It might be challenging to get a prescription for infusion treatment. But additional worries are the last thing you need when you already have to cope with various medical problems.
What is infusion therapy?
When a medicine is injected intravenously as part of infusion treatment, it is sometimes referred to as IV therapy. A needle is injected straight into the patient’s arm. This makes it possible to treat chronic diseases more effectively since it can be used to inject medication, antibiotics, and hydration into the bloodstream. Consequently, there is a quicker absorption rate, and you might get relief more quickly.
Although infusions are often administered in a hospital setting, they may also be delivered at a patient’s home or doctor’s office. It’s crucial to educate yourself about what to expect, which will help to decrease your worry before your first infusion, which may seem overwhelming.
What to expect from IV therapy
Nurses and other qualified medical personnel give infusion therapies. They are competent at preparing the drug, making sure the dosage is accurate, placing the needle in your vein or port, observing the infusion rate, and assessing the development and symptoms during the therapy. Be sure to let your doctor—or the nurses on the day of your appointment—know if you have any questions or concerns regarding the IV process.
You can prepare yourself for infusion treatment by considering the following points.
Plan your schedule
Following each infusion session, the patient must take some time to relax. You might need time off for your body to recover properly if you have had adverse effects. Do not overwork yourself by rushing to go to work or complete other responsibilities.
Follow the instructions of the doctor
It is usually advisable to clarify any particulars or directions your healthcare provider wants you and your nurses to follow with them before your visit. With some therapies, healthcare providers may advise you to have specific blood tests or take particular drugs before receiving an infusion. Additionally, the doctor may suggest that you drink plenty of water before the infusion treatment. It helps the patient to feel less anxious, and the procedure will go more smoothly if a patient takes the time to discuss these issues with the doctor.
Bring essential items or food
Unless specifically excluded by your practitioner, most facilities permit patients to consume food or beverages while receiving treatment. It’s usually a good idea to bring food with you if your preferred hospital does not offer any food items, particularly if you have diabetes or other conditions that necessitate frequent meals or snacks. As a general guideline, it is a good idea to bring easy-to-pack foods or snacks with minimal-to-no odors, so you don’t bother any other patients who could be in the same room as you. It is also necessary to bring a blanket, as the body temperature increases with the infusion, which may make you feel a little colder.
Make a list of your medications
It’s a good idea to compile a note of any drugs you might be taking before visiting. By having this list ready in advance, you can remember to inform the nurses about any medications that can interfere with your treatment.
Wear comfortable clothes
Loose-fitting clothing will facilitate the process of taking vital signs by medical experts, which is essential. Medical experts will also make sure the patient feels comfortable and relaxed. A patient’s clothes can influence the degree of comfort he/she feels.
What is Tepezza?
Tepezza is used for thyroid eye disease (TED) to reduce symptoms. It is a prescription drug used to treat TED in adults. The immune system targets the adipose and muscular tissue behind the eyes in cases of TED. Double vision, eye discomfort, and eye redness may all result from this. Other names for TED include Graves’ ophthalmopathy, Graves’ orbitopathy, and Graves’ eye illness.
Before taking Tepezza
Inform your healthcare provider if you have an inflammatory bowel illness, diabetes, or hyperglycemia (high blood sugar) to ensure Tepezza is safe for you to use.
Tepezza may be harmful to an unborn child. Use reliable contraception to avoid becoming pregnant while using Tepezza for at least six months following your final dosage. In case of pregnancy, always consult your doctor.
Administration of Tepezza
An intravenous (IV) infusion of Tepezza will be administered, which is an extended injection into a vein (often in your arm). The injections of Tepezza will be every three weeks. The average Tepezza therapy lasts five months. The infusion can be administered in a doctor’s office, an infusion facility, or a hospital. Sometimes, you can get Tepezza infusions at home.
The generic version of teprotumumab is Tepezza. Biological medicine is prepared using components from live things, but there is no biosimilar version of Tepezza. Teprotumumab is exclusively available under the trade name Tepezza.
Tepezza minimizes double vision and bulging of the eyes. It also relieves Thyroid Eye Disease (TED) symptoms, such as eye discomfort, redness, and edema. In this way, Tepezza is a prescription drug used to treat TED. Your healthcare professional should discuss the advantages and disadvantages of taking Tepezza.
Warning, Precautions, and Safety Information for Tepezza
Infusion reactions from Tepezza might occur in 4% of patients receiving Tepezza who have reported experiencing infusion reactions. Reaction with Tepezza infusion might occur during or 24 hours following the infusion. With Tepezza infusion, if you experience an infusion reaction, your doctor or nurse will reduce or stop your infusion while treating the reaction. In severe cases of infusion response, your doctor may discontinue your therapy.
