Knowing when to fold ’em

In an earlier post I mentioned my husband was trying a new medicine with some crazy side effects but some potential to improve his Cystic Fibrosis. We waited, did all the research we could, then decided to roll the dice and try it.

CFers that rolled the dice before us had coined the term “Hell Week” wherein the body adjusts to the new medication. Some CFers had a rough few days, some had a rough few months, some had no side effects at all.

Since Orkambi is such a specific medicine for such a specific population it is something of a celebrity in our minds – even hospital pharmacies don’t have access to it yet. When it finally arrived at our door after two months of waiting we greeted it with the appropriate pomp and circumstance, cradling the box it arrived in with the same tenderness we would carry a new baby.

He started Orkambi on July 8, deciding to give it six weeks come Hell or high water before giving up. In his mind Orkambi wasn’t the answer to everything, but it could hold the key to future improvements in his condition. He was choosing to be an optimist in light of all the answers the rapid research and push to approval didn’t provide.

At first he had flu-like symptoms. Fever. Increased cough. He was up most nights sweating profusely, then shivering. We had been warned and were not alarmed by any of this. Eventually his appetite was better and he started gaining weight. His sputum even got thinner and came up easier. So at first he was awful…and better.

He went in for PFTs (a measure of his lung functions) and had dropped 6%. The clinicians figured he must have caught a cold on top of the new medication and he would improve with time.

By September the flu-like symptoms had mostly passed but he still felt awful, had no energy, and his PFTs were hanging out in the low 50s (his baseline is normally in the high 60s). The CF team decided to cut his Orkambi dosage in half and see if that helped.

By the end of September when his PFTs continued to not improve he was admitted to the hospital for what he calls a “tune up.” These visits usually last a couple weeks, then he administers IV medicines at home for a while.

It was a couple weeks past the end of his home IVs when he came to me and explained he still felt lousy even after his hospital stay. This is uncommon, usually after a tune up he feels better.

“I want to quit,” he said in defeat, as if he had failed somehow. We talked it over, decided the only controllable variable that contributed to his drop in FEVs was Orkambi. For all of the trouble, money and side effects we were so hopeful to reap some benefit, but it didn’t go that way for us as it had for others.

He was a trooper. He stuck it out through [as best we can tell] a couple colds and a hospital stay. He made it much farther down the road with it than many have, and certainly longer than I would have tolerated.

We didn’t see it coming, but I’m beginning to suspect the hardest part of stopping Orkambi will be the giving up on something that held such promise. We knew it wouldn’t be instantly life altering, but we were hopeful it would make things a little better in the near future, and maybe a lot better in the distant future. Instead it was a long and winding staircase leading to a steep drop.

He stopped Orkambi a few days ago. He still feels lousy, but hopefully as the weeks go by he will start to feel better bit by bit.


Rolling the Dice

About a year ago the FDA approved a drug for people with Cystic Fibrosis harboring two copies of the F508del gene mutation called Orkambi. It’s not like many other medicines. It’s in a special class of medicines called gene modifiers. True to the name its job is to manipulate a defective gene. Welcome to the 21st century – we’re not just treating symptoms anymore, we’ve begun treating diseases at the genetic level.

Since my husband is a carrier of the double delta F508, we followed the drug’s development very closely for a few years. We read the outcomes of the two 24-week trials. We asked his specialists about it. When it was approved we read the blogs and message boards where CFers posted their experiences. But instead of jump on board we sat on the sidelines and watched the parade go by.

We waited for a few reasons. One reason was we felt there wasn’t enough research. Less than 800 people participated in both of the trials that lasted less than a year. There is still no research on what this drug might do after two years, five years, ten years, etc. The only other gene modifier that preceded it’s release (Kalydeco which targeted a different gene mutation in CFers) only preceded Orkambi by a few years. Frankly we really don’t know that much about the effects of manipulating someone’s DNA yet.

Another reason we waited was because the benefits weren’t that promising. Of the small pool of people they researched for Orkambi, some saw about a 3% increase in lung function. Compare this to Kalydeco where the average was 12%. It also seems less safe. Kaydeco is approved for ages 2 and up, Orkambi is not for anyone under 12. Perhaps not surprisingly there was a camp that asked the researchers to go back to the drawing board until they had something better.

