Someone I Love Has PH

Pulmonary Hypertension: Someone I Love Has PH A guide for kids and teens who have an adult in their lives with PH

This Booklet is For You! If your mom, dad or another adult in your family has been diagnosed with pulmonary hypertension (PH), this guide is for you. This booklet talks about two big questions you might have when someone you know has PH: 1. Pulmonary... what? This section tells you what PH is, what treatments there are, and answers questions you may have about your family member s illness. 2. Now what? This section talks about how your life might change when your parent or another adult in your family has PH, how other kids and teens handle having PH in their families lives, and ways you can learn more and fight back. Finding out that your parent or adult relative has PH can be scary or unsettling. Remember that lots of kids and teens out there have a relative who is living with PH. We ve talked to those kids and teens about what helped their families stay strong while getting used to PH, and that s what you ll find in this booklet. Adult living with PH is a mouthful! In this booklet, we refer to your parent or other adult living with PH as ALPH: Adult Living with PH 2 Someone I Love Has PH

Pulmonary... What? Having pulmonary hypertension (PH for short) means: Pulmonary is another word for lungs. The lungs are the organs that provide your body with the oxygen that it needs to function properly. Hypertension is a word that means high blood pressure. Your blood carries oxygen all over your body by traveling through arteries. When someone has PH, the arteries in their lungs and the right side of their heart don t relax enough to let the blood easily pass through. This makes the heart work extra hard to pump blood through the arteries. Because the heart has to work harder, it can start to become tired, especially during exercise or other physical activities. Having PH might make someone feel: Out of breath Dizzy Tired Like their chest hurts These are called symptoms. Symptoms can be clues that help the doctors figure out what s going on inside someone s body. Has your doctor ever taken your blood pressure on your arm? You have a blood pressure in your lungs, too! People who have PH have a higher blood pressure than normal in their lungs. 3

Why Do Some People Have PH? There are different reasons why people have PH: associated, familial and idiopathic. Associated: This means that PH may have developed as a result of a heart problem (either before or after heart surgery). This could also mean that the PH developed from another condition, such as lung disease, scleroderma or sickle cell disease. Familial: Some people get PH because they carry a gene in their DNA for it. Genes tell your body what color your hair will be, how tall you ll be, and everything else about your body. Not very many people have the gene for PH, and some people can have the gene for PH but never develop the disease. Idiopathic: This means the doctors do not know what has caused the PH. It can be frustrating, but sometimes even the doctors do not know why a person has PH. Doctors are working every day to learn more about why people get PH, and in the meantime there are lots of treatments to help your ALPH live with their PH. My parent has PH. Does that mean I have it, too? Not necessarily. Some people get PH because they carry a gene in their DNA for it. There is a special test the doctor can do to find out if your parent has the gene. If your parent does have the gene, they may want you to get a gene test as well. But doctors who have studied thousands of people with PH have learned that even if you have the gene, there s only a small chance that you ll develop PH. 4 Someone I Love Has PH

Is There a Cure for PH? There are medicines that help people who have PH, but right now there isn t a cure that takes away their PH completely. This is why PH research is so important! Thanks to PH research, doctors have developed a number of treatments for PH and more are on the way. The more research we can do, the closer we will get to understanding why people get PH, developing even better treatments, and ultimately finding a cure. To learn how you can help spread knowledge of PH, read PHA s guide, Doing My Part: Getting the Word Out About PH. Request your copy at: www.phassociation.org/parentresources Even though [my dad] has low energy, he still goes on walks and kicks a ball around with us. I have learned so much with my dad s PH. I don t care what he is not capable of doing I just focus on what he IS capable of doing. Alex, age 12 5

