Thrilling Case Study: Myasthenia Gravis Crisis, Renal Kidney Failure, Diabetes, UTI and Hypertension In One Patient!

“Amor Fati” – “Love Your Fate”, which is in fact your life. I had never met a person who was an embodiment of this particular quote by Friedrich Nietzsche until I did a case study on one of my patients in the surgical ward. I was scanning the board in the surgical ward which displayed the names of patients along with what they were admitted for. I decided to write up my case study on a patient whose left kidney was catheterized for the removal of pus. From a surgical point of view, I thought the history would be short and I’d be done soon so that I could get back to the medical ward where the rest of my colleagues were preparing their case studies.

Mrs. Naheed was eager to provide me information regarding her health when I told her I wanted to look into her case. With each successive question, she revealed more and more diseases. When I was done asking the necessary questions, she laughed and said – ‘I’m such a good patient, aren’t I?’ I asked what she meant by that and she cheerfully remarked, ‘Well look how many diseases you’ve seen in me! Isn’t that going to help increase your expertise?’ All I could do was smile and tell her that she was the bravest person I had ever met.

The Story

Naheed was around 20 years old when she suddenly developed difficulty in brushing her hair or getting up once she sat down. Next, water started regurgitating through her nose whenever she drank. She could not swallow well and started experiencing mild respiratory distress. These symptoms were not constant and were never too severe, only appearing during times of physical stress. When she turned 24, these symptoms suddenly aggravated and she could neither eat nor drink because food simply wouldn’t go down her throat and water would regurgitate through her nose.

Myasthenia Gravis Crisis Begins

She consulted a doctor and was diagnosed with Myasthenia Gravis. What is Myasthenia Gravis? This is a disease of the immune as well as the musculoskeletal system. The body’s immune system targets its own acetylcholine receptors or muscle specific kinase. Acetylcholine is a substance required for neuromuscular transmission of nerve impulses and without it, muscular activity is hampered. On the other hand, muscle specific kinase is required for the formation of the neuromuscular junction, the unit which is responsible for transmission of impulses from the nerve to the muscle so that contraction can be initiated.

Patient with Myasthenia Gravis

When its receptors are blocked, it cannot act and thus, a state of decreased or absent muscular movement ensues. The muscle groups most susceptible are the muscles which control eye movements, facial expressions, talking, chewing and swallowing. Less susceptible ones include those responsible for breathing and the muscles of the limbs.

Myasthenia Gravis is graded according to the number and type of muscle groups affected. Its hallmark is muscular weakness during times of muscle activity and consequent relief when one rests. Interestingly, medical science has no answer as to why the body begins to attack its own receptors or why particular muscle groups are affected.

You’re probably wondering why I explained this neuromuscular problem since I mentioned earlier that Mrs. Naheed had a kidney problem. Just hang in there folks! The story has just begun. As I explained, Myasthenia Gravis is an auto-immune disorder. So the logical treatment is to suppress the immune system so that it stops attacking the muscular nerve endings. Oh but wait! Did I just say we have to suppress the immune system? Wouldn’t that cause even more harm? Of course it would! With an almost non-functional immune system, a person can develop the most lethal types of infections which are usually go un-noticed in normal people since their immune system fights it all off.

However, steroids (which suppress your immune system) were started to control her myasthenic symptoms. This was successful. When Naheed turned 38, she developed hypertension and was soon started on anti-hypertensive drugs. When she turned 39, she developed Diabetes Mellitus, type II, and treatment was begun for this as well. (Read more about diabetes management guidelines if interested)

At the age of 40, she consulted her doctor with complaints related to none of the above mentioned problems. This time, she had increased urgency and frequency of urination, along with an unpleasant burning sensation during urination. The associated lower left flank pain was increasing and she got high fever. To those of you not concerned with medicine, this story makes no sense at all, does it? What does the neuromuscular problem have to do with the hypertension or Diabetes or the symptoms of urinary tract infection? Could they all be related? How did that all lead to her kidney problem?

Wait a second..Something’s missing!

None of the above questions can be answered without the addition of one major symptom which was unfortunately discovered at the very end of her ordeal: She was born with a constriction in her left ureter. In a normal individual, a small amount of constriction (such as in this case) would go unnoticed or cause very mild urinary tract infections on and off. Once your kidneys form urine, the ureters which connect the kidneys to bladder, carry the urine into the bladder.

Any constriction within this pathway causes reflux of urine and greater chances of infection, the degree of constriction being proportional to the severity of infection. In a healthy person with an intact immune system, mild infections are fought off easily and do not cause considerable distress or long-term harm.

In case of Mrs. Naheed, the symptoms of urinary tract infection started to appear when she was married at the age of 20 (that’s the time she got married and in her country of origin, i.e, Pakistan, physical relations start at this time, usually with just the spouse, and are an established risk factor for U.T.Is). These symptoms started worsening suddenly when her medication for Myasthenia Gravis was begun. These medications, amongst others, included steroids.

