Adjusting to this thing


#1

I am 26 yr old male from NE Pennsylvania. I am having a very difficult time adjusting to this disease. It has gotten worse within the last 3 or 4 years. I know that I have no choice but to do what I have to so I can stop getting sick as often as I do but I have always led a very unstructured and cluttered life. My treatments have gotten very demanding and I dont know how I am going to make them fit. I just have no idea where to start. One of my doctors told me I should think about moving to a different climate and another said not to. I have an appointment @ John’s hopkins in June and am working with a woman To get to the NIH. I guess I will start from there. I kindly received an email from a Lynn @ PCD.org but I was just wondering if anyone knows anything else. How do you find climate changes to affect you. Did you move because of a res. disease and did it benefit you or make it worse? If all it took was moving to make things just a little better I would do it in a heartbeat. Thanks.


#2

Hello,

I am so glad to hear that you will are trying to arrange a trip to NIH (to
see Dr. Olivier, I hope?). We know of several good pediatric doctors at
Johns Hopkins, but I don’t know of anyone in the adult area to recommend.
One frustrating thing with PCD is that even very good doctors are excellent
centers are not familiar with the disorder and do not necessarily keep up
with the research, so going to a site with experience and especially one of
the sites involved in research is (NIH is one) is the best thing you can
do.

I’m very sorry to hear you are having so much trouble. My daughter is 25
and she is also noticing that things get tougher to manage as she gets
older. She lives in Minnesota (I moved to Arizona, but she didn’t want to
move) so we have the same concerns about climate. I think that climate is
probably less important than finding the right doctor in most cases. After
being in Arizona for two years and speaking with patients, I would not
recommend this area for adult people with PCD. The care is very haphazard
and people with PCD seem to have disease that progresses more rapidly as a
result. That said, some people in the group find that a milder climate is
helpful for them, although not everyone has that experience. There are also
regional issues with infections that you may not hear much about until you
move into a certain area. For instance, in the southeast there are problems
with a bug called MAC (you can get it anywhere, but it is particularly
associated with the southeast) and in the southwest there is a fungal
infection called Valley Fever that can make people very sick. I would
definitely ask at NIH (they are MAC experts) what they recommend for climate
in PCD.

With regard to suctioning, yes it can be done at home, believe it or not!
There is a device called the Wiva-Vac that fits on standard vacuum
cleaners. Here is a link to the US distributor:
http://www.zapconnect.com/companies/index.cfm/fuseaction/companies_detail/eregnum/3005096857.html
Do you know where the made the openings in your sinuses? Sounds like you
may have had traditional sinus surgery instead of the endoscopic variety.

On Sun, May 4, 2008 at 11:57 PM, OzzyCoop <
kartagener-cpt8965@lists.careplace.com> wrote:


#3

Yes, I moved to Arizona in 1967 for my severe bronchiectasis when I was
20…I did not know I had
pcd then…
If you would get on the website of
www.pcdfoundation.org
you can e mail me privately or the yahoo group…
I know several who have moved to Arizona… for bronchiectasis…they moved
here as teens, got well,
but I did not…I was dx at 48, worked until I was 55…
many sinus , ear tubes, etc…so you get the picture.
I moved from Seattle…which has allot of rain…
Phx now has smog…but in Tucson, is less and other
places in Arizona…I cannot take cold winters…
----- Original Message -----
From: “OzzyCoop” kartagener-cpt8965@lists.careplace.com
To: elizabethashley@copper.net
Sent: Sunday, May 04, 2008 11:57 PM
Subject: [kartagener] adjusting to this thing.


#4

Hi Ozzy

As a family we made the move to a drier climate after several years of uncontrolled respiratory illnesses.? Early on I noticed that humidity seemed to affect our daughter.? In the new dry climate the PCD was still present, but more controlled.? When the PCD was diagnosed and new medicines and procedures were dispensed and introduced then it made all the difference in the world.? However, it took many years to resolve it too where it was less of an emergency.? There are plusses and minuses.? Too dry a climate may make it harder to clear out the sinus areas.? Too wet a climate may create even more mucous.? This may be the reason for your doctor’s giving different opinions.? So in general I would say humidity seems to affect PCD more than dryness.

