Thursday, January 14, 2016

Toddler Talk Part 7

Me: "Can boys do anything that girls can't do?
K: "Pee with a penis."

We live in a bilingual home where my husband speaks only Spanish and I speak only English to Kaylee. Sometimes having different languages directed towards different people can create some funny or confusing moments.

K: Are you going to your friend's house daddy?
Husband (In Spanish): Mande? (what?)
K: Oh, you are going there on Monday.

My husband I were simultaneously tickling K
K: Mas, Daddy! More Mommy! Mas, Daddy!

I watch my nephew (R)  two days a week and the things a 2 1/2 year old and a 3 1/2 year old say to one another keeps me laughing all day long.

Kaylee and a little girl chatting at the park
K pointing to R: "That is my boy. His name is R and he is my cousin."
Girl: "He isn't your brother?"
K: "Well, not usually."


K looks up to see R with both hands swirling her kiwi slices in her snack bowl
K: "Those are mine."
R: "What?"
K: "The kiwi. That bowl is mine."
R: "Oh! I just dancing them."
K: "Oh, okay."

The kids watched the garbage truck and after it drove by..
K: "I want to see him again"
Me: "He already did our garbage now he is going to another neighborhood. We can see him next week."
Neither kid budged from their spot.
Me: "Let's go in."
R: "He come back. We need be patient"

Tuesday, January 5, 2016

Four Months Of Orkambi

*This is a long version of my experience on Orkambi*

This is a post I keep starting, but can't seem to get myself to post. To be honest, my Orkambi journey started torturous at best. As much as I wanted to gripe and complain about the sickness that came with this new pill I knew I was unbelievably lucky to have the ability to even try this medicine. I know how many people with CF would love the opportunity to try this medication that was already in my hands and that none of them wanted to hear me complain. At the same time, when I felt as sick as I did, it was hard to feel grateful and lucky for a medicine that I wasn't sure could ever help me. And so I kept quiet and internally kept note of the changes, both good and bad, going on in my body. I am just finishing four months of Orkambi now and feel I have a clearer picture of what Orkambi means for me and my disease.

I started Orkambi feeling cautiously optimistic and well aware that the first month or so would be tough. However, I had no idea what the next several months had in store for me. My symptoms were not that unusual and many people that start the medicine experienced the same side effects. Some of the symptoms that came with this little pink pill were shortness of breath that was so extreme I had trouble walking across the room. In fact, there were times I felt like I was suffocating while sitting still. I had fevers, chills, aches, streaking of blood in my mucus, a cough that never stopped, but didn't feel productive, which meant I hardly slept. I was mentally prepared for these side effects, but I wasn't prepared for how long they would last. I was warned by fellow Orkambi users that the first week is the hardest and the worst of it was usually gone by a month. My symptoms seemed to get worse as time went on and as I passed the first and second month with no relief I started to lose hope that this medicine could work for me.

The physical aspect was tough, but the mentl aspect felt torturous. I dropped from 40% lung function (fev1) to 29% and despite the passing weeks, my numbers wouldn't budge. I worried endlessly as the weeks passed that I was harming myself rather than helping. I was so tight, coughing was not super productive and my exercise consisted of walking in slow motion around the house. I worried that my lack of movement and my tight chest meant that more mucus was pooling in my lungs and that I would end this Orkambi journey worse off than when I began. I felt like I was sicker than I had ever been in my life and I knew I was doing it to myself by taking this medicine. I kept waiting for a sign that things may improve, that my body was still okay, but nothing seemed any better.

And then one evening 2.5 months of worsening symptoms I broke down and cried. I had decided in the morning I would call the doctor and tell him that I just couldn't do it any longer. Physically, I was tired and mentally, I was terrified I was hurting myself and I don't have a lot of lung function to spare (at 29%). Orkambi didn't seem to be the right medication for me.

That night I went to bed and slept through the night for the first time in months. I didn't cough once! The next morning my shortness of breath had miraculously disappeared! It was a complete transformation. It was all I needed to stay on course and continue using Orkambi. My next clinic visit my lung function was 35% and although that was better than the previous 29%, it still wasn't to baseline. Regardless, I felt a huge sense of relief that at least my lungs were moving in the right direction.

