The White Man's Medicine Is Killing Me


The Mat, the Ta, the Po and the Ni are four rivers in Virginia. The Mat and the Ta join in Spotsylvania County to form the Matta, in Caroline County the Po and the Ni become the Poni. The Matta and the Poni converge to form the Mattaponi, one of the most pristine and beautiful rivers in the eastern US. The Mattaponi joins the Pamunkey at West Point to form the York River, which drains into the Chesapeake Bay just east of Yorktown.
Indigenous people, the original Americans, have lived in this region for about 15,000 years. Identifiable, distinct tribes date back 500-600 years. One of these tribes is the Mattaponi, who live in the region of the Mattaponi River and its tributaries. These Native Americans have lived in the region for a very long time and have a relationship with America that dates to Jamestown. Tribal leaders signed treaties in the 17th century with the early settlers of Virginia, and a reservation was created along the banks of the Mattaponi. The reservation now includes some housing, a church, a museum, a fish hatchery, a building that was once a school and tribal grounds.
The Mattaponi fared better than many other tribes in early America. They were not victims of a genocidal foot march to Oklahoma on the orders of an American president. Many heart wrenching accounts describe in agonizing detail the story of other tribes who fared much worse than the Mattaponi at the hands of the American colonists and their descendants in some of the darkest and most shameful chapters in American history.
Many Mattaponi continue to live in their ancestral territory and nearby parts of Virginia. These days, Mattaponi are pretty indistinguishable from other Virginians. There are few peculiarities of speech, dress or appearance to let a guy know when he is talking to a Mattaponi Chief or Princess. An occasional clue is a common Mattaponi surname, such as Custalow. I have run into a few residents and medical students who were Mattaponi. 
Per capita, Native Americans have served in the U.S. military more than any other ethnic group, including many Mattaponi. The Veterans Affairs hospital in Richmond provides care to many Native Americans who are military veterans, and one afternoon a Mattaponi Chief was referred to my VA Neuromuscular clinic because of muscle weakness. 
Chief had been experiencing muscle weakness for the preceding year or so. It began, as it often does, with trouble getting up from low places. He could no longer arise from a chair, or the couch, or get out of the car, with his normal ease and fluidity. When trying to get up he developed a hitch midway that required focus and some extra effort to complete the movement. As the condition progressed the hitch got longer and more of an additional thrust was required to stand. He attributed it to aging. Then he was no longer able to get up without pushing on the chair arms with his hands. When getting out of the car he would swing his feet out, then have to pull on the door frame and roof. He decided he should see a doctor. As he made the arrangements and waited for the appointment he noticed problems with his arms. The shoulders began to ache and give out before he could finish shaving or washing his hair. This history suggested a disorder of muscle, a myopathy.
He worked as a carpet installer and was by now having a lot of difficulty getting up from the all fours position where he spent a lot of his workday. The arm weakness made it more difficult to carry and position bundles of carpet. He became concerned about his ability to work and his livelihood. 
Chief was a Vietnam vet. Like many who served in that conflict he avoided talking much about it. In casual conversation he revealed he had been in the Army and spent a lot of time in the bush. Working at a VA hospital I had heard a lot of war stories. PTSD had recently been re-described and given a new name as the health professions recognized more and more Vietnam veterans with the same constellation of complaints: nightmares, flashbacks, anxiety and attempts to cope by drinking and drugging. 
But PTSD was not new with Vietnam. Soldiers thru the eons of human history have carried emotional wounds from combat. Herodotus described the psychic trauma of battle in ancient Greek warriors. Both Johnny Reb and Billy Yank suffered “irritable heart” from the American Civil War. The disorder was called shell shock in World War I and combat fatigue, battle fatigue, old sergeant syndrome or combat neurosis in World War II. An Army at Dawn, by Rick Atkinson, describes a twenty-year old-World War II infantryman in North Africa, the only survivor of a truck destroyed by a mortar shell, loping off into the night with a pair of suspenders in search of a tree from which to hang himself. Such is what combat can do to a man’s mind. The term PTSD was used to describe the disorder in Vietnam veterans. It's all the same. 
Chief never talked about it, but PTSD was one of the diagnoses in his medical record. Seeing him brought to mind jungles, destroyed villages, the cold-blooded execution of a Viet Cong soldier by an ARVN officer and 9-year-old Phan Thị Kim Phúc running naked down a road screaming from her napalm burns. All the tragedy and ugliness that was Vietnam. Long hair was common in the 60s and there were still a lot of old hippies around with ponytails. I figured Chief was just another old hippie. But his long hair was a Mattaponi custom, and he chose to honor it. His name was not Custalow; I did not make the connection. 
Chief's muscle biopsy showed he had polymyositis, an immune system mediated inflammatory attack on his muscles. Polymyositis causes progressive muscle weakness and is difficult to treat. Both the disease and the complications of treatment have potentially very serious consequences. When things go well, a patient with polymyositis is started on high doses of prednisone and the immunologic attack on the muscles is brought under control. The patient improves and regains lost strength, then the prednisone is slowly and carefully tapered off, lowering the dose bit by bit so the inflammation does not recur. Most patients are on prednisone for a year or more. That is when things go well. 
