A few replies...

[Guest guest]

Hi everyone,

I'm sorry I've been so silent recently, even when people asked for help

with specific issues.... I have tried to make some mental notes along

the way, but to be honest, there's so much going on in my life right now

that Internet stuff has really had to take a backseat. Even my adult

group probably thinks I moved to Africa and got eaten by a pack of wild

lions. :-) I think I mentioned that I've had some unexplained fevers

and some other junk we couldn't get to the bottom of.... well, " some " is

probably an understatement on the fevers -- I think that since the

beginning of February, I have had fevers topping out between 104 and 106

each day, and then I wake up literally drenched when they start to break

in the middle of the night. Needless to say, my life's been rather

consumed with 1) trying to keep up in school, 2) trying to get to the

bottom of these fevers, and 3) trying to maintain some semblance of a

normal life in between both of those! In the end, I opted to take a

leave of absence from school, effective about two weeks ago, and while I

think it was the right decision health-wise (and emotionally) it's been

frustrating personally, to see my classmates, roommates, and friends

moving forward, while I deal with health setback after setback. Long

story short, it's been a very complicated month full of lots of bad

info, but life goes on.... When I have a better sense of how this is

going to all play out, I'll send an update, but for now, I'll just say

that we know why I've had the fevers, and though I am still having them,

we at least know we're not missing some infection that's going to make

me septic in short order. So, we've backed off the IV antibiotics and

have gone back down to my maintenance dose of ampho (3x/week).

In the " life goes on " spirit, I'll take a stab at a few of the recent questions:

Urs - I think Duke will have to interpret her labs and compare them

properly.... I'm sure they're used to having to do so from any number of

different labs, given that they are a referral center for people around

the country. I hope they come up with a workable plan that keeps Macey

doing well year-round. I hope the GI stuff gets regulated, so that the

invasive procedures can be minimized. Sounds very difficult.

Beth A (Madison's mom) - the presence or absence of pneumovax titers is

only one thing to consider in coming to a diagnosis of a Primary Immune

Deficiency (the " PID " she was referring to, Pattie). It's not typical

to make no response to varicella vaccination, as said, but there

are a number of things to consider as part of the reason for that, among

them, " was the vaccine effective? " (i.e. the actual vial of liquid)....

was it refrigerated properly, was the concentration correct, was it

administered properly (vaccines supposed to be given IM can be less

effective if someone has poor technique and goes sub-cu, for example).

Also, was she sick at the time, and was she on IVIG? Vaccines are not

effective while on IVIG because the antibodies in the IVIG bind antigens

from the vaccine and deactivate them before the immune system can

respond effectively. Sometimes, having a fever or some other infection

can " tie up " the immune system enough to lead to ineffective response to

an immunization. All labs need to be interpreted in light of the

clinical situation, and if Maddie has chronic infections with low

absolute levels (even if some titers are in the protective range, or

some pre/post responses appear adequate), the simple presence of

pneumococcal antibodies can't make her suddenly well, and therefore, it

shouldn't be the sole consideration in IVIG administration, nor should

it be the only consideration in coming to a diagnosis. There is a

specific diagnosis that's sometimes made, called Functional or Specific

Antibody Deficiency -- usually made when all the other titers are normal

and there's a lack of response to pneumococcus. If that was the prior

diagnosis, and then all of a sudden, Maddie responded well to

pneumococcus, her diagnosis would have to be revisited, but if the

diagnosis was hypogam, all that implies is that she has low

immunoglobulins (IgG, IgA, IgM, etc). That's still true, and there'd be

no reason to change the diagnosis. BUT, in the end, short of insurance

company documentation, I think there's probably very little merit in

hunting down specific diagnoses or changing them every time new labs

come back. If the clinical picture changes rapidly or the treatments

depend on diagnosis, then it's more important, but if a child is IVIG

dependent, labs may go up and down over the years, and it simply causes

ulcers (for mom and dad!) to have to worry about what to call it every

six months! Hope that helps...

