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.

So here’s why I mention Ehlers-Danlos in my posts.

Ehlers-Danlos is a genetic tissue connectivity disorder. Belinda has several markers for this. I read back in the hospital that one thing that can happen to Ehlers-Danlos patients is super-slow esophageal emptying. So it makes me wonder. Does Belinda actually have Achalasia type 2, or does she have Ehlers-Danlos and that’s causing slow emptying? And if it is the case, is the POEM procedure still the right thing to do?

The POEM procedure involves a doctor going down her throat and making incisions all around the lower esophagus so it won’t open and close anymore with eating. It will just be open all the time. It’s apparently the best thing for achalasia, but it brings with it other problems. And it’s permanent. So obviously I just want to make sure. But when I ask, I either seem to be blown off or they don’t understand what I’m asking.

I decided to wait until we get the results from genetics to push it, but I know I have to. I just want to be understood, heard, and answered to my satisfaction.

I know some doctors don’t like it when you look stuff up online, but I’m not stupid. I just need some clarification. I don’t think it’s too much to ask.

This picture is from Feb. 22, from the OEC Youth Tour Banquet. She sure doesn’t look sick, does she?

So since then, I found a very small facebook group for parents of children with achalasia, and that’s helping some. It is good to feel less alone.

They put us in touch with a pediatric surgeon in Washington DC, at Children’s National, who performs more POEM procedures on children than anyone else. They also said that we should have a pediatric surgeon perform the procedure, instead of an adult GI doctor, which is what the OKC doctor is. They said a surgeon is preferred in case something goes wrong. That makes sense to me, even though going to DC doesn’t sound great.

We never got in to genetics at Children’s in OKC, but we were able to get a referral from her pediatrician to another geneticist in OKC. This is who the pediatrician wanted to send us to in the first place, but the geneticist, Dr. Polan, had left Integris. But I found out through the Ehlers-Danlos facebook group that she had started her own practice.

Belinda went to Dr. Polan on Feb. 13, way up on the north side of OKC. She checked Belinda out and said that she definitely has a connective tissue disorder, and the genetic tests should show exactly what. She got samples and sent them out. The next appointment for results is May 22. However, that’s the latest they can get back, and if they come in quicker that can be rescheduled. Dr. Polan wants her to go to a cardiologist and I don’t remember what else. I need to get with her pediatrician and get that set up.

Oh, and a few weeks ago, the genetics company called and said our portion of this was 2500 and I said, obviously, I don’t have that and then they sent me a form to fill out and then our cost is now $200. And I think that’s not cool because what if I hadn’t asked? Everything is so expensive. I think the CT Scan was 3,000? Like the Tiger King, never going to financially recover from this.

We had a video visit with Dr. Petrosyan (Dr. Kane wasn’t there but I don’t know why) from DC on February 20. Dr. Petrosyan said we should get the genetic testing back first before scheduling, because if it is Ehlers Danlos, that can create heart issues and they need to know before anesthesia. That’s scary since she’s already been under several times leading up to this.

Anyway, so now we’re waiting on genetics and I’m going to call them tomorrow and see how that’s going and if the results are back. The swallowing is getting worse. She’s barely able to get anything down now. She was able to sip down a quart of hot chicken broth throughout the day.

As much as she hated the tube, she said she might want to get it back in because she is hungry. She said maybe a g-tube, which goes directly into the stomach near the belly button, because she hated the ng tube in her nose. But then she found out it would probably leave a scar and she doesn’t want that. We need to get this taken care of quickly.

Today I asked for prayer in church and I broke down and cried and was hard to understand.

I am ready for this season to pass.

My facebook post from November 3, 2023:

And then this facebook post, from December 6:

So that was a major disappointment. I really felt like this was the test that was going to help us, and they didn’t even know when it would be done. I wasn’t sure if I should take her to another location, or wait, or what. Not knowing what to do is such a huge part of all of this.

But then, on January 12:

We ended up spending the night at a hotel across the street, since the weather was so bad and I did NOT want to take a chance on missing the appointment. From my journal on January 14:

Belinda and I are in a hotel now, at Embassy Suites in downtown Oklahoma City. I feel a little silly about it because we came to be safe from the terrible winter storm that was supposed to happen, since she has a procedure at Children’s in the morning, but the storm wasn’t much after all. However, I did see several stalled cars and one that had slid off the road, so maybe it was the right thing after all.

The next day we were at the hospital bright and early! But boy, Belinda had a HARD time with this procedure. She thought she would be asleep again through it, but she had to be awake for it. She cried a lot but she had to do it. It was like having the ng tube put in, sort of, which she hated. They put a little numbing stuff in, and then they put a tube in her nose and down her throat to her stomach. Then she had to drink little sips and big gulps while the tube tested the pressure. Dr. Altaf was surprised and told us so. With her other tests, things looked normal. But this was not normal.

Belinda also had to regurgitate right after the test, because she had to swallow a big gulp of water and it got stuck. They didn’t have a vomit bag in there! I quickly dumped out the cup in the sink and she used that. You’d think they would have been prepared for that to happen. I guess achalasia is very rare though.

My journal on January 16:

Yesterday the motility test showed that Belinda’s esophagus is very abnormal. Dr. Altoff’s prelimiary diagnosis is Achalasia type 2. She has another barium swallow study on Thursday now, and we’re trying to get her genetic testing pushed forward, to check the Ehlers Danlos possiblity before any surgery. She had a hard time of it but she did a great job. Then that night she tried to do a somersalt over my footboard to be fun and lighthearted and it was already broken and it completely broke and she fell. And then she cried and cried. I know it was good for her to get it out but poor baby.

My next facebook post was Jan. 30. This was a hard day.

Facebook post from Feb. 7:

The new doctor, Dr. Rumman, told us that Belinda would be a fine candidate for the POEM procedure, which is good news.

Facebook post from February 9:

They also said she might need antibiotics for her left lung, and she was coughing a bit, but her pediatrician said we would wait and see how it goes. And it went fine.

Continued in Part 4