My Meds

The primary ways in which CF affects me is my lungs, sinuses, and stomach (I have acid reflux). I am lucky because I do not need to take digestive enzymes before I eat. Here is a list of all of the medications I take.

Morning:

• Rinse my sinuses with saline (to wash out junk)
• Veramyst Nasal Spray (for my sinuses)
• Allegra (for my allergies)
• Albuterol (to open up my airways; I take this either via inhaler or nebulizer, depending on how wheezy I am feeling) 5-10 minutes
• Hypertonic Saline via nebulizer (to thin the mucus in my lungs) 10-20 minutes
• The vest (to shake the mucus out of my lungs; I can do this at the same time as albuterol and hypertonic saline) 20 minutes
• Advair (to keep my airways open)
• Colistin via nebulizer (an antibiotic taken every other month to kill the bacteria in my lungs) 10-20 minutes

Total Time = 25-50 minutes

Evening:

• Veramyst
• Albuterol via nebulizer
• Hypertonic Saline
• The Vest
• Advair
• Colistin every other month
• Pre-natal vitamins (my dr has all his CF patients take these, not just me)
• Mucinex (to keep my mucus thin)
• Zegrid (for my acid reflux)

Total Time = 30-50 minutes

So, as you can see, I spend 1-1.5 hours a day on my treatments. Add to that a full-time job, exercising 5 hours a week (also critical in CF care), a marriage, and a social life, and you may be able to see why time management is a big issue for me. I really don’t know how CF patients who have even more treatments do it!

Clinic Appointment

Greg came with me to clinic on Thursday. I am very glad he did because we got some great information, and they said, “You have to help her” to Greg multiple times J. Here’s a summary of the appointment:

• My lung function is still great (FEV1 of 111%).
• I need to do more to get myself above my baseline lung function, which means adding chest physical therapy to my daily routine, and there is no slacking off allowed.
• I can vest while pregnant. In fact, the vest may even rock the baby to sleep!
• I have to start getting more calcium because people with CF are at risk for osteoporosis (they don’t 100% know why this is the case). I need to be extra rigorous about this because, otherwise, the baby will take the calcium from my bones.
• We need to think about what life will be like after the baby is born - i.e., how will I get all of my treatments in if I am caring for a newborn? This was one of the times they said, “You have to help her” to Greg.
• I must allow my body to gain extra weight during pregnancy, just in case I get sick along the way. They said anywhere from 25-40 pounds. (Luckily, I am currently at a healthy weight of 125 lbs at 5’5”. If I were underweight like many CF patients, I would need to gain even more.)

I left the appointment feeling encouraged, but also frustrated that I have to add even more steps to my daily routine. I know I should be grateful for my health, and I am (very, very much so!), but I was already stressed about fitting everything in before they added more stuff! I will talk about that more next time.

What We Have Done So Far

Tomorrow Greg is coming to my CF clinic appointment. For those who don’t know, clinic is where I go to make my health is still a-ok. In addition to my CF doctor, I see a nutritionist, a social worker, and a respiratory therapist. I do pulmonary function tests (PFTs) to make sure my lungs are well, and I have a sputum culture taken to see if there are any nasty bugs growing in my lungs.

I go to clinic every three months, so this will be my last appointment before we start trying to get pregnant. I talked to my doctor, Dr. Smith, about getting pregnant at my last appointment, and he told me that I am so healthy I shouldn’t expect anything other than a normal pregnancy. This made me very happy because I was concerned that, while I was pregnant I would get infections more easily and not be able to take medications to treat infections. He also confirmed what I already knew about CF and fertility: men with CF are sterile, but women with CF are not. One thing I didn’t know was women with CF may be “hyperfertile” because, like everywhere else, our mucus, ahem, “down there,” (otherwise known as cervical mucus), is much thicker than normal. This may cause my husbands “boys” to become trapped inside me, (once they make it past a mucus plug), thus giving them a longer time to hang out and wait for the egg.

I also asked Dr. Smith what I must to do to prepare myself for pregnancy. In addition to the usual regimen of taking prenatal vitamins and staying in shape, he asked me to see my OB/GYN as soon as possible. Dr. Smith admitted that he is a pediatrician, and therefore doesn’t know enough about these adult things. He thought my OB/GYN may be able to tell me things he knew nothing about.

Onto my OB/GYN I went. We spent the entire appointment sitting in her office and talking. She told me that she expects it will be difficult for me to get pregnant, because my cervical mucus is so thick that my husband’s boys will have a hard time navigating it. She said I should come see her if we are not pregnant after six months of trying. This was pretty much the opposite of what Dr. Smith told me. I left that appointment feeling depressed.

I am really looking forward to tomorrow’s appointment because Dr. Smith will be discussing the results of Greg’s genetic test. Greg was tested for 97 variations of the CF gene several months ago, and the results were negative. This was great news, however this does not completely rule out the possibility that Greg is a carrier of the CF gene. There are hundreds of variations, and many more that have not been identified, but a few are the most common and Greg was tested for those. Tomorrow we will learn the odds of us having a child with CF, and if Greg needs additional testing.

My next post will be the outcome of tomorrow’s appointment!

First Post!

I can't believe I am doing a blog. It's not something I ever thought I would do. However, when my husband and I decided that we would start trying to get pregnant soon, I could not find much information on the internet about real women with CF having babies, and I thought sharing our journey could be helpful to other women with CF who are thinking of having children. I don’t know how good of a blogger I will be – I may not update this multiple times a week. But I will try to share any useful information as I learn along the way.

I think the best way to start is to share some information about myself, so here it is. I am 31, and I’ve known I have CF since I was a baby. My parents had me tested for CF because I “tasted salty” and, much to the doctors’ surprise, I tested positive. I have been blessed with great health for most of my life. I have only been hospitalized twice, and my PFTs are still over 100%. I am also pancreatic sufficient. I do suffer from bronchitis and pneumonia several times a year, and I’ve had my share of sinus problems, but, overall, I am so lucky. In case you are wondering, my genotype is R117H/DF508.

My husband, Greg, and I were married last summer and are very excited to start a family. However, we are not trying just yet. We would like me to be a Stay at Home Mom, so we are waiting until we have our financial ducks-in-a-row. We plan to start in just over two months. In the meantime, I am doing everything I can to get myself as healthy as possible.

That’s it for the first post! I will continue to share more about myself in the next few posts!