Posted (Tina) in All Posts on August-21-2008

Today was very busy and we’re tired! But I could not go to bed and leave everyone hanging! I will do my best to summarize.

I called The Children’s Hospital the other day and asked when we could schedule the surgery and bronchoscopy. When the phone rang this morning, they were calling to discuss time slots. I was trying to be proactive and get on their schedule. If I talked to Dr. Kays and he advised against surgery, I could always cancel the procedures. They penciled us in for Wednesday, September 3rd.

After leaving a message again for Dr. Kays this morning (I tried to stress that we have been in a holding pattern for 2+ weeks and that I really needed his thoughts), he finally called back this afternoon while I was driving Ella to the hospital. In a nutshell, he agreed with the doctors in Denver that she has a paraesophageal hiatal hernia. He noted that this is not uncommon for CDH patients, especially those that were missing a large part of their diaphragm. As she grows and the diaphragm stretches, one of the side effects can be a hiatal hernia. If she did not have any symptoms, he would hold off on surgery and let her continue to grow and get stronger. But since she is having so many symptoms, he recommends surgery soon. I asked if we held off on surgery and tried to manage her symptoms in other ways, could it heal or get better on its own? He said it will continue to stretch out as she grows and will worsen with time.

I expressed to him that I am nervous about having a surgeon here do the surgery since he knows her so well. He agreed with me because he knows that not that many surgeons here have experience with CDH babies. Also, that this area is right next to her diaphragm patch. At that point, he offered his time if the surgeons in Denver want to call him. He recommended that approach because she is not a straightforward case. He admitted that sometimes it is hard to get one surgeon to call the other, but if we can, he thinks it would be a good idea. Also, he welcomed us to come out to Florida.

The entire time I talked with Dr. Kays (15 minutes) plus the remainder of the drive, Ella cried. She was not consolable. I tried stopping the car and trying all the “normal” things. Nothing seemed to work. So, I just pushed through to the hospital so we could make our appointments.

First, we had the hearing test. By this point, Ella had been crying for 25-30 minutes and was exhausted. So she was asleep. They were able to administer 2 of the tests while she was at rest. One test required her to be alert and awake, which was not going to happen! They did see some things that seemed a bit strange, but since I have never thought she has experienced hearing loss and they did not have the awake test to see how she responded to noise, they deemed the tests inconclusive. They asked that we return in 3 months for a follow-up exam.

Next, we went downstairs to the blood lab. Fortunately, we were placed in a room with a nurse named Joe and he was a good stick. He was able to get into her vein within 2 attempts, which is good on Ella, and draw the necessary blood. It was over quickly. Since it always hurts Ella and she doesn’t enjoy the experience, the shorter the duration, the better. At least it went better than last week – – her arm is still bruised from 2 different nurses poking around several times looking for the vein.

Finally, we had a pulmonology appointment. Josh decided to meet us at the hospital. I was happy to see him because so much is at stake with Ella’s condition and we have some tough decisions to make. I wanted him to be able to interact with Dr. Abman and Dr. Lysinger, so that he could ask questions and we could get the information at the same time.

We had a great visit with both of them – – they are excellent doctors. They took so much time to talk with us and answer all of our questions and concerns. Ella retched during the visit several times and based on her body language, Dr. Abman thinks that she may have reflux occurring (related to the hiatal hernia). The Upper GI did not show refluxing, but that is not a perfect test for reflux (it could still be happening). He reiterated that many of her symptoms with the retching and even her pulmonary condition are likely affected by the hiatal hernia. He advised strongly that we get the surgery done soon.

We expressed that Dr. Kays agreed with the surgeons here in Denver. Dr. Abman admitted that he is biased, but gushed about how great Dr. Partrick is at surgery. This is consistent with what we heard when Ella was in the hospital. Plenty of nurses and doctors had nothing but great things to say about Dr. Partrick. However, Dr. Abman did think a surgical consult would be a good idea. Also, he is going to coordinate to ensure that a cardiac anesthesiologist is present for the procedure, due to her pulmonary hypertension and prior surgical history.