During the fourth month I suddenly starting seeing all the changes in my body I had secretly hoped would happen. I started coughing up old putrid plugs from my lungs. Sinus plugs were coming out at an alarming (yet wonderful) rate. I could climb stairs and even run in the yard with my daughter. My mucus lightened in color and was much less copious compared to years past. My mucus came up easily and I started to feel stronger and more sure of my body. my morning cough has completely disappeared. I am starting to see my weight steadily climb despite starting at a healthy weight to begin with.

Clinically, I am not sure my lungs seem much different, but I feel like I just came off a round of IVs. I am in extreme awe of how well I feel without needing a hospital stay and weeks of IVs. I feel amazing and I have done nothing to work for it, no oral meds, no hospital stay, no prednisone, this hardly feels like real life. Orkambi may be considered a marginally beneficial medication and yet I feel stronger, healthier, and my lungs feel clearer. I may not gain lung function or see a huge change clinically, but I am taking something that is making my body work more like it should and that is amazing!

Saturday, December 12, 2015

3 and a half

Dear Kay,

You are 3 1/2 today! It feels very strange saying that because after you accepted the fact that your birthday meant you were no longer two (which you resisted for a few weeks) you decided to skip year 3 and go right to 4. I assumed it was a phase, but here we are 5.5 months later I still have to tell people you are 4...in June...or else face the wrath of a child who has been called the "wrong" age. It feels like I am going back in time saying you are only 3 1/2.

Your age is not the only little fib you tell (and believe with your whole heart) these days, you also are convinced that you are fluent in French (on top of English and Spanish). Now, to be fair you know more French than I do, but counting to ten and saying a few catch phrases hardly counts as fluent. The other day I overheard you telling your 2.5 year old cousin (who has never said a word of French in his life), "J, I haven't heard you speak French in a while can you say, bon bon?"

Speaking of language, your understanding of Spanish had exploded these last 6 months. Unfortunately, I can't take any credit for this development. Your father speaks to you only in Spanish. Actually he does it so well that the other day when he slipped and said something to you in English you shouted, "But dad, you don't know English!" I guess you never noticed that your dad and I only speak English to one another.

My favorite part of the day is when you first wake up. You shout from your bed every morning between 7 and 7:30, "Is it morning?" When I shout that it is in fact morning, you crawl into our bed to snuggle before we start our day. You have always been a snuggler and that has not changed. You love showing affection and I call you my little romantic because you come up with the most sappy lines to show your affection and I love it!

Like all kids you age you have an active imagination and love to tell stories. You like to talk about when you were a grown up and I was little you took care of me. You also love to talk about made up memories from when you were a "tiny baby". The other day you told your Nana that you had a pet squirrel when you were younger and you went into great detail about this supposed pet from what it ate to where it slept. You also apparently had a run in with an alligator when you were a baby which doesn't say much for my parenting skills.

I am always saying every age is my favorite, but I absolutely love the relationship you and I have developed and continue to develop as you get older. You feel like my partner in crime and I feel thankful every single day that I was lucky enough to be your mom.

Love,
Mom

Saturday, September 19, 2015

Rain Showers and Orkambi

"Maybe we will see a rainbow" my 3 year old shouted with eyes wide at the thought.
My dad and I exchanged glances. While smirking I replied, "Maybe" not believing it one bit.
It hardly rains in the summer. We haven't seen rain in four months or so, but a recent drizzly day made its way into the end of summer leaving my daughter with the optimistic belief that she would see a rainbow. It was true the rain had stopped, but the sun was still hiding behind dense clouds and evening was quickly approaching. My daughter had a date with my father that evening and I was hoping the excitement from the date would be enough to make her forget the disappointment of a rainbow that would never be.

I started Orkambi today. The day I found out that Orkambi was approved, my application was accepted, and the pharmacy was preparing to ship my pills to me I was filled with a multitude of conflicting emotions. I am an optimist by nature, but a realist by life experience. My heart kept shouting, "This is a dream come true! Your body will be functioning more normally than it ever has before. This is what you have been waiting for" But my brain kept shouting just as loudly, "But the benefits are minimal and your lung function is already so low." My heart would rebuttal, "Improvement is not the point, stability is what matters. " The fight between my jubilant heart and my practical cautious mind roared on throughout the week

This morning I woke excited and anxious for the postal carrier. And once the meds were in my hand around 9am I suddenly felt nervous in a way I haven't felt in so long. This was the moment of truth. All the potential benefits and all the potential let downs were running through my mind. I wanted so badly to let my optimism take over my cautious mind, but I was equally concerned about being disappointed. I sat there for a long time before swallowing my first pill. It is okay to hope for the best, I kept telling myself. It is okay to be optimistic for once and believe that good things, no matter how small, are coming your way.

