Happy (or unhappy) Rare Disease Day!

Today is Rare Disease Day.  I usually don’t get too excited about it, but this year, I feel differently because of many different reasons.  I have a rare disease called Familial Adenomatous Polyposis (FAP), a genetic mutation on my 5^th chromosome that causes numerous adenomas (polyps) to grow on the colon, among many other dangerous and complicated health problems.  FAP causes a 100% risk of colorectal cancer, meaning every single person with FAP will develop colon cancer if left untreated.  It’s not if, it’s when.  In my family, because our particular genetic mutation codes for a more severe form of FAP, often called Garner’s Syndrome variant, I also, along with my affected family members, carry a 100% risk of small bowel cancer.  At 9, I had hundreds of adenomas (wart-like growths) along my colon, with several in dysplasia – which means they were in the very beginning stages of growing into cancer.  At 34, I had an adenoma in my small intestine right along my pancreatic bile duct that had dysplasia, meaning it was in the early stages of advancing to cancer.  I had surgery for that and ended up with severe pancreatitis for three months, where I was hospitalized six different times to treat the pancreatitis.  As a result the head of my pancreas has for the most part died, called atrophy, and does not function well.  I have permanent pancreatic insufficiency because of that surgery.   Then of course there’s the colectomy at 9, and many other abdominal surgeries, including removal of both my ovaries due to growths on them and removal of an aggressive, locally invasive rare abdominal tumor called a Desmoid tumor, a cause of my disease FAP.  I have had 9 surgeries in my abdomen that are directly caused by FAP. 

I live without my entire colon (large intestine) including my rectum.  I have a rectum made out of my small intestine.  This is the alternative to having an ileostomy – a poop bag on my side.  If I had to have an ileostomy, I’d be fine, but I was able to keep my sphincter muscle and have a makeshift rectum made out of my small intestine.  It’s a consolation situation.  I didn’t have to die from colorectal cancer, but not having a colon isn’t a picnic either.  I have 20 to 30 bowel movements a day, even through the night.  I haven’t slept a single night through the night without toileting, since I was 9 and had my colon removed. 

Why am I telling you this?  Because you probably don’t understand, unless you are my family or a friend of mine from FAP Family on Colontown (a private Facebook community for colorectal cancer patients, survivors, and their family members).  I’ve been living like this since 1984 and most people have no idea what I’ve been through, so why am I talking about it now?  Because I’m sick of hearing about all the useless complaining I hear every day quite frankly, about insignificant things that in the scheme of peoples’ lives, aren’t a big deal.  I’m sick of my disease being something to be ashamed of per society’s fecal phobias - something freakish, and something worth hiding. 

There are no famous people with a chiseled face, a smokin’ body, and a resume of smash hit movies with my disease that are champions and poster children for the cause.  You will probably never meet anyone else in your life with this disease.  If you don’t know my family members, statistically, you will never meet another individual with FAP.  There will be no online campaigns to raise money for my disease like an ice bucket challenge, NFL games raising awareness for my disease, or clothing and retail items that raise money for a particular disease or cancer.  My disease doesn’t benefit from the orphan drug program rewarding researchers for developing new drugs to treat rare diseases.  Nobody wants to research FAP, because there’s not that many of us and we just keep having our bowels hacked out each time we grow more polyps and more cancer and that is deemed acceptable to the medical community.  Most of us stay pretty silent and don’t complain.  We’re used to it.  It’s a lifelong disease that will NEVER go away and the cancer growths will keep coming, so you are locked into having endoscopies, colonoscopies, and sigmoidoscopies regularly the rest of your life, among several other ongoing tests.  If you don’t do this and you become complacent about the disease, you will develop cancer, 100%, not 30%, not 50%, no chance of NOT getting cancer. 

So here’s a truncated list of the negative ways my disease has affected me that I know most people have no idea is an issue:

1.Not only do I have to go to the bathroom all the time – not having your colon and having a prosthetic rectum causes chronic diarrhea and a need to evacuate such a tiny poop holder frequently – but I’ve had to do it in some of the most disgusting bathrooms you have ever seen in your life.  There is no hovering with FAP and no colon.  Hovering is a joke and not possible.  The worst bathroom I’ve ever experienced in the world was hands down in New York City.  I don’t even want to explain it to you. You think it’s so awful you’ve had to hover pee in a porta-potty, well you don’t want to know where and what I’ve had no choice but to go in (and I’m talking number two), and imagine there being no toilet paper too.  That’s an interesting situation to say the least.   

2. Having no colon as a child and learning how to reuse your prosthetic rectum takes months, so accidents happen in your pants, at school.  You never live that down.  You get bullied from it until the day you graduate from high school.  You never live it down.  You never live down being different.  This is not different in a good way. 

3.  You give it to your kids.  This is the best one by far, the best “gift” of FAP.  Both my sweet children have this and they are just as strong as I am.  They truly are the strongest kids I have ever seen and known and they have never complained once about having the disease, not once.  Z has had three abdominal surgeries to deal with his FAP, so far (there will be more in both my kids futures), and Bluma has had five abdominal surgeries from the disease. 

4.  People are ashamed of my disease.  We actually have family, not related to me (that’s as detailed as I’ll get) that are literally ashamed of my disease and my kids disease.  They are embarrassed by it and they are in denial about it.  We cannot talk about it around them because they can’t stand thinking they are associated with this disease, such an embarrassing and compromising disease.  A poop and butt disease, can you believe it?  What must I have done in a previous life?

5.  People think the disease is kind of funny.  People have laughed about the acronym of my disease FAP.  Apparently fap or fapping refers to something that guys do to pleasure themselves and more than one person has thought is so damn funny.  It’s just not.  It’s FAP.  It’s not funny at all.  It sucks.  I can assure you the disease and the acronym for it has existed decades before the juvenile urban dictionary definition evolved. 