How Can the Doctor Tell if Someone Has PH? These tests help the doctor know if your ALPH has PH and, later on, whether the medicines are helping their PH. Six-Minute Walk: This test looks at how far someone can walk in six minutes and what their oxygen saturation levels are before, during and after the test. During this test, your ALPH will walk up and down a hallway for six minutes. Oxygen saturation levels measure how much oxygen is in your blood. Someone who has PH may have lower oxygen saturation levels than someone who doesn t have PH. Oxygen saturation levels are also called O 2 sat. Echocardiogram (ECHO): This is a special machine that takes pictures of the heart. Your ALPH will put on a hospital gown and lie down on their back. The echo tech will place a little bit of gel on your ALPH s chest and use a special piece of equipment to take pictures of their heart. These pictures will show your ALPH s doctor how well the heart is pumping and estimate how high the blood pressure is in the pulmonary artery. This test can take a long time it often takes 45 minutes to an hour. Cardiac Catheterization: This is a procedure in which the doctor uses a small catheter (which looks like a long wire) to 6 Someone I Love Has PH

directly measure the blood pressure in your ALPH s heart and lungs and to see how well the heart is working. EKG: This test shows doctors the rhythm of your ALPH s heart and gives the doctor a rough measure of the thickness of the chambers of the heart. For this test, stickers are put on your ALPH s chest that are connected to a computer. The computer makes pictures of your ALPH s heart rhythm. If you have PH, your arteries look like the little straw instead of the big straw, so the heart has to push a lot harder to move the blood through the artery. Cardiac MRI: This is like a very fancy x-ray that gives the doctor an even better picture of your ALPH s heart. To take the MRI test, your ALPH will lie down on a bed, which moves them in and out of the MRI machine while the machine takes pictures of their heart. Your ALPH might get to wear headphones to listen to music while the MRI machine takes pictures of their heart. Tip! PHA has a Glossary of PH Terms on our website. If you and your ALPH need help with a medical term, you can look it up at: www.phassociation.org/glossaryofphterms 7

Treatments Have you ever taken medicine to help you feel better when you have a cough, headache or stomachache? There are several kinds of medications that can help people who have PH. The medications work in different ways, but all of them try to dilate (relax) the pulmonary arteries so that the heart doesn t have to work so hard to push blood through the arteries. Depending on the medication, your ALPH might take it in different ways. A special doctor, called a PH specialist, can tell you and your ALPH more about how to take these medicines and how they help. It s important to remember never to touch your ALPH s medicine unless they or another adult give you permission. Side Effects Have you ever taken cold medicine to help you breathe through a stuffy nose, and the cold medicine made you feel sleepy? The sleepiness is called a side effect. A side effect is when the medicine has an effect besides what you re using it for. Some PH treatments will have side effects. Your ALPH s doctor or nurse can explain what the side effects are for the treatment they are taking. 8 Someone I Love Has PH

Now What? Having a family member who has PH can be stressful. You may have new responsibilities, you might be worried or scared about your ALPH and you might be feeling like there s no one else who understands what you and your family are going through. This section covers ways to: Take care of yourself Help your family stay strong Find other families who are also living with PH Learn more about PH Finding Your New Normal When your family member has PH, a lot of things may be different than they were before. Getting used to these changes can be difficult and there may be good and bad parts about your family s life with PH. Families call these changes their new normal. Every family s new normal will look a little different. 9

Taking Care of Yourself Stress is an emotion we sometimes feel when our bodies are responding to different demands in our lives. It s our body s way of rising to a challenge and preparing to meet that tough situation with focus, strength, stamina and heightened alertness. A little stress can help you finish that school project or score in a sports game, but long-term stressful situations can be hard on you and your body. If you re feeling stressed, you might: Feel irritable or moody Get stomachaches or headaches Feel sad or depressed Have trouble getting to sleep at night Be more forgetful than normal These are all normal reactions to feeling stressed. The good news is that there are things you can do to help manage stress. Here are some tips that have helped other kids and teens: Stay Connected Talk to your parents about your thoughts, feelings and concerns. For tips on talking to your family, see page 12. Spend time with your friends. Stay involved with sports and activities. Join a PH Support Group to meet other families living with PH. Find a group near you by visiting: www.phassociation.org/findasupportgroup 10 Someone I Love Has PH

Check if your ALPH s hospital has a support group for kids and teens who have a parent or other adult living with a chronic illness. Take Care of Your Body Get enough sleep each night: 9 hours if you re a teenager, 10 hours if you re between ages 11-12, and 10-12 hours if you re age 10 or younger. Get some exercise every day. Try yoga or meditation. These help your body and mind relax. Be Creative Keep a journal so you have a place to let out your thoughts. Try drawing, painting, or other kinds of creative activities to find the one that lets you express your thoughts and feelings best. Ask For Help Talk to a counselor, teacher or adult who you trust. They can help you let out some of your feelings and may be able to offer good advice or tips. If you re feeling scared or overwhelmed, let your parents know. For tips on talking to your family, see page 12. Take Action Volunteer at or attend a PH event. Find events in your area by visiting www.phassociation.org/events. Talk with your teacher, friends or family about fundraising for PH research. Get more tips for this in PHA s guide, Doing My Part: Getting the Word Out About PH. 11

