Strategies to stay organized with PNH treatment

People living with paroxysmal nocturnal hemoglobinuria (PNH) face a number of challenges.

A blood disorder that causes the destruction of blood cells, even cases of PNH that are considered mild can cause symptoms that make life difficult—fatigue, headaches, chest pain, rapid heart rate, and limited exercise capacity. More severe cases can be debilitating. PNH can also cause life-threatening complications, including blood clots, which can cause heart attack, stroke, and damage to organs and limbs.

Some cases of PNH can be cured with an allogenic stem cell transplant (also called a bone marrow transplant), but this is an intense procedure that requires a high tolerance for side effects and a matching donor.

Most cases are managed with medications called complement inhibitors. These medications act on the complement system, a part of the immune system central to the blood cell destruction that occurs when a person has PNH. While considered a lifesaving therapy, complement inhibitors alter how the immune system works, can increase the risk of serious infections, and require careful monitoring for side effects.

Additional treatments can include anticoagulants (blood thinners) to treat or prevent blood clots, blood transfusions to treat anemia, and supportive therapies like iron supplements and folic acid supplements. Each person’s treatment plan will be different depending on the severity of PNH and individual needs.

Staying organized can help you manage PNH

Beyond the difficult symptoms of PNH, there are other challenges. Many people with the condition have had to see multiple healthcare providers to receive an accurate diagnosis. Treatment often includes high out-of-pocket costs, insurance pre-authorization, and frequent dosing. Though some therapies can be taken at home, others require a visit to an infusion center for each dose.

All of this needs to be managed on top of managing all other aspects of daily life, like work, household, family, relationships, and personal interests and goals.

If you or a loved one is living with PNH, it helps to stay organized. Here are two strategies that can help you stay on top of treatment and hopefully free up some time for the other important aspects of your life.

Keep a personal health record

A personal health record is a file that includes all documents and information relevant to PNH, as well as other aspects of health and healthcare. This file should contain:

• Copies of all evaluations, test results, and other documents related to your diagnosis
• Treatment records, including any therapies and medications you are currently taking or have taken in the past
• Dosing information for all medications you are taking, instructions from your healthcare providers, and copies of information on treatment side effects
• Copies of bills, receipts, explanation of benefits documents, and other information related to the financial aspects of treatment
• Other information related to your health, such as immunization records and records of any past illnesses, medical events, and hospitalizations
• Insurance information
• Patient education materials
• Your own notes from appointments
• Contact information for all of your healthcare providers
• A written plan of what to do if you need emergency care (your healthcare providers can help you put this together)

Keep a symptom journal

Symptom journals are useful for managing conditions like PNH because they help you track how you feel over time. It can be difficult to remember exactly how you felt on a specific day that occurred a week or a month ago. By keeping a journal, you’ll be able to capture that information, refer back to it later, and share it with your healthcare provider.

Some information to record:

• Any signs of dark or blood-colored urine
• Energy levels and fatigue
• Sleep, including duration and if you felt rested
• Any fever or other signs of infection
• Headaches, pain, or other symptoms like rapid heartbeat or difficulty swallowing
• Stressors and stress level (this is a potential trigger for PNH symptoms)
• Concentration and mood
• Anything that was difficult or challenging
• Any medications you took (for PNH or other reasons)
• Any questions you want to ask a healthcare provider
• Anything your providers have asked you to record

Include anything else you think is relevant but keep entries short and manageable, something that you can complete in a few minutes. Try to set aside a specific time each day. Also consider adding it to another part of your routine, like brushing your teeth or immediately after dinner.