The Importance of Caregivers

November is an incredibly important month for pulmonary hypertension, since it is the month where so many in the PH community try to spread awareness everywhere possible! But November is also special to many people who have caregivers, because it is Caregiver Awareness Month as well! A caregiver is defined as a family member or paid helper who looks after the care of someone who is sick or disabled, whether that person be an adult, a child, or an elderly person. A caregiver provides emotional and physical support as much as possible, and their helpfulness, thoughtfulness, and caring can be a blessing to the person being cared for. The following comments are from PH patients after they were asked to share what their caregivers mean to them. It is quite obvious that most of the caregivers play a huge roll in providing comfort for the PHer!

Jen C: Great - we all love our caregivers that are blessed to have them ! My hubby Manny (cg) is my rock - always there in good and bad days when it seems like the rest of the world has walked out on me !

Tiffany G: My caregiver (my fiance Mike) loves and accepts me unconditionally. There is nothing that he would not do for me and the PH cause. He takes me to all of my appointments, helps me understand the Doctors, picks up my prescriptions and eats the diet that I choose to make us more healthy. He also holds me when I am sad, crying and frustrated.

Barbara H: My caregiver who is my husband has been with me ever step of the way during this illness, I am so grateful for his kind, loving care, he has done without to make sure I get my medications and whatever I may need to survive.

Kevin B: Joann, my caregiver does everything for me, days I cannot get off the couch she is their, every doctor's appointment she is their, times I am mad at the world she is their to show me the good in my , when I'm ready to give up she helps me carry on. With out Joann I would not have the strength or courage to make it day to day, with out Joann I could not make it through this terrible sickness.

I want to also mention my son Josh who time after time has helped me through troubled times like when I awoke from passing out to find him laying next to me caressing my arm telling me "it's OK dad your OK" or the numerous times he has helped me while I was having a seizure or the times he has explained what PAH is to a doctor and EMS people that were unaware with what it was. To the day to day chores he does because I cannot. Between he and my wife I would never make it without them. I also have to include you folks my PHfamily and friends because you ALL help me even when your not with me because your always in my thoughts!

Kit B: My caregiver/partner has really stepped up at home where I need it. He cooks more, goes shopping for groceries, and tries to help see that I'm able to eat nutritious food even if I'm unable to cook it! In addition to that, he provides emotional support that others cannot. He never questions my struggles -- he accepts them and we work together to try and fix what we can.

Angie E: My caregiver, my husband, has fought for me through thick and thin and has saved my life so many times when there were too many doctors on the case and he was my point person, not taking "no" for an answer. He is always there when I need him even when I scream and yell because I am so tired of trying to breathe! God Bless Our Caregivers!!

Sylvia T: My caregivers, my husband and son, are always there for me, pushing me when I am down...loving me when I am having a hard day...making me laugh. They cook and clean and pay the bills. I always feel the love!

Bonnie H: My hubby, Brian, is my best friend and caregiver. If I can't do something I want to do, he'll figure it out so I can participate and not get left behind. He designed a garden closer to the house so I can grow my tomatoes and beans something I really enjoy. But just being there to make me laugh helps more than anything. I am loved.

Ruby Nan M: Louis, my husband, does every thing I need and never complains! He does the shopping, he does the cooking, cleans the kitchen, does most of the laundry, mixes my veletri, changes my cassette every night, sorts my pills once a month, etc. And he NEVER complains. I am very blessed. He always tries to lighten my load. He's the love of my life and my best friend and he always brings humor into the situation.

James R: My caregiver is my best friend, through thick and thin, she is also my wife. She never complains, and for that reason sometimes I forget I can be a handful to take care of, She is always there, even when I don't need her to be, She is my one true love and has been for close to 24 years now. I cannot imagine doing things without her, much less her not being a part of my life.