6.  You have to watch your kids suffer in the hospital, multiple times.  I’ve spent nearly three months of my life total just sitting in the hospital with my kids.  That’s just their FAP jail time, doesn’t include mine.  If I included it all, we’d be looking at many months in the health slammer.   And no, getting tubes in your kid’s ears or having a pin put in their broken bone does not mean you are an expert, can even relate to what it’s like for me as a FAP parent in any way, or tell  me you know how it feels to watch a kid have surgery.  This is a whole different ballgame, sorry, it just is.

7.  Your get discriminated against for your disease.  Yes, it’s really true.  I personally have stories and I know countless others of those with FAP and ileostomies that have been asked to leave well-known resort pools (because of their poop bag under their bathing suit), had a leakage accident on a plane and were treated with verbal abuse over it by the flight crew, had body searches from TSA because the xray machine detected the ileostomy and they had to make sure it wasn’t a bomb (well it ain’t a bomb, but if you want, it can be a shit bomb if you push on it), and so many others I can’t even list here.  My son’s former private school asked that he not attend school while he had his temporary ileostomy for two months.  This was several years ago.  Most people, even many lawyers, have no idea that ileostomies (a bag that collects poop externally under your clothes) are 100% protected under the Americans with Disabilities Act and you cannot legally discriminate or deny rights or access to those with ileostomies.  My son’s school was very ignorant and didn’t know this.  I had to educate them on this and they were begrudgingly forced to let him attend school while he had his ileostomy.  They have never and would never deny access to school to a physically handicapped child and have welcomed with open arms several students with physical disabilities and medical issues, including cancer, diabetes, and other medically noticeable diseases. However this was different to them and they felt they could shame our family into hiding my son at home while he had a poop bag.  We were supposed to be ashamed too apparently.  Society is compelled to feel compassion only for outwardly visible diseases that are deemed acceptable and that don’t involve excrement or excrement containing organs. 

8.  No, don’t tell me I’m thin because of not having a colon.  That’s not why I’m thin.  I’m thin because I work my ass off at the gym every day and I eat very little and count my calories like crazy.  I have a slow and crappy metabolism like many other people do but I do the work to combat it. 

9.  I get mini small bowel obstructions all the time.  I buy heat packs in bulk and at least once a week have one on my belly to get my bowels moving because some food is stuck in all the scar tissue riddling my intestines and strangling them.  I don’t talk about it ever because it’s just a normal part of my life.  I go the gym to move it out and I strap that sticky heat pack on my belly under my workout pants while I jump around in class.  Eventually, I get my bowels to move again, but I don’t stop moving, working, and living while it’s happening.

10.  When part of your dating ritual is informing a guy you like on the second or third date that you have a serious genetic disease that can be passed on to progeny, you know things aren’t normal.  I had to tell Marcus on our third date about my disease and all my surgeries, because I really liked him and knew that if we got serious, he would want to know because if he wanted to have kids and didn’t want the possibility of them having such a serious, lifelong disease, I would have to be honest and notify him, in case he didn’t want to go any further with me.  I wasn’t wasting anyone’s time.

11.  I live with no rectus abdominis and it is destroying and deteriorating my spine.  I had a massive abdominal muscle tumor due to FAP when I was 11.  The tumor consumed 90% of my entire rectus abdominis and in order to get clean margins to entirely remove the tumor so it would not return, I lost nearly all my stomach muscles, also called the six pack.  I have my lateral muscles, but they aren’t attached to anything and don’t totally function.  Through intense training and exercise regimens the past 8 years, I can do a one minute plank, all kinds of “ab” exercises, and modified sit ups by using my back muscles entirely.  But because of the strain on my back muscles since I was 11, not having front muscles to hold me up and straight, my spine has taken a huge hit and its deteriorating and has significant osteoarthritis and other abnormalities.  My back hurts regularly and I have to stay fit in order to lessen the pain and the deterioration. 

12.  My life is like one of those Crohn’s and IBD commercials where the sad lady misses her kids basketball game due to having to go to the toilet, or can’t eat the dinner with her family because her bowels are bothering her, except, I don’t pity party and I don’t miss stuff, I just run to the toilet quick and run back and I don’t let it affect my life one bit.  There also isn’t a happy little drug at the end of my life commercial to make it all great again so that I can play soccer in the backyard with my kids and feel totally normal again.  There’s no drug for FAP.

13.  I am uninsurable.  Technically not now because of ACA, but that’s going to go away in due time thanks to our wonderful congress.  I live in fear of not having insurance and not being able to afford it.  Lawmakers have been proposing for months to make people like me, pay very high amounts of money for their insurance, amounts that even my doctor husband cannot afford.  I MUST have insurance, or I can’t have my regular scopes, and then I’ll get cancer in my small intestine, 100%, not an exaggeration, it will keep happening and they will keep finding more as my life continues on.  I’m also nearly impossible to insure for life insurance.  I have a policy now, but you do not want to know how much I pay and it’s the only company that would approve me.  They must think FAP is something a man does to pleasure himself.

14.   I will be at war with this disease the rest of my life, however long that ends up being.  I will always have to worry about it, keep it in check, never become complacent, and beat it down the rest of my life.  It reminds me every day it’s here to stay and it taps me on the shoulder several times a day to tell me it’s still a monkey on my back.

I know everyone goes through stuff.  Some things are way worse than others.  I’m not here to say mine is worse than anyone else’s or ask for pity, because I don’t want that and I don’t do pity parties for anyone, including myself.  I want people to understand who FAP people are and what life is like for us.  I am here to say that people like me do exist and although there aren’t many of us and we aren’t cool and visible and vocal, and apparently should be embarrassed, our disease really sucks and it ain’t no picnic.  It’s a beast and you will probably never understand what it’s like.  But maybe it gives you a little perspective to see how pervasive a disease it is in my everyday life and how I just keep moving on in spite of it.

