Hi, I posted here before. about my daughter, Livvi, who was just diagnosed
with PCD after a ciliary biopsy 3 wks. ago. She’s in her 6th week of
Amoxicillan & she’s sick again, (very bad nose- sinuses-, bad deep
cough & fever). We go back to the Immunologist at Ann Arbor’s U of M
children’s hosp. today at 3:30. I’m assuming they’ll refer us to a
Pulmonologist since she doesn’t have one yet. Do you think that would
be her main Dr. then? I took her to her regular ped. yesterday, because
I was worried about her breathing & her sinuses. He admitted that he
did not know much about PCD & has never seen anyone with it before. He
was asking me lots of questions about it since I’ve done so much
reading. He really wants to work in cooperation with whoever her main
Dr. at U of M is going to be, and he seemed to be not quite sure what
antibiotic he should give her. He ended up changing her to Omnicef,
(which she’s been on many many times before), but it is only a 10 day
course. Did you all find that you had to educate your pediatrician
about this condition after diagnosis, or did you only see the
specialists and skip the ped. He’s just so close to home, and he does
want to help her. He just seems to not be sure of how he should
approach her treatment. I’ll up-date more after her appt. this
afternoon. Thanks, Missy (mom to 4 girls, 14, 13, 6 & Livvi 2-PCD)
do they have heating pads for children, i know it
helped me.
----- Original Message -----
From: “momto4girls” kartagener-cpt7723@lists.careplace.com
To: elizabethashley@copper.net
Sent: Monday, February 11, 2008 3:30 PM
Subject: Re: [kartagener] Livvi’s sick again-follow up appt. (after diag.)
today
Hi Missy,
It probably feels a little frustrating that you have to be the “educator,” but you have actually made tremendous progress in just finding an ENT who wants to learn and who wants to work with your pulm. You probably already do know more about PCD from the research you’ve done than a lot a doctors and that will only get more noticeable all the time. The important thing is to find people interested enough in the disorder and in your daughter that they will acknowledge your PCD “expertise” and will include you as a full partner in managing her care.
In an ideal world, your daughter’s pulmonologist would be her primary doc. However, a lot of how her treatment is managed will depend on your insurance and how the local medical community is organized. My daughter, who is 24, sees an adult CF pulmonologist at the U of MN. Because he pretty much wants to be made aware of anything that happens to her, he is her first call no matter what the problem is–even if it’s for a broken leg. She does not have a family practice doc or internist for coordination of care at this point because her pulmonology office wants to do it. They refer for gyn appointments, GI stuff, etc. It’s not because they want to be controlling, but because so many seemingly unrelated things may have an impact on care.
We had our appt. with the immunologists yesterday, and they referred
us to a CF pulmonologist at U of M and we also have an appt. with a
genetecist now. She is to finsih her 10 day course of Omnicef for her
sinus infection right now and then continue on with a month of
Bactrim and then one month of Erythromycin. After that, they’re going
to try her with no antib. and see how she does. I suspect she’ll be
sick within a week, because that is her pattern. We are doing the
daily sinus rinses with her, and we also just started doing nebulizer
treatments with albuterol while she’s sick. We’re going to go ahead
and keep our appts. at U of M, but I am also going to google Mayo &
Cleveland Clinics and see if I can find out how many PCD patients
the’ve treated. I think we will try to visit one of these places
either during Spring Break or in June when school is out, (we’re both
teachers). I love the idea of having a detailed letter concerning her
treatment that we could take with us to the ER or any Dr. if we
needed to. It’s just too difficult to try to explain her entire
background and history to every new person we come across. So many do
not understand. When I recently had my 6 yr. old at a Prompt Care
Clinic for simple URI, I explained that we needed to be careful of
spreading infection in our home due to Livvi’s condition. The Dr.