Infusion reactions that have been described have typically been mild to moderate in intensity. Transient rises in blood pressure, feeling hot, tachycardia, dyspnea, headaches, and muscle discomfort are a few signs and symptoms that may appear. Around 1.5 hours after an infusion, infusion reactions are possible. Recommendations can be made to pre-medicating with a corticosteroid, antihistamine, and antipyretic, giving all future infusions at a slower infusion rate in patients who have had an infusion response.
FDA Approval of Tepezza
The FDA approved Tepezza (teprotumumab) for treating individuals with thyroid eye disease. Thyroid eye disease is a rare disorder in which the fatty tissues and muscles behind the eyes become inflamed, pushing the eyes forward and outwards (proptosis). Tepezza was the first FDA-approved medication to treat thyroid eye illness that has been authorized.
Tepezza has been designated as an orphan medicine, which offers incentives to help and promote the production of medications for uncommon illnesses or ailments. The FDA Orphan Medicines Grants Program, which provides grants for clinical investigations on the safety and effectiveness of products for uncommon illnesses or disorders, also contributed to developing this medication.
It must be remembered that Tepezza should not be taken while pregnant, and women capable of becoming pregnant should have their pregnancy status checked before starting therapy. Such individuals should also get pregnancy prevention advice throughout treatment and six months after finishing Tepezza.
Side Effects of Tepezza
The side effects of Tepezza include dry skin, muscle spasms, nausea, fatigue, weakness, altered or diminished hearing, and muscle cramps. Inform your doctor or pharmacist as soon as possible if these side effects persist or become severe.
High Blood Pressure:
Increased blood pressure, a rapid pulse, feeling heated, a headache, and muscular soreness are a few examples of injection response symptoms. If you experience any of these symptoms, contact your doctor immediately. To reduce adverse effects, your doctor may advise you to take additional drugs (like acetaminophen and antihistamines) before each dosage.
Hair loss may be temporary due to Tepezza. After therapy, normal hair growth should resume.
Remember that your doctor has recommended this medicine because they believe it will help you more than harm you—several users of this medicine report no significant adverse effects.
Sometimes, severe side effects like bowel issues (such as diarrhea, bloody stools, stomach discomfort or cramps, or incontinence), menstrual cycle changes (such as missing or delayed periods, spotting), or other symptoms occur. In such cases, call your doctor immediately.
High Blood Sugar Levels:
The blood sugar level may sometimes increase due to this drug, which might aggravate or induce diabetes. In case of high blood sugar symptoms, like increased thirst or urine, immediately call your doctor. If you already have diabetes, check your blood sugar as advised regularly, and inform your doctor of the findings. Medication like antidiabetics, exercise routine, or diet may need to be changed by your doctor.
The medication may cause a very significant allergic response. Therefore, in case of any major adverse reaction symptoms, such as a rash, itching, or swelling (particularly of the face, tongue, or throat), severe dizziness, or difficulty breathing, get medical attention immediately.
Interactions of Tepezza
Drug interactions might alter how drugs function or raise the possibility of significant adverse side effects. Maintain a list of everything you take, especially herbal medicines, prescriptions, and over-the-counter medications, and provide it to your doctor and pharmacist. Without your doctor’s consent, never start, stop, or alter the dose of any medicines.
Fabrazyme is a prescription drug, and its generic is Agalsidase beta. To treat Fabry disease, it got FDA approval to be used in adults and children 2 years of age and older.
An uncommon hereditary illness called Fabry disease (passed down genetically through families). This condition is characterized by fat accumulation in the body brought on by a gene anomaly. This may cause symptoms that range from minor to severe and even fatal.
Indications for Fabrazyme
Patients with Fabry disease, a rare genetic condition, are treated with Fabrazyme. Many symptoms may be present in Fabry disease patients, including severe disorders, renal failure, cardiac issues, and stroke. The alpha-galactosidase is an enzyme that is insufficient in people with Fabry disease. Usually, this enzyme degrades a fatty molecule known as globotriaosylceramide (GL-3). Without this enzyme, GL-3 cannot be degraded and accumulates in the body’s cells, including kidney cells.
Because there are few people with Fabry disease, it is regarded as a “rare illness,” and Fabrazyme was named an “orphan drug” (a medication used in uncommon disorders) on August 8, 2000. Only those with a valid prescription may purchase the medication.
Approval of Fabrazyme
Fabrazyme therapy may provide long-term therapeutic advantages for people with Fabry disease. The Committee for Medicinal Products for Human Use (CHMP) was the decision. The CHMP determined that for long-term enzyme replacement treatment that in individuals with a confirmed diagnosis of Fabry disease, the advantages of Fabrazyme outweigh the risks. Fabrazyme was, therefore, recommended for commercial authorization by the board.
Fabrazyme was first approved under “exceptional circumstances” since there wasn’t enough information available at the time of approval due to the condition’s rarity.