-- I am so sorry that the kids have had such a difficult time

lately. I hope that recovers quickly from pneumonia and

avoids it! Nebulizers can be given for any number of reasons.... as

you said, CF patients do sometimes use them. Here's the spectrum of

meds that are sometimes given via nebulizer: antibiotics (Tobramycin,

for example, is nebulized for many CF patients and some with other lung

diseases like bronchiectasis); meds to open the airways by relaxing the

tight muscles that cause wheezing, etc (albuterol, salbutamol, I believe

is what it's called " down under " ); Intal (cromolyn sodium) - stablizes

one of the cell types in the lungs, decreasing histamine release which

can trigger bronchospasm/mucus, etc; Atrovent (ipratroprium bromide),

which can reduce coughing by acting on the nerves in the airways;

lidocaine (used in studies here for some severe asthma patients);

steroids (decadron, pulmicort - aka budesonide), which reduce swelling

in the airways; DNAse (pulmozyme), which breaks up DNA of the dead white

blood cells that form pus/mucus in the airways, loosening up the mucus;

straight saline (for simply adding some moisture to try to make mucus

thinner and more easy to cough up); racemic epinephrine (used in croup

or epiglottitis, when the airway is swollen and clamping down.... always

used in a hospital setting, in my experience). I'm probably forgetting

some, but that's what's coming to mind now. Hopefully that can help you

get a sense of why nebulizers are considered pretty helpful over here,

for much more than CF!

- 3.1 is low, on most scales, but not drastically. Viruses often

cause low WBC, because viruses tend to live in WBCs -- they " hijack " the

cell's reproductive and protein producing ability in order to reproduce

the viral cells and their products. The only way to kill many viruses

is to lyse (break open) or kill the cell that contains it, so one of the

ways the immune system kills a virus is to destroy the WBCs which

contain it. That means that for a short time, the WBC can go down.

It's just a sign that the body's doing what it's supposed to. A

drastically low count can indicate the same, and while there are serious

reasons for a low WBC, in light of her viral symptoms, my guess is that

this is simply a reflection of what's going on with her virus right now.

On the problem of the doc's office closing, there are actually a few

things you could do to avoid an ER trip, though your doc would have to

order this or help you pull it off. Rocephin can be given IM or IV. I

know she's been getting it IM, but there's no reason the doctor's office

couldn't put in a hep lock and just give you the med and some saline and

such, so that you could give her the med at home through the IV.

Rocephin is usually pushed slowly through an IV, over about half an hour

to an hour, but in reality, it can be given as quickly as one gram over

four minutes (the reason for doing it slowly has to do with what the

vein can tolerate, not any problem with the speed of the drug hitting

her system). Rocephin can be given once a day by IV, so it's not really

different than the IM dosing. The other thing they could do is ask for

a homecare company to come out to your home to give the IM shot or start

an IV and give the Rocephin dose (or show you how). Once the IV's

placed, pushing the med is simple, if they prepare it for you in the

syringe. You give saline to flush, give the antibiotic, give another

syringe of saline, and then take out the IV (or put heparin in it, if

you need it the following day, as well). Doing the homecare version

probably will cost a quarter of the ER, and it would certainly keep you

from having to be exposed to hospital germs. A comment on something

you said.... you mentioned that you couldn't feel sorry for her or you

wouldn't be able to do your job. I think part of your job is to

empathize with , to try to get inside her mind and how she might be

experiencing this. It doesn't mean you have to feel sorry for her, or

baby her, or pamper her because of her illness; it just means that you

keep in mind her experience of illness, and how she might be processing

it.... as a two year old, or as a twenty two year old. Acting tough so

that she will know it's not okay to feel sorry for her will ultimately

probably make her feel very alone in this. I think that Dale has said a

number of times that one of the best ways she's helped Katy is to sit

down and be angry with her, or be sad with her, and let her know that

it's okay to have these feelings, because we would all have those

feelings in this situation. But then it's also your role to learn how

to show her how to move through and forward, experiencing sadness,

anger, pain..... and ultimately, probably, gratefulness, a deeper

understanding of others' suffering and needs, and a sense of herself as

a strong person, no matter how fragile or how resilient her body may

ultimately be determined to be. I don't know if that's helpful or not,

but I think it's one of the ways I've figured out how to cope with

illness and its impacts on my life, and how I think I've been able to

continue to live a very full life, despite a lot of things that might

leave others flattened by pain, grief, or depression.

