Thinking things through - Little Lab? Faust? others?

[heidikk]

This long journal entry is my attempt to pull together the thoughts I have from experience and research. I know it is long, but if you wouldn't mind reading through it, I'd really appreciate your thoughts on - where I'm missing things or drawing wrong conclusions - and whether it would be helpful to share this with Dekorrah's CF team? Thanks in advance. - Heidi background - Sunday the 16th of June, Dekorrah had a
horrible bout of constipation. I know that's not uncommon with cf.
He wouldn't even nurse. I just prayed and held him while he
screamed. Finally about 2am, he managed to pass the thick sticky
stuff that was blocking him up and had what looked like a normal
bm. The rest of that morning he still was unusually fussy for
Dekorrah and appeared to be having some really painful gas. (This
was also at the tail end of his first cold for what that's worth.)
I called the clinic Monday morning and the nurse called me
back with three messages over the course of the day - the nurse,
the doctor, the nutritionist. By the time I actually called, he
was really back to himself mostly, but I called anyway to see if
there was a way to prevent this in the future. The theory is that
the constipation was instigated by becoming dehydrated. (from the
heat or from his cold maybe) That's something I'll have to be super
extra careful of and even more so since we don't have air
conditioning. The doctor said there was really nothing we could do
to prevent it in the future but that if it becomes an ongoing
problem, there is a medicine called miralax. The nutritionist said
to make sure he is getting his full 1/4 t of salt daily, try to
keep him cool, and delay solids (that the other nutritionist was
pushing) focusing on just getting him to nurse as much as possible.
For the next few days, he was intermittently acting like a
milder version of Sunday night was still going on. He would strain
harder than usual and cry about it with every bm, and his stools
were still usually of the thick and sticky looking stuff, though at
least not dry little bits like before. I was afraid he was gearing
up for another miserable night - or worse, the dios I kept reading
about. I was desperate to heal his GI tract and refused to just
accept this as par for the course and unavoidable.
The first thing I did was drop him back to breast milk
only. I also fed him every single waking hour. Within the first
day and a half he had cleared himself out. I thought since he'd
been eating about every 3 hours and was taking 3 creon 6000's at
each meal, it would make sense to give him 1 creon 6000 at each
hour's snack. I could quickly tell from his stools that he was
eating more than one capsule's worth of food at each feeding, so I
upped him to 2 and eventually to 2 1/2, which seems to be just
right.
Before the 16th, I'd weighed him and he was up about a
pound since his last appointment. After the problem was cleared
up, his weight was almost back down to where he'd started. My
guess was that most of that "weight gain" was actually just backed
up junk in his gut. On the positive side, not only is he now
completely better constipation-wise, but also the occassional
chesty cough that he had begun to develop well before his cold has
completely disappeared.
Through all of this I've been doing a lot of researching,
reading, and praying. I realized that since his birth I've been
praying hard for his lungs, but never thought to be praying for his
intestines. I have had some questions and found some interesting
information along the way.
1. Which comes first, inflammation or thick mucus?
Apparently I'm not the first one to ask this question. I found CF
articles that wrestled with this chicken or the egg dilemma and
found that there are at least some researchers who believe that the
mucus in people with CF in the absence of illness/inflammation is
no thicker than in those without CF. The mucus lacks some of the
mucins that help kill the bacteria caught within it. This bacteria
can then cause inflammation which leads to the thick, sticky, pus-
like mucus that causes "permanent" organ damage. (more on the
permanent question later.)
2. Babies with CF are born with normal lungs. Fewer are
born with normal intestinal health. Fewer still are born with a
properly working pancreas. Is there a connection from one to the
other? Could the intestinal problems cause or lead to the lung
problems? Again, I found evidence that other CF researchers have
looked into this question. There does seem to be a progression for
most from pancreas to gut to lungs. There also seems to be evidence
for intestinal permeability in children with CF. From what I've
read, IP is believed to be caused by inflammation from certain
foods being in the diet that the patient is either sensitive to or
finds it difficult to digest. The things that make their way
through the intestinal walls can cause inflammation throughout the
body, especially in the lungs. Intestinal permeability is
implicated in celiac, asthma, adhd, and aspergers syndrome, all of
which seem to be relatively common in children with CF.
more next post...