In our experience PCD seems to be more under controll?when the person is disciplined to take their medications and do regular exercise (to help the process of moving the mucous out of the system) regularly.? One way you can do this is to make up a type of computer spread sheet of all the procedures and?medicines that you need to do daily.? As you do each one you check it off.? Down the left hand column you put the date and across the top of the page you put the name of each individual medicine and procedure.? This helps to give your life some structure and as you check it off you feel more in control of your life.? You can make up these blank computer spread sheets so they can be run off on your printer.? Also it is good to leave some blank spaces across the top for?when you get sick so you can fill in new medicines.??If you would like I can email a blank example.? You can then reconfigure it so it fits your daily regimen.

PCD is one of those illnesses where structure?in your daily life really helps.? It can help?physically and mentally.???

This website will help you as each person or family has had different ways of dealing with PCD.? Use that knowledge to better your life.? Because each person’s case is different not all the information will help you.? It is trial and error as to what might help you, but at least you’ll have heard how others have managed PCD.

I wish you much luck and hope you continue to feel free to ask questions.

Sandy

P.S.? I have heard that Johns Hopkins has a great CF center which is an excellent place to start. We have found that finding good doctors for treating PCD is very difficult, but when you find one you like treat them like gold.

-----Original Message-----
From: OzzyCoop kartagener-cpt8965@lists.careplace.com
To: abq1ear@aol.com
Sent: Mon, 5 May 2008 2:57 am
Subject: [kartagener] adjusting to this thing.


#5

Hi,

I moved my family to Tampa, FL because of my son’s PCD. He always had a
harder time in the winter and I figured that it would be better in a warmer
climate. So far, it seems to have worked. We have been here almost two
years and we don’t regret it.

Rita Mercier
Chief Financial Officer
Tampa, FL VAMC
813-978-5818

-----Original Message-----
From: OzzyCoop [mailto:kartagener-cpt8965@lists.careplace.com]
Sent: Monday, May 05, 2008 2:58 AM
To: Mercier, Rita L.
Subject: [kartagener] adjusting to this thing.


#6

Is the endoscopic surgery better? I’m actually waiting to see the ENT now to get a second opinion on sinus surgery.

I had polyps removed years ago but they are wanting to do something more intense - which may be the one you’re referring to.

Sent from my BlackBerry® wireless device

-----Original Message-----
From: mmanion kartagener-cpt8965@lists.careplace.com

Date: Mon, 05 May 2008 09:07:46
To:dmhowell75@yahoo.com
Subject: Re: [kartagener] adjusting to this thing.


#7

NO, the endoscopic surgery is NOT better for those with PCD! The endoscopic version cuts the windows high up on the maxillary (under the cheek bones) sinuses; for those with PCD or CF, the mucus is too thick, and can’t travel uphill against gravity, to get to the windows when they are high up.

Unfortunately, we PCDers still need the old version, where they go in up under the upper lip and then go sideways. This way, the windows are MUCH lower down, gravity can help with the drainage, and there are some RESULTS from the surgery. Do NOT let any ENT or other doctor tell you otherwise!!! You will regret it for years to come, if you do!

That said, the old version is variable. Some people get wonderful results (I did) and others don’t get much from it. It’s a shame they can’t be sure you’ll get good results from it, as the old version is quite an ordeal to go through. The surgery itself isn’t all that bad, but having to keep all that packing in your nose and sinuses for several days is NOT fun!

Be sure you have (and USE) a good room humidifier during your recovery period, or you will wind up with a horribly sore throat; I know we feel we can’t breathe through out noses, but you’d be amazed at how well they humidify the air we breathe through our mouths! I didn’t know, and didn’t use a humidifier, and wound up not being able to eat or drink anything but ice water for 11 days, post-op! Also not fun! Also be sure to drink plenty of fluids (and I do mean PLENTY!)

Anyone interested in that Wiva-vac thing, be aware that the chamber that holds the mucus is VERY small—this was designed for children’s use. I have a medical suction machine, but I currently use a wonderful home-made arrangement that plugs into the hose on my vacuum cleaner. It uses an old mayonnnaise jar as the holding chamber. It works MUCH better than the medical machine, and is easier to clean.