MG - I don't think B cells have much use prenatally, as almost all the

Ig's a child has at birth are from the mother; IgG crosses the placenta,

predominantly in the third trimester, so IgG a child is born with is

mother's IgG. IgA is transmitted in breast milk, so a newborn's only

IgA is probably from the mother, as well. B cell function develops

gradually, and usually isn't complete until at least age 2. I'm not

sure I understand exactly where your confusion lies, so let me know if

I'm answer the wrong questions here.... A child is born with areas where

B cells will " live " in larger quantities than what normally circulates

through the blood -- these areas are known as glands or lymph nodes.

Adenoids are a form of lymph tissue, as are tonsils, and some areas of

the GI tract. Adenoids are simply collections of B cells, and what you

wrote into the group with demonstrates that they are colonized in the

first few weeks of life with various bacteria, simply by interacting

with the world. There's bacteria everywhere, and that bacteria can take

up residence in the adenoids and tonsils. The immune system can usually

keep that bacteria in check, but sometimes the adenoids and tonsils get

repeatedly and resistantly infected and have to be removed. If

adenoids are large, it may mean that they are swollen from infection

(fluid and other cells collects with inflammation and infection), or

that the B cells are reproducing at a very high rate, trying to take

care of the infection within. In a bit of irony, it's the lack of

function that may lead to the very large collections of cells -- if the

body thinks it's not taking care of an infection, it may get signals to

keep making more and more B cells, thinking it's improving the chances

of making Ig's (but in a very small child, usually they CAN'T make Ig's

yet, unfortunately, so making all those extra B cells really doesn't

have any added benefit, and just increases the chance of the breathing

problems and such that Zach experienced). It's possible to have MANY B

cells and very few immunoglobulins, which is probably the case with

Zach, because B cell function (which is what doesn't mature quickly) is

what is necessary to differentiate into plasma cells and memory cells

(and then have the plasma cells form Ig's). B cell reproduction and B

cell function aren't necessarily connected.

- I'm so glad that 's arm is doing better. What an ordeal! I

hope that he has a GREAT time playing baseball; I have great respect for

him, wanting to do his best no matter what's happened this year. I

think that attitude will probably help him rebuild and relearn the

skills he needs, and baseball will probably be a big part of that,

physically. I hope he become an All-Star.

- Congrats on those 7 extra years. I know they've been full of

heartache and joy, and it's a testament to your love and 's

fighting spirit that he's done so well. I hope that you have an amazing

celebration! I hope also that your family gets a break from this

overwhelming allergy season soon! Sorry I haven't added much about

's echo results -- I feel like cardiology is one of my weaker

subjects (it's so funny - it's my roommate's primary interest, but I

just can't seem to make things stick in my brain when they come to

cardiology!), and peds cardiology is different from adult, anyway, in

terms of normal values and the approach to things like functional

studies such as the echo. I didn't want to give you silly facts about

adult cardiomyopathies and such (as they relate to ejection fractions,

CHF, etc) because very little of it would have had much bearing on

's results. It sounds like they'll let you know if there's an

issue, and he sounds remarkably healthy despite all his little body has

been through. It's just amazing, isn't it? I know he still struggles,

but I just mean in terms of all the meds, all the massive infections,

weeks of bedrest, etc, etc, etc..... it's just remarkable how children

can rebound from things adults would consider insurmountable.

Pattie - glad to meet you! I have appreciated reading your posts

(particularly those about your approach to being the mom of three great

boys, two of whom deal with serious illnesses). Thank you also for the

info you took so much time to type -- thank you for re-doing all of

that! The book sounds very interesting and I think I'll have to invest

in that one. I am not a parent, but an adult CVID patient who runs an

adult PID group like this.... I'm not usually very " talkative " because

I'm so busy, but sometimes jump in when people ask me questions or I

think I have something to offer. I'm also in medical school, which (as

your experience in college proved to you!) can sometimes help with

interpreting this stuff. You practically need a degree to figure it all

out! I'm glad the neti pot has worked for your sinuses -- I do a

similar flush using a Water Pik (w/Grossan tip) and sterile water twice

daily, and I believe it's the reason my sinuses have remained patent for

more than 6 years after surgery, despite my chronic illnesses.

