem to bother him, whereas he normally screams and holds his breath until it is over. Also, he started looking a bit paler. We attributed this to him losing 23mLs of blood over the last week to labs being drawn. Mommy and Daddy just kept a close eye on him and if things didn’t get better on Wednesday, Mommy planned to express her concerns. On Tuesday a doctor from hematology came to Cael’s room to talk to Mommy. She informed her that the results of most of Caelin’s blood work came back normal with the exception of one test. This test measured the level of his Protein C and it was abnormally low. She said the numbers could be skewed because of the Lovenox and aspirin so they would retest. If after retesting him they are still low, he will be classified as having a Protein C deficiency. This means his blood is more prone to clotting. However, even after this second test, the numbers still could be incorrect because he is still on the medications- so they won’t really know if they are accurate until over the course of the first year of his life. This level will be tested immediately before his Glen and after, then a couple months later.

Tuesday was a milestone for Mr. Caelin- he turned two months old! In our one month post we said we really hoped he was not here for his two month birthday to wear his “2 Month” outfit… but he was. He of course looked adorable but he would have been more adorable at home. He weighed 4001 grams which officially changed his medication dosing weight. He is a growing boy, but still under weight.

Wednesday morning started off decent. Laurie, Caelin’s nurse practitioner, came into his room and said she had everything ordered for when we go home and she had started his discharge paperwork because she was feeling he would be able to go home within the next week. Mommy was ecstatic but when she looked at Cael and thought about how he normally acts, she knew he was not doing well… So when Laurie came back in Mommy told her that she felt like there was something wrong with Caelin. He had been over-circulating (oxygen saturation levels were in high 90s to 100 and they are supposed to be between 75 and 95), was tachypneic (had rapid breathing), was nasal flaring, pulling when he breathes, taking short inhales and holding his breath then grunting them out, had lower blood pressures, and he was dusky and pale and extremely lethargic.  Cael lacked so much energy, when you held up his arm, it just fell to the bed. He had no resistance to anything and when he would try to cry, he would simply succumb. Laurie heard Mom’s concerns, agreed with Mom after observing Cael for a few minutes, and recommended a chest X-ray and Echocardiogram.

The X-ray was fine but the Echo revealed Cael’s heart is not pumping as strong as it should and needs to. He has reversal of blood flow which essentially means the heart is not strong enough to pump the blood out, so it is flowing back in. He has a narrowing Aorta, which is a huge concern. If the Aorta is too narrow, blood will not be able to flow to the rest of the body, which is what had been happening over the past few days. This is why it became harder for him to breathe, why he turned pale, why he was increasingly lethargic and so on… his heart was not pumping blood. As mentioned in previous posts, Cael has a leaky Tricuspid valve- this was initially not a huge concern because the rest of his heart seemed to be working fine and they were confident the surgeon could address it when Cael gets his Glen (second stage of the Norwood Procedure). Now that his heart’s squeeze is weak and they realize his Aorta is too narrow for the size of his shunt, it is a bigger problem. The doctors said they were really surprised he had done as well as he had to this point. They also feel like it could be part of the reason Cael has not been tolerating high volume of feeds- his perfusion of blood to the gut is really poor. So up until this point, the shunt was the focus of concern with Cael’s heart and now that it’s working great, almost too good for Cael’s size, his aorta has become the major concern. To help his heart, they put him on IV Lasix (diuretic) to empty out extra fluid and gave him a blood transfusion. Almost instantly he turned into our normal Cael. His color started coming back, his breathing slowed down and looked easier, his heart rate decreased to the 140s from the 160s and 170s and his blood pressures were higher.

Over night he had a decent night. Thursday morning, the doctors were pleased with his progress and restarted his feeds. They ordered a follow up echo for today (Friday). So Thursday was a pretty good day. Caelin was back to screaming during diaper changes, holding his breath, making his heart rate drop, and setting off the alarms on his monitors. Oh how we missed his obnoxious behavior!

Thursday night he slept phenomenal! He woke up only for diaper changes and to be re-swaddled. The doctors came in during rounds and assessed Cael. They all agreed he looked much better than he did earlier in the week; however, Mom was still concerned because into the morning Cael’s saturation was back in the high 90s to 100, his blood pressures were lower, and he was taking more frequent breaths with some grunting. They said we would talk more after he got his echocardiogram. So they increased his feeds, ordered a Vitamin K shot for a retest of his Protein C blood draw, changed his Poly-Vi-Sol dose to once daily and moved on to the next patient. About a half hour later the ultrasound technician showed up to do the echo. She left and Cael took a nap. Within the next hour Dr. Nguyen, a cardiologist, came in to do another echo himself. He wanted to take a closer look at Cael’s aorta. While he was performing the echo, Dr. Wilmot, the cardiologist on staff this week, came in to discuss the images with Dr. Nguyen. Upon discussion and review, they concluded the narrowing had become more significant since Wednesday and has become more of a concern. Also, the squeeze of his heart is still not where it needs to be. Dr. Wilmot, who is a brilliant and compassionate cardiologist, carefully explained the next steps. First, he wanted blood pressures of all extremities. If the uppers are significantly higher than the lowers, it will support their theory of the narrowing being significant. We took them and his right leg was 80/50 with a means of 57. His left leg was 87/49 with a means of 57. His right arm was 116/60 with a means of 75. His left arm was 103/50 with a means of 62. So this did confirm their suspicion. Second, even though Cael has tolerated it, he does not want Cael's feeds to go up and stress out the heart, so he is remaining at 14 mL/hr. Next, he would talk to Dr. Stapleton in the cath lab about possibly placing a stent or balloon in his aorta until Caelin is older and large enough for the Glen. He also would speak with Dr. Quintessenza, the head surgeon, about Cael’s prognosis and ask for his opinion on if a stent is the right answer or if he would need surgery before his Glen. After speaking to both Dr. Q and Dr. Stapleton and other necessary members of the team, Dr. Wilmot would come back to talk to us to give us a tentative plan.

So needless to say, just Wednesday morning the team planned on having Cael home within a week… and now it may be another month or more before they are able to even mention that word again. We wish so badly that our son would just heal and come home but apparently that’s just not how it’s supposed to be. Never did we think we would have to watch our son go through so many struggles in his life…. And he is only nine weeks old. He has a milk protein allergy, was Jaundice the first three weeks of life, has had dozens of IVs, blood draws, blood transfusions, a chest tube, pacing wires, a nasal feeding tube, been intubated, went to the cath lab, had two upper GI tracks, a Bronchoscopy, a Gastrojejunostomy tube placed, all in addition to his initial open-heart surgery which required him to be on bypass and then an emergency bed-side reopening to remove the clot in his shunt. Our poor baby boy is going to have to continue to fight hard his whole life if this is any indication of how things will persist. We just hope he maintains the strength, resilience, and determination that he has show, for it is what a powerful Warrior needs, along with love, hope, and prayers, in order to be victorious.

We will post an update of what Dr. Wilmot says is going to be the next necessary step.