Meet Malea Wilson, a dedicated teacher, IgCares Patient Educator, and someone navigating the challenges of living with primary immunodeficiency (PI). Read on to explore her journey through PI treatment.
A Childhood Shadowed by Persistent Illness
I was that child who seemed to catch every bug, consistently bringing home various illnesses. My mom would take me to the doctor, and the response was often, “She’s just a kid; kids get sick. It’s just an ear infection. It’s just strep.” From frequent ear infections as an infant to the removal of my tonsils and adenoids at five, the pattern shifted to sinus infections and migraines. A perpetual cycle of health issues defined my childhood. My parents, trusting in medical advice, didn’t question further, and we adapted to a life marked by constant illness.
Upon moving to Chicago for college, my health took a turn for the worse. Living in dorms, training to be a teacher, and being exposed to various people increased my susceptibility to infections. It wasn’t until my senior year that I consulted an allergy and immunology specialist. Unfamiliar with immunologists, I assumed my frequent illnesses were linked to new allergens in the environment. The specialist, suspecting primary immunodeficiency (PI), initiated tests and administered a pneumonia vaccine. Ironically, I contracted pneumonia within six months, a strain I had been vaccinated against. Antibody tests confirmed their absence, providing the first concrete validation of my PI diagnosis.
Embracing the PI Diagnosis
While battling pneumonia for several months, the confirmation of my PI diagnosis brought relief. At that time, there were numerous unknowns, and I had no knowledge of PI or anyone living with the condition. Support groups never crossed my mind, and it took four years to realize there were others facing similar challenges. Initially, like many newly diagnosed PI patients, I followed my doctor’s guidance without question. The doctor connected me with a specialty pharmacy, marking the beginning of my infusion treatment.
Exploring Treatment Options: A Journey of Medication Trials
Discovering the right medication for my condition was a process of trial and error, involving numerous attempts with different drugs. Initially, I was placed on SCIg infusion, but the first product proved ineffective. Despite adjusting the dose, my health didn’t improve, and I experienced headaches with declining numbers. Transitioning to my second infusion product seemed promising initially, but I encountered two severe adverse reactions consecutively, leading to emergency room visits. One reaction manifested as extensive site swelling and pain, while the other hinted at the onset of aseptic meningitis. Considering a potential issue with the batch, we attempted a second round with the same drug, yielding the same negative outcome.
Subsequently, I switched to a third drug administered through both SCIg and IVIg modes. Although I began with SCIg, my neurological issues necessitated a substantial dosage, prompting a shift to IVIg. However, the demanding infusion schedule over four days each month, coupled with persistent headaches and the need for additional medications like prednisone, proved unsustainable for my full-time work commitment. Despite the option to infuse on weekends, the treatment’s time demands clashed with my lifestyle. Thus, I reverted to SCIg administration.
Initially successful with the first SCIg medication post-switch, my positive experience faced challenges due to supply issues and recalls. Using a drug designed for both IVIg and SCIg exposed me to availability risks, creating heightened concerns and instilling a sense of nervousness in my treatment journey.
Embracing Success with Cutaquig
When I discovered cutaquig, a recently introduced treatment option, I expressed my interest in giving it a try. Engaging in an informed discussion with my doctor, who values my proactive approach, we decided to explore this new avenue. Fortunately, I am blessed with a supportive and responsive healthcare partner who allows me to take the lead in managing my treatment journey. Unlike many patients navigating a new primary immunodeficiency (PI) diagnosis, I have learned to ask questions and actively participate in my disease management over time.
Some physicians might hesitate to prescribe a relatively new drug, opting for a more familiar option with known approval likelihood. However, I proposed cutaquig as my preferred choice and received the necessary support to make the transition.
Cutaquig has proven highly effective for me, providing over a year of virtually issue-free experience, including a notable absence of illnesses and infections. What sets it apart is its seamless integration into my daily life. As a full-time 7th and 8th grade English teacher, I can even infuse while teaching during remote sessions without anyone noticing. The success I attribute to cutaquig lies in its meticulous manufacturing process, designed by Octapharma to potentially minimize site reactions. While a touch of luck may play a role, my infection rate has significantly decreased since starting cutaquig.
Navigating cold and flu season without falling ill has been a remarkable achievement, allowing me to lead a fully normal lifestyle. Juggling a full-time teaching position, a part-time role as a Patient Educator for Octapharma, and pursuing a Master’s degree simultaneously, I have successfully managed a demanding schedule while being exposed to a multitude of interactions daily. This stark contrast from my childhood experiences is a testament to the positive impact cutaquig has had on my life.
Words to Live By
I have discovered that knowledge is key to effectively managing both your treatment and your life. Here are some valuable insights I’ve gained along the way:
You are Your Own Best Advocate
- Know your body better than anyone else.
- Don’t hesitate to push back and ask questions.
- If your healthcare provider isn’t comfortable with this, find one who is willing to work collaboratively.
- Be an active participant in your treatment.
Be Prepared for Timelines
- Diagnosis can be a prolonged process.