When things do not go well, the disease responds poorly to prednisone, or recurs when it is tapered, and other measures are required. Some of the other treatments include powerful immunosuppressants, such as methotrexate or azathioprine, compounds first used as cancer chemotherapy decades ago. These medications also have many side effects, most notably suppression of the bone marrow causing a profound depression of white blood cell and platelet counts. Intravenous immunoglobulin (IVIG) therapy involves infusion of immunoglobulins that can suppress the immune response. IVIG is usually well tolerated but complications can occur. There is little literature to support IVIG therapy in polymyositis and its use is generally a last resort.
Chief did not do well. His weakness did not improve and the muscle enzymes in his blood remained elevated. One reason a patient may do poorly is misdiagnosis. But neither the work up nor the biopsy had shown evidence of any other condition. He simply had steroid resistant polymyositis, an all too common and unfortunate condition.
Prednisone has a multitude of side effects, including weight gain, fluid retention, a predisposition to diabetes, osteoporosis, susceptibility to infection, psychosis, elevated blood pressure, cataracts and many more.  One of the characteristic side effects is swelling and fat accumulation in the face that can at times cause the face to take on a very distinctive, rounded shape referred to as a moon face. The skin becomes sallow and fragile with easy bruisability and fat accumulates behind the neck. When pronounced, the changes in physical appearance are so typical a physician can tell at a glance that a patient is on steroids. Chief had gained about 15 pounds, his face was puffy, his ankles were swollen and his blood pressure was up.
One of the most difficult aspects of managing inflammatory myopathy is that prednisone, the mainstay of treatment, can itself cause muscle weakness to develop. Determining whether persistent or recurrent weakness is due to such a steroid induced myopathy or is due to uncontrolled disease activity is no easy task. The stakes are high. Treatment of steroid myopathy is to lower the steroid dose; treatment of uncontrolled disease activity is to increase the dose.     
Chief showed little response to the prednisone. The weakness did not improve, the muscle enzymes remained high and other tests suggested continued disease activity rather than steroid myopathy. I started him on one of the big guns, azathioprine.  By this time the weakness was so severe he was unable to raise his arms up to shoulder level or to raise his knees against gravity. He had taken to a wheelchair. He was also having difficulty swallowing, a common manifestation of severe polymyositis. 
Some degree of bone marrow suppression and a decrease in blood counts is expected with azathioprine, but Chief’s white count and platelets tanked. Very low white counts predispose to infection; low platelets predispose to bleeding. I held the medication, sat tight and watched. The white cell count stayed in the tank. After several weeks off the medication the counts slowly returned to normal. I started him back on half the previous dose and the counts tanked again. He was not able to tolerate a dose of azathioprine high enough to control the polymyositis. There were similar complications during treatment with methotrexate.
So, Chief had not responded well to prednisone and had experienced steroid complications. Now he had developed side effects and was not able to tolerate azathioprine or methotrexate. We discussed his situation and he agreed to try IVIG.
IVIG has been a Godsend for neurologists. It is much safer than steroids and other immunosuppressants and several diseases respond to it, often quite well. It seems to control immune mediated disorders without suppressing the immune system and predisposing to infection. But there are no drugs without side effects and IVIG has some bad ones that are thankfully very infrequent. The major one is acute kidney failure, but the fluid load can also cause heart failure and rarely the hyper viscosity may cause a stroke or heart attack. All these we had discussed.
Chief was admitted to start IVIG. He tolerated the first infusion without difficulty but over the next couple of days his kidney function deteriorated. He was given some extra IV fluids. They did not help. Renal functions got worse and worse and his urine output began to fall. He went into frank kidney failure and had to start dialysis.
The nephrology team had gotten involved, and he was transferred to their ward for the dialysis. When I went by to see him it was during a dialysis session. He was laying on the dialysis recliner, a large needle in one arm, looking up with tired eyes from a puffy, steroid distorted face.  I asked how he was doing. 
He replied, “The White Man’s medicine is killing me.”
In the conversation that followed I learned he was Mattaponi, and that he was one of their Chiefs. We talked a little about the tribe and his leadership role in it.  I had known a little about the Mattaponi before, a lot more after.  
His renal functions gradually improved and the need for dialysis passed. He returned to clinic a few weeks later. Further IVIG was off the table. All we could do was continue the steroids and hope the disease would eventually respond or go into remission. He told me he had about given up on the idea of being able to walk. He just wanted to be able to crawl. As long as he could crawl around on all fours, he could lay carpet, at least part time, and earn a living more or less.
He began to miss scheduled appointments and after a while no longer came to clinic. He had given up on the White Man’s medicine. My last memory of him is of a proud Mattaponi Chief asking not if I could keep him walking, but if I could keep him crawling.