Tara S: My Caregiver/Partner Michael has been a blessing in my life. He has taken over so much of the housework (with a little bit of complaining)  but I know how much he loves me. He has to help me shower. He has to help me change my medicine out every two days. He does it all without complaint. He carries the laundry out of the laundry room for me so that I can put it away if I am feeling up to it and if I am not when he gets home he will take care of it. There is nothing that he won't do for. We are waiting right now for the call for Transplant. It is very nerve wracking. He has switched shifts at work to be home with me at night in case we get the call. We have been together for 7 years. I hit the lottery when I found him.

Alice Marie J: My care giver is my husband Dean, he helps me physically, emotionally and spiritually, he is my best friend, when I finally drop of to sleep I wake listening to him reading the bible to me! He is truly truly truly a gift from the Lord!

Shannon W: My best friend Susan. She has been there from the min I found out and has been there physically,emotionally,and spiritually. She took time to learn about PAH and how to help. The are days she has to keep me on my toes cuz I feel so down.

Neeta P: He knows it when I am ill or well. He knows it when I am blue or pink. He will help when the time is right. That is just when I am in need. This one is for all our caregivers. We are so very blessed to have them in our life. He is the only one who understands what I am going through and does whatever is needed to keep me comfortable, feeling better. On my good days, he lets me do what I can, on my bad days, he does it all and more without a peep, always cheerful and smiling, counting his blessings that I am alive and giving him company. Yes! he too reminds me when I forget to take my meds in spite of the alarm. He is the one who carries all the grocery, whether I accompany him or not, he is the one who is my moral, emotional, physical and spiritual support reminding me at the appropriate moments to be positive, to be thankful and to be happy. Its not how many breaths we take but, how many moments of happiness we have together in life that matters! I thank god for my caregiver every day of my life, for this beautiful gift He bestowed on me as my husband!

Stacey G: My hubby is my true partner in life in all ways. He caregives by giving me emotional support through the many ups/downs of having 2 chronic illnesses. He steps in when I can't and takes care of things to make my life easier, tells me I am beautiful despite my oxygen tubing and a line coming out of my chest and never gives me any worry that he won't be here for me when I need him.

Barbara T: I literally would not be on this Earth had my husband & caregiver, Chuck, given up on me. He took me to 14 different doctors prior to a correct diagnosis.

Lorrie T: I could not live w/o my caregiver! Joy has stood by me all along this crazy ride since dx 2 years ago. When I can not take care of me, she always steps in and mixes my meds. She is the most understanding of what we as patients deal with on a daily basis. I love her so much, she can't be replaced!!!!!

Elaine W: My caregiver helps me out in all situations, no matter how big or how small. I couldn't do it without her, she is special and deserves an award!!

Rozanne C: My caregiver is my partner, the love of my life for over 30 years. He is there for me, supporting me through physical, as well as spiritual challenges. And, I love him more each day.

Jennifer S: My caregiver and loving husband is my rock and the best advocate and partner anyone could wish for. I have no idea how it would face all of life's hurdles without him.

Kim F: My caregiver is my husband and he has been my rock for the last 5 years. He gladly took over all the daily house chores that I can no longer do and he waits on me hand and foot. He also learned how to mix my drugs and does that more then I do. I don't know what I would do without him he has been my best friend for over 30 years but in the last 5 has shown what better or worse really means.

Improper Diagnosis: The Challenge of PVOD

Improper Diagnosis: The Challenge of PVOD

Pulmonary Veno-Occlusive Disease (PVOD) is a disease of pulmonary venules and small veins, characterized by fibrous thickening and abnormal intimal cellularity, leading to vascular obstruction and pulmonary hypertension (2013, Istanbul, Pulmonary Vascular Research Institute Workshops and Debates). It is an incredibly rare form of pulmonary hypertension. With doctors knowing so little about the disease, it is often improperly diagnosed, or completely undiagnosed. Patients and caregivers share their stories and reflect on their journey.

