In an earlier post I mentioned my husband was trying a new medicine with some crazy side effects but some potential to improve his Cystic Fibrosis. We waited, did all the research we could, then decided to roll the dice and try it.
CFers that rolled the dice before us had coined the term “Hell Week” wherein the body adjusts to the new medication. Some CFers had a rough few days, some had a rough few months, some had no side effects at all.
Since Orkambi is such a specific medicine for such a specific population it is something of a celebrity in our minds – even hospital pharmacies don’t have access to it yet. When it finally arrived at our door after two months of waiting we greeted it with the appropriate pomp and circumstance, cradling the box it arrived in with the same tenderness we would carry a new baby.
He started Orkambi on July 8, deciding to give it six weeks come Hell or high water before giving up. In his mind Orkambi wasn’t the answer to everything, but it could hold the key to future improvements in his condition. He was choosing to be an optimist in light of all the answers the rapid research and push to approval didn’t provide.
At first he had flu-like symptoms. Fever. Increased cough. He was up most nights sweating profusely, then shivering. We had been warned and were not alarmed by any of this. Eventually his appetite was better and he started gaining weight. His sputum even got thinner and came up easier. So at first he was awful…and better.
He went in for PFTs (a measure of his lung functions) and had dropped 6%. The clinicians figured he must have caught a cold on top of the new medication and he would improve with time.
By September the flu-like symptoms had mostly passed but he still felt awful, had no energy, and his PFTs were hanging out in the low 50s (his baseline is normally in the high 60s). The CF team decided to cut his Orkambi dosage in half and see if that helped.
By the end of September when his PFTs continued to not improve he was admitted to the hospital for what he calls a “tune up.” These visits usually last a couple weeks, then he administers IV medicines at home for a while.
It was a couple weeks past the end of his home IVs when he came to me and explained he still felt lousy even after his hospital stay. This is uncommon, usually after a tune up he feels better.
“I want to quit,” he said in defeat, as if he had failed somehow. We talked it over, decided the only controllable variable that contributed to his drop in FEVs was Orkambi. For all of the trouble, money and side effects we were so hopeful to reap some benefit, but it didn’t go that way for us as it had for others.
He was a trooper. He stuck it out through [as best we can tell] a couple colds and a hospital stay. He made it much farther down the road with it than many have, and certainly longer than I would have tolerated.
We didn’t see it coming, but I’m beginning to suspect the hardest part of stopping Orkambi will be the giving up on something that held such promise. We knew it wouldn’t be instantly life altering, but we were hopeful it would make things a little better in the near future, and maybe a lot better in the distant future. Instead it was a long and winding staircase leading to a steep drop.
He stopped Orkambi a few days ago. He still feels lousy, but hopefully as the weeks go by he will start to feel better bit by bit.