 

[heidikk]

3. Why the pancreas? This question led me to a better
understanding of the exocrine system and the interesting fact that
both the pancreas and the lungs have cells called acinar cells. So
why is the pancreas first? (with the probable exception of the vas
deferens) Is it because those ducts are so tiny, they just get
sealed off first? Why do some children with the DDF508 mutation
grow just fine and don't find out until later in life that they
even have CF, while others are born with pancreatic insufficiency
and are quickly diagnosed with failure to thrive? Could it have
something to do with the uterine environment and the health of the
mother? Could there be a reason that my gallbladder failed after
my pregnancy with Dekorrah and that the only times I'd had issues
with my gallbladder were during that pregnancy and during my
pregnancy with Savannah (our full term stillbirth.)
This drew me to some fascinating topics such as
microchimerism - the transfer of cells both from mother to baby and
from baby to mother for the apparent purpose of healing one
another. It seems possible that in a pregnancy where the mother's
hepatobiliary and intestinal health is optimal, she may be able to
ameliorate the in utero effects of CF on the baby's pancreas. And
on the other hand, in cases where the mother's own pancreas may be
compromised, the baby's stem cells which include his CF genes may
actually exacerbate her deterioration in his attempts to heal her,
while bringing further detriment to his own.
So, after all that, the next question is what to do about
it. What is the best course of action, not just for CF'ers in
general, but for my Dekorrah in particular? What were we doing
wrong to bring us to the negative effects to his intestinal health
that we were beginning to see? At this point, the factors would be
breast milk, goats milk and yogurt made from goats milk, rice
cereal, and his enzymes. (He did also have one taste of baby food
beef, but while the timing of that was suspicious, it was less than
half a tablespoon in amount. We won't be trying that soon again
regardless.) I think the rice was a big part of my mistake. I've
found rice to be binding in other children of mine. Trying it on a
baby with CF, especially in the amounts that I did, was foolish.
The goats milk / yogurt combination I think has enough
positives to outweigh the negatives. On the one hand, dairy can be
suspect, though controversially so. I realize that the studies
were unable to prove that dairy consumption caused an increase in
mucus production. On the other hand, even those studies
acknowledged that: 1. Although there was not a clear increase in
mucus, there was a temporary thickening of the mucus already there.
2. Those participants who believed in the milk / mucus connection
did describe an increase in cough and congestion symptoms while
ingesting dairy products. And 3. There is suspicion that lactose
intolerance is a factor - perhaps showing that the symptoms
reported in some participants were not a result of their beliefs,
but rather that their beliefs were a result of the symptoms even if
only a portion of the participants experienced those symptoms.
Since for Dekorrah there is not only the CF, for which thick mucus
is already an issue without temporarily thickening it more by the
use of dairy products, but also a strong family history of lactose
intolerance, including dairy products in his diet is a questionable
thing. This is the sort of thing I meant when I asked long ago
whether an attempt to bring him up to the 50th percentile, even
were it successful, might be acheiving weight at a cost to health.
On the other side of the equation, by choosing the easier
to digest goats milk, we should be ameliorating that somewhat.
There are also the positives such as the fermenting of some of the
milk into yogurt containg the L. Rhamnosus culture that has been
proven so beneficial to CF'ers, and the cysteine available in so
few places that is an essential precursor to glutathione, a
powerful antioxidant that is extremely lacking in society in
general and CF people in particular. I will be watching how he
does with the goats milk more carefully as I am reintroducing it
now though. In retrospect, I realize that I should have realized
sooner that the lighter color of yellow and the fact that his
stools were formed were not good things for a baby of his age and
diet.

continue in next post again

 

[heidikk]

Last but not least is breast milk. This would seem to be a
given, and it is. There is the cysteine, the vitamin A, the
micronutrients, the anti-viral properties that attack bacteria, and
so much more - things that he really can't get anywhere else.But
there is also the side of trying to optimize what he gets from that
milk. One of those things that I've studied about and implemented
so far, is my taking DHA, which was shown to increase the amount of
lipase in a mother's milk. However, although my milk was
sufficient to get Kory past this bout of constipation, I would
guess that it can probably do a better job of healing Dekorrah's
gut if my own gut is in better health. Therefore, that is one
thing I'm attempting to work on through diet choices, exercise, and
supplements.
Finally, I come to a place where I wonder if Dekorrah's
pancreas health can be restored as his gut was. I know this is
generally deemed impossible. But I've also read that with the new
kalydeco drug, they are finding that areas of the lungs that they
thought were permanently damaged are being restored, raising
questions about how permanent these things are. Also, although
what they are studying at this point is the lung function, those
who are taking the drug have also reported returning pancreatic
function, need for fewer enzymes, etc. So, I have to believe it's
possible. I believe the answer - the best course of action - is to
focus on strengthening the immune system and healing the
inflammation wherever it may be, and to choose the diet according
to those goals rather than for the goal of simple weight gain. so - thoughts?