Get R' Done

Why haven’t you done it yet?  If you’re 50 or older (or people in your family have had colon cancer under the age of 50) and you’ve never had a colonoscopy, why haven’t you done it?  Do you know you could be silently and obliviously growing an ugly little wart-like polyp in the bellows of your large intestine?  It could be morphing into a gigantic cancer ball.  No, you wouldn’t know it.  You might have blood in your poo here and there, but likely, you may not have any.  You might not notice anything abnormal at all.  You might be constipated frequently, but lots of people are who don’t have colon cancer.  You could be tired, but who isn’t by the end of the day?  The biggest reason to pass off having a colonoscopy is that it just doesn’t sound like a whole lot of fun to have a tube rammed up your ass.

If you haven’t done it, it might be fear of the unknown.  Listen, I know it sounds really uncomfortable, not to mention embarrassing.  Let me assure you, the GI doctor has seen just about every shape and sized hiney possible in the world.  He’s seen hairy men’s ones, he’s seen big, juicy ones, he’s seen the cellulite all women have, and you can be sure he doesn’t care about your saddle bags.  He or she has seen and smelled it all and they don’t care, and neither should you.  Yes, it involves your poop chute.  Everyone has one and I will tell you with full certainty, the GI doctor doesn’t care about that either.  She’s used to dealing with crap, and frankly, she doesn’t care.  The GI doctor just wants to help you.  So help them help you.  You can do this! 

There’s really three steps, and they aren’t that bad, really.  1) colon cleanse – they’re popular now.  Vegans, yoga lovers, and health nuts seem to love to get the toxins out of their bowels and they swear by it.  You might feel completely squeaky clean and like you’ve hit the restart button on your bowels.  2) colonoscopy – don’t worry about the tube up your ass thing and it hurting, they completely drug you up and you’ll have an awesome trip.  I promise.  You won’t remember a thing and you’ll be as high as a kite.  It could be kind of fun.  3) recovery – all the sudden, you’ll be totally awake and you might have a lot of gas from the tube getting friendly with your intestines, but it’s all over, you get to know you did something really great for your health, and bonus,  now you can eat! 

I will tell you exactly what a colonoscopy is and hopefully, you won’t be confused anymore about having one.  The doctor needs to see the lining of your large intestine to look for the polyps.  Polyps are wart-like growths that are found in many colons.  They are overgrowth of normal intestinal wall tissue.  The problem with polyps is when they contain mistakes in their cells (in their DNA) that tells them to grow and grow and to never stop.  That’s what cancer is.  If the doctor sees suspicious polyps on your large intestinal walls, he/she will biopsy the polyp and possibly snare the polyp off the wall to remove it.  If the pathology says that the polyp has dysplasia (beginning stages of cancer) or is cancerous, then decisions are made about what to do next – usually removing the affected portion of the large intestine or full colectomy (removal of the whole large intestine in entirety).  If it’s invasive cancer, chemotherapy will be part of the treatment as well. 

How does the doctor see the lining of your large intestine?  He/she uses a long tube with a camera on the end of it called a colonoscope.  The scope is inserted up one’s anus and fed up through the entire length of the large intestine.  The camera feeds the visual of the inside of the colon to a TV screen where the doctor has a magnified view of the intestinal walls.  But before this test can be done, you must clean your bowels out.  The bowels are normally filled with lots of poop.  It’s hard to see through and the doctor would miss a lot of potential polyps if the large intestine isn’t really clean and free of everything you’ve eaten in the past few days.  How do you accomplish that?  Well, that’s pretty much done by a colon cleanse.  You will be given, by your doctor’s office, a drink to consume starting the afternoon before your colonoscopy.  You have to drink this liquid every so often and it causes you to have diarrhea, getting rid of the poop really fast, which is what you want.  The old way (which is a lot worse and I assure you that you wouldn’t like) was to stop eating solid food two days prior to the colonoscopy, followed by enemas - more than one - in the hours leading up to the procedure.  Luckily, you won’t have to endure any enemas, and you will only have to stop eating solid food the day before your colonoscopy.  Come on, it’s not that bad.  Drinking the liquid is pretty nasty, but it’s not the end of the world and neither is the diarrhea, it’s just once and it’s temporary.  And bonus, no enemas.  You can do it!

Once you’re all spic and span up your fanny, you’re ready to rock and roll.  You go to the outpatient center where your colonoscopy is, you get an IV (that is seriously no big whoop), and then nurses get you all ready for your trip down Versed, Fentanyl, and Valium Lane.  It’s awesome!  You won’t remember a thing.  When’s the last time you got to forget the whole embarrassing event happened?  Never mind your college days and too many drinks.  This is totally legit so just sit back and enjoy the drug ride.  It could always be worse.  And no hangover with this one.  Most likely you’ll be tired and groggy (and full of farts) for some hours after the procedure but just go home, veg out, watch some daytime TV, eat some great snacks, and milk it for all it’s worth.  You deserve it.  You just did the best thing you could ever do for your colon and your health.  Yay you!

So forget about your fecal phobias and all things poop and butt taboo that you might be feeling.  It’s ok.  It’s not as easy as having your blood drawn, an xray, or a mammogram, but as disturbing as the colonoscopy process might seem, it’s all worth it.  Catching any possible colon cancer early will without a doubt save your life.  Your spouse will thank you, your children will thank you, and you will be the most thankful.  If you are 50 or older or have family members who have had early stage colon cancer under the age of 50 or a strong family history of colon cancer in your family, please see a GI doctor or your primary care doctor to determine what you need to do right now.  If you’d like a GI doctor referral, please let me know.  I know a lot of awesome GI doctorss that I’m happy to recommend.  And if you’re scared of having a colonoscopy, just contact me.  I can help get you through it.  I’m happy to provide any information and support, so don’t hesitate to contact me.  For more information and resources on colon cancer screening, awareness, and support please visit www.ccalliance.org, www.chris4life.org, or www.fightcolorectalcancer.org.

I am the Warrior

A warrior is an individual who is experienced at combat, particularly within a tribe or clan.  It is also defined as a person who has shown great vigor, courage, or aggressiveness.  There are many ways to define what a warrior is.  I come from a long line of literal warriors.  I am a McFarland, or in Gaelic, MacParlan.  The name originated from the sons and progeny of Parlan.  The McFarland clan was known long before even the thirteenth century to be one of the most warring tribes in Gaelic land.  The family crest illustrates this war-like drive, the crest depicting a man above it holding a sword with the Gaelic words, “This I will defend.” 