Fabrazyme Mechanism of Action
Using Fabrazyme is a part of enzyme replacement treatment. Patients who need an enzyme are given it via enzyme replacement therapy. Alpha-galactosidase A, a human enzyme absent in those with Fabry disease, is replaced with Fabrazyme. Helped by “recombinant DNA technology,” the active ingredient in Fabrazyme, agalsidase beta, is created by a cell that has been given a gene (DNA) that enables it to generate the enzyme. The substitute enzyme aids in the breakdown of GL-3 and prevents it from assembling (accumulating) in the patient’s cells.
Agalsidase beta, an enzyme (a protein subclass), is an active component. This component works by substituting an enzyme either not created by your body or does not function properly.
Powdered Fabrazyme can be combined with a liquid to form a solution. A medical expert will administer the medication via intravenous (IV) infusion. There are two strengths of Fabrazyme: 5 mg and 35 mg.
The usual dose of Fabrazyme injection is every two weeks. The first dose is usually given as an infusion in a clinic or your doctor’s office. In the future, you may be able to have infusions from a doctor who visits your house.
Side effects of Fabrazyme
Severe Allergic and Hypersensitivity Reactions
During the infusion of Fabrazyme after its approval or the clinical studies, anaphylactic (allergic) or severe hypersensitive responses were seen in around 1% of patients. Patients have had potentially fatal extreme anaphylactic (allergic) or severe hypersensitive reactions when receiving Fabrazyme infusions.
The following are the allergic reactions that patients may experience while having an infusion of Fabrayzme.
The allergic reactions involved flushing, chest pain, irritation, nasal congestion, face, mouth, and neck swelling, constriction of the breathing airways, low blood pressure, hives, difficulty swallowing, rash, and breathing problems.
Individuals suffering from such allergic reactions need medical attention, such as heart and lung resuscitation, oxygen, intravenous fluids, hospitalization, and treatment with beta-adrenergic agonists, which help open the airways for breathing, antihistamines, and epinephrine (also called adrenaline), which aid in the reduction the body’s allergic reaction by reducing inflammation.
In such cases, doctors will immediately stop the Fabrazyme infusion and provide essential emergency medical assistance if you have a severe allergic or anaphylactic response. During your Fabrazyme infusion, the required medical care should be present due to the potential for serious hypersensitivity responses.
Formation of IgE antibodies due to Allergic reactions
In medical examinations, an allergic skin test response to Fabrazyme leads to developing IgE antibodies. The body’s immune system has been shown sometimes to generate IgE antibodies during an allergic response.
Adult patients with high antibody titers (units used to measure how much anti-drug-antibody your immune system produces) and those with anti-Fabrazyme antibodies were more likely to experience hypersensitivity reactions than adult patients with low antibody titers.
The doctor may want to test for IgE antibodies in case of allergic symptoms. The administration of Fabrazyme to patients who have had severe allergic reactions to Fabrazyme should only occur after considering the relevant hazards and benefits of continuing the treatment, only when under the close supervision of a licensed healthcare provider, and only when the patient has access to the necessary medical assistance.
The patients who have had adverse responses because of infusion of Fabrazyme are given anti-fever and antihistamine drugs before their subsequent infusions. In the case of using such drugs before their infusions, some individuals have experienced infusion-related problems.
If an infusion-related response develops, the symptoms may be alleviated by decreasing the infusion rate, pausing the infusion briefly, administering extra anti-fever, antihistamine, and steroid medicine, or any combination of these.
In such cases, a healthcare provider should usually discontinue the Fabrazyme infusion straight soon and should treat symptoms if you have severe infusion-related symptoms. The standard treatment for severe responses is to provide antihistamines, corticosteroids, fluids, and oxygen intravenously. Medical care should be accessible during your Fabrazyme infusion since serious infusion-associated events may occur.
Other Common Side Effects
Other common side effects due to Fabrazyme include burning or tingling feeling, upper respiratory tract infection, chills, fever, headache, cough, weariness, swelling in the legs, disorientation, and rashes recorded in 20% or more of patients.
Stelara, or ustekinumab, is a biological drug used to treat adults and children aged six and older with mild to severe plaque psoriasis, adults with moderate-to-severe psoriatic arthritis, and adults with moderate-to-severe Crohn’s disease and ulcerative colitis by reducing inflammation. After previous drugs have failed to work, this one is administered as an injection at home. IL-12 and IL-23, two inflammatory proteins, are blocked by Stelara, but a biological drug like this might take many months to start functioning. Long-term use of biologics is also common.
Stelara is indicated for treating plaque psoriasis and psoriatic arthritis—or both conditions—in adults and children who are at least six years old. Individuals with mild to highly active Crohn’s disease who have failed to respond to prior medications are prescribed Stelara, as are Adults who have moderately to severely active ulcerative colitis.
How to use Stelara
Injections of Stelara are given first, then again four weeks later, and every 12 weeks after that. In Crohn’s disease, it is administered as an injection under the skin, although it may first be administered as an infusion in the vein. Individuals under 100 kg (220 lbs.) get a 45 mg dosage, while those who weigh more than 100 kg receive a 90 mg dose. To lower the chance of infection, you must have a TB screening test before beginning this medicine. While using this drug, patients must see their doctor often for checkups.
