How did we find out about our children having CF?
When I was 22 weeks pregnant with (my eldest, 4 yrs old), her bowel was echogenic on the anomaly scan. They took blood samples to check for CF carrier status, and it came back we were both carriers. We refused an amnio, and when she was born she had a blood sample taken which came back at 8 days old to say she had CF.
With my son, we refused an amnio again, his bowel was fine on his anomaly scan, and he had a cord blood sample taken at birth, which came back positive for CF at 7 days old. There is no history of it in either of our families.
How has it changed our life? In every way imaginable. Not expecting grandchildren. Spend a lot of time at the hospital. Daily routine is EXTREMELY different from that of parents of healthy children, we spend 2 2/1 hrs a day doing physio, 3 hrs a day giving intravenous medicine at the moment, and have to give my son a total of 26 doses of oral medications per day, and my daughter a total of 11 doses of oral medications per day. I have had severe post natal depression after both children were born.
My marriage has been put under intense strain from all the emotional stress involved in caring for our children. Our children can't live a normal life, and we can't be normal parents. We don't think the thoughts that normal parents would. We don't take their health or life expectancy for granted. We try to make every moment count for them, because we don't know how many they have. We take tons of photos, to remember as much as we can of them growing up. Husband has had to have vasectomy as we couldn't possibly have any more children, even though we long to have another child.
Care from NHS: Mostly positive, but inconsistent depending on area. Care while pregnant with my daughter, was unsatisfactory. Were told very little when the problems initially came to light and weren't treated sympathetically or anything about it, no one tried to make it any easier for us, refusing us test results etc. Had awful birth with my daughter and nearly lost her, when they'd promised to get her out at the first sign of trouble during labour and left her for hours and she nearly died.
Care from our local hospital has been great, always there when we've needed them, given effective treatments to my daughter. We've had some disagreements over my son's treatment, mostly because they've not listened to a mother's instinct and I've been proved right in the end. They listen to me more now though.
Care from the larger teaching hospital was good when I was pregnant with my daughter and went there for specialist scans, but wasn't happy with the care my son received there when he went in for his portacath surgery. Very disorganised, didn't seem as clean as our local hospital, and no-one knew what was going on!
Care from our CF Community Nurse. IMPECCABLE. At the end of the phone whenever we need her. Very well informed, can guess quite accurately what the consultant will say to us. Explains lots of stuff for us. Comes to the house whenever we need her to. Helped set up my daughterís care plan at pre-school. Gave us my son's CF results in person. Couldn't manage without her.
How could you improve care? That's not an easy question. LISTEN to parents more, we know our children better than anyone, we are with them 24/7. Be more sympathetic. Be more supportive during pregnancy. Don't go back on promises. Don't be afraid to say 'I don't know' instead of guessing and being wrong and losing parents' trust.
Additional expansion of questions: Is there anything more you would like to say about the nurses in relation to the counselling received or other experiences you have had of them? Especially when they find out that the children have a genetic condition?
During pregnancy, we had VERY LITTLE counselling at all. No one seemed to have a clue what CF was at all, and no one would tell us anything. We had to find out all our information from the internet, and that gave us a very very very scary view of CF. Just a list of all the things that could possibly go wrong. They did finally put us in touch with the CF paediatrician, but he couldn't really tell us that much, as we didn't know for sure if she had it, and how bad she'd be at birth. We were able to ask him questions etc, but they sprang the meeting on us with absolutely no notice, so we didn't have chance to think up questions. The one good thing they did was show us round SCBU before she was born, which really helped when she went to SCBU (for reasons related to her traumatic birth, not to CF).
When they found out my daughter had CF, we just saw the CF paediatrician after that. They then directed us to the CF Trust website and got us pamphlets sent from the CF Trust especially for new parents, but to be honest, we weren't ready for more information. We just wanted to know what we needed to know for caring for her, not all the detailed background info. When we were ready, we researched it ourselves.