I am no stranger to the warrior drive of the McFarland’s.  My dad has always been driven and you didn’t dare cross him as a child because hell hath no fury like that of a McFarland.  If my dad got mad at my sister and me for something naughty that we had done, you knew to run if he put his index finger in his mouth and bit it.  You’d better run or you were going to get it, a spanking that is.  My dad and his brothers and sisters are very mellow and level-headed people, but if you piss them off, you’re going to know it.  They don’t pussy-foot around when they’ve been pushed over the edge and when they’re mad, they’re steaming.  

It’s a character trait that is not admired in our culture, but I have always been proud of my family’s directness and will to succeed.  I come from a typical Irish-American family that are proud and forthright people.  You stand up for what is right, you don’t coward, and you face any hardships life throws at you with strength and pride.  You certainly don’t piss and moan about it and you “take it like a man.”  I am proud to say I am a McFarland and this is my lineage and my destiny.

For the past few years, there has been mounting research studying the genetics of so called warriors - people who are aggressive or physically strong.  This research has identified a gene in them which produces a high level of an enzyme called Monoamine Oxidase A.  Monoamine Oxidases (or MAO’s) are enzymes in everyone’s body that breakdown neurotransmitters in the brain, such as dopamine and norepinephrine – which help regulate mood.  Simply put, people with high levels of MAO’s in their bodies are thought to have a higher propensity for depression, anxiety, aggression, and other psychological problems.  Monoamine Oxidase A is a form of an MAO that has been found in high quantities in people who are aggressive and have a high survival instinct.  The gene that codes for this particular MAO has now been dubbed “the warrior gene.”  It is widely accepted by scientists that people who are aggressive and have a strong will to persevere no matter what, most likely carry the warrior gene.  The newest research on the warrior gene shows that people who possess it are more likely to survive cancer and tend to have a better outcome after treatment.   

It is a joke between me and my husband Marcus that there is no way I don’t carry the warrior gene.  I am a McFarland, coming from ancestors that were savage warriors, and I have a disease that I have beat into submission.  And it has become an even bigger joke with my daughter, as she is the biggest warrior I have ever met in my life.  She makes my childhood fights with my mother look like pleasant conversations.  If Bluma had been born into a Native American tribe, she most certainly would have been named “Stands with a Fist.”  As much as she causes problems for Marcus and me parenting her, I wouldn’t want it any other way.  I am proud that she and my son are firecrackers, and although difficult to guide them, I know this character trait will serve them well as adults.

Why am I ending these 21 days of thoughtfulness and inspiration with a blog about being a warrior, of all things?  When I look back on my days as an FAP patient, my memories of being bullied, and my awkward teenage years, I feel that I was weak and insignificant.  But when I think about my life now, and how I currently feel about myself and my disease, I feel very much like I am a warrior.  I don’t cry about little things and I don’t whine about pain and tiny earthquakes in my life.  I see people around me crumbling from everyday events and then crying out in the community and social media for pity and support.  While everyone needs help from time and time and it does take a village, I wonder how so many people get through life when they are in so much need, so often, of emotional and physical support for every day dilemmas.  I am a warrior and proud to say, I can fight alone and have done it many times.  I’m not afraid to do it with merely my own two hands and I’m a better woman for it.  I think it is admirable and, depending on the situation, essential to go through that battle alone because you will discover that you are strong and can endure no matter what. 

You don’t have to win a war, or climb a 14er, or be trendy and run a marathon, or even battle cancer to be a warrior.  You don’t have to do anything incredible to be an everyday warrior.  You just have to take life as it comes, handle it, learn from it, and move on with strength and grace.  Writing these past three weeks has made me inspect my beliefs about persevering, surviving, and living meaningfully.  I was never very mindful of how much my disease had shaped who I was and who I’ve become.  I always thought I was different because I just am weird.  This experience has made me see that I am quite different from the people around me because my life experiences have been very unique. 

There is only a very small part of me that wishes I hadn’t gone through those things and that I’d be more like everyone else around me, ignorant of suffering if I may say so.  But when I really think deeply about it, to my core, I know that I am proud to be tough and I’m proud to think differently and live differently, and I wouldn’t want to be any other way.  I love who I have become and the most valuable thing I’ve learned the last twenty one days is that I’m thankful I went through everything I did when I was a child.  I accept that I have FAP and I feel positive about it because I embrace all the amazing lessons it has taught me and I am blessed to have received the gifts it has given me.  In spite of all the suffering and the pain FAP has caused me, the only impression it has left on my heart and my soul are its numerous, priceless lessons of strength and perseverance.  And maybe that is what a real warrior is.

It Could Always be Worse

When I was a little girl, my favorite story was called “It Could be Worse.”  The story is largely based on an old Yiddish folktale in which a man complains about the noise from his family, consults a wise Rabbi, who in turn tells him to add chickens, goats, and other livestock to his household in order to solve the problem.  The man goes to the town sage and says something like “Wise one, things are terrible for me.  My mother, my wife, and my six children live in a small house together and there is too much noise.  What should I do?  The wise elder tells the man “Get some chickens and put them in your house.  That will help with your problem of too much noise.“  The man, although a bit confused, does what the wise man says and gets a chicken to live with the already large family inside the little house.  Of course, this does not help, but instead, makes the problem worse with chickens clucking and feathers flying everywhere, and the man goes back to the wise sage for more advice.  The elder continues to make the life worse of the poor man and tells him to get a goat and then a cow, until the home if filled with utter madness.

