Cystic Fibrosis

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    Community Voices

    Being Diagnosed Late With Atypical Cystic Fibrosis and Having Trust Issues

    I have trust issues. And amazingly, they don’t stem from one too many “trust falls” gone wrong from my younger years (though maybe that contributed). My trust issues center around bigger things: trusting my body, trusting my doctors, and trusting… life. I was diagnosed with atypical cystic fibrosis (CF) one month and eight days before I turned 20. I had been complaining to doctors for as long as I can remember about having trouble breathing, being tired all the time, getting sick if I ate “normal” kid food, and of course, my bi-monthly “asthma attacks” that left me unable to talk, breathe, or swallow for up to a week. “It’s asthma,” my pediatrician would say. “She’ll grow out of it.” When my chronic digestive symptoms worsened around age 13, I was told to drink prune juice. When that didn’t work, it was medication. Nothing worked. My diet became more and more restricted to the five things I knew wouldn’t make me sick: lettuce, cucumbers, frozen strawberries, bananas, and protein powder. I got sicker and sicker, and it was clear that I wasn’t healthy. I started figure skating when I was 8 years old and quickly knew I had found my heart’s passion. I fell in love with it and trained to the best of my body’s ability — withholding from my coaches the pain it caused. I felt like figure skating seemed to wear me out and be a lot harder for me than for my training partners. Skating isn’t a sport that welcomes showing “weakness,” and since I was being told by all of my doctors I was “fine,” why would I say anything? After graduating high school, I moved and started training at an Olympic training center with renowned coaches from all over the world. I was living my dream—but trying to ignore the so-obvious fact that my body was breaking down. Just from my appearance, it was hard for anyone to ignore it — including my coaches. After only five months of professional training, I had a serious fall that took me off the ice and also led to my getting diagnosed with osteoporosis at only 18 years of age. I was terrified and heartbroken — being told I needed to stop skating because it was too risky for my bones was hard. I was no longer able to train or skate at all, so I moved home feeling utterly defeated. I knew something wasn’t right — everyone did — but I didn’t have any faith that doctors would finally be able to tell me what was wrong instead of just dismissing me as usual. Ironically, my saving grace came in the form of a lung infection — pseudomonas aeruginosa, to be exact. I woke up unable to speak or swallow, thinking my training-too-hard days were finally catching up with me — only to find out that my lungs were swimming with super-rare, hard-to-harbor bacteria. The urgent care doctor casually dealt me some medication and assured me I would be better in 10 days. Being the investigator — and skeptic — I am, I wasn’t convinced (or reassured), so I consulted “Dr. Google” to find out how in the world I came down with this rare infection. I learned that there are basically three conditions that can make you contract this bacteria — acquired immunodeficiency syndrome (AIDS), cancer or cancer treatment, or cystic fibrosis. I knew I didn’t have AIDS and I had not had chemotherapy or radiation, so all that remained was cystic fibrosis. “There’s no way I have that,” I said to my mom. “That’s that ‘baby disease!’ They would’ve caught that.” I pulled up an article about CF, reading through the symptoms just in case. As I did, my heart sank and tears flooded my face as I realized that everything made sense. All the puzzle pieces fit together. How in the world had the doctors missed this? I returned to my pediatrician — now no longer content to accept her prune juice and prescriptions. “I want you to test me for cystic fibrosis. I think I have it,” I insisted. She ceased her note-taking and looked up at me blankly. “I think you might be right,” she replied. I was referred to my nearest cystic fibrosis center for evaluation and testing. By the time of my appointment, I had struggled to eat and was so weak I could hardly walk. When the doctor at the cystic fibrosis clinic walked into my room, she took one look at me and immediately sent me down to the emergency room to be admitted. I had a complete abdominal obstruction, pancreatitis, four different lung infections, liver disease, and kidney disease. A few days later, I was also diagnosed with cystic fibrosis. A little less than a year after that, I got my genetic test results and was told it’s not “typical” CF, but a rare variant of it instead — one that doctors and researchers know absolutely nothing about. All they knew from clinical observations is that those of us with this form of CF fare no better than others with CF… and sometimes we’re even more affected. My initial shock-turned-sort-of-relief after actually having a diagnosis was smashed once I was told, “We don’t know how to help you.” The fact that my condition was getting more severe every month didn’t help. I started having one obstruction every week. I upgraded to GoLytely as my drink of choice — until even that stopped working. I did everything I knew to do, and my efforts did help — but only to keep things at bay. Eventually, the things I tried weren’t even readily doing that. In 2019, I had surgery for an ileostomy, which improved my quality of life and health tremendously… but not before I experienced literally every complication you can possibly have with an ostomy. One complication still lingers — prolapse — a common symptom in both CF patients and those with Ehlers Danlos syndrome (EDS), which I was also diagnosed with in 2020. Living with both cystic fibrosis and Ehlers Danlos syndrome is hard for me. The two diseases so often conflict with one another (Imagine a chronic cough with a hole in your abdomen and weak connective tissue!), and I honestly struggle to deal with them both. Most days, I feel terrified of when the other shoe will drop. I know I have more surgeries ahead — some of which will be harder than the ones I’ve already had. It’s another terrifying thought. So how can you trust that everything will be OK when nothing has been OK in a long time? I honestly don’t know. And after a long time of trying to figure it out, I’m not trying anymore. Trust is a brutal battle — just like an organ shutting down or an insurance pre-authorization. Sometimes trying to find the answer to unanswerable questions — like how to trust with a disease — isn’t necessarily the answer we maybe should be trying to find. Sometimes the only way to figure out how to keep going is to step back and realize we have been going — day after day, month after month, year after year. And even when it’s our bodies that so often betray us, we may need to step back and acknowledge that our bodies have enabled us to keep going. Sick or not, CF or not, EDS or not, it keeps surviving. And that body? The one that’s been through what it’s been through and still keeps on? That’s something we can trust.

