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Unless It's Too Late

Unless It's Too Late

The medical industry seems to becoming more odd as I watch physicians and patient interactions from what many would call the cheap seats. There is an advantage to being in the cheap seats; you can see the entire field of play, watching the good and the bad as well as the right and the wrong. Unfortunately, the bad and wrong are prevailing over the good and right. Which means patients suffer.

A bright, vibrant, intelligent adolescent girl came into my office a few weeks ago under her own power. She was a bit unsteady while she walked into the office because of dizziness. The young woman was well acquainted with the pediatric medical machine in Memphis, since she had been evaluated by multiple general pediatricians and specialists for an array of emerging medical problems.

Her initial complaint was an unsteady gait and dizziness. She was on a dance team, had straight A's in school, was well adjusted by any measure and had great supportive parents who are married and had been since she was conceived. She had to stop dancing due to her illness, and eventually needed to use a walker because of her dizziness. After the dizziness, she developed severe abdominal pain. She was evaluated by pediatric gastroenterologists who diagnosed ulcers in her stomach and duodenum. Her H. Pylori test was negative, so the physician believed "stress" was a major factor in this young woman's disease. She lives a stress free life other than her dizziness and stomach pain.

The gastroenterologist prescribed the usual acid blockers and dietary changes. Although the ulcers seemed to heal up, her stomach pain worsened. She started developing muscle weakness: first in her legs. That's when I came into the picture. She had been evaluated by countless physicians and no one could find anything wrong, but this young woman was deteriorating. He parents asked me to evaluate her.

The first action I took was to review about a thousand pages of medical records. The pediatricians and pediatric specialists and sub-specialists had tested her blood for all sorts of rare and exotic illnesses. None of those tests were helpful in any way. Perhaps they needed to be performed just to rule out diseases that rarely occur. No one could explain her progression from dizziness to abdominal pain to muscle weakness. I first saw her navigate our office building with assistance, but under her own power.

Our blood tests showed she had a Mycoplasma Pneumoniae infection that was active as well as a few other disturbances. We changed her food plan, and initiated antibiotics. Within three weeks, her condition deteriorated so rapidly that she was only able to get around via wheelchair. First she was able to use her arms, then she was so weak in her upper extremities also that she could not power herself around. I saw her every other day, and her ability to breath was being compromised. So she went to the LeBohneur Children's Hospital in downtown Memphis.

I spoke to the emergency physician after he evaluated her. She was admitted to the ICU for observation, since her efforts to breath were feeble at best.

Once in the ICU, the critical care specialists, pediatric hospitalists and neurologists swooped in to save the day. After they made their evaluations, they discovered they did not know what was wrong with this young vibrant adolescent. She was an enigma to them. After the physicians had a conference, it was the neurologist's best guess that this child was experiencing a Conversion Disorder. He told the parents that the disturbance was psychological in origin and with proper counseling, she would get better.

Can you say, shocker? Perhaps shocking? I examined this young woman in my office for several days, and her neurologic examination changed rapidly for the worse. When the psychiatrist saw her in consultation, he dismissed the diagnosis of Conversion Disorder and related he thought her illness was organic not functional. Bottom line, the neurologist did not know what was wrong with her, so in his worldview, it had to be of psychological origin.

What is a Conversion Disorder? It consists of symptoms or deficits that develop unconsciously and nonvolitionally and usually involve motor or sensory function. Manifestations resemble a neurologic or other general medical condition but rarely conform to known pathophysiologic mechanisms or anatomic pathways. Onset is almost always attributed to a major stress event. A typical Conversion Reaction occurs abruptly, almost linked to a stress event. A patient might manifest a paralyzed leg or a painful arm. The diagnosis can only be considered after all disease possibilities that can fully account for the patient's symptoms and their effects.

I thought she had manifested a disturbance similar to or consistent with a variation of Guillian-Barre Syndrome (GBS), which is an acute, usually progressive inflammatory polyneuropathy characterized by muscular weakness and mild distal sensory loss. It is thought to be a result of an autoimmune reaction. Diagnosis is clinical. There are several variants of GBS. In sixty to seventy percent of the patients, the syndrome begins five days to three weeks after a banal infectious disease, surgery or vaccination. Infection is the trigger in over fifty percent of the patients; common pathogens include Campylobacter Jejuni, enteric viruses, herpes viruses including Epstein-bar Virus and Cytomegalovirus and Mycoplasma Pneumoniae. One of the interesting findings patients afflicted with GBS is that only sixty to seventy percent have abnormal electromyography (EMG). That means thirty percent may not show abnormal EMGs. 