knew nothing about it and even asked, “Well how did she catch
that?”. I had already said it’s a GENETIC condition. What?! I am
very impressed with her Pediatrician though. He admits that he does
not know enough about it yet, but wants to know about it and work
with other Drs. so he can help her. I wasn’t sure if I would be
offending him if I gave him copies of my whole file I’ve accumulated
now on PCD from my research, but I should pass it on to him. I’m sure
he’s too busy to research it himself even though he wants to. I don’t
think he’ll mind. He’s always been a very caring Dr. through all of
her problems. I think he’s been frustrated at not being able to get
to the bottom of it, which is why he sent us on to U of M. We got the
pathology report from her ciliary biopsy at yesterday’s appt., and it
showed that almost all of her cilia were extremely abnormal, with a
variety of abnormalities and NO movement at all. Do you have any
knowledge or experience with what they do at Chapel Hill, (North
Carolina)? I know that they’re doing a lot of research there and they
need patients, (especially siblings, which we may have, 14 yr. old
will be tested Feb 25th), but I don’t know if that would be the place
to go for treatment or not. Thanks again for all of your responses.
That’s what I really need right now - to hear about other’s
experiences who have gone through this, and your suggestions have
been great! Thanks, Missy
Hi Missy,
It sounds like you are making progress with the doctors at least. In terms of spending time and money to make a visit to a site with PCD experience, be wary of relying on “reputation.” Cleveland Clinic and Mayo have stellar reps, but aren’t focused on PCD. We took my daughter to Mayo when she was young and they told us that her pneumonias were the result of “improper nose blowing.” Really. The one doctor there who is interested in PCD has some unique ideas about it that run contrary to most of the published research. He also is, in my opinion, pretty dismissive about the difficulties of managing care day-to-day. Granted I have not been involved with the site for years, but it would definitely not be my first choice. I don’t know much about Cleveland Clinic except that they were invited to participate in the PCD multi-center study and declined, graciously, because they felt they didn’t have enough patients or experience and it wasn’t an area of focus for them. Who knows, maybe as the research effort grows, they will eventually come on board. I have worked with some great doctors from Cleveland Clinic on other respiratory stuff (interstitial lung disease and PAP are biggies for them) and would be happy to get recommendations from them if you are interested. That said, if you are willing to make a trip, rather than going to a name center and hoping they will be able to help you, I would strongly recommend going to UNC or one of the other five PCD research sites. You won’t find doctors anywhere in the country who actually know more about PCD, do research and publish on PCD and have a genuine interest in the disorder. Combined, UNC and the other sites have seen hundreds of PCD patients and they communicate twice a month about PCD research outcomes and clinical problems they’ve encountered and how to manage them. In addition, they work with the international PCD research, which means the combined patient experience of this group of researchers is close to 1,000 patients. In contrast, even large centers around the country see only a handful of patients with PCD. UNC is the main site, has been doing PCD research since 1993 and has seen the most patients. The other sites are Wash U (St. Louis); Denver Kids; U of Washington (Seattle); NIH (Bethesda, MD); Hospital for Sick Kids (Toronto). These sites can see you through the research protocol and the initial visit and workup would be covered through research. There may be assistance for travel through Angel Flights for research participants. They can also see you for a regular clinical appointment. Here is the link for the research study which includes contact info for all five sites:
http://rarediseasesnetwork.epi.usf.edu/gdmcc/takeaction/findatrial.htm
Prior to working with the PCD Foundation, I worked in clinical marketing for the Vest system. Four of the six sites currently involved in PCD research were targets for the company because of their stellar reputations for CF research, even though the general population has probably never heard of them. When you see lists of top hospitals, remember that they are being judged and graded on how they deal with specific problems–generally the biggies like cancer and heart disease–and the pockets of truly astonishing work that gets done in rare disorders like CF and PCD may not even be represented in these numbers. A good example is Mayo Clinic in Rochester. They are an excellent facility for many things, but pediatrics was never a focus for the Mayo system, although they appear to be trying to improve that aspect of their care, and they have not devoted resources to genetic respiratory disease. In Minnesota, The U of MN is actually much better for these specific issues. I see that newer top hospital rankings state areas of expertise, which will help, but just be aware that reputation alone is no guarantee of knowledge when it comes to rare disorders. Many have been sorely disappointed when they thought they were getting the best of the best of care only to find out that the name center had no more experience than their family doctor.