    Holli Reherman

    Supporting My Independent Teenage Children With Cystic Fibrosis

    The room is dark and cold. I hear the familiar hum of an airway clearance machine in the next room overcoming the thunderstorm of white noise and air conditioning. It is 3:00 a.m., and the room next door belongs to my 12-year-old son. My kids have chaotic sleep patterns. Years of medications, hospital stays, and one parent who can’t sleep mean someone is always awake. I hear the choking sound of a mucus-filled cough. My throat catches, and without even being fully awake, I stumble out of bed to peek in the next room. My son, who is wise for his 12 years, reminds me, “Mom, this is supposed to make me cough.” I’m proud that he knows how to handle his own middle-of-the-night airway challenges now, but I don’t think I will ever sleep easily after hearing that familiar wake-up cough. It used to be easier, and it also used to be harder. My middle two children both have cystic fibrosis, so they’ve had extra routines from infancy. Extra nutrition, pancreatic enzyme tablets poured into apple sauce and spooned into gummy mouths. Cupping my hands and clapping my children’s’ backs while holding a nebulizer (and always getting an inadvertent dose of any medication they took), and wrestling a wiggling, often annoyed infant is what treatments used to look like. Changing a diaper quickly so that the familiar “baby boy shower” during a diaper change did not fountain upward and damage the PICC line in my baby’s head. Silly and serious memories were one and the same. With the exception of enzymes, medicines were either inhaled or liquid. Regardless of the medications’ method of absorption, one thing was constant: I ran the show. I was meticulous and anxious, not wanting to get anything wrong. There was so much to remember and so much to try to shoehorn into a “typical” childhood. I made the routines, I kept the routines, and with them, I maintained some vague sense of control in the face of an uncontrollable and uncertain disease that hovered over us. Like an unwanted cloud, ready to rain, rage, shade, or tornado at will, the disease remains. I stood ready to try to control the consequences of whatever this perpetual phantom storm. Then suddenly, it wasn’t my show anymore — but it never really was. My two children aren’t babies anymore. They have entered the teen years — or nearly so for my younger child with cystic fibrosis. They’re in the double digits — pending adulthood. I still fill the pill boxes. I make the refill calls. I remind my children of what to do all day long. But treatment challenges are no longer something I can shoulder for my kids. I grieve that I can no longer protect them from the time their condition takes each day — the responsibility of airway clearance, pills, inhalers, and breathing treatments. In my sorrow, I look up and see my children’s faces. They have this handled. They know what to do — but it still sucks. The irony of cystic fibrosis is that a healthier life is often a harder treatment responsibility. The teen years bring new challenges, like diabetes, but CFTR modulators bring me hope — beautiful glorious hope. These two amazing young people — like so many others before them — have got this handled. Whatever that storm cloud throws out, they have trained for it since birth. This moment has been coming, and now, I have to let go. I remember the preschoolers swallowing pills, the 8-year-old who was able to handle his own tube feedings after watching us, the 10-year-old who could talk to his nurses expertly about his fear of getting an air bubble in his IV line. My children know what to do. They will have my support and any help they will need for the rest of my days. The adage “Do for, do with, cheer on” rings true. So I lay back down in bed, closing my eyes to my son’s hum and cough. I don’t sleep, but as I listen to the familiar lullaby of a compressor, I grow into my new role in my children’s lives.

