Eiche fam

Near the end of September, Namine was in the hospital for nearly a week. The cause was an mysterious infection, but her time there led doctors to discovering a second hernia — in addition to the one discovered in May. We had an appointment with a doctor from the surgical staff (the same one we met during Namine’s hospitalization) to discuss our options for repairing the hernias.

Hernia complications

Namine has already had one hernia repaired: when she was two and a half, she had her g-tube (an abdominal feeding tube) removed. They had known about the hernia on her left side for a while, and as long as they had her on the operating table, they wanted to address that as well. This wasn’t the side where the first new hernia was discovered, but it is thought that the hernia on the left side isn’t new; doctors believe the old one has reopened.

In talking to the doctors about the hernia repairs, we were told that (in broad strokes) there are two ways to go about it. The first is to attach the muscle to the bone. This is what was done back in 2011, and is the preferred method. The second way is to use a synthetic mesh. It’s reliable — I actually have this from a hernia repair of my own before Namine was born — but they are more likely to need repairing again than the kind that only uses your own body.

To give a little context, let me back up a little.

Namine has an encompassing diagnosis called femoral facial syndrome. It’s intended to be a single term that lets her medical care providers know, in a broad sense, what disorders she was born with. More specifically, she has the diagnoses of double-inlet left ventricle heart defect, Pierre Robin sequence, and caudal regression syndrome.

It’s that last one, caudal regression syndrome (or CRS), that has wider-reaching implications. It is her legs that are most evidently affected by CRS, but everything from the abdomen down is potentially impacted. It is also responsible for Namine having scoliosis, as well as clubfoot in both feet. All of that is fairly well known and documented, but CRS has also impacted other things as well — some of which we’re only discovering now, fifteen years later.

We know how CRS has affected Namine’s spine, legs, and feet. What we didn’t know, until recently, is how it affected — or how much it affected — the shape of her pelvis. A typical pelvis has a natural curve to it, but one side of Namine’s doesn’t: it’s flat, or flatter, anyway, than is ideal, especially as concerns a hernia repair.

As I mentioned, the doctors would prefer to use the body’s own muscle and bone to repair a hernia, without resorting to using mesh. Due to the natural, and to be frank, atypical, shape of Namine’s pelvis, they are not confident they can. Therefore, they will be using mesh. And that leads to another potential complication: if they find any infection in Namine’s ovary, fallopian tube, or elsewhere, when they open her up, they will not proceed with the hernia repair. It is possible — likely, according to the doctor — to infect the mesh itself, where the infection will persist. In that case, the repair will have to wait until the infection has been completely healed.

If the hernia repairs can proceed, there is one more unknown. It would be preferred to perform laparoscopically — that is, with minimal damage to the skin and intact muscle walls — but depending on the complexity and positioning of Namine’s insides, that may not be possible. If that’s the case, they will have to perform an open repair, which means a longer incision, more pain, and a longer recovery period.

Caudal regression and the female reproductive system

Talking about the reproductive system can be a sensitive topic. I am fully aware of this, and before I even sat down to write this post, I asked Namine’s permission to write about this aspect of her care. She has always been enthusiastic and open to writing about her life, even though it’s most often me doing the writing. But this, I thought, was more private. As such, it needed her explicit permission. In response, she said: “For me, it’s just another medical complication.”

We maintain this blog, in part, to talk about our lives. In the general sense, sometimes it’s to say hey, look at this vacation, or that cool thing Namine got to do. But the other aspect is to help, or even just to have the potential to help, another family with complex needs. Even one of Namine’s complexities is very rare, but there are others born with at least one of her conditions. We hope that our life experiences can help another — if nothing else, to hold on to hope.

Those wider-reaching implications of CRS I mentioned above? Another is in regard to Namine’s reproductive organs to an unknown degree. Ultrasounds and x-rays have only revealed so much. When we had met with the gynecologist, she explained that there were a few unknowns, all related to CRS.

The first is whether or not Namine has a cyst on her right ovary. Based on imaging, that seems to be the surest thing. However, it might be, instead, that Namine has a dilated fallopian tube — meaning that it’s filled with fluid. We won’t know which is true (or if it’s something else altogether) until the day of surgery and doctors are literally looking at her insides.

The second is the state of Namine’s uterus. It’s likely, based on imaging (again, for what that’s worth), that she has what’s referred to as a uterine horn. That means that instead of the shape of the uterus being somewhat rounded at the top, it is instead concave, resulting in two protrusions — the “horns” — where the fallopian tubes connect to it. There is, however, the possibility that Namine actually has two separate uteruses. (Uteri?) We’re in unknown territory, and again, we won’t know the state of her uterus until she’s on the operating table.