Mysterious Symptoms—

“I started being seen by numerous doctors who all had different diagnoses. I spent hours sitting through medical tests and waiting for results. I felt like a lab rat always being poked and prodded. My freshman year (of high-school) the doctors’ suspected severe asthma. Today, they, and I know they were mistaken. I actually have Pulmonary Veno-Occlusive Disease (PVOD.” –Carson; St. Louis, Missouri    

 “..At every move, I was still gasping for breath. In late August we all knew I had reached a plateau as far as getting better. I was terribly worried since I could not breathe if I did anything strenuous – just walking and talking were exhausting. Finally in November, after many medical tests, and visits to various specialists, a biopsy and heart catherization needed to be done. Armed with those results they sent me to Boston to see another specialist. By the end of November, we had a diagnosis – Pulmonary Hypertension Veno-Occlusive Disease.” –Marie; Maine

“In November of 2010, my mom and I were flown out by Flight for Life and the University of California hospital for surgery of what doctors thought to be a pulmonary embolism. After 10 minutes of surgery, the doctor said it was something more serious.” –Kullie (as told by daughter, Lakesha; Colorado)

“We had spent the last year of her life (2009-2010) trying to sort out why she did not feel well, why she was so thin, why she didn’t want to eat, and why she complained of not being able to get her breath. We watched her slow down physically, finding new hobbies like reading lots of books instead of riding her bike or playing soccer. She had many appointments with several doctors... wrongly she was given a clean bill of health.” –Aine (as told by family)

Challenges and frustrations…

“Adjusting from having my parents around to take care of me, to taking care of myself during my first year of college was difficult. I struggled to do things for myself, and realized that I needed to take better care of myself. I also struggle with knowing when to tell people of my disease, or who I should tell and who I should not.” –Carson

 “For years, life had been too busy – I had lived on a diet of promises to myself that my time to relax would come when I retired; so I was ready and eager to start my new life. Instead on May 2 I found myself in the hospital emergency room.” – Marie

 “We know now that had the doctors looked further into just that one symptom—shortness of breath with exertion—or had the cardiologist seen her for a follow up; or had they not ignored her low blood states; or had they not labeled her with anxiety; or had the pediatrician followed up where the specialist left off, they would have found pulmonary hypertension...a lung transplant could have saved her life had they not ignored her.” – Aine’s family

Coping…

“Being physically active helped me adjust with stress.” –Carson

“My mom turned to her family, specifically my brother and I; we were her pillars, her strength and support.” –Lakesha

Why do you believe it is important to keep looking for answers?

“I think we put a lot of trust into doctors, but we need to make sure doctors are giving us the best [care]. We need to press doctors to keep working and finding information. It was a nightmare to find out there was a PVOD specialist right here in Colorado after being flown out to California for treatment.” –Lakesha

 Advice for Others…  

“Always keep a positive attitude and appreciate the little things. Cherish being able to do things and be optimistic. Keep trying to do things, it is so much better to try and have to quit, than to not try at all. You have to have an attitude of resilience.” –Carson

“My attitude was good and maybe that’s what helped me cope. I discovered there was life after diagnosis. This acceptance does not mean I liked it, I can dislike something and still accept it.” –Marie

Breast Cancer and PH: Similarities In Dealing With Both Ilnesses

How are breast cancer and Pulmonary Arterial Hypertension the same? They continue to be never ending. PAH has its tests, doctors appointments and effects so does breast cancer. Both can have lots of doctors appointments. Both have support for anyone who needs it.  I was lucky to find a local breast cancer support group and a cancer center. My doctor had flyers up  for a local PH run to raise funds for research. Online is another good place for support. PAH has helped me understand and use Twitter. Message boards are a good source to.

Eleven years ago I had early stage breast cancer. I had surgery, radiation and took medicine for five years. That medicine had terrible side effects. The hot flashes and night sweats were so bad it made me question taking the medicine. I took the medicine four years and decided I wanted a good nights sleep. Because I also have Cowden's Syndrome a mutation of my PTEN gene I am at a high risk for recurrence. So I have a mammogram or a breast CT every six months. This week I saw two doctors and had an ultrasound concerning the growth I have in the bra line of the  left breast  radiated field. I am currently waiting for tests results.

