Decisions…

Faith’s last few doctor appointments have left us with some decisions to make.

Decision #1-Tonsils and Adenoids Removal

We saw Faith’s geneticist about a month ago and she was concerned with Faith’s baseline breathing and recommended that we schedule a visit with Faith’s ear, nose, and throat (ENT)  doctor. Faith has always been a loud breather and even more so when she is sleeping. She often appears congested and sounds “nasally.” We have always just figured it was due to her small sinuses and narrow nasal passages. I figured we would go to the ENT and nothing would really come out of the visit. I am used to hearing something that amounts to there is nothing we can really do to help with the exception of major reconstructive surgery. So, at the appointment we discussed what was going on and the ENT wanted to scope (Flexible Laryngoscopy) Faith to get a better look at things. Faith has had it done several times and truly I believe it is one of the most traumatic things for her. I usually cry with her and HATE for her to have this done but realize that sometimes it is necessary.  By scoping, the ENT determined that Faith’s tonsils and adenoids are moderately enlarged. We are not at a point where Faith would absolutely have to have her tonsils and adenoids removed but we discussed that we will probably need to make a decision in the next few months. The concern is that this surgery would be risky for Faith due to her other medical issues. The ENT isn’t worried about the surgery itself but the recovery. Of course, anesthesia is something that we consider as well when making decisions due to Faith’s endocrine issues. The decision would need to involve all of Faith’s specialists as well as us weighing all the pros and cons of the surgery. Personally, I feel like I don’t want to do it unless it is absolutely necessary and the ENT feels the enlargement is becoming more severe.

The enlarged tonsils and adenoids could be making breathing more challenging for Faith especially when she has any type of respiratory virus.  However, Faith’s low muscle tone in her neck and trunk and her narrow nasal passages don’t help things either. Unfortunately, we can’t fix those issues so I would hate to put her through a major surgery and then find that she still has problems which seems very likely to me. It is possible that sleep could improve with the surgery but I am not going to hold by breath on that either especially when Faith’s sleep issues point directly to neurological issues and a circadian rhythm sleep disorder. Additionally, we don’t have any reason to believe that she is having concerning sleep apnea. For now, we will be following up with ENT over the next few months and make a final decision as we re-evaluate.

Decision #2- Removal of Extra Teeth

View of Faith’s extra teeth

For awhile now, we have known that Faith has some extra teeth. Right now, Faith has 4 extra teeth. Two of the four are still coming through but they are visible. Faith has been seen by a pediatric dentist several times and he feels they need to come out. The question has always just been “when”. Due to Faith’s medical issues, they would have to be removed under anesthesia in the hospital. The dentist will not remove the teeth at his office. Since this would require anesthesia, we wanted to wait until Faith would require anesthesia for something else so we could do it all at once. We are definitely going to wait for the two coming in to completely get through so we can get all four out at once. Faith has had a few tongue bleeds but otherwise no major problems with them yet. Faith’s geneticist did some research and found another older boy with SOX2 that had extra teeth as well. We figured this was just another midline defect that resulted from the SOX2 gene mutation and that appears the case as of now. We are being conservative right now but will need to decide when to remove them.

Decision #3- Conformers/ Ophthalmic Plastic Surgery

Faith has had conformers for over three years now. It has been a very slow process. Her opening are still very small and narrow. Faith is still wearing peg conformers. We do know that children with anophthalmia especially those with SOX2 seem to have a much harder time making progress toward getting prosthetic eyes. At our last visit with the ocularist, she recommend that we go see an ophthalmic plastic and reconstructive surgeon to get his thoughts and for him to get an idea of where Faith is at with her progress. We figured we would get to this point so we weren’t surprised. We started the process with conformers to help Faith’s face grow symmetrically and to prevent additional ENT problems that could result from asymmetrical growth. Mike and I are truly ok if Faith never gets prosthetic eyes. I know some families really want their kids to get them so they look like other kids. I am not opposed to the idea but I refuse to put Faith through surgery after surgery for cosmetic reasons especially when there are no guarantees. God created Faith without eyes and I have accepted that. I don’t connect with Faith through her eyes and never will so I almost feel that Faith getting prosthetic eyes would be odd and uncomfortable at this point. Maybe if Faith’s eyes were our only issue to worry about or if the process was more successful from the start then our thought process might be different but really Faith just needs to be healthy and happy and I don’t believe that Faith getting prosthetic eyes would contribute to either of those.

Again, we are just going to the surgeon to discuss where we are at in the process and to get his thoughts. We feel we at least need to do that much. He may suggest doing something aggressive or he may very well say just keep going with the conformers now which we would be fine doing. We do want to make sure that she has the conformers in to continue stimulating the orbits and for symmetrical growth of her face. I just don’t think we are up for anything real aggressive at this point in time.

We have a lot to think about but aren’t stressing out about any of the decisions. Thankfully, nothing is urgent or absolutely necessary at this point so that gives us time to make the best decisions for our super little girl.

Keeping the faith,

Bridget