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Lung bleeds (hemoptysis)

Question
Is there something to prevent lung bleeds? My son has recurrent lung bleeds and he is fed up with it. Already 4 times he had embolization done and he doesn’t want it anymore. Is there something that can straighten his veins ?
Answer
Dear ECORN-user,

The lung has 2 systems of blood supply. The first system is called the lung veins and the lung arteries. This system is responsible for about 98 % of the perfusion of the blood to the lungs and is under low pressure. It’s function is to take up oxygen from the air into the lung blood vessels. Oxygen is then transported to all organs in the body. Just like the others organs, the lung gets an oxygen supply via the bronchial arteries. This second system only supplies 2% of the blood to the lungs but functions under high pressure (equal to the pressure in other arteries throughout the body, at the pressure that is called blood pressure).
It might sound strange but 95% off all lung bleeds in CF occur in this second blood supply system. In CF, several factors can contribute to the coughing up of blood. In a minority of situations (7%) major lung bleeds, even massive lung bleeds, occur. For these, age and poor lung function are the main risk factors. These bleeds out of the high pressure bronchial arteries occur because of the chronic infection in the airways leading to overgrowth and tortuous course of the bronchial arteries to the lung. The inflammation and damage to the airways can cause the local feeding bronchial artery to burst and bleed. These bleeds under high pressure can cause major blood loss.
Apart from major lung bleeds, patients can cough up minor streaks of blood, usually mixed with sputa. This mainly occurs at the time of worsening of lung infection which makes the mucosa more vulnerable so that minor blood loss occurs. Rarely, vigorous chest physiotherapy can lead to production of bloody sputa because of ‘barotrauma’ due to pressure changes in the chest.
An extra element in patients with CF is fat malabsorption, responsible for poor uptake of the fat soluble vitamins A, D, E and K. Vitamin K is a important for blood clotting. Deficiency of vitamin K will make you bleed more easily or, when a minor bleed occurs, it can worsen in case of vitamin K deficiency.
The treatment of lung bleeds is similar in CF and non CF-patients with this difference that in CF, vitamin K deficiency should be excluded and if present treated. Several other drugs are used in the treatment of lung bleeds. There is little proof for the efficacy for tranexaminic acid (Exacyl) and desmopressin (Minirin). Apart from giving these ‘clot promoting medicines’, the cause must be treated. Since worsening of the infection is the most frequent cause, the patient should also be treated with antibiotics. In case of severe life threatening bleeds, first the oxygen supply via the non-bleeding lung should be safeguarded. Often, putting in a tube with a balloon to occlude the airway on the side with the bleed may be necessary. At times one can try to stop the local bleed with medication (via bronchoscopy). Another option, but not available in all centers, is ‘embolization’ whereby the vessels that cause the bleed are closed off. This treatment is however not definitive and re-bleeds do occur in the same location as well as in another site. When the bleeds occur repeatedly in the same lung and in the same location, local surgery can be considered. But for patients with CF this is not really advised. When patients repeatedly have severe lung bleeds and embolization does not solve the problem, a lung transplant should be considered.
Kind regards
Pieter Goeminne
01.12.2011
1.12.11
Please find more information on this topic under the former question:

ecorn-cf.eu/index.php?id=65&L=0&tx_expertadvice_pi1[showitem]=760&tx_expertadvice_pi1[search]=haemoptysis