Cumberworth Crew

Cumberworth Crew

Cumberworth Crew

Please donate to our team for the 2020 Virtual Walk!

We've all had a strange year! Time to do something good!
:)

NOV
1

Message from Randy 2019: Last year, Liana and my daughters, Sonora, Alina, and Joule, participated in the Los Angeles Immune Deficiency Foundation (IDF) Walk for Primary Immunodeficiency (PID). This year, we are both planning and hosting the first Annual IDF walk here in Tucson (www.walkforpi.org/Tucson). Many of you know or have heard somewhat what has happened to our family over the past few years. I know all of us when we see sick or suffering children on TV or elsewhere you can’t help but feel empathy. Like most of you I always felt a strong sense of empathy towards them, but it is completely different when it happens to your own family. The IDF walk for PID we are hosting is dedicated to raising money and awareness to help improve the diagnosis, treatment and quality of life of persons with primary immunodeficiencies. Below I’ve posted a short summary of what happened to our family, how it has changed our lives, and what is currently required for our treatment. In May of 2010 I retired from the Air Force from Misawa AB Japan. It is somewhat strange retiring and moving on especially after living the past 7 and a half years overseas. We chose Tucson because that’s where Liana and I had met and Liana was fortunate enough to be offered a job at the University of Arizona, while I was set to hang up my flight suit to become Mr. Mom for our children who were ages 6, 4, and 2 at the time. Less than 2 weeks after we arrived in Tucson, I had a fairly severe stomach ache that had lasted over a week. I attempted to see a doctor, but it was nearly impossible due to the strange status of being on terminal leave. Finally in the middle of the night it got so bad I woke Liana and went to the ER for how bad the pain was. It turned out that it was my appendix. It had “leaked” instead of the normal appendicitis presentation. During the surgery it was found that the leak had caused gangrene to spread throughout my abdomen and it suddenly it had become life threatening. The treatment required cutting a much larger section than normal and leaving the wound open to be cleaned and packed twice a day with wet wound gel to cover the wound. Liana trained with the medical staff so that she would be able to do this at home for me instead of having to be an inpatient in the hospital. After 3 months it seemed like things were going my way. I was up and around and the last part of the wound would be closed in a week or so. That’s when things really went downhill. Less than a week from when I expected to get the O.K. from the doctor that I was finally healed, I awoke in more pain than I thought humanly possible. Every joint in my body was swollen and inflamed from the little joints in my fingers, hands, toes and feet, to my knees, shoulders and hips, to even my jaw making it difficult to talk and unable to eat solid foods. I went to the ER and they were unable to give a diagnosis. It appeared to be Rheumatoid Arthritis, but it normally didn’t present itself this way. It was as if I had it for years and had let it go completely untreated. They treated me for weeks with high doses of cortical steroids which returned me to feeling normal. But within a week off of the steroids the pain and stiffness began to return. Within the next year I went from needing a cane, to a walker, going as far as needing an electrical wheelchair at times. I spent my weeks with often 2 to 3 doctor appointments going to several different specialists. I went to several specialists and pushed to have my consult sent to hospitals such as the Mayo Clinic. About 6 months after it had started, I began to have different organs fail. I started to require multiple different hormone replacement therapies. Despite seeing dozens of specialists, no one could find a reason. After a year they began listing me as having serum negative Rheumatoid Arthritis, and Lupus. Additionally I started to become routinely ill. I almost always had some sort of a cold, and was encountering pneumonia more than once a month. Finally one of the doctors made a discovery that my antibodies were extremely low. They ran some further tests and determined that I could not make most of the primary antibodies that the immune system uses anymore. I began treatment with high doses of Immunoglobulin. (Immunoglobulin is a blood product where the antibodies are filtered out of blood and blood plasma donations. A quick note it takes approximately 130 plasma donations per year to make the Immunoglobulin one person alone need for treatment. That’s over 500 blood donations required for me and the girls so please give blood and plasma). This helped stabilize me and while I was in severe constant pain all the time, I was able to get out of bed for a couple hours every couple days or so. As you can imagine this took an incredible toll on our family. Not only could I not work, but instead of me taking care of my young kids, often they had to take care of me. It was hardest on Liana who was working and trying to keep up with all my needs. Unfortunately the worst was yet to come. Sonora, Alina, and Joule, like myself, had fairly normal healthy childhoods. But in the fall of 2016, the entire family contracted a severe viral flu. After weeks of being sick, the three girls never fully recovered. They started complaining of headaches, joint pain, and fatigue so bad they at first could no longer go to school, then they could not get up off the couch to do anything, Finally they could more often than not, not get out of bed. We began taking them to specialists. For the next two years we again went through the same set of tests I had faced while they continued to grow sicker and worse. We had suspected that my