negative genetic testing, two postive sweat tests

uncertainty

New member
I hope it is ok to post here. I have not been diagnosed with CF, however, I have also been told that there is a possiblilty that I do still have some mild form of the disease. My history is a little complicated, so bear with me. I am 42 years old now, and I underwent CF testing at UNC hospital some 15 years ago. I had two postive sweat tests at that time, yet genetics were negative.
As far as I know, I was diagnosed with asthma at around six years old. Unfortunately, my mother had many mental health issues, so regular treatment and check ups did not happen. I did have a couple of hospitalizations, and numerous ER trips.I had a long hospitalization during my toddler years for severe anemia secondary to malnutrition, and I have always been very thin, even today. I will not deny that dietary habits likely add to that, but minimally so. I had three benign growths removed from my esophagus at the age of ten. After that, I recall only a few ER trips, but no regular MD visits.
In early adulthood, I began seeing doctors. The first asthma/allergist I saw suspected CF, and sent me to UNC for workup. Pancreatic function was normal, IGE level high, chronic sinus disease present, and another infection showing on a lung CT scan. At this time, I was told my lungs showed COPD. Two sweat tests were postive, yet I was not ever formally diagnosed with anything other than severe asthma. The meds they prescribed did little for the repetive lung infections, though they were somewhat helpful with breathing. I had sinus surgery as well, and that provided some level of relief also. Even still, not much did anything to prevent the infections that often occurred in my lungs, and in my mid twenties, I had what was deemed as Guillian Barre syndrome. UNC said it likely stemmed from a pneumonia I was fighting, yet even at that time, there was some uncertainty. There were some MDs who thought I could have had steroid toxicity, but in the end, the DX was Guillian Barre. I spent a year learning to walk and use my hands again. I have been to MD very little since then. Mostly, if it has been bad enough, I have gone to urgent care places for antibiotics. I kept quiet about all the medical history as I was afraid I would not get the antibiotics, but rather get sent for a bunch of testing that never helped much.
It has been hard at times not to have a regular MD, but I have a horrid anxiety when it comes to being a patient. I lost a sister in 2007, and my griefeventually led me to seek grief counselling. Eventually, the counselling went deeper, and soon, my health was mong the issues. ThisDr. worked diligently and patiently with me to help work through my some of my anxiety with doctors. My fear is that my lung damage stemmed from childhood neglect and a very rare abuse called munchausen by proxy. The psychologist thought it was important that my medical MDs knew of this as it could be significant, and eventually, I was brave enough to try seeing one. It was fine at first, ande I felt alright telling her about the abuse. It did not seem to matter to her, so I was relieved. Over the next year or so, I saw her a few times and tried numerous reflux medications as it was thought it may help with the SOB ( sometimes caused by reflux). They never helped, and the first severe cold Igot that winter was one of the worst. She gave me steroids and antibiotics, but after finishing them, I was no better, and was in much pain. I went back, and she wanted to change the reflux meds. At that point, I told her I wanted nomore reflux meds as I did not think I even had relux. Basically, she got angry and told me I should have come back sooner if the pain was really that bad. It had been less than two weeks, and I returned after the antibiotic course. She asked what I expected her to do, and I suggested a CXR. She ordered it though stated she did not think it was necessary. The CXR was normal, and of course, I got a callincinuating the pain was from the chronic cough, and nothing more. I was instructed to take advil and do another course of antibiotics. I knew that something more was wrong though, so I went to see an ashtma/allergist. She was very thorough, noted the postive sweat tests from UNC years earlier, and sent me to DUKE for a revaluation. Duke performed a CT scan that showed a colonized infection in my right lung and a broken ribon the left. They also found no evidence of reflux, and also evaluated me for vocal cord dysfunction, which I do not have. I was tried on Xolair for high IGEs, and a repeat genetics test for CF was done, still showing negative. The xolair was disconitued after three months as all of my symptoms worsened. I have not been back to a doctor since that time, about three years ago.
I am a mother now. My youngest boy ( he is 6)has developed some respiratory issues over this past year or so, mostly a cough that seems to stay around. He has frequent nose bleed and nasal congestion, and I suspected allergies. Placing my anxiety aside to do what is best for him, I scheduledan appointmentwith the allergist I saw several years ago. I did not stop seeing her because I did not like her, I stopped because I feel very confused and uncertain about what I really have. She was very nice and was very happy to see me in her office again. She is actually good with my anxiety, and took it into account when evaluating my son. She thinks he needs to have a sweat test, and I am so terrified. And I know that it is not helpful to speculate, but I worry that if he is positive, he will also test negative genetically, so what does that leave for him? I feel no one has really ever known what to do with me, and mostly, I have felt to be more of a lab rat than an actual person.I do not want this for my son. I also realize that my own reluctance to see MD has likely worsened my symptoms, and of course, I cannot allow that for my son, either. I just want to feel that the doctors will do the right things for him.
Anyway, sorry this is long and complicated, and please accept my apologies if this is not the appropriate place to place such inquiries. I am just curious if anyone has received a CF dx without having a postive gentetics test? Two asthma/allergists believeI have some variation of CF, but UNC would not really say yes or no. Of course, that was fifteen years ago, but DUKE did repeat genetic testing nearly three years ago and no identifiable mutations were found. Any input is appreciated.
 