 

[Aboveallislove]

I don't have any specific thoughts on lots of what you wrote, but wanted to share what I did have some thoughts on based on experience:
1) CF doctors are pulmonologists. They aren't really trained in the GI track and most GI doctors aren't good at CF GI stuff. I was calling 2-3x a week for 6 months with concerns which were put down to "normal" constipation when we ended up in ER and hospital with DIOS.
2) The "sticky" poop, which i described as "clay" consistency (NOT color) is a sign of a CF GI problem in my opinion. If you search on here you'll see a mom of a little one with a feeding tube which was oozzzing that and NO ONE knew what it was. That clay consistency poop is what DS had along with marble stools and liquid stools and was a dead give away to a back-up. Liquid stools don't mean they have diarhea and nothing blocked--it could be "overflow" where it is going around poop.
3) The "cough" could very well be an acid reflux cough because when stuff is backed-up the acid comes up and not down. That is another sure sign of a back-up for us.
4) No one knows why some have more GI than others. Ours son has had no lung issues to date, thank God, but daily GI issues. I was violently ill his entire pregnancy and wonder if that was connected to his GI issues. Hard to say.
5) You are so lucky to be able to nurse and give him that wonderful "cure all."
6) Big hugs.

 

[heidikk]

thanks, Aboveallislove. I love your thoughts. of our points: 1. that explains some things. I've wondered why it seems all anyone cares about are lungs when it seems to me like we could avoid an awful lot of lung stuff if we took care of the GI stuff. 2. Yes, that clay stuff is was I was seeing when he got bound up. It looked like when you make fresh peanut butter and it comes out of the machine in a thick sticky tube. Fortunately we are no longer at clay or at liquid so I think there's no danger he's still stopped up and I'm just not recognizing it. He is back to more of a soft frosting consistency (closest analogy I can think of right this minute) and a good mustardy yellow. And he's no longer in any distress when trying to go. It looks just like the picture for normal breastfed baby poop on the website. LOL 3. Good thought. He's not a spitting up kind of baby at all, but when he does culture anything, it's e-coli, which I was told can come from reflux, so that's definitely a possibility. 4. Isn't it fascinating to think of the connection between our kids' symptoms and our symptoms during pregnancy? 5. I am so thankful that God has allowed me that privilege. I do not fault those who can't and surely did not mean for it to come across that way. I pray that it didn't. 6. Thanks, and to you too as we walk this road.

 

[Aboveallislove]

re #5 you didnt AT ALL. i was just thinking how great that was...I nursed as much as I could and pumped and added formula to supplement but DS just didn't nurse well, which was sad.

 

[LittleLab4CF]

Hmmm. A great logical thought!!! Allisabovelove has been immersed in this to exhaustion for some time. I started a "constipation compendium" for her about a year back? I am about ten pages into a good BM but now I see how facts need to be in context. I.e. Enzo's poopedium. Maybe Heidikk's format, complete with questions, some rhetorical, can serve to describe infant and adult CF below the diaphram.

I am disturbed that GI problems are less glamorous, along with the profession. Both adult CFGI doctors I know, took years to find. Hopefully I can offer some thoughts before the topic expires.

LL

 

[ashamalie]

Wow, totally unrelated thought here...
I wish I were HALF as intelligent as OP! That was very well put together and informative.

 

[heidikk]

LL, somehow I missed your poopendium til now. Great post LOL and thank you for it. I'll have to dig for the constipation one. I am glad to hear that my writings came across as logical at least. My biggest fear with bringing my research to Kory's doctor is that I'll be seen as "just a mom" and a mom in denial besides. Sometimes I'm tempted to just nod my head at his appointments and go home and do what I want.

 

[LittleLab4CF]

heidikk,

Thanks for enjoing my light hearted poopendium. Although I have a many posts on constipation, or more to the point, an end to constipation, the "Constipation Compendium" is still on my computer. Everytime I start on it, I'm pooped by the time I re-read it, so it has a good title at least! I just feel there is more in me still. (LOL)

LL

 

[Aboveallislove]

hee hee LL. And since we're on some poop humor, I thought I'd share yesterday's: Had to call the on-call GI tonight b/c of red specks in DS stool to make sure it wasn’t a concern and they didn’t want me to bring in to the lab for testing. (DH was kinda worried and I'd rather be safe and check then worry about it--likely just chew-able vitamin.) Anyway, after discussing with very stoic doctor who concluded it wasn’t blood and not a concern, she said if it happens again to they could test a sample at the lab. So I said, “so if it happens again, I call. And what should I do with the poop?” She said, “well, I’d throw it away.” I burst out laughing and said “yeah, I wasn’t planning on keeping it. I meant how do you want me to take/keep another sample for the lab if it happens again.” Dr. Stoic actually started giggling.

