Just Breathe

Sometimes I can get caught up in my own illness and how it affects my life. When I'm inclined to throw myself a pity party, I think about my nephew, Colby, who is just 9 and is fighting Cystic Fibrosis. I asked my sister-in-law, Hollie, to write this guest post, because I knew she could convey the daily effects of CF to you much better than I can.

“Have you taken your meds today? It’s time to do your smoke medicine. Do you want the vest or manual chest PT? I think you should wear a mask today at the store. How many times have you pooped today? Make sure we don’t forget your meds for school. How about your puffer? Did we do that today? Please bring me your milk bag and extension tube........”

And so begins another day. These are the conversations between mom and son that have essentially become co-workers, fighting CF. All the strange things that must be considered and monitored. I wonder what it feels like to be a mom that doesn’t have to inspect a 9 year olds poo. Or get to just worry about friends at school or grades. When there’s a sniffle, just to make chicken soup and baby their child, instead of sheer panic. I wonder what that’s like. Because my days are quite different than most mothers. They are filled with medical regimens, physical therapy, inspecting and listening, worrying and fretting a hospital admission and administering countless prescriptions………… sometimes I wish I could just breathe.

In 2002, my first born son came prematurely and in distress. I barely held him before he was rushed to Childrens’ Hospital from the small town hospital he was birthed at in Jacksonville, AL. At two days old he had his first surgery for an intestinal blockage. The doctors told us that there was a long road ahead of us, but not to worry. They were going to take good care of him and I just needed to get some rest for the journey.

It was a month before my tiny little boy started to turn the corner. We were finally moved to a private room and out of the special care unit. I was elated to get to hold him anytime I wanted and put him in his clothes. However, just as we were settled into our room and enjoying progress and each other; his DNA tests came in…..Cystic Fibrosis. I had heard of it, but had no clue what it was.

Cystic Fibrosis (CF) is a terminal, genetic disease that affects many organs of the body. The disease causes the cells of the body to not allow salt to travel into the cells and effectively throughout the body. The lack of salt in his cells means that fluids in his body become thick and sticky. Mucus becomes our enemy and hides away in the lungs, the digestive system and the reproductive system. The mucus plugs ducts that prevent semen from being released; therefore, he will be sterile. The mucus plugs the ducts in the pancreas that release enzymes and break down food; therefore, his body cannot digest food on its own. Mucus will soon inhabit his lungs and the body will work against itself to try and fight it, creating permanent damage and eventually destroying the lungs.

For the first four years, CF was really little more to us than some capsules sprinkled on applesauce before each bottle or meal. These capsules had man-made enzymes that digested his food. Of course, not perfect and he still had trouble with greasy, messy stools but he was gaining weight and thriving. I was encouraged that he had no lung infections and easily fought off colds through those years. Maybe he was the exception! But the doctors assured me, it’s not IF it’s WHEN.

At four, my son was hospitalized for the first time for what we know affectionately call a “tune-up”. It is a period of 10-14 days of breathing treatments, IV medications and chest physiotherapy (or chest PT) that helps loosen mucus from his lungs. He sailed right through it. Encouraged still! We had to be the exception!   

As the years passed on, the “tune-ups” became more frequent; the medicines added to, the at-home regimen lengthened. Getting a cold became harder for him to fight off and almost always meant a hospitalization was imminent. It’s not IF, it’s when.

Now at age 9, this disease has officially taken over our lives and daily thoughts and worries. Lung damage has been confirmed and it’s now a fight to recover what can be and maintain the rest. We have had three “tune-ups” in the last 6 months and now plan on another three for the remainder of the year. Doc says it time to fight. Based on the most recent tests, his lungs are operating at around 60%. The remainder is damaged and unhealthy, but he is optimistic that we may be able to recover some of that. A portion is already permanent and irreversible.

While our fears are growing there is hope in sight. With the help of the CF Foundation, researchers have been relentless in finding a way to move the salt in the bodies of CF’ers more effectively and have by all accounts found a “fix”….for 4% of the CF population. My son is not included in that group. We must still wait and fight. It is hoped that in the next 4-6 years that there may be such a fix for his strain of CF. But, the damage done until then cannot be reversed. What is done, is done.

