What it Means to be Strong

We are Bill and Victoria Strong. Our precious daughter, Gwendolyn DeBard Strong, was born perfectly healthy October 4, 2007. This is the journey that follows... the good, the bad, and all things Gwendolyn.

10.4.07 Gwendolyn DeBard Strong

Born - 10/4/2007 at 8:39am
Weight - 6 lbs. 9 oz.
Length - 19 3/4 in.

12.14.07 Infant Botulism Diagnosis

Gwendolyn was admitted into the ICU this afternoon and they are treating her as if she has infant botulism; a very rare (a few hundred cases a year in the country), typically soil born illness that basically leads to paralysis in the limbs and if untreated can quickly make it difficult for the patient to breath, swallow, and eat. The good news is that when we brought her in to the hospital she was still doing quite well, is still eating well, and seems relatively comfortable. We've been told that the antidote is extremely effective so assuming the diagnosis is correct once the antidote is administered she should quickly improve and should more or less fully recover within 3-4 weeks in the hospital. The antidote is very controlled and protected by the federal government (don't know why yet) but we received approval tonight and it is being flown in from some Army base as I type this. It should be here in the next few hours and will be administered immediately over a few hour period. It's obviously early and we're learning more by the minute but the pediatric neurologist and ER doctor we saw this afternoon seemed pretty confident that this is what she has. We're still in complete shock and scared and just trying to take it one step at a time.

4.3.08 SMA Diagnosis

As parents, we have learned the worst: Gwendolyn's illness is terminal. This is the most difficult news we have ever had to face and now share with those who care for us and our little girl. We are devastated, confused, and in disbelief. We have more questions than answers at this time, as we are still coming to terms with the enormity of it all.

What we do know is that Gwendolyn's illness is a relatively rare genetic neurodegenerative disease called SMA Type 1, which stands for Spinal Muscular Atrophy, also known as Werdnig-Hoffmann Disease. It is caused by a missing gene combination responsible for the production of a protein that is critical to the survival and health of nerve cells in the spinal cord that the brain uses to develop and control the muscles. We are apparently both carriers of one copy of this recessive, defective gene and unknowingly passed our copies on to Gwendolyn.

Given Gwendolyn's improvement during her hospital stay in December/January and thereafter, we remained hopeful that the original Infant Botulism diagnosis was correct. But the doctor's were always forthright that SMA had never been completely ruled out. Gwendolyn's clinical fact pattern was always described as "atypical" of both Infant Botulism and SMA. There are and probably always will be many, many unanswered questions around her specific case relative to the "typical" SMA case. Over the last month Gwendolyn's movement progress slowed and SMA was spoken of with more frequency and so a genetic blood test was ordered several weeks ago. We learned the results of that test on Monday. Unfortunately, we are told that the results of this test are 100% accurate. The prognosis is not good -- most babies don't make it past two years and many begin to plummet around eight months old. There is no treatment except to keep Gwendolyn as comfortable as possible, as happy as possible, and cherish every second we have left with her.


2.10.09 Honest Things

I've come to terms with the fact that I'm going to lose Gwendolyn to SMA, but I'm terrified of watching her slip away from me.

I appreciate when people ask questions about Gwendolyn; I hate when people don't and instead choose to make ignorant assumptions.

I worry about our $5mm health insurance cap from time to time; it didn't even cross my mind when we set the policy up 5 years ago at age 27.

I think of Gwendolyn's funeral often and I'm comforted every day when I walk past our church where it will be held.

I get anxious knowing that we'll have to save Gwendolyn's life at some point again; it comforts me to know we are well equipped to do so.

Most of the time I feel like it's next to impossible to focus on anything other than Gwendolyn and Victoria; but, they're all that matters right now.

Wherever we go, I know where the nearest pediatric intensive care unit (PICU) is and I know which one is best suited for SMA and our philosophy of care.

I'm now a complete germaphobe, but it's life or death for Gwendolyn.

I have a hard time remembering what it was like to not have a child with such "special" needs.

I've become comfortable with empathy; I'm not okay with sympathy.

I worry about the toll this is taking on Victoria, Gwendolyn, our family, our friends, and me.

It bothers me that we have to keep a bag packed for hospital emergencies; it stresses me out that we'll be using it again.

I wish that when people were unsure of what to say, they'd just say nothing.

I understand that all parents have to make weighed decisions, but every-single-simple-decision-I-make-for-Gwendolyn-has-actual-life-or-death-consequences.

I've become accustomed to the constant sounds of Gwendolyn's machines, but I think that when they are gone the silence will be deafening.

I worry about keeping Gwendolyn stimulated - bringing the world to her - but I know I am doing the best I can.

I'm no longer self-conscious about how "different" we are; whatever that means.

I've always had a "life is short" mindset; I never thought my daughter would be the one to teach me what that truly means.

I'm the furthest thing from a doctor, but I am one of Gwendolyn's caretakers and I know what is best for Gwendolyn.

When I'm in crowded places, I sometimes think about how many people around me don't know they are carrying the SMA gene; that makes me sad.

I've thought about whether we made the right decisions, not for us, but for Gwendolyn; her zest for life and playful smiles lead me to believe we have.

It's exhausting to constantly have to fight for everything and educate others; I wish the system were different and this weren't so, but I'll never stop fighting.

It kills me that our family is going to shrink, when it should be growing.

I am mindful that every time I hug and kiss Gwendolyn goodbye or goodnight that it may be the last time; and I make certain to cherish every single joyful one of them.