 

[Helenlight]

Hi, just thought I'd add my 2 cents in case it might be helpful.Our girl is DDF508, 20 months old now. She had no noticeable issues until 5 weeks old, at which point she got a lung infection, after which we noticed that she wasn't gaining weight as well as she had been. She was diagnosed at 9 weeks, after which she was put on enzymes and the works. We haven't noticed significant GI issues, though she has had pretty chronic thick lung mucus since that first infection. We have only now seen some really positive results after starting hypertonic saline. So in terms of which issue starts first, and how they are all connected- I find that an interesting area too. I think the inflammation side of things is a crucial point and one that needs to be taken into account. Our daughter has had some allergies, food sensitivities, and asthma symptoms- which run in the family. Lungs will typically get inflamed if airborne allergens are present, or if a triggering food is consumed, which means mucus production goes up. Many members of our family have problems with dairy, which can definitely cause a 'mucus-like substance' to form in the back of the throat, sinuses, and eustachian tube (and probably in the gut and other areas too). Of the two studies I have seen that seem to conclude that dairy doesn't cause mucus, at least one of them said this may not be true for people who are allergic to it. Dairy allergy is usually to the protein casein, and often not a lactose (milk sugar) problem (breast milk has lactose in it too). Goats milk contains similar proteins to cows milk and so can cause the same issues. Through my own experience and experimentation, and that of my family members, combined with information I have come across in my research, dairy intolerance (or allergy, if that's what it is) can cause a number of problems: The clogging 'mucus-like' substance, inflammation, gas, bloating, constipation, milia (milk spots on the face), tonsil stones, ear aches and headaches, and eczema. We have tried our daughter on occasion with dairy (there is quite a lot of pressure to put her on it), but every time we do she gets a really snotty nose, tries to cough up gunk that I can hear is in her throat (not lungs), is irritable, itchy and gassy. I also find that my diet can affect her too (I breast feed too), so that's something to take into consideration. I'm always interested in the effect different foods have on the body, and I've just started thinking about different foods and the types of enzymes required to break them down. I feel there must be limitations to what the enzymes given to PWCF can actually break down. They are pig pancreatic enzymes, so suitable for a wide variety of foods, but probably not all. Casein in cow milk breaks down to a casomorphin called BCM7 (bovine casomorphin) which it seems some people can't break down effectively and it causes an inflammatory response in the body.In your case it might be a good idea to come off the goat milk for a while to see what happens. Perhaps try a probiotic powder formulation instead of the yoghurt. Breast milk has a lot of the important probiotics as well (one of the main reasons I'm still feeding!). We add coconut milk or cream and spirulina to fortified rice milk as an additional beverage, instead of cow or goat milk. Soy or almond milk is an option too if you can find a good one.I really think you are on the right track in terms of focusing on the inflammation- when the body is inflamed it is being damaged, without the inflammation a lot of healing can go on. In terms of healing the pancreas- my knowledge is limited here, but my thoughts would be that you can probably limit damage, but crucially that gateway protein caused by the DF508 gene mutation is still going to be there- UNLESS of course you are on a drug that corrects it. Then there is a chance that as the mucus thins again, your body can start to heal. So it probably depends partly on the age and health of the person when they start on the new medication. Hopefully we will come to know more and more about the process that is going on and what steps we can take to keep PWCF as healthy and well as possible through everyday means like diet and exercise etc as well as everything else.Good luck

 

[Helenlight]

Argh it didn't keep my paragrah breaks- sorry!

 

[heidikk]

no problem, Helenlight, I've noticed the frustrating problem of paragraphs on this forum. At least I haven't figured out the right way to do them yet. You've added some very interesting points - maybe the progression isn't always the same as I have surmised. I wonder if it is because of factoring in the asthma. I also find it interesting that you've seen improvement on hypertonic saline. I asked the doctor about starting saline nebs "prophylactically" for Dekorrah but he disagreed and pointed to a study that showed hypertonic saline to be no more effective than regular saline in children under 6. (I wish there was comparison between those with saline of either kind and without anything.) I'm trying to find out if there's some way of getting salt air into his lungs without a nebulizer, short of moving to the coast. Anyone have experience with a salinizer? I've also read about the question of lactose vs. casein being the issue. I know lactose intolerance runs in my family. Don't know about casein. What I do know is that my oldest few children, when starting cow milk at around a year (or formula earlier for the few that I tried that route), ran into problems with constipation, ear infections galore, and eczema. When we stopped drinking milk and only used it as an ingredient, those problems went away. I've yet to run into a doctor though who believed it was a true allergy. Most look at me like I'm a crazy woman. LOL And that's why we're at the goats milk compromise for Dekorrah. I don't like it. In fact, I'm already beginning to notice some issues in just the last couple of days starting to add it back in. He's had some green mucousy poop and fussy gassiness. I don't know if it's because he didn't have enough time to heal before bringing it back, or if it's the goats milk itself. I had really hoped that it would work as it's only been with the goat's milk that he's gained enough weight to keep them somewhat off my back. Nobody cares that my other children also stayed around 15 lbs or so, only slowly gaining from that point, and most of them being still less than 20 at a year old. It took Dekorrah longer to get to that 15 point, but he's at 16.5 now. This is 50th percentile for our family. But that doesn't matter. So we look for ways to add more than breast milk to his diet regardless of the fact that every time I've tried to add other foods earlier than 8-9 months or so with our other children, they've had constipation problems, and sometimes even closer to a year. I feel like beating my head into a wall trying to figure it all out.

