As the topic in Crip Camp last Sunday addressed creating community, I thought I would revisit the history of the groups in the USA that offer resources to those specifically who caregive and parent children and teens living with cystinosis and those groups addressing cystinosis adults:
Original foundation that organized the cystinosis community and petitioned approval of cysteamine therapy-Cystagon treatment made available for the first time. First foundation to provide funding in 1985 to Dr. Jerry Schneider’s research on determining carrier status, as well as some of Dr. Doris Trauner’s (Angela Balantyne, Ph.D) neurologic research; also provided grant funding to Dr. Jess Thoene, at a time when funding for rare disease research was drastically cut, Dr. Corinne Antignac at Necker Hospital in Paris.
Sixteen different countries now provide support to newly diagnosed families as well as others at advanced stages in their cystinosis journey: Australia, Belgium, Brazil,
Canada, France, Germany, Ireland, Italy, Mexico, the Netherlands, New Zealand, Turkey, South Africa, Spain, United Kingdom, and Venezuela. cystinosisfoundation.org
Original focus was on more funding for cystinosis research. The first studies focused on gastrointestinal issues and feeding issues both for children and adults.
Now the CRN is a private, non-profit 501 (c) (3) corporation, all volunteer dedicated to supporting and advancing research, providing family assistance and educating the public and medical communities about cystinosis. Clinton Moore is current President and Christy Greeley is the executive director. cystinosis.org
Day of Hope Annual Family Conference and Natalie’s Wish Fundraising Event
This private fund provider has the mission to finding better treatment and an ultimate cure for cystinosis. First research grants were issued to Dr. Jerry Schneider and Dr. Ranjan Dohil to find a delayed-release form of cysteamine (now known as Procysbi) and to Stephanie Cherqui, PhD for stem cell and gene therapy research. Since 2003 the CRF has issued 151 mulit-year grants CRF funded research studies in 12 countries. CRF researchers have published 67 journal articles.
The CRF sponsors a biennial 2008- CRF International Research Symposium for CRF funded researchers and scientists. In 2018 CRF will host the 6th symposium in Irvine, California. The (CRF sponsored biennial and invitation only) co-chairs are Corinne Antignac, MD, PhD, Julie Ingelfinger, MD and Stephanie Cherqui, PhD.
The CRF, in collaboration with other cystinosis advocacy groups around the world, established the first and only international patient registry. The Cure Cystinosis International Registry (CCIR) is a hub of information for patients, researchers and clinicians.
2017- Future By Design (Aimee Adelmann and Jennifer Loglisci)
CRN has funded an adult only groupspearheaded by Jennifer Loglisci and Aimee Adelmann with the hopes for adults living with cystinosis to impact the future of their treatment. This group was disbanded by CRN in October 2018. And CRN announced the formation of Adult Leadership Advisory Board (ALAB)in January of 2019. The goals for ALAB are similar to those of Future By Design with training for leadership skills provided by CRN.
Next Generation of Cystinosis a 501 (c) 4 (pending) volunteer group led by majority cystinosis affected adults without the supervision of caregiver/parent-led organizations to address the continued unmet needs of adult issues in Cystinosis and also to address policy issues affecting rare disease people. Please read about NGC here in their introduction digitally published by their partner Patientworthy.
I have found the disability community to mean home and family to me because my needs will be met if I ask for help. It is not special that I have needs or uplifting in the way others express feeling in their gladness that their needs are not like mine. It’s just me with a lot of other people who have various mild to severe needs different than mine. We learn from each other in describing what it takes to live in our bodies through our days.
We also talk about our other identities besides disability. While health is what brings us together, many intersections cross-over and makes each of our stories unique and special over our disability and disease differences.
When the topic came to sex and disability, my experiences and knowledge with this human act has been shaped by my identity within a strict religious framework. I was taught sexual education by my mother from Catholic textbooks about men and women and what they do to conceive a child after marriage. Even though my family attended a fundamentalist Baptist church, my mother used the Catholic books because the fundamentalist culture didn’t recommend teaching children and teens about reproduction but to prolong the teaching until an opposite-sex couple was engaged to be married.
Religious identity is complicated for me as a disabled female. I still adhere to certain doctrine and theology inside of reformative Christian belief. However, I need sex education that considers my choice to not be sexually active for the purpose of having children, and also the reality that legal marriage is something I may never participate in as someone receiving medical benefits. Its 2020 and upon legal marriage, I would be at risk to loose the benefits I have in receiving healthcare.
I have yet to attend a cystinosis conference that hosts adult meetings with speakers relevant to the spectrum in disabled experience with relationships whether they are sexual and/or emotional and mental. It’s not enough to have a facilitator who is a social worker by trade and a round table discussion of cystinosis adults who aren’t sure if this is a safe space to open up about something so vulnerable and intimate.
