A Look Back

As I prepare for my routine follow up with my GI this week, I look back on how my health once was. Each appointment, my blood labs and UA are reviewed. I'm usually never within range for several electrolytes, but my labs are still better than what they once were. During the years after my surgery for my straight pull-thru, I battled the odds with my out of control electrolytes. I had weekly or more frequent appointments with my GI to review my labs in order determine if I needed to be hospitalized again just yet and to review any recent tests I had underwent. My labs included a sodium as low as 111 (should have had a brain seizure), potassium as low as 1.9 (should have had a heart attack), protein as low as 3, low magnesium and b-12, hemoglobin as low as 4. My medications included potassium, sodium tablets and drinks (spoons of salt in a one ounce cup to be swallowed 3 x day), magnesium, iron, polyp growth reducers, bowel control pills and shots, pain medications, protein pills and foods (shakes, bars, and whatever else my mother could cram in me. Ick!) and of course b-12 shots. I may be forgetting some, as you're aware memories tend to blur when dealing with health events.
I had a team of specialists completing tests and consulting with my doctor. My doctor consulted other specialists around the world and I even traveled out of state for continued testing to try to determine what was causing my out of control electrolytes. And oh were some of the tests dreadful, especially when they were completed repeatedly! Everything from simple x-rays to scans with rectal tubes inserted for contrast to be seen in my cavity (this was the most painful) and even making my blood radioactive and injecting the radioactive blood back into my body. After a year, it was decided I had to undergo exploratory surgery as my lost option. It was during the exploratory surgery that it was discovered that I had an intestinal stricture from excessive scar tissue and a hole in my intestine from an ulcer. You see, the stricture made bowel control and nutrient absorption virtually impossible. Stool was unable to pass through the stricture resulting in extreme pain and constipation until I involuntarily vomited, which released the pressure allowing stool to pass through the stricture. Only it wouldn't stop, because there was literally a liter or more of liquid in my intestine that was finally able to be voided. This cycle is what led to my uncontrolled electrolytes.

Scar tissue was removed and I was placed on TPN to allow the intestine hole to heal.
I also underwent hyperbaric treatments where I received 100% oxygen therapy for around 2 hours 2-3 times weekly for a couple months or more. I don't recall the full number of sessions, just that I was extremely concerned about being able to avoid using the bedpan in the hyperbaric chamber with 4-6 other people watching me. Fortunately I never had to use that bedpan, sometimes I don't know how but so grateful I made it!
After a few months of TPN only, which meant nothing by mouth not even liquid for an extended period of time, I slowly was able to begin drinking and eating foods. I started off with one ounce of ice chips once an hour, to a few sips of water every predetermined interval, to a set amount of water at every interval to being able to drink liquids regularly again. I did the same with food. I was only allowed to eat one egg, with salt or pepper only for seasoning, for breakfast everyday. Then I graduated to my breakfast egg and a small salt and peppered boiled chicken breast with a small amount of unseasoned rice for lunch. Later I was able to also have dinner, and it was the same meal as for lunch. After several weeks of this exact same menu everyday, I was able to include a little more variety. This variety came in the forms of any high protein food, mainly lean meats at every meal and several protein bars and protein shakes everyday. The protein bars and shakes were extremely harsh on my body causing unceasing severe stomach cramps and pain.
I hated eating, for weeks it was the same bland food and then when I could have some variety it was only foods that tasted awful and caused extreme pain.

Over time I had my port removed, was hospitalized on average once every 3-4 months for electrolytes and blood transfusions until 2007, when I received my last blood transfusions. My hemoglobin was 4 and I received 4 transfusions. Funny thing is, I didn't even know my hemoglobin was so low and was in complete surprise when my doctor personally called me at work that day and demanded I go straight to the hospital to be admitted. I argued with her about finishing work or classes that day, going home to pack some clothing like I did almost every other time she ordered me to be directly admitted to the hospital. Many times I would advise my professors that I would be admitted to the hospital at the end of class for "internal bleeding" (nicest and simplest way to say 'uncontrollable bloody stools' I could think of) for an undetermined amount of time and would return to class as soon as I was released. I learned over time that others thought I was crazy for not directly going to the hospital as instructed by my doctor. And this last hospitalization, my doctor was not allowing any room for my day's agenda.