The cost didn’t go unnoticed either. Orkambi packs a price tag of over  $250,000 a year. The initial stories were copays of a few hundred dollars a month IF your insurance would cover it. Last night Orkambi was highlighted on the Nightly Business Report because NICE (the UKs government healthcare) – not wanting to set a precedent for backing a medicine that would cost an estimated $100 million annually – denied it. Vertex, the company that developed Orkambi, cites seventeen years of research as justification in the cost. The landscape is still shifting. The specialists tell us the copays now are between $10-$20 for each prescription.

But the final and most compelling reason we hadn’t jumped on the bandwagon yet was the effect the drug has on the body of a CFer. Orkambi is not a cure, nor does it claim to be. It also doesn’t guarantee an improvement of any sort. The best they can tell us is he might see as much as a 3% improvement in lung function and potentially fewer exacerbations.

What we have seen across the various media reviews is Cfers coining the term “hell week” for the initial phase while the body adjusts to the new medicine. They warn the first part – that can last a month or more – is like the worst flu you’ve ever experienced. Many have given up and stopped Orkambi before their “hell week” ended. It must be bad. CFers are not a troupe of pansies.

What began to change our minds now that the drug has been out a year was our last visit to the adult CF clinic. The doctors had changed their tune. An attitude of “it costs too much and doesn’t do enough for all it puts you through” had become “you are a great candidate for Orkambi and it’s a fantastic drug.” Either Big Pharmaceutical got to them or enough patients made it through hell week and saw enough of a change it became worth it to pitch it to other patients.

For the past week we have talked every night. We’ve talked about the good and the bad. The potential and the unknown. The risk and the reward. I am one of those people that won’t take a Tylenol without very good cause and he was participating in drug trials as a kid. We are a good blend of overcautious and experimental.

The trouble with treating CF is they are still discovering things about it. There is still so much they don’t know. For the majority of his life, the medicines he has taken have been giant question marks. Taking a new medicine and not knowing what will come of it is old hat to him. All of the talking and reading and researching and asking has led us to a place where he wants to try Orkambi. It feels like a real life version of the game ‘Would You Rather…?’

He has convinced me all that we don’t know about it might be good. If a defective gene is the problem, what if making that gene behave appropriately for a year, two years, ten years, twenty years reaps results we can’t even imagine? What if each of the effected organs begin functioning better after a period of exposing them to a physical environment with less mucus blocking important passageways? He ultimately convinced me that “what if…” doesn’t have to be a bad thing. It could actually be a very good thing.

So we are rolling the dice. The prescription has been requested and we are waiting for a call from a specialty pharmacy. He tells me he will stick it out for six weeks and hope hell week ends before the six weeks is up. I have arranged a private meeting with my boss to prepare for potential “I need to leave work right now” moments. All of the ducks are lining up for us to step into 21st century medicine.

Fish Tank Granola

Greetings again, everyone! Welcome to the wonderful world of medicine and the adventure it is being married to someone with Cystic Fibrosis.

In my last post I gladly announced my husband does not have tuberculosis. Yay! That in fact he has a non-TB mycobacterium with a difficult name to pronounce. Tomorrow we will wake before the sun rises to drive to a research hospital to meet with the top lung expert in the state of Oregon about his – say it with me, kids – chelonae infection.

So what is chelonae? That is an excellent question. A question I took to the internet to find an answer for. WebMD believes it is a skin infection you get from tattoo ink. That is where the easy, breezy understand it in one paragraph and move on with your day starts and ends. The rest – and more accurate – of the information on chelonae can be found in dry medical journal studies that are neither conclusive nor comforting.

To someone like my husband, who eats, breathes and lives in medical jargon, these articles are interesting and provocative. To someone like me, they are enigmatic and full of made up words.

Whenever I need to be cheered up, I ask him to read French to me. It’s hilarious to hear someone with a Texas accent and no formal education in French take a stab at it. When he needs cheering up, he asks me to read from a medical journal. After a few sentences of struggling through Latin-based anatomical jargon, I get creative and go for the first word that comes to mind that most closely resembles the word on the page. This is where my reading aloud about chelonae got us:

“Currently, the epidemiology and clinical features of the NTM diseases are dominated by their occurrence in patients with HIV infection and, more recently, in patients on tumor necktie factor a Tunisia pathway blocker. However, disease caused by this group of orgasms continues to occur in persons without delectable seismic immune dysfunction (e.g., recipients of prostitute knees, fish tank granola, hot tub lung).”

Once I get a few paragraphs into it, and he stops laughing, he gives me a definition I can understand. The short and sweet version is that he has a NTM (non tuberculosis mycobacterium) infection in the soft tissues of his lungs that will take about a year to treat.