Printer

Active member
Up until reading your post, I thought that I was the most misdiagnosed person in the world. I may have to take second place to you.

The most important thing that you have to realize is the past is water under the bridge and you need to start fresh. When you had the genetic testing 15 years ago even 3 years ago, they most likely tested for 250-500 mutations. Today there are almost 2000 known mutations. You need a FULL CF SEQUENCING, nothing less.

I can tell you that, should you be dx with CF and begin treatments, your quality of life will improve drasticly.

Lastly, you need to go to an APPROVED CF CENTER AND BE SEEN BY A CF SPECIALIST. Only then will you have a real opportunity at a proper dx.

Good luck,
Bill
 

uncertainty

New member
Thank you for your reply, Bill,as well asyour offer to help. It is my understanding that both UNC hospital and Duke University are CF accredited. The first workup many byears ago was at UNC, and entailed pancreatic function testing, too. I do not believe that Duke did anything more than repeat the genetic testing. The MD I saw seemed very interested in whether I had vocal cord dysfunction, but when I did not, she offered little regarding the chronic cough and frequent infections. She suggested leukotrienes ( sorry for spelling).reventative antibiotics, and to continue the Xolair. I did try for another month, but the Xolair felt to me to make things worse. I improved within a few weeks of stopping the injections. I haven't been anywhere since then. It has become an issue again, though, in light of my son's symptoms ( though I was worse at his age than he is). He is scheduled for a nsweat test at UNC on July 9. I have been torn with what to do, but I spoke with a very nice lady at the CF foundation. She told me I should be getting treated for at least CFTR related disorders, and that there are differences between this and asthma treatment. I do not undersrtand why it would not have already been suggested, and the MD at Duke never even talked about the history of positive sweat tests or the possiblitity of this CFTR related disorder, so I do not know.I just do not know, Bill, and I think I would be more sick if it did have CF, yes? It is so hard as my allergists think it is more than just asthma. and there is some level of explanation for COPD signs at such an early age. I understand that the University of Iowa is one of 15 aprroved for CF research, and I have thought of contacting them. I do not have a current physician, and I cannot self refer, so, I'm not sure what to be doing. Again, Bill, thank you for your effort.
 

Melissa75

Administrator
You might consider PM'ing a woman on this site: Jennifershope
She has been dx and un-dx and re-dx with CF. Her sweats were positive but then attributed to thyroid issues. Of her two gene mutations; one was called disease-causing, then called benign, then disease-causing again. She had the nasal potential test--even that outcome was controversial. And she's your area (NC) as well.