Other ways to judge a center/doctors experience in PCD are to check PubMed, the National Library of Medicine catalog of peer-reviewed publications, and see who is doing research and/or publishing on PCD. This information is provided by Congress and is freely available to everyone using a simple search engine. There are currently 1,467 articles published about PCD since 1950. If you browse recent articles you will notice that certain names come up over and over again. This will give you an idea of the level of interest and expertise of the doctor/facility. You can also go on to the facility website and do a search on primary ciliary dyskinesia in the “conditions we treat” category. If a site doesn’t reference PCD, it doesn’t mean they can’t treat it, but it does mean they don’t see a of it and they don’t consider it an area of comfort for their clinic.
There is no perfect choice for consultation with a disorder as rare as PCD, but at least these days we have UNC and the research sites as a first step. I would definitely recommend starting there.
Thank goodness I live in Arizona, we had some
cold weather here and rain, and I am now back
on IV’s I also have a weak immune system…and
get IVIG.
----- Original Message -----
From: “momto4girls” kartagener-cpt7723@lists.careplace.com
To: elizabethashley@copper.net
Sent: Monday, February 11, 2008 3:32 PM
Subject: Re: [kartagener] Livvi’s sick again-follow up appt. (after diag.)
today
laural, i hit reply, thus not having to resign up…
the thing is, I thought by hitting reply these
were private messages…they are not…?
----- Original Message -----
From: “lars” kartagener-cpt7723@lists.careplace.com
To: elizabethashley@copper.net
Sent: Tuesday, February 12, 2008 8:40 PM
Subject: Re: [kartagener] Livvi’s sick again-follow up appt. (after diag.)
today
Does anyone have any tricks for soothing a little one’s bad, bad ear ache besides Tylenol? Livvi has been on her Omnicef for 4 days now, still has a bad nose & cough & this afternoon she’s feeling awful & crying a lot with a bad ear ache. I’m thinking she probably needed a different antib. but not sure what to do about it today. No one at the ER here would understand about PCD. I guess I’ll just wait & call her Drs. tomorrow. Her fever is only around 100, so not bad now.
Thanks, Missy
Hi, Missy,
I can totally sympathize with Livvi, having gone through many horrible ear infections in my life. I hate to say go ahead and take her to the ER, but couldn’t you call the emergency service at her pediatrician, and get them to phone in a different antibiotic for her, to your pharmacy? That’s what I do, if I’m not sick enough to get admitted to the hospital, but need a quick change like this.
One thing I’ve learned about PCD—you DON’T sit and wait and hope it’ll get better on its own—it almost never does. MOST antibiotics should be showing you some sort of improvement within 72 hours; if she isn’t better after that, then it’s very likely that that antibiotic will not kill whatever bug she has growing in there, so the sooner she switches to another one (hopefully the right one!), the sooner she will feel better, and the less permanent ear damage will be done.
As to the ER people, they don’t really HAVE to understand about PCD much, to understand that this antibiotic isn’t doing what it’s supposed to, and that your child is in a lot of pain. It’s helpful if they understand that PCD is one of those conditions that causes “glue ear”, and that, because of that, it’s highly unlikely that Livvi’s Eustachian tubes are open enough to let the infection drain that way. It might just ‘light a fire’ under their butts to change the antibiotic for her faster. But it’s really important for most of us with PCD to NOT WAIT to jump on bugs that get a ‘toe-hold’ on us, because they tend to progress SO fast, rather than get better, like with normal people.
To give you an example, this has happened several times to me. I have been fine all day, raking leaves and gardening, or hiking in the mountains and horseback riding, swimming—just active and perfectly okay. On these occasions, usually around 4 or 5 in the afternoon, I’ll feel like lying down for a nap, so I do. In an hour or two, I will wake up with chills and fever, and wind up getting admitted to the hospital that night.
One time, I was at work when this happened. At 7 am, I was fine. At 8:30, I felt a little chill, so I took my temp, and it was normal. By noon, my temp had risen 5 degrees. Needless to say, I was pretty sick. Many of us PCDers do not GET any warning that we are sick. It just comes ‘out of nowhere’, as far as we can tell. Livvi may be a PCDer like that—this ear infection sounds like she might be, and it sound pretty bad.
