My treasure (Draft)

This story starts in early February during my last few weeks working as inpatient wards senior on the neurology service. I was in the process of trying to figure out where to work next year after I finish residency. I exchanged emails every day regarding job offers and contract negotiations, as usual I was pretty tired all of the time.

A patient was transferred from a nearby hospital in Raleigh for evaluation of neuromuscular causes of shortness of breath. She was in her late 60s, and I will refer to her as Senora Alvarez. She was from Venezuela and spoke Spanish only. She reported that over the past 8 months she had progressively worsening difficulties with breathing. She felt weak and had gradual weight loss as well. Sometimes it was difficult for her to climb stairs and swallow food. At the outside hospital, she required a tracheostomy because of constant ventilator needs. She could mouth words, but needed to write things down to communicate with us. A percutaneous gastrostomy feeding tube was placed to provide her with nutrition. The neurologists in Raleigh had difficulties agreeing on her diagnosis. Her blood tests came back positive for acetylcholine receptor antibodies suggesting that she may have myasthenia gravis. She received a round of plasma exchange, and reports that she felt better. However in spite of this, somewhere along the lines, another neurologist had told her that she has ALS and would most likely be dead in 3 months.

Because of the uncertainty of her diagnosis, she was transferred to our hospital. She was small and frail, but her face was bright and she often wore a smile. She had fasciculations (muscle twitching) of her tongue and in her thighs. She had muscle wasting as well, most noticeable in her hands. Her reflexes were brisk. She was still able to swallow, eat, and walk, but she required a breathing machine often as her carbon dioxide levels in her blood were significantly elevated. My co-residents and I had no doubts that she had ALS, but there was disagreement among the neurologists at the outside hospital where she had an EMG that was not consistent with ALS. Afterall, her bloodwork suggested that she has myasthenia gravis and she had a good response to plasma exchange.

We obtained an EMG which had findings which were not classic for ALS. So then we obtained a nerve conduction study with repetitive stimulation along with a single fiber EMG. The electromyographer reported that there was likely 2 separate processes going on such as neuromuscular junction disorder and motor neuron disease, but the findings were not specific for any unique disease process. So this only added more uncertainty as to what was going on. The patient started to develop worsening hypercarbic respiratory failure and had to be briefly transferred to the intensive care unit for further ventilator optimization. We asked another neuromuscular specialist to evaluate the patient, and thankfully he agreed that she had ALS. Finally our suspicion had been confirmed.

The family had requested that we not discuss Senora Alvarez's diagnosis with her. They cried, but remained strong. They did not want Senora Alvarez to worry, and this seemed to be in keeping with there culture. They told me that the best possible outcome at this point would be to get her out of the hospital so that she could die at home surrounded by family. I told them that this was not an option, but that I would make sure to tell her when her family was with her. I also said that I would wait until later to discuss prognosis. Later that evening, 2 of her sons and her husband came by and I told her that she has ALS and she also probably has myasthenia gravis. She asked if there was a cure, and I said no.

I purposely did not try to overwhelm her with a grim prognosis. I thought it would be best to ease her into a reality which I'm sure she already saw coming. The next day we had another family meeting to discuss prognosis and goals of care. Just prior to this meeting, her sons showed me a video on his cell phone of Senora Alvarez dancing with her grand daughter several months prior. Her nurse, 2 sons, one daughter, the interpreter, and a medical student were all present. We discussed that she had ALS, and that this is the reason why she is so short of breath. We discussed that the natural progression of this disease is for her to become slowly weaker, and that there are no treatments available to reverse this process.

I did not tell her how long she had left. If I had to guess, she probably had anywhere from 2-10 months. We discussed what she would want to do if her heart were to stop beating, and she decided that she would want CPR to bring her back if this were to happen even though it my fracture her ribs.

I offered her IV IgG immunoglobulins or a second round of plasma exchange to treat what is most likely myasthenia gravis. We decided to do a treatment of IVIG but I do not think it helped. In my heart I think the only reason I had suggested it to her is to let her know that we had not given up on her.

We discussed that over time, the expectation is that she would continue to get worse, but that she would not be alone. She had a whole team of physicians, nurses, and therapists caring for her.  I told her that she would need the ventilator to live. She would always have the ability to tell us to make her more comfortable and help her transition on to death if she did not want some of the life sustaining measures we were offering her. However, given that Senora Alvarez still had the ability to walk, eat, and communicate, it may be too early for this. She had a large family who loved her very much.  She agreed and wrote down on her paper, "Yes, they are my treasure!"