PH and breast cancer raise money. I am slowly finding what I can do to help others with PH. I have gotten back into art. Last year I entered a bra in our local theme bra decorating contest.  Women helping women a local group in Medina County help local women fund cancer screenings with a theme bra decorating contest. There is a big fancy dinner. All the bras are on display. Votes cost one dollar. There are six categories this year. Last years winners were really good. The librarians entered Fifty Shades of Pink bra. The ladies who work at the oldest school in Medina entered a bra decorated to look like a brick. It was called she is a brick house. The event raised enough money to help 26 women.

This year I used the global genes theme Hope its in the Genes. Global genes is a support organization for people with rare diseases. I have Cowden's Syndrome. Cowden's Syndrome is a rare disease where the   PTEN gene is mutated. That gives me a high risk for many cancers including breast cancer.   My bra is decorated in denim. I think it turned out really good. Hopefully it makes lots of money. 

*Story written by Tami A.*

Losing Weight While Dealing With PH -Tami's Story

My name is Tami Arnold. I have pulmonary arterial hypertension, right side heart failure, a large painful arteriovenous malformation, and Cowden's Syndrome.  I had early stage breast cancer in 2002 and colon cancer in 2008. I like food, especially ketchup, tomato sauce and chocolate. Sunday evening was date night for me and my husband. That meant Chinese food. It got so bad the staff knew what we want before we told them. I never met an ice cream I didn't like. For a long time I ate way too much so...I gained weight.

I like working out. Exercise class was fun. Having thirty minutes on the treadmill listening to my music is great. The feeling I got completing a personal workout goal was great.  I grew up in an active family.  My dad was a distance runner. We were on the swim team in the summer. Swam during the day. Fall was swim team at the Y and gymnastics after swim practice. As an adult I did exercise classes, worked out and did spinning class with my husband. For along time I didn't see the connection between what you ate, exercise, and how much I weighted. Lots of things helped me put everything together. A big part was when I found out about my PAH and right side heart failure. Over the years my arteriovenous malformation  needed embolismed to help with the pain. In 2007 I had an emblozation. I left the hospital with an upset stomach, shortness of breathe and heart palpitations. Before the end of the week I was back in the hospital. After a two week hospital I had a PAH and right side heart failure diagnoses.

Two thing helped me realize I needed to do something about my weight.  I felt really bad. So bad that when anyone mentioned my weight I didn't care. While recovering I realized I didn't weigh that much when I was pregnant. Who ever thought my weight would be almost 200 pounds. Our health insurance was offering a rebate to anyone who joined Weight Watchers. You had to pay in full, were only allowed to miss three meeting and the insurance company got your results. The rebate made me want to give it a try. After talking to my husband he wanted to join to. That was great. We had support at home.

Because I was working we ended up going to different support meetings. My first  meeting was not bad. You stand in line and step on the scale for a total stranger. I am 5 ft 5. My starting weight was 189. I was upset with my self and grateful no one saw the scale. The lady who saw my starting weight was very encouraging. She told me I didn't have much to lose, then pointed to her before and after picture. The weeks I gained weight because of fluid retention she was understanding.  Every week I got in her line. My first meeting, I was the only newcomer. After telling the instructor about PAH, her first words were follow your doctors advice first. All she knows  is the program. I still follow her words today.

The first couple of weeks were frustrating. Along with counting points I had to look at sodium. Yes we got starter recipes.  We didn't have most of the ingredients in the kitchen. I never liked math let alone counting points and keeping track of what I ate. So I decided to try new things with an open mind. We bought bananas, carrots and any fruit in season on sale in the house to snack on. I tried lots of new things for dinner. Who knew chicken could taste so good. I marinade chicken in mustard and lemon juice. Before 2007 I didn't like any kind of mustard.  I made fish for dinner using just the microwave.  I can make  three kinds of chili, better than my mom. We still have steak. Now we have one steak and split it. Yes there are time I think I'm going to scream if I see another baked potato. But I make great baked steak fries. Fried rice takes time but I getting good at making it.  Because of right side heart failure I don't have much of an appetite, so I started eating only when I was hungry and stopped when I was full. That seemed to work for me. I did not count points.

