Liver Transplantation for Alpha-1 Antitrypsin Deficiency: What You Need to Know

Introduction:

Alpha-1 antitrypsin deficiency (AATD) is very bad for your health. Among Europeans and Americans, it is very common. Our liver makes the alpha-1 antitrypsin enzyme, which protects our lungs from unnecessary damage. In AATD, a faulty production of alpha-1 antitrypsin (AAT) damages our lungs, causing breathing issues. Such problems appear at a young age. However, a defective AAT can also harm our liver. Such liver damage can be fatal, which can lead to liver failure. In such cases, a liver transplant can save a patient's life.

What Is the Need for Liver Transplantation for Alpha-1 Antitrypsin Deficiency?

Eligibility Criteria: As already discussed, AATD has dangerous effects on the lungs and liver. However, not every patient is suitable for a liver transplant. Only one percent of AATD patients require a liver transplant. If you are suffering from end-stage liver disease, then only a liver transplant is necessary for you. Such severe liver damage causes cholestasis (obstruction of bile flow in the liver), which leads to a jaundice-like condition. Gradually, such prolonged damage causes cirrhosis of the liver. Even in serious cases, this might lead to liver cancer. Eligibility for liver transplant in alpha-1 patients;

• Persistent jaundice (yellowing discoloration of skin) in children.
• The abdomen increases in size due to the gathering of fluid.
• Bleeding inside the gut.
• Increasing the size of the liver and spleen.
• Encephalopathy or brain problems.

Evaluation Process: If you are suffering from AATD, doctors usually perform a liver function test to understand the condition of your liver. However, this test alone is not useful in predicting the condition of the liver. The following tests are done to understand the condition of your liver;

1. Ultrasound and liver stiffness measurement through ultrasound.

2. Magnetic resonance imaging of the liver.

3. A computed tomography scan (CT scan) of your liver.

Surgical Procedure: Before the transplant process, doctors use immunosuppressive medicines to lower your immune response. This also lowers the chances of organ rejection. A liver transplant includes taking out the faulty liver and replacing it with the donated one. It consists of two primary steps: first, your damaged liver is removed, and then the donor's liver is placed in its place. There are two ways to change your liver.

The most common technique involves detaching the main vein leading to the heart and replacing it with the main vein from the donor liver, known as the inferior vena cava. Sometimes, doctors use a "piggyback" method, where they join the new liver with your main vein. Here, they cut all channels that carry blood to the liver along with the bile duct.

Post-Operative Care and Recovery: After the surgery, you need to stay in the hospital for seven to fourteen days. The first few days after surgery, you need a complete bed rest. After a few days' rest, you can start walking. Even after surgery, you have to take medicines like Cyclosporine or Tacrolimus to lower your immune response. More importantly, you should stay and eat healthily in the future.

What Are the Risks and Benefits of Liver Transplants in AATD?

1. Potential Complications: After surgery, you can see some issues. These problems are graft rejection. During a liver transplant, doctors put a healthy portion of the liver from the donor’s body into the patient's body. However, our body often identifies this as a foreign body and reacts like that. This causes rejection of the transplanted portion. This leads to serious complications like death. Doctors use immunosuppressive drugs to lower the body's reaction. This can cause lifelong difficulty due to lower immunity. However, recent research shows that in 15 percent of cases, such lifelong support is not required.

2. Immunosuppression: Even after a successful liver transplant, patients suffer from a low level of the body’s defense system. In AATD patients’ defence is already low, as a result after liver transplant, further reduction in immunity is seen. Patients often suffer from different infections due to low defense in the body.

3. Other Health Issues: You have to be serious about your health. You may see heart problems and high blood pressure. Also, it may cause kidney problems and nerve issues. Prolonged periods of immunosuppression can increase the chances of cancer.

4. Survival Rates and Long-Term Outcomes: Alpha-1 antitrypsin deficiency liver transplant success rates are very high. In the past, such operations used to fail due to a lack of modern things. If one of your children has undergone a liver transplant for AATD, the survival rate of the surgery will be 90 percent after one year. After five years, the survival rate for your child will be 78 percent. Even in cases of adults, the one-year survival rate is almost 89 percent. After five years, your survival rate as an adult will be 75 percent.

5. Quality of Life Post-Transplant: After a liver transplant, you can feel a little weak, tired, feverish, or a loss of interest in food. Low defense mechanisms in your body cause frequent infections. That is why you have to take care of your health and lifestyle.

6. Post-transplant Care for Alpha-1 Antitrypsin Deficiency: Wearing masks, avoiding alcohol and smoking, and eating healthy food, is important.

After a liver transplant, you slowly feel better. Your liver comes back to normal. Problems like digestive issues and jaundice also subside. You can also observe improvement in lung issues due to an increase in AAT level.

What Are the Alternative and Adjunct Therapies?

Now, doctors try to think beyond transplants. It is risky and costly, too. Moreover, as a liver transplant patient, you have to be careful throughout your life.

1. Emerging Treatments: Doctors are using gene therapy for AATD. In gene therapy, a healthy copy of the faulty or missing gene is added to the patient's body. Apart from this, gene therapy can move faulty or unwanted portions of the gene. In recent times, doctors have been using gene therapy to modify liver cells. These modified liver cells can replace defective liver cells in AATD. As a result, newly formed liver cells can improve the level of AAT in patients’ bodies.

2. Clinical Trials: As this is a new type of treatment, doctors are still experimenting with gene therapy. Most of the clinical trials are done on animals to date. Though the number of human clinical trials are very less, they show positive results.

Conclusion:

Alpha-1 antitrypsin deficiency liver complications can go really bad. Regular doctor visits are necessary to avoid any complications. Timely medical intervention, like a liver transplant, cannot only save your life but also give you freedom from this. But remember, even after the transplant, you have to look after your liver. You must stay healthy and eat healthily.

Key Takeaways/ Note From iCliniq:

Alpha 1 antitrypsin deficiency can have a bad effect on your liver. A liver transplant can be a true lifesaver for such cases. However, a tiny number of patients require liver transplants. Even after that, you have to be serious about your health. A proper and timely doctor visit can not only save your life but also minimize disease complications.