New York, New York, United States
I remember my first nosebleed. It was the late eighties and I was at the age where it was still permissible for me to sleep on the floor of my parent’s bedroom. Ostensibly this was due to their superior air conditioner, but in truth I was afraid of the dark. My oldest sister, a perennial curfew breaker, entered my parent’s room just before eleven to clock with the warden. Roused by her entrance I laid momentarily in the limbo between asleep and awake trying to process the unfamiliar, metallic taste in my mouth. “Anthony!” my worried sister shouted, queasy as she stared at my bloodied pillow. My parents and I were momentarily startled by her shriek, but the sight of my pillow, while comically macabre, caused us no real alarm. It was clear what had happened. I had had a nosebleed. It was hardly a noteworthy occurrence. Half of my family experienced regular nosebleeds; some lasted minutes, others hours. They were just a normal part of life. Apathetically, I headed to the bathroom to clean myself up. “Are you OK?” my sister called after me, her voice slightly trembling. “Yeah,” I replied, somewhat annoyed with her concern. “It’s only blood.”
Though we did not know it then, my family carries the gene for Hereditary Hemorrhagic Telangiectasia (HHT), a rare genetic disorder in which malformed blood vessels lead to bleeding of the nose, GI tract, and other vital organs. In some cases, arteriovenous malformations (AVMs) occur in major organs. These vascular abnormalities can create serious medical complications. We had always accepted our nosebleeds as an inherited familial trait without understanding or questioning the root cause. Occasionally a doctor might notice the telltale telangiectasia (dilated blood vessels near the surface of the skin) on our hands and tongues and suggest that our nosebleeds might stem from something called HHT. Other than its name, the doctors rarely seemed to know much about the disease and never showed any signs of concern. Rather than seeking further information, my family continued blithely on, viewing our affliction more as a quirky inconvenience than a serious malady. This all changed in my teenage years.
My maternal grandmother, Nana, was the first to experience major complications from HHT. By the mid-nineties her nosebleeds had become nearly impossible to control. She was chronically anemic, which resulted in frequent periods of dizziness and disorientation. She began to require regular hospitalization to successfully stop the bleeding and frequent transfusions to compensate for the loss of blood. Eventually she became dependent on regular transfusions to maintain her quality of life; without them she would become weak, demented, and minimally responsive. Ultimately she was moved to a care facility that could accommodate her increasing medical needs. In 2004, after nearly a decade plagued by related medical issues, Nana passed away from complications due to HHT.
Nana’s swift decline was a shocking wake up call to the severity of this disease. Shortly after her death my aunt and uncle followed similar trajectories, ultimately dying from HHT as well. Though my family had begun amassing information about the disease, there was very little material on the internet and knowledgeable professionals were rare, remote, and expensive. My mother was dejected. She had watched hopelessly as her once vibrant family succumbed to a fate to which she herself felt inevitably tethered. It seemed she was next in line.
As time passed, my mother’s nosebleeds increased in severity. Though she managed to avoid hospitalizations and transfusions, we worried for the future. My family became more vigilant in educating ourselves, hoping to provide a more preventative and proactive approach to her care. HHT awareness was on the rise. An internet group called “CureHHT” consolidated information about current research and symptom management while also providing listings of HHT-savvy doctors. New information and resources were certainly encouraging, but the bottom line remained: there was no cure for HHT.
In 2015 Mom’s health began to show signs of steep decline. Familiar symptoms such as dizziness, weakness, and confusion all pointed toward acute blood loss, but much to everyone’s surprise, her blood counts were normal. Her symptoms got progressively worse until doctors eventually discovered that she was suffering from severe heart failure; her aortic valve would need to be replaced. Done laparoscopically, this would be a fairly standard, low-risk procedure, but as with most heart valve procedures, she would require blood thinners indefinitely. This created a devastating catch-22. The blood thinners could make it inordinately more difficult to ebb Mom’s already troublesome nosebleeds, but forgoing them would put her at frighteningly high risk for a stroke. The doctors provided arguments for and against the surgery and post-operative care, but were hesitant to take strong positions in a seemingly no-win situation. How to proceed was largely up to us. After much deliberation and consultation, we devised a plan in which she would have the valve replaced and then introduce a low level of blood thinners into her system while monitoring her bleeding. The dosage could then be slowly increased based on tolerance. The plan was by no means ideal. It was riddled with calculated risks, but all things considered, it appeared to be our best option.
