Today Nikki from Children’s National called me and then got Southwest Airlines on the line to set up Belinda and my flight. It was very easy since Nikki handled everything. I just had to say things like where we were coming from and what days and times we needed. It is really nice that Southwest works with Children’s like that so people can have help with getting their kids these important surgeries!

Our flight is at 4:10 pm on April 21 with a layover in Nashville. We could have gotten a direct flight, but we are needing to leave as late a possible since Belinda has a dance competition that day. We still have to see how she will do with the competition since she will be on day 3 of a clear liquid diet at that point. She can have apple and white grape juice, and broth, so I’m hoping if she can get those down it will help her get quick energy before her dances. She wants to participate so much and I don’t want to take that from her. It’s the last competition of the year.

We are scheduled to return to Oklahoma City on April 25, and that’s a direct flight. If all goes as planned, we should be back here just before 7 pm. Perfect timing to just go home and REST!

After that, Belinda doesn’t have anything big until the Spring Formal on May 2. She will probably have to have soft foods at that point (mashed potatoes type of thing) but that should be okay.

Please pray for my anxieties about the flight. It’s funny that I have taken so much in stride, but I worry about flying. I haven’t done it very much, and this will be Belinda’s first flight. Also pray for the doctors and nurses that will be performing the procedure, and that Belinda does well in surgery. A small percentage of children who undergo this procedure have narrowing of the esophagus after, that requires more procedures. Please pray that Belinda stays healthy, and above all, that God’s will be done in all things!

It looks like surgery will be April 22. I am supposed to get a call to confirm that. This is such a busy time of year for Belinda. She will have a dance competition that ends on April 21, and then we will have to get on a plane and be in Washington DC that night. We will need to stay there until the 24th or 25th. The dance competition should be able to get her dances scheduled early in the day. I hope that doesn’t make too much trouble for her team. As long as that works out this will be a good time for it. She won’t be able to eat much at her spring formal in early May, but at least it will be over and done with and she can get some healing in before she goes to Girls State.

Once I’m 100 percent sure on the date, I’ll need to schedule flights and things. The hospital said we might be eligible for a waiver for some of the flight costs. Here’s hoping.

Dr. Polan’s office just called because the genetics test is back early! We set an appointment for April 18 for us to get the results. I asked if there wasn’t anything sooner and there isn’t, which is difficult to accept but I guess that’s the way it goes.

At least it looks like we will know the results before surgery. Even if it doesn’t make a difference in the surgery, I’ll be glad to have the information.

Here’s a short article about the doctors who will be performing Belinda’s surgery, and info about the POEM procedure as well.

https://innovationdistrict.childrensnational.org/poem-procedure-is-safe-and-effective-for-children-with-esophageal-achalasia/

This morning Belinda and I had a zoom visit with Dr. Timothy Kane, the Division Chief of General and Thoracic Surgery and Program Director of the Pediatric Surgery Fellowship Program in the Joseph E. Robert, Jr. Center for Surgical Care at Children’s National Hospital in Washington, DC.

Dr. Kane was really nice and helpful, and answered the question I’ve been struggling to get answered for so long!

I told him how I had read that many children with Ehlers-Danlos (if that is what Belinda has) have slow emptying of the esophagus and asked if Belinda could actually be experiencing that, but it was mimicking achalasia on the tests, and if it was that, if the POEM surgery was still the best option.

And he didn’t raise his voice, he didn’t talk over me without answering my question, he didn’t do any of that! He said it was a good question. And then he answered it. Amazing.

The doctors at Children’s National have treated patients with Ehlers Danlos, and those patients that aren’t experiencing achalasia pass both the manometry and Endoflip tests. Belinda did not pass either for several reasons, including the fact that the tests show her sphincter is tight. Her esophagus is also dilated because it is getting stretched by food that cannot get down, but in Ehlers Danlos patients, the sphincter is not tight and the esophagus is not dilated.

He said that although achalasia and Ehlers Danlos are not related, he has treated a few patients that have both. Those patients may take longer to heal after surgery, but he said that was really just for open surgery, not for endoscopic surgery like POEM.

Belinda also had a question, asking if she would be able to do backbends and things like that as a competitive dancer. She had concern that her stomach contents might back into her esophagus after surgery if she attempted those tricks. Dr. Kane said it shouldn’t be a problem at all. He has treated other athletes (he mentioned hockey players, wrestlers, and football players) and they have not had issues like that.

I asked if he thought there was any other reason to delay the surgery to wait for the genetics testing results, and he said he did not. So we’re moving ahead!

They are scheduling surgeries around April 17 right now. Belinda’s dance competitions for the year will finish on April 21, so that week will probably be when we schedule!