Back in 2014, I was doing research for my book addressing church leaders to examine the amount of sex abuse (particularly with women and children) going on in their institutions and to stand for the victims instead of siding with predators. I found those with health disabilities are 10x more likely to be abused than their peers. (If interested in the reality I lived in among strict religious persons who ignored abuse, I have an article available here.)
Trauma-informed facilitators are a necessity in sex discussions as well as speakers who have background in sex therapy and study of human sexuality. Many people with disabilities become parents and live with partners and spouses. Many make other decisions regarding their bodies and partners. Inclusion is important because disabled people have sex or have sexual interests too. And we are often excluded in spaces wherein abled persons get to share first time experiences, things they didn’t know but figured out how to do, and the other side of the spectrum of pain and hurt with people.
In consistently checking my own heart and mind on why my feelings are the way they are when certain events happen, I have come across a principle greatly abused in the cystinosis rare community.
I point this out so that the systemic problems can be fixed, relationships can be restored, and people can move forward. In the cystinosis communities, there is a need for changing narrative, not an erasure of the caregiver/parent perspective, but a shift in how topics are discussed.
I want to recognize the other cystinosis adults before me who have spoken to this effect but were also ignored or re-cast in the light of “they just decided they had other things to work on besides an active role in our organization” and other euphemisms for lack of cystinosis adult participation.
I also want to recognize I am pointing this out because of the observations Black people have made for centuries, and are currently making in light of current anti racist education taking place on a global scale in 2020. As well as those of LGBTQIA identies who have also been pointing this principle of changing narrative, not erasure, to the public for decades in this country.
I also want to recognize I am not comparing oppression: being judged by skin color, sexual identity, and disability are three separate kinds of oppression (and certainly not the only kinds of oppression as gender, class, and religion could be discussed as well) and many people have experienced six, three , or one oppression inside themselves. Which leads me to say I am not participating in what some call “the oppression Olympics” to label those who compare “who has it worse” but rather, I am noting the empathy we as a community should have for these different kinds of abuses on each other as humans. Be patient with oneself first, which is what I find hardest of all.
What is this principle that able-bodied people can also learn in their current efforts to be anti racist and less homophobic and transphobic? It is the principle of inclusion: when a community of people allow those most impacted (those with lived experience) to speak first and listen to their stories before speaking about one’s experience with observing the issue (those who have only considered/witnessed things associated with the topic).
I believe the cystinosis community has a problem with allowing cystinosis adult narrative to lead their organizations because for decades most cystinosis persons died as minors (children or teens). Parents had to lead and champion what other able-bodied persons would not. With this no longer the case, their are a plethora of cystinosis adults who are skilled in leading businesses and non-profits.
Now would be the time to not create a cystinosis adult group within a caregiver/parent-led organization, but to support cystinosis adult groups that are majority led by cystinosis adults.
Now would be the time to create boards in your organizations with majority cystinosis adults in the positions to impact change. If the caregiver/parent-led organization is still informing the cystinosis adults and not the other way around, the organization is still controlled by the feelings and thoughts of able-bodied people and not those most impacted.
Let’s get real here. Why are we still not talking about social security income and Medicaid/Medicare and other resources for single-payer adults within rare community? Because the people leading rare community are of a white, middle-high class, hetero married, able-bodied demographic that does not understand the struggle with obtaining rare medication and ongoing hospitalizations threatening their own job status and health to the point of exhaustion or death. They may understand the struggle on the phone between pharmacy, doctor, and insurance but rarely do able-bodied persons connect with cystinosis adults as equals in this fight.
The history in the USA on disability as discussed by Patrick Cokley has typically been from other people’s ideas of another’s work ability. Disability only gets discussed in regard to whether or not you are a good worker. Production. Production. Production.
It is time to let disabled people, including cystinosis adults, discuss their experiences and reality in the workplace, school/college, and home.
And no, not just allowing cystinosis adults with the same narrative as their able-bodied caregivers and parents repeatedly have the floor but giving all cystinosis adults with diversity of thought permission to share their lived experiences.
There is a thing called “tokenism” when one only has a Black person, LGBTQIA person, or disabled person speak for performative reasons: you want the inclusive pat-on-the-back but not the reality of equity in one’s organization.
Anita Cameron said it better regarding offering that one place out of twenty to someone impacted at your organizations meeting place: “You can be at the table and still be on the menu.”
So, as I stated at the beginning of this article, my feelings and thoughts reflected in my heart led me to write here tonight. Why am I struggling with anger and sadness and why am I so exhausted right now in this world?