I've been very blessed since then, although its a never ending battle, I'm able to maintain my electrolytes well enough to not require hospitalization. I hope my lucky streak will not break for decades to come.

Back Me Up

The majority of the time I am filled with thoughts of gutting myself to relieve stomach pain. But on occasion, I think about gutting myself from the backside as well to relieve back pain.

After five abdominal surgeries, my abdominal muscles became severely weakened and were no longer able to provide proper support to my back. In high school the back pain progressed to the point that when I leaned over, my back would give out and I was unable to lift my torso up and straighten myself again. After  consultation with my GI doctor I was referred for physical therapy to re-strengthen my abdominal muscles to relieve the back pain. I completed my physical therapy sessions with great success.

Through the years though and after two more abdominal surgeries, I've noticed my back beginning to pain me more frequently. It is typically brought on after the initiation of severe stomach pains from bloating and cramps. I believe this triggers the back pain as excessive pressure is placed around my torso. So instead of coping with one pain, I find myself literally surrounded with pain battling two fronts. I've realized with repeated experience that in order to prevent or at least delay or reduce the occurrence of back pain, I must carefully consider my previous, current, and future food intake. As I discussed in Evils of Food there are a multitude of variables in relation to eating that affect my daily health and life. The stomach pains require sitting or lying down for relief, unfortunately this does not provide the same amount of relief for back pain.

I've often considered returning to physical therapy and perhaps that may be in the near future.

Break in the Storm

In the midst of a large storm, beyond obvious concerns of physical and possessional safety, I found myself repeatedly concerned with bowel safety. Making silent appeals to the universe for the power to stop flickering and remain on without further difficulty and for the weather to remain mild enough that we may remain at home without danger to ourselves in fear of not having access to a restroom.

Do you ever find yourself with such concerns?

When growing up, our home had a well rather than using the town's water and everytime the power went out, so did our ability to use the restroom. If we had enough weather notice to suspect power interference then we would fill several gallon buckets of water in order to restock the toilet tank. Depending on the length of the power outage I would then be forced to flee to city to stay with relatives.
Even to this day, in spite of having access to city water, I still find myself worrying about power outages in relation to restroom access.
Or the fear of the house becoming so damaged from a severe storm resulting in leaving the property or becoming trapped in the house and injured like so many that are viewed on the news channels. Think of the additional injury of being found in a compromising situation due to SBS or other health issues brought on by crisis situations or fleeing for safety without adequate accomodations or when necessary.

Perhaps these are silly or irrelevant concerns to others, but to us with our lives constantly being assaulted by health concerns and bathroom habits, is it a surprise that even during times of potential crisis that we concern ourselves with the most basic needs?

Evils of Food

There are so many activities and hobbies that I enjoy that I want to participate in but sometimes I just can't. Sometimes walking  inside from the car is a difficult task in itself much less any real physically strenuous activity.
The evils of food, that wonderful tasting, life preserving particles of food create infuriating, excruciating obstacles. How I hate thou!
Due to having a total colectomy and the majority of my small intestine removed, I have Short Bowel Syndrome and must take medicine to help keep my stomach and bowel calm and controlled in order to function. In addition to SBS issues, food also causes severe stomach pain, cramping, and a delightful soundtrack of very loud stomach rumblings that you can even see move across my stomach like some alien life form trying to break forth from my abdomen. Granted, the rumblings and pain have improved some since the subsequent years after my last two surgeries. My stomach noises could easily be heard across a very large room and once caused my professor to literally jump in the air in fright. I previously absolutely could not allow myself to eat any ethnic foods due to debilitating pain for the period of 1-2 days. Now I am able to eat a variety of foods as long as I'm wise about my intake.

My itinerary isn't planned out by what I want to do but what I think I will be able to do, depending on how my stomach and intestine is reacting to the food I've eaten. And not just the food I've eaten recently or even anytime during that day,  but what I've eaten the day before. You see, there are an extreme amount of variables related to food that affect my body and it's performance. I must consider:

1. How do I feel the day before the activity
2. What did I eat the day before the activity - best to eat something light and small
3. Am I having a lot of stomach pain and cramping
4. What time do I need to wake up in the morning in order to allow my stomach/bowel medicine to start to work
5. Mustn't eat breakfast if it can be helped
6. If my medicine still hasn't started working, I must weigh the benefit vs the harm of eating breakfast as sometimes breakfast will cause my medicine to kick in but other times it will make my bowel worse thus the debate
7. Don't eat anything else until after the activity and drink very little fluids until afterwards
8. Stand and walk as little as possible until activity has started
9. Take sitting breaks as frequent and as long as possible during activity in order to prolong ability to participate in activity

If any of these variables come into play, the activity is frequently very difficult to participate in and enjoy. 