And for those of you who were wondering, yes, hot tub lung is a real thing, prostitute knees and fish tank granola were my creations. 😉

CFers Mac & Cheese

This recipe is part of a regimen to add weight to my CF DDF508, GERD, CFRD 35 y/o husband.


box of mac & cheese (any brand, the cheap stuff with the powdered cheese will do)

4 Tbsp salted butter

1/2 cup heavy cream

1/2 cup shredded cheese (any cheese you prefer, Gouda, Cheddar, Havarti, etc)


Cook macaroni in water following package directions.

After draining, stir in butter and heavy cream.

Stir in powdered cheese and shredded cheese, turning pan on to low heat if needed to fully incorporate.


Approx. carbs for the whole dish: 111

Approx calories for the whole dish: 2,193

Breakfast Skillet

This recipe is part of a regimen I feed to add weight to my CF DDF508, GERD, CFRD 35 y/o husband.


2 medium Russet or Yukon Gold potatoes (or sweet potatoes)
3 strips bacon
1 large bell pepper, diced
1 cup mushrooms
1/2 cup onion, diced
1/4 teaspoon salt
1/4 teaspoon ground pepper
4 large eggs
1/3 cup shredded cheese (just about any kind will do)


Place a 10 – 12 inch skillet on the stove. Before you turn the heat on, lay out your three strips of bacon on the pan. Then, turn the heat on low.
Cook the bacon on both sides just before they become crispy. While waiting on your bacon to cook, wash and scrub the potatoes clean, then dice them.
Remove bacon from heat, leave the grease in the pan, and set bacon on a plate lined with paper towels to absorb some grease.
Preheat oven to 400F degrees.
Turn the stove heat up to medium. When bacon grease begins to simmer, add the potatoes and onion and give them a quick mix.
Allow to cook for about 2 minutes, untouched.
Add the chopped pepper, salt, and pepper. Stir things around once or twice.
Allow to cook until potatoes become brown, stirring occasionally.
Break the bacon into the pan with your fingers, stir in the mushrooms, and cook for 2 minutes.
Remove skillet from the heat and flatten out the top of the hash.
Using the back of a spoon, make 4 shallow indentations into the hash.
Crack an egg into each indentation.
Top with shredded cheese.
Transfer skillet to the oven and bake until the egg whites set, about 5-8 minutes.
Season with salt and pepper to taste.
Serve immediately.

Makes 4 servings. Approx total carbs per serving: with Russet potatoes, 35g carbs; with sweet potatoes 19g carbs

Sweet Potato Salad


for salad:
1 medium sweet potato
1/2 medium onion, sliced
1 tablespoon olive oil
1 tablespoon smoked paprika
1 cup cooked grain (brown rice, quinoa, millet, oats, etc)
2 to 3 handfuls spinach
for dressing:
2 tablespoons lime juice
1 tablespoons honey
2 tablespoons olive oil
1/4 cup cilantro, minced


Cook grain of choice (rice, quinoa, barley, etc.)
While grains are cooking, cut sweet potato and in to 1/4” cubes.
Toss with 1 tablespoons olive oil, onion, and smoked paprika.
Cook in skillet at medium to medium-high heat until tender.
Whisk together lime juice, honey, cilantro and olive oil.
Toss grains with desired amount of spinach and dressing.
Top with cooked sweet potatoes.

Makes 2 servings

Total carbs per serving: 53g

Italian Stew

This recipe is part of a regimen I feed to add weight to my CF DDF508, GERD, CFRD 35 y/o husband.

1 tablespoon plus 1 teaspoon olive oil, divided
16 -ounces of gnocchi (or shelf-stable gnocchi from the store), cooked
1 medium yellow onion, thinly sliced
4 cloves garlic, minced
1/2 cup water
6 cups chopped geen leaves, (ex: spinach, chard, collard greens)
2 diced roma tomatoes
1 15-ounce can white beans, rinsed
1/4 teaspoon freshly ground pepper
1 tsp Italian seasoning
1/2 cup shredded mozzarella cheese
1/4 cup finely shredded Parmesan cheese

Heat 1 tablespoon oil in a large nonstick skillet over medium heat. Add gnocchi and cook, stirring often, until plumped and starting to brown (5 to 7 minutes).
Transfer to a bowl.
Add the remaining 1 teaspoon oil and onion to the pan and cook, stirring, over medium heat for 2 minutes.
Stir in garlic and water.
Cover and cook until the onion is soft (careful not to burn it).
Add greens and cook, stirring, until starting to wilt, (1 to 2 minutes).
Stir in tomatoes, beans, Italian seasoning and pepper and bring to a simmer.
Stir in the gnocchi and sprinkle with mozzarella and Parmesan.
Cover and cook until the cheese is melted and the sauce is bubbling (about 3 minutes).