I am not angry and sad where I hate people in the cystinosis community. I am angry and sad that in spaces that have been marketed as safe for a cystinosis adult and places wherein I have been told by the caregivers and parents themselves on many separate occasions to tell them the truth and “not let parents speak over me”, that those same good people shut down honest, poignant, and vulnerable speech whether subconsciously or with great purpose. Let me repeat: these are the good people who donate money and time.
I promise you that your identity in your caregiver and parent role is not being erased to no longer engage in rare disease life. If a disease you do not have but manage as a job or because your child is a minor takes over your reason for existing to the point you cannot listen to those most impacted, you may want to find out why you are controlling your profession or offspring so damn much.
Seriously. Get help. And don’t put the burden on cystinosis adults. We have bodies with cystinosis that are overwhelmed and stressed enough and we don’t get to express any feelings about that without being checked by others IN PUBLIC for having “attitudes”.
Along with this photo taken right after the stay-at-home orders in Wisconsin were given (May 26 but I think this state released a bit earlier than that date…more like May 20), I submitted this paragraph for Aware Causes to donate to a fibromyalgia organization:
“I was told by doctors I had fibromyalgia as early as 2014. In 2012, I was clinically diagnosed with general anxiety disorder and ongoing post traumatic stress disorder. I believe that I endure nerve and muscle pain due to how I was repeatedly not validated when I told adults I was sexually abused as a child and when I would report sexual harassment. Some of my nerve and muscle pain is also due to the rare disease cystinosis, but this is not something everyone with cystinosis will live with daily. The purple aware pin also speaks of awareness for domestic abuse/violence. I want to validate that this happens in religious and church families too. The Love your genes hat I am wearing comes from activist Wheelchair Rapunzel.” @cystinosisociety @wheelchair_rapunzel 💜 #awarecauses
Disabled people and those who live with chronic and rare diseases have lives outside of medical spaces. Medical spaces can come with their own instances of trauma, but able-bodied people need to keep in mind that we are people with whole lives too, and trauma can exist for us in multiple layers and spaces.
I highly recommend the book Don’t Call Me Inspirational by Harilyn Rousso to both caregiver/parent and teenagers/adults! The complexities of who we are as humans on this planet is what makes our stories ours. Taking away humanity with a label instead of describing a part of someone with a label is truly a weird nuance of United States 21st century living.
Meanwhile, I experienced a great session with the mental health guide pictured below as well as the returning India Harville from Crip Camp Virtual (she was pictured on my first blog post about Crip Camp).
Lysosomal Diseases exist everywhere around the globe. The story I am posting today is written by Goran Kos who emailed his experiences with Adrenolykodystrophy with me:
“Hi everyone, I don’t really know how to start. I think that the best option is to go chronologically.
I was born in 1990, and since my birth I was a sick child. I remember from my childhood that I was sick a lot; I was susceptible to infections of the respiratory tract and my general health was very bad. Nobody knew why, everyone had their share of ideas, but nobody actually knew anything.
In spring 1997, I got ill. I was just 6 years and 6 months old, and my health got bad. I was visiting my grandparents when I got sick. I couldn’t move, couldn’t get up.
Soon they moved me from one ER to another and from there to our capital city. Their first and only idea was that I had an inflamed appendix and their solution was simple – operation.
Thanks to a good old doctor, maybe some better version of our Dr House, I was diagnosed with Addison’s disease. I am sure that I wouldn’t have survived that appendix operation. My body was weak and fragile.
Before my 7th birthday I was skinny, my skin was somewhat “brownish”, my legs had an “O” bent (they were bent outward – genus varus). For Addison’s they gave me my therapy and I was fine. I gained weight, my health was better, appetite strong and I was “normal”.
By my 10th birthday we already knew a possible cause of my sickness. A rare genetic defect caused a bunch of weird little fatty acids to accumulate on my adrenal gland, and caused Addison’s disease. That defect was called Adrenoleukodystrophy and I wasn’t the only one affected in my family.
Since late 1980s my maternal aunt had symptoms of progressive weakness in her legs. She struggled with her disease until her death in 2017 (she died from lung cancer).
From my 7th birthday to my 21st birthday I was “fine”. My health wasn’t perfect, but I functioned more or less like my peers. I went to school, did my share of mischief, and my good share of video games.
In summer of 2011 I got sick; I had what is called acute adrenal crisis. My body was in some sort of shock, my supply of cortisol was low, and soon I ended in a hospital. I got on some antibiotics and after 10 days they let me go home. I was fine.
In summer of 2013 I started showing symptoms of tremor in my legs. I have developed the same phenotype of my illness that my aunt had for almost 30 years. Since then I have had more and more of problems with my legs and my walk: bad balance, weaker legs, and less general strength.
I don’t know what to say to you who read this, maybe that illnesses come and go and some remain part of us for our whole life. We should not stop and surrender.