Side effects of Stelara
Common Side Effects of Stelara
The common side effects appear when Stelara is injected; at the injection site, there can be bruising, itchiness, discomfort, redness, swelling, or skin hardening.
Reactions at the injection site often subside within one or two days. Back discomfort, sinus/throat pain, or headaches may also occur. Inform your doctor or pharmacist as soon as possible if any of these side effects persist or become worse.
This drug might impact the immune system. Your body’s capacity to fight an infection may be diminished. You may be more prone to severe conditions, like lung infections, bone/joint infections, skin infections, sinus infections, or bowel/gallbladder infections.
Additionally, fighting an illness you already have may be more difficult. If you develop any signs of disease, you must immediately consult your healthcare professional. Symptoms include nausea/vomiting that doesn’t stop, painful/frequent urination, aggravated tenderness and swelling, redness at the injection site after two days, fever/chills, cold/flu symptoms, unusual vaginal discharge, burning, itching, odor, or severe pain in the stomach.
Remember that your doctor has recommended this medicine because they believe it will help you more than harm you—many users of this medicine report no significant adverse effects.
Call your pharmacist and doctor immediately in the case of severe side effects, such as a cough that won’t go away or shortness of breath.
Serious Side Effects of Stelara
Stelara may result in PRES (posterior reversible encephalopathy syndrome), an uncommon (and sometimes deadly) disease. If you get a headache that won’t go away, seizures, abrupt eyesight changes, or mental/mood abnormalities, seek medical attention immediately.
With the use of Stelara, there is a minimal chance that you may get cancer, such as skin cancer. Consult your doctor about the advantages and disadvantages of the proposed course of therapy.
Call your healthcare professional immediately if you have symptoms like strange lumps or growths, unexpected skin changes, swollen glands, or unusual weight loss.
Effect of other drugs on Stelara
Stelara may interact with other medications, such as over-the-counter drugs, prescription drugs, supplements like vitamins, and herbal medicines. Inform your doctor about your existing medications and any new or discontinued ones.
Before starting Stelara, consult your doctor
Stelara could weaken your immune system and raise your chance of developing various infections. Some people using Stelara get severe infections like tuberculosis (TB) and infections brought on by bacteria, fungi, or viruses, which may need hospitalization.
Before beginning the Stelara medication, your doctor should test you for TB. They should also keep a careful eye out for any TB symptoms while you are on Stelara.
If your doctor believes you are at risk of TB, you could get TB therapy before and after taking Stelara.
If you are suffering from any infection, you must not take Stelara until your doctor gives the all-clear.
Before initiating Stelara, you must consult with your doctor if you have the following symptoms:
Red or painful skin
Shortness of breath
Blood in phlegm
Stomach pain or diarrhea
Warnings and precautions for Stelara
Never start Stelara when there is a clinically significant active infection. Consider stopping Stelara until the infection clears up if a major infection or clinically significant infection develops.
Theoretical Risk of Specific Infections:
Patients with hereditary IL-12/IL-23 deficiency have been linked to severe infections from mycobacteria, salmonella, and BCG vaccines. Consider diagnostic testing for these infections as warranted by the clinical situation. Before beginning Stelara medication, people with tuberculosis (TB) should be tested for the disease. Before giving Stelara, begin latent TB therapy.
The chances of cancer development increase with the use of Stelara. Its safety in individuals with a history of or currently being treated for malignancy has not been examined.
Anaphylaxis or other clinically severe hypersensitivity events might happen.
If you suspect PRES, get treatment at once and stop taking Stelara.
During post-approval use of Stelara, cases of interstitial pneumonia, eosinophilic pneumonia, and cryptogenic organizing pneumonia have been reported. If the diagnosis is validated, stop taking Stelara and start the appropriate therapy.
What is a Copay Accumulator?
A copay accumulator, also known as an accumulator adjustment scheme, is used to prevent manufacturer copay assistance coupons used by insurance companies and pharmacy benefit managers (PBMs) from being utilized to cover two prices:
the maximum amount you must pay out of pocket.
Pharmaceutical firms try to develop programs to cover patients’ out-of-pocket expenses. Some payers diminish the value of these programs by using up the funds allotted for them while also needing patients to cover their deductibles and co-insurance costs up to their out-of-pocket costs to get their medications.
Use of Copay Accumulator Program
A copay accumulator program alters the way an insurance provider administers and accounts for payments made, using a copay card from a prescription manufacturer. The copay card’s contributions often cover your deductible and other out-of-pocket expenses. It may protect you from having to pay considerable amounts out of pocket each year when you take a costly specialty drug, such as a biologic.
Although accepted at the pharmacy, the copay card’s contributions will no longer be used to cover your deductible and other out-of-pocket expenses if your insurance provider starts using an accumulator program. Instead, any payments made with your copay card will be sent directly to your health insurance provider, and not deducted from your deductible, or applied to your out-of-pocket expenses.