I could hear the giggles and high pitched voice of my little girl running up the front steps. I greeted both her and my dad at the door with a big smile. Before I could even ask how the date night went my daughter, hardly able to contain her excitement shouted, "We saw a rainbow, mommy! We really did!"

Sunday, September 13, 2015

Forgetting Enzymes

CFers take enzymes every time food passes our lips. It is so engrained in our daily routine that we often take them without much thought. It is so routine that there are times we may wonder, "wait, did I take my enzymes already?" It happens to me more often now that I have a little one that distracts me during meal time, but let's be real, it happened often enough before kids. Recently, my dad found the perfect solution to this annoying problem at the local pharmacy.

 

His great find is a lid that goes on regular prescription bottles (the translucent orange kind) and keeps track of how much time has passed since the bottle was opened. So let's say you ate breakfast at 7am and you sit down for a snack at 10am, but you can't remember if you already took your enzymes. You can take a peak at the lid and if the bottle says 3 hours have passed since you opened it you know you better get swallowing. And if it says 1 minute, you are good (as long as you didn't accidentally place your napkin on top of your pile of enzymes by accident not that I have ever done this ehem).

The downside is they don't fit on the plastic Zenpep bottles, but if you have CF you probably have a few extra orange bottles on hand (or will once your next shipment of prescriptions arrives) that you can use. These were clearly from Rite Aid, but I assume most major pharmacies have the exact same product. Hopefully this will answer the age old CF question of, "Wait, did anyone see me take my enzymes?"

Wednesday, August 19, 2015

A Green Tomorrow

To plant a garden is to believe in tomorrow.
- Audrey Hepburn

I started my first vegetable garden 5 years ago, in pots that were much too small, outside my apartment's front door in a much too shady and busy walkway. Throughout the day I would move the pots holding my precious tomatoes and peppers to wherever the sun was filtering through the branches of an overgrown pine tree. It was the worst conceivable place to grow a garden, but that little potted garden that gave me a total of three tomatoes and two stunted bell peppers ignited a passion in my soul that I have never been able to shake.

Today, the first sight you will lay your eyes on when you go in my backyard is a large display of raised beds that is home to everything from asparagus to watermelon. My mornings and evening are spent tending to my garden and my kitchen is home to the rewards these plants offer in return for my care and attention. It seems at times my daughter was raised in the vegetable garden and the age of two can rattle off facts about garlic scapes, and the importance of ladybugs, and that melons have both female and male flowers.

I always found it funny that so much of my life is avoiding thoughts of the future, a future that statistics and doctors assure me won't be long. Yet, I spend my time on a hobby, a passion, an obsession that is all about forward thinking and constant planning for the future. The seeds I so careful sow in the ground in spring comes with so much hope of thick green vines heavy with melons that will fill my stomach and soul mid summer. I cover my strawberries today to protect them from tomorrows ravishing birds. Asparagus is planted with the hope that in three years time I will have spears to grace my dinner table.

If you ask me about the future of my garden you will grow bored and restless long before I stop chattering on about expansion, and artichokes, and aphids. If you ask my about my future, the future of my health and my life you will be greeted with a harrowing silence. You will see an empty vastness in my eyes so foreign in a woman still in the prime of her adulthood.

The garden is a way to plan for the future and put my hopes and dreams into a tomorrow that feels so uncertain. Somehow it feels safer to make plans for my plants than for myself. If I never get the harvest of cherries it is much less heartbreaking than never seeing my daughter get ready for prom.

So for now I carefully tuck seeds into the soil so that in two months from now the ground will be stuffed with plump carrots. And as for what lies in the future? The carrots are all I can be certain of.

Wednesday, August 12, 2015

Clean and Care Free

You know that feeling you get after filling your gas tank? The relief when that little voice that keeps nagging, "your tank is getting low" and "don't forget you need to fill your tank soon" is finally quiet? That voice that you didn't even consciously know was bothering you until it is finally gone?

Yeah, I get that same freeing relief times ten when I know all of my nebs are clean and dry!