We don't pay much attention to some of the small changes that occur in our bodies, do we? But sometimes those small changes, for example, something like a change in the appearance of your urine, can be a sign of a more serious health problem than you think. Today we are going to talk about one such rare, but very concerning kidney disease. This is called Complement 3 Glomerulopathy, or C3G for short.
Simply put, what is C3G?
Think of your kidneys as a super filter that cleans the blood in your body. Inside this filter are very fine, mesh-like structures called glomeruli. These are what filter the waste products from the blood and then excrete them as urine.
We all have an immune system in our bodies. It's like a defense army that fights germs that enter our bodies. One special force in this army is what we call the complement system. Normally, this force helps us, but in the body of a person with C3G disease, the function of this complement system is disrupted. Simply put, this defense army has a mistake, and they start attacking the delicate filter in our own kidneys.
This attack damages the filter mesh, and things like protein and red blood cells that should normally be in the blood start to accumulate in the urine. Over time, this damage increases and the function of the kidneys gradually decreases, eventually leading to kidney failure and the need for dialysis.
What are the symptoms of this disease?
Not everyone with C3G disease will experience the same symptoms. Some people may have several of these symptoms, while others may only have one or two.
| Symptom | This means... |
|---|---|
| Change in the appearance of urine | If your urine appears cloudy, foamy, or dark in color (red, pink, or brown), this is because protein and blood cells are present in the urine. |
| Body swelling | Swelling may occur, especially in the legs, ankles, hands, or around the eyes. This happens when the body is low in protein. |
| Joint pain | Joint pain can occur due to a malfunction in the body's immune system. |
| High blood pressure | When the kidneys are damaged, it also affects blood pressure control. |
| Frequent fatigue | When the kidneys are not working properly, waste products accumulate in the body, causing you to feel constantly tired and weak. |
| Frequent infections | A malfunction in the complement system can also weaken the body's ability to fight germs. |
| Eye problems | Some people may also experience vision problems. |
Let's learn about the new drug called Fabhalta (Iptacopan)
As a treatment for C3G disease, doctors usually prescribe medications that control blood pressure and reduce protein excretion in the urine (such as ACE inhibitors, ARBs). They also prescribe medication for high cholesterol. Sometimes, medications that control the activity of the immune system (glucocorticoids, mycophenolate mofetil) are also used.
However, Fabhalta is unique in that it is the first drug approved to reduce protein excretion in the urine of adults with C3G disease.
How does this medicine work?
Remember that confused defense system in our body (complement system) that I mentioned earlier? The drug Fabhalta blocks the activity of a special protein in that army called Factor B. To be precise, it calms down the confused army that attacks its own kidneys. This reduces the damage to the kidneys and controls proteinuria.
How should I use the medicine?
Fabhalta is a capsule that you take by mouth. It can be taken twice a day, with or without food. The most important thing is to swallow the capsule whole. Do not break, crush, or chew the capsule. If you miss a dose, take it as soon as you remember and then take the remaining doses as usual.
Studies on the effectiveness of Fabhalta have shown that people who take this medicine have reduced protein excretion in their urine by about 35% within 6 months. It has also helped maintain or improve kidney function.
Side effects and how to manage them?
Like any medicine, Fabhalta can have side effects. The most common side effects are symptoms of a cold, such as stuffy nose, runny nose , cough, sneezing, and sore throat.
However, there is one serious thing we need to be very careful about with this medicine. That is, because this medicine controls part of the complement system, there is a risk of serious infections caused by certain types of bacteria (Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae type B). These infections can even be life-threatening.
Therefore, you should be up to date with all required vaccinations (especially meningitis and pneumonia vaccines) at least two weeks before starting this medication. Your doctor will advise you on this.
| If you have these symptoms, see a doctor immediately! | |
|---|---|
| Fever (with or without chills), chest pain, cough, difficulty breathing, rapid heartbeat. | |
| Body aches and flu-like symptoms. | |
| Clammy skin. | |
| Persistent headache, fever, nausea, vomiting, neck or back stiffness. | |
| Feeling more tired than usual, confusion, and changes in vision. | |
Remember, these are not all side effects. Talk to your doctor about any symptoms that are bothering you.
Tests and other matters required during treatment
- Blood tests: While you are taking this medicine, your doctor will do regular blood tests to check how your body is responding to the medicine. Fabhalta may increase your cholesterol and triglyceride levels, so you will need to have these checked regularly.
- Interactions with other medications: Some other medications you are taking may affect the way Fabhalta works. Therefore, it is very important to tell your doctor about all the medications you are taking, including vitamins and herbal remedies. He or she can then decide on the best treatment for you.
- Special Safety Program: Because of the risk of serious infections with this medicine, it is given under a special safety program (REMS program). Your doctor will tell you about it and give you a safety card. It is important to keep the card with you throughout your treatment and for two weeks after stopping the medicine.
Take-Home Message
- C3G is a rare but serious disease that damages the kidneys due to a malfunction in the immune system.
- Fabhalta (Iptacopan) is a novel, targeted therapy approved to reduce protein excretion in the urine of C3G patients.
- There is a risk of serious infections when using this medicine. Therefore, it is imperative to get the necessary vaccinations before starting treatment.
- Notify your doctor immediately of any unusual symptoms or side effects. Be especially aware of signs of infection, such as fever and severe headache.
- You should never start, stop, or change the dosage of your medication without your doctor's advice.
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