I think you need to find out why your sweats are positive. Maybe you do have CF. And if you don't, you have to treat your health problems regardless. The dx of CF can be important in terms of what meds and treatment you have immediate access to, BUT if you see a good doctor and stay on top of things, you can get CF-type treatment whether you have the dx or not. If you are pancreatic insufficient, you can get enzymes. If you have copious thick mucus infected with bacteria, you can have antibiotic treatment, mucolytics, physical therapy and more. If you have allergies, you might still try immunotherapy since you didn't have a good experience with xolair. Also, visit NoExcuses blog and read all she has to say about allergies.
http://noexcusesnoexcuses.blogspot.com/search/label/Allergies

FWIW, my sweats were negative as a baby and as an adult. With the bronchiectasis dx alone, I have access to plenty of treatments and meds, and my sputum is cultured regularly. I have also had drs eagerly test me for vocal cord dysfunction and reflux in hopes of discovering they are the cause of my issues. I do have reflux on the tilt test, but my endoscopy and Bravo test are normal (apart from the pile of mucus they viewed in my stomach and assumed, correctly I think, that is what I swallowed from my lungs/coughing). So no reflux, but no handstands for me after I eat either.
 

Printer

Active member
Uncertainty:

Massachusetts General Hospital in Boston is an APPROVED CF CENTER. There are about 2000 Doctors working for MGH. Less than 10 Doctors at MGH are CF SPECIALISTS. The same is true for every CF Center in this country It is not enough to be seen at a CF Center.

If you had a brain tumor, you would want a BRAIN SPECIALIST. The same is true of CF. I repete that I doubt that you were screened for the almost 2000 known mutations, which for people like me is like not being screened at all. You need to have a FULL CF SEQUENCING done.

You should also know that all sweat tests are not equal. That too should be done in the CF Center portion oh the hospital.

Bill
 

uncertainty

New member
Thank you both for your input. Melissa, I have heard of the nasal differential testing, but it is my understanding that is is still a research test, but does carry promise. I was told it is only done a JohnsHopkins in Maryland, and in many places in the UK. I would like to have the sweat tests repeated, as maybe they were somehow false positives, but I have been told it is unlikely in lieu of the repetitive lung infections and other symptoms. My thyroid function is normal as far as I know, and it was checked with the positive sweat tests results. It is scary to hear of the lady you speak of and her struggle with getting an appropriate diagnosis.
Bill, yes, I would be interested in being checked for all the mutations, and it is something I will look into. I also looked up the MD I saw at Duke as I was not sure if she is a CF specialist. She is listed as critical pulmonary care and asthma/allergy, but she is not with the CF clinic. So, yes, perhaps I need a CF specialist. The CF foundation also strongly recommended this, but it's hard to have faith in UNC or Duke right now because of so much uncertainty. Is Boston more equipped, do you know? And can I be evaluated in another state, and still get treatment in my own if it is needed? I know you probably can't answer those questions, and I will look into it as well.
Again, thank you both, and Melissa, we share the same name. <img src="i/expressions/face-icon-small-smile.gif" border="0">
 

Printer

Active member
I can answer that YES you can get a 2nd opinion in another state. I would recommend Craig Gerard MD at Boston Childrens, Adult CF Clinic.

Bill
 

uncertainty

New member
Bill, thanks again for your opinion. I am looking into the possibility of going to another state, so I appreciate your referral. It's tough as I worry that if genetics remain negative, I will still be a "gray area" patient, and quite honestly, I find this to be very difficult. Mostly, it is because it does seem that the MDs are never quite sure how to treat what, and even when I know I need antibiotics, it has sometimes been a struggle to get them. And I assure you that I hate taking medication, so running to the MD is not something I do very quickly. That said, when I do go because I know something is wrong ( I believe I can actually feel lung iunfections), they look at me like I'm a little 'off' rather than a woman who knows her body. And I have been told I have so many things, so it is frustrating. But again, Bill, thank you so much. My son gets evaluated at UNC next week, so getting through that is taking precendence over me right now. I am glad to have had the courage to post here, though, as now, I feel a little less lonely and hopeless.
 

