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Expert Opinions

Expert Opinions

Last week, my uncle was admitted to a large teaching hospital in Royal Oak, Michigan for a blood clot in his leg, extending up into one of his pelvic veins. My cousin notified me of his admission. She asked my advice on what treatment would be appropriate for him. Sounds simple enough, right? Standard treatment for a blood clot should not be so difficult. Wrong!

My uncle, as well as my brother, suffer from a rare illness called HHT: Hereditary Hemorrhagic Telangiectasia also known as Osler-Weber-Rendu syndrome. The disease afflicted my grandmother and father, too. Simply stated, one of the problems associated with this disease is spontaneous bleeding from capillary beds in the nose, mouth, and gastrointestinal tract. Patients typically die of acute hemorrhagic anemia. My brother has had several severe nose bleeds. As a result of one, he has passed out and needed emergent transfusions and cardiopulmonary resuscitations in the hospital. I study HHT treatment. I do not have the disease. I have watched specialists treat my family, ultimately performing procedures that made the condition worse. These specialists tend to fly by the seat of their pants since they rarely see HHT patients in their practices. My grandmother succumbed in the emergency room of the same hospital my uncle is in, after an intravenous infusion of two units of packed red blood cells. I told the physician on the phone not to give her blood; it would kill her. But he said to me that since I was not bedside, he would do what he thought was best. After she died, that physician did not communicate with me in any fashion. He was ignorant about her disease and profoundly ignorant of the effects that two units of blood has on the body in general.

In addition to my uncle’s HHT, he also has survived lung cancer and is currently in the throes of battling Hertel Cell cancer of the Thyroid. He takes chemotherapy every day.

His cancer condition, as well as his chemo, makes him prone to form blood clots. His current medical event is his first blood clot. Anyone can see that fully anticoagulating him would most likely be disastrous. Except for the specialists who are caring for him. Ignorance is Bliss.

I told my cousin not to allow them to implant a Greenfield Filter surgically. She said they floated that idea to my uncle. I said, “No Way!” I also told her to stop them from giving my uncle any of the newer drugs for anticoagulation such as Xarelto. The standard drug warfarin would be too difficult to control in him: No to warfarin also. He would have to do low molecular weight heparin, probably a subtherapeutic dose. I told her I would call Mayo clinic to get the opinions from Dr. Iyre, a world renown expert in HHT and hopefully affirmation of my thoughts.

I was lucky enough to speak to Dr. Iyre. He was not available when I contacted Mayo Clinic in Rochester, Minnesota. The secretary of his department took my number; he called me back. He was kind, soft-spoken, and straightforward about his advice. He affirmed my thoughts about treating the blood clot after telling him the ins and outs of my uncle’s condition. He said he would be available to the physicians by phone in Royal Oak. The drug of choice to treat my uncle outpatient was Lovenox at 1 mg/kg per day. One dose in the morning.

When I texted my instructions to my cousin, she relayed them to the physician-in-charge. They took issues with my directions. I received a call from an oncology fellow in the hospital. She was a foreigner and spoke very little English. The conversations began. She became frustrated with me; I grew frustrated with her. Back and forth we went, identifying the risks of my uncle’s condition. She probably thought I did not understand the complexities of his situation. I held my ground. She then gave the phone to the Pulmonologist in charge of my uncle’s care.

He identified himself so swiftly that I could not catch his name. He had a mid-eastern accent. English was not his first language. He immediately asked me what published evidence-based data did I have to justify an approach to my uncle that would give him subtherapeutic anticoagulation when he has a fresh clot in his leg and pelvic vein. I was not surprised at his attitude. I answered, “There are no studies to evaluate the proper therapeutic dose of anticoagulation in patients with a fresh blood clot in his leg with complicating factors of HHT and Hertel cell cancer and undergoing chemotherapy at the same time. My Uncle’s case is a complicated, rare event. He is an N of one.” I would not allow him to take control of the conversation by intimidating me. I asked, “What’s your evidence-based data?” He backed up, “I have none.”

I told him I spoke to Dr. Iyre at Mayo Clinic. Dr. Iyre affirmed my thoughts and concluded Lovenox would be the best outpatient treatment. The specialist did not recognize Dr. Irye’s name. How would he? He is an HHT specialist at Mayo, not a pulmonologist in Detroit. The specialist was hesitant to give my uncle the drug at the dose I requested. He was worried about the clot, not my uncle’s nosebleeds. Both could end his life, the clot was less likely to cause mortality, but a profuse nose bleed when he is fully anticoagulated would most likely end his life.

At that moment, I got a bit enraged. I can’t remember the exact words, but I told him I would not permit him to kill my uncle. He could call experts in the field and get advice like I had. He refused to contact anyone. That just didn’t go well with me. I increased my voice intensity and forcefulness. I told him his hospital physicians killed my grandmother by acting in ignorance in her case of HHT, that would not happen with my last remaining uncle. I was firm. I was authoritative. And he finally said “Fine. We’ll do it your way. He must see a doctor at Cleveland Clinic in follow-up. They have an HHT center.” I responded, “Did you call them to consult?  Speak to anyone there? Get a doctor’s name?” He replied, “No. I do not consult on the phone. I’m done here.”  He gave the phone to the female physician as he walked out of the room. He was angry. The woman on the phone said, “Everything you want be done. Thank you.” She gave the phone to my cousin. The battle was over. My cousin told me to calm down.

I spoke a few minutes with my cousin. She did not understand the dynamic. The pulmonologist was treating me like I was an ignorant medical student who could not grasp the seriousness of my uncle’s blood clot. He was unaware of the issues associated with HHT stating, “What’s a nosebleed going to do? He hasn’t had a GI bleed.” He did not know my uncle had a colonoscopy looking for a GI bleed. The suspicion of GI bleeding is always a factor in HHT patients.

My uncle is home recovering. He is taking injections of Lovenox daily. His nose is not bleeding. I need to follow up with him soon to make sure all is well. One thing to recognize about illness and rare disease, not all specialists are created equal, nor do they have a desire to do the right thing. Many have the desire to do their job which may not include contacting an expert in an illness with which they are unfamiliar or ignorant. If they cannot have a rational conversation about a treatment plan for a complicated medical problem, find another specialist.

A specialist whose righteousness is higher than the patient’s need for the best, most appropriate care is a dangerous doctor.

Who is on your list of specialists in your wallet?

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