The treatment of GBS is either an invasive procedure called Plasmapheresis or intravenous infusion of immune globulin (IVIG). I will not go into the pros and cons of each of the treatment options, but of the two, IVIG is my choice in her case.

When this young woman was admitted to the hospital, it trigger a host of consultations, all of these physicians disagreed about the diagnosis which naturally leads to no agreement about the treatment. It is as if she was a controversial bill in the US congress: no one can make a decision about what direction is best. If there is a therapy that might work, IVIG might be that treatment. Why?

It has been used in a number of illnesses with great success including GBS. The US Food and Drug Administration has approved the use of IVIG for the following conditions : Allogeneic bone marrow transplantation, Chronic lymphocytic leukemia, Common variable immunodeficiency (CVID) - A group of approximately 150 primary immunodeficiencies (PIDs) that have a common set of features (including hypogammaglobulinemia) but that have different underlying causes, Chronic inflammatory demyelinating polyneuropathy (CIDP) - Solely Gamunex, Kidney transplantation with a high antibody recipient or with an ABO incompatible donor, Primary immunodeficiency disorders associated with defects in humoral immunity, Immune-mediated thrombocytopenia, Kawasaki disease (see the Kawasaki Disease Diagnostic Criteria calculator), Hematopoietic stem cell transplantation in patients older than 20 years (Gamimune-N only), Chronic B-cell lymphocytic leukemia, Pediatric HIV type 1 infection.

Its reported off label uses have been: Aplastic anemia, Pure red cell aplasia, Diamond-Blackfan anemia, Autoimmune hemolytic anemia, Hemolytic disease of the newborn, Acquired factor VIII inhibitors, Acquired von Willebrand disease, Immune-mediated neutropenia, Refractoriness to platelet transfusion, Neonatal alloimmune/autoimmune thrombocytopenia, Posttransfusion purpura, Thrombotic thrombocytopenia purpura/hemolytic uremic syndrome, Conditions in which acquiring an infectious disease could be deleterious include low birth weight (i.e., < 1500 g), solid organ transplantation, surgery, trauma, burns, and HIV infection, Epilepsy and pediatric intractable Guillain-Barré syndrome, Chronic inflammatory demyelinating polyneuropathy, Myasthenia gravis, Lambert-Eaton myasthenic syndrome, Multifocal motor neuropathy, Multiple sclerosis, IVIG may be helpful for recurrent pregnancy loss, Asthma, Chronic chest symptoms, Rheumatoid arthritis (adult and juvenile), Systemic lupus erythematosus, Lupus nephritis, Systemic vasculitides, Dermatomyositis, polymyositis, Inclusion-body myositis, Wegener granulomatosis, Adrenoleukodystrophy, Amyotrophic lateral sclerosis, Behçet syndrome, Acute cardiomyopathy, Chronic fatigue syndrome, Congenital heart block, Cystic fibrosis, Autoimmune blistering dermatosis, Diabetes mellitus, Acute idiopathic dysautonomia, Acute disseminated encephalomyelitis, Endotoxemia, Hemolytic transfusion reaction, Hemophagocytic syndrome, Acute lymphoblastic leukemia, Lower motor neuron syndrome, Multiple myeloma, Human T-cell lymphotrophic virus-1–associated myelopathy, Nephritic syndrome, Membranous nephropathy, Nephrotic syndrome, Euthyroid ophthalmopathy, Opsoclonus-myoclonus, Recurrent otitis media, Paraneoplastic cerebellar degeneration, Paraproteinemic neuropathy, Parvovirus infection (general), Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome, Progressive lumbosacral plexopathy, Lyme radiculoneuritis, Rasmussen syndrome, Reiter syndrome, Acute renal failure, Thrombocytopenia (nonimmune), Streptococcal toxic shock syndrome, Uveitis, and Vogt-Koyanagi-Harada syndrome.

I did not list the diseases for which IVIG was used as treatment to impress you. I just wanted to show how many physicians had had the courage to be creative in their treatment approach to diseases that are classically treated with other methods.

What's holding everyone back from just treating her with IVIG? I do not know: Pride, insecurity, need to be right, do not know what to do, just ignore the illness and it will get better, it is out of the box, no data that it will work. I will let you be the judge of their decisions.

But I ask myself each day she's in the hospital and just unable to move: Why not? It's safe and the down sides are minimal. It's never too late to do the right thing. Never.

Unless it becomes too late.

Doc 

Posted by Amanda Sanders at 9:45 AM
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