People with Alport syndrome can develop chronic kidney disease, where the kidneys accumulate damage over time. This is due to an inherited mutation in an essential structural protein, called type 4 collagen, which forms a major part of the filtration unit of the kidneys.
Once damage has progressed past a certain point, end-stage kidney disease results — the kidneys can no longer function. The kidneys are essential to filtering excess fluid and waste products from the blood, among other tasks. As a result, once the kidneys fail a patient must undergo frequent dialysis to manually filter the blood.
Kidney damage from Alport syndrome is permanent, and currently, the only way to restore kidney function once the patient has end-stage kidney disease is through a kidney transplant.
Some patients may be eligible for an early, or pre-emptive, kidney transplant, before end-stage kidney disease develops, possibly avoiding the need for dialysis.
What is a kidney transplant?
A kidney transplant is a surgical procedure to replace the damaged kidneys with a healthy kidney from a donor. Since only a single kidney is needed, it can come from either a deceased organ donor or be donated by a living person.
Patients who do not have a living donor are put on a waiting list for a kidney transplant. It may take a long time before a kidney that is a suitable match to the patient becomes available.
Once the transplant has been done, the patient will have to take immunosuppressant medications to prevent the body from rejecting the new organ. This is an immune reaction against the new organ that can occur when the patient’s body recognizes the kidney as foreign and attacks it in an attempt to remove it.
Immunosuppressants dampen the immune response, making it less likely to target the new organ. However, these medications can have several side effects, and in many cases, the patient must continue taking them for as long as the new kidney is functioning.
The transplanted kidney may not start working immediately, which will require the patient to continue undergoing dialysis until they start producing urine as normal.
Kidney transplant outcomes in Alport syndrome
A kidney transplant is not a cure for Alport syndrome, and other symptoms of the disease, such as problems with hearing and eyesight, will not improve following a transplant. However, if successful, the patient should no longer need to undergo dialysis and complications associated with kidney failure and issues with blood clotting may be reversed.
Outcomes of kidney transplants in Alport syndrome patients are similar to those of patients with end-stage kidney disease from other causes, as demonstrated in a 2016 study published in Renal Failure. The study monitored 51 kidney transplant patients with Alport syndrome compared with 3,430 non-Alport kidney transplant patients. The 20-year graft survival, or the percentage of transplanted kidneys still alive and functioning after 20 years, was 46.8% in Alport syndrome patients and 30.2% in the other patients. Similar studies carried out in New Zealand and Turkey produced similar results.
The type of disease-causing mutation does not appear to affect this outcome, based on a study published in Kidney International Reports. Among 41 Alport syndrome patients, organ and patient survival rates were similar regardless of mutation.
Risks of a kidney transplant
Even with immunosuppressant medications, it is possible for the transplanted kidney to be rejected by the patient’s body. In general, the longer the transplant is successful, the lower the risk of organ rejection.
Symptoms of the body rejecting the transplant are not always obvious, since medications can reduce their intensity. Signs can include a fever, decreased urine output, swelling, weight gain, pain around the kidneys, and high blood pressure.
Patients should be monitored with routine blood tests to quickly identify early signs of rejection. By altering the medication, the rejection may be prevented if noticed early enough.
It is also possible for the transplant to fail if blood cannot reach the organ; for example, if the artery supplying the kidney is blocked by a blood clot or if arterial stenosis (narrowing of the artery) occurs.
All surgical procedures carry the risk of infection, and this risk can be increased by the immunosuppressants required to stop transplant rejection because they prevent the immune system from working normally.
Bladder or urinary tract infections can be more common following a kidney transplant, and patients should be closely monitored. Symptoms can include a burning sensation accompanying urination, and a frequent or painful need to urinate despite having little or no urine to pass.
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