After two and half Months of Orkambi not sure what is going on?

[rosesixtyfive]

My son has been on Orkambi all of January and all of February. He got sick with a sinus infection at the end of January and ended up going on antibiotics February 4th. Nothing we have tried has made him better. This is the first time this has ever happened. The green mucus from the sinus infection became a cough. We were on a sulfa-based antibiotic until February 13th then started Augmentin, then went into hospital February 28th and came out March 2nd with a picc line and Cipro and cef abx that we are now using at home. Nothing has made him better. Nothing has lessened the cough. (not extra vests, not Sudafed, not the antibiotics, not prednisone, not extra albuterol puffs). He did have a fever of 102 for one night after coming home from hospital, so he could have a cold right now causing the cough. We are confused about what is going on. This is the first time he has ever been hospitalized for a cough. His main problem is severe sinusitis, and even now the cough still sounds upper respiratory. Lots of things are swimming through my mind - like is the Orkambi making the mucus too thin, is this not going to work for us? Are we making him sick to eventually make him better? It's such new territory even for the doctors. Lung capacity was significantly down at date of hospitalization, but cough has continued to stay in head and he doesn't feel too bad. Does anyone have a similar experience whose primary problem was severe sinusitis and the need for sinus surgeries, then started Orkambi and had a decline in health?

Thank you,
rosesixtyfive
mother of Sam, ddf508, culture Hflu

 

[kenna2]

I'm sorry you're dealing with this struggle. Orkambi has different affects on everyone. I actually had sinus surgery in December and that really helped a lot with my cough and breathing. What I do know from reading about others experiences with Orkambi is that lots of people get congested and do require hospitalization with the start of this med. Some have had their lung function decline as well. The title of the thread on this site about peoples reactions from it is called "for those taking Orkambi". I read it multiple times to prepare myself for what was to come when I started in July. So your son isn't the only one who has experienced these symptoms. You also have to remember, with this med, it's changing how his whole body works and naturally his body is going to be throwing a fit because of it. It's also getting mucus that's been stuck and buried deep down in his lungs out. Some people have seen results right way, some have not. Some people have stopped taking it because it became too much for them. Some people took awhile to get their symptoms to stop and felt better. If he is one of the people who cannot handle Orkambi, just remember there are other drugs coming down the pipeline that are made to do the same thing. Just some other ideas that might help:

1) Did his doctors start him off with the full dose (2 tabs twice a day)? If so, talk to them and see if they can reduce the dose so he can tolerate it better. My doctor started me out on 1 tab twice a day to get my system used to it. She then increased it over the next couple of weeks since I was tolerating it well.

2) See if your CF doctor can recommend an ENT doctor who sees/ specializes with CF patients. Many of us have had sinus surgeries (some multiple) and I know my cough improved after I had mine after being on Orkambi. My sinuses really are the main source that caused my cough. Having that cleaned out and starting fresh made a huge difference.

3) Also ask to see an Infectious Disease doctor. I have the same mutation as your son and some of the medicines that I normally get for CF make me sick. There are a couple that my Infectious disease doctor has recommended and have worked for me. Sometimes you have to play around with combinations of meds till you find the right ones that work.

Good luck!

 

[Ratatosk]

DS has always had sinus issues, his nose never runs, so if he does get a head cold we end up doing sinus rinses to get that junk out. When he first started Orkambi his symptoms were similar to a head cold -- stuffy nose, scratchy, hoarse throat --- coughed a little bit but it was mainly due to post nasal drip. He also had digestive issues. Seemed to last a few weeks.

IMO I concur with seeing an ENT specializing in CF, as well as an ID or at the very least have them run cultures to see what he may be growing. Could be he's on the wrong antibiotics for whatever he was culturing or it could be he needs to do some sort of medicated/antibiotic sinus rinse or sinus nebulizer treatment.

DS did catch some sort of bug a month or so ago. Started with a scratchy throat, then digestive issues (vomiting and other) and he ran a fever for over a week. Then he developed a head cold and we're still dealing with sinus issues, so we've been having him do sinus rinses to clear out that crud. Otherwise since being on Orkambi, his breathing has been much quieter -- no mouth breathing, snoring, cough/laugh....

 

[rosesixtyfive]

Thank you so much for your responses. This gives me some direction to take in my discussion with Sam's CF team. He has had two sinus surgeries in his life. He is now 7. During his admission last week, his ENT did not advise sinus surgery. He said he was congested, but there was still plenty of room for Sam to breathe. We will also ask to see an infectious disease doctor. He sees an ENT, but it is not an ENT who specializes in children with CF.
I will try to post an update after he goes off of the IV meds.

 

[Ratatosk]

Just a little side story about ENT's here. Years prior to ds being put on Orkambi, he gradually had difficulty breathing through his nose. Was a mouth breather, snored, would cough/laugh or gasp if he spoke really fast... We couldn't get any thing out of his nose with sinus rinses, when before we'd get a lot of thick, crud. Our local CF doctor was sure he had polyps, was sure he needed surgery and wanted us to see the local ent, but I was concerned because there are a number of CF kids in our town who have had many surgeries and kept having issues. Could be the nature of the CF beast...., but wanted a specialist Our CF doctor referred us to an ENT specializing in CF care because he felt in some cases sinus surgery wasn't warranted. So we made an appointment and the CF ENT scoped ds' and said there was a lot of swelling, no polyps. Even called in a colleague because there was something going on with irritation and his septum. Ordered a script for a nasal steroid and a CT scan. CT scan showed typical CF sinuses, but by that time, the nasal steroids got the inflammation down and ds was able to breathe much more clearer. So in our case it was a very simple solution, but it took a person familiar with CF. If we'd have stayed local -- we would've been pressured to have him go thru surgery to see what was going on....

 

[MissAlexsBP]

Hello!

There have been many responses to this on our Facebook Page! Several Facebook members with Orkambi experience write:

1. "It's not for everyone. I was on it for 4 or 5 months and didn't feel any different. Made me feel short of breath. So I just stopped taking it. If it makes you feel bad, just stop, it may not get bettet."

2. I'm 34, was diagnosed at 6 months. I was on orkambi for barely a full month, 2 years ago, i declined so bad and so rapidly that i almost got admitted to the hospital. Stomach/digestive issues, shortness of breath, extreme coughing (to the point of vomitting), and general exhaustion. I could barely get out of bed. Within a week of stopping the med, i was so much better. Unfortunately it took me abt 6 months to fully recover."

3. "My daughter 33 took it for over a year , she just had to stop taking it. She was having hormonal problems and had more exacerbations. : ("

4. "and they have the nerve to charge exorbinate amounts of money for this "experiment" !!!! They get paid, but don't have to pay for the recovery of those who have been harmed by it. Just "oh well"...!!"

5. "My friends daughter was on it for a few years and had a rapid decline she's getting better now that they stopped it."

Hope these responses help!