Dr. Abman confirmed that they have scheduled the bronchoscopy for the same time as the surgery. He confirmed that she likely is having some “floppy” muscles around her airway (which is common when babies are intubated for a long time). This could explain why she requires more air during the day than at night (normally our bodies are working harder to breathe when we sleep).

Then, we talked about her labs. Sigh…her BUN is 19 which is not great. We started to worry a bit because when she weighed in on their scale, she was 17 lbs, 4 ounces. Normally, we would jump up and down. But just 2 weeks ago in the hospital when they dried her out, she was around 15 lbs, 6 ounces. Ella gains true weight slowly. And even though Dr. Perry increased her food 100ccs over the past week (more fluid intake), there is no way that she gained nearly 2 pounds in 2 weeks. So, maybe she is getting some pulmonary edema again. That would certainly explain some of the extra oxygen requirement and heart rate going faster. Also, her potassium was low so they gave us a prescription to administer it to her at home for the next week.

In preparation for surgery (which is less than 2 weeks), I asked if we should do labs again next week. I certainly want her respiratory status to be good before she undergoes a major procedure. Even though they do not have clinic hours next week, Dr. Lysinger made an appointment just to see Ella. I thought that was really cool. So, we are going to the hospital next Thursday again for labs and a follow-up appointment to weigh her, check to see if she is getting wet, dry or staying the same along with her potassium levels.

Dr. Abman noted that Ella has not shown signs of pulmonary hypertension and stated that a side effect of the sildenafil could be an increased heart rate when it is paired with bumex. Since both of those scripts have been increased, that might be a cause too. So, he cut her sildenafil down 33% (a third less).

Josh asked specifically if there was anything else that we could do in the mean time to make Ella more comfortable, but something that would not have extreme side effects. We expressed how well Ella seemed to do when taking the steroids, but we know those have extreme side effects. So, they decided to place her on a inhaler of Flovent. So, now twice per day we have to use this inhaler with a spacer and mask. Tomorrow will be the first time we try that method. But because it is an inhaled steroid, very little is absorbed into the blood. So the side effect is minimal but it might help open her airway and make her more comfortable, especially prior to the surgery.

When we left, Josh and I both felt confirmed that Ella is supposed to have this surgery…and that she is supposed to have it here in Denver. On the one hand, we are nervous and it is scary but we know that the Lord will be with her and that He has a plan for her.

The title of the post might have thrown you for a loop. Today is the first day that Ella ever made noise in syllables. She kept trying “duh, duh, duh”. Before she would make noise, but it was a more continuous noise like she was testing her vocal cords. This was a more intentional noise, “duh, duh”. Admittedly, it made our hearts melt. She is normally so quiet; it is so good to hear her trying to make more noise.

Psalm 27:14
Wait for the LORD;
be strong and take heart
and wait for the LORD.

This has been our frame of mind for the past weeks. We have been actively seeking Him…waiting for an answer on the next steps for Ella…waiting on the next steps for me and if I should secure a job…waiting for God’s timing and for him to tell us “yes”, “no” or “wait”. Recently, it has just been “wait”. So although not everything has been uncovered, the reality of the Bun having surgery on September 3rd has hit home. Once again, trusting in Him and His plan and provision for this beautiful miracle He placed in our life.

Please pray that Ella will be able to maintain and get stronger for her upcoming surgery. Also, that we can coordinate a surgical consult; we feel that is important.

Finally, for us to be steadfast in Him. These next couple of weeks will be tough in many respects and the thought of seeing our baby girl endure more major surgical procedures is not a fun thought. Our journey has resonated with the truth that He is in absolute control and ordains all of our circumstances. We want to cling tightly to that truth as we enter into the next valley and always be aware that He is working around us all the time; we just have to look beyond our immediate perspective to see how he can take a painful situation and turn it into good for His glory.