Death in the Family

Yesterday my 20-year-old nephew, A., died from injuries he sustained in an automobile accident. I am waiting to hear when the funeral will be held. Dear Husband and the girls are headed for LA. I will be headed for Philly, either by car with my other brothers or by plane. A. was my fourth brother's only child. He survived childhood leukemia--a real miracle child for them. I last saw him when my mother died, 7 years ago. He was a devout child and wanted to be a priest. He was sweet and smart. He was very troubled in recent years and I gather his behavior became erratic and there were many problems. I don't know the whole story and probably never will. I know it caused my brother a lot of pain. And now this.

The bad news and the, well, what is the good news?

Tuberous Sclerosis Complex is a genetic disorder that causes benign tumors to grow on vital organs. When they grow in the brain, as they are for Firecracker, they cause seizures. They can also grow on the kidneys, lungs and heart, and the skin is usually involved as wel. "Benign" is something of a misnomer, since they can disrupt organ function just by being there. The specialist is pretty certain that Firecracker has TSC. In fact, when he examined all the films again with a neuroradiologist, he found 4 lesions, not the 2 he originally told us about. Unless there is a compelling reason not to, she will have brain surgery to remove the lesions. And she will have to have her other organs scanned to make sure they are clear.

There's no cure for this stupid disease, and there's no way to predict its course. It can cause just a few problems or it can endanger your life. All the information I've read states that TSC patients live a normal life span, at least statistically, but just how disabled they might be during that lifespan is unknowable. Some children are autistic and some severely retarded. Firecracker is neither of those, but the lesions are causing seizures, learning delays, and behavior issues.

We're waiting for the results of a genetics test. That will take 2 months. Because it's a genetic mutation, any children Firecracker has could also have the disease.

Dear Husband thinks I'm in denial, that I'm not here, because I'm not constantly in tears. I think I feel more fear than sadness. A kind of cold, clawing fear that has settled into my stomach. It makes me tense and then tired, waiting to find out when, how, what next.

God, what are you thinking?

This is one of those times when my patience is tested. I think as a family we've been through quite a lot in the past few years. But, no, there's still more. Dear Husband has recurrent sinus infections, which landed him at the ENT. The ENT found polyps and a deviated septum, but he also referred Dear Husband to an immunologist, because he has stopped responding to oral antibiotics. The immunologist took 6 vials of blood and ran every test imaginable.

The upshot is that Dear Husband has some sort of auto-immune disease, the exact nature of which is still to be determined. He's not making enough IGg or some such. The treatment for this is monthly IVs of gammaglobulin or whatever the stuff is.

He also has celiac disease. We've been kind of wondering about that possibility for a while, and his primary care doctor had a test run that came up negative. The immunologist, however, found several indicators (anemia and other nutritional deficits, IBS, lactose intolerance and other measures)and said the best indicator of all is how Dear Husband feels when he eats wheat.

Could life get any more complicated? Our family looks like this:

Me:
ductal carcinoma in situ, mastectomy and reconstruction, possible tamoxifin therapy for 5 years.
bipolar II

Firecracker:
Hypothyroidism
Epilepsy
Asthma
Reflux
learning delays

Dear Husband:
Interstitial Cystitis
Irritable Bowel Syndrome
Primary immunodeficiency
Celiac disease

The only healthy person in this family is DramaQueen.

But, hey, I found out that Outback has a gluten-free menu, and we have a gift card. I guess some provision has been made.

So, God, enough already.

Oh, did I mention that having primary immunodeficiency completely destroys Dear Husband's dreams of becoming a nurse, a career he has set his sights on for the past few years?