Taking Care of One Another If your parent or another adult has PH, you may want to be there for them but not feel sure what to do or say. And your ALPH might be worried about how you feel about their PH and not sure what to do or say to support you! It s OK to not feel sure this is new for everyone in your family. Talking it Out When your parent or relative is diagnosed with PH, some things may change suddenly. Other things may change slowly. Families say it helps to talk about these changes and how they make each person feel. Sometimes it can feel hard to start that conversation, though. Here are some tips for what to say and what to keep in mind. With your siblings Your siblings, both older and younger, are probably feeling many of the same things you are. They may be frightened, overwhelmed or confused, and they may want to help but not feel sure how. You may feel like you have to act like the strong one. But if you let your sibling know that you re feeling the same things, you may be able to team up and help one another feel stronger. When you want to have a conversation with your sibling: Remember that your sibling may be feeling frightened or angry. If they react badly the first time you approach them, give your 12 Someone I Love Has PH

sibling some time to cool down before you approach them again. It s OK if you don t have one big conversation that covers everything. You and your sibling may want to have lots of small conversations to check in with one another whenever one of you needs to talk. Everyone handles stress differently, and that s OK. It s important to respect the way your sibling wants to handle their feelings, as long as they are doing it in a way that doesn t hurt themselves or others. With the adult in your life who has PH Your ALPH may be feeling a lot of hard feelings like stress, anger and confusion. They are also coping with their PH symptoms and trying to learn about PH and their new medications. When you want to have a conversation with your ALPH: Put yourself in their shoes and imagine how your ALPH might feel about what you plan to say. If it might be hard for them to hear, how can you make it easier? Sometimes just reminding them that you love them, or starting with something you appreciate about them, makes it easier for them to hear something hard. For example, you might say, Thank you for helping me with my homework last night. I love when we can spend time together. But I wish we had more time together. Try to pick a time when your ALPH isn t distracted. Be understanding that PH may make your ALPH tired, and you might have to continue the conversation later if your ALPH needs to rest. Remember that your ALPH is human, too. They may snap at 13

you or say the wrong thing. When we re stressed and tired or not feeling well, it can be harder to be patient with the people around us. Remember that your ALPH isn t really upset with you, they just aren t feeling well. With your parent or other adult who is well Sometimes things don t change a lot after your ALPH is diagnosed. But if your ALPH is feeling sick, your parent or other adult who is well has probably also taken on a lot of new responsibilities, like driving your ALPH to doctor s appointments, preparing their medicine and doing some of the activities that your ALPH used to do before they got PH. When you want to have a conversation with your adult who is well: Put yourself in their shoes and imagine how they might feel about what you plan to say. If it might be hard for them to hear, how can you make it easier? Sometimes just reminding them that you love them, or starting with something you appreciate about them, makes it easier for them to hear something hard. For example, you might say, Thank you for all that you re doing to take care of me and Mom. I really like helping out, but I m feeling a little overwhelmed right now. Would it be OK if I take two days a week off from doing the dishes so that I can take a little time to see my friends? If you can t think of any solutions, you can ask your adult to help you think of one that works for both of you. Remember that they may be feeling stressed or have a lot on their mind. This might make them seem less patient and more 14 Someone I Love Has PH

distracted, but they still love you just as much as they did before your ALPH got PH. Try to pick a place where you won t be interrupted. Try to pick a time when they aren t in the middle of something. If you need to talk and are having trouble finding a good time, you could say: I was hoping we could talk, just you and me. Could we have a little time alone sometime today? Be patient when they don t have the answer to your questions. They have a lot to learn about PH, too. You can even offer to help find the answer using the resources on page 21. You could say: I don t really understand what this means. I was thinking I could find some information about it using this website and we could look it over together, or, I have some questions that I was hoping you or I could ask the PH doctor at the next appointment. Remind them that you love them and appreciate what they are doing to take care of you and your ALPH. Remember that it s OK to talk to someone outside your family, too. A school counselor, social worker or a pastor can be a really good person for this. Your family may also want to join a PH Support Group to meet other families living with PH. You can find groups near you by visiting: www.phassociation.org/findasupportgroup. 15