We did have one session of genetic counselling, just after my son was born, but by then it was far too late. In fact, it was more of an inconvenience than anything, as it was another hospital trip, time off work for my husband because he had to be there too and all they did was explain how CF is carried, which we already knew! They even told us that there was nothing else they could say to us, just to let them know if my daughter or my son wanted advice about family planning in the future!
Our care would have been improved by the doctor who had to explain to us all about echogenic bowel actually KNOWING what echogenic bowel was. He read off a sheet to us about it, the same sheet we'd already read in the waiting room 10 times. Then he offered us an amnio and possibly a termination, which we didn't want. He totally gave the impression of not caring, and not wanting to care, not wanting to be there.
Our care would have been improved by the doctors involved being more empathetic to the traumatic events of the day. Being more honest with us. Knowing more about CF!
And when they rang us to say our results were back, we had to battle to get them to tell us over the phone, as we didn't want the results in person, we wanted time to absorb the results before seeing the specialists. I know that CF is fairly rare in the grand scheme of life, I'd never even heard of it before they said my daughter might have it at 22 weeks pregnant, so I know it's unreasonable to expect every doctor and nurse to know lots about it, but it would have made things so much easier if midwives knew more about echogenic bowel and CF, especially as they are now planning to test for CF routinely on the Guthrie Test throughout the UK.
Another little thing that could make things easier, actually reading my daughter or my son's notes, instead of asking us the same questions about 10 times a day! Every doctor would ask what meds they were on, what doses, what times, and want their full history from us, every time! Sometimes even asking us two or three times within the same conversation! All that information should be in my daughter or son's notes, and we found it incredibly frustrating having to keep repeating ourselves, and also it was rather unsettling, to think that such basic information wasn't being absorbed, didn't help us feel confident in the treatment our children were receiving!
Also, what makes our life harder is seeing registrars on Childrens Ward, or even consultants, who know very very little about CF. A CFer is managed very different to your average child, antibiotics are prescribed earlier, extra tests are performed, etc, and yet time and time again we have to fight tooth and nail for the appropriate treatment from registrars and consultants on the ward. I now know that if we are asked to go to Childrens Ward for either of the children to be examined, we won't get any appropriate diagnoses or treatments out of any doctors who don't know anything about CF.
One example, we had a registrar swearing blind that my son was fine, that all his problems at that time were upper respiratory, and that no treatment was necessary. We fought for a second opinion and he ended up looking at my son's x-ray, which the second doctor suggested having done, which clearly showed a patch of infection in his lower lung, and he was put straight onto an iv (intravenous fluids).
I know that unless we see one of the doctors who know a bit about CF, we will be back at the hospital a couple of days later, if not sooner than that, fighting again for treatment because symptoms are worsening.
Did you want to know how each individual nurse responds to finding out that your daughter or son has CF? Is that what you meant? In that case, sometimes they don't know what it is, or don't know much about it, and usually want to talk about their treatments and their history.
We've never had any negative reactions to health professionals finding out about them having CF, and usually nurses are very supportive. I couldn't wish for better nurses on our local children's ward, they are very empathetic, supportive, making cups of tea, feeding me when I'm staying on the ward and can't get anything to eat from the canteen, talking to me when I get upset about the doctors being so frustrating and not listening to me, and they genuinely care about my son (my daughter has always been very well, so they rarely see her, don't have the same relationship) and want to see him get well. They were also excellent at teaching me to give iv's through his port during his current course of ivs. I view them almost as friends.
The only negative thing has been all the students, they all want to interview us or prod his port or watch procedures, and while I don't mind this at all, as it all raises CF awareness and helps improve the treatment for our children in the future (hence this interview!), it does leave us feeling like a bit of a freak show sometimes! We've had a midwife use me for her study for her training, a problem pregnancy study! We've been to meetings to help develop pamphlets giving parents of newly diagnosed children information, we've had countless students observe consultations or treatments, I've been interviewed a few times, and just the other day we had students examining my son's port. As I've said, I don't mind, but it is ... odd, for people to want to stare, or to prod, or to talk to us, all because of CF.