The copay accumulator program transforms the possible out-of-pocket expenses from $0 to now completely covering the deductible, followed by any co-insurance or co-pay amounts. You are left to bear the bill when your copay card expires and therefore make no progress toward meeting your yearly deductible.
People sometimes don’t notice the change until the copay card’s remaining balance is reached. They don’t realize their deductible and out-of-pocket limit have remained unchanged until this occurs. They must immediately begin paying down any applicable deductibles and co-insurance/copays until they have reached the plan’s out-of-pocket limit.
How to Address Copay Accumulators
Prepare for a surge in copay accumulator plans by anticipating it.
The benefit inquiry process extensively examines the patient’s plan to find any that contain an accumulator adjustment program.
Educate people when you notice they are enrolled in plans with an accumulator adjustment scheme.
Talk to patients about how a specialty drug may affect their insurance and how their financial obligations may change throughout their therapy.
Working on a Copay Accumulator Program
In the past, a person may have obtained financial aid from a medication manufacturer, and relying on the insurance plan, would have contributed against their deductible and out-of-pocket expenses. Pharmaceutical firms would often provide financial assistance to those with inadequate insurance in order to pay for pricey prescriptions. The individual purchasing the medication would ultimately save money—sometimes hundreds of dollars.
Where ASCO Stands on Copay Accumulators
According to ASCO’s Position Statement on Copay Accumulators and Copay Maximizers, the use of copay accumulator adjustment and copay maximizer programs for cancer patients is unacceptable. The statement also offers the following suggestions:
To further protect patients, federal and state governments should pass legislation that forbids copay accumulator adjustments and copay maximizer programs.
The Centers for Medicare and Medicaid Services (CMS) should outlaw the use of copay accumulator adjustments in the programs they administer and regulate.
Commercial insurers and PBMs should immediately stop using these programs.
Public and private insurers and PBMs should, at the very least, guarantee openness by outlining the copay accumulator adjustment program’s structure for users, as needed by the Centers for Medicare and Medicaid Services Summary of Benefits and Coverage Instruction Guide.
Copay Accumulator as an Evolving Policy
The use of copay accumulator and maximizer programs is growing, and recent legislative changes made via federal regulation and state legislation have raised additional questions about the future of the program and the interests of the organizations they touch.
The use of copay adjustment schemes, such as copay accumulator and maximizer programs, by payers and pharmacy benefit managers (PBMs) to reduce plan sponsor exposure to specialty prescription prices has increased in recent years. Although both programs utilize distinct tactics, they aim to use manufacturer copay assistance while keeping those funds from contributing to a patient’s commercial insurance deductible and maximum out-of-pocket limit.
According to a recent review of commercial insurers, 83% of participants are in plans with copay accumulator schemes, while 73% are in plans with copay maximizer programs. According to the data, 26% of the cost adjustment programs were for medical benefit goods, and 77% were for pharmacy benefit products. For payers, manufacturers, and patients, accumulators and maximizers provide new problems and issues. Since these programs are anticipated to expand in the ensuing years, recent regulatory and legislative action at both federal and state levels raises further concerns.
Implementation by Insurance Companies
Insurance providers and PBMs that have reportedly begun implementing these initiatives include:
Cigna Blue Cross Blue Shield
Various insurance providers and PBMs may use differing terminology for these programs. The one from Express Script is referred to as the “Out of Pocket Protection Program,” whereas United Healthcare refers to it as the “Coupon Adjustment/Benefits Plan Protection Program.”
The infusion-related reaction is characterized by an adverse response to the infusion of pharmacological or biological substances.
A drug that releases cytokines may produce an acute infusion reaction. Within 24 hours after the end of the medication infusion, signs and symptoms often stop developing. The common reactions due to infusion include lethargy, malaise, allergic reaction/hypersensitivity, arthralgia (joint pain), bronchospasm, cough, dizziness, dyspnea, fatigue, headache, hypertension, hypotension, myalgia (muscle pain), and nausea. Rigors and chills; Rash and desquamation; Pruritis and itching; Sweating; Tachycardia, urticaria, and vomiting.
Infusion or acute infusion reactions, like anaphylactic reactions, may happen during or shortly after the infusion. Patients must carefully identify the symptoms to classify them as an “infusion-related response” and distinguish between the phrases “infusion reaction” and “anaphylaxis.” Infusion reactions often refer to symptoms that appear shortly after receiving an infusion and are not always associated with hypersensitivity or anaphylaxis.
The common terms for infusion-related reactions include anaphylaxis, anaphylactoid responses, and cytokine release syndrome, which are frequent with monoclonal antibodies (mAbs) and promptly tied to drug delivery.
Common Signs of Infusion Reactions
The following are the common symptoms of infusion reactions.