Now, in the meantime, you could try letting her hold a heating pad to her ear, or a warm compress (be VERY careful it isn’t too hot—those tales you hear of people being burned by these things are NOT old wives’ tales—I have been burned pretty badly, myself.) If that doesn’t help, you COULD try cold compresses, but I have never tried that, personally. She will be able to tell which–if either of them—is helpful pretty quickly.
You might also try doing extra sinus rinses, as the main reason that pressure builds up inside the ear is that the Eustachian tube which leads from the ear to the throat gets clogged with mucus and then nothing can drain from the middle ear. So the pressure builds, because the bacteria keep breeding in there, growing all the time. If the pressure is not relieved in some way (Eustachian tube drainage, eardrum lancing, myringotomy tubes), it will eventually progress to the point where the infection itself will rupture the eardrum—certainly the most painful way to let the infection out, and the way that also causes the most suffering and the most damage.
This last way was the way they always let ME go as a child, and it was absolute HELL. Some infections were so painful, my mom would be holding me, trying to comfort me, and I wouldn’t even know she was there, I was so wrapped up in the pain. And I can recall one or two when I was older that were so bad, I couldn’t sleep for four or five days straight, waiting to get in to see the ENT. My parents didn’t believe in the ER for ear infections, I guess. I never asked them. But it was a really traumatic experience, going through this every time.
So, in my opinion, I think it would be the good thing all the way around, to try to do SOMETHING for her today, and not wait till tomorrow. You’re not just working to save her from pain NOW, but also from the cumulative damage that each one of these infections does to her ears. You won’t get the chance to go back and do this over again, and there’s no way to tell exactly how much damage she’s getting from each infection.
Sorry I don’t have more to offer you!
Laurel (52, PCD/SI, SC)
momto4girls kartagener-cpt7723@lists.careplace.com wrote:
Does anyone have any tricks for soothing a little one’s bad, bad ear ache besides Tylenol? Livvi has been on her Omnicef for 4 days now, still has a bad nose & cough & this afternoon she’s feeling awful & crying a lot with a bad ear ache. I’m thinking she probably needed a different antib. but not sure what to do about it today. No one at the ER here would understand about PCD. I guess I’ll just
wait & call her Drs. tomorrow. Her fever is only around 100, so not bad now.
Thanks, Missy
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Laurel, Thank you so much once again for the great information and suggestions for Livvi. She finally fell asleep right now. Maybe the Tylenol helped, but I know I need to do something. I’m wondering if she’ll wake up with a higher temp. She too has typically been hit extremely fast and out of nowhere by bad infections. On more than one occasion, she has been running around, playing and laughing in the afternoon and then admitted into the hospital by that same evening with sky high fever, dehydrated & awful infection. Thank you again, and I’ll let you know how she’s doing.
Missy
My parents used a heating pad…when I was young…the thing
is with PCD, it is a life long endurance…i still go to the ENT
every month at 63…When I was in high school, my friend wrote
in my HS book, I would be more fun, but I am always at the
doctor’s, and then I had another friend tell me she was afraid
of me because she lost her grandmother and was afraid I was
going to die…
There should be a book entitiled
Kids with Chronic Illness go through the darndest things" blm
maybe we should start a forum just for that…
I’d really like it if you would, Missy. I’m one of the ones who couldn’t have kids (at least, not without medical help, which I could never afford, back then), so one of the things I make an extra effort on is answering parents when their kids are having a rough stretch, or if I see a place where saying something to a parent might save their child(ren) from having to go through some of what my sister and I had to go through, all those years.
Is Livvi on nebs or at least an inhaler (with spacer) every day? If not, I am personally a VERY firm believer that ALL PCD kids should be. No parent wants to make their kid take medicine they don’t need, and neither do I. But my KS sis and I are prime examples of “locking the barn after the horse has been stolen”. They didn’t know we had KS/PCD back then, and all the docs we saw felt that our crummy lungs and ears were due to the rotten sinuses. They COMPLETELY focused on treating the sinuses to the total exclusion of our lungs, and the only real treatment we got for our ears was antibiotics when we had an ear infection, and a surgical rebuild of my left eardrum for me, when I was 8. I’m sure if myringotomy tubes had existed back then, my parents would have gotten me those, if they could have—I don’t think they did, although it would have been nice if at least one of my docs would have been willing to lance my eardrums when I got bad infections—it might not have
saved my hearing much, but it would have saved ME an awful lot of agony during those years when I was getting such constant ear infections.
