Treatment of Cystic Fibrosis

Part 3

As previously mentioned there is currently no known cure for cystic fibrosis. For some who suffer the condition this can be a life-long sentence of frequent hospital visits and for others the condition may cause very little suffering. The age that a cystic fibrosis patient is likely to die is totally unpredictable, and it is not fair to say that the person who suffers the most symptoms on a regular basis is likely to die sooner than the person who has one chest infection per year. The not knowing is often the hardest thing to cope with for parents who have children with the disease.

In as much as there is no cure for the disease therapeutic treatments are improving all the time and symptoms can be markedly improved using a combination of therapies. Antibiotic treatment is one of the primary treatments used in the management of cystic fibrosis. Without the use of antibiotic therapy patients would die of serious infections such as Staphyloccocus aureas, Hemophilus Influenzae, Pseudomonas aeruginosa and Burholderia cepacia. To minimize the risk of developing such infections it is strongly recommended that patients are immunized against influenza, pneumonia, whooping cough and measles (Cystic Fibrosis Trust, 2002).

In some cases of infection antibiotics can be administered at home without the need for hospitalization, this can be via oral or nebulized administration, or intravenously given by specialist nurses that visit the home. Patients with the disease are nursed at home wherever possible, as frequent hospital attendances can increased the risk of acquiring infections.

The metabolism and clearance rate of antibiotics in the cystic fibrosis patient differs from the patient who does not have the disease therefore treatment regimes differ accordingly as standard treatment does may not be sufficient to eradicate the infection (Sorgel et al, 1996).

For patients that receive intravenous antibiotic therapy on a frequent basis the impact of this on their veins can be problematic and crucial therapy can be interrupted when venous access is not possible. In these cases the patient will have a ‘TIVAS’ (totally implantable venous access system) inserted which is required to be undertaken as a minor surgical procedure. The benefit of these systems is that due to their increased durability patients can receive intravenous antibiotics at home more easily. These systems do however carry their own risks and the most common ones are blockages and increased risk of infection (Burdon et al, 1998).

In terms of therapy that cystic fibrosis patients receive, physiotherapy is one which compliments the medicinal therapies that the patients very much rely on. Even though antibiotics treat the infection, until they become therapeutic the patient still have to endure the unpleasant symptoms of excessive mucous secretions lying on the lungs and in the respiratory tract.

At times the mucous is too thick to excrete by using methods such as specific coughing techniques to loosen and expectorate the mucous, times like this call for intense physiotherapy by means of percussion, postural drainage and hand vibration techniques to the chest to carefully tease, or in some cases force the mucous to loosen in readiness to cough up.

Chest physiotherapy for some is a daily occurrence, and for some this will mean several sessions in a day. The therapist will administer treatment which the patient cannot deliver themselves however parents and carers are also taught vital techniques to enable physiotherapy to be given at times when it is most needed. Without the physiotherapy treatment many patients would have a shorter life span due to permanent lung damage occurring long before it needed to. Evidence suggests that even in patients with mild chest symptoms, daily physiotherapy maintains the chest in a better condition than if treatment were omitted (Reisman et al, 1988).

To compliment the effects of the physiotherapy treatment the use of steroids is also commonplace. Medications such as this allow better patency of the respiratory tract as a result of reduced inflammation and increased air flow. The use of oral or inhaled steroids is one which must be considered carefully as there are negative effects of the use of these treatments also therefore caution must be applied in each individual case.