Q: What is the recommended venous access device in people with inhibitors: port-a-cath, Hickman line, shunt, other?

A: There is no recommended venous access device for people with haemophilia with inhibitors. The decision should be made on the individual patient’s demands, i.e. venous situation, how venous punction is perceived, current treatment (prophylaxis or on-demand-treatment, ITI, ITI regimen) etc. If a central venous line becomes necessary (usually for long-term treatment) we usually recommend to use fully tunneled implantable devices such as port-a-caths because they allow almost normal activities (swimming etc) in contrast to devices as hickman lines.

In some patients some weeks of intensive treatment have to be managed and so called pic lines can be used to bridge this period. All these lines have the risk of infections or occlusions. They should be used under aseptic conditions. AV-fistulae (shunts) have become very popular in some centres. However, they have to mature after surgery until they can be used. Surgery might be challenging particularly in small children and experienced surgeons are needed. Patients with AV-fistulae can also develop complications (inadequate maturation, AVF overflow, thrombosis, limb hypertrophy etc).

After all it is always an individual decision whether a patient needs central venous access or not and if so which one is the most convenient access device.

Q: My son has hemophilia A with inhibitors. He never had a “implantofix”. We always used two veins to infuse the factor. Do you consider that we should alternate the access using different veins or we should try to use always the same in order to make up a “natural access canal”?

A: Of course you can continue to use both veins. It is always good to have another safe access if one vein can’t be used any more. But it is very important to take care of the veins after each venipuncture (press on site of injection for several minutes after each punction, use cream (local massage to keep the skin /scars soft and healthy). You should try to use thin needles whenever possible, since they do not cause large wounds. Many patients, particularly children want their injection at the same site, since it doesn’t hurt so much when you inject in scared skin. If you decide to start to use a new vein consider using Emla before injections to reduce pain, particularly in children.

Q: My personal case: I always had poor veins, when I was a child I just had infusions on basilic veins (left and right arms). Nowadays that veins are scared I find it is really hard because the needle needs to cross the “hall”, you know? It hurts. Parents, pay attention please. If it’s possible, try find new veins and change location of infusions. Since 2016, I have a Venous Access too, because after severe bleeding I had infusions each day for one week. I didn’t have more veins after that, the solution was the port. I thought, well, now I have a “USB port” the easiest way to get factor. Isn’t it! I need someone to do the work for me. Luckily my daughter is my nurse at home. My concern about CVC are about: – I can sleep from the same side of CVC? How is this device locked in the central vein, is it possible with movements remove the device? – Can I go to Sauna or Turkish bath, in hotel?

A: I try to avoid the use of CVLs whenever possible. But if they have to be placed in adult patients they should be placed at a place where the patient is able to use the device by themselves. However, this was obviously not possible in your case – but you have your daughter who is supporting you. After a few weeks when the whole postoperative haematoma is completely reasorbed the port usually doesn´t move any more, some surgeons even fix it in place. In rare cases the port chamber might move slightly, however the catheter stays in the vessel. So you shouldn’t be afraid to sleep on the same side where the port is placed or to be physically active. But the most important thing is – you should feel comfortable. Concerning Sauna, Turkish bath or swimming I usually advice my patients not to do it immediately after using the port (wait a few hours – the injection wound should be closed).

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