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The focus of the Rheumatologist during treatment of Pearl's illness was the secondary effects of taking high doses of prednisone for a long period of time. His obsession with this appeared to have contributed to undertreatment of GCA most of the time - insufficient prednisone was the result. Pearl's quality of life I believe would have been significantly improved had the GCA inflammation been managed to maintain normal C-rP and sed rate without the distraction of potential side effects. Those serious effects that she experienced were generally corrected by additional therapy - medicinal or surgical. Some secondary effects that people can experience fortunately were not by Pearl. I will list mainly those she did experience and the impact or corrective measures that were taken to remedy or tolerate the condition.
Fluid retention : This was controlled by use of Lasix pills - about two a week were found to be most tolerable and effective. Since potassium can increase blood was monitored; seldom was increased potassium a problem for her though there were some anxious periods of intense consultation.
Hypertension : Blood pressure became elevated significantly during the first year on prednisone. The Cardiologist managed this using the combination of an ACE Inhibitor, a Beta Blocker, and a Calcium Channel Blocker. The Beta Blocker we learned later was a potential contributor to leg arteries constriction but the Cardiologist insisted that it was good for her so she continued to take it at his direction. Pressure was maintained at a low normal level through most of her illness; her heart remained strong and functioned well. She endured no strokes or aneurysms.
Muscle weakness _ Pearl’s muscles continued to weaken throughout her illness. Attempts to get her to exercise were not successful because of her general weakness. In the earlier years she tried water exercises and short walks, but later she was too debilitated. After loss of shoulders function due to torn rotator cuffs and other shoulder joint damage her options were reduced even more. Several times during her illness her legs buckled from under her. In her final months she had open skin scrapes to deal with so she was transported in the house with a wheeled chair which I pushed. She recovered sufficiently after the wounds healed to walk by herself once again but we were concerned about her stability – her right knee tended to buckle on occasion without warning.
Loss of muscle mass - This became evident as the years on prednisone went by. It was disappointing for her that her arms and legs were losing mass while her torso, face and neck retained the bloated Cushingoid appearance.
Osteoporosis - Pearl was placed on bisphosphonates and calcium supplements with Vitamin D as soon as prednisone therapy was started. She developed osteoporosis in the first couple of years of her illness. As prednisone was reduced her bones improved to the point that in the later years of illness the diagnosis had improved to osteopenia. This is a condition between normal bone density and osteoporosis.
Vertebral compression fractures - A flaw in a vertebra was observed in a bone density scan done in 2003. A subsequent spine xray showed that some compression had taken place in the vertebra, causing a slight kink in the spine, but it was more of a curiosity item since she suffered no ill effects or symptoms from the condition.
Aseptic necrosis of femoral and humeral heads - The only bone that fits this category for certain is the left shoulder blade which was diagnosed as necrotic and fractured into two pieces. The humerous joints of both shoulders were affected by PMR or arthritis to some extent. Significant dissolution of Pearl’s left clavicle had occurred – these conditions became apparent after her rotator cuffs were torn in prescribed physical therapy early in 2004. X-rays and MRIs were used to determine the extent of damage present. The left shoulder was deemed inoperable while the right could be repaired to eliminate pain but not function., though not until inflammation was gone.
Peptic ulcer with possible perforation and hemorrhage - Stomach ulcers were a constant threat because of the 18 or so prescription medications Pearl generally was taking during her illness. She had GERD and irritable bowel syndrome before the illness so her GI tract was monitored closely and medicated. Pepcid, used before her illness, was eventually replaced by a proton pump inhibitor; that was replaced during her final months with Carafate to allow use of methotrexate for steroid-sparing. She had several upper endoscopies to monitor her stomach and throat – all showed no ulceration.
Pancreatitis - Throughout her illness pancreatitis was not mentioned nor signs of it observed. The word first came up in the final hospital ER visit when the doctor advised me early in the morning shortly after admittance that Pearl couldn't receive corticosteroids because she was treating Pearl for severe pancreatitis. Pancreatitis is diagnosed when lipase and amylase are elevated - they hadn't been measured until two hours after Pearl went into shock, 12 hours later. So the diagnosis of pancreatitis that morning appeared to be speculative rather than based on facts.
