All I want for Christmas is a kid who can breathe,OR Anya's second hospitalization

If you have been following a while you probably have gathered that CDH babies are different. It can be difficult to anticipate what may happen during their care. Anya has proven this to be true; additionally she has added her own brand of weirdness to what is known. The past 24 hours have reinforced this.

Over the past several weeks Anya has been crabbier, and it was very difficult to pinpoint why. She had had immunizations, she was starting more therapies, and she was possibly teething were theories. It was also possible that she is more irritable than Arwen was; Arwen was a very laid back baby. My first real indicator that something was wrong was sweating: Anya had been sweating a lot more than usual. It was known and expected that she would sweat when stressed and her oxygen was getting low, but now she was doing it without any stress at all. Last week Wednesday Adam and I had spent several hours in the early morning trying to figure out if anything was wrong. Her saturations on the machine showed 85-91%, but she was pink, and then at one point she started smiling and cooing at me.

That is it, I decided, that number cannot be real, and I turned the oximeter off and went to bed. Generally speaking it is better to look at the patient’s behavior and not the numbers. We had an appointment with Newborn Medicine at Children’s the next day and I completely forgot to discuss the incident. Dr. Najaf was thrilled with her weight gain (as usual) as well has the milestones she had been reaching. We went over a Denver screening test that revealed that gross motor skills are the only big delay Anya has. I had no basis for concern that Anya’s oxygen was not doing well.

Because this is the thing: babies who are not oxygenated appropriately do not grow well, they do not reach milestones well, and they are usually pale.

Sunday Anya was sleeping a lot and she was sweaty almost constantly. I hooked her up again to the oximeter: 85-91% again, but still pink, still responding well. Turned up her oxygen and did not get much improvement. Still questioning the validity of the numbers, I started calling doctors, which led to us taking Anya to the ER. We had to put her up to 1 liter to get her sats up, and usually she is on 0.5 liters.

I have been avoiding the ER at all costs. I have been very concerned how other physicians would respond to her. Anya’s normal is a lot different than ‘normal’ normal and I have been afraid they would want to treat all of the strange things that are ‘wrong’ with her. Sunday though it could not be avoided. You can only second guess yourself so much, and we had to get some answers to what was going on. It turned out everyone was really great!

As her usual it was nothing obvious. The same questions were asked of us over and over: any nasal drainage? Coughing? Fever? Problems with tolerating feeds? No, No, No, and No. Doctors would walk in with one look on their face that would turn into pleasant surprise and then it would change to concern when they looked at how she breathed. Anya looks quite sick on paper, in reality looks healthy, but her respitory rate and the way her chest moves when she is breathing make her look like she is in distress. Mostly her breathing was the same as her normal throughout the ER stay.

There was concern that it could be from pulmonary hypertension, a leftover issue from her CDH (more on that later). She had just had a cardiac echo on Thursday that had shown no changes, so this was unlikely. Her blood work showed a slight increase in her white count so we were told rather feebly that she might have a cold. Since we had the ability to monitor her at home we could decide whether we wanted to have her admitted or go home.

Going home to wait for cold symptoms to surface, only to have to go back to the ER was not an appealing option to me, so we decided to stay. Besides, I had been questioning whether some of her behaviors indicated something was wrong or not for over two weeks, and with monitoring some answers might surface.

May I add that after we arrived at the ER Anya was grinning cheekily at everyone? And generally did not look like she was in any distress at all? Still, any attempt to wean her oxygen to normal was unsuccessful.

 

In the morning after an uneventful night Dr. Najaf came in to see her. It is her opinion that there was no cold other similar problem causing the issue. Anya has pulmonary hypertension because she has CDH. There is high pressure in the vessels and the part of the heart that goes to the lungs because they are trying to pump a normal amount of blood into small lungs. This can further stress the lungs out causing a cycle where the oxygen drops. It is possible that with her weight gain, doing new things, or many other factors, Anya simply needs more oxygen right now.

So the plan is to keep her on 1 liter and increase as needed with any activity or stress. In a week we will revisit this and see if she can be weaned down. Normally I would be really let down that she needed double and sometimes triple the amount of oxygen than what she needed before. Instead, I am just relieved that she does not have something more sinister going on.

The visit was helpful in other ways. Dr. Najaf and her nurse Donna came in to discuss her treatment as I was feeding Anya per tube. Lately she has been getting very upset at the end of her feeds, coughing and retching and acting very uncomfortable. I had attempted to explain what was going on but it was difficult. Dr. Najaf got to see this behavior for herself and now thinks Anya may have some reflux despite the Nissen, and that it could be causing inflammation that is irritating her. Anya was started on Prilosec today. It is possible this may not only help her discomfort with tube feeds, but with oral feeding as well. Anya is not taking anything by mouth right now at all.

So we are very happy to have her back home, but very glad that we went in. Hopefully the changes made will help Anya be more comfortable, help her to work on her therapy more, as well as eating. Thanks to everyone who prayed and continues to pray for Anya, and much thanks to my sister and my mother in law who shuffled Arwen’s care so I could stay with Anya in the hospital!