Lung Transplantation

Question

Good afternoon,

three weeks ago, our baby son (8 weeks old) was diagnosed with Cystic Fibrosis after the values of his neonatal screening were increased – a nightmare!!!

First examinations concluded that he is missing a pancreatic enzyme. He now has to take pancreatin, simethicone, a vitamin compound, and ACC juice. He is being breast-fed fully and has been gaining weight properly from the beginning.

In addition, we are already supposed to do physiotherapy. And here comes my problem which keeps me awake at night: the physiotherapist told us that all CF patients will require a lung transplant at some point. Is there any truth to that statement?

She also said that our son was short-winded and would therefore probably get oxygen through a nasal tube soon. The lung is not affected, though; our paediatrician and our CF doctor find the lung to be completely normal. How can the therapist make such a statement simply from looking at our son?

We are very upset and obviously do not want to continue working with this therapist.

Answer

Hello,

This is indeed very unfortunate. Therefore, although I am not a paediatrician, I will answer really quickly so that you will not have to wait for too long.

It is completely normal for the lung to be okay at this age – and with optimal care in a competent CF center, it should stay that way for as long as possible. Your indication of a proper weight development is already an important and good piece of information, and you can be happy about that, since it shows that everything is going well.

Concerning your concrete questions: not all CF patients want or need or get a lung transplantation – we strive to defer lung damage through inflammation and infection for as long as possible. Our long-term goal, of course, has be the ability to completely avoid lung disease.

And even though I cannot determine from here whose statements about the condition of the lung are correct, I can still say that normally, at 8 weeks and with proper growth, an oxygen tube will not be needed anytime soon.

It definitely is a burden to be confronted with the diagnosis and to imagine what that means for your son’s and your family’s future. Therefore, it is all the more important not to miss anything (the screening helps a lot in this respect) and to quickly receive proper care in a competent CF center. There will be a lot of things for you to deal with now, and I cannot play down the diagnosis or make it look harmless, but panic and horror scenarios will not help – it is important not to allow oneself to be driven mad. One does not have to draw out now every problem that might arise at some point – some things need to be tackled only at the time when it is necessary.

Apart from the connection to a CF center (in case you are not yet visiting a special-care clinic, you can probably find addresses via Mukoviszidose e.V.), getting in touch with CF-experienced parents who have been through this and know what to pay attention to “now” and which problems to confidently put off until “tomorrow.” You can get addresses of local contact persons via the office of Mukoviszidose e.V. (on their homepage, www.muko.info).


Good luck and enjoy your little son.

Prof. Dr. T.O.F. Wagner

01.12.2008