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    Megan Glosson

    How Roe v. Wade Being Overturned Harms People With Health Conditions

    This past Friday, the Supreme Court voted five to four in favor of overturning Roe v. Wade. This landmark court decision from 1973 established the constitutional right to abortion. Now, individual states will get to decide whether or not they will allow abortion. People across the country are experiencing mixed feelings about this perplexing court ruling. However, many people, including the justices who voted in favor of overturning the court’s previous ruling on the matter, are not thinking about the way in which this decision will impact the millions of American women and people with a uterus who live with chronic health conditions. So, here are just some of the people the justices of the Supreme Court of the United States failed to consider when they overturned Roe v. Wade and all but outlawed abortion for over half of the states in our country. 1. The Transplant Recipients Whose Medications Make Pregnancy Problematic Although it is possible for a transplant recipient to get pregnant and carry a baby to term, there are many potential complications. First and foremost, many anti-rejection medications can cause birth defects that would make life unsustainable for the fetus. They can also build up to toxic levels in the fetus’ bloodstream, which can lead to other complications. Although there are some medications that are safe for the fetus, any change in immunosuppressants must be made gradually so doctors can measure if these medications are actually doing their job (because not every medication works for every person). Also, because medication levels are based on weight, pregnancy can impact the medication levels in a way that leads to organ rejection during the pregnancy, making it a life-threatening situation for parent and fetus. 2. The People Whose Autoimmune Disorder Could Cause Complications Many autoimmune disorders cause your immune system to attack healthy tissue. This means that a pre-existing autoimmune disorder can interfere with the pregnancy by harming the fetus. Even if the autoimmune disorder allows the pregnancy to continue, the mother’s antibodies can enter the fetus’s system and disrupt its development and growth. Furthermore, some people don’t even know they have an autoimmune disorder until their pregnancy triggers it. In these cases, a person may find that being pregnant is interfering with their life so much that it’s not possible to continue living while pregnant. 3. Those Who Live With a Genetic Disorder That Could Prove Fatal for the Baby Living with a rare disease is not an easy road. However, some genetic disorders can be fatal, and passing them down to a child can increase the risk of fatality for the baby. These conditions include Huntington’s disease, vascular EDS (vEDS), cystic fibrosis, Marfan’s syndrome, and many other genetic disorders that someone can either have or be a carrier for. Even if the baby makes it through delivery, they will have a hard life (assuming they can sustain life). 4. The People Whose Endometriosis Caused an Ectopic Pregnancy People with endometriosis are twice as likely to experience ectopic pregnancies than the average person. Unfortunately, there’s zero possibility of an ectopic pregnancy becoming viable, no matter what marvels of modern medicine an OBGYN can perform. Usually, ectopic pregnancies are treated with injections that end the pregnancy or surgery to remove the fallopian tube. Either way, these life-saving medical procedures can be considered forms of abortion, and would now be punishable by law in some states. This means that people could face jail time for something completely out of their control just because they chose to save their own life. And without Roe v. Wade, there’s nothing a person can do about it if their state’s court system decides to rule against them. 5. Those Whose Cancer Treatment Would Affect the Fetus Breast cancer is the most commonly occurring type of cancer for women, and breast cancer rates are on the rise for women of childbearing age. Unfortunately, many of the recommended forms of cancer treatment can cause harm to a fetus and are not compatible with pregnancy. There are instances where a pregnant person with cancer can either wait until after their child is born to undergo treatment or select treatment methods that are least harmful to the fetus. However, there are also times when someone may need to decide whether it’s better to end a pregnancy during the early stages so they can undergo cancer treatment, or risk bringing a baby into the world while also dying. It’s not an easy decision to make either way, but the overruling of Roe v. Wade now makes it even more challenging. 