By the way, chances are good that Livvi’s ears won’t be QUITE so susceptible by the time she turns 6 or 7. The Eustachian tubes are much more horizontally oriented when we are young, which means they don’t drain the middle ears of mucus and infections as well, and also that bacteria can migrate up into the middle ear a lot easier, as they’re not going uphill, so to speak. As Livvi grows, the infections should slow down gradually. By the time they did that first surgery on me when I was 8, I was only getting a couple of infections a year, usually—HUGE improvement over nearly constant infections when I was little.
I don’t always just offer my personal experience and opinions, either. Until I was sidelined by my lungs and had to go on disability in 2001, I was a respiratory therapist for 23 years, so I also have a little clinical knowledge to offer. Just don’t EVER think that I know everything, as I am just as ‘in the dark’ as anyone else who isn’t in the field, when it comes to new developments/knowledge. I try to learn about anything new I hear about, but that doesn’t mean I even hear about it very quickly. I learn something new every day.
But about neb treatments; bronchiectasis is NOT a reversible condition, and once those airways are damaged, it only gets worse. So dealing with PCD/KS is really a PREVENTIVE situation. You are going to have to do the best job you can, every single day, to prevent any damage you can for as long as you can, to Livvi’s lungs, sinuses, and ears.
In many conditions, people can turn to surgery of various sorts, to repair damage of this type. I won’t say that surgery can’t be HELPFUL to us, but in our case, the surgeries never seem to really FIX any of our problems. So we may get improvement for a while, but it rarely helps permanently (or even for very long, sometimes). Therefore, the only real option left to us is to prevent the damage in the first place, for as long as we can. And that means starting EARLY, BEFORE the damage can be seen, if at all possible. It isn’t like there’s a chance that there might not BE any damage, if you don’t. There is GOING to be. It’s just a question of WHEN it will happen to her.
So you need to be as demanding as possible, for Livvi’s sake, to get her docs to look at her as her own person, and to understand that her needs are very, very different from most children’s. She NEEDS to be on daily nebs (probably at least 3 times a day, too, not just once) of some type, or at the VERY least, an inhaler with a spacer daily, to help stave off lung damage as long as you can.
If you are willing to fight (if necessary) to get her these things, she may NEVER have to sit on the sidelines of life, just gasping to get oxygen from a tank like me and many of my generation. It really KILLS me, to see that these things are at least AVAILABLE to this generation of PCDers, and some of them aren’t getting them====mostly because of the ignorance of docs on PCD, too. I mean, every parent I’ve heard in here is fairly desperate to get some help for their child(ren). None of them have seemed to have a ‘whatever’ sort of attitude. They mostly say that ‘their doc doesn’t want to give young kids drugs like that’, or 'he’ll only let us rent a machine for a couple of weeks when he’s really bad." I would like to give docs like that a REALLY good lecture about PCD!!!
One of my major pet peeves, I’m afraid, sorry. But I can’t stress this enough----keep coming to this group whenever you can; we haven’t been really active in here, lately, but you can almost ALWAYS find some valuable info in the posts, and you’re ALWAYS welcome to ask any question; someone will be sure to answer, even if it’s only me! And Livvi’s whole life is going to depend in part on what YOU can do for her today, not just her childhood and immediate future, because so much of the quality (and even quantity, maybe—some of us who went without treatment in childhood have died in their 30’s, 40’s, and 50’s, which is pretty young, by American standards) of our later lives depends on what kind of treatment we got in childhood.
So, remember when you’re fighting a doc to get something Livvi NEEDS, that you’re not just getting her through THIS infection, you are also fighting to give her the best future life possible. Even with the best of treatment, Livvi’s going to get SOME damage; the whole idea is to prevent as much of it as you can, for as long as you can.