Swelling of eyelids, tongue, lips
Respiratory issues, like shortness of breath
Notify your healthcare provider or infusion nurse as soon as possible if you notice any of the symptoms mentioned above while receiving an infusion. After your infusion, notify your healthcare team immediately if you experience these symptoms at home.
When to Call Healthcare Provider?
The doctor or team of medical professionals supervising your treatment will also monitor your vital signs, look for any infections that may still be active, and ask about your general health.
You must call a professional and highly skilled healthcare provider to handle any infusion reactions in case of an infusion reaction so they may stop or lower your infusion rate.
Your medical staff will keep a careful eye on you throughout the entire process.
It’s critical to contact your healthcare professional immediately if you experience any signs or symptoms up to 24 hours after your infusion.
When to Call 911?
One of the most common infusion reactions is a severe allergic attack called anaphylaxis that must be treated on a priority basis. If you have an anaphylactic shock, your healthcare provider will inject epinephrine (adrenaline) immediately. In case of severe reactions, dial 911 for immediate medical assistance. It may be fatal if neglected.
You must ask for emergency assistance if the person exhibits respiratory problems or lack of circulation symptoms. Call 911 if you know the person is affected by the infusion reaction or any symptoms.
It’s critical to remember that epinephrine passes over time. In such a panicked situation, you must need emergency care, even after receiving an injection; as soon as you administer the epinephrine, dial 911 or immediately arrange a trip to the hospital.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune ailment with inflammatory characteristics, and which affects the central nervous system (CNS). It manifests as optic neuritis, myelitis, and brain lesions. Relapses or attacks occur in patients with this fatal condition at intervals of weeks, months, or years, followed by remissions.
Managing Fatigue and Loss of Vision in NMOSD
Relaxation (or rest) is a crucial component of the overall therapy strategy for individuals with NMOSD, as well as exercise to maintain a sufficient fitness level. NMOSD sufferers often experience fatigue, which has been linked to poor sleep, pain, and sadness. It may be helpful to manage energy utilization while carrying out everyday tasks to lessen weariness. Maintaining energy levels may be achieved by setting priorities and completing the most important activities first thing in the morning. Exhaustion can be minimized by striking a balance between sleeping and doing your daily business.
NMOSD’s visual impairment may greatly impact the quality of life. Patients may be able to continue their daily routine using technology like voice-activated software and tactile devices.
Managing Pain and Diet in NMOSD
The problem of chronic pain in patients with NMOSD may last a lifetime. Although it might start in particular locations on the body, pain typically spreads over the whole body. The mental anguish of the patient might be made worse by persistent pain. Patients try to handle the impact of pain on their everyday tasks with the assistance of a medical team that consists of physiotherapists, pain doctors, nurses, and psychologists.
The arms and legs of the patients with NMOSD may become weakened, numb, or paralyzed during an NMO flare-up. Moving, working, or everyday tasks may be more challenging. To assist you in improving your arm and leg function, your doctor can recommend physical or occupational therapy.
Therapists may show you how to utilize aids like canes and walkers. Additionally, they may design specialized physical treatment regimens to strengthen and stabilize problem regions.
If an NMOSD patient wants to feel better, he can do the following things.
Yoga: It reduces pain, keeps you flexible, enhances mood, promotes better sleep, and relaxes the body.
Exercise: It can help overcome bowel and bladder problems, boost energy, helps with depression and fatigue, and promotes better sleep. Before starting any exercise routine, you must consult with your doctor.
Living With Vision Problems
Your spinal cord and optic nerves are most impacted by NMO, also known as Devic’s disease or NMOSD. Your eyes communicate with your brain via optic nerves. NMO damage may result in issues like:
Loss of vision
Difficulty in seeing colors in the dark
NMOSD may sometimes result in spasticity, a painful disease marked by stiffness and muscular spasms. To maintain your flexibility and enhance your mobility, a physical therapist may give you exercises to do, and you need to use splints, a sort of brace.
The treatment of NMOSD-related spasticity can be by:
Botulinum toxin injections: often called Botox; it relaxes your muscles. The results persist for many months. If just a few of your muscles are spastic, it will work well for you.
Serial casting: This treatment holds your ankle, wrist, or elbow joints in positions that gradually increase joint mobility using a succession of casts.
Bowel and Bladder Control
Living with NMOSD is very difficult. One problem is bladder and bowel control. Your bowel and bladder function may suffer if NMO damages your spinal cord. Constipation is common. You may have incontinence (problems regulating your urine and intestines), or urgently need to use the restroom. Your signs may improve if you:
Do regular exercise
Consume a fiber-rich diet
Drink lots of water
Do not eat citrus fruits, including tomatoes, and acidic foods; also avoid caffeine.