Abdominal distention : Swelling of the stomach was always present but generally was thought to be due to the bloated torso effect of the prednisone. I don't know if there is any significance to this term other than general swelling. Her liver condition and other stomach organs were always considered to be normal by specialists who on occasion felt her stomach or performed biopsies.
Impaired wound healing : This occurred only one time and in that case it was due to insufficient prednisone, not because of the prednisone. The occasion was ulceration of her right foot that developed after leg artery blockages were observed and prednisone dosage was decreased instead of being increased. Hyperbaric oxygen (HBO) treatments were successful in assisting the ulcer to heal. Future ulcerations were aided by increasing prednisone. HBO therapy was never needed again though she suffered many skin scrapes and additional foot ulcers.
Thin fragile skin : Pearl continually was bothered by skin tears, some from so slight an abrasion as wiping her skin after a shower with a bath towel. Her skin was always very tender : if I brushed her with a finger it felt to her like I had scratched her with my fingernail. A skin scrape or wound would occur in almost every test she had done where people would have to touch her in the process. She used copious amounts of over-the-counter body lotions for skin firming and conditioning on much of her skin daily.
Petechiae and ecchymoses : Purpura (large flat red spots from bleeding under the skin) was a constant condition during Pearl’s illness. It was aggravated when Plavix was administered starting in 2000. Wherever Pearl was touched or pressed a red spot would form within minutes. These generally were benign but at times were disconcerting. They eventually were absorbed and disappeared within a few days.
Vertigo : Occasional bouts of vertigo were experienced. These were normally traced to a medication however there might have been some that were prednisone-related. But for those few instances vertigo was not a significant problem during her illness.
Headache : Pearl had significant headaches in the two months before diagnosis with GCA. Once therapy started the headaches disappeared. They re-appeared as prednisone dosage was less than needed to suppress symptoms and inflammation. During the last several years of her illness Pearl took Darvocet N 100 three times a day to manage pain that was mainly in her shoulders and legs pain but included headaches too.
Development of Cushingoid state : Within a few months after prednisone treatment began Pearl took on this typical appearance, gaining about 30 pounds. She recovered some of her normal appearance in 2002 but a return to higher doses of prednisone brought back the Cushingoid appearance. The bloat was severe enough to cause protrusion of internal body parts through her vaginal opening on a few occasions – this was a temporary condition that corrected itself within a week or so without special treatment or surgery.
Secondary adrenocortical and pituitary unresponsiveness, particularly in times of stress, as in trauma, surgery or illness : This condition was present because of long-term prednisone use. I believe lack of supplemental hydrocortisone when in the hospital was the cause her death. I couldn’t convince the hospital ER doctor of this danger until shock set in and recovery was impossible.
Manifestations of latent diabetes mellitus : While this was always a concern for us Pearl fortunately didn’t experience it. We knew it was a threat to prednisone users. Pearl was restrictive in her diet but did not avoid sugar–rich foods and candies entirely. Her weight was 120 pounds before diagnosis, so at 63 inches tall her body had been in the petite category. After gaining 30 pounds she continued to be petite. Her final weight was 10 pounds above her pre-illness weight.
Posterior subcapsular cataracts : Pearl had successful cataract surgery on both eyes in 2002. Her ophthalmologist stated the cataracts were not caused by prednisone.
Exophthalmos : Pearl had one occurrence of a bulging eyesocket. This occurred in the orbit behind her blind eye in 1999, a year after diagnosis of GCA. Subsequent increase in prednisone corrected the condition. MRI showed nothing other than the presence of inflammation.
Urticaria and other allergic, anaphylactic or hypersensitivity reactions : Pearl was continuously plagued by hypersensitivity reactions, mainly hives. She used Benadryl or increased prednisone to correct the condition. She had occurrences of angina - these couldn’t be traced to a heart condition but she took nitroglycerine pills on those occasions. She wore a nitroglycerine patch daily which helped to reduce the number of episodes. The Cardiologist considered these to be artery spasms. I don’t know if these were GCA-related, caused by hypersensitivity or due to some other unidentifiable cause.