Kim ('s mom) - You're right -- my story is a very long one! I

have been sick for as long as I can remember, and I've had a number of

rare complications of CVID. In fact, they don't even really know if I

have CVID (that's a silly way to say it, but my labs are actually more

consistent with what they might call Combine Immune Deficiency, because

of my severe lack of T cells and poor T cell function, on top of the

lack of Ig's). But in general, particularly when I was younger, I

dealt with many of the same things the children on this list do --

constant infection (sinus, ears, lung, GI), asthma, fatigue. Despite

those infections and being hospitalized probably 4-6 times a year, I

wasn't diagnosed until age 19, when my T cells really hit bottom and I

became critically ill. PID wasn't a common diagnosis, especially when I

was little; my dad was a doctor, so I never really had a pediatrician

(he would bring home antibiotics if I needed them.... though I did have

a pulmonologist, and everyone thought I had very severe asthma and

moderate allergies, and that was the full explanation for why I was

regularly in the hospital and in the PICU in respiratory failure). To

some extent, I am SO THANKFUL I didn't have a diagnosis then (even

though it probably cost me some lung tissue damage), because I was

*determined* to do everything I wanted in life, and I would have been

crushed if I'd had to be isolated, or someone told me I couldn't do

something I wanted to do because of health. And my parents did

something VERY important with me -- they taught me that I was in control

of my body, and I was in charge of my healthcare. That meant if I

wanted to go to camp, or spend the night at a friend's, or ultimately,

go to college 2000 miles from home, I could, because I never had to

depend on them to tell me when to take my medicine, or have them say, " I

don't know if you should do that, because your lungs are bad " -- they

let me tell them if I felt okay, and they knew that I knew what the

possible consequences were (just as now, I know the potential

consequences of the exposures I face in medical school, but I am

careful, I know myself, and I know that living my dreams, even when I

have to pay some prices like give myself IVs or get extra rest or take a

leave of absence, is what keeps me ALIVE). I wasn't the greatest

athlete (though I figure skated for many years), and I tended to focus

on " quieter " activities like piano, writing, student council and such

because I knew I couldn't run and dance and tumble (though I took years

of choir and voice lessons, which all my docs have said served me soooo

well because they taught me how to breathe, even when my lungs were

horrible! So sports and voice lessons and stuff can be excellent ways

to build upon health, if that's something someone loves enough to put

the energy into it. I just wasn't very good at sports -- I'm kind of

uncoordinated, I think -- so it seemed pointless and I didn't like

them!) But in the end, I got to figure out who I was, what I loved,

what I was good at, and what

I believed in enough to fight for. I am pretty sure that I have a

semi-complete introduction in the archives here somewhere (I'll check).

If not, I'll give the address of my website (which I really don't like,

because I hate the picture on the main page, but can't change because

the web design program is no longer made for Mac's and I haven't had

time to do a whole new one! Also, it's about two years old, so much of

the information on the last couple of years is outdated, and there's

been a lot that's happened in those last few years, both good and bad.

I'll check, and if it's in the archives, I'll send the message number

and a brief update with it. If not, I'll send the website with a quick

update, too. I hope it helps -- it was written with the intent of

parents reading it, so that you might understand some of what your kids

go through, and how I coped with things. Thanks for asking about it!

Dale - I know you're in Chicago, but just wanted to say hi and let you

know I'm thinking of you guys.... I'm in the middle of a message to you

about college stuff, and I hope you know you can always write or call if

you need help with organizing infusions, healthcare, etc. in college. I

went to school at Dartmouth, which has a good hospital, but no

immunology department (at that time) and a very, very small town (and

horrible student health service, IMO), so I learned a lot along the way!

Hope you have a wonderful visit with , and that Katy perked up

physically enough for her to enjoy the trip!