 

[heidikk]

on the inflammation and the pancreas question - I agree, "when the body is inflamed it is being damaged, without the inflammation a lot of healing can go on." What I think we need to do is find the cause of the inflammation and stop it or at least treat it as naturally as possible. At least start there and then if necessary move to bigger guns. Especially if it's true that the cf'ers mucus is *not* thicker in the absence of inflammation/infection. Treating it with saline showed improvement for your daughter's lungs. It seems so often, even when doctors acknowledge the inflammation angle, their response is to prescribe anti-inflammatory drugs. Those tend to come along with some pretty serious side effects such as kidney failure, liver failure, ulcers etc. (I've watched my husband's GI health deteriorate terribly being on NSAIDs for back problems over 15 years.) Isn't the liver at least something we should be especially careful about with our CF kids? There are so many dietary choices, herbs, essential oils, etc that seem to be mostly ignored except by individuals trying to work it out themselves. Have you seen this study? http://www.greenmedinfo.com/article...tic-defects-associated-cystic-fibrosis-animal I knew that turmeric was anti-inflammatory, but to think that it showed the ability to also correct the underlying cftr defect, even in homozygous subjects? I find that amazing. Certainly enough to send me in the direction of trying some new Indian recipes.

 

[Helenlight]

I totally understand about doctors thinking we are crazy sometimes! I bet some of them wish mothers would just get out of the way so that they can have an easier job ;-) I understand that to a degree, but really- we know our kids best, right? We are with them every day, noticing what is happening for them, and everyone is different- so not all treatments are going to work the same for every patient.


I agree about the fact that some drugs can cause inflammation and damage to the body, especially in individuals with allergies and sensitivities to various things. One concern I've had is that both of our daughter's prescribed vitamin supplements have sodium benzoate in them, which is known to become benzene in the presence of ascorbic acid (vitamin C). One of the supplements has vitamin C in it! benzene can cause cirrhosis of the liver, which is the second leading cause of death after lung failure in PWCF. I know the quantity is small but it's a known carcinogen that we don't know the 'safe' amount of, and she gets a dose every day. That kind of thing worries me when I'm sure it's possible to make these things without sodium benzoate in them. Anyway, I digress!! I hope you figure things out.

 

[MotherofCFBoy11yrs]

There was a commentary on the hypertonic saline trial urging doctors not to discard a promising treatment because of it: http://211.144.68.84:9998/91keshi/Public/File/40/307-21/pdf/jed120022_2316_2317.pdf. A newer trial has proved that the use of hypertonic saline in young children brings their lung clearance index closer to normal: http://www.atsjournals.org/doi/abs/10.1164/rccm.201302-0219OC.We find hypertonic saline very good and it's the only way my son can cough up anything. After starting HS he does not have to undergo nasopharyngeal suction to get a sputum sample, he simply inhales HS and coughs into a jar - at home, and I take the sample to the lab. He inhales the HS with a mask so his sinuses get cleaned up as well (it's gentler and probably more hygienic than a nasal rinse).I think that in terms of the airways, there is agreement that inflammation is caused by infection, so the best way to reduce inflammation is to treat infection. Studies about CF lung microbiota show that there are all kinds of bacteria in CF lungs which are not detected by normal laboratory tests. Excessive mucus probably means that there is an infection because the mucus in CF airways is normal when there is no infection (I've read that for example chronic PA infection destroys the normal mucus, which is protective, and results in the production of a lot of discharge).

 

[heidikk]

thanks! I'm printing out that first one to bring along to Kory's next appt. and try to bring it up gently. Kory's dr. participated in that hypertonic saline trial so he's kind of invested in it.

 

[LittleLab4CF]

You certainly have been thinking the processes of CF through life and through the body. I don’t know if anybody has considered both in this way. Using Dekorrah as an example of a baby with CF. This response will take some refining and I have sources of information that haven’t come in yet.

