Updates Head to Toe

Hair- Keira has a couple of nickel-sized bald spots on the top of her head; apparently casualties of wearing her CPAP at night. I’ve bought her a satin-lined night cap to wear under her CPAP from now on to prevent pulling, but so far I’m not seeing the hair grow back.

Brain- Last year, Keira was diagnosed with “Continuous Spike Wave Cluster Syndrome.” Never heard of it? Neither had I. Basically, her brain activity is really funky when she sleeps. The neurologist said that little is known about the condition except that kids who are found to have it tend to show developmental regression. So, for that reason, she felt it was important to treat it. Bring on a new medication: Depakote. Interestingly, since taking this medication, Keira seems to sleep more and is more energetic in her therapies in general. Her most recent EEG looked much better, too. Her EEGs still aren’t normal, though we still have never detected an actual seizure and that’s good news.

Eyes- She is still benefitting from glasses. We are so thankful for the developmental ophthalmologist, who practices functional medicine in a way that is altogether different than the ophthalmologist or optometrist. Last week she got a new pair of glasses and she’s looking so grown up. We still don’t know how well she sees, exactly, but we do know that the glasses minimize her “googly eyed” look and seem to make it easier for her to control her eyes.

Ears- My biggest stressor in the past year was probably Keira’s rapid loss of hearing. A key feature of CMV, we weren’t altogether surprised by this, but it still sent me into a tailspin. Anticipating near or complete deafness to come, I spent months worrying about how to maximize her eyesight, learning sign language, learning about cochlear implants, trying to obtain a electronic communication device, and trying experimental treatments. Thankfully, Keira’s most recent hearing test showed that her hearing had stabilized, or in other words, it hadn’t continued to decline. She currently wears a hearing aid in each ear and is taking Valgancyclovir (the same antiviral medication that she took at birth) in hopes of mediating the hearing loss.

Mouth- Keira had 5 teeth pulled about 6 months ago. Because she doesn’t chew and work her mouth the way you and I do, her teeth are likely to resist coming out on their own. Also, pulling some teeth in advance will hopefully prevent overcrowding in her microcephalic (too small) head. She looked so cute without her front teeth, but it seemed that the adult teeth would never grow in. Last week, though, they finally broke through!

Throat/Lungs- Along with the spike wave syndrome, Keira was diagnosed with sleep apnea and we’ve spent the last couple of months getting her used to a full-face CPAP mask. Honestly, it’s gone much better than I’d hoped. Even though Keira never seemed overly tired and didn’t have difficulty sleeping before, she does seem more alert and engaged now that she wears the CPAP at night. 



Stomach- Keira continues to do well with the Nourish we’ve been giving her through her G-tube. She’s been growing quite a lot and seems relatively healthy, overall. We have been so excited to see her interest in eating orally improving. She almost always wants to taste what we are eating at the family dinner table and in therapy, she is working on chewing without a tether! She still is not able to take any substantial volume, but it’s fun to have her socializing with us in this way.

Hips- We went through a spell when Keira’s hips seemed really uncomfortable, particularly in the mornings. Given that she has already had 3 hip surgeries, it was good news/bad news to find out that her X-rays looked clean. The pain may be a combo of growth and the hardware that remains in her left hip from her last surgery. Next month, Keira will have surgery to remove those pins and hopefully that will increase her comfort.

Arms/Legs- K’s arms and legs are perennially stiff. She often wears her clothes to bed as opposed to changing into her PJ’s because it’s just so hard to move her limbs. We rely on the baclofen pump and Botox injections to help with this, but they are both limited. For more than a year we continually increased the baclofen pump dosage, hoping to further improve her tone and discomfort. Basically, doctors suggested that we continue to increase it until we saw negative side effects. Unfortunately, we seemed to reach that spot a few months ago. For the first time, we dropped her dose back because her dose increase had coincided with poorer head control and increased coughing/retching/choking. It appeared that the baclofen was relaxing her neck/shoulders area too much, and in a potentially dangerous way, so that set the limit on how much it could help her arms and legs.

Hands/Feet- We focus Keira’s Botox injections primarily on her hands and feet. Unfortunately, she can only safely get these injections every 3 months. And the Botox is at its best for only about 1 month out of each 3. Still, it’s probably worth it. Keira’s toes get so gnarled that they overlap each other and make it difficult (sometimes impossible) to wear any kind of shoes. However, at it’s peak, the Botox has her toes looking nearly normal and for a couple weeks she can sport her cute sandals or boots.