6. The People Who Live With Mental Health Conditions That Require Daily Medications There are countless mental health conditions that require daily medications. Some of these conditions include depression, anxiety, bipolar disorder, and schizophrenia. However, even with the wide variety of available medications out there for each and every single mental health condition, the American College of Obstetricians and Gynecologists still says a majority of these medications are not safe during pregnancy, especially during the first trimester. Whether a pregnancy was planned or unexpected, a pregnant person who uses one or more psychiatric medications may be forced to decide whether or not they want to expose their fetus to the risks associated with the medication. In many cases, these medications can cause harmful birth defects or even harm the fetus in a way that makes life unsustainable. Therefore, these individuals need as many choices as possible available to them, including the right to terminate the pregnancy if that’s what they and their medical team feel is best. 7. Those Whose Epilepsy Puts Them At Risk for a Stillbirth Women with epilepsy are up to three times as likely to have a pregnancy that results in stillbirth than women who do not live with epilepsy. Sometimes, there’s no way of knowing whether they will experience a stillborn birth, whereas other times an OBGYN may no longer detect signs of life before the pregnant person even hits the third trimester. Without the option to abort, these individuals will be forced to carry a pregnancy to term even though the fetus will no longer grow and develop. 8. The People Who Almost Died With Their First Baby and Don’t Want to Go Through That Again There’s a lot that is still unknown about how pregnancy impacts the body. Conditions like pre-eclampsia are largely undetectable until it’s too late, as are other rare pregnancy complications. However, people who experience these issues during their first pregnancy are more likely to experience them again. This means a person may take active measures to avoid additional pregnancies. Unfortunately, no form of birth control is foolproof, and a person can still end up pregnant even when actively avoiding it. Should these individuals have to go through the same hell they endured during their first pregnancy if they don’t have to? And is that really someone else’s choice to make? 9. The Trans Man Who Would Struggle With the Dysphoria of a Pregnancy Thanks to the advances in modern medicine, trans men can do many things to counteract the gender dysphoria they experience. However, up to 30 percent of trans men still experience unplanned pregnancies. These pregnancies can lead to depression and other concerns due to the mixture of dysphoria and judgment from society. Before the overturning of Roe v. Wade, trans men could decide whether or not they wanted to go through with a pregnancy. Now that it is overturned, trans men in states with abortion laws in place may have no choice, and this combined with the stigma they likely already face due to society’s general view of the trans community in their geographic location, could cause depression and suicide rates to climb even more. This list isn’t exhaustive. However, it does provide a view into just how many people will be impacted due to the Supreme Court’s decision to overturn Roe v. Wade. In many cases, people who live with health conditions are already marginalized and mistreated by medical providers and society as a whole. Now they may face even more problems and harsh judgment just for making decisions that can help them continue to live. This isn’t the type of treatment anyone deserves, especially people who already have to fight for their right to live day in and day out.