“June 16[SUP]th[/SUP] Dekorrah had a horrible bout of constipation”. Doctors, parents and family medical guides can’t seem to agree on the definition of constipation. If there is a typical bout of constipation, most likely several days of food not moving, cramping and malaise, define the outward symptoms. The stool produced from the slow bowel transit has turned to dry clay hence the medical term ‘I think I just @#$% a brick’. As chyme, is extruded from the stomach into the duodenum, it is pretty watery. As enzymes and such reduce the chyme to nutrients, amino acids and most of the water is reabsorbed through the intestinal walls. With normal bowel transit, about 92% of the water is reabsorbed out of the feces. When feces aren’t cleaned out in a timely manner the bowels squish and spasm away and as much as 98% of the water, compacting it into something akin to a fireplace log.

Hard stool constipation can be made better. Instead of three freight cars trying to force the colon around enough to transit from the vertical ascending colon into the (up under the ribs) transverse colon, while another attached freight car tries to make the impossible corner into the descending colon. Then the sigmoid colon…

I dealt with porcelain cracking, bunghole ripping lumps of dry clay for decades. To add insult to injury, a high pressure gassy diarrhea was usually between a couple immovable hard spots. With a working diagnosis of Irritable Bowel Syndrome (IBS), I never could figure what helped and what did harm.

After six weeks blocked up solid, I was looking at surgery or an equally desperate act trying to get things moving. Skipping the details for now, soon I was re-acquainted with that peanut butter extrusion that I can squeeze out until I am too pooped. Substituting hard constipation for DIOS is something I wonder about every day. Somewhere between the two is a normal stool. So, why is this so hard to figure? One reason is the indirect methods doctors use to tell US how our guts are. Someday I am going to order an extra heavy duty Nerf mallet to pummel the next doctor who looks me in the eyes and proudly tells me what I DON’T have. GI doctors, who know DIOS, realize it is just like red hot coals, burning and burning deeper into the gut. And this is just one source of pain with CF. It is a good one however.

For CFers, constipation is just a bowel movement that won’t come naturally. Hard constipation is just what it sounds like, a hard, sometimes very hard stool. Distal Intestinal Obstructive Syndrome (DIOS) is the peanut butter, super sticky, irritating and horribly painful stool. I have been trying to keep current on the topic of DIOS, and it almost depends on the author when it comes to defining it.

As an adult male, I imagine DIOS as a TV cutaway of a sink drain. You can see a waxy solid adhered to the pipe wall, leaving a very tiny pathway for water and junk down the disposal. Doctors are quick to point out that DIOS can occur in both the small and large intestines.

More on DIOS and at least my theories of what might be going on, later. You carefully went through the thought of dehydration as constipation’s source. One primary way of improving the pliancy of stools is good hydration. Something like Gatorade or a drink that can be salted is preferred over any direct delivery of salt. In solution, salt will stay in the intestines and draw moisture into the stool via osmosis. It only works if the gut is salty and the body well hydrated.

On the subject of foods, I am no zealot for any camp. Being informed and rational is the best advice when it comes to navigating these waters. I have been trying to contact friends who used a very well thought out means and methods for introducing foods to baby for best tolerance and development. The first year is definitely mother’s milk. My heart goes out to every mother, but today they have double breast pumps that emit a recording of the baby crying to some scent to stimulate your emotions that put out more good stuff in the milk. And they give them to new mothers!

They had specific milestones for the introduction of Goat’s milk, soy and a mix of holistic and fast foods etc. I have studied every argument for decades on the merits of food introductions at certain ages to modifying a diet for a child such that they can never leave sight of the home garden. Try new ideas slowly. You might want to see how something affects you first, but I am not eating baby food carrots!

Mirilax or Polyethylene Glycol (PEG) is an inert hydrophilic gel or soluble fiber, sort of. PEG expands, widening its chemical bonds to saturation with water. Psyllium husk is not something I would try blind. As a soluble fiber, compared to PEG, they have their pluses and detractions. Mirilax is almost universally recommended because it seems to be gentler. When you properly mix water/juice/milk with PEG, just imagine that glass, bowl or bottle as a solid that will keep that amount of water bound up from ingestion to defecation. The mass and volume will remain unchanged as well.

When PEG rich chyme oozes from the stomach into the duodenum, the stomach has kneaded and worked on with amylase and HCL. No matter how hard the digestive enzymes and bowel spasms labor to remove water, PEG keeps stool pliant and bulked. Bulking laxative action depends on stimulus and gravity. A normal bowel senses a large weight and soon the bowels start to move. As long as that stimulus works, it is a good one. Infants should be encouraged, but this is again between parents and doctor.