You have probably heard this story at least once or twice, and you know the conclusion of the story and the subsequent moral it is trying to teach children.  After adding all the chaos with a bunch of barnyard animals to his already crowded and noisy home, the man can’t take it anymore and removes all the animals from inside his house.  Once they are gone, his house is quiet and enjoyable.  He notices the laughter of his children and the coziness of having his family all around him.  The Rabbi was indeed wise as he taught the poor man that although his life was difficult living in a small house with too many people, too much noise, and not enough to go around, his life could be so much worse.  He learned this first hand when he had to also live with a bunch or loud and crazy animals.  The simple man figured out that, although he didn’t have much, he had a family that was dear to him and he now was thankful for his simple and modest life.  He wasn’t able to see this gift until things were awful for him and he then realized his life was never so terrible to begin with. 

Jewish teachings are full of wonderful and thoughtful lessons.  Reading that story when I was little really had an effect on me.  I remember understanding the meaning in the story and thinking it was pretty special.  I didn’t realize then how much I’d have to reteach this lesson to myself and my children when I grew up.  There have been many times in my life - when I was sick in the hospital, when I was suffering with chronic abdominal pain, when my kids were ill in the hospital, and when my kids suffer from their bowel problems – where I’ve had to call on this teaching to bring me back down to earth and ground me.

Everyone has a sob story.  Everyone has had a few bad things happen to them, some really small and insignificant, but everyone thinks they’re a big deal.  We all get sucked into drama and the dregs of a painful event.  And many of us have trouble pulling ourselves out from the pile to see that it just really isn’t that bad.  It’s easy to get caught up feeling sorry for yourself and feeling like you’re alone and beaten down.  But the truth is, no matter how bad it is, it just could always be worse.  You might not feel like looking at it that way and you might want to keep wallowing in self-pity, but you need to look around and see that it just could be multitudes worse.  In situations when I’m feeling bad for myself because something negative is happening in my life, I remind myself of exactly what could be happening to me that would be so, so much worse.  For instance, when my kids were struggling in the hospital during their surgeries and treatments for FAP, I would look around me on my kids’ hospital floor.  I didn’t ever have to go far to see much worse suffering.  I saw a child walking down the hallway, only three years old, holding her mom’s hand and smiling in spite of her bald head and chemo treatments.  My child could be undergoing chemo and battling cancer.  It could be worse.  In the playroom, there was a seven year old girl with traction bandages over her arms and much of her chest, and her face was badly scared.  I would find out later she practically lived at the hospital because she had been burned over 70% of her body and had been receiving major skin treatments at the hospital for two years.  Her brother died in the fire.  It could be worse.  On the elevator, a mom and her son got on.  He was clearly a quadriplegic and his physical function was minimal, but he talked with his mother and they seemed to just go about their day as they waited for the elevator to arrive on the ground level.  It could be worse. 

If you aren’t looking around you for the signs that God is sending you when you are in the pits of despair feeling sorry for your situation and complaining to anyone who will listen about the terrible situation that has befallen you, then you are missing a divine opportunity to thrive.  Sure, everyone has to grieve and everyone has to feel the pain of losing, having less, and seeing things not working out right, but sometimes it’s just not bad enough to warrant such drama.  I hear people complaining everyday about small things, to a level that causes them extreme anxiety, fear, and pain.  I hear people complaining about the contractor not finishing their remodel on time, or their children getting tons of colds and viruses, or their vacation flight was majorly delayed to the Caribbean.  I have no idea why they are complaining, and they sound ridiculous.  You have the money to remodel your house, you have healthy children who might get little sicknesses but they aren’t dying of a disease, and you are lucky enough to be the minority in America that can afford an expensive vacation to paradise.  I even see people creating massive drama about pretty small things like minor surgery, a broken wrist, or treatable and avoidable problems like allergies. 

It could always be worse – you could be diagnosed with stage 4 colon cancer (you’re probably going to die if that’s the case), your arm could have been severed in a terrible car accident instead of slipping on the ice and simply cracking it, and your child could have a virtually untreatable disease like Cystic Fibrosis or be waiting for a heart transplant instead of just avoiding eating dairy.  Nobody wants to hear that their problems aren’t that big of a deal, but people need to start hearing it and listening to it.  This doesn’t mean it doesn’t suck for you and that it isn’t unfortunate, but the truth is, you just shouldn’t be whining because it simply could be so, so much worse for you. 

Even through my disease and all my health problems, I think every day how much worse it could be for me and so I won’t complain.  I could have not known I had FAP and later found out as a young adult that I had late stage colon cancer.  I might have survived, but I probably would have died.  If you’ve going through chemo right now for something like breast cancer or prostate cancer, chances are statistically, you’re going to survive.  You’re going to live and be ok and enjoy more and more years on this earth with the people you love.  That a wonderful thing, not a sad thing.  Sure, it sucks to go through chemo and treatment, but it just could be so much worse.  You could be dying and watching the last days of your life go by.  But that’s not you and you get to keep moving forward.  You get to live, I get to live, and we shouldn’t be wasting this precious time focusing on the little blips on our radars.

The moral of that story “It could be Worse” is so incredible.  It’s not out there to minimize what’s going on in your life.  It’s there to pull you back up.  It’s a pick-me-up, when you’re having trouble seeing through the shit.  It’s there to keep you moving forward and upward.  It’s there to keep you in reality, and it’s there to keep you actually living – I mean finding the little joys in life that are priceless and being present to fully receive them.  If you’re not thankful for how your life is now, even if it could be a lot better, then you’re wasting time on negativity.  It won’t get you anywhere.  Looking for the little nuggets of pleasure around you will get you so much farther than having a pity party for your life.  I for one am celebrating delightful things every day, no matter how crappy of a day or life I’m having.  And I welcome you to join the party with me.     

Reach for the Brass Ring

When I was young, my parents used to take my sister and me to an old amusement park in the hills of Pennsylvania during visits to aunts and uncles in the summers.  It’s an amusement park, called Knobels, which has been frozen in time.  It’s nearing one hundred years old and it has historic rides to go along with its nostalgia.  One of oldest rides is its unique carousel.  It is one of the largest working carousels in the world, with 63 hand carved horses, made in 1913 in New York and purchased for the park in the 1940’s.  This special carousel is one of the last working ones that has a brass ring dispenser.  If you are sitting on a horse on the outside row, you can reach out and grab a brass ring from the dispenser as you sail past it.  A long time ago, they used to give you a free ride ticket for grabbing a brass ring, as a prize. 