Causes of NMOSD
The clinical condition of NMOSD has been linked to two distinct antibodies. An assault on the aquaporin-4 (AQP4) water channel, found in the optic neurons and spinal cord, is the most frequent cause in the adult population. Proteins called aquaporins (AQPs) move water across cell membranes. An antibody biomarker termed the NMO-IgG is detected in the blood of almost 70% of people with NMOSD (anti-AQP4 antibody). More than half of all NMOSD with pediatric-onset is caused by autoantibodies against myelin oligodendrocyte glycoprotein (MOG), which is present in up to one-third of NMOSD patients who test negative for antiAQP4 antibodies.
Treatment of NMOSD with SOLIRIS
The FDA-approved drug for individuals with neuromyelitis optica spectrum disorder (NMOSD) is SOLIRIS (eculizumab). The patients of NMOSD are anti-aquaporin-4 (AQP4) antibody-sensitive. Data about the safety of SOLIRIS is not available for children with NMOSD.
Mechanism of action of SOLIRIS
In contrast to conventional multiple sclerosis lesions, NMOSD lesions exhibit substantial complement activation, eosinophilic infiltration, and vascular fibrosis. The primary humoral immune system-mediated attack on AQP4 is the pathophysiology of NMOSD. A humanized monoclonal antibody called eculizumab blocks the terminal complement protein C5 from cleaving into C5a and C5b. Although the precise mechanism for its effectiveness in NMOSD is unknown, it is thought to be connected to the prevention of the membrane assault complex’s development, which is mediated by C5b.
The Clinical Indication for SOLIRIS
Eculizumab, or Soliris, was the first medication approved by the FDA for treating adults with anti-aquaporin-4 (AQP4) antibody-positive NMOSD.
Previously, the FDA had approved Soliris for the treatment of the following conditions.
It is advised to administer SOLIRIS intravenously once per week for the first four weeks, followed by the fifth dose one week later and then every two weeks.
Common Side Effects of SOLIRIS
Severe allergic reaction from SOLIRIS is the most common side effect.
What is Plaque Psoriasis?
The most prevalent type of psoriasis is plaque, which results in dry, raised skin patches (plaques) coated in grey or silver scales. Based on skin tone, it may appear differently, ranging from pink on white skin to dark or grey on brown or Black skin.
Causes of Plaque Psoriasis
Medical experts are uncertain about the causes of plaque psoriasis. It is regarded as an autoimmune condition in which your immune system considers healthy cells as if they were infected. Because of this, new skin cells develop considerably more quickly than usual and accumulate in dense regions.
How Is Plaque Psoriasis Diagnosed?
Plaque psoriasis is classified as mild, moderate, or severe depending on the area and intensity of the plaques, the proportion of skin affected, and other factors.
Plaques may manifest as scalp psoriasis or genital psoriasis. However, they often appear on the hands, feet, elbows, knees, and back.
What is Skyrizi?
In April 2019, the FDA authorized Skyrizi (its generic name is risankizumab-rzaa) and is indicated for managing moderate-to-severe plaque psoriasis in adults. Moreover, in January 2022, the FDA approved Skyrizi for treating adult patients with active psoriatic arthritis (PsA).
Your doctor could advise you to take Skyrizi if you have plaque psoriasis. It is indicated for patients whose condition might benefit from systemic medicines or light therapy, as your entire body is affected by systemic treatment.
After two beginning doses, the SKYRIZI therapy may help you maintain smoother skin with four doses annually. Three out of four participants in clinical studies had 90% clearer skin after four months (16 weeks). Nearly 90% of those who had a 90% improvement in skin clarity maintained it for a full year. In clinical studies, many participants with plaque psoriasis saw clearer skin after a year.
Mechanism of Action of Skyrizi
Skyrizi (risankizumab-rzaa) is a humanized immunoglobulin G1 (IgG1) monoclonal antibody and an Interleukin-23 antagonist. It specifically binds to the human interleukin 23 (IL-23) cytokine’s p19 subunit and prevents it from interacting with the IL-23 receptor. A naturally occurring cytokine called IL-23 has a role in inflammatory and immunological reactions. The release of cytokines and chemokines that promote inflammation is inhibited by risankizumab-rzaa.
How to take Skyrizi?
The method to take Skyrizi will be explained to you by your health care professional. They’ll also go through the dose you need and how often to take it. Adherence to your doctor’s recommendations and following your doctor’s instructions for doses is essential.
Skyrizi is injected beneath the skin and comes as a solution within single-dose prefilled syringes. Your doctor will probably administer your first several Skyrizi dosages. You may begin administering Skyrizi injections to yourself at home after your first few doses. Your doctor will show you how to do it. For further details on administering Skyrizi by yourself, you can also check out the manufacturer’s website.
At the starting week of treatment (week 0), Skyrizi is administered through injection under the skin, then again on week 4, and then every 12 weeks after that.
Sickle cell disease manifests as a genetic blood condition known as sickle cell anemia. The change in the red blood cells usually observed forms when you have sickle cell anemia, going from being round, flexible discs to rigid, sticky sickle cells that obstruct blood flow. About half of those with sickle cell anemia survive into their 50s through early identification and the latest therapies.
