Ivacaftor is one of the newer medicines that is showing great potential for the treatment of cystic fibrosis for G551D and other 96 variants.
Ivacaftor-
What is ivacaftor-
Ivacaftor is a CFTR (cystic fibrosis transmembrane conductance regulator) modulator.
It is an agent which helps in the potentiation of mutant CFTR gating in cystic fibrosis patient’s genes.
It is the first approved medicine of this class for human use in patients with this disease.
What is the mechanism of action of ivacaftor?
It can potentiate the CFTR channel opening and thus facilitate ion transport. It overcomes the G551D CFTR gating defect which helps in improving the condition of the patients suffering from cystic fibrosis.
Benefits of ivacaftor-
For patients who have G551D CFTR gating defects when taking this medicine can help the patient overcome these gating defects and provide relief which includes-
- Improvement in lung function which helps in improving the breathing in the patients.
- Weight gain is also seen in the patients with the improvement of some of the gastrointestinal symptoms.
- Sweat chloride values of the patients shows good improvement and are reduced quite well compared to any other treatment methods used before this medicine.
- This medicine has been approved in the USA for 96 variants of CFTR other than G551D.
With continuous administration of this medicine, lung function improvements are significant and can be seen in the patients.
Lumacaftor and Tezacaftor-
How Lumacaftor and Tezacaftor works-
These medicines are used in F508del processing abnormalities and they help in improving this.
FDA (Food And Drug Administration) USA has approved these medicines for this disease.
This medicine has corrector molecules in them which help to repair the misfolding of the CFTR.
The three medicines when taken together in combination can help to improve pulmonary function.
Elexacaftor is a newer medicine in this group and works by different mechanisms and has shown a good effect on the disease.
This Triple medicine combination therapy has proved beneficial for the patients and improves the patient’s conditions.
This Triple combination medicine helps to improve the breathing of the patients and also bring down the sweat chloride number to normal level.
Precautions while taking the combination of these three medicine-
Doctors generally monitor all the vitals and blood reports of the patients before and during the therapy with these medicines.
Liver Function tests are monitored closely to check for any side effects during the course of medicine to keep the liver healthy.
If the patient is taking any other medicine along with the above-mentioned medicine then it is important to look for any interaction with these medicines as it can lead to some side effects or weakening of the medicine effects which can lead to worsening of the patient’s conditions.
How to take ivacaftor?- Route of administration
It is available in the form of Tablets and Granules and is taken orally in the dose as advised by your doctor.
After how much time does the patient’s condition improve?
If taken as prescribed by the doctor without missing the dose then this medicine can show its effects and in seven to Eight Months patient’s respiratory capacity can improve.
Important points-
- Always consult your doctor about your conditions with the doctor and take only those medicines which are prescribed by the doctor.
- If your doctor prescribes you the above-mentioned medicine then take it as advised by the doctor.
- Do not stop these medicines on your own and if you get any side effects after taking this medicine then inform your doctor before stopping these medicines.
- Always give your complete medical history and personal habits history, so that the medicine can be prescribed depending on your medical and personal habits history.
- Eat healthy food as advised by your healthcare professionals and follow a healthy lifestyle.