You can do a heck of a lot, even before you find a doc who will start treating her lungs. ANYTHING that gets her breathing deeply or moving around—ultimately, anything that gets her coughing that junk out of her lungs----will work wonders at helping prevent the lung damage. If she likes blowing bubbles, do that every day. Have her blow out candles one by one, let her jump on trampolines, encourage her to sing loud, happy songs, have tickle-fights with her. Let the kids have pillow-fights----OH, I remember coughing my GUTS up, when we did that as kids! As she gets older, encourage dance (especially really active stuff like rock or square dance or folk dancing), or help her find a sport or activity she really LOVES. What you’re looking for here is something that she will ENJOY doing, so she’ll be pretty willing to do it every day. Some of us play harmonicas for the lungs—excellent exercise, and really gets us coughing. We even have one little 7-year-old whose
mom posted a pic of her on the CarePages, of her playing her harmonica by holding it with her FOOT! So informal respiratory therapy doesn’t HAVE to be boring or painful.
Have you been taught how to do CPT on Livvi yet? That would also help a lot. I can describe the technique pretty well to you, but I’d send you to the CF website to look at drawings of the different positions.
You’re entering a whole new world, here, now that you know what’s wrong with Livvi, and I can’t help all that much, but I am glad to offer what I can to you. If I can save your child or any other from some of the pain and lack of appropriate treatment that my sister and I went through, it’s worth taking some of the time I have left here to do that.
Laurel
momto4girls kartagener-cpt7723@lists.careplace.com wrote:
Hi,
Livvi’s ear pain doesn’t seem to be as bad today, but she is still complaining of it some. She’s on Tylenol too, so maybe that’s helping her. She still has her bad nose & cough too. Luckily, we had a snow day today, so we’re all able to stay home and take her out in these 10 below wind chills! We did extra sinus rinses, Tylenol & put in a call to her Ped. office this morning --no response yet! (other than "The nurses have been really busy all day & will call you back at the end of the day!) I also called her Immunologist’s office at Ann Arbor & they said to call her Ped. since he’s the one that prescribed it to her. I don’t feel that we’re being taken seriously. She’s not deathly ill right now, but she’s not feeling well, and as you said Laurel, if things aren’t handled in a timely manner, she can get there fast. I managed to get our Genetecist appt. made this morning - Apr. 10th & her pulmonologist appt. - April 16th, the soonest possible.
Also, I just received 2 replies from pulmonologists at Mayo. I emailed them to ask if anyone there has any expertise in PCD. They both replied that yes, they do have experience with it at Mayo, and one of them, Dr. Mark Wylam said that he’s an advisor to the PCD Foundation & trears a few patients with it. They both said they would be happy to see her. We’re thinking about driving to Mayo over spring break, (beginning of Apr.), just to see what they have to offer. We would probably still try to go to N. Caro. in the summer too.
Laurel, you asked if anyone had taught us the chest therapy yet, and no they haven’t. Could you give me the web site that shows how to do it, since we can’t get in until April? Also, you asked about nebulizer teatments. We have a nebulizer from when our oldest was little, and her ped. just gave us prescript. for Albuterol last Thurs, so we’ve done that a few times when her cough has been really bad, but it makes her hyper. What do you know about Xopenex? Someone else mentioned it, that it doesn’t have as many side effects.
Thanks for listening once again! Missy, mom to 4 girls (14-testing soon, 13, 6 & Livvi 2-PCD)
I meant NOT taking her out in the 10 below wind chills! Missy
Hi, Missy,
I’m really glad Livvi’s feeling a little better today, but ready to SCREAM because you’re absolutely right----these docs are NOT taking you seriously when you try to tell them about Livvi’s illness. Not surprising, of course—American docs are notoriously arrogant, believing that their patients couldn’t POSSIBLY know more about their own condition that the DOCTOR could, but it’s extremely frustrating and infuriating, ESPECIALLY when it’s a child, who has a CHANCE at preventing permanent damage, if they would just LISTEN!!!
