FAQs About a Kidney Transplant for Fabry Disease

Fabry disease is a rare genetic disease characterized by the buildup of a fatty molecule — globotriaosylceramide Gb3 or GL-3 — inside cells, interfering with their function. The accumulation of this molecule can lead to kidney disease, among other problems.

The kidneys filter waste from the blood to keep us healthy. A buildup of Gb3 can prevent the kidneys from clearing waste, allowing waste products to build in the blood.  Gb3’s accumulation can also, in itself, permanently damage the kidneys.

Many Fabry disease patients manage their kidney symptoms with a combination of dietary changes and medications. But if symptoms are left untreated, kidney failure can occur. People in kidney failure need to be treated with dialysis or through a kidney transplant.

Here are a few FAQs and answers about kidney transplants.

What is a kidney transplant?

A kidney transplant is a surgery in which doctors place a donor kidney in your body to help ease the stress on your own kidneys. Generally, it is not necessary to remove the damaged kidneys.

There are benefits to getting a kidney transplant. You will be healthier and feel more energetic, and there will be fewer restrictions on your activities, though you will still need to follow a heart-healthy diet.

Drawbacks also are known. Aside from the risks of the surgery, as a transplant patient you will have to take medications to prevent your immune system from rejecting the donated kidney. You will have to be on these medications, which can make you more susceptible to infections, for the rest of your life.

Another consideration is that the donated kidney may not last forever. The length of time a transplant “lasts” is different for every person, but many people need more than one kidney transplant.

What is a preemptive or early kidney transplant?

A preemptive kidney transplant is a transplant given before you need to start dialysis. An early transplant, conversely, is a kidney transplant given when you are on dialysis, but before your kidneys have failed.

There is some evidence that getting a preemptive or early transplant can improve health outcomes following the transplant. However, studies have not been done specifically in Fabry disease patients to determine if preemptive or early transplants are of particular benefit.

Who can get a kidney transplant?

To be eligible for a kidney transplant, you need to be healthy enough to have the operation, as well as free of cancer and infections. A psychological evaluation is also necessary prior to surgery to make sure you are a good candidate for the surgery. Any problems that arise can usually be addressed prior to surgery.

If you are older or have underlying conditions, like diabetes, you may still be eligible for a kidney transplant.

How do I start the process?

Talk to your doctor. They can give you a referral to a transplant center in your area. Alternatively, you may be able to contact the transplant center directly.

The first stage of the process is an evaluation. Transplant center physicians will want access to your medical records, and they will require additional tests and scans to determine whether a transplant is the right treatment option for you, as well as to identify any problems that might make surgery high risk.

How do I find a donor?

If someone you know wants to donate a kidney to you, they will also have to be evaluated at the transplant center. They will undergo a physical screening process to make sure they are healthy enough for the surgery, as well as a psychological evaluation and counseling to ensure that they understand the long-term effects of kidney donation.

If you don’t have a compatible friend or relative who is willing to donate a kidney to you, you can be place on the national kidney transplant waitlist for a healthy kidney from a person who has died and donated their organs. It may take a long time for a compatible kidney to become available. Many patients need to begin dialysis while on a waitlist. You will also need frequent blood tests, making sure that you are ready for the transplant as soon as a kidney becomes available.

The transplant center will need to know how to get in touch with you at all times. Doctors must perform the surgery as quickly as possible when an organ becomes available.

What happens during surgery?

If you are receiving a kidney from a living donor, doctors will schedule the surgery at a time convenient for both of you, and perform both surgeries on the same day. If you are receiving a kidney from a deceased donor, doctors will perform the surgery as quickly as possible once the organ becomes available.

The surgery itself takes about four hours. You’ll be put under anesthesia (medication that numbs pain and makes you sleep) during the surgery. Doctors will place the new kidney in your abdomen, and connect it to your urinary tract and blood vessels. They will not remove your kidneys unless there is a problem with one or both of them.

What happens after surgery?

You will be sore after surgery, but you should be out of bed within a day or two. Most patients can go home within a week.

If your donated kidney comes from a living person, it should start working almost immediately. However, if you received a donor kidney from a deceased person, it may take longer to start working (up to a month). Many patients need to be on dialysis for a few weeks after the transplant surgery.

Your doctor will talk to you about the medications you need to take, and the diet you will need to maintain your health, and the health of your new kidney.

You will have frequent checkups for the first year after the transplant. These will include blood tests every week, for the first few weeks post-transplant, to make sure that your new kidney is working.

What are anti-rejection medications?

Anti-rejection medications are immune-suppressing medications that prevent your immune system from attacking the donor’s kidney. Without these medications, the donated kidney would not survive long after transplant. Because these medications suppress the immune system, you will need to be careful about infections.

What happens if my body rejects the new kidney?

The blood tests will tell your doctor if you are rejecting the new kidney. Rejection is very rare now with improvements in anti-rejection medications, but it can still happen.

Watch for symptoms like pain, difficulty urinating, swelling over the kidney, and fever. Contact your doctor immediately if you have any of these symptoms. Having a rejection reaction doesn’t necessarily mean that you will lose the kidney, but you may need additional treatment.

How will a kidney transplant affect my sex life?

Many patients find that their sex life improves after recovering from the surgery. There are also indications that male fertility may be increased following transplant surgery.

Women who have had a kidney transplant can become pregnant, but it is very important that pregnancies be planned. Most doctors recommend that women wait at least one year after kidney transplant surgery to become pregnant. Some medications  can be harmful to the developing fetus, and must be stopped at least six weeks before conception.

Last updated: July 24, 2020

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Fabry Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health providers with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.