I have many memories of going to Knobels over the years when I was little and now as an adult, taking my kids there each summer when visiting family.  But my most vivid memories are those of my sister Jennifer, who was (and still is) the master of grabbing the brass ring.  When I was little, Jenn would run to the outside row of horses as soon as she gave her ticket to the carousel operator.  She was so excited to have the chance to take a brass ring.  When the ride would start, she would immediately be ready, hanging her whole body out from her horse, ever so precariously, like she was on the verge of falling off her horse.  She would reach her monkey arms out as far as she could, and as she passed the apparatus loaded with the brass rings, she would snag one, almost each time she went around.  I would usually be behind her on an outside horse too. 

The first time she ever got one, I was probably in middle school.  She was three years older than me and taller.  I saw her do it the first time and I thought “I want to get one too.”  I stretched out from my horse just a little, not much, and I reached for a ring as I approached them.  I just couldn’t reach one.  I saw the look of joy on her face and the effort it took and I wanted to do it too.  But the difference between me and her was that I didn’t think I could do it.  I wanted to, but I wasn’t willing to go out on a limb.  I was afraid I’d fall off my horse, and I told myself I was smaller (I was stunted in growth too from all my surgeries and illness) and couldn’t quite reach one.  This was my excuse.  I was negative and I told myself I couldn’t do it.  I never thought I was quite good enough to do amazing things and certainly wasn’t going to go out on a limb trying, unlike my sister. 

I envied my sister’s attitude of “I can do it, I can get a brass ring.”  Why couldn’t I grab a brass ring?  But what I should have been asking myself was why wouldn’t I grab a brass ring.  I thought I couldn’t, but I wouldn’t take the risk, so of course I failed, and on top of it I made excuses for why I didn’t.  After all, there were kids my size and even smaller grabbing brass rings left and right.  It would take me until I was well into my adult years to realize just how much this very situation impacted my life almost daily.  I looked around and saw happy people, living successfully, making their way through the world grabbing wonderful things all the time.  I thought “why can’t I have that too.”  But I wasn’t looking at myself and why I wasn’t stretching out and grabbing at life too.  I felt like I couldn’t or I shouldn’t.  I was used to suffering, feeling low, feeling less-than, and feeling frankly – screwed over. 

I felt cheated and deprived by God, by life, by my parents, by society, you name it.  I was letting my childhood and my disease affect my life in a painfully negative way.  I thought I didn’t deserve the brass ring, beyond not even being able to go for it.  My disease had made me feel so small and so insignificant for so many years.  The aftermath of my surgeries had left me so scarred from such things as the pain and the bullying, that I was paralyzed from self-defeating and negative feelings.  I wasn’t thriving yet.  I was letting the world and my life beat me down.  But as I grew older and wiser, I started to find my voice and I started to see things just a little brighter.

I didn’t have to suffer anymore and I didn’t have to hold myself down.  The bullies were gone, I didn’t live with my parents anymore, and nobody was telling me I couldn’t be successful at life.  I was telling myself this.  I let all those bad things from my childhood and my disease infect my head, and I was listening to the ghosts from my past.  I needed to start listening to me and what I wanted and what I needed to accomplish in life.  What is it?  It’s still evolving and developing every day, but since I started reaching for greater things, I’ve decided - I want to be a loving wife who deserves her loving husband, a caring mother who deserves to be needed and loved by her children, and an exceptional person, not just a good one, who always listens, always lends a helping hand to others, and makes a positive contribution to the world and the lives of the people she knows. 

It is said in a Jewish Midrash teaching, one must do at least three things before they die – plant a tree, write a book, and make an effort to have a child or raise a child.  It means you should leave the world a better place than how you came into it.  It means you should leave a legacy, an influence on the people around you.  My biggest goal in life is to leave this world a better place than how I found it and positively influence the lives of others.  I certainly have made my own life better than how it started in my early years.  I don’t think I have achieved enough positive effect on other’s lives yet, but I actively work towards it every day.  I believe I deserve that and I believe I can do that.  I believe I can reach for the brass ring and give it my all and not worry about falling flat on my face anymore, and I believe I deserve to succeed.  I only hope I can convince other people the same - that we all deserve and should reach for brass rings, no matter how much is pressing against us.  We’re not always going to get them every time around, but if we stretch ourselves out there, we’re going to get it sometimes.  And I hope I’m there to see it happen because if I can do it, you can do it.

Resilience

My kids were unfortunate enough to be born with Familial Polyposis too.  They had something going against them from the minute they drew their first breath.  Although they were otherwise perfectly healthy, there was a ticking time bomb inside of them.  But we are fortunate enough to know that my kids have FAP.  Not everyone is that lucky.  Up to thirty percent of people with FAP have no family history of it and they are the first person in their family to ever have the disease.  This is called an index case.  It’s the first person in a family line to be born with a spontaneous genetic mutation.  The area on the 5^th chromosome where the FAP mutation lies is an area that’s known to be hypermutable.  This means that a particular area along the chromosome is known to be abnormally unstable.  There are many other places along human DNA, on other chromosomes, that are also hypermutable and result in a number of other genetic diseases. 

The point I am making is that I am lucky enough to have the knowledge of my family history of FAP, so my family knows to look for its ugly presence.  If you have an index case, you are even unluckier, because there is no reason to ever think you’d have an extremely rare genetic mutation in your DNA.  These individuals are going about their adult life quite normally until they are derailed with some kind of abnormal symptom – usually blood in the stool, which leads them to all kinds of doctors and testing, and ultimately, a devastating diagnosis of colon cancer.  It would be a horrible way to find out you have FAP.  I can only imagine the shock and extreme confusion this would cause to find out you have one freakish and uncommon disease most doctors have never heard of. 

