Posted (Tina) in All Posts on June-5-2008

Wow, today was exhausting.

It started with trying to find a pharmacy that would make Ella’s heart medicine correctly. We thought we located one, so we dropped off the prescription yesterday, for pick up today. Instead, they called and told us that based on the way they would compound the medicine, it would be $428.99 for a 30 day supply!

It was $72 for a 30 day supply in Gainesville. But we are low and only have enough medicine for another 24 hours, so I was stressing out. I finally found a place that would turn it around quick enough and would make it correctly. They were nice enough to accept my fax of the prescription to start making it (luckily I had made a copy), but they needed the original prescription when we pick it up. So, this meant an extra 30 minute drive across town to pick up the original prescription. All said and done, the new pharmacy will make it for a cost of $196 for a 30 day supply. This means more back and forth with the insurance company to fight for them to cover the medicine that she needs.

We left the house around 12:15pm to head to Children’s Hospital. Once again, the building is impressive.

First, Ella was scheduled for an echocardiogram. The technician was great; you can tell that they work with kids all the time. After about an hour, she reviewed the images with the cardiologist. He came in and explained that there were a couple of images that they were not able to get, so they might have to sedate her. Ella has never been sedated for an echo, so I was not real thrilled about the idea. I explained my position, so he went ahead and tried to get the measurements themselves. This meant another 45 minutes of sitting still for Ella.

When the echo was complete, they were still not able to locate 2 measurements of pulmonary hypertension. They felt it was because Ella was moving too much. It was indicated that it is likely that she will need sedation the next time they perform an echo.

From there, we went over to pulmonology. We were 45 minutes late to see the pulmonologist because the echo took so long. When we went back, they wanted to weigh her and get all her vital stats. When she weighed in, she was 14 pounds, 3 ounces. This didn’t sound great to me because she weighed in downstairs the other day at 13 pounds 12 ounces. All of a sudden, I began to wonder if she has started to retain fluid.

We are pleased that we picked Dr. Steve Abman to monitor her heart and lungs. He is knowledgeable, has great bedside manner and you get the sense that he truly cares. That is so important to me. He did a few things: increased her dosage of sildenifil, decreased her oxygen to .5 liters of oxygen via her nose cannula, ordered an overnight saturation test with a pulse oximeter, and ordered a swallow study to ensure that she is not micro-aspirating. Also, Dr. Lewinsky was present for the exam and meeting too. This ensures that 2 pulmonary doctors are familiar with Ella, in case we have any concerns.

Dr. Abman wants to see her again in 2 months, for a follow-up echo and visit. He asked who we picked for her primary care, so we mentioned Dr. Steve Perry and his face lit up! He ensured that they will coordinate her care together. Apparently, they have worked together frequently. That was a total blessing. I definitely wanted a pediatrician and pulmonologist to work together on Ella’s care. So this was a big accomplishment for the week.

Dr. Abman’s approach to pulmonary hypertension is different than Dr. Kays. In this way, we almost have to re-train our minds. Dr. Kays wants Ella to maintain high saturations whereas Dr. Abman feels that her prime saturation range is between 92-94, awake or asleep. His basis has more to do with the in-depth research and studies showing that there can be too much pressure in the lungs when the pulmonary vessels are too dilated. He did mention that he reviewed the echo on CD from 5/1/2008 and thought it looked good. He is positive about her prognosis moving forward.

Tonight, we have Ella on .5 liter and she is saturating 97 with a heart rate of 125, as she is on her way to deep sleep. According to Dr. Abman, we could wean the oxygen more. He gave the flexibility to wean until we got her to the 92-94 range. This is such a different approach to Ella’s care. We will see where she settles in. Either way, they will be coming to give us a special pulse oximeter that will track her heart rate and saturations while she is asleep one night. Once this is reviewed by Dr. Abman, he will be able to tell if this is the right level of oxygen therapy for Ella.

Before we left, we mentioned the concern about fluid retention. The doc suggested that we go down to the Special Care Clinic and use the exact same scale, so we can see if it is accurate. I’m so glad that he made that suggestion. She weighed 6.2 kg the other day and 6.3 kg today, on the same scale. This makes me believe that maybe she is okay on the fluid side. We’ll just have to continue to monitor it.

I’m so glad that my Mom is still here to help me with Ella for the next week. It makes me realize how tough it is going to be to pull off life in solo mode.

We’re just so blessed to have this little girl. Each day we are around other people and share our story, we are reminded that she is truly a miracle.