CF stands for Cystic Fibrosis which is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
- clogs the lungs and leads to life-threatening lung infections; and
- obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
Symptoms of Cystic Fibrosis
People with CF can have a variety of symptoms, including:
- very salty-tasting skin;
- persistent coughing, at times with phlegm;
- frequent lung infections;
- wheezing or shortness of breath;
- poor growth/weight gain in spite of a good appetite; and
- frequent greasy, bulky stools or difficulty in bowel movements.
Becoming a parent to a child with special needs is not an easy situation or one that any parent wants to face, but the fact of the matter is that it happens, and some parents accept it and do their utmost to turn a difficult situation into a positive one. One such parent is a friend of mine and fellow lactivist who runs a blog entitled A day in the life of a CF mom. Over the months of reading her blog posts and talking to her, I've come to see what a truly dedicated and well-informed mother she is. Happily she accepted an interview to provide more insight into what it's truly like to deal with Cystic Fibrosis.
Wendy : Kacie, tell us a bit more of what CF truly encompasses
Kacie : Cystic Fibrosis (CF) is a genetic disease that is inherited from a person's parents. Lucy has CF because her father and I are both silent carriers of the disease. If Lucy's father and I had more children, there would be a 25% chance of each child being born with CF. CF is often mistaken as a lung disease but really it is a genetic disorder that effects the cells in the body so that they don't move sodium chloride in and out normally. Because of the sodium chloride abnormality, it causes the mucus membranes in the body to be thick and sticky. The lungs and pancreas are the hallow organs most effected by the mucus. The thick lining inside the lungs makes it easier and more susceptible to lung infections causing lung damage over time. The sticky mucus inside the pancreas makes it almost impossible to digest food properly without the use of digestive enzyme supplements that have to be taken with every meal to ensure proper digestion, and to prevent malabsorption. There are so many advances in the field of CF but the average age of a person with CF today is only 37 years old. There is no cure for CF.
Wendy: At what point was your daughter Lucy diagnosed with CF, did you suspect something was wrong before you got the diagnosis?
Kacie: I had a very normal and happy pregnancy with no signs of anything being abnormal, even having a chemical-free and intervention-free labor and birth. I didn't suspect anything was wrong until my water broke, there was a lot of green meconium. When they placed Lucy directly on my stomach, I noticed first, her huge blue eyes, and then her very distended, very bloated belly. There was so much fluid in her stomach that it was putting pressure on her lungs, and she was wheezing and gasping for air. They took her away to pump her stomach and take x-rays to be sure that her lungs were okay. Her lungs were 100% but they did find multiple blockages in her intestines, called meconium ileus. The meconium, a baby's first poop, was too thick and bulky to pass while in utero. It stayed in her small intestine, and the intestine closed up around it, pinching the intestine shut. When my water broke during labor, and it was green, it was caused by her not being able to pass amniotic fluid any longer. She was instead regurgitating it. Lucy had surgery to repair the intestinal blockages at 18 hours old but Lucy was not officially diagnosed with CF for anther 2 weeks after birth. Only about 10% of infants with CF are born with meconium ileus.
Wendy: What were the first thoughts that ran through your mind at that point?
Kacie: I have never been so scared in my life. In the first 30 seconds of seeing my new baby, she was ripped out of my arms for inspection. I had feelings of guilt in the beginning. I had done everything an expectant mother should have done. What went wrong? What did I do that could have caused this? Just being in the unknown is a horrible feeling. After Lucy was diagnosed, I had already had a chance to relax and compose myself, and feelings of guilt had started to subside and were actually replaced by anger. Why me? Why my baby? It really wasn't fair. I had so many questions!
Wendy: Since you were exclusively breastfeeding Lucy, did the diagnosis affect this? What did your doctors recommend and what did you do?