I'm going to go eat some dinner, but first I just wanted to put my two

cents in about lifestyles and choices about foods/exposures/etc. I

agree with Dale that there are times when it's necessary to put your

foot down about keeping exposures limited and taking no chances with

health. And that this whole issue really comes down to what's right

for you and your family, because we're all individuals and we have

different needs. I eat fruit, and I rarely worry about where it comes

from. There were some Guatemalan raspberries and some foreign

strawberries that had some problems, but to be honest, in an economy in

which we demand all fruits/veggies year round (vs. seasonally), we are

importing fresh produce all the time, and very rarely does the consumer

know where it came from because in a big chain grocery, the produce

manager sometimes doesn't know where it came from! If you really want

to know, you can go to Farmers Markets and only go to the vendors who

sell there regularly, appear to have good produce, and answer your

questions, or you can go to smaller markets, who often can tell you

where they get their produce, but if you wash it well, you are usually

going to be fine. I don't use Fit because I think it's a waste of money

compared to good old water and elbow grease. I eat from salad bars

(would starve in the hospital cafeteria if I didn't!), but don't buy

things made with mayo and other red flag spoilables, or cubed meat

that's sat there for hours (or dressing that's not chilled properly).

But if you want to get really paranoid, you can catch more from the

ketchup at Denny's or other places with Heinz ketchup in refillable

glass bottles, than you can from most salad at salad bars. I can tell

you if you want the details, but in general, let's just say I don't eat

ketchup in such places anymore (and I believe that a french fry isn't a

french fry without ketchup, so believe it or not, I usually keep some

ketchup packets in my glove compartment or in the zipper pocket of my

purse!). But I eat fast food (even hamburgers sometimes), cook chicken

and ground beef at home, eat at friends' homes, and eat out regularly.

I eat imported cheeses, any sushi that doesn't contain raw fish (you can

get California rolls with crab and avocado, or sushi with cooked

fish/eel), and raw veggies (I'm eating a bag of baby carrots as I write

-- the one thing about raw veggies in bags is that I take them out of

the bag and wash them again before using them. There have been some

salad-in-a-bag things that had bacteria in them, and I just think it's

good personal policy to wash raw things no matter how I buy them). I

drink water from the tap and eat ice (in fact, I'm rather addicted to

it, due to a bizarre side effect of iron deficiency! Drives my mother

NUTS). If I had well water, I might do more to purify it or drink

bottled, though. The only place I don't eat things is in the hospital

(on trays, when inpatient), and that's simply because I really don't

believe it's food. <grin> I am actually about the least picky eater I

know, and I have only had about five cases of true " food poisoning "

(documented by cultures) in my life. Sure, I get GI things, but who

knows where most of those come from.... it's just as easy to catch

something from the dollar bill in your purse that 785,000 other people

have touched since it was printed (I'm not kidding -- money is one of

the dirtiest things in society -- ever been to a casino and played the

slots? Note the black scum on your hands after playing with money for

an hour).

I guess, especially for the newer people, I should say here that I

*don't* have a mild case of CVID... in fact, according to most of my

physicians, I have such an unusually complicated and severe case of it,

they don't usually use that label anymore when describing my case to

other doctors, because they believe they need to use my clinical status

to describe how profoundly ill I have been (and continue to be). A

hem/onc walked into my room this week and said, " I just read the

clinical summary your internist sent over, and I honestly cannot believe

you are alive to talk to me right now, forget completing two years of

medical school! " . I'm happy to go into detail if necessary, but it

seems silly to me (and depressing!) to talk about how sick I am, so in

general, I don't. But I just didn't want people to think that it's

okay for me to take all these chances because I am very healthy, or

rarely get ill, or have a case of CVID that responds well to IVIG so

that I have a very " normal " quality of life. I do these things in spite

of having rather poor health most of the time, and probably BECAUSE I

have rather poor health. I know there are no guarantees, because I've

been too close to dying too many times to think that PIDs can't be

fatal.

I worked at a day care center in high school, and as a babysitter every

weekend and all summer, every summer. I went to college 2,000 miles

from home and lived in the dorm (with public bathrooms and cafeteria

food). I worked with homeless teenagers for two summers in college,

and then again in a residential setting worked with homeless pregnant

and parenting teen parents after graduating. In that job, I lived in

the house as houseparent/counselor for 48 hours at a time each week,

during which time it was my responsibility to teach our residents how to

cook healthy food (and then I ate whatever they made -- even though

sometimes, despite my hawk eyes and best intentions, their kitchen

hygeine left something to be desired). I rocked their sick children to

sleep when they were exhausted or comforting their other children, and I

wiped the little ones' green runny noses when necessary (though left all

diapers to the moms). I believe that children with fevers and

infections deserve touch and comfort, and if I couldn't give it to them

when their mothers couldn't, they would have cried themselves to sleep,

and it was worth a risk to me to ensure they were cared for properly.