It has made my life SO much more comfortable, I can’t say enough about the benefits of suctioning. Also of sinus flushes (I use hypertonic saline, which gets TONS more stuff out, and does it a lot more easily, too.) Sinus flushes alone are a tremendous help, if you can’t afford a machine or can’t get your doc to prescribe one for you. If you DO suction, though, always be sure to keep your mouth open a little bit, so that suction cannot build up and possibly cause damage. We have pretty weak eardrums, usually. If your mouth is open, the suction can’t build up enough to hurt you.

Laurel (52, PCD/SI, diabetes, SC)

— On Mon, 5/5/08, dmhowell75 kartagener-cpt8965@lists.careplace.com wrote:

From: dmhowell75 kartagener-cpt8965@lists.careplace.com
Subject: Re: [kartagener] adjusting to this thing.
To: shannielars@yahoo.com
Date: Monday, May 5, 2008, 11:51 AM
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Is the endoscopic surgery better? I’m actually waiting
to see the ENT now to get a second opinion on sinus surgery.

I had polyps removed years ago but they are wanting to do
something more intense - which may be the one you’re
referring to.

Sent from my BlackBerry® wireless device

-----Original Message-----
From: mmanion

Date: Mon, 05 May 2008 09:07:46
To:dmhowell75@yahoo.com
Subject: Re:
[kartagener] adjusting to this thing.

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#8

Thanks for you responses everyone. Some of you might know of the history of the Scranton/Wilkes-Barre area of Pennsylvania. Back in the days of mass coal production this area was right at the top. Until recently you couldn’t drive 1 mile without seeing an old breaker or the old group houses that the miners lived in. Old mine shafts are all over as well. Someone once told me that they were conducting research into residual gas build ups and stuff like that that might seep out of the ground. This area is right in the middle of the mountains. It is a big valley and I think the crappy air just blows around. I know it may sound funny but I was in Orlando this past spring for a couple of weeks and I felt incredible. I spent time in North Carolina with my brother a few years back and I also felt great (not perfect but much better). I wasnt sure about moving because I would imagine Florida gets very hot and humid in the summer. We were there in February and it was already 85. The heat wont bother me but the humidity does. Also walking in and out of air conditioners gets me going too. I wont use an air conditioner. I was trying to find somewhere that might stay mild all year around but I dont know. I have a hard time with the winter as well. Here it has been hot when it should be cold and cold when it should be hot. I would handle the cold much better if it would stay cold but it is the chnages. I seem to always get sick in between seasons. Oh well. Moving is for a few years down the road anyhow. First things first. I cant wait till I go to NHI and John’s Hopkins. I am going to have so many questions.


#9

laural, I did n’t know you had a trach machine at home…I like
the trach machine because of the different size caths I can buy
from the medical supply store…
I have windows too…but windows do close up…I am afraid
the endoscope surgery is what they do now…it goes faster…
and now they have packing that dissolves…
----- Original Message -----
From: “lars” kartagener-cpt8965@lists.careplace.com
To: elizabethashley@copper.net
Sent: Monday, May 05, 2008 9:10 AM
Subject: Re: [kartagener] adjusting to this thing.


#10

With all due respect, we get call and e-mails from many more patients than you are in touch with, so I’d say we have a better grasp on the situation. After you’ve walked a mile in the shoes of some of the folks we’ve corresponded with, maybe your attitude about the situation would be different. You have a right to your opinion. Michele’s wasn’t actually an opinion but rather a fact! Folks don’t tell you what to day. maybe you should exercise the same respect.

betts kartagener-cpt8965@lists.careplace.com wrote: Michele…don’t say that about doctor’s…maybe there
is on who has had bad luck, but it is up to the patient
to find good doctor’s…patient s can always s fire their
doctor’s


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#11

Yeah, I’ve had it for years. Hardly ever use it any more, since I got my Wonderful Wally-Vac, as that works much better, and I don’t have to mess with sticking a catheter up my nose. MUCH less pain!