PEG is in my diet for CF and the fact that I don’t eat enough food to stimulate a bowel movement until it becomes a problem. PEG is most often the bulking laxative of the elderly. I have a concern about PEG. Being inert, it neither cultures nor kills bacteria. Our sticky CF mucus sounds a lot like this in a way. Everybody complains of bloating and gas accompanying PEG as part of the daily protocol. I have made hundreds of nutrient concoctions for specialized growth in the laboratory and this is a good recipe. An inert gelatinous matrix thoroughly mixed with all the phlegm, mucus from the ENT area, lungs, and of course our over active gut, combined with…dinner!

CFers report doctors seeing two unique substances during endoscopy. From the top down, they often find a “ball” of mucus in the stomach. What? You mean this was just worthy of an observation? This is probably an ongoing source of thick and sticky mucus exiting the stomach in the chyme. Not exactly on topic, but an endoscopy of the bowels using a standard prep, CFers often leave greenish mucus lining the colon, even stopping a procedure. Strange but true, C. diff and CF are known to produce this impassible goo. And again, if it is mentioned, it comes on the heels of advice to clean out better next time. Just some stuff in the way of the exam?

Please understand I realize everything your baby is going through, you are imagining something worse yet. You need to wear a hat that doesn’t also fit your baby. I understand how critical this is. Although the likelihood of his organs being torn life from limb are miniscule, I have watched my very composed wife go completely berserk watching what used to be limitless options close before her eyes.

You seem to have his Creon administration handled. You have a good way of monitoring his enzyme intake. I will caution you as everybody else about the habit of doctors to prescribe too much enzyme. In a way, until you get to fairly high levels of Creon or such, too much is Ok. Others have the metrics of home monitoring enzyme intake and hopefully they can contribute. Not forgetting my missive to Enzoxx a while back.

 

[LittleLab4CF]

Now I have a reply that spans more than one post. Sorry to all

I noted your concern, pleasure and worry over Dekorrah’s weight. Pediatricians have two baby weight/size charts. One for formula fed babies and another for milk of mom. Of course the baby is going to be a little smaller. Everything I have learned, accepts that the introduction of “formula”, organic or Nestle at proper developmental landmarks evens the score quickly. Most CFer’s with CFGI syndrome can drop a considerable weight from a long overdue bowel movement. People who don’t have CF have something that defies nature in my experience so we aren’t exactly a club.

If it were as simple as the chicken or egg, we would know so much more. Scorp, a new contributor noted that many decades back he was reliably informed that a cure for CF was in sight. I relate to that anger and from time to time, I have been complicit in passing thinly supported discoveries as the future, even the near future. Time has served to humble me and most of my generation.

CF is a monogenetic disease, a condition involving an error within only one gene. Although the initial data showed CF to be an autosomal recessive disease, meaning asymptomatic parents that have one copy of the same CFTR mutation have a one in four chance with each child being genetically CF. This simple explanation is full of holes but for now let’s say it is gospel.

The easiest explanation of CF, caused by a defect from birth, is at the cellular level. The CFTR gene was identified to be active in mucosal cells, although they exist in every body cell. In mucosal cells, a perfect CFTR gene makes the correct number of perfect chloride ion channels. This process is interfered with in CF, resulting in a thick, viscous and sticky compound.

This compound may help define the chicken or egg contest winner. I was absolutely dumbfounded when I examined a couple drops of fluid drawn from my pancreas during a pancreatic function test. As I rubbed the clear material between my fingers, it reminded me of extremely sticky tree sap. It had little odor but balsam seemed to be a good comparison. I could not imagine this surface asphyxiation, almost like a chemical patch, caused by a fluid like material that seems alien to a human body. This is straight from the pancreas.

If my pancreas can put out a cup of ultra toxic, intractably adhesive biological weaponry per hour (with secretin stimulus), what else is making feces so difficult to move? When we end up with an absurd sticky digestive cocktail from the pancreas combined with all the swallowed phlegm and mucus, plus the odd, sticky mucus your malfunctioning stomach can make… leaves no doubt. The thick mucus came first.

The pancreas and the lungs are made from acinar cells, as well as others, but these are special. Although their function of providing protective mucus is similar, this treads on embryology and how similar cells differentiate. Lungs and their alveoli are similar in structure to the pancreas and acinus sacs that excrete that produce a toxic enzyme cocktail, load in some sodium bicarbonate and finally protective mucus to envelope the inner fluids until they drop into the duodenum. If you have any doubt about how lungs and the pancreas have structures. Next time you attend a “Body Works” art exhibit, ask one of those white jacketed doctors to show you a full pancreas. Any full pancreas in the exhibit will do. Just like the lungs, unless something drastic soon after death is done to plasticize the lungs and pancreas, it flattens like some multi-color gauze and lace fabric.

I appreciate the discovery that both cell types are in common areas where CF does the most harm. This may not be the first time somebody tied the two together, but if I learned about this, it was long forgotten. Thanks for placing this in the light.

