CF is more than just lungs
Today the CFTrust launched its CFTruths campaign once more concentrating on the pulmonary effects of CF. While the effects of thick mucous in the lungs is the most serious issue in most with CF limiting the discussion to just that seriously short changes most. Bowell and pancreatic problems are well recognised and attributed to the viscous mucous but is that all. Many pwCf have a plethora of Co-morbidities and renal and hepatic failure are all too common. Obstruction of airways and ducts does not explain failure of the endocrine glands such as the Islets of Langerhans in the Pancreas (causing Diabetes), nor Adrenal dysfunction causing Hypokalaemia and Addisons Disease. Hyperparathyroidism too occurs despite the Parathyroid Gland having no goblet cells secreting mucous. Heart failure was reported as long ago as 1980.
Many of these so called Co-morbidities cause considerable distress and disability in pwCF, are often overlooked by the superb Respiratory Physicians leading CF Teams. Unfortunately too, specialists in other fields rarely understand the role of the faulty CF gene in their cause.
The thick mucous is well ascribed to the failure of the Chloride transport from the cell caused by the faulty CFTR channel and gene. If the Chloride (an anion) is not being pumped out then the levels within the cells must be high and must be balanced with cations esp Potassium (K) and Calcium (Ca). High K within the Adrenal cells will surely make the cells ‘think’ K is too high and increase Aldosterone secretion, causing hypokalaemia. Similarly high Ca within the Parathyroid cells will affect PTH and the Ca metabolism balance. K & Ca are also involved in the function of muscle cells (incl Heart Muscle), neurons and to some extent every cell of the body. No wonder pwCF are so complex!
We do need the CFF to lead research into these co-morbidities. Cardiologists, Endocrinologists and other Specialists need to understand the effect of the faulty CFTR channel and gene is having in the organ they specialize in. To date, they usually don’t. Cardiologists do not understand why pwCF and heart failure often have severe problems with many heart drugs. ACI’s, ARB’s & Ca Channel blockers often increase blood pressure rather than decrease it.
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