illness may have a genetic component, because both my sister and my mother had symptoms similar to mine and theirs. You cannot imagine the frustration of having your kids too sick to even get out of bed, the anger you can have towards doctors that telling you it must be psychological because their blood tests aren’t showing anything or to have the doctors simply refuse to run tests that may show something simply because because your insurance company refused to pay for it (even though you are offering to pay for the test out of pocket). Or finally the fear you can have when you receive warnings from the school and the city stating that you could be investigated by Child Protective Services because of the number of absences your children are accumulating without a medical excuse because the doctors are unable to determine a definitive diagnosis. We finally removed them from school on a “Religious Waver” to continue to home-school them ourselves as best possible while continuing to research what was wrong with them. We finally received a break in 2017. Liana had sent a summary of my medical records into a study being conducted by the National Institute of Health. They were interested in the possible genetic link of my disease to my sister’s and my mother’s. After running multiple genetic tests, each more in depth, one of the top Geneticists in the world took me in a research subject. The found there were two possible genes that were likely to be the cause of my disease. One was fairly well known, while the other was completely new. There was only one other case in the world that they knew there was a mutation in this gene, but my mutation was different from theirs. The NIH fortunately picked up the girls as patients as well and have determined that all three contain the same genetic mutation that I have. Additionally we have found a new Pediatrician Immunologist that determined the girls needed immunoglobulin therapy as well. The immunoglobulin therapy helped tremendously. While it in no way was a cure, it did allow the girls to begin to have the energy to some days get out of bed. One of our girls has been able to return to school nearly full time, another part time, and one receives at home tutoring from the school district. While Liana deserves the most credit for getting the girls and myself to where we are today, the girls themselves have shown an inner strength I never thought possible, and they continue to do so day after day. I was devastated to find out that I would be in such severe chronic pain for the rest of my life, but it was indescribable to find out that all three of my kids had the same underlying issue, and worse instead of coming out of remission at the age of 40, they had all developed their symptoms in their early teens, and would be fighting with it for the rest of their lives. Fortunately there is hope. Though grants to organizations like the NIH, and private fundraising such as the IDF walk, fascinating research that seems almost out of science fiction is taking place. Currently at the NIH they have taken the mutated immune gene from my blood, cloned it and graphed it onto the DNA of mice currently under study in a laboratory. While still in the very early stages of testing, the hope is that they will discover how the mutation affects the immune system, and develop new drugs and treatments that may present a new treatment. While I can’t be up more than a hour or two a day, I don’t consider myself completely bedridden anymore. In 2017 a new drug based on genetic sequencing was passed by the FDA. It blocks one of the many channels of inflammation that my disease seems to use primarily, so I am more functional today than I have been in the last 5 years. I want to thank all those that that helped our team from Tucson be the third biggest fundraising team at the National LA IDF walk last year. I want to once again thank those that have donated in the past and apologize for not thanking our donors personally, but my new drug makes me more susceptible to infection and I have been in and out of the hospital quite often and it fell out of my cross check. I want to thank Tucson Unified School District, Catalina Foothills School District, Canyon View Elementary, Esperero Canyon Middle School, and University High School for working with us to provide education to our girls despite their unusual needs. If you live near Tucson, please consider participating in the walk (Dec. 14, www.walkforpi.org/Tucson). If you can’t and wish to donate directly or to one of the teams participating please consider that as well. (www.walkforpi.org/tucson, donate to the Cumberworth Crew). Thanks again to everyone that has offered support to me and my family over this difficult time. It really has mad a difference.

2019 in Vancouver - looking strong!

Message from last year 2019: Dear Family and Friends, Last year, we received incredible support from our friends and family for the Los Angeles IDF walk, making us the third highest fundraising team! Thank you! This year, we are upping our game and organizing a walk in Tucson. Would you consider donating again? We are grateful for any little bit you can give! I have written the story of our experience with PI on our team page: www.walkforpi.org/Tucson/Cumberworth From that page, you can click on my name, or another name on our team, the Cumberworth Crew, to donate, or click https://www.walkforpi.org/tucson/ and donate directly without going through our team page. You can also donate through facebook, twitter, instagram: https://www.facebook.com/walkforPI/ https://twitter.com/TZtheIDFZebra https://www.instagram.com/p/B2ooNQHg4wF/ Thank you again for your support last year. I hope you will consider helping us make our own walk even better. Thank you, Liana, Randy, Sonora, Alina, Joule

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