Steroids: Treating one, damaging another!

Steroids are produced by the body in times of stress and suppress the immune system and help the body handle stress. Their release is tightly modulated and thus, when given as a medication, this delicate balance is thrown into disarray. Mrs. Naheed’s immune system had to be suppressed to stop the antibodies which weren’t letting her muscular function remain normal. It is crucial to control this disease not because it causes limb weakness but because it affects respiratory muscles and those involved with swallowing, therefore putting the patient at risk of developing severe respiratory failure and obviously, difficulty while eating.

The former puts the patient at high risk of sudden death. Therefore, despite their deleterious effects on the body, steroids have to be given. Other immune-suppressants are also available but they have worse side effects and cannot be given for a long time. Getting back to our story, Naheed’s symptoms of U.T.I became more recurrent but only became markedly distressing for her when the steroidal dose had been considerably increased, which was 20 years after her steroid therapy was initiated (the body becomes resistant to small doses so their dose has to be increased successfully to achieve the same effect).

It’s fascinating that her with mild doses of steroids, her immune system was functional enough to counter the infections but suppressed enough to stop the Myasthenia. The recurrent urinary tract infections caused damage to her kidney which eventually added up to produce mild hypertension which was diagnosed when Naheed was around 38. When she was 39, she was diagnosed with Diabetes.

This could either be coincidental or due to the Myasthenia Gravis because latest research has proven that patients with this disease are at greater risk of developing Diabetes Mellitus type II (since that is also an auto-immune disorder). In Diabetes, the glucose which is normally taken up by the cells of the body remains in the blood because the body loses its ability to use glucose.

This glucose is food for bacteria which grow so massively that the immune system cannot fight them off. In a patient with an already existing auto-immune disease who is being given drugs that suppress the immune system, there is literally no hope of fighting any infections successfully.

Let’s put it all together:

Here’s the scoop:

• Naheed was born with a constriction in her left ureter and had mild symptoms of U.T.I on and off.
• She developed Myasthenia Gravis at the age of 20.
• She was given immune-suppressive drugs for treating the Myasthenia.
• Her U.T.Is started increasing in frequency (not severity).
• The recurrent mild U.T.Is caused renal damage and hypertension (15 years after steroidal therapy was started).
• She developed Diabetes type II 16 years after the initiation of steroidal therapy for the Myasthenia.
• The Diabetes caused a sudden increase severity as well frequency of the U.T.Is
• Her renal functions declined markedly and over a period of 8 years, she lost her left kidney. Renal kidney failure, that is.

Did she really have to lose her kidney?

Why did she lose her kidney? Couldn’t she be treated for it? Of course she could! But she wasn’t just born with a ureteric defect with acquiring an auto-immune disorder when she was only 20, she was born in a third world country. That is a place where even reaching the right doctor gets very hard sometimes. Here’s why she lost her kidney:

• Her major problem was the ureteric defect. If that had been treated (by a very simple surgery), she wouldn’t have had the recurrent infections. Without these, her kidney would be just fine. This problem was diagnosed when she was 47 – 8 years after she started getting clinically significant U.T.Is. Before that, she never noticed considerable symptoms which would make her consult a doctor.
• She didn’t pay much attention to her symptoms since she wasn’t educated and lived in an area far away from a good health center.
• She considered the symptoms of the U.T.Is a minor problem and decided not to go far away to see specialists, like she had when she was diagnosed with Myasthenia, Hypertension and Diabetes (which were much more serious diseases in her opinion).
• She was diagnosed to have pus in her left kidney – something which could be surgically treated but in her case; surgery was not an immediate option since her Myasthenia Gravis was an absolute contraindication to general anaesthesia which is required during major surgeries. However, the high risk surgery was eventually attempted successfully when her left kidney became a harbour of a large amount of pus – something which put her at great risk of widespread bacterial spread into her body and eventual death.

Some would blame doctors, others would blame her. Simply said, one single factor cannot be the root problem of this issue – multiple factors were at play. Having been in her position, I don’t think I’d be able to accept life if I were to live it that way. But like they say, you’re never given more than you can handle, she was coping with it so well that she definitely had no problem with the way her life had turned out to be.

Amor Fati after all!

I asked her how she could be so happy and she said: ‘ I have 3 healthy kids and I have lived to be almost 50 – doctors say I can live just fine for another 20 years with my right kidney. I’ve seen many people who don’t even have that. I’m thankful for all that I have. Everyone has one problem or another; I guess mine is this variety of diseases. It’s good you talked to me and took my history, see you learnt so much!’

I don’t know how many diseases I learnt about, but I do know she taught me to love what I have. Amor Fati after all!