Helping Out PH can make a lot of everyday activities harder. Here are some tips if you want to help out with these everyday activities: Make a list of activities you usually see your ALPH do that you think you could do. This might include things like: Washing the dishes Feeding and walking the dog Spending time with your siblings Share your list with your ALPH and explain that you d like to help out. Ask them to tell you which of these activities would be most helpful to them. Together, you can decide which activities you can take on. Remember that as you get older, you can talk to your ALPH about doing different things to help out for instance, when you get your driver s license you could offer to help do the grocery shopping. While it s great that you want to help out, remember that you still need to take care of you! You re part of a team that includes you, your ALPH, their doctors and anyone else who helps take care of your ALPH s PH or helps your family. Being part of a team means that you don t have to do everything yourself. If you start to feel like you re doing too much and don t have enough time for yourself, talk to your parents. Together, you can find the right balance between helping your ALPH and taking care of yourself. 16 Someone I Love Has PH

Feeling it Out Sometimes you might have feelings about your ALPH s PH that you wish you didn t feel. You might feel: Embarrassed if they use a wheelchair in public. Impatient if they can t walk as quickly as other adults. Disappointed if they can t come to your event. Guilty that you are healthy while your ALPH is not. Angry at people who don t understand PH. These are all normal feelings. And your ALPH probably feels them, too! They might feel: Embarrassed to be using a wheelchair in public, especially when people say they don t look sick. Impatient and frustrated that they can t keep up with other adults or kids. Disappointed to miss your event. Confused about why they have PH. Angry at people who don t understand PH. Frustrated when people say they don t look sick. My mom has had PH for almost 10 years. You don t have to be diagnosed with PH for it to affect you. It affects the family, too. I am a fighter like my mom, and I won t stop spreading awareness for her and everyone affected by PH until there is a cure. Paige, age 10 17

Sometimes you might feel guilty for being embarrassed, impatient, disappointed or angry with your ALPH. Instead of feeling guilty, you can accept that these feelings are normal and talk about them with your ALPH. Try to remember that you and your ALPH are a team. You could say something like: I get frustrated when people stare at us at the mall. Maybe we could make a funny sign about PH so that when they stare, they ll learn something. I m sad that you won t be able to come to my game next week. I wonder if someone could take a video. That way I could show and tell you all about it later. Sometimes I feel really upset that this happened to our family. Do you feel that way, too? What Happens Next? Your ALPH may have good days when they have lots of energy, and bad days when they are too tired to get out of bed or may need to go to the hospital. Sometimes it might not be clear why your ALPH is having a good day or a bad day, and you might not be able to predict ahead of time which kind of day it will be. This can be frustrating and stressful. Here are some tips from other kids and teens who have an ALPH. 18 Someone I Love Has PH

Take it one day at a time. Make the most of the good days, but respect when your ALPH needs to rest or save their energy. Maybe you can find some quiet things to do together, like watch a favorite movie or listen to music. Remember that your ALPH feels just as frustrated as you do when they have to cancel plans. Remember that we don t know exactly why your ALPH has good days and bad days it s no one s fault. Stay on schedule if appropriate. If you drop everything anytime your ALPH is having a bad PH day, or refuse to make plans in case, it can make it even harder for you to de-stress and feel like you have a life outside of your ALPH s PH. Obviously some plans (like going to school) may be more important than others. You and your parents can decide based on how important your plans are and how your ALPH is doing. Work with your ALPH to arrange a back-up person who can drive you to school or events when your ALPH is having a bad PH day, and remember that your ALPH wants you to live a happy and healthy life. If you need to stay home because of a bad PH day, have a favorite activity, book or movie on hand so that you can still have something to look forward to. Have someone you can talk to during a crisis. Some of your friends may be more comfortable than others with providing support during an emergency. Try to find someone in your life a friend, relative or a counselor who you can call or text when you re upset or afraid. Even while you re supporting your ALPH, you deserve and need support, too. 19