These children went to a daycare center for homeless children during the

day -- a repository for all sorts of viruses and bacteria, believe me!!

-- but I couldn't make them stay away from me all weekend until I was

sure they had no illnesses, as that would have been impractical and

unfair to them. Part of my job was to model nurturing and parenting

behaviors to our clients/residents, and that means hands-on work. I

wash my hands religiously (and OFTEN!!), whether I believe someone is

sick or well. Medicine teaches you to remember that all people should

be treated as if they have a contagious illness. I therefore live my

entire life with the precautions of believing everyone around me is ill;

it doesn't stop me from doing anything I would normally do, it just

means I have to make sure to wash my hands, ask people to wash theirs,

use sterile technique when administering my IVs and changing the needles

or dressings. I love interacting with people, and I believe that I

have a gift for interacting with people who are hurting and sick

(especially children), and limiting that would be tantamount to killing

a piece of myself that I love. So I am just careful, but not isolated

or isolating. I have a cat, and I change his litter box - with latex

gloves and a mask - and wash my hands and arms thorougly after. But boy

does he make me smile, and he brings me great joy when he curls on my

stomach and purrs and gives me kisses, when I'm lying in bed feeling

sick and lonely. I travel on airplanes, without a mask, very

frequently (because I have a boyfriend of 2.5 years who lives in another

state, and parents who live in another state, and grandparents who live

in another country). But would I rather not see them? NEVER. Would I

rather wear a mask? No.... it would be hot, claustrophobic,

attention-grabbing, etc. I have had to travel on a plane using oxygen

(the airline provides it with a doctor's letter), though, and that is

also VERY attention grabbing. But that's a choice that has serious

consequences if I become hypoxic, so I'm willing to do things that cause

people to stare at me sometimes. I used to be on oxygen 24/7, and

people sure stared then, too. So it's not that I won't do the things

that are necessary to maintain my health, it's just that I decide which

are important and which don't seem worth it to me. And that's what it

comes down to -- is it worth it? All of our choices in life have

consequences. We pay prices for (or obtain benefits from) every

decision we make, about health or otherwise. Would I demand a private

room for my PID child in a hospital (or a room with a non-contagious

person -- though remember that even a surgical patient can start

coughing from something they caught the day before the surgery and

didn't know about!)? Definitely. Would I make people wash their hands

around my child? Definitely. Would I be careful about exposing my

child to people who are obviously sick? Sure (unless it was something

REALLY important, like a birthday party or a very rare opportunity).

Would I isolate my child from all children, on the premise that some

could be sick and I didn't know it? NEVER.

I pay the price for my choices at times. Certainly I have gotten food

poisoning, but I cannot think of a single event that could have been

avoided by doing something rigidly or limiting something in my life on a

regular basis. It's just a chance we take for eating food. Catching

viruses is the chance I take for being a med student and interacting

with sick people. But it's also the chance I take from interacting with

my peers, my friends, my teachers, and my doctors. And the chance I

take from going to the grocery store, or opening the main door to my

apartment building. It's the chance I take for breathing. The fact

that I get run down and more susceptible to infection is the chance I

take for socializing with my friends (to all hours) or staying up all

night reading a good book, or staying up all night on call, or the

chance I take for living.

My illness does not make me want to limit my life. It makes me want to

expand it -- it makes me want to experience everything I can possibly

experience, have conversations which make me think, interactions which

make me laugh and cry. It makes me live more intensely, love more

intensely, laugh harder, cry more, care more. I might not live as long

as my peers, but whether I live three more days or fifty more years,

you'd better bet that when I die, I'll be living.

This is something I'm passionate about, and it is very much because of

what Dale said about the risk of PIDs -- the real risk is that you won't

live your life fully because of fear. Infections can be treated. Bones

can be mended. Fevers can be lowered (or lived with). But there's no

medicine for a broken spirit.

Just my thoughts....

My best to you all!

Take care,