Very scary but Belinda is excited to be able to eat and drink normally again. And her first time in a plane! Also scary for me but exciting for her, ha ha!

I got a response back from Children’s National yesterday and we scheduled a virtual visit with Dr. Kane on Thursday morning to see if we can come up with a new treatment plan for Belinda without the genetic testing results.

She did better yesterday and was able to keep more food down. Today she seems more cheerful.

The genetics testing company called me this morning for genetics counseling. It was mostly what to expect and they asked for permission to do testing for a wide variety of things as well, which I approved. More information seems good to me. I asked if the results could be expedited. She said right now Belinda’s results look to be back at mid to late April, but if her geneticist called them and asked them to expedite so the surgery could be scheduled, they would probably do that. So I sent Dr. Polan an email asking her to do that.

Hopefully things will move faster. But if we have to go ahead and have surgery without the results, I guess we’ll have to do that. I just wish everything would fall into place!

I did not call Children’s about getting the ng tube back in. On bad days, we want that. On good days, we don’t. It’s hard not knowing what to do.

I’m so thankful that she was able to get more down yesterday! Thank you to everyone for the prayers. <3

Today we prayed for Belinda at church. After that, she was drinking a clear Ensure drink I got for her, in the hopes she could swallow it since it wasn’t thick at all, but the sugar started making everything stick and she had to go the restroom to get it out. She and I went to the kitchen after that and she tried to get more out there and take tiny sips of water. We ended up in the privacy of the children’s church office where she cried a lot. It’s just so much for her to take on. After some time in there, I coaxed her back to the church service. We stopped at the refreshment table and I got a coffee cup full of warm water for her to sip on, and that seemed to help clean things out. I really regret buying those drinks. It seemed like such a good idea, but it was not.

We had communion after that and I wondered if she would be able to handle the tiny wafer and cup of juice. Afterward she told me it was stuck in her throat, but it was small so it was bearable.

We had a church lunch and she ate all right but ended up needing to use one of her little bags to regurgitate in after. I hope she got some of it down.

The afternoon and evening was hard too. I had her try to drink a Complete nutrition drink and she couldn’t get it down. It was from the refrigerator, so room temperature will be the next thing we try.

She was able to drink about 15 ounces of chicken broth. Warm liquids seem to open things up better for her. But I don’t think she can subside on broth. She says that she is starting to feel really tired all the time, and she can’t think properly. She’s scared that if this isn’t figured out by this summer, she won’t be able to hack it in the Harvard summer school program she got into.

I told her I would call Children’s tomorrow about getting an ng tube in again. She’s worried that it will be a problem with dance. Her final two dance competitions are the second and third weekends of April. I think maybe she could get the tube in now, then take it out before the first dance weekend. She can’t do several of her dance tricks with an ng tube in. It’s expensive to get one put in but I am at a loss on what would work better.

I also did research tonight on what other parents with kids with achalasia are feeding their children. I have looked before but didn’t stress on it since she was doing okay. Tomorrow we have co-op and I’m going to bring broth and soup and a room temperature Complete drink and hope for the best. Maybe tomorrow will be a good day again and she’ll be able to eat nachos in the lunchroom. I hope so.

I also emailed my contact at Children’s National and updated her on what’s going on, to see if there’s anything we can do. Maybe we should just move ahead with the surgery without the results of the genetic testing. Hopefully I will hear back from her in the morning.

We went to Belinda’s pediatrician today. They are so good to her there, and they really listen. She said that since it’s only 10 pounds she’s not overly worried but wants Belinda back in a month. She wants Belinda to try to drink Ensure shakes or something like that. High calorie ones. Belinda hates sweet stuff like that and we’ll probably have to water it down to boot but we’ll see how it goes.

The ng tube makes it difficult for her to dance and she still has two competitions this season. A g-tube directly into the stomach is prone to infection and Belinda discovered the scar could look like a second belly button and she’s not okay with that.

The pediatrician is going to find a specialist in Ehlers-Danlos to refer her to, so we can make sure this is achalasia and not Ehlers-Danlos slow esophageal emptying. She mentioned a rheumatologist office she knows but also said we might have to go to Dallas or the Mayo. Mayo does televisits so that’s something.

We have to wait to see the cardiogist after we get a diagnosis of Ehlers-Danlos, from a geneticist or other doctor.

Called the geneticist office this morning and the results are not in yet. They said that if they do come in before May 22, they will call and get her in faster. I also confirmed that nothing was waiting on us – I was concerned that maybe I was supposed to pay the $200 to the genetic testing company to get things moving. But we have done what we are supposed to do, and now we just continue to wait.

Then I called the pediatrician’s office and told them what was going on and they got Belinda an appointment today at 2:30 pm. It will be good to go to her, get her caught up on everything, and get her advice. She always takes really good care of Belinda.