I need to make a distinction, that my diet habits to help control my health issues and to feel good are not bound by the same diet recommendations of Crohn's and Ulcerative Colitis. 

Those with Crohn's and UC frequently are recommended to following certain diet protocols as certain foods will increase or agitate inflammation causing symptoms to worsen. 

For me, food in general is upsetting to my body. I feel my very best when I don't eat. When I don't eat I am not struck with stomach pain and cramps from eating or SBS issues. Only problems are that I really enjoy the taste of food and one must eat to survive. 

So I try to eat light, non-greasy, small portion foods whenever possible as these do not upset my stomach and bowel as much as a heavy, large meal does. 

I don't know if there is another that experiences the same issues with food in the same way as I do, although I imagine it is similar to Crohn's and UC flare ups. But either way, it makes the day very hard to plan.

The FAP Beginning

For those of you that are new to the world of FAP, you must first understand the genetic disease.

Briefly, Familial Adenomatous Polyposis is a rare hereditary genetic mutation on the APC gene that predisposes an individual to colon cancer due to hundreds to thousands of precancerous polyps developing in the colon at an early age. Those with FAP develop significantly more polyps and at an earlier age than those without FAP. If the FAP polyps are not treated, the individual will inevitably develop colon cancer. Due to the autosomal dominant hereditary nature of FAP, the offspring of an affected individual has a 50% chance of inheriting FAP.
There are associated manifestations of FAP as well. These include:

• Congenital hypertrophy of retinal pigment epithelium (CHRPE)
• Osteomas, supernumerary teeth, odontomas
• Desmoids, fibromas, epidermoid cysts
• Stomach, duodenal, and other small bowel adenomas
• Gastric fundic gland polyps
• Brain tumors
• Increased Risk for Gastric Cancers and cancers of the Thyroid, Pancreas, Liver, Central Nervous System, Adrenal, and Bile Ducts

FAP is sometimes referred to as Gardner's Syndrome. This was described as a variation early on, prior to the level of understanding about FAP that is now known and is no longer acknowledged by the National Comprehensive Cancer Network (NCCN). 

FAP occurs due to a mutation on the APC gene and has been associated with mutations between codons 178 and 309, between codons 409 and 1,580, corresponding to exons 5-8, 9-14 and the first half of the large final exon. While the APC gene is the only gene identified to lead to FAP, there have been over 300 different variations of mutations found on the APC gene thus far.

Attenuated FAP is a variation characterized by a significant risk for colon cancer but fewer colonic polyps (average of 30), more proximally located polyps, and diagnosis of colon cancer at a later age; management may be substantially different. AFAP mutation on the APC gene is usually associated with mutations at the extreme 5' (before codon 157) and 3' (after codon 1,595) ends of the APC gene, and in the alternatively spliced region of exon 9 (codons 213-412). 

If a brain tumor occurs in someone with either FAP or a hereditary non-polyposis colorectal cancer condition, it has been referred to previously as Turcot Syndrome. However, the NCCN recommends against using this term as it is imprecise. 

NCCN recommends genetic testing to occur in infancy for children suspected of having FAP with colonoscopies occurring annually upon age 10, if symptoms do not present earlier requiring colonoscopy. For monitoring of hepatoblastoma to occur every 3-6 months from birth to age 5. NCCN guidelines also provide detailed recommended monitoring guidelines for the additional cancer risks associated with FAP. These can be viewed by creating a free account with NCCN. 

To learn more about Familial Adenomatous Polyposis visit these resources:

• National Comprehensive Cancer Network - FAP Guidelines for Testing and Management. Updated Annually
  
• Hereditary Colon Cancer Foundation Patient Guide to FAP. 2015
• National Organization for Rare Disorders FAP Report. 2014
• StatPearls: Familial Adenomatous Polyposis. 2023
  
• Medscape: Familial Adenomatous Polyposis Report. 2019
• The Genetic Basis of Familial Adenomatous Polyposis and its Implications for Clinical Practice and Risk Management. 2015