So, we hold fast. Praying that the scientists, the researchers, the doctors are successful in their quest. I can’t even fathom the possibility and we can’t rest until then. We can’t get complacent. We can’t stop fighting. But, if AND when, I will finally be able to make chicken soup when colds move through our home, I will be able to relax in the evenings and not have to do breathing treatments and chest PTs. I will not have to shook my other two children to grandma’s house while we weather another “tune-up” and not have to schedule our lives around air quality and medical equipment.

I will be able to just breathe.

And my son will be able to eat without pressure, poo without inspections, play without worries of germs, sniffle without fear of a “tune-up”.

But best of all, he will be able to just breathe.

Shaky Mommy's notes: 

Colby is participating in a walk to benefit Cystic Fibrosis on May 19. You can help Colby meet his fundraising goal by donating here or by buying a t-shirt here.

Hollie and her kids have created a great video to educate children and adults on what CF is. Below that video is an incredibly adorable video of Colby's first stay in the hospital that shows how much he missed his brother while he was away...this is a must see! So sweet! 


Please give the "CF: I know what it is, do you?" page a like on Facebook and help us spread the word about Cystic Fibrosis.   

Breast Milk Jaundice

Jaxon is still looking a little yellow, especially in his eyes. We also had several people mention to us over the past few days that they thought he looked yellow. Concerned that his jaundice was lingering,  we called the pediatrician. She told us to come in and have him tested. His bilirubin level was 8.4. The doctor said that it’s caused by breastmilk jaundice. I was concerned that I would have to stop breastfeeding for a couple of days in order to allow the bilirubin to clear out of his system, but the doctor said there was no need for that. She said his levels were not high enough to be dangerous and that it would all clear up in a couple of weeks.

He is gaining weight - he was 9 lbs 14 oz at the doctor’s office.   

We also found out that his newborn screening came back and everything is normal – no CF. That was very good to hear. 

Got to go-the boy wants to eat...again!

Jaundice

After 24 hours in the hospital, Jaxon had still not pooped. He had peed on the nurse when he was first born, but had not peed since that time. While he was nursing wonderfully, and I was sure that he was getting colostrum, I knew how important that first bowel movement was. I started supplementing with some formula from the hospital to try to help him along. Jaxon finally produced a soaking wet pee diaper, but the poop was still not coming. Finally, after more than 36 hours, he produced what is known as a meconium plug. I had never heard of this before.

The doctor told us that in some cases a meconium plug can present when a baby has cystic fibrosis. Since CF is part of the newborn screening, he told us to be sure and ask the pediatrician about it at our first visit. *Update-  we did this when we went for our first bilirubin check and we were told the newborn screen was not back so we will have to wait for the 4 week visit to get this information.*

Jaxon’s jaundice levels were higher than what they like them to be (12.4), most likely because of the meconium plug and delayed pooping. He was placed under the lights at the hospital, complete with baby protective eyewear, which he hated. He cried almost the entire time he was under the lights. I hated it. I got out of bed and sat beside him (not an easy task with my stitches and pain). Jason and I took turns holding his hand and rubbing his head to comfort him. I was so glad when they came in and said that the doctor had decided to just go ahead and release him with a light to take home. 

We were sent home with a “glow bed” and instructions to keep him on it at all times unless we were feeding, changing, or comforting him. Not fun at all! But sooo much better than the lights he had been laying under at the hospital. With the glow bed, he didn’t have to wear the eyewear, and he could be covered up, which seemed to make him feel more secure.

Jaxon on his glow bed

The home health nurse came on Sunday to check his levels – up to 13.4. On Monday, we went to our pediatrician for a bilirubin check. His levels were down to 12.5. On Tuesday we went back for another check, but saw a different pediatrician in the practice who said that since the levels were dropping and he was gaining weight, there was no reason to stick his little heel again. She also told us we could discontinue use of the light! Yay! I am very glad to have this behind us.