Because of fluid retention my weight loss was up and down. That made losing weight  very hard and frustrating. My husband Dave lost weight every week. Doing it together helped. Dave learned to cook. If I found a recipe I liked, one of the first things I looked at was the  sodium contain. Who knew ketchup and spaghetti sauce have so much sodium. We now have a kitchen full of spices. It had been a long time since I used the crock pot. The last time I opened the canned soup, added water and put the meat in. Crock pot cooking has come a long way since them. Now a days you brown the meat, add fresh vegetables, spices. No more canned soup. That adds way to much salt. Last Thanksgiving we cooked a small turkey for two in the crock pot. It turned out great and we had leftovers for a couple of days.  Don't get me started on healthy microwave cooking and why I like fish.

I have to do whats best for me when it comes to exercise. Because of  PH, heart failure issues, and  I can not feel the bottoms of my feet an issue left from chemo. After breaking my foot I do things at my pace. The walks might be slower by I'm walking.   In Ohio there are lots of reasons its hard to exercise every day. As the days get shorter I'm only about to go with hubby and the dog on the afternoon walk. Snow and bad weather will make even that walk harder for me.  In bad weather I try to use my environment for more exercise.  If possible I make an extra lap around the store. 

My doctor keeps telling to to focus on what I can do not what I use to do and quit letting pride get in the way. That is really hard for me.  We went to an Indians baseball game this July. Our last row seats were hard for me. Baseball games mean LOTS of walking. The last two flight of steps were straight up. I had to stop twice. My brother saw that. 

I am learning my mom and dad had good ideas about food and exercise. My mom only bought one box of cookies every two weeks. We had one box of sugared cereal every two weeks. That's enjoying something you like while eating healthy. I still eat chocolate and ice cream. Just not every day and in smaller amounts. I like to cook again. Since 2007 I found out fast food isn't fast. What I can make is fast, tastes good and low in salt. I have given up the salt shaker. Found is better with less salt. Exercise is what you make of it. Start small and do something every day. My husband Dave and I have kept our weight off since 2008. Teamwork helps. He loves to cook. I get new food ideas from all kinds of places. On Twitter I found someone who's blog had the best ideas about easy,cheap, low salt homemade pizza. We had pizza for dinner last night. Reward your successes. I like the occasional peanut butter cup. Shopping for a smaller size never gets old. Yes I still look at my skinny reflection whenever I can and smile. My weight today is 153. Tonights dinner is opened back fried chicken made with corn flake crumbs with my baked steak fries and cole slaw.

Double Jeopardy -Rare disease x2

In 1973, at age sixteen, while living in Jamaica, WI, I noticed strange things happening to my body. My athletic, lithe self was slowly becoming tight, stiff and painful and I was tired all the time. This phenomenon could not be seen by anyone, but I definitely felt it. Over the next few months, my lack of speed was obvious in my track and field competitions and in my basketball practices. The pain and fatigue became overwhelming and as a result I had to drop out of high school my senior year.

A diagnosis of scleroderma (rare disease #1) was made about a year after my initial symptoms and the doctor told my parents I had two years to live. This time frame was kept secret from me for about 10 years. I spent 4 months in the hospital and my dramatic physical decline coupled with what I now realize was a deep depression kept me in a downward spiral to the point where I essentially became an invalid. During a period of about 8-10 years after my initial diagnosis, for no apparent reason, I began feeling better. The darkness cleared and I slowly began to regain my independence. With family support and by the grace of God, I was able to finish high school (at 26), get my driver’s license (a big deal at the time), graduate from college (at 34) get my master’s degree (at 35), travel extensively over Europe and the Caribbean, purchase my own house, mentor numerous children, and work in a career as an accountant for 25 years before being hit by PAH in 2009 (rare disease #2).