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    Community Voices

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    Hi, my name is bearcuddler76. I’m new to The Mighty and look forward to sharing my story.

    #MightyTogether

    #CysticFibrosis

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    Self-Advocacy Can Be A Struggle

    Part 1 of 5 Response to dismissal from services letter

    Dr W,

    I had held you in such high regard. You seemed to be a doctor who genuinely cared. You spent longer than your HMO allotted 15 minutes with your patients, asking appropriate questions, explaining things in layman’s terms, and answering questions even with the client might have thought their question would be considered stupid.

    You knew a good deal about what I had been and was going through:

    The family murder/suicide
    The fact that I was dealing with unprocessed #Trauma from being enlisted in cleaning up the bloody crime scene because the police nor coroner’s department perform this service
    That I was turned away for #MentalHealth services to process this #Trauma by 25 clinics in Oregon and 5 clinics in Nevada citing Medicare

    Quotas
    That calling the Oregon State Ombudsman’s and Governor’s Advocacy offices only resulted in me being told they only help with Medicaid
    That I make $200 too much with my SSDI and tiny pension from the County of San Diego Department of Health and Human Services to qualify for anything but Qualified Medicare Benefits under Medicaid with a $2600 spend down which is impossible to meet
    That a clinician in Nevada, who said she would work with me, fired herself 2 weeks in saying my family issues were too triggering for her
    That calling NAMI only resulted in advice to lodge a complaint with the Federal Comptroller
    That the representative at the Federal Comptroller’s general phone number informed me that filing a complaint had no effect on obtaining care and that I would hear nothing about the disposition of any investigation nor whether it would be determined if an investigation was even warranted
    That the primary care physician in Salem, OR, refused to even consider that I might have a mild or moderate form of #CysticFibrosis despite:

    The progressively worsening cough I’ve had since as far back as 12 years old
    I have chronic #HereditaryPancreatitis
    My daughter has one marker for the SPINK1 mutation
    Two of my granddaughters have both markers and have a heterozygous manifestation of the hereditary #ChronicPancreatitis
    That my sister and her son have a genetic mutation of the CFTR gene

    That after the murder/suicide and subsequent denials for #MentalHealth services, my daughter and the rest of my family expected me to still be on my best behavior and ended up going no contact with me for small disagreements which unnecessarily escalated because of their lack of compassion
    That I was battling with my apartment management over their willful negligence regarding

    The plumbing leak
    The storm damage in the ceiling
    Mold growth in the apartment with dissemination into the air through the HVAC system
    The snakes in the apartment; which, took an adult protective services worker’s involvement to remedy

    That the only resolution offered me regarding my living conditions by three legal aid agencies was a 14/30—an ultimatum I was unable to follow through with because I had no money to move
    The fact that, because of the mold exposure, I came to your office to be tested for mold in my lungs only to discover from the lab results of the sputum sample that there was actually Pseudomonas aeruginosa in my lungs
    I am having trouble with medical transportation because:

    I have no vehicle
    My son, friends, and neighbors have no desire to help
    My Humana Medicare Advantage Plan offered a choice between in home services should I become unable to care for my own hygiene or of receiving medical transportation services. I chose the in home services because it had been helpful in the past after back surgery and I had always been able to secure medical transportation in the past from Johnson County #MentalHealth Transportation Services
    When I had trouble getting a medical ride from JCMH, I was informed by the program director that the program is officially only for the work and school rides of clients and because of short staffing caused by #COVID19 exceptions are now an extremely rare occurrence
    That I was turned away from 37 different agencies which provide medical transportation because either I’m not a Medicaid client or because, I live on the outskirts of De Soto, I am out of the service area
    That ride-share is unaffordable to me—it cost me $80 round trip from De Soto to KU Med West to provide the lab with the sputum sample you requested

