Monday, April 3, 2017

Week 189: In bloom

Spring has definitely sprung in Wisconsin, even if the temperature is still be quite chilly. The forsythia has its first tentative yellow blossoms, the garlic and rhubarb in our back yard have sprouted, and my new hobby of leafing through seed catalogs and dreaming is in full effect.

With these blossoms comes our old friend pollen. We know that Lemon has seasonal allergies. We deployed the bedroom air purifier, complete with brand-new HEPA filter. We went up from one daily dose of Zyrtec to two. We added Flonase back to our daily routine. And, I think, by and large, our approach is keeping things in check so far. Lemon still has some coughing and sneezing, but not too much. Of course, my subconscious refuses to update its operating parameters with information about the new season, so every time I hear Lemon cough in his sleep, I bolt awake, with my subconscious saying "Red alert! Cough detected!" Then, it takes a moment or two for my conscious mind to over-ride the distress signal with a "Hush, it's just allergies. And it's 1 a.m."

We continue to make incremental progress towards the new things I'm working to access for Lemon. After several phone calls with 4 different offices, I now have a range of estimates for how much it will cost us to take Lemon for a consult in Milwaukee. Of course, they can't tell you exactly which of the 5 numbers they quoted will be the right one (and actually it's 10 numbers because there's one bill from the hospital for the use of its space, and a second bill from the organization that employs the doctors for the use of the doctor's mind). But, we have a sense of what the minimum and maximum charges are and are comfortable with moving forward. So, I'm hoping to find time to reconnect with the social worker there and actually schedule something for June, once Lemon is done with school for the year.

We're also incrementally closer to getting started with feeding therapy. Our insurance agreed to cover at least the first few sessions, which is great, so I need to fill out a few more forms (there are ALWAYS more forms) and then we can schedule his two-hour evaluation. We're supposed to bring a selection of several foods that he will eat, and several foods that he's likely to reject. Suffice to say, the latter category will not be an issue. He did totally surprise me by eating several pieces of fresh mango at dinner tonight, so that's something.

This week featured some exciting news from the CF research world: Vertex announced the results of two clinical trials on its latest drug combination that treats the root cause of CF. You can read the press release here. One trial tested the drug combination in patients with two copies of the common F508del mutation, and the other tested the same combination in patients with one copy of F508del and one "residual function" mutation. A residual function mutation is one in which the CFTR protein (that is missing or defective in patients with CF) isn't totally missing/defective. Patients in both studies saw a significant improvement in lung function compared to patients who took the placebo, and patients with two copies of the F508del mutation also had a very significant reduction in pulmonary exacerbations. This is very exciting, even though Lemon's second mutation isn't traditionally classified as a "residual function" mutation. It does mean that we are one step closer to a drug combination that will directly help him--take a look here to see all the exciting things that the CF Foundation has coming down the line.

As you all know by now, research costs money. We're in the midst of our annual Great Strides fund raiser now, to help provide the CF Foundation with the resources it needs to continue this amazing work. To those of you who have already donated to our campaign, thank you, thank you, and thank you. For those of you who haven't donated yet, please visit our fundraising page. With the announcement of these latest clinical trials, we're so tantalizingly close to the medicine that Lemon desperately needs--please help us get there!



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