Compared with healthy red blood cells, sickle cells have a shorter lifespan. Anemia is called sickle cell anemia since a patient doesn’t produce enough healthy red blood cells.
Children born with sickle cell disease hardly ever make it to adulthood. Nearly half of those with sickle cell anemia now reach their 50s owing to early identification and modern therapies. Sickle cell anemia patients continue to remain at risk for possibly fatal medical issues. Furthermore, medical professionals have procedures that lessen the likelihood of problems and soothe symptoms. Still, no appropriate medical therapy for sickle cell disease is accessible in many parts of the world.
Symptoms of Sickle Cell Disease
The symptoms of sickle cell disease often appear in newborns around the age of five months. Each person’s symptoms are unique and evolve gradually. The following are the symptoms of SCD.
Sickle cells are more delicate than normal red blood cells and often disappear or die after 10 to 20 days. Almost 120 days is the average cell life span in this way. This leads to the loss of red blood cells, called anemia.
Fatigue results from a lack of red blood cells because it affects the transport of oxygen throughout the body.
Pain attacks are a common sign of sickle cell disease. Pain in your chest, belly, joints, and bones is brought on by red blood cells with a sclerotic form that obstructs blood flow via small blood arteries. The pain’s level and frequency might change, and sometimes, you might need to visit the hospital.
Hands and feet swelling:
Swelling results from sickle-shaped red blood cells obstructing blood flow to the hands and feet.
Risk of infections:
Ulcers may develop when sickle cells harm your tissues. Also, it’s possible you might get infections if they affect your spleen. Healthcare professionals usually give SCD patients immunizations and medications to avoid potentially harmful infections.
Jaundice or Yellowing of Skin and eyes:
It may happen due to damaged sickle RBCs.
Sickle cells may get lodged in the blood arteries that nourish your eyes, negatively affecting your retina and impairing your eyesight.
Children with SCD may develop more slowly than other kids. Teenagers affected by SCD may reach puberty later than similar-aged teenagers.
One more severe and unexpected consequence affecting sickle cell patients is the chance of a stroke. The damaged cells might block the main blood arteries that provide oxygen to the brain. Severe brain injury may occur if the blood and oxygen supply to the brain is eliminated. A stroke caused by sickle cell anemia increases your chance of a second and third stroke.
Treatment of Sickle Cell Disease
Hydroxyurea (Droxia, Hydrea, Siklos)
Using daily hydroxyurea lowers the incidence of painful crises and may lessen the requirement for hospitalization and blood transfusions. However, it could enhance the chances of infections. If you are pregnant, avoid using the medication.
L-glutamine oral powder (Endari)
This drug recently got approval from the FDA to treat sickle cell anemia. The likelihood of pain can be minimized by using this drug.
Adults and children over 12 may take this drug to treat sickle cell disease. This medication, when taken orally, may increase blood flow throughout the body and reduce the risk of anemia. The possible adverse effects are headache, nausea, diarrhea, exhaustion, rash, and fever.
For sickle cell-related pain issues, your doctor may also prescribe narcotic analgesics (painkillers) to reduce pain severity.
What is Adakveo?
Adakveo is a brand-name prescription drug called Crizanlizumab. This drug is FDA to avoid vaso-occlusive crisis (VOC) in adults and children 16 years of age and older with sickle cell disease. VOC is another name for a pain crisis.
The hereditary disorder sickle cell disease alters the structure of your red blood cells. Most often, sickle cell disease is discovered in newborns.
Few individuals with sickle cell disease have a pain crisis. Pain crises often result in intense pain. Additionally, this may result in a blood clot, raising the danger of organ damage and stroke.
Administration of Adakveo
Adakveo is administered as an intravenous (IV) infusion into your arm—it is a liquid solution. (An infusion is an injection that is slowly injected into your vein.) The dosage of the drug is 100 milligrams of the drug per 10 milliliters of solution.
Adakveo may be administered in an infusion suite, a medical facility designed exclusively for infusions. It can also be given to doctor’s clinics and hospitals. Rarely, a nurse may be able to provide the infusion to you at home.
Adakveo is a member of the class of drugs known as selectin blockers. Crizanlizumab is the active ingredient of Adakveo.
FDA approval of Adakveo
In November 2019, the Food and Drug Administration (FDA) authorized and approved Adakveo. The first targeted therapy for a pain emergency is adakveo. This indicates that it functions differently from other pain relief drugs. Adakveo identifies blood cells and prevents blood clots as opposed to alleviating the pain.
Important Adverse Reactions of Adakveo
Some adverse effects include nausea, joint discomfort, back pain, and fever. Fewer than 10% of patients receiving ADAKVEO noted clinically significant adverse effects such as oropharyngeal pain, infusion site reaction, diarrhea, vomiting, pruritus (including vulvovaginal pruritus), musculoskeletal chest pain, and myalgia.