Sounds like CF to me, but pulm doesn’t agree...Advice??

Alorance9571

New member
I had a gene sequencing test done, and it came back as follows:

Finding: Presumed Positive

They found two disease causing mutations, G542x (now called 1624G). The other, is 3120G (now called 2988G) Both are heterozygous, but the testing couldn’t tell if they were on different chromosome copies. The report states that parental testing (which isn’t possible in my case) is the only way to tell if they are on different chromosome copies.

I’m seeing my Pulm for m. abscessus, and he doesn’t think I have CF, and says I’m simply a carrier, because neither mutation was homozygous. But, from what I’ve read, that doesn't matter. If you have two mutations (assuming they are on different chromosome copies), you have CF.

Symptoms (since childhood, 35yrs old now) are:
-Chronic infection of lungs
-sinusitis with polyps
-easy dehydration (I’ve found that no amount of water keeps me hydrated in the heat)
-a lot of dry salt on arms, neck and chest when I get too dehydrated and hot
-heat intolerance
-frequent constipation
-nausea (likely due to the dehydration)
-fatigue (also likely due to dehydration)
-frequent low potassium levels (leading to wicked foot cramps!!)

I can count on one hand the number of well days I’ve had in my life. I’m pretty much always dehydrated, I sweat a lot. In the last couple of weeks since researching CF, I’ve found salt to be the ingredient I may be missing. So, I’m testing that now.

Am I just reaching here, or does this sound like CF to you all? I’m awaiting a referral to a CF clinic, but I think my doc wants to send me to National Jewish in Denver. It typically takes a month or two to get an appt there.

From all of my research on this site and elsewhere, it seems that being diagnosed with two mutations is common, so my doc is really confusing me right now.

I really appreciate any advice you can give me!!

Amy
 

Aboveallislove

Super Moderator
Either the pulmonologist doesn't know what he is talking about or he did a poor job explaining it. Probably 40-50% of CFers are heterzygotes--having one copy of 2 CF mutations. Have they done a sweat test? It sounds like you are NOT at a CF clinic? If so, I wouldn't have them do anything else, but immediately get an appointment at a CF clinic with a CF specialist.
 

Alorance9571

New member
Not at a CF clinic yet...I'm waiting on my infectious disease doctor to give me a recommendation. His nurse called me with a recommendation for a pulm at a hospital that has a CF clinic (at UT Southwestern), but said that the doc would rather have me go to National Jewish. I asked her to get clarification on why I need to go to Nat Jewish now, and to make sure a pulm is who I need to see about all of this anyhow. I may go ahead and just call the number listed on the hospital website without waiting for a referral though.

No sweat test yet. But it's one of the first things I'm going to ask for when I get in to see a CF doc.

I really appreciate your response! I wanted to make sure I had my info correct....!

-Amy
 

Printer

Active member
Having been in your situation, I would tell you to get away from that Doctor as fast as you can. Get a new PCP who will, without question, give you a referal to an Approved CF Center and see a CF Specialist.

Unfortunally, the world is full of Doctors who can't spell CF, much less dx or treat it. You don't need to continue seeing one.

Bill
 

Alorance9571

New member
Thank you for your replies! It has been extremely frustrating dealing with the doc's. I've been going from doc to doc for 35 years, and luckily a surgeon I was seeing for my abscesses a couple of months ago mentioned CF, and suggested the genetic testing. I'll call the CF center on Monday and make an appointment. And in the meantime....arm myself with as much information as I can on this.
 

Beccamom

New member
I'm 36 diagnosed at 35. If you have the option to go to National Jewish I would recommend that. Make sure you see a pulmonologist specifically on the CF team.

Best wishes



Thank you for your replies! It has been extremely frustrating dealing with the doc's. I've been going from doc to doc for 35 years, and luckily a surgeon I was seeing for my abscesses a couple of months ago mentioned CF, and suggested the genetic testing. I'll call the CF center on Monday and make an appointment. And in the meantime....arm myself with as much information as I can on this.
 
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