I had my windows put in 35 years ago, and one of them is still open, after all these years. They WILL do the new windows unless you insist that they do they old surgery on you. Everyone else but us and CFers do fine with the new, higher windows, but WE need the lower ones. And don’t let any doc, no matter HOW many fancy degrees they have on their wall, tell you otherwise! We HAVE to have to lower windows, because our mucus is too thick to travel uphill against gravity, to get to the higher windows. And we don’t have working cilia to help it get there, either. We NEED the lower location so that gravity can work FOR us, not AGAINST us.

Laurel (52, PCD/SI, diabetes, SC)

— On Mon, 5/5/08, betts kartagener-cpt8965@lists.careplace.com wrote:

From: betts kartagener-cpt8965@lists.careplace.com
Subject: Re: [kartagener] adjusting to this thing.
To: shannielars@yahoo.com
Date: Monday, May 5, 2008, 12:42 PM
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laural, I did n’t know you had a trach machine at
home…I like
the trach machine because of the different size caths I can
buy
from the medical supply store…
I have windows too…but windows do close up…I am afraid
the endoscope surgery is what they do now…it goes
faster…
and now they have packing that dissolves…
----- Original Message -----
From: "lars"
To:
Sent: Monday, May 05, 2008 9:10 AM
Subject: Re:
[kartagener] adjusting to this thing.

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#12

Hey, my disease is doing great, I did not go on oxygen
until I was 60… I worked 32 years…I went skiing
and played tennis until my 40’s…I loved the move here.
I once went to Kans…for my schooling in nursing, but never made it through
the winter…zero degree put me in the hospital with double pneumonia…i was
18, i really thought I was a goner…I moved back to Seattle , worked
three years , the doctor said, I would live to be 30 in Seattle, and 50 in
Arizona…so here I am…63…
Michele…don’t say that about doctor’s…maybe there
is on who has had bad luck, but it is up to the patient
to find good doctor’s…patient s can always s fire their
doctor’s…If I were working again though I would work
for the government. post office,etc…they have great benefits …and you
don’t have to work all of your life…
My ENT in phx, has saved me from getting more
surgeries, I have had tubes, and 12 sinus surgeries,
he now suctions my ears and sinuses…we have
a ear specialist at St Joe’s…
Anyway…we do have a CF clinic, that takes adults
too , I think the pcd thing, I checked they only had
one patient in their 20s with pcd…
Michele is right about smog…but I know of people
who moved to places without smog…and do great…
Bullhead City…Arizona…even got off of their oxygen…
The main thing, as you can see I have been active…
And I would not recommend the move if you feel
the weather would not help…the one thing about having
the sun, there is less molds…and three days of 100 degrees gets rid of
allergies…but that will not be until
later May or June…Monsoon, is when I do worse,
it is high dewpoint that does me in…
Michele sorry about your daughter, but she probably
would not do well, if she did not want to move out where
you are…the choice has to be hers…
Attitude has so much to do with our illness also…
I just know of someone who moved to Prescott, az and
to her the sun is healing…but to me that is high altitude…
Even Tucson is a 1000 feet higher then Phoenix…
I think if you just stay where you are, and do what
your doctors say…for me it was my doctor telling me
to move to ARizona…, I do miss the water in Seattle…
Seattle is smog free, just about though…but too humid
and wet for me…
----- Original Message -----
From: “mmanion” kartagener-cpt8965@lists.careplace.com
To: elizabethashley@copper.net
Sent: Monday, May 05, 2008 6:07 AM
Subject: Re: [kartagener] adjusting to this thing.


#13

Just want to comment on the endoscopic surgery. I had it done in 2004 and they now put in what they call “lower windows” which are level or almost level with the base of the maxillary sinus. These are very helpful for PCD.

The physician doing the surgery needs to be aware that you need the “lower windows” and not the traditional “upper windows” which are higher up. I have both since my “upper windows” from 1972 never closed up - the upper windows really don’t do us much good.

Hope this helps.