Kacie: I had always known that I was going to exclusively breastfeed my children so the decision was made before Lucy was even born. When Lucy arrived, and because she had the blockages, I was not able to nurse her for 2 whole weeks. Imagine my frustration and heart ache to not be able to nurse my child when she cried for me! She never ingested a single thing until 2 weeks old, waiting for her insides to heal up. She was given fluids and nutrients through an IV while I pumped, pumped and pumped, and saved every last drop of milk I could get. I made the NICU nurses write in huge, red lettering, "MOTHER'S BREAST MILK ONLY!" in her file so she would never be given formula. The very first thing that went into Lucy's repaired intestines was exactly 5ml of my milk. She was not to be given more than 5-10ml at a time to insure that the surgery went well. After 2 days of "testing" her new tummy out, we were finally, at last, able to nurse. I will never forget the first time either! She latched on like she had done it before. We were off and running. The doctors were very pleased that I was so adamant about breastfeeding. The recommendations about switching to formula came later. Lucy was not gaining as much as she should have been gaining according to the AAP weight charts. When she dipped below 50th percentile, it was mentioned that we may have to supplement with formula for added calories. I told them that giving her formula would never happen in a million years! I went home that day and started nursing her around the clock like normal, but I added in feedings as well, even when she really didn't even have hunger cues. I became a human pacifier because I refused to put her on formula. Extra nursing worked. She gained enough weight to get above the 50th percentile so Lucy's nutritionist never mentioned formula to me again.
Wendy: How has breastfeeding proved to be a great help in relation to Lucy's diagnosis and her illness in general?
Kacie: I make jokes that my boob has been my best friend and still is! Lucy has been in and out of doctors offices her whole life. Her life is very chaotic and stressful because of CF. More stress than a child should have to go through: blood tests, needles, throat cultures, wearing a vest that shakes her chest and taking up to 9 different medications a day. By nursing Lucy on demand for the last 2 years, we have created a safe place for her to go where she knows everything will be okay. Nursing is her neutral place, a safe place, the quiet place among the storm. I am more in tune with her and her physical and emotional needs because of our nursing relationship. Nursing her for this long has helped her immune system. She is sick less often and when she does get sick it is very short-lived. If I ever get a cold or illness, she has never gotten it from me. She is receiving the antibodies from me that are needed in fighting the infection. I really cannot think of one negative thing to say about breastfeeding Lucy.
Wendy: Could you describe what a normal day is like for you and Lucy?
Kacie: Lucy and I are both night owls so we leisurely get up anywhere between 9 and 10 am, earlier if we need to be somewhere. She nurses when she wakes up. Since I am Lucy's very own short order chef, I ask her what she wants to eat for breakfast. I oblige by making whatever she says (but make it as high calorie as I can.) Her pancreas doesn't work for digestion so I have to help out matters by increasing the chances that she will absorb some fats and she takes digestive enzymes in pill form (7 per meal) to break down food. At breakfast time, she also takes a vitamin designed for CF kids and a probiotic to aid in intestinal health. After eating her delicious high calorie meal, we begin breathing treatments for her lungs. These breathing treatments take up to an hour, consisting of 3 different inhaled medications given with a nebulizer and a mask while she wears a vest that vibrates and shakes her chest to loosen the mucus in the lungs. After breathing treatments, we get ready for our day. Bathe and shower, get dressed, sing songs, pack a high calorie snack and drink, and then go and do what we need to do before it is time to eat again. Then a high calorie lunch is served.We then do another breathing treatment with her nebulizer and vest. She plays until nap time. She nurses at nap time. Nap time is when I get on the computer and clean up around the house a little bit. When she wakes up from her nap, she likes to nurse again, then I get another snack ready for her. She plays more around the house, playground if it's nice or we run more errands. Dinner is more high calorie food followed by dessert or another snack.We do her final treatment of the day consisting of 3 meds and vest, lasting almost an hour. She plays and nurses off and on all night until bedtime.
Wendy: With new advancements in the field of CF, new medications are lengthening the life span of people diagnosed with CF, how many meds does Lucy take? How did it start out? Is it difficult to get her to take her medications?
Kacie: I have been so lucky from day one. Lucy takes her medications like a professional and has rarely complained. I really try to make it fun. Make it a game for her instead of a hassle or 'bad thing.' As a newborn nursling, I had to open the digestive enzymes into a tablespoon of applesauce each and every time she breastfed. With practice, Lucy was taking entire pills by 15 months of age. In the beginning, as an infant, I used to do all of her breathing treatments and inhaled medications at night when she was sleeping. I would set my alarm, give her her meds and she would never know she did it. Now, at 2 years old, she holds the mask herself, and nearly does it on her own. Here is a list of her 8 meds:
- Miralax (laxative) half cap daily in juice to prevent rectal prolapse from recurring.
