We didn’t realize just how ill my mother was. As my mother walked into the emergency department, her shockingly low hemoglobin (Hb) level was 3.5 g/dL. Normal hemoglobin levels for a woman range from 12.0 to 16.0 g/dL. The nurse confided that she had never seen such low hemoglobin in a person who was conscious much less alert and ambulatory (however weak). The doctor said that such a low Hb level could cause a heart attack.
Hemoglobin is the protein molecule in red blood cells which carries oxygen throughout the body. According to the Mayo Clinic, "a low hemoglobin count is a common blood test result. In many cases, a low hemoglobin count is only slightly lower than normal, isn’t considered significant, and causes no symptoms. A low hemoglobin count can also be caused by an abnormality or disease. In these situations, a low hemoglobin count is referred to as anemia."
After an endoscopy, it was determined that bits and pieces of the large amount of Motrin which my mother had taken during the previous week for a case of sciatica likely got "stuck" in the intestines and burned small holes through the lining. The gastroparesis caused by my mother’s scleroderma is likely to blame for the Motrin’s lack of movement through the gastrointestinal track. It took four units (or one-half gallon) of blood to bring my mother’s hemoglobin level up to a safer 10.4 g/dL before she was released to go home.
An endoscopy is a gastrointestinal exam which allows the doctor to view the lining of the digestive tract. For an endoscopy, a flexible tube with a light and small camera is inserted through the throat to exam the esophagus, stomach, and duodeum (upper part of the small intestines). For a colonoscopy, a small camera is inserted through the rectum to exam the colon and large intestines.
Chronic Bleeding and Autoimmune Disease: GAVE Syndrome
My mother began to feel much, much better when her severe anemia was helped by the four units of blood she received during that first hospital visit in October 2010. However, during the following 12 months, she found herself in the hospital two more times to receive additional units of blood.
Something else was going on besides bleeding ulcers, but the doctors were not exactly sure what that was"¦…until her last hospitalization. After more testing with a traditional endoscopy and an endoscopy/colonoscopy conducted with a tiny camera which was swallowed, a new gastroenterologist diagnosed a condition called Gastric Antral Vascular Ectasia or GAVE syndrome.
Gatric antral vascular ectasia is an uncommon cause of gastrointestinal bleeding which is sometimes called "watermelon stomach" due to the appearance of the lesions found in the stomach. It can present symptomatically as iron-deficiency anemia, due to chronic slow bleeding, which is resistant to treatment with iron building strategies. Patients may become dependent upon blood transfusions.
The causes of GAVE syndrome are varied, but is often associated with systemic illnesses, such as cirrhosis of the liver, autoimmune connective tissue disorders, bone marrow transplantation and chronic renal failure. Some studies have indicated that approximately 60% of GAVE patients have an autoimmune connective tissue disease, particularly scleroderma, systemic lupus, and atrophic gastritis with pernicious anemia.
In a recent retrospective study of 264 patients diagnosed with scleroderma, 5.7% had been also diagnosed with GAVE syndrome. In another trial where asymptomatic scleroderma patients had to undergo endoscopy before entering the study, 10.8% of them had undiagnosed GAVE syndrome. Other autoimmune diseases which may be associated with GAVE include Raynaud’s phenomenon pernicious anemia, autoimmune hypothyroidism, primary biliary cirrhosis, polymyalgia rheumatic, and rheumatoid arthritis, according to a review article in the Journal of Rheumatology.
Management of GAVE Syndrome
Conservative symptomatic therapy includes iron supplementation, proton pump inhibitors, and blood transfusion if the anemia is severe and symptomatic (as my mother’s was). Some reports in the literature explore treatment with estrogen-progesterone combination hormone therapy. Patients with limited cutaneous systemic sclerosis typically do well with these conservative therapies.
Patients with diffuse cutaneous systemic sclerosis may be more difficult to treat and need recurrent endoscopic interventions and blood transfusions. One unique endoscopic technique is a series of treatments using argon gas to coagulate (or burn) the GAVE lesions. This is the approach which my mother’s doctors are now taking. She has had one procedure so far and will undergo a second procedure next week. The gastroenterologist expects that it will take at least four rounds to effectively treat my mother’s condition.
Our fingers are crossed that the argon gas treatments will stop the bleeding and greatly improve the quality of my mom’s health. Simply being able to maintain a normal hemoglobin level will help to eliminate one potential source of severe fatigue, weakness, and shortness of breath. In the meantime, it is wonderful knowing that blood transfusions are available when needed.
If you haven’t considered being a blood donor, please do so. Blood is needed not only for accident victims and patients in surgery, but for patients living with various diseases which affect their ability to produce and maintain sufficient quantities of certain blood components. As we say goodbye to National Blood Donor Awareness Month, please help to spread the word regarding the need for blood donors throughout the year.
To learn more about blood, organ, and tissue donation, read these posts:
(more posts to come)