ecorn-cf: App. question list

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R553x/IVS8-5T-TG12: Hello, concerning the mutations mentioned above, we have been told repeatedly that this most likely means mild progressions of CF. But what does that mean? Does my daughter have a normal life expectancy? She is five years old and does not show any effects in the lungs or other organs. She was diagnosed by chance. She was supposed to get her genetic type for alpha1-antitrypsin insufficiency determined since the laboratory told us she had antitrypsin insufficiency. Since the antitrypsin genetic type was normal in a second test, the blood was examined further, which showed the above mutations. Many thanks C.M. 2nd question on this topic: Hello, we have been told repeatedly that our daughter has the R553x/IVS8-5T-TG12 mutations and hence a mild form of cystic fibrosis. Do these mutations already mean a higher life expectancy, or can one even reckon with a normal life expectancy? Can these mutations potentially lead to asthma or asthma bronchialis? Many thanks C.M.; 06.09.2010

Germ colonization with atypical cystic fibrosis: Hello, is the probability of germ colonization as high in atypical cystic fibrosis (R553x and IVS8-5T-TG12 mutations) as in typical cystic fibrosis? Many thanks. Kind regards C.M.; 06.09.2010

Progression with R553x/IVS8-5T-TG12 mutations: Hello, My daughter has been diagnosed with atypical cystic fibrosis. What does that mean? Do you possibly have any experience with the mutations mentioned above? Many thanks C.M.; 06.09.2010

3 sweat tests – 3 different results, part II: Dear Dr. Sommerburg, I am submitting my question again, since it was not transmitted completely the first time. My four-year-old son was admitted to the hospital with obstipation. Upon admission, his stool values were at 207 ug/g and the sweat test (sodium iontophoresis) at 30 mmol/l. Further tests during his stay – after purging measures with the help of Practo-Clyss®) – returned the following values: stool < 50ug/g; sodium iontophoresis 49 mmol/l. The head physician recommended further tests after two weeks. We had those done now, with the following results: stool at < 50 ug/g and sodium iontophoresis 38 mmol/l. The head physician thinks all these values are within the normal range, except for the stool values. He suspects those to be only temporary, though, due to preceding infections, one asthma attack and stomach flu. He ascribes the differing sweat test values to my son’s condition on the days of testing. Can I assume that everything is okay, or what should I do now? My pediatrician is trying to push me into the CF direction and urging me to take more tests. I would be grateful for a quick answer. Kind regards; 06.09.2010

MRSA- germs: About 2.5 years ago I had an empyema of the knee joint because of MRSA after an injection and had then be operated 9 times on the knee and femur. It has been treated in the beginning with Linezolid (without success), then with Vancomycin. Discharge of the hospital after 3 months. After a rehabilitation and longlasting physiotherapy and hard training I am able to walk again and to bend the knee to 110 degrees. Sportive activities like before the infection (jogging etc.) is not possible anymore. My question is, if there is the possiblity that remaining MRSA-germs could have encapsulated in the joint or the thighs, which could be probably getting active again at a knee-prosthesis operation, which is probably necessary in the next years? Is it realistic at all that after the time-interval of 2.5 years bacteria could still be encapsuled in the leg?; 06.09.2010

Nausea and vomiting in severly ill people: Despite 24-hour oxygen administration, my son suffers from nausea and vomiting in connection with food intake. This often seems to be the case in the late stages of CF. Is there a connection with the lung/heart problem (tachycardia) or a medicinal therapy? It would be important for him to gain weight given the upcoming transplantation. His diabetes is adjusted quite well and the oxygen saturation okay.; 06.09.2010

MRSA: Dear expert team, I have a tremendous problem. Our little Louis is almost 19 months old now and has CF. My partner’s family does not want to have anything to do with the disease, dismiss everything and think that if they only see him every three months they do not have to pay attention to anything (we live in the same street). My partner’s grandmother has diabetes, can hardly walk and has recently been admitted to three different hospitals including a lung clinic due to acute disturbed functions. I am terrified now that she might have MRSA, and I do not know what to do. We avoid any contact completely now, of course. How could we find out whether grandma has such a germ? What are we supposed to do now? Please answer me honestly; we are done with this family anyway, but how long does such a germ live? Regards Michelle; 06.09.2010

Hygiene: Hello, I have a question concerning hygiene with CF children. Some think that it is necessary to have the child shower and change all of the clothes. I usually do this every other day. Would it not be an exaggeration to do this more frequently? My daughter is five years old, has two rabbits and a dog, and I pay strict attention to her washing her hands whenever she was in contact with the pets. After she goes riding, she always changes all her clothes immediately. In addition, I always put any clothes into the dryer after washing to get rid of any germs. Earlier, I often only folded them… Sometimes I wonder if it would not be reasonable to keep folding the clothes without putting them in the dryer or ironing them. Which hygiene measures are necessary, which ones are exaggerated?!? Many thanks.; 06.09.2010

Food: Dear expert team, I have read all questions in this forum and, interestingly, have come across the question about chees. Up to now, I have not given Louis any blue cheese for fear of possible fungi. This has been clarified now. ;-) Are there any other foods which one does not think of at this point, but which people with CF should perhaps rather not eat? This is concerning “strange” fungi or other “CF-specific” germs. And food intolerances notwithstanding, of course. Many thanks Michelle; 06.09.2010