Laura


#14

also with sinus surgery…I am fortunate enough to find an ENT that believes
in suctioning…he was against the Pic
when i was diagnosed with destructive cilia…in 1990…
he thought the pic line spread more infection…I can’t do
washes either, that solution gets into my ears. then I am
worse and more plugged up…
Everyone is different…not all cilia is the same…even if
cilia is working the same people have the same problems
as we do…asthma. repeated pneumonia, otitis media…
etc…I have had 12 sinus surgeries…and suctioning was
the best…I have had both upper and lower windows that
have closed, in fast I have no maxillary sinuses now…
they do not show up on the CAT scans…Endoscope,
I have had diseased bone removed…frontal sinusitis…also.
surgeries started when I was 12…even moving out to
Arizona, I still continued to have sinus surgery every three years until I
found this ENT that worked for me…
i am also low igg igm iga…low iga alone can cause
sinupulmonary disease…I do get IVIG…to help with
infections…t and b cells are borderline…I do have
pseudomonas, which IVIG cannot help…so I still
need to be on antibiotics…
----- Original Message -----
From: “lynniegirl88” kartagener-cpt8965@lists.careplace.com
To: elizabethashley@copper.net
Sent: Monday, May 05, 2008 12:12 PM
Subject: Re: [kartagener] adjusting to this thing.


#15

Hi Laural, Just for clarication, endoscopy just refers to how the surgery is done--not where the windows are located. The frustrating thing is that many ENTs are not aware that the location of windows in PCD needs to be done the "old-fashioned way" with the windows low in sinus so you have to be sure to check out what your doc plans to do prior to the surgery.

Betty, You sound like you take it as a personal affront that I am not impressed with the medical care provided by adult doctors in Phoenix. For the most part, I am not and I stand by that assessment. Remember, I chose to move out here. I like Phoenix and am not trying to “knock” it. I just also recognize that it is ranks near the bottom on measures of health and education and that low ranking is clearly evident to me in the inadequate treatment many PCD patients (mostly adults) receive out here. Unlike most of the rest of the country, a large number of adults in their 30s and early 40s out here have end-stage lung disease and are facing transplant—due in large part to negligent care. That is not an opinion—it is the fact.

 The topic of this thread had to do with whether or not to move. My post was meant to point out that climate is just one of many reasons to consider a move and climate alone can't make up for bad care. If the weather is a huge contributor to your disease than it may be worth the tradeoff to risk less aggressive care for a warmer winter, but I would want more than just personal opinions form posters to a forum to rely on to make such a big decision. This is a REAL and serious issue--especially for adults. When you moved in 1967 no one knew about PCD, so you had little to lose by choosing AZ. That is not the case anymore. If it is possible to get better care elsewhere and better care is more important to you than good climate, there are other options. If the weather bothers you and you believe it is a major factor contributing to your disease, by all means make a move--just be sure you know what you are getting into.

I would also point out to you, Betty, that you have received the bulk of your adult care in Phoenix, so really have no other gauge by which to measure quality of care. It may be (and I’m not saying that it is, because I do believe you have good doctors) that your expectations of care have been lowered to meet the prevailing standard. This just means that what patients in some areas have come to expect represents an inferior standard. In a sense they are manipulated to accept what should not be acceptable.

Finally and for the record, I am discouraging Meghan from moving out here because I think it would ultimately be worse for her health. Her "attitude" has nothing to do with it. She clearly gets better care in Mpls than most adult patients do here and I can not in good conscience recommend she move away from good care to face a fight for less than adequate care. Forums encourage people who would otherwise be more thoughtful to be very dismissive and insensitive to the concerns of others. You are entitled to your opinion, but when you mischaracterize mine or throw in derogatory comments about someone who is not even posting to this forum and so has no way to defend her/himself, I feel compelled to call you on it.  Bottom line, I am worried about Meghan's lungs if she moves out here--not her attitude.


#16

The climates do have an effect on the severity of the symptoms, but as PCD patients we’re ALWAYS being infected with bacteria. The ciliary functions are too slow or not working to clear up the mucous that accumulates in your lungs. That’s why the body releases a coughing mechanism. Mucous is the easiest target for bacterias and viruses. I have experienced the warmer climate to be more conducive than the harsh cold winters we experience in NY. However, if it gets too warm, it feels like the mucous starts to melt and make its way up through your esophagus as the coughing mechanism.

Hm…I just wish we could jolt these cilia brushes to start cleaning up and doing their jobs. I’ve had pneumonia more than I can remember.

I feel a bad cold coming on right now. Woe to autumn/winter.