JennifersHope

New member
Hi.... I was the misdiagnoses queen. I was diagnosed with CF at age 29 via three positive sweat tests but also have a disease called Addison's disease which can cause false posituve sweats when the diesease is not properly controlled.

My whole life I had chonic cough, bronchitis, pneumonias etc. I was dx at Columbia Hospital in NYC then received care in a local CF center where I lived.

I then moved to SC and went to MUSC where they felt I did not have CF..i pursued testing, FIrst I had the full genetic testing which was Df508 and a Q1330E and at the time they were not aware of the significance. I went to UNC and had a Nasal PD, just one while I was sick and it was totally negative. MUSC used that as ground to undiganose me and stopped all my CF medications. I progressively got worse and worse.

I went to local dr and was found to have MRSA, PA and MAC and was hypoxic. I then pursued a more specialized out of the normal CF center and went to National Jewish Hospital in Denver Co, Mostly it was for my MAC infection. I was put back on all my CF medications and my lungs are doing amazingly well, I was also put back on enzymes which though I am overweight cut down on the amount of times I was going to the bathroom a day but a lot.

If you do not fit the normal CF sterotype it is very hard for some doctors to understand it, even CF doctors. I highly respect my CF doctor that undiagnosed me with CF. He was a great guy. He was right about a lot of my other health issues..that are even bigger then the trouble CF is causing one of them is Vocal Cord Dysfunction. I have that and I have one of the most severe cases that National Jewish has ever seen. They considered and recommended have a trach put in me because I have been put on life support so many times for my airways shutting down.

National Jewish has an amazing VCD clinic and I spent three weeks there learning techniques. I do not have asthma. I also have very severe reflux which causes me to be short of breath too.

Overall, I would say no one can say for sure but I have never met a man more smart about atypical CF then Dr Nick from National Jewish hospital. He is outstanding. If you can go there and get his advice, I highly recommend it.
 
S

stephen

Guest
If you are looking fro another opinion, I can highly recommend National Jewish Health in Denver. They are consistently rated the #1 respiratory hospital in the US - and for good reason. Seven years ago, after seeing numerous "Top Doctor" specialists at some of the top New York hospitals over the course of many years, my pulmonologist suggested I go the National Jewish. They have a program where as an outpatient you are evaluated daily over the course of up to two weeks. Each day's tests are based on the previous day's results which are evaluated by a medical team. My evaluation lasted five days. It included extensive pulmonary, blood, digestive, hearing, and most importantly full genetic tests. The result was a diagnosis of CF. The trip from New York to Denver was a life changer, thank G-d. I was 63 years old at the time. For anyone wanting/needing another opinion, my money is on National Jewish! Stephen 70 years old with CF (D1152H, G542X); Bronchiectasis; FEV1 47%
 
U

urmysunshine54

Guest
Uncertainty - my heart truly goes out to you for what as been the ordeal of a lifetime. You've come to right place - as this forum has both empathetic and truly educated CFers that are so great at sharing their knowledge and experiences. I'm in my late 50's and was just diagnosed with CF by clinic this year (postive sweat test, no CF genes, but some kind of mutations)...so I'm defintely one of the new 'kids' on the block. I have so many questions (3 bouts with pneumonia in the last 6 months) and get so many insightful replies, comfort and support here. Quick background...I've been sick for several years with sinus infections, 2 sinus surgeries, regular bouts of bronchitis and pnuemonia and culture all kinds of bacterias.