So I count myself lucky.  I get the privilege of knowing my family’s genetic history and I was blessed enough to get to know at the very beginning of my children’s lives that I would have to protect them from this monster.  They say knowledge is power.  It truly is.  Both my children hit the ground running with the testing and screening to keep the disease at bay.  FAP can cause liver cancer in children under the age of six.  My cousin Kevin died from this very cancer and he did have FAP.  Starting at six months old, both my children had liver ultrasounds and their blood drawn to check their liver enzymes, and they did this every six months until they were six years old.  From an early age, my children grew used to being poked and having to sit still to have tests done.  I’ve never understood those parents who cry and act overly-dramatic when their child needs to have a shot or their blood drawn.  It’s not a big deal honestly.  Children don’t like it, but ten minutes later, they’re over it and honestly, they might remember it, but they don’t dwell on it like many parents do.  Get over it – your child will be just fine, it’s just a little poke and in the scheme of things, it could be so much worse, like surgery.

And surgery is just what was on the menu for my kids.  Marcus and I decided when Zehariah was very little that he, and any sibling he might eventually have, would have to have their colectomy young.  Why?  Because it’s not fair, nor ethical, to put a thirteen or fourteen year old through colon surgery, an ileostomy, and temporary incontinence.  It’s hard enough to be a teenager in America, with all the social media, bullying, and peer pressure.  Try being a teen who is running to get to the bathroom in time at school, only to miss and soil their pants.  How embarrassing and how cruel to knowingly do that to a teen.  Many FAP parents do choose surgery during the teenage years for their children, and this happens to be the most common time that FAP patients have surgery.  It makes no sense to me and quite honestly, I find it sadistic.  

In my opinion, having gone through the surgery myself, the best time in your life to be in this delicate position is when you are a small child.  Five and six year olds are not quite aware of the social world around them yet.  Bullying, peer pressure, and picking on other children hasn’t developed yet.  Five and six year olds don’t know that pooping your pants is a problem and if you do, they don’t really care.  They like to talk about poop and farts and they are fascinated with it too.  They haven’t learned from their fecal phobe parents yet or society that pooping is something to be ashamed of.  When children are small, their memories are not very detailed and they adapt to pain and change in body habits much easier.  The older you get, the harder it is to go through the bowel changes that the surgery causes and the harder it is to heal and adapt to.  For so many reasons, the younger you go through surgery, the better.

So, Marcus and I decided very early in our children’s lives that they would have surgery at the end of kindergarten, just before they turned six respectively.  It’s not a big deal to miss a few weeks of kindergarten, and when you come back to school, the kids don’t really care what happened to you, they just want to play and be silly.  They don’t feel afraid of you, like you have the plague or something, and they have no need to make fun of you because that’s not on their radar.

I did the same thing for each of my children when they had their colon removal surgeries.  I prepared them emotionally and physically for the surgery.  I took them to psychologists before their surgery so they could learn what to expect and feel safe to talk to someone about their fears, other than Marcus and I.  Both Zehariah and Bluma went on tours of the hospital, to see where they would have surgery and where they'd recover.  They got to see the play rooms and the fun places of the hospital too.  I invited friends, family, and classmates to visit Z and Blu at the hospital, so they felt like they weren’t isolated.  I decorated their hospital rooms with their artwork and the artwork of their friends who would come to visit them at the hospital. I bought them presents and wrapped them up so they had gifts to open while they were sick and recovering there.   I wanted my kids to experience a hospital stay I did not.  I wanted them to have a “trip” to the hospital, not a prison stay at the hospital.  I wanted them to experience positive things, instead of all negative.  Sure, they experienced some pain, but it wasn’t all bad.  There were friends and family there, presents to open, toys to play with, AND, they didn’t have to go to school! 

I, quite honestly, tried to trick their brain into thinking the whole thing was more fun than agony.  And to this day, almost six years post-op for Zehariah and two years post-op for Bluma, my kids don’t remember much pain.  They just remember their hospital rooms, some of the gifts they received, and friends coming to visit them.  They don’t remember the gory details, and that’s exactly my goal.  I don’t want them to have the emotional baggage I do.  I want them to grow up tough and resilient, but not scarred from their battle with FAP.  So far, they have shown me that they are the toughest kids I know.  Both of my kids faced their surgeries head-on, showing bravery and strength, getting through their hospital stays with courage and positive attitudes, and I know they are stronger for it and will get through anything life throws at them.  And no matter what, I’ll always be there to support them and cheer them on. 

Blue Genes

When I was nine and sitting in that hospital bed having that annoying gene conversation with the psychologist, I never realized then how much those “blue genes” would affect the decisions I made as an adult.  At nine, the last thing on my mind was growing up, getting married, and having kids.  I didn’t start thinking about that until after college.  Even when I was in a serious relationship in college, I never thought about having kids or the possibility of passing on FAP to unsuspecting tiny humans.  It wasn’t until I was maturing and growing up emotionally that I started to think this disease might be a total deal breaker for some men.

Let’s face it, it’s not for everyone.  I wouldn’t fault a person who didn’t want to breed with someone who had FAP.  It’s a lot to take on.  You are signing for the likely possibility (a 50/50 chance with each kid) of having a child/children who need extensive screening, medical intervention, and several surgeries in their lifetime.  That’s not something for just anyone, or someone with crappy insurance either.  It takes a special individual, or in my case a special man.  That man would have to really love me and be willing to be tough with me.  Not only would he have to accept the possibility of having “sick” children, he’d also have to put up with my medical problems and loose bowels.  I certainly couldn’t marry a man who was a fecal phobe or have any issues with poop or talking about poop.