Depending on the type of gnocchi you use, the carb count will widely vary. Below are carb counts from just the 16 oz of gnocchi.

Goat cheese gnocchi: 12g carbs

Ricotta cheese gnocchi: 108g carbs

Gluten free gnocchi: 116g carbs

Pumpkin gnocchi: 136g carbs

Potato gnocchi: 10g carbs

Marriage to a Terminal

Normally this is where I post recipes for CFers and their families to find high calorie meals. Today I want to post something more personal.

Soon my partner and I will celebrate five years of marriage (nine years as a couple). This does not make me an expert, but I think I have something to offer those in relationships with CFers thinking about becoming more serious. When we were engaged I sought out any information I could find for what to expect once we were married and frankly, there wasn’t a lot out there other than doom and gloom. So I thought I’d offer up an inside scoop with my top 5 things you can likely expect when you marry a CFer that are also true, but not all doom and gloom.

#1 You know each other, inside and out

The things that take senior citizens fifty years of marriage to do without blushing will cross your threshold in the first six months. You will truly know what it means to intimately know your mate. You will be the second opinion on if today’s phlegm is the usual color, or darker than yesterday. You will argue with him about how much blood is too much before you call the pulmonary specialist. You will discuss and compare details of your bowel movements to see if something is out of whack or if dinner last night upset both of you. You will know the smell his body makes when his sugars are too high. You will hook up his IV medications in the middle of the night so he can sleep. You will visually check places he cannot see. You will rub creams on places he cannot reach. You will have no qualms about asking him to do the same for you. After a while you will lose the need to do things like close the bathroom door.

As strange and unromantic as it may sound, it’s actually kind of nice. Partly because you are completely comfortable with each other (farting contest anyone?) and partly because you benefit from CFers being medical geniuses. Just as I take care of him, he takes care of me. My spouse knows where to rub my stomach one direction to ease nausea, and another location and direction for gas pains. He knows how to mix a [what he calls] “cocktail” of pain pills to target the specific headache I may have. He knows my body so well he can predict my menstrual cycle better than I can.

#2 No one will get your humor

There’s a fine line between humor and tragedy. CFers don’t walk that line well. Things that are hilarious to them are wildly inappropriate for the average person to laugh at. There’s a different outlook from a group of people who defied death as kids while watching friends around them succumb to their mutual disease. I think in many ways humor becomes a coping mechanism. To many CFers death is funny. Illness is funny. The conversation about what to do with their remains is worthy of a joke. If you let them write their eulogy, one quarter of it might be sentimental and the rest will be a standup comedy act. My partner and I have even joked about what his last words and last gesture will be. It is hard for them to take life too seriously because they are already inundated with tragedy in their daily lives. Humor eases the difficult conversations. Humor eases the difficult decisions. Humor about macabre things will work its way into both of your daily lives. Hospital stays are called “vacations” and “tune ups,” Pneumonia is labeled “a tough cold” and death is a betting matter. People outside of your marriage will not understand this. You will have to learn to reign it in at social functions and cue your CFer when it is inappropriate to joke about illness, death, and tragedy.

#3 Smokers will dictate your social life

Smoking is not a matter of personal choice, it’s a public health concern. When you’re with someone with limited lung function the locations of smokers will trump your social plans. As a couple we can’t go to bars because of the smokers. We can’t enjoy a meal Al Fresco because smoking is allowed on the patio. We can’t enjoy a stroll through a historic downtown square because there are smokers on every corner. A nice picnic in the park? Think again, smokers are there. Headed to a great outdoor concert? Nope. Smokers there too. Look a new coffee shop! Nope, a sea of smokers has created a cloud of filth for anyone within 15 feet of the door. Let’s play with our dogs in the front yard? Nope, five of our smoking neighbors are outside, lit up, and headed over to say hi. How about a ride on a beautiful day with the windows down and the radio up? Nope, every stop light has a smoker enjoying the nice day with their windows down too. It’s a serious thing. I’ve even ended friendships because they married a smoker. For some reason smokers think it’s fine as long as they are downwind of you, or if they just step outside for a minute. I can’t tell you how many people have tried to hide it from us as if we wouldn’t figure it out the second we stepped inside their place. Smokers: you’re not fooling anyone with the cologne, breath mints and incense. You stink even after you put it out and you bring all of those pollutants inside to the rest of us on your clothes and hair. It’s everyone’s air to share. Kick the habit already!