If we surrender our destiny is taken from us. If we lose control over what we can control, than we have lost our lives: fight, fight until you win or you can’t speak or move anymore. I shall fight until my last day on this Earth.
There is a patriotic song from my country that has a nice line:
‘i kad nam muške uzmete živote,
grobovi naši boriće se s vama!’
‘and when you take our lives
our graves shall fight with you’
Mi Znamo Sudbu”
Goran found an English translation of the whole poem for me to ponder:
We Know Destiny/We Know Our Fate
By Aleksa Santic ( Alexa Shantic)
Serbian Patriotic Song from a Bosnian Serb-English translation
We know our fate and all we’re in for, o! Yet our hearts in fright will not shiver! Oxen bow down but men do not quiver, For God unto men freedom did bestow.
Our strength is a mountain stream, Nobody shall ever harness its flow! This people is used to dying in woe- By virtue of death itself to redeem.
Our way, that of a god-man, we know, And mighty as a mountain river’s flow, We shall all tread bold the mud thro’!
Far and beyond, to Golgotha one strives, But even if you take our lionhearted lives, The graves of ours shall be fighting you!
What is Crip Camp: the Virtual Experience? First of all, you must know that Crip Camp is a documentary currently on Netflix that highlights a summer camp for disabled people in the 1970s. These campers lives were impacted so much that many of them went on to become leaders in the movement for disability justice.
A note from the film team of Crip Camp states:
“Crip Camp is the story of one group of people and captures one moment in time. There are hundreds, if not thousands, of other equally important stories from the Disability Rights Movement that have not yet received adequate attention. We are committed to using the film’s platform to amplify additional narratives in the disability rights and disability justice communities – with a particular emphasis on stories surrounding people of color and other intersectionally marginalized communities. We stand by the creed of nothing about us, without us. For too long, too many were excluded, and it is time to broaden the number of voices and share the mic.”
Second, you need to know that “Nothing About Us Without Us” isn’t a white American people chant. It was coined by South Africans and according to a Crip Camp post on May 13, 2020 on Facebook, “it shows that no decision can be made without full participation of those impacted by the decision”. In the cystinosis community where I have had lived experience the last ten years, everything goes on in the organizations without adults living with cystinosis impacting any decisions. The abled adults and caregivers inform us and not the other way around.
Between May 17-August 30, 2020, the Crip Camp Impact Program has a free online Crip Camp 2020: The Official Virtual Experience. I signed myself up as I am lacking in education surrounding the intersectionality of racism, disability, and other marginalized communities. Please register at cripcamp.com/officialvirtualexperience.
My last post speaks to being active in disability justice, and I have a long way to go in educating myself to bring this not only to the cystinosis community, but in relearning the ways I have learned internalized ableism, racism, sexism, and ways discrimination and trauma have happened to marginalized groups of people like trans, non-binary, and queer people of color. Religion, class, and family of origin and chosen family play into racism and disability as well. Please take the time to read about 5 Disability Justice Activists to Know This Asian Pacific Heritage Month (the month of May).
[Image Description: Crip Camp quote from Haben Girma, whose headshot is photographed in the bottom right corner, that reads: ” I spent time with people who had different disabilities. We learn so much when we can share ideas and experiences from people who are different from us. That’s when I started seeing myself as a Disabled person. Capital “D” with pride. Haben is the first deafblind student to graduate from Harvard law school.]
There were three speakers my first Sunday session on May 17th and I am going to share their photographs and descriptions so my readers can look up their activism.
May 24, 2020 our Crip Camp was cancelled due to the passing of Stacey Park Milbern, a mixed race Korean and white queer disability activist. I did not know her before her passing. She is quoted as saying, “I want to leave a legacy of disabled people knowing we are beautiful because of who we are, not despite it.” Stacey was the co-producer if the Crip Camp impact campaign. She also created the Disability Justice Culture Club.
Yesterday, May 31, 2020 was another round of three speakers. I will post their pictures and image descriptions below:
Recently, Next Generation of Cystinosis posted this statement on our Facebook page in light of current events that are traumatic and painful for our Black brothers and sisters, and frustrating for white allies:
“Next Generation of Cystinosis stands with Black and Brown people in this time of conflict. As persons living with an ultra-rare disease making us vulnerable to Covid-19, we cannot physically stand with you, but we do stand with you in spirit. We seek to hear you and work with you during this struggle. As people with disabilities we wish to use our voices to lift up yours.
We see the senseless violence toward Black and Brown people and we call for an end to it. We call for justice: justice for George Floyd and all who have died at the hands of police officers.”