While I'm still learning about all of this, just wanted to provide you with some moral support. Please let us know how both you and your son are doing...my thoughts and prayers are with you.
 

uncertainty

New member
Thank you everyone . . . . . i'm sure you can all imagine at some level the frustration with being diagnosed as a 'maybe'. I have been so turned off by MDs, and now, not having a regular MD is a problem, too. I need one to refer me to a CF clinic, atleast this was what I was told by UNC. I have read so much since they have wanted my son to be tested, and I cannot help but wonder why I have yet to be referred one, despite repeated negative genetics. If I am understanding what I have been reading correctly, characteristic features combined with laboratory evidence is criteria, yes? If I remember correctly, my sweats were in the 70s both times, and there was no thyroid malfunction or pancreatic malfunction. As for respiratory symptoms, well, there have been many, and this continues to be a problem. I do have asthma with high IGE levels, and I have been told I have chronic bronchitis as well as COPD ( COPD first told to me when I was around 21yo). I feel very fortunate that I am doing so well, and really, I have only been hospitalized for pneumonia about five or six times the last fifteen years, minus the Guillian-Barre Dx in my mid 20s. I will be honest and say I have not had much workup since then due to my hesitancy to continue the struggle to "know" what is really going on. Seems I may never really know, and this feels it will be a problem getting appropriate care. I have done well using urgent care centers as needed for antibiotics, but I admit that things seem to be worsening slowly, and as I am a mother now, my health has become more important. I also have great concern about the possibility of my son having a positive sweat, but also negative genetics. It worries me thatthey are putting him through this simply because of my testing, which apparently indicates nothing, but is significant??! I tell you, I like to think I have a decent level of intelligence, but I am stumped on this one. The CFF told me last week that I indeed met their criteria for DX, so I am confused because of not receiving it at a place they accredit.
Again, thank you all, and I have been reading about the Denver center. My son goes for a sweat next week at UNC, and I spoke with a lady there thatsaid they may be able to refer me to their clinic without seeing me as my insurance does not require an authorization to see a specialist. Most clinics require a physician referral, though, and to get one, I must start over with a regular MD; and honestly, it is an exhausting process as many of you have seemingly experienced. I am really surprised a CF specialist was not suggested by Duke's asthma/allergy department when I was there three years ago, with another colonized infection! I don't know . . . maybe I do not get the common bacterias associated with CF, and so perhaps it is just asthma, but it sure would be a comfort to be even a little more sure. I don't recall ever having cultures anywhere except during the hospitalizations (staph and pneumonia).
Anyway, thank you allfor listening, sharing your input,and being so kind even though I am not a CF patient. Maybe I will feel better with it all after my son's testing.
 

JennifersHope

New member
Call National Jewish they will deal with your insurance. I was there 43 days...but in their outpatient program three weeks! It changed my life!
 

JustDucky

New member
I really do hope everything falls into place for you.. If your insurance doesn't require a referral, then just make the appointment. My insurance was like that, I never needed any referrals, I could just call up any specialist and make an appointment. It just makes things a whole lot easier if you don't have to involve a GP who knows absolutely nothing about you, your fate is basically in their hands if that is the case.

I was in diagnostic limbo for so many years as well, even called a hypochondriac at one point....so many of my systems were just going haywire. I honestly felt like an old car, fix one problem and another problem shows up.. In a way, I could sympathize somewhat with what the doctors were dealing with, so many things were going wrong and they didn't know why. Some of them handled it better than others, yet there were a few who became dismissive. Not only did I have problems with my lungs, but I also had autoimmune tendencies, digestive problems, developed diabetes, wound up with a myopathy that severely affected my breathing, etc..the list goes on. To make a very long story short, a new GP on my case suspected CF because of the severe respiratory and digestive issues that I was experiencing. My sweat tests were in the 50's and genetics ultimately found one mutation and a variant, and so at 33 years old, I was diagnosed with atypical CF.
As time went on, more and more pieces of my crazy puzzle came together......I was finally diagnosed with Ehler's Danlos syndrome, which explained my joint pain and dislocations, stretchy skin, severe reflux disease and SVT.. They also found out that I had Hashimoto's, which destroyed my thyroid. The myopathy is still somewhat of a mystery as they don't know why it happened, just that it did. As a result, I am trached and vented eve's and nights to give my tired muscles a rest.

Complex, yes.......it took decades to figure this out. Hang in there, go where the top notch doctors are and it sounds like Nat'l Jewish would be right up your alley. Being that you have two positive sweats and the symptoms that you describe, CF should definitely be on their radar. I can certainly understand your frustration. If you get a CF diagnosis, you will finally get the treatment that you need and I promise you that your health will improve.. Let us know what happens, I will be thinking of you.