Okay, let’s get on with things that WE can do to help Livvi. First of all, it is my firm belief (and it’s also what the PCD experts at UNC/Chapel Hill believe, too) that ALL PCDers should be doing neb treatments daily, for prevention, if nothing else, because it’s not like our cilia work on some days and not on others----even if you’re not HEARING the junk, believe me, it’s down there in her lungs, causing damage. And nebs tend to help a little with sinuses, too, especially if you give them by mask, or try to teach Livvi to inhale through her mouth and exhale through her nose.
Xopenex is basically albuterol with the side effects taken out. It’s a left-handed drug (if you’ve never heard of it, there are some things that scientists can take a part of the molecule from the right side of the chemical compound and move it to the left side, and this will change the compound in desirable ways.
The most familiar of these left-handed things is Splenda. It IS made from sugar, but by moving that part of the molecule, they take the calories out of it; the human body can’t RECOGNIZE it as sugar. With Xopenex, they took albuterol and did that to it, and it helps cut down on the high heart rate and jitteriness and hyperactiveness that albuterol can cause. Xopenex is better for parents AND kids; parents, because the child is less of a handful when using it, and for kids, because it never hurts to keep the heart rate down and the kids not bouncing off the walls. So, yes, that would definitely be good for Livvi, as it would allow you to give her treatments much more frequently with far fewer bad side effects.
CPT stands for chest physical therapy, and it is a mainstay for many of us (as well as Cystic Fibrosis patients) for airway clearance. The whole idea of treatment for PCD is airway clearance. Keep the airways clear, and there is far less of a breeding ground for bacteria, viruses, and fungi to grow in.
But it isn’t EASY to keep our airways clear, as the cilia don’t work properly (in some cases, not at all). So we have to do everything we can to make that easier. This includes being absolutely sure we get enough water/fluids every day, as that is a key to keeping the mucus thinner.
Most of us do CPT at some time in our lives, but some of us find other ways of keeping the airways clear that also work for us. CPT is a lot of hard work, but if you can’t get your insurance to pay for a Vest (a machine hooked up to an inflatable vest that vibrates the ribcage, getting that mucus moving in the airways----several in the group have them and swear by them, but I have tried them several times, and never noticed any benefit----I think maybe it’s not intense enough to reach to the areas where I need it, but I have other ways I use, like a flutter valve, which works very well for me. And my lungs are pretty bad, too, which also probably makes a big difference—I’m end-stage, so getting things moving for me is a lot harder than it used to be.
Anyway, the basic technique is pretty simple, although you have to practice it a bit before you feel confident. You position Livvi in one of the positions (I’ll give you the CF website for those) and then you cup your hand as though you were going to scoop up a drink of water with it. Then you just ‘clap’ your hand over her ribcage at a fairly good rate (about 1-2 claps per second; this doesn’t have to be exact—experiment til you find a rate that seems to be doing something for Livvi, like make her cough.)
You can’t really do this wrong as long as you remember two things; you should ALWAYS hear a sort of popping sound (very distinctive, you shouldn’t have any trouble telling it when you hear it) and it shouldn’t HURT her. If she says it hurts, change your hand position just slightly til it doesn’t, anymore.
You can practice this real easily on yourself til you get the hang of how you need to hold your hand. Sit on a straight chair so you have some support for your back, and ‘clap’ on your own bare inner thigh. When you hear the popping sound, you’ll know you have it right. And you will see just how hard you can clap without hurting—you will be AWFULLY surprised!
Most parents try to do CPT twice a day, at least. This is HARD WORK, so if you can, you might check into a Vest for her—quite a few of the kids in the group have them, and it seems like they help kids a lot more often than they help adults, to me. Also, it’s not like she’s not going to need CPT for the rest of her life; they can change out the Vest as she grows, so that she can use the same machine for years to come. But get them to let her TRY one a time or two before you tackle the insurance company about it—they run around $16,000 each, last I heard, so insurance companies usually want some indication that it will DO something for her before they’ll pay for it. Ask her lung doc, but go through ANY of her docs to get it, if you have to.
Now for the CF website: http://www.cff.org/
Once you go there, click on “Treatments” in the blue menu bar, then on “Therapies” on the right side of the next screen. From there, you click on “Postural Drainage” on the right side of THAT screen. They have drawings illustrating hand position and positions for the child to be in. Livvi’s probably small enough that you could do some of those positions with her on your lap, which is easier on your back.