Knowledge is Power These days, there are a lot of ways to get more information. The first people you should ask are the trusted adults in your life who know about PH: Your ALPH, another parent or a doctor or nurse who helps with PH care. The most important thing when you are looking in other places for information is to make sure that your information is reliable, up-to-date and accurate. This means: Reliable: Is it a good source? A lot of websites have bad information information that isn t true, or that uses facts but misinterprets them. When using a source, ask yourself: Is this a medical organization or an organization that works with PH families? Where did they get their information about PH? Up-to-Date: Does the source list a date? More PH research is going on all the time we know more this year than we did two years ago, and we re learning more all the time. If your information is old, it might not be correct anymore. Accurate: Is the information true? Do other websites or books you trust have the same information? Does it match what your ALPH s PH specialist tells you? 20 Someone I Love Has PH

When you are looking for information, be sure to talk about what you find with your ALPH, your adult who is well, or your ALPH s PH specialist. Everyone s PH is a little different, and it s important to discuss the information you find with someone who can tell you how it applies to your ALPH. Here are some suggestions for good sources of information on PH: Online www.phassociation.org www.phassociation.org/phkids. The Pulmonary Hypertension Association is a national organization dedicated to finding ways to prevent and cure pulmonary hypertension, and to providing hope for the pulmonary hypertension community through support, education, research, advocacy and awareness. www.lpch.org/kids. The Lucile Packard Children s Hospital has a pulmonary hypertension clinic. They designed this website for kids visiting their PH clinic, but you can learn about a lot of the tests your ALPH will have, too. www.mayoclinic.com/health/pulmonary-hypertension/ DS00430. The Mayo Clinic website is run by the Mayo Foundation for Medical Education and Research. Their hospital includes a pulmonary hypertension clinic. 21

www.nhlbi.nih.gov/health/health-topics/topics/pah. The National Heart, Lung, and Blood Institute is part of the National Institutes of Health. This part of the American government provides money and laboratories to do medical research. In-Person Your ALPH s PH specialist and PH nurse. Ask if you can come to an appointment and ask questions, or give your list of questions to your ALPH. Your local PH support group. You will likely meet patients at your local PH support group who have been living with PH for a very long time. Some support groups also bring in medical professionals to talk and answer questions. In Print Pulmonary Hypertension: A Patient s Survival Guide. The Survival Guide is a book for patients and caregivers that was written by PH patient Gail Boyer Hayes and is published by the Pulmonary Hypertension Association. Learn more at: www.phassociation.org/survivalguide or 301-565-3004. http://science.education.nih.gov/supplements/nih10/ Diseases/pdf/Rare%20Diseases.pdf. You can read about the history of rare disease research by jumping to page 21 in this lesson plan for teachers. 22 Someone I Love Has PH

When my mom was diagnosed, I didn t want to talk about it. Then at PHA s International PH Conference, Mom forced me to go to the caregivers support group. It was the best thing she could have done. At first, I was nervous. I sat in a room with husbands, wives, parents, and siblings, all older than me. When they came and talked to me and asked to hear my story, I told them everything. They didn t question [what I said], but asked about school and things to do in Kansas. After they hugged me, gave me phone numbers and email addresses, they told me that I was a strong kid, that I was such a good daughter, and that I was doing all I could. They urged me not to push myself too much and to remember that I was never alone. I fought back tears as they hugged me. For the first time since my mom was diagnosed, I didn t feel alone. Karel, age 16 We know that learning about PH and how to help your ALPH is a lot! Remember that you don t have to do everything right away, and you don t have to do things perfectly. What counts is finding ways to learn what you can, help when you can, knowing the adults in your world (including your ALPH) still love you and showing your ALPH how much you still love them. If you have any questions, you can contact the Pulmonary Hypertension Association anytime by emailing: Outreach@PHAssociation.org or calling 301-565-3004. 23

This resource was medically reviewed by Allyson Rupp, LCSW, of Stanford Hospital and Clinics, and Dr. Robert Schilz of Case Western University School of Medicine. 801 Roeder Road, Suite 1000 Silver Spring, MD 20910 301-565-3004 Patient-to-Patient Support Line: 800-748-7274 www.phassociation.org Kids@PHAssociation.org