Here are some stories from three mothers about what it is really like to have a child with Cystic Fibrosis. When our daughter was diagnosed with CF at five weeks of age, it shocked us completely. We had no family history of Cystic Fibrosis, in fact I really didn't even know what it was. As the paediatrician explained the condition, it's effects and the treatment required, I asked the big question... Is it fatal? I will never forget that feeling when he answered, 'Yes'. My heart felt like it literally broke.
In the weeks that followed we met with a Respiratory Physician, Gastroenterologist, Dietician, Physiotherapist and Social Worker. We watched videos, read books, and sourced information from the internet. We became members of a Cystic Fibrosis Association and learnt all we could about CF-as we now called it. It was a steep learning curve, that's for sure. So as well as caring for our first newborn, we took on the tasks of administering enzymes which were needed to digest breast milk and assist in normal growth, chest physiotherapy twice a day and daily antibiotics as a Staphyloccocus prevention. Every cough, cold and runny nose was full of anxiety and worry about whether it would turn into something more sinister. I remember staring at my sleeping baby girl and thinking she looked so normal. How could anything be wrong with her? And what was happening inside her tiny body?
As our daughter began to grow and develop into her own little person, the CF stuff seemed to consume us less. She became used to daily treatments and she became a sweet, bright, happy little girl who happened to have CF. Our daughter loves school, her friends, gymnastics, polly pocket, dressing up, riding her bike and lollies. CF is just a small part of the beautiful little girl she is! And for that, we are TRULY grateful. She can run and play, laugh and learn and for now, CF doesn't limit her in living and loving life. She continues to have physio twice a day, swallows 20 capsules a day for digestion,needs a high fat, high energy diet, many courses of antibiotics, hospital admissions for more serious infections, blood tests, sputum samples, chest x-rays and lung function tests at CF Clinic-all a very normal part of our families life.
Our child is usually compliant with treatments, food can often be a battle but overall we manage the practical aspects of CF just fine!
As a mother, the emotional aspects are my biggest challenge. I think about the questions my child will ask in regards to her life expectancy, how will I answer them, how will I cope as her health deteriorates, will I be good enough? As a parent, you don't expect to outlive your children, so knowing this, is something I think about from time to time and something I struggle with. Of course nobody knows what the future will bring, new and improved treatments have extended the life expectancy considerably. But for me personally, being realistic about my childs health issues and life expectancy is part of accepting and dealing with CF.
Having a child with CF has really created an awareness in so many other conditions, disorders and special needs. I often reflect on how families manage with their various challenges and am grateful CF is all we have to deal with. I am also so thankful for the efforts of medical staff and charity organisations and the generousity of organ donors and their families ability to give such an amazing gift in their time of grief.
Having a child with CF has made us realise how lucky we are!
Lea, NSW
________________________________________________________________________________________________
Amber loved shopping, boys, clothes, makeup and hanging out with friends, just like other high school girls, but Amber was different in one way-she had Cystic Fibrosis. Even with CF, she didn’t want anyone looking at her differently or pitying her, or worse, avoiding her because of her disease.
Amber required breathing treatments several times a day. When she wasn’t strong enough to use a mechanical device, she had to have someone, usually mom, pound on her lungs to get the mucus out. At home, taking medication was no big deal, but at school it was a completely different story. It was extremely important to her that no one knew. She had to work hard sometimes at keeping it hidden, especially when she had a port a catheter in her chest, but she did it...and she attended school as long as she was well enough.
Two days before Amber started her senior year, she got very ill. She had contracted C-Diff. C-Diff is harmless to those who don’t have compromised immune systems, but for someone weakened by illness, or on long-term antibiotics it is quite serious. Her nurse practitioner started pushing the CF doctor to get Amber started on the lung transplant process. Amber improved for a couple weeks, but her lung function never increased and her infection kept mutating and the doctors had no antibiotics left to give her.
On September 17, she got ICU psychosis, which is a sign of impending death. She was on continuous oxygen now. Her kidneys started to shut down and she lost control of her bowels and bladder. At one point in the middle of the night she pulled her mask off and said, “Mom.” I leaned over and told her that it was okay. She had fought hard and she didn’t have to fight any more. After that she went into a coma. I made the decision to take her off life support
Amber’s high school closed the day of her funeral so teachers and students could attend. Kids from her previous school attended as well as all of her doctors and nurses, which is unheard of. The funeral home said it was one of the largest funerals they had ever witnessed. Over 200 came to the gravesite.
Amber never gave up hope that she would be cured. She was planning on a career in nursing, wanted to get married and planned to adopt a baby with Downs Syndrome because having her own baby would have been risky to her health. Amber left an impression on everyone she touched. She was a sweet child, an angel on earth.
Cystic Fibrosis is a terrible disease and no child should have to suffer what Amber suffered. Although CF affects thousands of children yearly, very little is known about it. One doesn’t hear the term unless they know someone affected, and then they have to search to find out about it. For a disease that affects so many, it is not “popular.” There are no televised fundraisers; no celebrities out fighting for the cause; a “portion of the proceeds” of products doesn’t go for research. It is an ignored disease and awareness needs to be raised. These kids need our help.
Liz, USA
Amber passed away 5 years ago - she was 17.
____________________________________________________________________________________________________
When our daughter Sophie was born, everything looked great. Within a day though, we were being told they suspected she had a bowel blockage, and the possible scenarios. So she was rushed to NICU and told that a surgeon would see her in the morning. Matt and I spent a sleepless night in the maternity ward, listening to other babies crying, wondering what was happening to ours.
So, I could write a novel about Sophie's ordeals in hospital, but in brief...
She had meconium ileus - meconium so sticky it had to be scraped from the bowel. All I could think about was my baby being cut open! We were told that the nature of the blockage made it highly likely she had Cystic Fibrosis. The surgeon asked if we knew what it was, and I replied, "is it something to do with the lungs and they usually die young?" Yes.
So at 8 days old, Sophie was having her second surgery to remove another blockage, and a second ileostomy hole in her stomach. She had this rotten ileostomy bag attached to her that spewed yellow muck all day and usually leaked and burned her skin. She was not feeding, and existing on TPN. So when she was seven weeks old, NICU decided it was time to move her to the ward, and there we lived for 8 weeks - two more bowel surgeries, and much despair. When Sophie was about 14 weeks old, we nervously took her home with a central line still attached, scars all over her, and malnutrition. And then tried to settle into the routine of nebulisers, physiotherapy, medicines that made her scream.
Sophie has since been reasonably well, with only one hospital admittance. I put it down to good inherited immunity, and that she is so very active. She is now nearly eight years old and constantly questions why she has to have enzymes when she eats and do PEP and physio. She already thinks that it is a drag to have CF and that she is different. But I tell her that she has benefits - she will never have to watch her weight, she can eat anything she likes, especially high salt, high fat, high energy foods. And that doesn't exactly excite her, as she struggles to eat anything (partly because she is a fussy eater, and partly because CF children have no appetite).
Does she know what the future holds? No, she doesn't. And nor do we. We hope for a miracle cure, just like all the parents before us hoped. We hope that she will live to find love, have a career, and maybe even have children.
CF affects lungs, digestion, reproduction.... life.
Kellie, NSW