CF and the Appendix.... Chances are you have appendicitis but don't know it!

LittleLab4CF

Super Moderator
For too long I have been looking for relief from nausea. That was at least two years ago and it I'm sure now that it's been a significant player in my health for much longer. Being sick is just part of CF's definition, like infection, you might never know you have CF if not for chronic infections and maldigestion/malabsorption neonatal testing not withstanding. Being sick and feeling sick are not necessarily one and the same. Most of us just power through infection. I don't know many diseases where an "infection load" is part of our vital signs. Infection eats away at our bodies for the entirety of our lives.

When I was born in 1950, CF was a disease of the pancreas. It's similar in cell types to the lungs and because the pancreas is entangled with everything else in the gut, every non cancerous abdominal complaint is abundant in CF. My GI doctor recently broke with the Party line and made some disparaging observations about the quality of treatment and care of digestive health for adult CFers. For good reason IMHO.

Our sinuses, ears, nasal cavity and mouth, especially teeth are on the losing side of flesh eating infections. Hence dental issues, sinus reconstruction and aspiration caused by an ever present infectious glob of mucus sitting atop the epiglottis, destroying it and its perfect seal. That leaves the modern CF patient with a slowly deteriorating set of lungs, once lost, lost forever. Pulmonary health is both critical and urgent. And it's in no small way why so much attention is given to it at the expense of the rest of the patient, especially below the lungs.

Nausea is the pits. I have a pathway to manage pain, the opioid crisis not withstanding, and there's a plethora of treatments for nausea from milk and saltines to ginger, aromatherapy, OTC antiemetics, and of course pharmaceuticals. I've tried them all and have benefited from them all. Like pain that simply cannot be fully controlled, there's a limit to how much nausea can be eliminated.

I have had a pain in the lower right side of my abdomen, or the right fossa if you use the medical term. At first it wasn't focused, a meal would swell my belly with gas and everything felt like it was on fire. Losing weight below 125 pounds has been a line in the sand for me ever since I was 125 pounds when I was 20 years old. My father and I passed each other on the weigh scale in 1970, he was going to to die soon.
His post mortem showed several CF related health issues, but they weren't associated until my late diagnosis. That line in the sand is somewhat born in fear because it My nausea quotient, as I call it, has been off the charts and I weigh a paltry 114 pounds.

Wednesday, October 23rd, I had my appendix removed in an outpatient procedure. Yeah, another old Primary care doctor warned me that despite the tiny incisions, my body had been assaulted. That was a long time ago and it was my gallbladder. When I came to after the appendectomy, my first thought was, "I'm not going anywhere today". I was wretching and hurling bile, not ready for prime time. Then I remembered, the alternative was a night in the hospital. Fortunately the medications for pain and nausea kicked in and I went home.

Thursday I saw the surgeon who was delighted to say that her evaluation was wrong about the need for the surgery, warning me ahead of time that she saw no evidence of appendicitis. It was entangled with the layers of mesenteric membranes and nearly encased in scar tissue. The appendix had to be cut into two parts for the removal. A normal appendix has a small opening (lumen) that runs the length of the tiny vestige and it has been recently established as a kind of safe for probiotic organisms. My appendix was like 97% of us CFers, with the lumen obliterated by fibrous scarring, making it sealed from the rest of the intestines and worthless as a probiotic safe.

The surgeon who removed my gallbladder in 1997 made a point of saying there was evidence that gallbladder attacks had been going on for many years. He explained that each attack of a certain severity would split the mesentery and the uncovered gallbladder would adhere to the liver or such that built up layers of scar tissue. My post-op appointment was enlightening to say the least. When I asked if her difficulty in cutting the appendix loose from the mess of mesentery tissue was telling me that I have been dealing with appendicitis for a long time, she went into considerable detail about what she saw and what it all meant/means. Like the gallbladder, it showed years of chronic appendicitis and the terrible effect on my large and small intestines.

I'm happy to say that I am hungry, a sensation I have missed for years. The recovery is going well but I was told that it will take three months to heal and a year before my body adjusts to the surgical trauma.

What bugs me no end is the amount of time it takes to be diagnosed and the hit to my quality of life. I've never made any bones about my intention to leave this Earth on my terms. Before appendicitis and subsequent appendectomy I was just initiating talks with a specialist about ending my life. At 114 pounds and no desire to eat, I look like I'm on death's door and frankly was there mentally. Not depressed but so tired of swallowing extra saliva that happens just before vomiting, but usually batting it back. I'm guessing my GI doctor is wondering what the hell GI health specialists do to keep current, diagnose things like appendicitis when a patient has developed eating aversion. I will never understand why so many common CF issues aren't being caught at the first attack. I've had years pass by and for the most part gotten less than help.

If you are plagued by nausea and eating makes you sick, consider the appendix, you have a 97% chance of having appendicitis. Don't count on imagining, it will be negative and don't necessarily expect any great pain or palpable mass, the appendix is going to be a hard little finger that can make life not worth living.