- PancreaCarb MS4 (digestive enzymes) 7 pills before ingesting any food.
- ADEKs (chewable vitamin) specially formulated for CF kids with fat soluble vitamins A, D, E and K that she takes once a day.
- Probiotic to help aid in her intestinal health that she takes twice daily (same as yogurt probiotic but higher dose.)
- Xopenex (inhaled levalbuterol) opens up lungs. 2-4 times daily depending on wheezing and cough.
- Pulmozyme (inhaled enzyme) loosens up mucus in lungs to aid in expelling it. Once a day.
Tobi (inhaled antibiotic) fights a Pseudomonas bacterial infection in the lungs. Twice a day for an on and off duration of 6 months.
- Cipro (oral liquid antibiotic) fights bacterial infection. Twice daily for 2 weeks.
Wendy: What does your daughter's 'diet' consist of?
Kacie: Lucy is on a very high calorie diet. Her CF nutritionist recommends that she have an average intake of 1700 calories every day. She is only 2 years old so it is very difficult to pack that many calories into a little body, and little food. I take normal toddler table food and add calories to it all. Add butter, olive oil and mayonnaise to everything. She does drink whole cow's milk at meals for the calories and extra vitamin D. To her regular milk, I add half and half to it! Her veggies are usually sauteed in butter or oil and then served with a mayonnaise dipping sauce. A lot of salt is added to her food to make up for what the cells in her body got rid of. She eats always eats quality, homemade food. Never candy, soda or other empty calories. She eats when she wants to. She has awaken in the middle of the night and was hungry so I made her an entire meal. A CF mama will never refuse food to her child!
Wendy: Is Lucy still breastfeeding? Has this proved a hindrance or a help?
Kacie: Lucy and I just hit our 2 year milestone in our breastfeeding journey, and we don't have any plans on weaning in the near future. I could never say anything negative about nursing Lucy. Breastfeeding has helped me, and her CF team of doctors, keep her happy and healthy. She is well-round, smart, caring and a very confident little girl.
Wendy: What difficulties have you experienced with either, relatives, friends, or doctors, due to Lucy's illness?
Kacie: My family has been supportive through the entire thing. I have never gotten any negative comments from my friends either, more questions and curiosities about nursing. Lucy's pumlonologist has made a few funny comments to me like, "Oh my gosh! You are still nursing her?" I find it so disturbing that doctors today can't find the benefits of nursing a child long term, and then question a mother's choice to do so. I just reply with things like, "Yes, we are and she is thriving because of it."
Wendy: What joys and hopes do you have for Lucy and the future?
Kacie: I have the same hopes and dreams for her now as I did when I found out I was pregnant. I want her to be independent, self-confident, nurturing and kind, show compassion to others less fortunate than ourselves, be a citizen of the world. She can and will do anything she puts her mind and heart into. She already shows that she is smart, loving, kind, compassionate and she is only 2! I cherish every moment that I have with her, and I feel like she knows how I feel and reciprocates those feelings back to me. I want Lucy to be happy with herself and never feel sorry for herself just because she has Cystic Fibrosis. I want Lucy to live the best life she can.
Wendy: If you could reach all parents who just got a positive diagnosis for CF of their baby/child, what would you say to them?
Kacie: I would tell them that there is only a short period of time to grieve and be angry. Spend the rest of your life educating yourself about CF so that you may become a better parent and caregiver. Knowledge is power. Find support groups or other CF families that are going through the same things as you. The worldwide CF community is friendly and will become your go-to when you need answers. Spend everyday loving and nurturing your child. Every moment counts in life, every breath counts. Fight for your child and their right to good health and happiness. Only you can be your child's voice, an advocate. Speak up and take a stand, if you think something is wrong. A parent's instinct DOES count and should be listened to. Love, kiss and adore your child and treat them like you would any other. They are normal children who just happen to be diagnosed with Cystic Fibrosis.
Kacie, with heartfelt gratitude, I would like to thank you for letting us see what it's truly like to deal with Cystic Fibrosis on a day to day basis. It's not easy, but you are a sterling example of a loving, nurturing parent. I have learned a lot from your example