This past week was an incredibly busy one! Tuesday we had Liliana’s Pulmonary Hypoplasia Clinic and Thursday Friday was her 6-month well visit.

The PHP clinic on Tuesday was so crazy. We left home around 7 AM and arrived at the hospital just after 8 AM. Here’s how the day progressed:

  1. Hearing Test: Liliana had a tympanogram performed, and the results were a bit unusual. I don’t understand them, but it had to do with the pressure and possible congestion in her ear due to her feeding tube. She also had a hearing test where we sat in a booth (I was holding her in a sitting position) and the audiologist would call her name and play tones out of certain speakers for her to respond to. These were at different sound levels, and Liliana did respond to her name, but at a sound level that’s higher than normal.

    Liliana did not respond to the tones, but the audiologist thinks that it’s just because of her development level. We should be going back in about 5-7 weeks (and we have to get her ears cleaned of all the wax) for another hearing test, at which she should be able to respond to the tones.

  2. Meeting with the Pulmonary Doctor: The pulmonary doctor checked her breathing, the size of her chest, and basic development. He also explained to me what would happen at the Pulmonary Function Test, which was happening later in the afternoon.
  3. Meeting with Nutrition: The nutritionist came in, and after discussing Liliana’s current eating habits and her dismal weight growth, we came to a new feeding plan. We’re to feed her 90 ml of milk/formula that’s doctored up to be 24 calories per ounce for 2 weeks. If she’s doing okay with that, we’re to increase it to 100 ml. The nutritionist did say that she should be able to take 115 ml from the size of her stomach, so hopefully we’ll be able to get to that point sometime soon. Until then, we’ll take baby steps to make sure she can keep everything down.
  4. Meeting with General Surgery Doctor: I told her about Liliana’s night-time crying fits and how we weren’t sure if it was teething pain or pain from her gallstones. We would put Orajel on Liliana’s gums and she’d still be irritable and fussy, sometimes crying for hours no matter what we did. To see if it was her gallstones, the doctor ordered bloodwork and an ultrasound. Everything else seemed fine.
  5. Meeting with Speech Therapist: The scheduling was weird for this because they had the speech therapist meet with us after the point that Liliana is NOT supposed to eat or drink anything due to the pulmonary function test. We managed by using the pacifier, which Liliana still does not suck on (she chews on it instead). The speech therapist did give me a tip to point the pacifier up to touch the roof of Liliana’s mouth, and to possibly dip the pacifier in her baby food so she can suck the food off that way.

    Liliana seems to love this particular speech therapist. Once during this meeting, the woman was talking and Liliana laughed for no particular reason than she enjoyed her company. We’ve only been able to get Liliana to laugh once and we had to boodle her for it.

  6. Bloodwork: Not fun.
  7. Pulmonary Function Test: We had the pulmonary function test (PFT) scheduled at 1, so after the bloodwork was done we went to the Pulmonary Lab in the hospital’s South wing. We were shown the PFT room and settled down a bit while the nurse readied the medications. I was sitting in a chair with Liliana sitting upright in my lap. She was so tired, she fell asleep. After all, I had to make her sleep-deprived for this test. I couldn’t wake her up either, she was so out of it.Once our pulmonary doctor arrived with the pulmonary technician, the equipment was readied, Liliana was sedated with chloral hydrate, and once she was in deep sleep she was placed inside a sort of sleeping chamber.

    I took this opportunity to finally eat some lunch. I think it was around 2 PM, and I hadn’t eaten much since 7 AM that morning so I was starving!After getting back from a satisfying lunch, Liliana was still in the midst of the PFT. She woke up a few times, and sometimes air leaked out of the air mask on her nose and mouth, so the test took a lot longer than anticipated. I think the actual test ended around 4 PM. In any case though, the results were good and showed that her breathing was on par with that of normal babies her height.

  8. Surprise Echocardiogram! Someone had apparently ordered an echocardiogram, possibly because Liliana’s blood pressure was low in the morning. Two people came in with an ultrasound machine to the PFT room as the PFT was ending, and performed the echocardiogram as the PFT’s results were being tweaked and analyzed.
  9. Last Minute Ultrasound: There is at least one good thing about CHOP. The people there will try their hardest to accommodate you. Since we wanted to check on Liliana’s gallstones, General Surgery made an appointment for us with Radiology for an ultrasound. The first appointment was at 2:30, then was moved to 4:30 since the PFT was taking longer than usual. After the PFT and echocardiogram was done, what time did we arrive to Radiology? Just before 5, and then we finally got in to do the ultrasound at 5:30.I saw quite a few blotchy things on the ultrasound, some of which the sonographer measured. I haven’t heard anything yet about what was found.
  10. FINALLY. HOME! After driving home (45 min – 1 hour), Liliana was starving. She hadn’t had any milk after 7 AM, had only a little bit of water between then and 11 AM, and didn’t get much after her PFT because of the ensuing ultrasound. Sean fed her a whole lot of peas/green beans (she shoved it into her mouth and when she wasn’t being fed she was crying), some of which she threw up, possibly because her stomach was so upset from being empty. That day was really rough on her.

With the pediatrician, I told her about the small bump on Liliana’s head. It’s right above her hairline, and looks to be in the middle between the two hemispheres of her skull. The pediatrician wasn’t sure what it was, but suggested it could be where the bones overlap and is a bone overgrowth. For now, we’re just going to watch it.

I also told her about Liliana’s curved spine. She took a look and also noticed that Liliana has a tuft of hair in the small of her back, which she says is indicative of an underlying spine condition. I always wondered why that hair wasn’t going away, and thought it was something newborns got and eventually lost.

Anyway, she’s having an orthopedic doctor contact us to set up an appointment for an evaluation and X-ray. The pediatrician also is ordering an MRI for us to check on the brain, spine, and spinal cord.

All in all, there are still a lot of issues left to deal with. We have our GI follow-up later this week, and we’re going to see another speech therapist next week to try to get Liliana to drink milk. It seems like things just keep getting busier and busier…