- Authorization for specific treatments may take up to a month.
- Understand that improvements won’t happen overnight; medications take time to take effect.
Cultivate a Support System
- The disease can be isolating.
- Utilize online resources to learn from others’ experiences and discover helpful tips.
- Join supportive groups like IDF and engage in their events.
- Explore resources like IgCares for information, support, and community.
Proactivity and Self-Reliance
- Keep a supply of emergency medications at home.
- Thoughtfully equip your living space, considering unforeseen circumstances like a pandemic.
- Establish an effective storage and organization system for your supplies.
Maintain Self-Discipline in Therapy
- Adhere to your infusions and stay compliant.
- Plan infusions around your needs and schedule for flexibility.
- Effective planning and preparation enable a more adaptable schedule.
Live and Enjoy Your Life
- Primary immunodeficiency (PI) is not a death sentence; view it as a temporary challenge in life’s journey.
Cautions and Crucial Safety Details for cutaquig® (Subcutaneous Human Immunoglobulin – hipp, 16.5% solution)
Alert: Blood Clotting
- The potential for thrombosis exists with immune globulin products, including cutaquig.
- Factors contributing to the risk may encompass advanced age, prolonged periods of immobility, hypercoagulable conditions, a history of venous or arterial thrombosis, use of estrogens, presence of indwelling vascular catheters, hyperviscosity, and the presence of cardiovascular risk factors.
- In cases where patients are susceptible to thrombosis, it is advisable to administer cutaquig at the lowest feasible dose and infusion rate. Adequate hydration should be ensured in patients before the administration of cutaquig. Thorough monitoring for signs and symptoms of thrombosis is crucial, and blood viscosity should be assessed in patients at risk of hyperviscosity.
Cutaquig® (Immune Globulin Subcutaneous [Human]-hipp) is a 16.5% immune globulin solution designed for subcutaneous infusion (IGSC). It is indicated as a replacement therapy for primary humoral immunodeficiency (PI) in both adults and pediatric patients aged 2 years and older. This encompasses various conditions, such as common variable immunodeficiency (CVID), X-linked agammaglobulinemia, congenital agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies.
Important Safety Information
Cutaquig is contraindicated in individuals who have experienced anaphylactic or severe systemic reactions to the subcutaneous administration of human immune globulin or to any components of cutaquig, including Polysorbate 80. Additionally, it is contraindicated in IgA-deficient patients with antibodies against IgA and a documented history of hypersensitivity.
Warnings and Precautions
Severe hypersensitivity reactions are possible with cutaquig, even in patients who have previously tolerated human immune globulin treatment. If a hypersensitivity reaction occurs, discontinue the cutaquig infusion promptly and initiate appropriate treatment. IgA-deficient patients with anti-IgA antibodies are particularly at risk of severe reactions.
Thrombosis is a potential complication following the use of immune globulin products, including cutaquig. In patients susceptible to thrombosis, administer cutaquig at the minimum dose and infusion rate feasible. Ensure adequate hydration in patients before administration and closely monitor for signs and symptoms of thrombosis. Assess blood viscosity in patients prone to hyperviscosity.
During and after the infusion of cutaquig, falsely elevated blood glucose readings may occur with certain glucometer and test strip systems. When administering cutaquig, measure blood glucose using a glucose-specific method.
Aseptic meningitis syndrome (AMS) can occur with cutaquig. AMS has been reported following intravenous and subcutaneous administration of human immune globulin and may manifest within 2 days post-treatment. Discontinuation of immunoglobulin treatment has led to remission within several days without sequelae.
Acute renal dysfunction/failure, acute tubular necrosis, proximal tubular nephropathy, osmotic nephrosis, and death may occur with human immune globulin use, particularly those containing sucrose. cutaquig, however, does not contain sucrose. Monitor patients for signs and symptoms of renal dysfunction and assess blood urea nitrogen, serum creatinine, and urine output in patients at risk of acute renal failure.
Closely monitor cutaquig recipients for clinical signs and symptoms of hemolysis, especially in patients with pre-existing anemia and/or cardiovascular or pulmonary compromise. If signs and symptoms of hemolysis are evident after cutaquig infusion, consider appropriate confirmatory laboratory testing.
Non-cardiogenic pulmonary edema may occur in patients administered human immune globulin products. Monitor for pulmonary adverse reactions, including transfusion-related acute lung injury (TRALI). If TRALI is suspected, conduct appropriate tests for the presence of anti-neutrophil antibodies in both the product and the patient’s serum. Manage patients with TRALI using oxygen therapy with adequate ventilatory support.
Cutaquig, derived from human plasma, carries a potential risk of transmitting infectious agents, such as viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
Interactions with Other Drugs
Following the administration of cutaquig, a temporary increase in various passively transferred antibodies in the patient’s blood may lead to false-positive serological test results, posing the risk of misinterpretation.
The predominant adverse reactions (occurring in ≥ 5% of study participants) included local infusion site reactions (such as redness, swelling, itching), headache, fever, dermatitis, asthma, diarrhea, and cough.