Jenn 40 wCF
 
K

katethekid

Guest
I have had everything from negative to positive to borderline sweat tests. My dr's say it's because I have one of the more rare mutations. I am going to have the full sequencing done soon to see if we can pinpoint it. I had lung problems when I was younger, but after age 8 or so they almost disappeared until the last couple years. However, my pancreas started causing problems in high school. My bile ducts were completely blocking off. Several surgeries eventually helped, but still didn't solve the problem. I hope they can help you soon <img src="i/expressions/face-icon-small-smile.gif" border="0"> Hang in there.
 

uncertainty

New member
I am a bit speechless at the empathy in all of your replies. I feel somewhat out of place as my physical issues now are not nearly as complicated as what most of you describe. It may very well be that I have nothing more than chemically induced lung disease, but man, those two sweat tests are really causing questions. It is not much comfort to know that having them repaeted now may not matter, either. I have placed call to both National Jewish and the University of Iowa. I spoke with a nurse at National Jewish, but shefelt she would rather have a more experienced CF nurse speak with me, so I am suppose to receive a call back. Same with the University of Iowa ( it is suppose to be one of the 15 accreditted CF research hospitals). Both sound like all of you and the folks from the CF foundation, strongly recommending more evaluation for me, so I am taking steps. Right now, I just want to get through my son's test, and hopefully, have that worry lifted/changed/confirmed, whatever may be the case.
Again, expressing gratitude to those of you who have reached out to me seems so insufficient, but I do sincerely appreciate the sharing of your knowledge and experiences. It has been more helpful than you can know. Hope you all have a great July 4th!
 