    That I was having problems getting treatment for the Pseudomonas in my lungs from the pulmonary clinic when the Levofloxacin you prescribed me

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    Self-Advocacy Can Be A Struggle

    Part 2 of 5 failed to resolve the symptoms, including the pulmonary nurse putting in my chart that I was being non-cooperative with regards to picking up the #Smoking cessation medication which I, myself, had asked for and did, in fact, pay the $90 copay to have the prescription delivered to me
    When I called your triage nurse asking for your help in receiving the treatment you referred me for she said it is not your problem
    When I brought this to your attention you told me to speak with the pulmonary nurse
    That I spoke with the pulmonary nurse supervisor and KU Patient Relations representative, to no avail
    That I spoke with the Humana Quality Assurance Social Worker who added my complaint to an ongoing CMS investigation of KU
    That I was experiencing debilitating side effects from the Levofloxacin making it difficult to walk and had to hire an IHSS housekeeper who stole the tetracaine left by the EMT when I suffered an inadvertent chemical burn in my eye because of cognitive impairment caused by the Oxycarbazine prescribed to me by my psychiatrist for management of anger resulting from all of the aforementioned maltreatment and I had to fire her

    Knowing all of this, you made sure I was sent a dismissal from services letter from the entire KU Physician’s Network after I sent you an angry message through the My Chart system upon discovering that I had been given yet another misdiagnosis which, because it was a #MentalHealth diagnosis rather than my symptoms being appropriately assigned to #Migraine aura, being in my chart might give any doctors who would work with me from that day forward an inaccurate presupposition of what it would be like to work with me.

    You knew my outburst was way out of character for me after having been my Primary Care Physician since 2009. Yet, you failed to respond with something along the lines of, “This is very out of character for you. Are you having trouble receiving #MentalHealth Services? Do you need a referral for #MentalHealth Services through KU? Would you like another Neurology referral to see if that diagnosis could be reassigned to the Intractable #Migraine Aura?” You know I have never in the past been a difficult person to work with. And, even if I were, difficult people still need healthcare services.

    I received no warning, no offer of a behavioral contract, and no resources were included with the dismissal letter to assist with finding a new Primary Care Physician. In addition, the Dismissal letter from the KU Physician’s Network constitutes patient abandonment because I was dismissed from services during a critical juncture in my treatment and the 30-days allotted me for urgent care was insufficient when you know I live in a semi-rural area and lack medical transportation. There was also no follow-up from the KU Med West Social Worker to ensure I had been able to obtain appropriate care after the dismissal letter was sent.

    Because of the dismissal letter, I was unable to keep the appointments for the CT scan to determine whether I had a true infection or a colonization of the Psuedomonas in my lungs and sweat chloride test to determine whether I have a mild or moderate form of #CysticFibrosis . I vehemently fought for these tests after the pulmonary doctor requested the referrals and the pulmonary nurse subsequently dropped the ball on putting the referrals through. Then, it was discovered by the lab through a follow-up sputum sample that I now have Fusarium in my lungs for which the pulmonologist you referred me to refused to treat me saying, “Everyone has Fusarium in their lungs,” and also sent me a message through the My Chart system saying, if I didn’t like the treatment I was receiving—which was none—to find another doctor. The dismissal letter from the entire KU Physician’s Network took these tests and appropriate treatment for the Fusarium away from me.

    I tried going to the Overland Park Regional Hospital ER when I was having trouble breathing. Remembering me from the 5/2021 visit wherein I requested an anti-#Anxiety prescription after my daughter publicly accused me of pimping her out to her husband before they were married, had five children together, and had a successful business under her contractor’s license, the Overland Park ER doctor thought I was just there in an attention seeking capacity. Even though I was unafraid that I would be in trouble over the accusation because a judge would question the validity of the claim since she asked me to move to Oregon to caretake and supervise her children in her absence, I was still very di