A lot happens in a year…

Over a year ago we were still settling into our new home and getting used to being back in Colorado. We still miss our Texas friends and family – though we are pleased to be spared from the Dallas July heat – yet have been happy to see many of them here in Denver over the last two years. There is much to report with Claire: while we’ve been settling our mundane day-to-day, there has been a lot of changes for Claire, some fun, others unsettling. Major surgeries, enhanced ability to communicate, new diagnoses, new meds, and unexpected hospital stays are the high level items that define the last year for our family.

Claire’s had her first of two surgeries in January. Hip reconstruction was necessary due to a series of compounding drivers: Claire doesn’t get the necessary weight into her frame to promote healthy skeletal structure, her spasticity continues to stress and strain her bones, and her advancing scoliosis limits the time she can spend upright. These have contributed to her hip sockets degrading. Our care team elected to reconstruct her hip socket by removing bone slivers from her femur and bulking up the socket. This required an ostectomy: the heads of both femur bones (the portion that sits in the socket) were severed, a portion of the bone was removed, and then the heads were reattached with brackets and screws.

This necessitated a three-day stay in the inpatient unit at Children’s Hospital Colorado. Those three days were focused on pain management so we’d be confident Claire would go home as comfortable as one could having just had their largest bones cut in two. The next three weeks were the most taxing for Claire and the family, both physically and emotionally. This was the cute recovery phase, marked by sharp, piercing pains as the her body worked to heal. The second three weeks were a slow uphill climb to recovery with fewer sharp pains.

One of the decision points through this was what type of cast to put on Claire. The two choices are a Spica cast or a Hip Abduction (soft) cast. Because of Claire’s size and mobility, we opted for the soft cast. There were costs and benefits to each. With the soft cast, it is more comfortable and can be removed for easier cleaning. The tradeoff is that it is more flexible, allowing small micro movements while the bones heal: and Claire felt these movements. It was also more difficult to clean. On day three back at home, Claire soiled the cast and we elected to remove the cast for cleaning. The ensuing 45 minutes was a war zone and no feelings were spared. Working to immobilize certain joints and moving others so you can clean up your 10 year-old child is a horrible experience. Claire provided a soundtrack of screams as we worked to get to a point where we could hold her, comfort her and cry with her. It’s these recovery struggles that are preying at our minds a we prepare for the second surgery of the year.

Claire is scheduled to have her scoliosis correction surgery in mid-September, again at Children’s Hospital Colorado. Tiffany was operating on feedback she had received that the scoliosis surgery is more taxing than the hip surgery, until three weeks ago. Because the back is easier to immobilize and has fewer articulation points, the recovery should be less grueling. Tiffany was relieved to hear this because she was dreading a worse experience than the hip surgery. While that is better news, the truth is the scoliosis surgery itself is still higher risk. Because of the hardware involved and the size of the operating field, the risks of infection are some of the highest for surgical procedures. Infection occurs in 10-20% of patients depending on the quality, reputation and expertise of the hospital and surgical team.

Children’s Colorado recognizes the risks and has developed a high risk care pathway for each scoliosis patient: this pathway requires meeting with multiple specialties to assess what needs to be managed before, during and after surgery. Claire is cleared on all fronts except one: her weight has been falling the last few months.

Bouts with allergies on a regular basis have caused Claire to struggle with nutrition retention: in short, when allergies kick in, Claire can’t keep anything down – food or liquids – for 36-48 hours. We’ve discussed Claire’s limitations with managing her own saliva secretions: she forgets to swallow, she gags herself and triggers a vomiting session. When allergens start swirling, it make the secretion management much worse. Because of Claire’s ulcerative colitis, vomiting once causes an irritation in her stomach for those 36-48 hours and it rejects whatever nutrition we supply. This caused an ER trip recently due to dehydration and a spiking fever, so getting this managed is critical.

Furthermore as a result of the nutrition battle, Claire dropped more than six pounds since March. We started a couple new medications to combat: one calms the stomach and the other moves food through the digestive tract faster. The result is Claire has started putting weight back on over the last month. So fingers crossed we will keep the surgery date in September.

There are some additional medications we have introduced in the last year to the digestive ones mentioned above, due to one primary issue: Claire’s epileptic seizures have increased in strength and frequency. Another EEG was ordered for Claire at the end of 2016: the results came back that she was having near constant seizures through her sleeping hours. Before introducing a new psychoactive med, we decided to pursue a personal trial with CBD oil. We used Charlotte’s Web for an 8 week period before deciding that it was not impacting her seizure activity at the rate it needed to forgo additional neural damage. We do believe, however, it is having a material impact on managing some of Claire’s Ulcerative Colitis flares.

Two weeks ago Claire had blood taken and her White Blood Cell count was low. We have removed her ulcerative colitis meds over the last two weeks because her care team believes they may be causing the low count. To date she has not had any flares. We hope that between the probiotic (VSL #3) and the CBD oil, her colitis flares are managed. Time will tell.

Back to the epilepsy episodes: Claire is now on a small dose of Kepra. This followed an initial five-week attempt to use high-dose Valium to remedy the seizure activity. It didn’t work. We are in the middle of the experimental phase with Kepra working on getting the dosing right and evaluating if it is having the right therapeutic impact. More to come.

That constitutes the majority of the clinical report with Claire. I include this information for many of our readers who have followed because they have family that share a similar story to Claire’s. If you have questions about any of these items, leave a comment and we’ll be happy to provide more detail.

The last portion of this update is about Claire’s enhanced ability to communicate through technology. We got a new Tobii Dynavox machine about 6 weeks ago. We used to have a Dynavox (pre-Tobii merger) and did not get good use from it. It was unreliable and sketchy. The new one is awesome. Claire is still learning how to use it, but she’s getting better and better. Autumn and Heidi have assumed the role of making sure Claire has sassy, feisty and current phrases and slang to use. If Tiffany does something silly, it’s likely that Claire will add her “OMG” as commentary.

One of our favorite early stories with the Dynavox (we call it her “Talker”) involved one of her therapists. After a long therapy session, her therapist asked her if she wanted to say goodbye. Claire’s response: “Go away please. I’m tired.” Then Claire closed her eyes and went to sleep.

All of us are enjoying Claire’s new avenue to communicate with us. We are thankful she has a voice again.

The rest of the family is doing well. Lots of change to accompany Claire’s year, but nothing material to warrant complaint. We’re just happy to be a family and share our time together and with friends.

Thank you again for your continued support for Claire. We hope you and yours are doing well.

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