LittleLab4CF

Super Moderator
If it looks like a duck, walks like a duck and quacks, chances are it's a duck. A few weeks ago, two topics covered a lot on 'when it is CF or when it isnot CF', and M470V? Before genetic testing for CF, the sweat test was, and by all measures, still the gold standard for diagnosing CF. From your post, I can't tell if your genetics were negative because you didn't have two identical CF mutations,rather theCF mutations they foundwere a single copy. If you have none of the nearly 2000 known CFTR gene mutations, you do not have CF. Genetics is considerably more noble than seeing how you sweat. CF is a monogetic autosomal recessive genetic disorder. The short version isthe condition of the CFTR (cystic fibrosis transmembrane regulator)geneis solely responsible for having CF or not.
Of the 2000 mutations, you must inherit one copy of the same mutation from both parents in order to present CF. Many CFers have two or more identical pairs of various mutations, this polymutation andconditions that two identical DNA sequences in the geneare missing, can signal more severe presentation of CF, but not always. The assumption is since the initial analysis of the CFTR gene mutation was recessive, then when a single copy of a mutation or mutations show up in a genetic test, the working gene or a combination of single mutations the gene functions normally.
Nothing could be further from reality. Two people with identical mutation pairs most often will present CF differently. One may have mild pulmonary symptoms and the other may require a double lung transplant at 15. Other sequencing errors such as M470V with a 7T/7T polymorphism once thought to have no effect, current thinking is it can be just like classic CF, but generally more mild pulmonary presentations. A polymorphism is just extra copies of DNA sequence before moving on. As an example the 7T polymorphism the double helix repeats thymine seven times. It is sort of a DNA stutter.
Having a single copy of a mutation or mutations makes you a carrier but the recessive and dominant bit is like a mixed race person. You probably would have a number of features that lean one way or the other rather as a mix. Mendelian genetics where everything is blackand white works doesn't cypher with CF. Recessive genes can present anywhere on the scale although the same recessive mutation follows the same general range of presentation. We are finding that a carrier can present classic CF symptoms. This sounds like you can have CF for all practical purposes except the genetic analysis, which ultimately is just information.
You don't mention where your father is or was in all of this and most often a single parent can get away with munchausen by proxy. My parents were as close to perfect as any child could hope for. However my sister, 7 years younger had severe bipolar I. I saw munchausen by proxy, in action with her two daughters. I lost her in 2008 adding to the statistics of the number one source of mortality in mental illness, a drug overdose took her and left my wife and I with a 10 year old niece. Considering how limited conventional doctors are in understanding the ramifcations of depression, excepting psychologists etc. dealing with the complex issues surrounding munchausenby proxy may be left on the editing room floor for a psychiatric professional. CF doctors are most often pulmonologists and for treating these symptoms, they cannot be beat. When they get into CF genetics or GI issues, again, they are most often winging it. I found that utilizing specialists for my special issues works best.
Guillian Barre can interfere with muscles and nerves of the face and neck. Specifically what I wonder about is whether you asperate some of everything you swallow. GB and Parkinson's syndrome are both able to interfere with the mechanism of swallowing, either messing up the timing such that the epiglotis may not fully close in time, or the epiglotis may prematurely open. The worst is when a step get out of sequence. A good ENT can knock this off quickly. It sounds like you have a non productive "smokers cough". CFers have this presentation quite often. I might suggest a visit with a GI specialist because leisions in the esophagus aren't to be taken lightly. Two close friends had esophageal cancer. One is alive, one didn't make it, and both hurt me to the core. Gastritis is symptomatic in CF as are the conditions resulting from gastritis. Gerd, aspiration etc. each or all could be a silent presentation.
For the record, I am 62 dx in 2001 via sweat test and I have chronic acute pancreatitis. My pancreatic function test yeilded a new record for bad. I was tested genetically in 2003 and I am a carrier; S1235R and M470V with a7T/7T polymorphism. This may change soon as I am planning to be re-tested. I cringe everytime I see someone dx/ 3mo. or at birth. Born in 1950, there is little doubt my father had CF. His dx was postmortem. Sincemy father and I shared the same doctor, I was his medical miniature. In retrospect, it is beyond my comprehensionour doctor, from my birth til 20yrs never tested me for CF. My father died from malnutrition.
What caught my attention is your son has been recommended for a sweat test, and understanding too well the odd looks, confusional paralysis from your doctor, and now his doctor. Few things are as taxing as being sick with no better dx than " you have the worst case of whateveryou've got, that we have seen". This is no time to be terrified. A positive sweat test is all you need. Not! When I was talking about how much more impressive genetics is over a test older than your doctor, too many medical professionals, including Jerry Nick at National Jewish, yield to the genetics god. Jerry has taken his learning curve seriousely for several reasons. Most of the knowledge about the clean cut genetics of CF have been tossed in the can as 30 CF mutations / deletionsandrepeated sequences has grown large in a fairly short time and many CFTR mutations have not been vetted as harmful or harmless and a growing number of mutations aren't behavingby the classic genetic rules. Third is an aging population of CFers that has grown large enough that adult CF management now is encroaching onconditions of old age. My example is I now have Parkinson's disease. Right now I manage it so well unless you were examining my colon, Parkinson's wouldn't be on the radar. Dr. Nick is fantastic and I believe his prodding has opened up a new field of CF management.
Whether your son gets an official CF sticker, for that matter if you finally get a judgement of CF, which by your sweat test, you have with the cavaet that a new genetic test shows absolutely no CFTR gene mutations. In both cases it still doesn't matter.I am not surprised that many older CFers were diagnosed later in life. They, like you, like me, have picked and either kept or fired doctors depending on whether they can treat the symptoms and complaints of the many areas affected whether it is from the phase of the moon or a bone solid sweat test and genetic analysis.
I have CF but genetically I am a carrier. I also hold a doctorate in genetics and I could do a post doc thesis on this very frustrating issue. Let the genetic pharmaceutical developers focus on the genetics and lower the gaze of doctors from the gene god and look after the person in front of him or her. I hope this helps.
 