And then, I met my incredible husband Marcus.  I remember going on our first date.  I had met Marcus at synagogue (I know, it’s a little cheesy).  We had a fifteen minute conversation after services and I enjoyed talking to him.  We exchanged contact information and about a week later, we met at a wine bar to talk some more.  About an hour and a half into our “date”, I decided it would be best to tell Marcus that I had the family curse – FAP.  I wanted to be honest and direct.  I didn’t want to waste Marcus’ time or mine.  If he couldn’t handle it, then I’d move on and so could he.  I felt like if I didn’t say something now, I’d be lying and hiding it from him.  Marcus was in medical school and I knew he was intelligent and would understand medically what FAP meant and the details that involved having FAP. 

So, I spilled the beans about my disease.  I felt like I was dropping a wrecking ball right in the middle of our table.  But Marcus didn’t miss a beat.  I told him what Familial Polyposis was, because even some doctors have never heard of it – luckily there aren’t that many of us out there.  He understood what I was explaining.  He engaged me, talking about the disease that had robbed me of a normal childhood.  Then I asked him if it concerned him that I had FAP.  I spelled it out for him that if I ever decided to have kids, I could pass it on to them.  He nodded and said he was aware of that.  Upon saying this, Marcus said he wasn’t scared of me.  He said it didn’t change what he thought of me and he said he enjoyed my company so far and he certainly wasn’t going to run away from me knowing that I had a genetic disease.

I was relieved, because I really liked Marcus.  He was intelligent, quiet, and very kind-hearted.  He was exactly the kind of man I wanted to be around.  After that night, Marcus and I spent more and more time together and if you know me, you know the rest of the story.  At that time, I knew Marcus accepted me and my disease, but I didn’t know what his family would think about it.  I still don’t really know.  I think they are very uncomfortable with it.  I don’t think they know how to relate to it.  I guess it’s not their fault as they never had to face any significant medical problems with their children.  It’s foreign to them, so I think it’s easier for them to keep their distance physically and emotionally from this issue.

After Marcus and I became engaged, we started to talk about whether we could have kids or not, and how that would work.  I knew we would have a 50/50 chance of having a child with the disease.  Did I really want to have a child with Familial Polyposis and bring someone in to the world to suffer?  I wasn’t sure what to do.  If I was going to do it, I couldn’t wait forever.  I only had one ovary and the other was severely scarred and was constantly making cysts and causing me problems.  Marcus and I decided that if we were going to have children, we for sure would have the fetus tested for FAP in the very early months of pregnancy so that we could make an informed decision about what to do.

That meant that once I was pregnant, I would have chorionic villi sampling of the placenta.  At about eight weeks, a doctor takes a sample of the placenta, sends it off for DNA testing, and that testing determines if the fetus is positive for the FAP gene mutation or not.  The process takes about 4 to 6 weeks.  So this is exactly what Marcus and I did.  We did this, to put it bluntly, so we could decide whether we wanted to terminate the pregnancy or have a child with FAP.  Unfortunately, we have a very black and white view of abortion in this country and it’s hard to talk about making a decision like this without the fear of judgment from “pro-lifers.”  I think anyone who would argue it’s a good thing to bring a child with a rare and serious disease into the world, needs an examination by a qualified psychiatrist.  And it’s also judgmental for someone to say they would never dream of even considering terminating a pregnancy like this.  It’s easy to judge when you’ll never be confronted with a situation like mine.

So when I became pregnant with Zehariah, I wanted all the information about whether he had FAP or not, so that I could make an informed and fully conscious decision one way or the other.  I had the chorionic villi sampling done at six weeks, and just before I was twelve weeks pregnant, I got the news I dreaded.  The fetus was positive for the FAP mutation.  I told the genetic counselor on the phone that my husband and I would make a decision in the next few days and call her back.  I didn’t know what to do and Marcus wasn’t sure either, but he wanted me to see a movie that might help us decide what to do.  The movie was “Gattaca.”  If you haven’t read the book or seen it, it is a science fiction film where a genetically inferior man assumes the identity of a genetically superior man.  During the course of the film, you learn that it doesn’t matter how “inferior” genetically you are, you are capable of amazing things if you have the will and work ethic to succeed.  Seeing the film made me realize that I had achieved lots of wonderful things in my life in spite of my suffering and physical struggles.  I had been through a lot of pain, but I was still happy and thriving.  Wouldn’t I be able to teach this to my child too?  Would he resent me for making him live to suffer?  Would my child thrive too? 

I didn’t have the answers to those questions, but I thought that if I could make it through and still be happy, then I could feel alright putting a child with the same genetic disease on this earth because I believed he would make it through too.  We decided not to terminate the pregnancy and six months later, a very healthy and complete little boy named Zehariah was born.  As soon as I held him, I knew the day would come where we’d have to watch him wheeled away to the OR, but in that moment, I was sure I could keep him safe and happy.  Four years later, when we had our daughter Bluma, I choose not to find out if she carried the FAP mutation until she was born.  I figured I’d brought one into the world, how could I go through that decision again?  Maybe it was selfish, I don’t know. 

After Bluma was born and they put her in my arms, I began feeling her head for osteomas.  Those are benign bony tumors found on the skull and flat bones, such as the rib cage, of people with FAP.  Osteomas are only found on people with FAP, so if you have them, you probably have Familial Polyposis.  As I ran my fingers all over Bluma’s small newborn skull, I felt a perfect bump just behind her ear.  It was certainly an osteoma and she had one in the exact same spot as I.  Instead of looking at my daughter to see whose nose and lips she had, like most other people do upon meeting their baby, I was discovering that my baby had my genetic disease and I had to take that in and process it.  My heart sank and I was very low for a few minutes, but then I reminded myself that I had an amazing husband, a beautiful son, and now we had added a little girl to our family – what a joyous day.  I decided to not dwell on discovering that Bluma had FAP, but to celebrate how wonderful life was for us and the magnificent gifts I have been given in my life. 

There are always total bummers in life, and some of us get a lot more of them than others.  I’ve had a lot of shitty stuff happen to me, but I choose to focus on the fabulous.  My husband, my son, my daughter, and the life I’m living are pretty damn fabulous.  You can choose to wallow in the pain of not having an ideal life, or you can keep moving positively forward with what you have.  The choice is always your own to make.