I may be reiterating this, but it is crucial to understanding the background of an adult living with a rare disease not fitting into the organizations already out there designed to support children and teens living with cystinosis as caregivers and parents see fit.
The thing I may be reiterating is that I did not grow up surrounded by families with children who also had my disease and their non-cystinosis siblings for my family to communicate with and vice versa.
My mother received regular phone calls from Jean Hobbs-Hotz of the original Cystinosis Foundation, and once we had a pediatric nephrologist who saw me after diagnosis cystinosis at age 5, that was it for outside help.
I wasn’t contacted by any Cystinosis Research Network or Cystinosis Research Foundation. In 2011, Jennifer Caughlin of Caughlin Research invited my mother and I to a townhall. Even at that time, my mother felt like she was no longer a fit in the community as a parent of an adult with cystinosis.
It took me a bit longer for my displacement to sink in but my outsider lense became apparent as soon as I felt the USA organizations were interested in me and my skills as a token to promote within their established leadership, and not an adult who has lived experience with cystinosis, is a subject matter expert on SSI and disability needs, and is also a trained speaker and writer.
I thought about sanding down my edges to be the puzzle piece that fit inside their youth group type adult groups–both inside of Future by Design and in applying for Adult Leadership Advisory Group. I didn’t want any conflict of interest in my advocacy outreach to different rare disease adult groups so I opted to continue studying my communication profession from a far rather than within the parent organizations.
Because I am not some adult who has aged-out of the youth group, I am a privileged adult woman who is college-educated (yes, it is through my white, and then middle-class privilege that I am in institutions of higher education, not my merit) and possessed of leadership skills in speaking and writing on certain subjects and topics, I have embraced a controversial existence.
I have almost 33 years of lived experience which include the microaggressions and out-right abuses from institutions for being disabled, female, and queer. Yes, it was at a CRN conference in 2017 where I was uplifted because an editor from Patientworthy noted my professional value and human worth beyond medicine that I started writing regularly for that rare news website.
It was Horizon who noticed I would speak with honesty and truthfulness about what needs in the adult community are being either blatantly ignored or being re-packaged as work the adults do in support for the teens and children or just being re-narrated under the umbrella of comfortable rhetoric to assuage caregivers and parents.
With Patientworthy and Horizon asking questions and introducing me to a wider audience within rare, I wanted to do more for the adult community than just offer tokenism or patronizing support in the form of segregated groups when simply giving board positions to those adults with cystinosis qualified to lead would have answered adult pleas for resources and help from the established organizations. Keeping adults with cystinosis in previous positions and listening to their suggestions by leading with their recommendations would have acknowledged the dignity of older adults living with cystinosis, I would be remiss not to mention here.
When my friend Mika Covington messaged me to ask about joining her effort to start an organization that would be led by adults with cystinosis for adults with cystinosis, I said yes to informing the community about adult needs and educating the public about the existence and persistance of adults living with cystinosis.
It is so important that those of us adults living rare are visible and leading our own narrative instead of sifting it through able-bodied caregiver and parent lenses. Why? Because those who care most for us, do not necessarily live the daily stress of life along with a rare disease.
They don’t date with the thought of when do I reveal my condition to a potential partner. They don’t work with worries about bosses and coworkers inquiring about the smell of our treatment in our bodies, and if or when we should let jobs know about outcomes scheduled medical appointments or emergency visits have on our health. They don’t apply to secondary education keeping communication open with professors and teachers. They don’t worry about keeping compliant on medications when state insurance is our only option in certain times of our lives and many times we don’t have a good option for paying for drugs with our employer’s plan.
They don’t worry about living with other conditions besides cystinosis. Many of us in the cystinosis community are also the 1 out of 5 with mental conditions. Many of our stories also include other traumas like sexual abuse, domestic violence and neglect, and living below poverty levels. Some of our stories depend on background, race, ethnicity, culture, gender, faith, and sexuality and how living with cystinosis shapes those aspects as well.
We need something we have created ourselves. Not something caregiver and parent approved as we are the parent and caregiver, some of us of more than just our own lives but of biological and adopted offspring and spouses/partners.
I am incredibly proud of certain peers of mine making things happen on their own. Those who have started groups under the organizations only to see it disappear: you did something no one else as done before and you were the inspiration for the current group assisting CRN.
Please. Reach out to Next Generation of Cystinosis if you are an adult living with cystinosis and be a part of history as this is a group of adults living with cystinosis using their skills to create and lead our own narrative in the community and the world.
We do consider people who are non-cystinosis as advisors, but our bylaws state our group will always be at least 60% run by those living with cystinosis in their bodies. We recognize ally support is essential, especially as they listen and ask questions for our improvement.
If you are in need of inclusive community, I hope you find that space where you can grow be it with us or in the adult and teen community groups or your own inspiration of creation.