With older kids and adults, we use both hands for the clapping and vibrating, but Livvi is probably a little too small, yet, for that. I didn’t describe the technique for the hand-vibration because I felt that they describe it better than I do. Also, the vibration is a WHOLE lot easier if you just go out and buy a good, strongly-vibrating vibrator and use that on her; I spent YEARS doing this daily to everything from tiny preemies to centenarians, and I can tell you, doing the vibration by hand it hard as heck on the person doing it! You have years ahead of you, doing this, so making it as easy as possible on yourself is a very good idea!
BTW, you may find that it works better if you use one hand for one side of her and the other hand for the other side. I also want to remind you that, since Livvi has the reversed organs, you’ll have to reverse those drawings when you pound on her, as the right and left lungs are different.
How’s she feeling today?
Laurel
momto4girls kartagener-cpt7723@lists.careplace.com wrote:
Yes, I DID put in the “not” on my own—figured it had to be a typo!
Laurel
momto4girls kartagener-cpt7723@lists.careplace.com wrote:
Hi Missy,
I’ve been trying to decide how to respond to your last note in an appropriate and diplomatic way–should I say anything publicly or not?-- and finally decided I just have to be open about clarifying this issue so no one gets a mistaken impression. Dr. Mark Wylam is not now, nor has ever been an adviser to the PCD Foundation. I don’t know why he would have characterized himself this way. We did correspond briefly many, many years ago, but that has been the extent of his involvement in Foundation activities. Since there are no other “PCD Foundations” in North America, I’m really not sure what he is referring to. The current list of PCD Foundation Medical/Scientific Advisory Board is available on our website if you are interested.
In terms of finding a center that currently follows a number of PCD patients, if you don’t want to go to any of the six PCD sites, you might want to consider the U of MN as a possible alternative. The CF Center at the U of MN follows more than a dozen people with PCD–a large population for any single center. Several people who are seen there post to the forums (it’s pretty much the same folks at all three major PCD forums) so you could get feedback on their experiences, which I believe are mostly positive, and get doctor referrals. In terms of adding centers to the PCD “centers of excellence” network, U of MN is at the top of the list for Minnesota.
I want to highlight, again, that there are six centers specializing in PCD in the US and Canada right now–six PCD Centers of Excellence. They are research sites, but they also see people for clinical appointments. One of the reasons the PCD Foundation worked so hard to get this network up and running is so patients would not have to take the “crapshoot” approach to diagnosis and treatment that so many of us had to endure, but could be seen at sites with knowledge, experience and a reliable track record for diagnosis and treatment. This network will be growing all the time and I highly recommend using these resources since they are now available. The expertise of these centers represents the very best of the international consensus on how to make the diagnosis and treat the problems associated with PCD. Is the network perfect? By no means–but it is a darn good start and we have focused on recruiting researcher physicians with experience and stellar reputations within the pulmonary community. Our goal is to have at least one such center in every state by the end of the decade.
Missy, I’m not sure if you ALSO sent this to Michele, but you did send me a copy. How’s Livvi doing today? Did any of the docs FINALLY do something for her?
Laurel
momto4girls kartagener-cpt7723@lists.careplace.com wrote:
Thank you Michele, I really appreciate you clarifying that. The email response I got from Mayo really was misleading then. I respect your opinion, and we really want to go where the most knowledge and expertise in PCD treatment is, so we’ll do a little more reading and checking and maybe wait to go to N Carolina in the summer if we can. Thanks for letting me know.
Missy, mom to 4 girls (14-testing soon, 13, 6 & Livvi 2-PCD)
Hi Laurel, Yes, I sent it to Michele too. Livvi seems to be doing better today. Her ears are not hurting as much. Her Ped. FINALLY called in a different antibiotic last night, so she switched form Omnicef to Augmentin. She already has a presciption for 1 month of Bactrim after finishing the Aug. & then 1 month of Erythrm. after finishing the Bactrim. Hopefully this one will help clear her up quickly. Take care, Missy