Have you ever felt a little scared about the anesthesia you're given for an operation? It's normal for some people to feel that way. But imagine what would happen if some of the anesthesia drugs you're given weren't exactly right for your body. Today we're going to talk about one such rare, but very important condition to be aware of. That's a condition called `(Pseudocholinesterase Deficiency)`.
What is `(Pseudocholinesterase Deficiency)`? Let's understand it simply, shall we?
Okay, this name sounds a bit long, doesn't it? Let's keep it simple. `(Pseudocholinesterase)` is a special enzyme `(Enzyme)` in our body. One of the main functions of this `(Enzyme)` is to break down (that is, `(metabolize)`) in the body some of the drugs that are given to relax muscles , especially when we are anesthetized (we call these `(Choline esters)`). Imagine, during an operation, your muscles need to be paralyzed for a while. These are the drugs that are given for that.
Now, in a condition called `(Pseudocholinesterase Deficiency)`, what happens is that this `(Enzyme)` called `(Pseudocholinesterase)` in your body is either not produced enough , or it is not functioning properly . What happens then? The drugs that are given to relax the muscles (`(Succinylcholine)` and `(Mivacurium)` are two examples of this) are not removed from the body quickly. They stay in the body for a long time.
As a result, your muscles may remain numb for a long time after anesthesia, and you may even have difficulty breathing. This means that you may not be able to move or breathe on your own until the effects of the medication wear off.
How common is this condition?
Actually, this condition called `(Pseudocholinesterase Deficiency)` is not that common . On average, only about one in 3,200 or 5,000 people in the population experience this condition. That is, a very small number.
What are the symptoms? How do you recognize this?
Most people with this condition don't know it until they receive the type of anesthetic drug I mentioned earlier, called ``Choline esters.'' That's when the main symptoms appear:
- Muscles that are excessively relaxed (i.e., lifeless) for a longer period of time than would normally be expected.
- Even the muscles that help you breathe become temporarily inactive .
It may take a few hours for these symptoms to subside. This time varies from person to person. During this time, your medical team will keep you under anesthesia and help you breathe through a ``ventilator'', an artificial breathing machine, until you are able to breathe on your own.
What are the causes? How does this happen?
This condition (Pseudocholinesterase Deficiency) can be inherited (i.e. inherited), or it can develop later in life .
Inherited causes
People who are born with this condition have a change, or mutation, in a gene called `(BCHE)`. This `(BCHE)` gene is what tells our body to make the enzyme `(Pseudocholinesterase)`. So, when there is a change in this gene, either your body produces very little of this enzyme, or it may not produce it at all.
Causes that develop during life (Acquired)
Sometimes, this condition (Pseudocholinesterase Deficiency) can occur throughout life. It can be caused by various diseases and certain medications.
Some diseases that can cause this condition:
- Malnutrition
- Severe burn injuries
- Kidney disease
- Liver disease
- Hypothyroidism (underactive thyroid)
- Cancer
- Pregnancy
Some medications that can increase the risk of this condition include:
- MAO inhibitors (e.g. Phenelzine (Nardil®))
- Birth control pills (Oral contraceptives)
- Echothiophate eye drops
- Metoclopramide
- Cyclophosphamide
- Pyridostigmine
How do doctors diagnose this?
If you have difficulty controlling your muscles or breathing after general anesthesia, your doctor may suspect pseudocholinesterase deficiency. To confirm this, they can do a blood test to check your levels of the enzyme pseudocholinesterase.
If someone in your family has this condition, a doctor can do genetic testing to find out if you have it too. This involves taking a blood sample and checking to see if you have the (BCHE) gene mutation.
If your doctor suspects you have this condition, they will use other types of muscle relaxants that do not rely on the enzyme ``Pseudocholinesterase''.
What is the treatment?
If you develop symptoms of `(Pseudocholinesterase Deficiency)` after you have been given general anesthesia, you may need urgent medical attention. The medical team will monitor you closely until you recover. If necessary, they will keep you under anesthesia and use a `` mechanical ventilation`` machine to help you breathe until you can breathe on your own.
What should you expect if you have this condition?
If you are diagnosed with `(Pseudocholinesterase Deficiency)`, the best thing you can do is inform your doctors about it – especially the doctors who will be seeing you for surgery .
Also, you should tell your family about this so they can be tested for the `(BCHE)` gene mutation. If so, they should also inform their doctors before any surgery.
It's also a good idea to wear a medical ID bracelet so that doctors can know if you need to have emergency surgery.
Can this be prevented?
Pseudocholinesterase deficiency is a congenital condition that cannot be prevented. However, if someone in your family has it, you can be tested for the BCHE gene mutation before undergoing general anesthesia. This can help prevent complications during surgery.
If you have the genetic mutation, your doctor will likely skip the Choline esters and choose a different anesthetic. Rocuronium is the most commonly used anesthetic for people with Pseudocholinesterase Deficiency.
You may be able to reduce your risk of developing Acquired Pseudocholinesterase Deficiency later in life by treating underlying conditions, such as malnutrition or chronic kidney disease (CKD). Also, if you are taking any medications that reduce the production of the enzyme Pseudocholinesterase, you may want to talk to your doctor about changing your medication.
What are the contraindications, that is, what medications are not good to use for people with this condition?
If you have been diagnosed with `(Pseudocholinesterase Deficiency)`, you should avoid the following types of anesthetic drugs :
- Succinylcholine `(Succinylcholine)`
- Mivacurium
- Procaine
- Chloroprocaine
- Benzocaine
- Tetracaine
Questions to ask your doctor
If you have a condition called ``Pseudocholinesterase Deficiency'', whether you were born with it or developed it later, you may want to ask your doctor some questions like:
- What are the exact contraindications for me, that is, what medications should I avoid?
- Is this condition something I was born with or is it caused by another illness?
- Is it a good idea to have genetic testing done on my family?
Going into surgery can be a bit stressful. Many of us are so focused on the operation that we don't even think about the possibility of an adverse reaction to the anesthesia. There is no cure for Pseudocholinesterase Deficiency. However, knowing whether you have it can help you avoid the temporary muscle weakness and breathing difficulties that can occur after surgery. If you have Pseudocholinesterase Deficiency, tell your family members about it. They can also get tested for the gene and avoid any unnecessary complications that may arise during their health journey.
Finally, things to remember
So, `(Pseudocholinesterase Deficiency)` is a bit of a scary name, but it is very important to be aware of it. Especially if you are going to have surgery, or if someone in your family has experienced something like this , it is best to talk to your doctors about this. Remember, awareness is often the best shield that can save us from unnecessary problems!
` Anesthesia, pseudocholinesterase, enzyme deficiency, surgical complications, genetic condition, muscle dysfunction, anesthetic drugs
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