If you have white privilege and you belong to the middle to upper class in the United States, you have had access to medical care and other patient families living with your condition. You have probably had resources like departments of vocational rehabilitation and have had your needs accommodated to attend a college of higher education or job placement.
I fall into these spaces as I have had assistance as a white disabled female in university (side note: I did not have access in Bible college because it was an unaccredited religious school with no students with disabilities services). I was taught by someone associated with a department of vocational rehabilitation to create and write specific cover letters to go with resumes. I apply for government grants and loans for schooling and have received them due to my income falling below poverty level.
The advice given to me concerning absences and late work and mobility was keeping in contact with each professor and teacher. In fact, much of my self-advocacy with my doctors, pharmacy techs, and insurance billing was practiced from middle school onward because of the amount of communication I needed to have with my schools and work places. I am skilled in spoken and written forms of communication and it would be appropriate for someone like me to work in this field.
When I started getting the emails due to the coronavirus in my current university inbox, my first reaction was to chuckle as the communications department and chancellor was asking for encouragement for those who needed to be absent from class for longer periods of time. Instructors were actually requested not to ask for medical proof of absences as well as no longer using the dean’s office to submit documentation for medical absence. Students were asked to directly communicate with staff about their condition in order to ensure timely work accomplishment.
Campus culture is not so far removed from societal expectations in the work force as many people think. And this is made clear when one has lived in a disabled body since birth and had to acclimate to school policy instead of the school policy being lenient in fair ways to their existence and participation in the school.
Work and job placement for someone who has had to ask for access and accommodations in education is only exasperated because of the reality of employers paying the disabled person for a job. If the disabled person has the job capabilities just like an able person, corona virus has now ensured that it is doable to work from home while getting paid, online is an alternative for in-class absences, and online is a place where work-related conferences can take place. It will be telling who society has placed more worth and value upon if we go back to limiting access and accommodations after the general population pandemic is over.
So my secondary reaction is anger. I feel angry as my energy and mental and emotional space continues to be taxed because living inside a disabled body is inconvenient in a world designed for the well side of humanity and nothing in between, let alone the ill side.
I thirdly feel a sense of justice in my anger for all people living with disability and hope our reckoning comes because of the environmental changes occurring as coronavirus is affecting humans globally.
And in all reality, blogger and media specialist Jessica Jondle and I posted updated information for adults living with our disease, cystinosis, and the current covid 19 pandemic:
Here is a statement offered by Dr. Richard Simon, pulmonologist and cystinosis researcher at the University of Michigan, to our adult population:
“I have been asked if the respiratory problems that can occur in some adults with cystinosis should place them in the ‘high risk’ category for severe consequences if they become infected with the SARS-CoV-2 virus that causes the disease, COVID-19. Before directly answering this, I believe it worth noting that the majority of adults with cystinosis are kidney transplant recipients and are taking immunosuppressive medications to prevent rejection. This places them into the ‘high risk’ group as defined by the Centers for Disease Control and Prevention (CDC). Therefore, the CDC recommendations for the high risk group should apply to most adults with cystinosis, regardless of their respiratory status (https://www.cdc.gov/coronavirus/2019-ncov/index.html, see ‘Older Adults and Medical Condition’ section. More information about COVID-19 for transplant recipients can be found on the website of the American Society of Transplantation (https://www.myast.org/covid-19-information).
Some adults with cystinosis develop weakness of the muscles that are used for breathing. The lungs themselves in people with cystinosis are usually normal, unless they have another lung condition unrelated to cystinosis. If muscle strength and endurance are normal in someone with cystinosis, then the risk of severe disease from COVID is no further increased above what it would otherwise be. But those who do have significant respiratory muscle weakness will have more problems if they do develop pneumonia as part of COVID-19. A person with pneumonia needs to ventilate more to compensate for the lungs being inefficient at getting oxygen into the body and carbon dioxide out. Furthermore, it takes more work to inflate lungs that have pneumonia because of secretions plugging up some of the airways and the lungs themselves are less complaint. Therefore, people with cystinosis and significant respiratory muscle weakness who develop pneumonia during COVID-19 have a greater risk of needing intensive care unit treatment and breathing assistance with mechanical ventilation than if their respiratory muscles were strong. For this reason, I would consider people with cystinosis and respiratory muscle weakness alone to be in the “high risk” COVID-19 group. All of the CDC recommendations for those at high risk would apply to them.
Determining whether someone has respiratory muscle weakness is best done by formal pulmonary function testing that measures the maximal amount of air someone can blow out after taking in the deepest possible breath. A level below approximately 70-80% of normal would suggest respiratory muscle weakness. Pulmonary function laboratories can measure respiratory muscle strength by additional methods. Another suggestion that respiratory muscles are weak is if someone becomes uncomfortably short of breath after climbing less than 2 flights of stairs, assuming there is no other reason for it. For more information, it is best to consult your healthcare provider who knows most about your cystinosis.”