uncertainty

New member
Littlelab4CF,
Thank you for your reply, but I am afraid it has left me a little more confused. If all I need is a negative full genetic sequencing test done to ensure I do not have CF, then I will have the lab test immediately! I am trying to find out which one was performed by Duke, but the one done at UNC tested only for 33. Also, there is certainly a lack of consensus about how many mutations have been found as I have been given four different numbers over the past ten days, and all were provided by CFF accredited centers. One of those resources was the foundation itself, and they have stated that I do not need to have a positive genetic screening for diagnosis. So, as you may be able to imagine, it can be confuising for those of us that fall into this questionable realm. I will fight receiving the DX with every ounce of my being, just as I always have, but there seems as much uncertainty now among the experts as there has always been. Granted, there have been tremendous strides in detecting CF and CF carriers, but I am not convinced that genetics knows everything, yet. That said, a mutation finding seems to the significant factor in my case, so send me where they test for the most, and as far as I am concerned, that will be all that is needed provided the test shows no mutation. Sounds simple enough.
My father was not around during my childhood years, but he became a regular part of my life at the age of 16 ( DSS involvement). He has bad sinus disease, and he fought esophageal cancer fives years ago. Hehas been cancer free for two years. History about his biological father is not available, as he never knew him. His mother is healthy, but also suffers sinus disease. My mother's family history is negative for anything. I have four siblings, one deceased ( by suicide). I share boths parents with only one, my oldest has a different father, my youngest a different mother. They are all mostly healthy.
MBP is rare, LittlelabforCF, and though I doubt it is as close to being as rare as what the medical/mental health world believes, there is very little known about survivors of this or the long lasting impact that such experiences may have on physical health. My mother attempted to make all of us (three grew up with her) ill, or pretend to be ill,at some point, but I, having respiratory illness already, was an easier target. I have always felt very strongly that my experiences have produced permanent effects, how could such a thing not? Many lung diseases have been linked to exposures to harmful toxins, and well, it seems almost wreckless not to consider that possibility. I'm not certain there would ever have been any question had I not had the positive sweats, and had I been honest regarding my abuse when I first began seeing MDs in early adulthood. I carried far too much shame during that time, but that is less of an issue now, and I have learned to be honest about it. There is some part of me regretting this decision, but what's done is done now, and I must go from here. My next step is having the full sequencing test performed, and should my son's sweat test be positive, I will insist this be done for him as well.
Again, thank you for the reply. I found it a bit hard to follow on the genetics info, but hearing that no mutations found in the full 2000 search panel means no CF sure sounds terrific to me!
 
M

momofmia

Guest
I can't believe it. My daughter is m470v and 7T/7T.
Chronic sinus infections, sinus surgery, ear infections, PICC lines, oral antibiotics, Pneumonias, but NOOOOOO she doesn't have CF. Well now a diagnosis of Bronchiectasis and after years of me begging for pulmozyne and Hypersal, now they RX it, after the damage is done. oh 3 negative sweat tests also.
Since being on Pulmo and Hypersal, no fevers and no antibiotics for almost ten weeks. I am grateful that I was finally able to get Mia the meds she needed. Her health and her PFT's are absolutely improving;this is what I've been praying for for 14 long years. They Rx'd Hypersal for the Bronchiectasis, pulmozyme is only indicated for CF yet it was RX'd and Mia's health has greatly improved
She is still not diagnosed except for Bronchiectasis, she has been accepted into a trial for a definitive diagnosis, we are waiting for the call. It will either be at NIH or Duke. Nasal potential and many other tests will be done, they think she may have PCD, although she had two biopsies that were negative. I assume they will also perform genetic testing for that lovely disorder. Please tell me where I might find info on the change of thought on m470v 7T/7T. I am growing weary and need to have a diagnosis. Insurance will not pay for Pulmozyne as my daughter is NOT DIAGNOSED. She has absolutely no Pancreatic Insufficiency as all of her bloodwork, stool samples and ultrasounds were normal. She has no GI symptoms except for GI disturbance with antibiotic use. She is 50th percentile for both height and weight.




Don't Ever Give Up
A Mothers Instinct should never be dismissed.
Mia's Mom
 
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