My house is right around the corner from the bus stop and one cool March day, as I left my home to run an errand, I heard the familiar diesel engine sound of a bus slowing down. So, like I had so easily done countless times before, I ran to catch the bus. But this time even though I caught the bus, I was completely and almost totally out of breath the point of near fainting. Luckily there were available seats on the bus. After a few minutes the palpitations slowed and I was able to relax. At my next doctor’s appointment I mentioned the incident to my rheumatologist who immediately knew that it could probably be pulmonary arterial hypertension (PAH) and referred me to a PH specialist.

Dr. Roxanna Sulica from Beth Israel Hospital in NYC did a right-heart catherization and determined that I indeed had PAH. Since then my life has taken a different turn. I retired a year after my diagnosis and, after repeated allergic reactions to Letairis and Tracleer, I am now stable on Adcirca, and I get tremendous benefit from pulmonary rehab, support groups and the educational conferences and forums.

To everyone with PAH and/or scleroderma, realize that there are going to be stages to your life and you will have to keep making adjustments to your “normal” every time you experience a setback or a disappointment. However, your life can still be rewarding and full even with 2 rare autoimmune diseases.

Somebody once said, “If you choose to sing, you will always find a song,” and I choose to sing.

MY BIO

I was born and raised in Jamaica, WI.  I moved to the United States in 1983.

I retired in 2010 at 55 years old from a 25-year public service career as an accountant with NYC government.

I now volunteer with an organization called New York Cares and support community service projects close to my heart, especially those that benefit the well-being of the disabled and other disadvantaged groups.

My PAH is secondary to scleroderma. I have experienced practically all the manifestations of both diseases. I do not travel out of the country as extensively as I used to, primarily because of the many adjustments needed for air travel with PAH. There are times I cannot get out of bed, but when I can I make sure I get out of the house. I am determined to keep a song in my heart because it makes me feel better and reinforces my usual positive, hopeful outlook on life.

I live in Brooklyn, New York and I have a host of family members and friends close by for support.

Josiah's Journey with PH+

by Jan Windz

When we met Josiah he was living in a group home for medically fragile children. At that time he was 4 years old, very tiny, non-verbal and had just started to walk. He was wholly g-tube fed and on oxygen 24/7. He would become cyanotic when you took off his oxygen to pull his shirt over his head. He was considered to be terminally ill and non-adoptable. His prognosis was that he would be dead within a year without a heart lung transplant. However, he was not considered a candidate for a transplant because he was in the foster care system. He carried diagnoses of primary pulmonary hypertension, complex congenital heart disease, severe infantile scoliosis, and Noonan's syndrome. He was also, at that time, thought to be autistic.

We had been approached about adopting Josiah because we already had some experience with transplantation. On August 31, 2001 I was blessed to be our adopted son, Felix's kidney donor!

As soon as we brought Josiah home I got him in to the pediatric cardiologist at UC Davis Medical Center in order to have them prepare the packet for transplant evaluation at Stanford. About a month after we sent the package to Stanford I received a call from Dr. Jeff Feinstein, at the Wall Center for PH, Lucille Packard Children's Hospital. He said "before we jump to transplant, why don't we actually try treating the PH!"

Since Josiah has begun being treated by Dr. Feinstein he has become a new child. He has now been on Tracleer (Bosentan) for almost three years. For us this has been an absolute miracle drug. He has never had any side effects. The child who became cyanotic in moments of being off of his oxygen now needs to be reminded to wear it. He can go outside without it and ride his bike for 30 minutes at a time! He still gets most of his nutrition from his g-tube feedings, but he is taking food orally as well. He hasn't stopped talking in the past two years (except when he is asleep)! He is learning, loves school and has only had one, very brief hospitalization since he has been with us. A year ago Sildenifil was added to his regimen. While the affect hasn't been as dramatic as with the Tracleer, it does seem to be helping as well. We are currently anticipating surgery in December for his scoliosis. Dr. Feinstein will work with the surgeon, also at Stanford, to be sure that Josiah will tolerate the surgery.

We don't know what the future will bring. Josiah still has severe pulmonary hypertension. However, he is currently doing incredibly well. And every day brings new advances in medicine.

We are truly blessed.