Richard H. Simon, MD Professor and Associate Chair for Faculty Affairs Department of Internal Medicine Pulmonary and Critical Care Medicine Division University of Michigan Medical School
I feel so lost in my disabilities because no one sees disability. Society thinks if disability rhetoric is erased then the medical costs, treatment options, and mental and emotional health can be regulated and eventually be absent from the world altogether.
This lack of disability language is glaringly apparent in conferences and spaces reserved for those who live in bodies that adapt to living as their bodies allow. Especially in a rare disease space will no one normalize the word “disability” because that word is equated with “less than” be it less intelligence or less accolades on how inspiring one child is over another and which one of us is worth the time, space, and care more disabilities entail.
Caregivers and relatives complain about their offspring or those in their care be they children, teens, or adults. But when these children, teens, and adults offer up their own complaints they are asked to be thankful for the able-bodied caretaker and relative in their life for meeting their needs and cares. Not an equality of discussion on the topics and issues but on respect for authority.
The able-bodied caregivers and relatives have resources, energy, and validation to control the narrative of healthcare.
I just represent the work they do. Not even the poster child, because that would mean I’m beautiful. No, I’m the freak in their circus show–the entertainment as its cute and annoying I have PTSD not another disability to manage and bring awareness and education to as disabilities are more present in humans than not.
I sit and exist in rare disease spaces so those more able-bodied can feel whatever it is they need to feel and then energize themselves to go out and love and excel in a world unconcerned that the able are exploiting the disabled by disregarding what we really need out of our own healthcare.
Look what she can do. Look what Rebekah is: a speaker, a writer, an actor. We created her and without us she is nothing.
Leave this Disabled Body
By Rebekah Palmer
Slip on my shoes
tie the laces tight
and walk a mile
perhaps go even two.
the accolades you will begin to receive
will be because you were born, what body you’re in,
The following interview I conducted was recently published on Patientworthy on January 17, 2020:
Let’s Have A Conversation About What Affects Adults With Rare Disease
Since April 2019, I have been actively seeking out adults (18+) who are willing to publicly share their stories about their lives with rare and chronic diseases. I feel it is essential that the rare community include and listen to those adults living with the illness in their body otherwise legislation for rare community, social structure in rare communities, and the general public knowledge of living rare and chronic will not gain any traction in the future of medicine.
Recently, I spoke with a beautiful adult woman named Kara. She has lived with Acromegaly for the last 23 years of her adult life. Acromegaly is different for every person diagnosed. For Kara, she had a couple precursor diagnosis,Polycystic Ovarian Syndrome and insulin resistance, before she found doctors and specialists who would take the time to diagnose and treat her Acromegaly and Small Fiber Neuropathy.
Kara has a bachelor of arts degree in Graphic Design. She currently lives in Greater New York City with her husband and is active in the arts and advocating to have her life with rare disease validated. She uses #AcromegalyLife and #DisabledArtist on her social media accounts to bring awareness for others to understand what living rare and chronic can look like.
I was struck by our conversation on the phone when Kara related that adults living with rare and chronic conditions tell their story so much in the medical world that it has become exhausting to relay it over and over again in the community support groups. She continued to explain her meaning by saying adults have lived either their childhood and teen years or both (or having received a diagnosis as young adults) repeating their symptoms and diagnosis information. With this background, adults would rather have conversations about the issues affecting them instead of repeating the logistics of what parents, medical providers, caregivers, and advocates have been saying about them for years.
I decided to ask her questions about some issues that need to be heard by the world from the mouths of the adults living with the disease themselves instead of the caregivers:
1. When we spoke on the phone a few days ago, you mentioned “I’m tired of telling my story; I want to have a conversation”. I thought this succinctly and adequately revealed what adults living with any kind of chronic and rare disease feel like, especially if they have been dealing with illness since birth or later teen and college-aged years. Could you tell me more about the conversation that needs to happen in the rare community and in the general public (i.e. the phrase heard by many adults “But you don’t look sick” and “do you work”?)
In the early stages of my chronic illness self doubt crept in. I did not feel confident that I was still creative. That I was still a graphic designer who had a talent and could work with others. I hurt – chronic daily migraine, neck and arm pain shut down thought processes and creative flow. Eventually, I got over it. If I can get over it so should the community at large: I got over my doubt and did not lose my creativity or ability to do the work. If I can get over my insecurity, then employers should be able to get over the fact that I have an illness. I am still creative and professional. They have to get past the “sick” part and offer options to be inclusive.
