When your doctor tells you or your loved one that they have cystic fibrosis (CF), you probably have a lot of questions about the medication they will prescribe. Since it is a complex condition, it is important to be fully informed about the medication they will prescribe. Today we are going to talk about one such specific medication. It is a combination pill called ORKAMBI, which is a combination of two drugs called Lumacaftor and Ivacaftor.
What kind of medicine is this? What exactly does this do?
Simply put, cystic fibrosis (CF) is a condition caused by a defect in a protein in our body. This causes the secretions in our lungs and organs, especially mucus, to become very thick and sticky. Think about it, normally the mucus in our lungs is a bit runny, so it comes out easily. But the mucus in a person with CF is actually thick, like glue. This thick mucus gets stuck in the lungs, causing difficulty breathing, coughing, and frequent infections.
Lumacaftor; This drug called Ivacaftor works by going to that faulty protein I mentioned and fixing it to some extent. It's like fixing a broken gate. When this protein starts working properly, the way salt and water move through the body's cells is balanced. Then that thick, sticky mucus becomes a little thinner and starts to be easier to remove from the lungs. This can control many of the symptoms of CF.
But one thing to remember is that this is not a cure for cystic fibrosis (CF). It is just a treatment that helps control the symptoms and make life easier.
Things you need to tell your doctor before taking medicine
Before starting this medication, it is very important to inform your doctor about your complete health history. Because some conditions require special care when using this medication. If you have any of the following conditions, be sure to tell your doctor.
| Health condition or opportunity | Why do you need to tell the doctor? |
|---|---|
| Liver disease | This medicine can affect the liver. If you already have liver problems, your dose may need to be adjusted or you may need to be checked more often. |
| Kidney disease | It is also necessary to be concerned about kidney function. |
| High blood pressure | The medication can cause blood pressure to rise further, so it's important to be aware of that as well. |
| If you are pregnant or trying to get pregnant | You should definitely talk to your doctor about the safety of this medication during pregnancy. |
| If the baby is breastfed | Discuss with your doctor whether the medication passes to the baby through breast milk and its effects. |
| Allergies to other medications, foods, or dyes | This information is important to know if you are allergic to this medicine or any of its ingredients. |
How do you use this medicine?
This is the most important part. If you don't use this medicine correctly, the results may be reduced.
- Take with a fatty meal: This is the most important rule . For this medicine to be absorbed properly by the body, it should be taken with a fatty meal. For example, take it with something like eggs, butter, cheese, peanuts, avocado. If giving it to a small child, you can give it with breast milk, powdered milk or full-fat dairy products like yogurt, cheese.
- Take it exactly as your doctor prescribes: take the right dose, at the right time. Continue using it until your doctor tells you to stop.
- Do not take with grapefruit juice: Avoid drinking grapefruit or grapefruit juice while taking this medication.
- When given to children: This medicine can be prescribed to children over 6 years of age. However, your doctor will give you special instructions about this.
What if I take too much medicine?
If you think you have taken more than the prescribed dose of medicine, immediately call the National Poisons Information Centre at the Colombo National Hospital, or go to the Emergency Department (ETU) of the nearest hospital.
What if I forget a dose?
If you forget to take a dose, take it as soon as you remember. However, if it is less than 6 hours until your next dose, do not take the missed dose. Skip it and take the next dose at the scheduled time. Never take two doses at once.
Other medications that may interact with this medicine
This is something to be very careful about. ORKAMBI can interact with many other medications. This means that taking it with other medications can change the effects of ORKAMBI, change the effects of other medications, or cause serious side effects.
Therefore, you should tell your doctor about all the medications you are taking (medicines for other illnesses, vitamins, herbal remedies, Ayurvedic medicines, dietary supplements).
Below are just a few of the medications that may interact with this medication. This list is not complete.
| Do not take this medicine with any other medicine. | |
|---|---|
| Artemether, lumefantrine | Ranolazine |
| Lurasidone | Some medications given for HIV and Hepatitis C viruses |
| Please inform your doctor as this may interact with your medication. | |
| Some antibiotics (e.g. Clarithromycin, Erythromycin) | Antifungals (e.g. Fluconazole, Ketoconazole) |
| Medicines for epilepsy (fits) (e.g. Carbamazepine, Phenytoin) | Medication for depression or anxiety |
| Immunosuppressants (e.g. Cyclosporine, Tacrolimus) | Types of steroids (e.g. Prednisone) |
| Birth control pills (Estrogen/Progestin hormones) | Warfarin (a blood thinner) |
| Painkillers like ibuprofen | St. John's Wort (herbal medicine) |
Other things to consider when taking the medicine
- Regular medical checkups: Your doctor will tell you to come to the clinic regularly. Go at the right time and get things like blood tests done. If your symptoms don't go away, tell them about it.
- Watch your eyesight: This medicine has a small risk of causing cataracts, especially in children. Therefore, you should have your eyes checked by an ophthalmologist at regular intervals while taking the medicine. If you notice any changes in your eyesight, tell your doctor immediately.
- Be careful with birth control: This is very important if you are using hormonal birth control pills. ORKAMBI may make them less effective. Therefore, be sure to talk to your doctor about using an additional form of birth control, such as a condom or diaphragm .
Side effects that may occur due to the medicine
Like any medicine, this one can cause side effects in some people. Some are serious, some are common.
| Serious side effects that should be reported to the doctor immediately | |
|---|---|
| Allergic reactions | Skin rash, itching, swelling of the face, lips, tongue or throat. |
| Difficulty breathing or chest pain | If you have difficulty breathing or feel your chest tightening. |
| Symptoms of liver damage | Pain in the upper right side of the stomach, loss of appetite, nausea, light-colored stools, dark yellow or brown urine, yellowing of the eyes or skin, and extreme fatigue. |
| Increased blood pressure | The doctor will check this out. |
| Change in eyesight | If your vision feels blurry. |
| Side effects that are usually not urgent, but should be reported to the doctor if they persist | |
| Stomach ache | Gas |
| Diarrhea | Nausea |
| Headache | Tiredness |
| Irregular menstrual cycles in women | |
How do you store and dispose of the medicine?
- Storage: Keep the tablets out of the reach of children and pets . Store at room temperature (between 20-25 degrees Celsius). Do not use expired medicine.
- Disposal: The best way to dispose of expired or no longer needed medication is to take the pills out of the container, mix them with something like used coffee grounds, soil, or cat litter, put them in a tightly sealed bag, and throw them in the trash. Do not flush medications down the toilet unless your doctor tells you to.
Take-Home Message
- Always take ORKAMBI (Lumacaftor; Ivacaftor) with a fatty food such as butter, cheese, or eggs.
- This medicine is for you only. Do not share it with anyone else.
- Inform your doctor about all other medications, vitamins, and herbal remedies you are taking.
- Seek immediate medical attention if you experience serious side effects such as vision changes, difficulty breathing, or pain in the upper right side of your stomach.
- Keep your scheduled clinic dates and tests with your doctor.
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