The valve was successfully replaced. Mom was expeditiously released from the hospital and began taking blood thinners as planned. A week into the regimen she suffered her first post-operative nosebleed. The blood flow was heavy but stopped after about ten minutes. We were optimistic. It appeared that the blood thinners would not completely prevent her blood from clotting. Two weeks after the surgery, however, my sister got a distressed call from my mother reporting a problematic bleed. My sister was alarmed. My mother has always been something of a martyr when it comes to her health, rarely calling attention to her nosebleeds. She has developed the rather repugnant ability to, through a series of sniffles, redirect the blood to the back of her throat where it can be discreetly swallowed, concealing the nosebleed from onlookers. This phone call could not be good. My sister arrived fifteen minutes later to a horrific, gruesome scene. My mother, though still conscious, had collapsed to the floor. A substantial trail of reddish-brown blood traced her path from the couch, across the living room floor, to the threshold of the kitchen where she lay gasping and choking in a literal pool of blood. No longer possessing the strength to hold a towel to her nose, the blood poured freely down the side of her face and onto the floor as she painfully vomited up copious amounts of blood and enormous blood clots. Rather than wait for an ambulance, my sister found the presence of mind to get my mother’s nose packed and transported her to the hospital. I do not know how my sister had the strength to face what she faced that day. I like to think I would have handled it with the same amount of capability and grace. I pray I never have to find out. “Any time I thought I was going to be sick or pass out,” my sister would later report with a forced smile, “I’d just take a deep breath and remind myself, it’s only blood.”
Once at the hospital the doctors were able to stop Mom’s bleeding, but a transfusion was necessary. She had simply lost too much blood. Mom was devastated. She had always resisted transfusions, believing that the first would mark “the beginning of the end.” Following the transfusion, we decided it would be best to discontinue the blood thinners until her blood count was back to normal. The following day she had a stroke.
The next eighteen months were a blur of doctors’ appointments, medicine adjustments and cessations, nosebleeds, cauterizations, small strokes, hospitalizations, and rehabilitation. One day my mother said to me, “I don’t know how much longer I can do this. I have no quality of life.” My heart broke. “Please just hold on a while longer,” I begged, “I know it’s rough but I think we are making progress.”
Last week my phone buzzed. It was a text from my father. My body tensed. I opened the message to find a picture of my mom by a hotel pool. “She looks like the Duchess of York,” my father wrote. “We’re having a great time in Florida. Call you soon.”
Mom has been stable for almost two years. Her nosebleeds are severe but manageable and though stroke activity has ceased, she will always be at high risk. To date, she has had only the one blood transfusion and while the strokes have left her with memory and balance impairment, she shows improvement every day. We consider ourselves quite lucky.
This year I turned forty. With every drop of blood, I think about how HHT will affect my coming years. I try to keep a positive outlook and resist mentally condemning myself to a future of complications. The disease expresses itself in various degrees of magnitude in different individuals and I have every reason to hope and believe that my HHT will be less aggressive than that of my relatives. I keep up with new advances in treatment and eagerly hope for a breakthrough in the maintenance or cure of HHT, but until then, all I can do is hold a tissue to my nose and remind myself that it’s only blood.
ANTHONY CHIOCCHI received a bachelor’s degree in psychology from New York University. He worked briefly in research before pursuing his entrepreneurial dream of opening a restaurant. Currently he lives in Manhattan and spends his summers in the beach town of Cherry Grove, Fire Island where he owns and runs a seasonal bar and restaurant. Writing has always been a passion and he spends much of his free time working on fiction and personal essays.