Not everyone with acromegaly becomes so damaged. I feel like I am made of broken popsicle sticks held together with duct tape. My husband hates this phrase but it has been my truth for a very long time. It is also a very vivid description of what I feel and it helps people understand.
But what about last Month… The weather was crisp and clear, my head was my manageable seven out of 10 on the pain scale (yes, every doctor realizes that it is absurd that I learn to function at a constant seven but an eight forget it)… So on this Tuesday I can paint a flower or two. I can make it into a pattern, put it on mugs, home decor or sell it out right. But people don’t see me. I need to be acknowledged for my talent and my disability.
2. You were quite vulnerable and honest about your life as an adult with an advanced degree who wants to work but is hindered by symptoms and side effects of ongoing illness. You mentioned feelings of being stuck between “I don’t feel well enough” and “I can start but can I finish?” Could you describe how these feelings and physical barriers in Acromegaly dictate your life in paid and unpaid jobs?
A year ago a dear family friend was opening a gym on a shoestring budget. Remember I am valuable, I am talented and I am worthy… But my schedule is wonky due to health. It is my choice to give him my talents and in turn he sends me a little bonus thank you now and again. It covers a blowout (it’s too painful to do my own hair) or a massage or a new pair of pajamas. I live in pajamas or let’s be politically correct, loungewear.
I have a few of these friends that are close to my heart, they deserve professional design to represent their business and I get to be creative.
Creating, be it graphic design, oil painting or watercolor soothes my weary soul. It is therapy.
I have one “real” client. A summer youth program. The youth program hits all the markers, a fresh design, kid oriented (my favorite) and a short and flexible time frame. They get a sweet deal and my soul gets refreshed.
I want to work but I cannot commit. Why? Because yesterday I was able to walk and today I am unable to hold my head up… I don’t know if this will last a day or weeks.
Who would hire me? I would need to work from home. I would need my hours to be random and flexible. As long as I can make the deadline – why should they care? But they do.
The youth program is run by two people. The head of the program knows my honest truth and I appreciate him. He is kind, compassionate, respectful and a rarity, easy to work for.
I want to be heard, seen and respected because of my abilities and my disabilities.
What if Social Security Disability had a program that acknowledged my education and valued my talent? What if companies registered with the program and had deadlines but planned ahead, budgeting in extra time so that I may fulfill their needs? What if they were willing to hire designers for a fair wage, on a per project basis that didn’t affect government disability? Because let’s be honest, a project or two does not make a salary and it does not better my financial situation. But that money does pay for a massage a week or a personal trainer because I need to be watched because of structural damage – and I could go back to Pilates!
Sidenote: pills do not fix me. If insurance paid for Pilates, Rolfing, Massage – I could have a better quality of life. But it doesn’t, so I’m always stuck. And doctors are constantly telling me to do these things… where is the money coming from?! I’m allowed to have a hobby but it doesn’t pay for a massage a week. It’s random at best.
What if I bought all the never covered supplements doctors recommend but I cannot afford each month? What if doing a project to earn the money for these life-changing things wasn’t held against me and threatened my Social Security Disability?
Might I have a better quality of life? Don’t I deserve that! Don’t we all? But none of that exists. I feel cheated. I wish my body worked. I wish I could have a regularly paying job.
I fake being well… When I can. If I can’t, I’m on the couch or desperate to get there. Sometimes my voice betrays me – no matter what I try.
But I’d still like to be offered meaningful projects that fit my abilities as an educated, talented individual. This would work for so many of us with degrees — just looking for a few projects here and there.
Why is it one or the other? Why are you penalized if you are sick? Why are we dismissed? Isn’t being chronically ill punishment enough? Yet everything else is taken away and it doesn’t have to be.
The rare disease and invisible illness communities needs to join as one. Not just each disease in its own bubble. We have similar if not the same experiences.
We, the disabled, the largest minority in the US need to band together. We check off all the boxes, sex, race, religion, economic status… But there is no bridge. There is no support. Why can’t we change this?
I can’t volunteer in real life. I can’t commit to Thursday at 4 PM every week. Who knows how I’m going to feel from day today, if not hour to hour.
Being in a government program means being subjected to one size fits all solution. When in life does one size fit all?
I miss real life encounters. They are hard and exhausting but filled my soul for a bit… And I’ll take it but it’s rarely an option offered me. I wish I had the energy to start something, but I don’t. I like to contribute and be a part of society.
I know we deserve better and I know it is possible. The fact that it is possible but no one with authority and ability ever bothers to try leaves me trapped. No one wants to be trapped, yet I am in so many ways.
Although all these negative things are my truth, I try not to dwell. The other part of my truth is that I look for the joy in life and hold on to hope. It’s better than the alternative.