Gyuck,

LL
 

Redonfire

New member
Thank you for your post. My son has been having digestive issues, pretty much since birth, that have developed into gastroparesis by the age of 16. He is now 26. After too many hospital trips, and a lot of research, we are convinced that appendicitis is the culprit. How did you convince your doctor to take out your appendix? We had a wonderful GI Dr. in Denver, then she moved, and we have recently moved and are currently looking for a new GI doctor.
 

LittleLab4CF

Super Moderator
Redonfire, and anyone else who might need to get approval for an appendectomy,
The first thing I did was convince myself that I had an appendicitis. One of the drawbacks of the information age is the ability to find out just about everything about anything and one of the bits of arcane mumbo jumbo is that CFers don't develop acute appendicitis. I'm repeating myself but 7% of the general population will be treated for acute appendicitis compared to a mere 1.1% of CFers.

It's not like our GI tract is healthier, for acute appendicitis you need to have the tiny lumen, or narrow canal open to the inside of the intestines in order for bacteria and maybe some partially digested food or fecal matter to infiltrate the appendix. We start out normal but the first time an infection sets in, the lumen could be obliterated because our extra thick mucus won't be able to drain away the infection. So what does that mean for the CFer?

Once the appendix is sealed off, the remaining mucosa within the appendix can still pump out mucus which dries up making it rigid and constantly sore. Chronic Appendicitis results from the rigid appendix rubbing and poking around the abdominal cavity while attached to a special place in the bowel tract. It turns out that the cecum, a junction of the large intestine, small intestine and the appendix, is close by to the junction of the decending colon and the rectum. The appendix is even closer and when one is acting up so is the other, or it could be.

Desperation was driving the search that landed on appendicitis. Almost overnight I found myself with a stool stuck right at the junction of the decending colon and the rectum. Some serious paristaltic stimulant drugs and such saved me from more serious medical intervention. This was a first for me and I was frightened by the implications. As soon as the impaction passed I was thrown into diarrhea and back.

By this time I had a focused pain in the region of the appendix. This may be more obvious to a younger person who hopefully isn't suffering epic pancreatic pain. My pancreas and the pancreas in most all CFers can't move the digestive enzymes completely out of the ducts because of our thick mucus. Enzymes that remain attack the exquisitely enervated pancreas and in time a vital organ is destroyed. Because the pancreas is so entangled with the digestive system, the pain can exceed what most people experience with kidney stones and giving birth. I am going to be continuing this with links to the medical literature supporting my argument for an appendectomy.

Be back soon,
LL
 
Last edited:

Redonfire

New member
Thank you very much for your quick reply. I am trying to build a case for the GI team here in the hospital where my son currently is. After 9 years of his suffering we are convinced it is the appendix that is causing the problem. He is exhausted and tired of being in pain, and we are desperately seeking a solution. Thank you for your intelligent and well thought out post.
 

LittleLab4CF

Super Moderator
Redonfire,
I've included the literature titles and links to them. There are more out there but I don't think you'll need them. My doctor said that I had educated him about appendicitis in adult CFers and ordered a surgical consult. He's always been open to any ideas that are well supported. Let's hope your son's doctor is too.

When something happens to me like an exponential increase in my nausea, I start searching through medical literature, usually searching Google and Google Scholar. My wife has been a great help, usually by distilling my complaints down to something that makes my search succinct and revealing. She looked up appendicitis in our Mayo Clinic Home Medical Guide. It never occurred to me that I should read up on it, after all, I knew what it was.

Seeing how many symptoms I had, I did a more focused search: "Cystic Fibrosis and Appendicitis" brought up several articles, many from the prestigious medical journal, GUT. The first one is golden, and written 15 years ago, referenced in many subsequent papers.

"Appendicitis in an Adult Patient with Cystic Fibrosis: A Diagnostic Challenge" the title of the first article is fairly short and to the point. https://www.ncbi.nlm.nih.gov./pmc/articles/PMC2095722/ Beginning with an actual patient, the authors say that all the standard imaging, abdominal and pelvic CT scan, and an ultrasound was unremarkable, medical terminology for no charge historically, and/or no enlargement or tissue indicating an infection. The appendix was removed and the outcome was a good improvement. The authors investigated the post mortems on CF patients and 49 out of 51 CFers had chronic appendicitis, that's 97%. It's a good number of patients for statistical analysis.

Other links were published by GUT or RSNA.

Review of the Physical Manifestations of Cystic Fibrosis in the Adult Patient

I love this title.

Cystic Fibrosis below the Diaphragm: Abdominal Findings in Adult Patients

If you need more information please let me know. Personally I think if you're convinced of appendicitis, you can get it done by advocating for your son, and his advocating for himself. Medicare doesn't even have to authorize the surgery, it's approved already.

Since you are in Denver, I have a suggestion for a great GI doctor. I haven't sent any private messages with the new format, assuming it's available, I would be happy to share.

Best of Luck, and please let us know how it goes.
LL
 
Top