#4 Fights are fast

Believe it or not, it’s not hard to fight with a sick person. Even terminal people do some really dumb and insensitive stuff. But its hard to fight for long. Because you know the time you have together is limited. We still fight plenty. We fight about our families and our pillow space and how far over the car should be parked in the garage and how the roll of toilet paper should be installed. But the fight is usually over the same day it starts. Sometimes within minutes. There are so many opportunities in a day to take care of each other when you live with a CFer. Fights blow in and out like clouds. It doesn’t matter how mad I am at him, I will remind him to take his dinner pills. It shows him I care. Boom. The fight is over. It doesn’t matter how mad he is at me, he will make dinner when I work late (not eating is not an option for a CFer). It shows me he cares. Boom. The fight is over.

#5 Healthcare is paramount in any big decision

Aside of the two kinds of medical insurance we have (which frequently argue about which one should pay), we also pay a premium for hospital insurance. To not have these would cost us thousands (no, seriously tens of thousands) of dollars a month in prescriptions alone. This is not to say everything works peachy keen without weekly calls to insurance companies and doctors to get referrals, prescriptions, argue about procedures, and process orders on time. My partner (who spends a few hours a week making these calls) has every number on our medical insurance cards committed to memory.

Where most people are putting aside money for retirement, we are putting aside money for medical costs later in life when we expect insurance coverage to be worse and procedures to cost more. When looking for a new job my first criteria are if the benefits include medical insurance, if the benefits includes the spouse, at what percentage and how long is the probationary period? Even a probationary period of three months can put us in a bind if he gets sick.

Before I married a CFer I went to the doctor begrudgingly maybe once a year. Now I’m on a first name basis with many in my spouse’s medical care team and see them quarterly. Where healthcare may have been an afterthought before, it is a priority now. And it is one of the first hurdles to clear in any lifestyle changing decision.

For those out there in committed relationships with CFers, does my list apply to you too? Anything you’d like to add?

Sweet Spare Ribs

This recipe is part of a regimen I feed to add weight to my CF DDF508, GERD, CFRD 35 y/o husband.


6 country style pork ribs
2 cups onion slices
2 garlic cloves, minced
1/4 cup BBQ sauce
2 Tbsp. Worcestershire sauce
1 tsp. dry mustard
3 Tbsp. brown sugar
1 Tbsp. olive oil
1/2 cup vinegar
1/2 cup water
1/4 cup hot sauce (optional)
2 Tbsp. lemon juice
1 1/2 tsp. salt


Preheat oven to 450 degrees F.
Grease a shallow baking pan, place ribs inside and bake for 30 minutes.
Meanwhile, in a large saucepan, cook onion and garlic in oil until soft.
Pour all other ingredients except ribs into skillet and simmer for 10 minutes.
Reduce oven temperature to 350 degrees F.
Brush ribs with sauce from skillet, and pour remaining sauce into pan.
Bake uncovered at 350 degrees F for 1 hour, basting every 20 minutes

Approx carbs in entire dish: 70g

Hawaiian Chicken Stir-Fry


16 oz broccoli florets
2 cups diced, cooked chicken
15 oz can pineapple chunks, in juice (juice will be separated out for sauce)
1/4 c. water
2 tbsp. vinegar
2 tbsp. soy sauce
1 tbsp. cornstarch
1/2 tsp. onion powder
1/4 tsp. ginger
1/4 to 1/2 tsp. crushed red pepper
1 tbsp. olive oil
2 packages Ramen noodles


For sauce stir together pineapple juice, water, vinegar, soy sauce, cornstarch, onion, powder and red pepper and ginger. Set aside.
Preheat wok or skillet, add oil, stir fry broccoli for 2 minutes. Remove.
Add chicken 1 to 2 minutes and push from center of wok.
Stir sauce, add to center of wok. Cook and stir until thick and bubbly. Cook and stir 2 minutes more.
Add broccoli; mix.
Stir in pineapple.
Cover and cook 1 minute.
Serve over Ramen noodles.

Serves 2

Approx total carbs in one serving: 80g