Why the Pancreas? That resonates with me. No CFer will claim their brand of suffering is better or worse than anybody else’s. Many like me are pleased to admit to a great life and by self declaration call my CF relatively mild. That said, I have dealt with unreal pain from decades of pancreatic autodigestion. By all measures, my pancreas should be fried but it continues to sear and spit with pain.

So many pancreatic problems start with one of the less inspired designs of joining the main bile duct with the main pancreatic duct making a short, vulnerable common bile duct. The first lump of cholesterol filled bile that plugs the sphincter of Odi can spell the end of the pancreas in a very short period. I believe many young PI patients are smack in the middle of a crisis of fine duct systems in the lungs, liver, pancreas and intestines soon after birth.

Possibly the common bile duct is blocked just at entry into the duodenum. Pancreatic secretions stay in the pancreas until the mess breaks down and pancreatic autodigestion begins. Bile and what my old GI doctor called “bile sludge” develop stones from a pretty early age and possibly a terrible night of colic was really terrible. This plumber’s nightmare begins with a common bile duct? Then the pancreas’ duct system is unusually small as is the bile duct system in the liver. Both bile and pancreatic juices are so unusual in CFers, if we could, we’d remove it all and re-install the right stuff. If only. My point is the pancreas and liver have abnormally small ducts throughout and they are more porous than non-CF duct tissue. I wouldn’t jump to microchimerisms to explain the added complication of a hot gall bladder during pregnancy. Chances are very good your genetic contribution to Dekorrah’s CF, leaves you a carrier. Many carriers have the very same tiny duct systems, though the mucus may be unaffected, or much less so. Both my brother and sister were CF free but especially my brother has gall bladder problems, small estuation tubes and so on. And with my sister’s daughter so went her gall bladder. A condition common in CF involves the bile duct system, with or without a gall bladder.

Primary Sclerosing Cholangitis (PSC) is a form of narrowing and scarring of the bile duct system. And mom’s feel it around pregnancy. For a bunch of people who gall bladder’s are removed by thirty, PSC is one that might point people in the right direction.

I hesitate to delve into microchimerisms. For a long time the mother/baby barrier has been the rule. A chimera is a mythical beast that has features from many powerful animals. A microchimera can be a genetic feature transferred to or from the mother and baby during pregnancy. Although it is more a Trojan horse, the mitochondria also transfer genetic material during sexual reproduction. A sperm, gene or the father’s genes from a migrating baby cell into the mother can occur as well as some or all mitochondrial DNA. I suggest we use PM’s to continue discussing microchimerisms. It has real potential relevance but it is sooo hard.
Try not to beat yourself up on trial and error that goes error. I noticed you related a sense of self flagellation a couple of times, being foolish or such. Dropping your kid while swinging baby by an ankle is foolish, ok, criminally negligent, but considering the depth of your thinking, pacing through the logic is far from foolish. Fools do nothing, Leaders do something, it may be right or wrong, but action was taken and that is half of genius.

It looks like I am going to be upping the priority on feeding a baby during the first two years. Sorry, I realize a hundred very good diets for baby are all over. There is merit in graduating from milk of mother to buttermilk and goat’s milk and introduction of rice and then other glutens. I will pm what I find. Assuming it isn’t two days of oral instructions, a couple guides will put formula and goat’s milk in perspective. I am from the generation who is still punishing Nestle for introducing dry baby formula in countries without potable water. Natural baby formulas are much closer to mother’s milk and adhere to gradual introduction of food into the gut.

Lungs have to come out working so I won’t agree that the GI tract is hit first. I also could be wrong, but every CFer I know has a constant battle with GERD and aspiration. Aspiration for several days is almost sufficient to book a hospital room for the coming pneumonia. Lungs aren’t to be casually serviced. We live minutes without air.

DIOS seems to generally mean that an intestinal obstruction is formed by a sticky mass, adhering to the intestinal wall. Hard constipation used to give me the satisfaction of seeing red segments of intestinal lining dividing the other clumps with their one red side. With DIOS, it can occur anywhere in the small intestine through the large intestine. Most DIOS that occurs in the small bowel is near the distal end. This however may be the beginning for a concentric colon liner.

In short, DIOS, or meconium equivalent, possibly leading to acquired megacolon is all caused by what? The stomach makes its vividly described, icky, sticky chyme, and once in the duodenum, dry, chalky bile chowder joins with indescribably sticky pancreatic goo and it is time for the real misery. As goes the lungs and pancreas, so go the intestines. Unless you have actually felt this unworldly goo oozing from the wall of the bowels, it is hard to imagine all of it churned and dried into something that needs some real cleaning out. Not so fast.

LL